Color Atlas and Synopsis of Clinical Ophthalmology - Wills Eye Institute Oculoplastics 2 PDF [PDF]

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CO LO R ATLAS & SYNO PSIS OF CLINICAL OPHTHALMOLOGY

W i l l s Ey e I n s t i t u t e

Oc u lo p la s tic s S ECON D EDITION

EDITOR

Robert B. Penne, MD Director and Attending Surgeon, Oculoplastics Service Co-Director, Ocular Cicatricial Pemphigoid Clinic Wills Eye Institute Philadelphia, Pennsylvania

SERIES EDITOR

Christopher J. Rapuano, MD Director and Attending Surgeon, Cornea Service Co-Director, Refractive Surgery Department Wills Eye Institute Professor of Ophthalmology Jefferson Medical College of Thomas Jefferson University Philadelphia, Pennsylvania

CO LO R ATLAS & SYNO PSIS OF CLINICAL OPHTHALMOLOGY

W i l l s Ey e I n s t i t u t e

Oc u lo p la s tic s S ECON D EDITION

Senior Executive Editor: Jona han W. Pine, Jr. Senior Product Managers: Emilie Moyer and Grace Capu o Senior Manufacturing Coordinator: Benjamin Rivera Marketing Manager: Lisa Lawrence Creative Director: Doug Smock Production Services: Ap ara, Inc. © 2012 by LIPPINCOT WILLIAMS & WILKINS, a Wolters Kluwer business First edition, © 2003 e McGraw-Hill Companies, Inc. wo Commerce Square 2001 Market Street Philadelphia, PA 19103 USA LWW.com All righ s reserved. T is book is pro ec ed by copyrigh . No par o his book may be reproduced in any orm by any means, including pho ocopying, or u ilized by any in orma ion s orage and re rieval sys em wi hou writ en permission rom he copyrigh owner, excep or brie quo a ions embodied in cri ical ar icles and reviews. Ma erials appearing in his book prepared by individuals as par o heir of cial du ies as U.S. governmen employees are no covered by he above-men ioned copyrigh . Prin ed in China 978-1-60913-265-1 1-60913-265-3 Library o Congress Ca aloging-in-Publica ion Da a available upon reques Care has been aken o con rm he accuracy o he in orma ion presen ed and o describe generally accep ed prac ices. However, he au hors, edi ors, and publisher are no responsible or errors or omissions or or any consequences rom applica ion o he in orma ion in his book and make no warran y, expressed or implied, wi h respec o he currency, comple eness, or accuracy o he con en s o he publica ion. Applicaion o he in orma ion in a par icular si ua ion remains he pro essional responsibili y o he prac i ioner. T e au hors, edi ors, and publisher have exer ed every e or o ensure ha drug selec ion and dosage se or h in his ex are in accordance wi h curren recommenda ions and prac ice a he ime o publica ion. However, in view o ongoing research, changes in governmen regula ions, and he cons an ow o in orma ion rela ing o drug herapy and drug reac ions, he reader is urged o check he package inser or each drug or any change in indica ions and dosage and or added warnings and precau ions. T is is par icularly impor an when he recommended agen is a new or in requen ly employed drug. Some drugs and medical devices presen ed in he publica ion have Food and Drug Adminis ra ion (FDA) clearance or limi ed use in res ric ed research set ings. I is he responsibili y o he heal h care provider o ascer ain he FDA s a us o each drug or device planned or use in heir clinical prac ice. o purchase addi ional copies o his book, call our cus omer service depar men a (800) 638-3030 or ax orders o (301) 223-2320. In erna ional cus omers should call (301) 223-2300. Visi Lippincot Williams & Wilkins on he In erne : a LWW.com. Lippincot Williams & Wilkins cus omer service represen a ives are available rom 8:30 am o 6 pm, ES . 10 9 8 7 6 5 4 3 2 1

To Devany, Daniel, and Mara— the source of pride and balance in my life

Abou he Series he beau y o he a las/ synopsis concep is he power ul combina ion o illus ra ive pho ographs and a summary approach o he ex . Oph halmology is a very visual discipline ha lends i sel nicely o clinical pho ographs. While he seven oph halmic subspecial ies in his series—cornea, re ina, glaucoma, oculoplas ics, neurooph halmology, uvei is, and pedia rics—employ varying levels o visual recogni ion, a rela ively s andard orma or he ex is used or all volumes.

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T e goal o he series is o provide an up- oda e clinical overview o he major areas o oph halmology or s uden s, residen s, and prac i ioners in all he heal h care pro essions. T e abundance o large, excellen -quali y phoographs and concise, ou line- orm ex will help achieve ha objec ive. Chris opher J. Rapuano, MD Series Editor

Pre ace his ex is aimed a assis ing physicians (oph halmologis s and nonoph halmologis s) in recognizing he mos common oculoplas ic condi ions. Many oculoplas ic condi ions can be diagnosed on simple visual examina ion, which makes his a las an ideal resource o have in emergency depar men s and in he of ce. I provides a solid basis o pho ographic and descrip ive in orma ion o diagnose oculoplas ic

condi ions. Once hese condi ions are recognized, he ex describes o her es s ha may be needed and he di eren ial diagnoses ha should be considered. T e managemen op ions or hese condi ions are also described. Rober B. Penne, MD Editor

vii

Acknowledgmen s

S

pecial hank o my colleagues who provided assis ance: Edward Bedrossian, MD; Jurij Bilyk, MD; Richard Her le, MD; Ka e Lane, MD; and Mary A S e anyszyn, MD.

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Con en s Abou he Series vi Pre ace vii Acknowledgmen s viii

SECT IO N I: EYELIDS Ch a pt er 1 Benign Eyelid Lesions 2 Papilloma 2 Seborrheic Kera osis 4 Cu aneous Horn 6 Epidermal Inclusion Cys 8 Molluscum Con agiosum 10 Xan helasma 12 Syringoma 14 Apocrine Hydrocys oma 16 richoepi helioma 18 Nevi (Nevocellular Nevi) 20 Hemangioma o he Eyelid (Cherry Angioma)

22

Ch a pt er 2 Eyelid Inf ammation 24 Chalazion 24 Hordeolum 26 Floppy Eyelid Syndrome 28

Ch a pt er 3 Eyelid Neoplasms 30 Kera oacan homa 30 Ac inic Kera osis 32 Len igo Maligna 34 Basal Cell Carcinoma 36 Squamous Cell Carcinoma 40 Sebaceous Adenocarcinoma 42 Malignan Melanoma 44 Kaposi’s Sarcoma 46

Ch a pt er 4 Eyelid rauma 48 Marginal Eyelid Lacera ion 48 Canalicular Eyelid Lacera ion 50 Dog Bi es 52 Eyelid Burns 54

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x

CO NT ENTS

Ch a pt er 5 Eyelid Malpositions 56 En ropion 56 Acu e Spas ic En ropion 56 Involu ional En ropion 58 Cica ricial En ropion 60 Ec ropion 62 Involu ional Ec ropion 62 Paraly ic Ec ropion 64 Cica ricial Ec ropion 66 Mechanical Ec ropion 68 Symblepharon 70 richiasis 72 P osis 74 Congeni al Myogenic P osis 74 Acquired Myogenic P osis 76 Aponeuro ic P osis 78 Neurogenic P osis 80 T ird Nerve Palsy 80 Myas henia Gravis 82 Marcus Gunn Jaw-Winking Syndrome 84 Horner’s Syndrome 88 Mechanical P osis 90 rauma ic P osis 92 Pseudop osis 94 Brow P osis 96 Derma ochalasis 98 Blepharochalasis 100 Eyelid Re rac ion 102 Eyelid Dyskinesis 104 Benign Essen ial Blepharospasm 104 Hemi acial Spasm 106

Ch a pt er 6 Congenital Eyelid Anomalies 108 Blepharophimosis 108 Epican hus 110 Epiblepharon 112 Congeni al En ropion 114 Congeni al Coloboma 116 Congeni al Dis ichiasis 118 Ankyloblepharon 120

Ch a pt er 7 Miscellaneous Eyelid Conditions 122 Ocular Cica ricial Pemphigoid 122

CO NTENTS

SECT T IO N II: L LACRIMAL ACR R I M AL LA APPARAT PP PAR R AT T US S Ch a pt er 8 Lacrimal Obstructions 126 Congeni al Obs ruc ions 126 Congeni al Nasolacrimal Duc Obs ruc ion 126 Dacryocys ocele 128 Lacrimal Fis ula 129 Acquired Obs ruc ions 130 Acquired Nasolacrimal Duc Obs ruc ion 130 Canalicular Obs ruc ion 132

Ch a pt er 9 Lacrimal In ections 134 Dacryocys i is 134 Canaliculi is 137

Ch a pt er 10 Lacrimal Sac umors 140

SECT S ECT T IO N III: III I : T H E O R RBIT BIT T Ch a pt er 11 Orbital In ections 142 Orbi al Celluli is 142 Orbi al Abscess 146 Phycomycosis (Mucormycosis) Aspergillosis 152

150

Ch a pt er 12 Orbital Inf ammation 154 T yroid-Rela ed Oph halmopa hy 154 Idiopa hic Orbi al In amma ion (Orbi al Pseudo umor) Sarcoidosis 164 Wegener’s Granuloma osis 168

Ch a pt er 13 Congenital Orbital Anomalies 170 Microph halmos 170

Ch a pt er 14 Orbital Neoplasms 174 Congeni al Orbi al umors 174 Dermoid Cys s 174 Lipodermoids 178 Vascular Orbi al umors 180 Capillary Hemangiomas 180 Cavernous Hemangiomas 184 Lymphangiomas 188

160

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xii

CO NTENTS

Hemangiopericy oma 192 Orbi al Varices 196 Ar eriovenous Mal orma ions 200 Neural umors 204 Op ic Nerve Gliomas 204 Neuro bromas 208 Meningiomas 210 Schwannomas 218 Mesenchymal umors 222 Rhabdomyosarcoma 222 Fibrous His iocy oma 226 Lymphoproli era ive umors 228 Lymphoid Hyperplasia and Lymphomas 228 Plasmacy oma 232 His iocy ic Disorders 236 Lacrimal Gland umors 240 Epi helial umors o he Lacrimal Gland 240 Miscellaneous Orbi al umors 246 Secondary Orbi al umors 246 Me as a ic Orbi al umors 252

Ch a pt er 15 Orbital rauma 258 Orbi al Frac ures 258 Orbi al Floor Frac ure 258 Medial Wall Frac ure 262 Orbi al Roo Frac ure 266 Zygoma ic Frac ure 268 Miscellaneous rauma 272 Orbi al Hemorrhage 272 Orbi al Foreign Bodies 276 Mucocele 282

Index 285

CO LO R ATLAS & SYNO PSIS OF CLINICAL OPHTHALMOLOGY

W i l l s Ey e I n s t i t u t e

Oc u lo p la s tic s S ECON D EDITION

C H AP T ER

Benign Eyelid Lesions PAPILLOMA

A

papilloma is a common benign, of en asymp oma ic, skin lesion ha occurs mos commonly in he in er riginous areas (axillae, in ramammary, and groin) bu is also commonly seen on he neck and eyelids. Papillomas are of en numerous on he eyelids when presen , and he number ends o increase wi h age. Synonyms: skin ag, acrochordon.

Epidemiology and Etiology Age: More common in middle-aged and elderly people Gender: More common in emales E iology: Unknown History Mos commonly asymp oma ic bu may become ender af er rauma. Wi h ime, lesions may become crus ed or hemorrhagic. Examination

Lesions are sof ; skin-colored, an, or brown; round or oval, peduncula ed 2

papillomas ( Fig. 1-1A & B). T e lesion is of en cons ric ed a he base. Size ranges rom less han 1 mm o 10 mm. Special Considerations May grow or become more numerous during pregnancy More common in obese pa ien s Dif erential Diagnosis Peduncula ed seborrheic kera osis Dermal nevus Soli ary neuro broma Molluscum con agiosum Conjunc ival papillomas ( Fig. 1-1C) can appear on he eyelid margin bu have a di eren appearance and he base o he lesion is rom he conjunc ival sur ace. Treatment Excision by simply snipping he lesion a he base Prognosis Excellen . Pa ien s may develop o her papillomas wi h ime.

Papilloma

3

A

B

C FIGURE 1-1. Papilloma. A. Multiple small papillomas o the upper eyelid. B. Larger papilloma o the right lower eyelid. Conjunctival papilloma. C. Papillomatous lesions may grow rom the conjunctival sur ace and protrude onto the eyelid margin. T ese papillomas are f esh colored and more riable than cutaneous papillomas. Conjunctival papillomas can be associated with a viral origin.

4

1 BENIGN EYELID LESIO NS

SEBORRHEIC KERATOSIS

T

he seborrheic kera osis is one o he mos common benign epi helial umors. T ese lesions are heredi ary, are rarely seen be ore he age o 30 years, and will con inue o increase over a li e ime. Some pa ien s will only have a ew, and o hers can have hundreds over heir body. Epidemiology and Etiology Age: More common as pa ien s age. Rare be ore age 30 years. Gender: More common and more ex ensive in males E iology: Unknown Inheri ance: Probably au osomal dominan

Lesions vary in size rom 1 mm o 6 cm. Special Considerations Mos common on lower lids Dif erential Diagnosis Pigmen ed ac inic kera osis Verruca vulgaris Pigmen ed basal cell carcinoma Pathophysiology Epidermal lesion. Benign proli era ion o kera inocy es, melanocy es, and orma ion o horn cys s.

History Lesions are of en presen or mon hs o years and are of en asymp oma ic. T ey are mos common on he ace, runk, and upper ex remi ies.

Treatment Excision-ligh elec rocau ery or cryo herapy will permi he lesion o be easily rubbed or curet ed o . T e underlying base can hen be re rea ed wi h cau ery.

Examination Lesion s ar s as a a , ligh an lesion. Wi h ime, he lesion becomes more pigmen ed and will become eleva ed ( Fig. 1-2A). As hey age, he lesion’s sur ace becomes “war y” ( Fig. 1-2B).

Prognosis Excellen wi h rare recurrence Pa ien s will of en have many lesions and will develop addi ional lesions over ime.

Seborrheic Keratosis

5

A

B FIGURE 1-2. Seborrheic keratosis (A&B). Seborrheic keratosis are common lesions o the eyelids. T ey tend to get darker as they have been present longer, as seen in B.

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1 BENIGN EYELID LESIO NS

CUTANEOUS HORN

C

u aneous horn is a clinically descrip ive erm or lesions wi h exuberan hyperkera osis. T e e iology o his hyperkera osis can be variable and biopsy o de ermine he cause is required.

Epidemiology and Etiology Age: Older adul s Gender: Equal in males and emales E iology: Hyperkera osis is associa ed wi h a varie y o underlying lesions History Lesion may grow slowly or rapidly. Examination Raised lesion, of en on a s alk, usually whi e in color. T e sur ace is hyperkera o ic ( Fig. 1-3).

Special Considerations Biopsy o hese lesions is required o rule ou malignan lesion a he base o he lesion such as basal cell carcinoma or squamous cell carcinoma. Dif erential Diagnosis T is is a descrip ive erm and no a pa hologic, diagnos ic erm. T e base o his lesion may be a seborrheic kera osis, verruca vulgaris, basal cell carcinoma, or squamous cell carcinoma. Laboratory Tests Pa hologic evalua ion Treatment Excisional biopsy wi h pa hologic evalua ion Prognosis Good

Cutaneous Horn

7

A

B FIGURE 1-3. Cutaneous horn. A cutaneous horn has a hard, rough sur ace that is white in color. A. T is lesion is less pointed, but some end in a point, giving them their name o cutaneous “horn.” B. Cutaneous horn o the lower eyelid.

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1 BENIGN EYELID LESIO NS

EPIDERMAL INCLUSION CYST

C

ommon whi e o yellow cys seen around he eyes and elsewhere on he ace. Easily rea ed wi h excision.

Epidemiology and Etiology Age: Any Gender: Equal in males and emales E iology: Arises spon aneously rom he in undibulum o he hair ollicle or ollowing rauma ic implan a ion o epidermal issue in o he dermis. History May have his ory o rauma o he area Lesions usually grow slowly or a period o ime and hen remain s able. Examination Smoo h, round, eleva ed cys

T e underlying cys is whi e and can of en be visualized hrough he hin eyelid skin ( Fig. 1-4). Special Considerations T ese cys s may become secondarily in ec ed and cause a celluli is. Dif erential Diagnosis Molluscum con agiosum Chalazion Syringoma Treatment Excision; at emp should be made o ei her excise he en ire cys wall or i lef a he base, i should be des royed wi h cau ery. Prognosis Excellen . Recurrence is rare.

Epidermal Inclusion Cyst

9

A

B FIGURE 1-4. Inclusion cyst. A. Inclusion cyst o the le upper eyelid. B. A smaller cyst o the le lower lid. Patients with inclusion cysts on the eyelids o en seek treatment be ore they become very large.

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1 BENIGN EYELID LESIO NS

MOLLUSCUM CONTAGIOSUM

M

olluscum con agiosum is a sel -limi ed viral in ec ion charac erized by skincolored papules ha are of en umbilica ed a he cen er. In immunocompromised individuals, his may no be sel -limi ed and can lead o large cosme ically dis guring lesions. I hese lesions are loca ed on he eyelid margin, hey may cause a ollicular conjunc ivi is. Epidemiology and Etiology Age: Children and young adul s Gender: More common in males han emales E iology: Viral lesions spread by skin- oskin con ac History Spon aneously occurring lesions Known con ac wi h o her person wi h lesions is no usual Examination Single or mul iple small 1- o 2-mm papules ( Fig. 1-5). Rarely, hese lesions can become larger. T ey are pearly whi e or skin colored wi h a cen ral kera in plug ha gives hem heir cen ral umbilica ion.

Special Considerations I hese lesions are loca ed on he eyelid margin, hey may cause a mild o severe, chronic ollicular conjunc ivi is. In immunocompromised pa ien s, his viral in ec ion may no be sel -limi ed and can lead o large, cosme ically dis guring lesions, especially on he ace. Dif erential Diagnosis Epidermal inclusion cys Syringoma Kera oacan homa Laboratory Tests Direc microscopy o he kera in plug wi h Giemsa s ain shows “molluscum bodies.” Treatment T ese lesions will regress spon aneously over ime excep in immunocompromised pa ien s. I removal is desired, small lesions can be rozen or he core can be rea ed wi h elec rodesicca ion. Curet age or direc excision is also e ec ive. Prognosis Good in heal hy people T e chance o in ec ing o her people is low when he lesions are presen bu in ec ed pa ien s should avoid skin- o-skin con ac .

Molluscum Contagiosum

11

A

B FIGURE 1-5. Molluscum contagiosum. A. T ere are three lesions on the upper eyelid. I the lesions are on the eyelid margin, the eye itsel may be injected with a ollicular conjunctivitis. T is patient also had similar lesions on her leg. B. Multiple lesions o the eyelid margin. T ere was a mild ollicular reaction in the in erior ornix. (Courtesy o Jurij Bilyk, MD.)

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1 BENIGN EYELID LESIO NS

XANTHELASMA

X

an helasma are yellowish plaques ha occur medially on he upper or lower lids and are classic in appearance. T ey end o enlarge wi h ime and may or may no be associa ed wi h hyperlipidemia. Synonyms: xan homa.

Special Considerations I LDL is eleva ed in he lipid pro le, i is a sign o a amilial lipopro ein disorder. Dif erential Diagnosis I he xan helasmas are presen bila erally, no o her lesions look like his. Early, a xan helasma can look like an inclusion cys or syringoma.

Epidemiology and Etiology Age: More han 50 years o age. I younger, mus consider a amilial lipoproein disorder. Gender: Ei her E iology: May or may no be associa ed wi h hyperlipopro einemia

Laboratory Tests Labora ory evalua ion o lipid pro le

History T e lesions are no ed or mon hs o years wi h slow enlargemen .

Treatment Excision is mos common. Elec rodesicca ion, laser, and applica ion o richloroace ic acid are o her rea men s.

Examination Sof , yellow-orange plaques loca ed medially on he upper and/ or lower eyelids ( Fig. 1-6)

Pathophysiology Macrophages con aining drople s o lipids orm xan homa cells. T ese xan homa cells hen accumula e orming he xan helasma.

Prognosis Good, bu wi h ime addi ional deposi ion may occur and he lesions reappear.

Xanthelasma

13

A

B FIGURE 1-6. Xanthelasma. T ese lesions are in the classic area o the upper eyelids. T ey are still relatively small but with time, the lipid deposition will continue and they will enlarge. Less commonly, they can occur in a similar position on the lower eyelids.

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1 BENIGN EYELID LESIO NS

SYRINGOMA

S

yringoma presen as mul iple lesions on he lower lids o women. T e onse is usually insidious. Pa ien s ypically presen wi h cosme ic concerns because o he numerous “bumps” on he lower eyelids. T e challenge can be excision o he large number o lesions presen wi hou causing scarring or an ec ropion.

Lesions occur commonly on he lower eyelids bu may occur elsewhere on he ace, axillae, umbilicus, upper ches , and vulva. Dif erential Diagnosis Very ew o her lesions look similar or presen wi h numerous lesions on he lower eyelids. A single lesion can look like an inclusion cys , basal cell carcinoma, or richoepi helioma.

Epidemiology and Etiology Age: Begins in puber y Gender: Occurs in women and may be amilial E iology: An adenoma o he in raepidermal eccrine duc s

Pathophysiology Benign adenoma o he in raepi helial eccrine duc s Pa hology shows many small duc s in he dermis wi h comma-like ails wi h he appearance o adpoles.

History Lesions no ed on he lower eyelids wi h insidious onse . Lesions may be presen elsewhere on he ace, axillae, umbilicus, upper ches , and vulva.

Treatment Pa ien s of en reques removal on a cosme ic basis. Removal is by elec rosurgery or direc excision.

Examination Lesions are 1 o 2 mm, skin colored or yellowish, and usually appear in mul iples ( Fig. 1-7).

Prognosis A large number on he ace can be di cul o remove. Addi ional lesions may grow af er excision.

Syringoma

15

FIGURE 1-7. Syringoma. Multiple lesions in the classic area o the lower eyelids. T ere can be just a ew lesions or even more than in this patient.

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1 BENIGN EYELID LESIO NS

APO CRINE HYDRO CYSTOMA

A

pocrine hydrocys oma is a very common lesion arising along he eyelid margin. I is a clear, cys ic lesion ha ransillumina es, al hough he overlying skin may give i a bluish color. Epidemiology and Etiology Age: Adul s Gender: Equal E iology: Cys orma ion rom he glands o Moll along he eyelid margin History Cys no ed and may slowly enlarge Examination Cys ic lesion near or on he eyelid margin ( Fig. 1-8).

Lesions are ranslucen or bluish and ransillumina e. T ere may be mul iple lesions. Dif erential Diagnosis Cys ic basal cell carcinoma Eccrine hydrocys oma (re en ion cys o eccrine glands) Pathophysiology T is lesion is an adenoma o he secre ory cells o Moll and no a re en ion cys . Treatment Marsupializa ion o he cys may be adequa e or super icial lesions, bu deeper lesions require comple e cys wall excision. Prognosis Excellen . Rare recurrence af er excision.

Apocrine Hydrocystoma

17

A

B FIGURE 1-8. Apocrine hydrocystoma. A. T is lesion on the upper eyelid transilluminates with the slit beam. On excision there will be a gush o clear f uid. B. Multiple lesions. Lesions are o en smaller than these and are di cult to photograph.

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1 BENIGN EYELID LESIO NS

TRICHOEPITHELIOMA

T

richoepi helioma is a benign leshcolored papule ha arises rom an immaure hair ollicle. I can occur on he eyelid margin bu more commonly elsewhere on he ace, scalp, neck, and upper runk. Epidemiology and Etiology Age: Firs appears a puber y Gender: More common in males E iology: Benign appendage umor wi h hair di eren ia ion History Lesions o he eyelid and orehead appear a puber y and can slowly increase in size and number.

Examination Small pink or skin-colored papules ha can increase in size and become qui e large ( Fig. 1-9). Special Considerations May be con used wi h a basal cell carcinoma, especially i i appears as a soli ary umor. Dif erential Diagnosis Epidermal inclusion cys Basal cell carcinoma Syringoma Treatment Excision wi h pa hologic evalua ion Prognosis Excellen

Trichoepithelioma

19

FIGURE 1-9. Trichoepithelioma. T ese pink or skin-colored lesions can occur on the skin or eyelid margin. T ey can enlarge and be con used with a basal cell carcinoma. (From Fitzpatrick B, Johnson R , Wol K, et al. Color Atlas and Synopsis of Clinical Dermatology, 4th ed. New York, McGraw-Hill, 2001.)

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1 BENIGN EYELID LESIO NS

NEVI (NEVOCELLULAR NEVI)

N

evocellular nevi are small (less han 1 cm), circumscribed, acquired pigmen ed lesions ha are made up o melanocy ic nevus cells loca ed in he epidermis and dermis bu rarely deeper.

Epidemiology and Etiology Age: Appear in early childhood and reach a maximum size in young adul hood. T ese lesions gradually involu e and disappear by age 60 years. T e excep ion is he dermal nevus, which does no involu e. Gender: Equal E iology: Groups o melanocy ic nevus cells loca ed in he epidermis, dermis, or, rarely, in he subcu aneous issue. History Pigmen ed lesion ha is s able or involu ing Lesions are asymp oma ic. Examination Nevi can be grouped as ollows ( Fig. 1-10): Junctional nevi: Round or oval, a , or very sligh ly raised lesion, less han 1 cm in diame er. an or brown in color wi h smoo h regular borders. Compound nevi: Round, eleva ed, dome-shaped lesion wi h smoo h or papilloma ous sur ace. Dark brown in color bu

becomes mot led as he lesion evolves in o a dermal nevus; of en has hairs growing ou o he lesion. Dermal nevi: Round, dome-shaped, eleva ed nodule, skin-colored, an, or brown wi h elangiec asias. T ese do no disappear wi h age and may become more peduncula ed. Special Considerations Any enlarging lesions, hose changing color, or becoming irri a ed in any way af er age 20 years need o be biopsied o rule ou malignan change. Dif erential Diagnosis Seborrheic kera osis Malignan melanoma Derma o broma Basal cell carcinoma Laboratory Tests His ologic examina ion i biopsied Treatment Observa ion, unless he lesion changes color, i s borders become irregular, or he lesion begins o i ch, hur , or bleed. Any o hese are indica ions or excisional biopsy wi h his ologic evalua ion. Prognosis Rare chance o malignan rans orma ion

Nevi (Nevocellular Nevi)

21

A

B FIGURE 1-10. Nevi. A.T is small nevus o the lower eyelid is amelanotic except or a ew pigmented spots. Nevi on the eyelid margin will o en mold against the eyeball, as in this picture, but cause no discom ort or corneal changes. B. A split nevus where nevi cells were split congenitally as the eyelid ssure ormed. T is nevus is very dark and shows the variation that can occur in the color o these lesions.

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1 BENIGN EYELID LESIO NS

HEMANGIOMA OF THE EYELID (CHERRY ANGIOMA)

H

emangiomas o he eyelid are raised, red, benign lesions ha appear on he eyelids in adul hood and can increase in size bu usually remain less han 3 o 5 mm. Epidemiology and Etiology Age: Adul hood Gender: Equal E iology: Unknown History Usually appear spon aneously and can increase in size over a shor ime. Pa ien s may have o her similar lesions elsewhere on heir body.

Examination Raised, brigh red, blood- lled lesions ha can occur anywhere on he body ( Fig. 1-11A) Lesions may be single or mul iple. Dif erential Diagnosis Pyogenic granuloma ( Fig. 1-11B) Melanoma Laboratory Tests Pa hologic evalua ion af er excision Treatment Excision usually or cosme ic reasons, less commonly o have he lesion evalua ed pa hologically. Prognosis Excellen

Hemangioma of the Eyelid (Cherry Angioma)

23

A

B FIGURE 1-11. Eyelid hemangioma. A. Very red, blood- lled lesion that may be just slightly raised or very elevated as in this picture. On excision, there is usually a small gush o blood, but these lesions usually do not bleed excessively. arely, these can bleed actively so the surgeon must be prepared. Pyogenic granuloma. B. A pyogenic granuloma can look similar to a hemangioma, but it usually is solid, not blood lled, and o en somewhat papillomatous.

C H AP T ER

Eyelid Inf amma ion CH ALAZION

A

ype o ocal inf amma ion o he eyelid; i is a common lesion o he eyelid. T e mos common cause is blockage o a meibomian gland o he eyelid. Chalazia can presen wi h an inf amed, ender, red eyelid or as a discre e non ender lump in he eyelid. Epidemiology and Etiology Age: Any Gender: Equal E iology: Focal inf amma ion o he eyelid resul ing rom he obs ruc ion o he meibomian glands History O en presen wi h acu e onse o ocal eyelid inf amma ion. T e inf amma ion will resolve bu may urn in o a chronic cys -like lesion. T e onse may be more insidious wi h appearance o he cys -like lesion wi h minimal inf amma ion. Examination In he acu e process, he eyelid may be di usely in lamed wi h pain ocally over 24

he involved area ( Fig. 2-1A). here may be poin ing over he blocked meibomian gland. As he inf amma ion resolves, he resul ing lesion is a rm mass in he arsal pla e wi h or wi hou residual inf amma ion ( Fig. 2-1B). Special Considerations Chronic, nonresolving chalazion needs o be biopsied o rule ou a carcinoma. Dif erential Diagnosis Sebaceous adenocarcinoma Squamous cell carcinoma Basal cell carcinoma Pathophysiology Blockage o he eyelid glands resul s in release o he gland con en s in o he arsus and eyelid, resul ing in an inf amma ory process. he in lamma ory process is hen walled o wi h ime, resul ing in he cys -like lesion. T e exac role o bac eria in his process is unclear.

Chalazion

Treatment When in he inf amma ory phase, ini ial rea men is warm compresses and s eroid an ibio ic drops or oin men . As he lesion becomes cys ic, rea men is hen excision via a conjunc iva incision. Injec ion o s eroid in o he lesion may also be e ec ive. S eroid injec ions need

25

o be used cau iously in pa ien s wi h darkly pigmen ed skin, as hey can cause depigmen a ion. Prognosis Good T ese lesions can be mul iple and are rarely resis an o rea men .

A

B FIGURE 2-1. Chalazion. A. A f rm, ormed lump o the le lower eyelid. T ere is still some in ammation o the chalazion. T e eye is red rom blepharoconjunctivitis, which o en is part o a chalazion. Most o the time, the eye is white and quiet. B. A chronic chalazion o the le upper eyelid with some crusting over the chalazion rom external drainage.

26

2 EYELID INFLAMMATIO N

HORDEOLUM

A

n acu e in ec ion o he glands o Zeis (ex ernal hordeolum) or meibomian glands (in ernal hordeolum). I presen s as a red, inf amed, ender eyelid. In prac ice, he erms chalazion, hordeolum, and stye are o en used in erchangeably (and incorrec ly). Synonym: s ye. Epidemiology and Etiology Age: Any Gender: Equal E iology: Acu e bac erial in ec ion o he glands o Zeis or he meibomian glands History Sudden onse o ocal inf amma ion o he eyelid cen ered around a gland o he eyelid

Examination Red, swollen, ender eyelid, o en wi h a ocal area o in ec ion around a gland o he eyelid ( Fig. 2-2) Dif erential Diagnosis Presep al celluli is Eyelid abscess Pathophysiology Eyelid gland becomes in ec ed probably associa ed wi h blockage o he gland. Treatment Warm compresses and opical s eroid/ an ibio ic drops or oin men Rarely, his can evolve in o an abscess, which needs drainage, or a celluli is ha requires sys emic an ibio ics. Prognosis Excellen

Hordeolum

27

A

B FIGURE 2-2. Hordeolum. A. Acute in ammation o the le lower eyelid caused by blockage and in ection/ in ammation o a meibomian gland. T is lesion may resolve as the acute in ammation resolves or evolve into a chalazion. B. Blockage with in ection/ in ammation o the glands o Zeis is involved in this eyelid lesion. T is lesion is on the external eyelid in the area o the eyelash ollicles. Hordeola usually resolve without sequelae but can sometimes become chronic and take many weeks to resolve.

28

2 EYELID INFLAMMATIO N

FLOPPY EYELID SYNDROME

F

loppy eyelid syndrome is seen in obese pa ien s, many o whom have sleep apnea. T e eyelids become very loose and f oppy ei her as a primary process or secondary o chronic rubbing o he eyelids a nigh . T is syndrome mus be considered as a cause or chronic, papillary conjunc ivi is.

Epidemiology and Etiology Age: Adul s Gender: Males more commonly a ec ed E iology: Unknown. T e eyelid laxi y and loss o s ruc ure may be rela ed o chronic mechanical eyelid rubbing or may be due o some inna e abnormali y o he pa ien ’s eyelids. History Pa ien presen s wi h chronic papillary conjunc ivi is ha is usually bila eral and may give he his ory o his eyelids spon aneously ever ing a nigh . T e pa ien may complain o chronic nonspeci c irri a ion. T e symp oms are o en worse on he side he pa ien sleeps on. Examination Eyelids are f accid and easily ever ed ( Fig. 2-3).

T ere is chronic papillary conjunc ivi is wi h a kera i is o en wi h di use super cial punc a e kera i is. ypically, he palpebral conjunc iva has a velve y appearance. T ere is a high incidence o obesi y in hese pa ien s. Special Considerations T ere is a signi can incidence o sleep apnea in pa ien s wi h f oppy eyelid syndrome. All pa ien s need o have sleep s udies. Dif erential Diagnosis O her orms o conjunc ivi is in a pa ien wi h eyelid laxi y Pathophysiology T ere is loss o elas in bers in he arsus, bu he cause remains specula ive. Treatment Pa ching or a shield over he eye can be at emp ed bu is usually no help ul in he long erm. Horizon al eyelid igh ening is usually required. Prognosis T e laxi y will recur wi h ime. Horizon al igh ening will relieve sympoms or a ime.

Floppy Eyelid Syndrome

29

A

B

C FIGURE 2-3. Floppy eyelid syndrome. A. Patient with mild ptosis but complains o chronic irritation o the eyes. His eyes are white but he has moderate corneal superf cial punctate keratitis. B. Upper eyelids are easily everted and the undersides o the eyelids are red with a di use papillary reaction. C. T e eyelids are very loose and, once everted, will o en remain everted even with blinking.

C H AP T ER

Eyelid Neoplasms KERATOACANTHOMA

K

eratoacanthoma presents as an isolated lesion on the ace with a very unique appearance. he lesion is dome shaped with a central keratin- illed crater. It grows rapidly over weeks and may undergo spontaneous regression over months. Once considered benign, most pathologists now consider this a low-grade squamous carcinoma. Epidemiology and Etiology Age: Most of en older than 50 years o age; rare younger than 20 Gender: More common in males than emales by a ratio o 2 to 1 Etiology: Unknown; ultraviolet radiation and chemical carcinogens may have a causative role. History Rapid onset o growth over a ew weeks T e lesion is of en asymptomatic except or cosmetic changes. T ere may be occasional tenderness. 30

Examination Single, dome-shaped nodule with a central keratotic plug T e lesion is rm and is slightly red to light brown in color ( Fig. 3-1). Special Considerations Once considered a benign lesion, there may still be some con usion in the literature about whether this is a squamous carcinoma. T e lesion must be treated as a low-grade squamous carcinoma. Dif erential Diagnosis Basal cell carcinoma Hyperkeratotic actinic keratosis Squamous carcinoma Laboratory Tests Histopathology o the excised lesion Should be excised with rozen section guidance or with Mohs surgery Treatment Excision with pathologic evaluation T ese lesions will sometimes spontaneously regress over a ew months to a year.

Keratoacanthoma

T e need to rule out a squamous cell carcinoma and the cosmetic appearance should lead to biopsy and excision be ore spontaneous regression, especially around the eyelids.

31

Prognosis Good Depending on the size o the lesion, reconstruction o the de ect may leave some eyelid changes.

A

B FIGURE 3-1. Keratoacanthoma. A. Lesion o the lef upper eyelid that grew over 2 to 3 weeks. It was excised without recurrence. B. Large lesion o the lef lower eyelid in a 40-year-old patient. T e appearance could be that o a squamous cell carcinoma; however, the history o growth over 4 weeks and the patient’s younger age point to a keratoacanthoma. T is lesion was excised without recurrence.

32

3 EYELID NEO PLASMS

ACTINIC KERATOSIS

T

hese lesions may be single or multiple on chronically sun-exposed skin. T ey appear as dry, rough, scaly lesions that are stable but can rarely disappear spontaneously. Synonym: solar keratosis. Epidemiology and Etiology Age: Older than age 40; rare younger than 30 years Gender: Higher incidence in males Etiology: Sun exposure over time in a airskinned white population results in actinic keratosis. History Extensive sun exposure in youth Lesions present or months. Examination Rough, slightly elevated, skin-colored or light brown lesions with hyperkeratotic scale ( Fig. 3-2) Special Considerations It is estimated that one squamous cell carcinoma will develop per 1000 actinic keratoses. Dif erential Diagnosis Squamous cell carcinoma Discoid lupus

Laboratory Tests Pathologic evaluation i biopsied Pathophysiology Repeated solar exposure results in damage to the keratinocytes by the cumulative e ects o ultraviolet radiation. Treatment Prevention through early and li elong use o sunscreen Excise nodular lesions and submit or pathologic evaluation. Most at lesions respond to liquid nitrogen or topical application o 5% 5- uorouracil cream over a ew days to weeks. opical imiquimod cream has also been approved or treatment o actinic keratosis. T ese three treatments (liquid nitrogen, 5- uorouracil, and imiquimod) must be used with caution around the eye and should be avoided in lesions at or near the lid margin. Prognosis Some actinic keratoses may disappear spontaneously but others remain or years unless treated. Incidence o squamous cell carcinoma developing in these lesions is unknown but has been estimated to be one squamous cell carcinoma in every 1000 actinic keratoses.

Actinic Keratosis

33

A

B FIGURE 3-2. Actinic keratosis. A. Multiple actinic keratoses on the cheek and brow with signs o chronic sun damage. B. Lesion involving the lower eyelid. (Courtesy o Jurij Bilyk, MD.)

34

3 EYELID NEO PLASMS

LENTIGO MALIGNA

L

entigo maligna is a at intraepidermal neoplasm and the precursor lesion o lentigo maligna melanoma. T e lesion has striking variations o brown and black ( Fig. 3-3), of en described as a “stain.” Epidemiology and Etiology Age: Median age is 65 years Gender: Equal incidence in males and emales Etiology: Sun exposure is a de nite actor.

Of en appears like a dark “stain” on the skin Special Considerations T is is a premalignant lesion and should be excised because o the chance o development into a lentigo maligna melanoma. Dif erential Diagnosis Seborrheic keratosis Actinic keratosis Malignant melanoma Laboratory Tests Histopathologic evaluation

History History is usually not help ul, as exact onset o lesion is unclear.

Treatment Excision with margins sent or pathologic evaluation

Examination Flat, dark brown or black color, sharply de ned edges

Prognosis Excellent i excised be ore developing into a melanoma

Lentigo Maligna

35

A

B FIGURE 3-3. Lentigo maligna. A. A large macule with irregular borders and di erent shades o brown. (From Fitzpatrick B et al. Color Atlas & Synopsis of Clinical Dermatology, 4th ed. New York, McGraw-Hill, 2001.) B. Recurrent lentigo maligna o lef brow.

36

3 EYELID NEO PLASMS

BASAL CELL CARCINOMA

B

asal cell carcinoma is the most common type o skin cancer. It is locally invasive and aggressive but has very limited capacity to metastasize. I neglected, it can invade the orbit, especially i located in the medial canthal area. Most commonly, it occurs on the lower eyelid and is treated by complete surgical excision.

Epidemiology and Etiology Age: More than 40 years o age. Rare cases do occur in the 20s and 30s Gender: Males more than emales Etiology: Sun exposure and air skin with poor ability to tan are risk actors. reatment with x-ray ( or acne) increases the risk. Incidence: 500 to 1000 per 100,000 people History Slowly enlarging lesions in sun-exposed areas T e lesions may be associated with bleeding. Examination Round or oval, rm lesions with depressed center T e lesions are pink or red with ne thread-like telangiectasia. T e center may be ulcerated. Basal cell carcinoma may also appear scar-like or be cystic ( Fig. 3-4A–D). Special Considerations Aggressive treatment o basal cell carcinoma o the medial canthal area is indicated because o the risk o orbital extension rom the medial canthal area.

Basal cell carcinomas almost never metastasize. Sclerosing basal cell carcinomas have poorly de ned margins and may recur. Basal cell nevus syndrome is an autosomal dominate syndrome in which patients develop multiple basal cells at a very young age ( Fig. 3-4E and F). Dif erential Diagnosis Squamous cell carcinoma richoepithelioma Laboratory Tests Lesions are sent or pathologic evaluation. Treatment Complete surgical excision with pathologic evaluation Frozen sections or Mohs surgery is needed to assure complete excision. Reconstruction o the de ect is then completed at the same time. reatment with radiation should not be used or lesions around the eye unless the patient is not a surgical candidate. Imiquimod cream can be used as a nonsurgical option i the lesion is not close to the eyelid margin. Prognosis Good when promptly and completely excised Neglected cases can invade the orbit and brain and have the potential, in rare cases, to be atal.

Basal Cell Carcinoma

37

A

B FIGURE 3-4. Basal cell carcinoma. A. Classic appearance o a basal cell carcinoma. T is lesion does not involve the eyelid margin, but large lesions such as this one are a challenge or reconstruction because o the chance o lower eyelid ectropion. B. Notching o the eyelid margin is a sign o an eyelid neoplasm. T is basal cell carcinoma has caused a notch and demonstrates the smooth pearly borders o a basal cell carcinoma. ( continued)

38

3 EYELID NEO PLASMS

C

D FIGURE 3-4. (Continued) Basal cell carcinoma. C. Basal cell carcinoma may present as pigmented lesions, especially in patients with darker pigmented skin. Note the pearly edges on the in erior part o the lesion. D. A cystic lesion can be a basal cell carcinoma. T is lesion is larger than most hydrocystomas and has a slightly violaceous hue. T is cystic basal cell carcinoma was lled with a thick clear gel-like material, which is classic. (continued)

Basal Cell Carcinoma

39

E

F FIGURE 3-4. (Continued) Basal cell carcinoma. E. Basal cell nevus syndrome with many basal cell carcinomas all over the ace occurring at a young age. F. Pits o the palms o the hands that are of en seen in basal cell nevus syndrome.

40

3 EYELID NEO PLASMS

SQUAMOUS CELL CARCINOMA

S

quamous cell carcinoma is a malignant tumor o epithelial keratinocytes. It is of en the result o exogenous carcinogens (ultraviolet exposure, exposure to ionizing radiation, arsenic). T ese lesions are much less common than basal cell carcinoma on the eyelids and are usually success ully treated with excision. Epidemiology and Etiology Age: Older than age 55 years Gender: Males more commonly involved than emales Etiology: Sun exposure and air skin with poor ability to tan are risk actors. reatment with x-ray ( or acne) increases the risk. Incidence: 12 per 100,000 white males; 7 per 100,000 white emales; 1 per 100,000 blacks History Persistent keratotic lesion or plaque that does not resolve af er 1 month must be considered a potential carcinoma, especially in sunexposed areas. Examination wo types o lesions: Di erentiated lesions are keratinized, rm, and hard.

Undi erentiated lesions are eshy, granulomatous, and sof . T ese can present rom very small to large. T ey may be crusted with bleeding or smooth ( Fig. 3-5). Dif erential Diagnosis Actinic keratosis Basal cell carcinoma Keratoacanthoma Laboratory Tests Pathologic evaluation Treatment Complete surgical excision with controlled margins Frozen sections and Mohs surgery are both appropriate options. Imiquimod cream can be used as a nonsurgical option i not close to the eyelid margin. Prognosis Excellent unless the lesion is neglected Squamous cell carcinoma rarely spreads via lymphatics, blood vessels, or along nerves.

Squamous Cell Carcinoma

41

A

B FIGURE 3-5. Squamous cell carcinoma. A.T is is a very large squamous cell carcinoma that was neglected. It now in ltrates the entire lower eyelid. Note the crusting on the lesion, which is usually present with squamous cell carcinoma and is less common with basal cell carcinoma. B. Smaller lesion o the lower eyelid that shows crusting and an irregular, erosive central area.

42

3 EYELID NEO PLASMS

SEBACEOUS ADENO CARCINOMA

S

ebaceous adenocarcinoma is a highly malignant and potentially atal tumor that arises rom the sebaceous glands o the eyelid. Early, this tumor can be di cult to recognize and once it grows, it is di cult to contain, as it can have skip areas. Early recognition and aggressive excision are the keys to success ul treatment. Epidemiology and Etiology Age: Usually greater than 50 years o age Gender: More common in emales than males Etiology: Arises rom the meibomian glands, glands o Zeis, or rom the sebaceous glands o the caruncle, eyebrow, or acial skin. History Of en starts as a chronic blepharitis or nonresolving chalazion Patients may have a chronic red, irritated eye or months to years. Examination Multiple potential presentations: Nodular lesion simulating a chalazion Unilateral chronic blepharitis Cellular membrane growing over the conjunctiva Destructive, of en ulcerated, lesion on the eyelid margin ( Fig. 3-6) Occurrence in the upper eyelid is twice as common as the lower eyelid.

Special Considerations T is lesion is the great masquerader. Delay in diagnosis as a carcinoma and subsequent growth o the lesion add to the poor prognosis or this tumor. Dif erential Diagnosis Basal cell carcinoma Squamous cell carcinoma Chronic blepharitis Chronic chalazion Pathology It is important to alert your pathologist i this sebaceous adenocarcinoma is suspected. Special stains/ studies are per ormed on lesions that are suspected to be sebaceous adenocarcinoma. Without these stains, the lesion may be misdiagnosed. Treatment Diagnosing the lesion is of en the biggest challenge. Biopsy o any suspicious lesion is the key. Once diagnosed, complete excision with wide, controlled margins is the treatment o choice. T ere can be skip areas, so care ul ollowup or recurrence is needed. Prognosis T is is a potentially lethal tumor that must be treated aggressively.

Sebaceous Adenocarcinoma

43

A

B FIGURE 3-6. Sebaceous adenocarcinoma. A.T e eyelid margin is red and in amed with notching. B. When the eyelid is everted, there is an in ltrative lesion o the tarsal conjunctiva.

44

3 EYELID NEO PLASMS

MALIGNANT MELANOMA

M

alignant melanoma is a rare but very dangerous malignant lesion o the eyelids. Sun exposure as a child is an etiologic actor. Despite aggressive surgical excision, this can still be a atal tumor.

Dif erential Diagnosis Nevus Pigmented basal cell carcinoma Laboratory Tests Specimens are sent or pathologic evaluation.

History Pigmented lesion with recent growth or change in appearance

Treatment Complete excision with aggressive, controlled surgical margins T e deeper the lesion, the wider the margins required. Sentinel lymph node biopsy is usually required. Melanomas are best excised via a team approach at centers experienced with melanoma treatment.

Examination Pigmented lesion with irregular pigment deposition, irregular margins, or just increase in size ( Fig. 3-7) T ere may be ulceration and bleeding.

Prognosis Dependent on the depth o the tumor Eight-year survival rate is 33% to 93%, depending on depth o melanoma invasion ( Table 3-1).

Epidemiology and Etiology Age: T ird decade and beyond Gender: Equal between males and emales Etiology: Sun exposure and genetic predisposition

TABLE 3-1. Survival in Relation to umor Depth Depth (mm)

8-Year Survival (%)

3.60

33.3

Malignant Melanoma

45

A

B FIGURE 3-7. Malignant melanoma. A. Lesion o the right eyebrow that has grown over a ew months. T e lesion has irregular areas o lighter and darker pigmentation. B. Malignant melanoma o the lower eyelid.

46

3 EYELID NEO PLASMS

KAPOSI’S SARCOMA

K

aposi’s sarcoma is a vascular neoplasia that can involve multiple systems. It is a rare lesion o the eyelids but when present, is usually associated with a compromised immune system, most commonly HIV disease. Epidemiology and Etiology Age: Any Gender: More common in males Etiology: Vascular neoplasia is of en associated with immune compromise in the United States.

History Rapid growth o lesion may occur. Patients most commonly are HIV-positive, although other orms o immune compromise may predispose patients to these lesions. Examination Elevated dermal lesions that are red or purple ( Fig. 3-8)

Dif erential Diagnosis Pyogenic granuloma Chalazion Hemangioma Melanocytic nevus Laboratory Tests Pathologic evaluation i biopsied Evaluation o immune system i indicated Treatment Excision with pathologic evaluation Cryotherapy or intralesional chemotherapeutic agents may be used or local control o the lesions. Radiation treatment or some large lesions Prognosis Patients who develop lesions associated with HIV of en have a short survival and die rom advancement o the HIV disease. Patients with primary Kaposi’s sarcoma may survive or years.

Kaposi’s Sarcoma

A

B FIGURE 3-8. Kaposi’s sarcoma. Lesion o the lower lid in a patient with AIDS.

47

C H AP T ER

Eyelid rauma MARGINAL EYELID LACERATION

M

arginal eyelid lacera ions are mos commonly associa ed wi h rauma o he en ire orbi al area, and of en here are o her associa ed injuries. T e ex en o laceraion can vary grea ly. Promp , me iculous closure is he rea men o choice. Epidemiology and Etiology Age: Any age. Second hrough our h decades mos common. Gender: Males are more commonly a ec ed. E iology: Blun rauma (e.g., s ), direc cu (e.g., glass, kni e), or dog bi e mos commonly History rauma his ory is variable rom minor o major injuries. I is impor an o de ermine he cause o he rauma o know whe her o suspec oreign bodies.

48

he amoun o orce causing he injury will help de ermine he likelihood o more signi ican injuries o he orbi and globe. Examination Mus evalua e globe and orbi or injuries. Evalua e he ex en o he injury o he eyelid and be sure ha he lacrimal sys em is no injured ( Fig. 4-1). C scanning may be required i o her injuries or oreign bodies are suspec ed. Special Considerations Dog bi es (also ca s and humans) require copious irriga ion o he wound and special care because o he grea er risk o in ec ion. e anus immuniza ion mus be up o da e. Treatment Me iculous closure o he wound wi hin 24 o 48 hours

Marginal Eyelid Laceration

Surgery can be done in he o ce or emergency room set ing unless he lacera ions are complex or in children, where an opera ing room set ing wi h general anes hesia is required.

49

Prognosis Good. T e more complex he wound, he grea er he chance o scarring, which may hen require secondary repair a a la er da e.

FIGURE 4-1. Marginal eyelid laceration. Cen ral eyelid lacera ion rom an umbrella ca ching under he eyelid. Isola ed marginal lacera ions wi hou canalicular involvemen are more likely due o some objec direc ly cut ing he eyelid. Canalicular lacera ions are more of en because o earing and s re ching, as he medial lid is he weakes area and he rs o ear.

50

4 EYELID TRAUMA

CANALICULAR EYELID LACERATION

Probing o he canalicular sys em may be necessary i a lacera ion is suspec ed ( Fig. 4-2).

T

Special Considerations T e ar her he lacera ion is medially rom he lacrimal punc a, he more di cul i is o nd he dis al cu end.

he medial eyelid is he weakes area o he eyelid, so any horizon al rac ion on he eyelid is more likely o resul in damage o he medial eyelid and he canaliculus. Eyelid rauma requires care ul inspec ion o he medial can hal area o recognize he lacera ed canaliculus. Repair wi h silicone in ubaion is he rea men o choice.

Epidemiology and Etiology Age: Any age. Second hrough our h decades mos common. Gender: Males are more commonly a ec ed. E iology: Usually a earing injury, as he medial eyelid is he weakes area o he eyelid. History rauma his ory is variable, including blun orce, dog bi es, and, rarely, sharp objec s. Examination Evalua e eye and orbi or injuries. Any cu medial o he lacrimal punc a mus be evalua ed or a canalicular lacera ion.

Treatment Surgical repair wi h anas omosis o he canalicular ends and in uba ion o he lacrimal sys em Loca ing he dis al end o he canaliculus may be di cul and of en requires loupes or an opera ing microscope. Depending on he severi y o he injury and he pa ien ’s coopera ion, surgical repair is usually per ormed in he opera ing room set ing wi h local or general anes hesia. T e ubing is lef in he lacrimal sys em or 6 weeks o 6 mon hs, depending on severi y and he individual prac i ioner. Prognosis Good. Even he injuries ha resul in a scarred canaliculus usually do well, as mos pa ien s do well wi h one unc ioning canaliculus.

Canalicular Eyelid Laceration

51

A

B FIGURE 4-2. Canalicular laceration. A. Any cu or ear medial o he punc um, no mat er how super cial i appears, needs o be explored or involvemen o he canaliculus. T is lacera ion involved bo h he upper and lower canaliculi. B. T e punc um and cu canaliculus can be seen a he medial edge o he cu eyelid. T is eyelid was nearly comple ely avulsed.

52

4 EYELID TRAUMA

DO G BITES

D

og bi es are highly variable in heir ex en bu generally do no involve injury o he globe i sel . Promp repair wi h copious irrigaion of en gives airly good resul s, depending on he ex en o he original injury.

Epidemiology and Etiology Age: Mos commonly children; adul s less common. Gender: Equal E iology: In children, he dog may be rying o bi e he child on he nose o show domina ion, which is wha happens be ween dogs, and usually does no represen an at ack. T e eyelid lacera ion is he resul o he dog’s canine oo h ca ching he eyelid and earing he medial lid ra her han a rue bi e o he eyelid. History Of en, he child knows he dog and here is a single bi e and no a vicious at ack. Examination Evalua ion o he eye and orbi or o her injuries

T ere is mos commonly a single bi e wi h mul iple areas o injury. T ere may be punc ure wounds or larger ears and gashes ( Fig. 4-3). Special Considerations T e bi e mus be repor ed o he heal h depar men or ollow up o be sure he dog is properly vaccina ed or rabies. e anus immuniza ion mus be up o da e. Treatment T e eyelid lacera ion mus be rea ed as ou lined previously. T e risk o in ec ion in animal and human bi es is high because o he bac eria in he mou h. Copious irriga ion o he wounds is he only rea men o proven bene o decrease he risk o in ec ion. T e use o broad-spec rum, sys emic an ibio ics has no been proven o lessen he chance o in ec ion bu should be considered. Prognosis Good. T e more severe he injury, he grea er he risk o pos opera ive de ormi y.

Dog Bites

53

FIGURE 4-3. Dog bite with eyelid lacerations. Dog bi es are usually a single bi e, bu he amoun o damage can be highly variable rom mild o severe. T e eyelid damage is usually rela ed o earing as he dog pulls away and he ee h ge caugh on he eyelid. Bo h he medial and la eral can hi are orn and mul iple punc ure wounds are seen.

54

4 EYELID TRAUMA

EYELID BURNS

E

yelid burns are usually associa ed wi h signi can burns o he res o he ace and body unless hey are elec rical or chemical. All burns ake days o weeks or he ull issue dea h and necrosis o mani es . Recons ruc ion can be very di cul because o poor vasculariza ion.

Epidemiology and Etiology Age: Any Gender: Males more commonly a ec ed E iology: Burns ha involve he eyelids are usually associa ed wi h burns over a large percen age o he body.

T e concern is o pro ec he cornea wi h lubrica ion. Wi h ime, he eyelids will scar, resul ing in poor closure and more corneal exposure ( Fig. 4-4). Treatment An ibio ic oin men and copious corneal lubrica ion Sys emic an ibio ics are usually par o he sys emic care. As he burns heal, cica ricial changes become more prominen and he use o skin graf s is required.

History Generally associa ed wi h o her acial burns unless he e iology is elec rical or chemical.

Prognosis Dependen on he severi y o he burns

Examination Burns o he eyelid vary in dep h and severi y.

Severe burns may require mul iple surgeries and skin graf s o pro ec he cornea.

Eyelid Burns

55

A

B FIGURE 4-4. Eyelid burn. A. Elec rical burn wi h necrosis o he upper eyelid and underlying scleral necrosis. Elec rical burns can ake weeks or he o al amoun o issue necrosis o become apparen . B. Mol en lead was splashed on o he eyelid and in o he eye. Wi h hermal burns, he ex en o damage is eviden more quickly. No e he rela ive lack o vasculariza ion along he lower eyelid margin rom he burn. T ere was par ial necrosis and loss o par o he eyelid margin over ime.

C H AP T ER

Eyelid Malposi ions ENTROPION ACUTE SPASTIC ENT OPION

A

cu e spas ic en ropion is he resul o eyelid swelling along wi h orbicularis spasm ha resul s in a emporary in- urning o he eyelid. A cycle o corneal irri a ion rom he en ropion, causing more eyelid spasm, causing more irri a ion, mus be broken so he eyelid can re urn o normal. Some o hese pa ien s will have underlying involu ional changes (laxi y) ha may resul in a recurren en ropion. Epidemiology and Etiology Age: More common in older pa ien popula ion Gender: Equal occurrence in males and emales E iology: Ocular irri a ion or in ammaion causes con inued orced blinking and closure o he eye. T is will lead o in- urning o he lower eyelid in eyelids ha have involu ional changes predisposing hem o en ropion (see “Involu ional En ropion”).

56

History ecen surgery on he eye or recen onse o ocular irri a ion Examination Lower eyelid en ropion ( Fig. 5-1) wi h associa ed involu ional ac ors such as horizon al laxi y and orbicularis override. In addi ion, here is a separa e iden if able irri an o he eye. his irri an may be kera i is, oreign body, su ure, or jus in lamma ion pos opera ively. Dif erential Diagnosis Involu ional en ropion Cica ricial en ropion Pathophysiology Involu ional changes o he eyelid allow he orced closure o he eyelid orbicularis muscle o override he arsus and drive he eyelid margin inward oward he eye.

Entropion

Treatment rea men o he underlying ocular irri aion or in amma ion will resolve some cases. T is involves rea ing he ocular irri a ion and s abilizing he eyelid o hal he addiional irri a ion he eyelid is causing. S abilizing he eyelid may involve aping he eyelid ou or Quicker su ures.

57

Some cases will hen resolve; o hers will become an involu ional en ropion and need more ex ensive surgery. Prognosis Excellen . ecurrence in pa ien s wi h signif can involu ional ac ors o he eyelid may develop an involu ional en ropion a a la er ime.

FIGURE 5-1. Acute spastic entropion. Pa ien wi h a corneal abrasion. Con inued irri a ion and blinking leads o an en ropion.

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5 EYELID MALPO SITIO NS

INVOLUTIONAL ENT OPION

E

yelid laxi y bo h horizon ally and ver ically predisposes o he ins abili y o he lower eyelid. T e addi ional ac or required is he abili y o he pa ien ’s orbicularis muscle o override he arsus and drive he eyelid inward. Pa ien s presen wi h red, irri a ed eyes rom he eyelid margin and he eyelashes in con ac wi h he eye i sel .

Epidemiology and Etiology Age: More common in older pa ien popula ion Gender: Equal occurrence in males and emales E iology: Horizon al laxi y and orbicularis override resul in inversion o he eyelid. History Acu e onse o eye irri a ion. T is irri aion is some imes in ermit en in na ure and becomes more cons an . Examination Inver ed lower eyelid wi h in erior corneal superf cial punc a e kera i is (SPK) or corneal abrasion ( Fig. 5-2) En ropion is usually associa ed wi h horizon al eyelid laxi y.

Orbicularis muscle override is o en no ed as ullness over he arsal pla e when he lid is en ropic. T e en ropion can be in ermit en and no always presen on examina ion. Placing opical anes he ic drops in he eye, having he pa ien close he eyes orce ully, and look downward will usually bring ou he en ropion. Dif erential Diagnosis Cica ricial en ropion Acu e spas ic en ropion Pathophysiology Aging o eyelid issues resul s in laxi y and s re ching o suppor ing s ruc ures. Treatment Surgical correc ion is based on correc ing he ac ors con ribu ing o he en ropion, usually horizon al shor ening o he eyelid and igh ening he eyelid re rac ors in any o mul iple ways. Prognosis Excellen . T ere is a 5% o 10% chance o recurrence over 5 o 10 years.

Entropion

59

FIGURE 5-2. Involutional entropion. Lef lower eyelid en ropion wi h involu ional changes. T e rolled in orbicularis muscle can be seen driving he eyelid margin inward on he lef .

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5 EYELID MALPO SITIO NS

CICAT ICIAL ENT OPION

C

ica ricial en ropion is caused by conjuncival scarring pulling he eyelid inward. Generally, rea men is surgical, bu def ning and rea ing he cause o he conjunc ival scarring mus be done f rs or mos cases will recur. Occurs in he upper or lower eyelid.

Epidemiology and Etiology Age: Any age Gender: Equal occurrence in males and emales E iology: Scar issue on he conjunc ival sur aces resul s in shor ening o he pos erior lamella, physically pulling he eyelid inward. Fac ors include: Surgery Conjunc ival scarring diseases (e.g., ocular cica ricial pemphigoid, S evens-Johnson syndrome, rachoma) rauma Conjunc ival burns (e.g., chemical) An iglaucoma drops History Chronic low-grade in amma ion over mon hs o years resul s in he en ropion ha hen causes more irri a ion. T e o her scenario is a his ory o rauma or surgery resul ing in an en ropion and associa ed irri a ion. Examination Care ul evalua ion o he conjunc iva or signs o scarring causing inversion o he eyelid.

T is may include evalua ion o he o her eyelids o de ermine i he en ropion is isola ed or involving all our eyelids, which may help de ermine he e iology ( Fig. 5-3). Special Considerations Mus de ermine he e iology o he conjunc ival scarring be ore rea ing. Any progressive disease mus be quie ed be ore surgery can be done on he eyelids. Dif erential Diagnosis Acu e spas ic en ropion Involu ional en ropion Laboratory Tests Conjunc ival biopsy wi h immuno uorescence es ing i ocular cica ricial pemphigoid is suspec ed. Treatment De ermine he e iology o he conjunc ival scarring. Quie any ac ive disease. Surgical correc ion o he en ropion wi h a marginal ro a ion or buccal mucosal gra is hen he rea men o choice. Prognosis Variable depending on he e iology En ropion secondary o rauma and surgery usually do very well. Progressive disease processes, such as ocular cica ricial pemphigoid, can make i much more di cul o preven recurrence o he en ropion.

Entropion

61

A

B FIGURE 5-3. Cicatricial entropion. Ex ernally (A), i is di cul o di eren ia e his cica ricial en ropion rom an involu ional en ropion un il he eyelid is ever ed (B), and he cica ricial changes are no ed pulling he eyelid inward.

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5 EYELID MALPO SITIO NS

ECTROPION INVOLUTIONAL ECT OPION

I

nvolu ional ec ropion has he same involuional ac ors as in an involu ional en ropion (e.g., horizon al laxi y, ver ical ins abili y). T ese pa ien s do no have hyper rophic, spasic orbicularis muscle o override, so he uns able eyelid sags ou ward ins ead o being driven inward. Symp oms are less acu e and no as severe as in involu ional en ropion. Many pa ien s will have mild involu ional ec ropions and may be asymp oma ic. Epidemiology and Etiology Age: Incidence increases as age increases. Gender: Equal occurrence in males and emales E iology: Eyelid issue laxi y, especially horizon al laxi y History Insidious onse o ocular irri a ion and/ or earing Pa ien may no e redness and in ammaion o he eyelid margin. Examination Eyelid sagging in eriorly and away rom he globe sur ace ( Fig. 5-4)

Mus look or he amoun o horizon al laxi y, corneal exposure, and s enosis o he lacrimal punc a. Special Considerations arsal ec ropion is comple e eversion o he eyelid and indica es de achmen o he lower eyelid re rac ors. T is condi ion mus be recognized, as i requires bo h horizon al igh ening and rea achmen o he re rac ors. Dif erential Diagnosis Cica ricial ec ropion Paraly ic ec ropion Treatment Mild ec ropion wi h only mild exposure symp oms can some imes be rea ed wi h ocular lubrica ion. De ini ive rea men involves horizon al eyelid shor ening and possible punc oplas y. Prognosis Excellen . ecurrence a er surgery is es ima ed a 5% o 10%, bu is higher he longer he ollow-up is done and he more severe he ec ropion was a he ime o he repair.

Ectropion

FIGURE 5-4. Involutional ectropion. Bila eral ec ropions wi h very lax eyelids. No e he red palpebral conjunc iva rom chronic exposure.

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5 EYELID MALPO SITIO NS

PAR LYTIC ECT OPION

P

araly ic ec ropion is he resul o emporary or permanen seven h cranial nerve palsy. T e lower eyelid sags away rom he globe, resul ing in loss o pro ec ion o he eye and inabili y o he lacrimal sys em o collec ears. Pa ien s wi h less severe palsy and o her eye pro ec ive mechanisms in ac presen wi h earing. Pa ien s wi h more severe palsies and poor eye pro ec ion mechanisms presen wi h corneal breakdown. Epidemiology and Etiology Age: Any age Gender: Equal occurrence in males and emales E iology: Facial palsy e iologies include: Bell’s palsy Surgery: in racranial or acial S roke umor

Evalua e severi y o acial palsy, degree o ec ropion, amoun o corneal exposure, amoun o lagoph halmos, and presence o an in ac Bell’s phenomenon. Special Considerations Mus check or corneal sensa ion as loss o corneal sensa ion will make all exposure symp oms much worse. Any unexplained acial palsy mus be worked up. Dif erential Diagnosis Bell’s palsy versus nonresolving acial palsy

History Previous onse o acial palsy Depending on he severi y o he acial palsy, he ec ropion may have onse a he same ime or he eyelid may slowly sag wi h ime. T e severi y o he condi ion depends on he severi y o he paralysis, corneal sensa ion, and ocular lubrica ion.

Treatment rea men depends on he an icipa ed dura ion o he paralysis. I spon aneous improvemen is an icipa ed, hen rea men wi h lubrica ion and a emporary arsorrhaphy i severe corneal problems are presen is indica ed. I corneal exposure is s ill a problem wi h lubrica ion use and he paralysis is long erm, hen horizon al eyelid igh ening is used o rea he paraly ic ec ropion. Placing a gold weigh in he upper eyelid may also be required. arely, a permanen arsorrhaphy may be needed.

Examination T e lower eyelid is ound o be sagging away rom he globe ( Fig. 5-5).

Prognosis Variable. T e ec ropion ends o recur over ime i he paralysis is permanen .

Ectropion

FIGURE 5-5. Paralytic ectropion. Righ lower eyelid ec ropion as he resul o a acial palsy.

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5 EYELID MALPO SITIO NS

CICAT ICIAL ECT OPION

C

ica ricial ec ropion is caused by mechanical shor ening o he an erior lamellae o he eyelid pulling he eyelid down and ou ward. T is resul s in earing and corneal exposure. More common in he lower eyelid bu can occur in he upper eyelid.

Epidemiology and Etiology Age: Any age Gender: More common in males because o higher incidence o rauma ic even s. E iology: Scarring o he an erior lamellae o he eyelid pulls he eyelid ou ward. T e e iologies include: rauma Surgery Derma i is Skin carcinoma History May include a specif c his ory such as rauma or surgery I a chronic derma ologic condi ion is he cause o he scarring, his may be a known or a previously unrecognized condi ion. Examination Ex ernal scarring or skin changes are no ed on he upper or more commonly he lower eyelid.

T is scarring resul s in shor ening o he eyelid skin and ou - urning o he eyelid margin ( Fig. 5-6A). Special Considerations Mus always consider a skin carcinoma as he possible cause o scarring o he skin. I he cause is unclear, a biopsy is needed. Dif erential Diagnosis Impor an o di eren ia e involu ional ec ropion rom hose wi h cica ricial changes. Treatment rea men o any underlying derma ologic condi ion is impor an . In rauma ic or pos surgical cases, he scarring should be le or 6 mon hs or longer unless exposure or o her problems necessi a e earlier rea men . rea men involves lysis o any deep scar issue wi h horizon al igh ening. I he skin shor ening is severe, ull- hickness skin gra s will be required. Skin gra s have he po en ial or scarring and a cosme ically no iceable area a he gra si e ( Fig. 5-6B). Prognosis rauma or surgically induced cases do well wi h repair. Chronic condi ions o he skin end o resul in recurrences.

Ectropion

67

A

B FIGURE 5-6. Cicatricial ectropion. A. rauma o he lef lower eyelid resul s in scarring o he skin wi h ver ical shor ening as well as scarring in ernally wi hin he eyelid. B.A f er repair, using a skin graf .

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5 EYELID MALPO SITIO NS

MECHANICAL ECT OPION

M

echanical ec ropion is a rare cause o ec ropion in which a mass o some ype pushes he eyelid ou ward. here are usually associa ed involu ional changes ha allow he eyelid o be pushed ou ward. Epidemiology and Etiology Age: Older pa ien s Gender: Equal occurrence in males and emales E iology: Gravi y pulls he eyelid away rom he eye or pushes he eyelid away rom he eye secondary o a mass. Causes o he mass e ec include: Derma ochalasis Edema Chalazion Eyelid umor (e.g., hemangioma, inclusion cys )

History Pa ien may be asymp oma ic, have symp oms o corneal irri a ion, or have redness and irri a ion o he eyelid. Examination Mus de ermine he degree o involu ional changes o he eyelid as well as he e iology o he mass dis or ing he eyelid. T e amoun o corneal exposure and any corneal scarring should also be no ed ( Fig. 5-7). Dif erential Diagnosis Involu ional ec ropion Cica ricial ec ropion Paraly ic ec ropion Treatment Excision o he mass and correc ion o he involu ional ac ors o he eyelid Prognosis Good i he mass can be elimina ed

Ectropion

69

A

B FIGURE 5-7. Mechanical ectropion. A. Chemosis rom an in amma ory process mechanically pushes he lower eyelid ou ward. T ere are usually some involu ional changes presen o allow he eyelid o be pushed ou . Resolu ion o he chemosis allowed he eyelid o re urn o a normal posi ion. B. Mechanical ec ropion rom a chalazion. Even wi h chalazion resolu ion, he ec ropion may remain.

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5 EYELID MALPO SITIO NS

SYMBLEPH ARON

S

ymblepharon is scarring be ween he bulbar and palpebral conjunc iva. T is may be associa ed wi h ac ive in amma ion or here may be no in amma ory signs. Epidemiology and Etiology

Age: Any age Gender: More requen in women E iology: T e ollowing can resul in scarring o wo conjunc ival sur aces: Chronic blephari is Previous rauma Conjunc ival scarring diseases (e.g., ocular cica ricial pemphigoid, S evensJohnson syndrome) A opic disease Eyelid surgery Conjunc ival burns Chronic glaucoma drops, especially mio ics History T ere may be no his ory, jus asymp oma ic symblepharon no ed on examina ion. Pa ien s wi h his ory o eye or eyelid rauma or in amma ion may also have symblepharon. Examination Scarring o he conjunc ival sur aces may be very sub le wi h sligh in erior ornix shor ening or i may be very obvious wi h large conjunc ival bands be ween he eye and eyelid ( Fig. 5-8).

Mus be sure o examine under he upper lid or conjunc ival scarring, as early signs are some imes more obvious here. Special Considerations I is impor an o de ermine he cause o he symblepharon. I asymp oma ic, he symblepharon may require no rea men excep looking or he cause o he scarring. uling ou a progressive conjunc ival scarring disease, such as ocular cica ricial pemphigoid, is impor an . Dif erential Diagnosis T e di eren ial diagnosis involves de ermining he cause o he symblepharon, no whe her he process is a symblepharon. Laboratory Tests Conjunc ival scarring o unknown e iology requires a conjunc ival biopsy wi h immunouorescence es ing o rule ou ocular cica ricial pemphigoid. In rare cases, squamous cell carcinoma may cause symblepharon; here ore, pa hologic evalua ion should be considered in selec cases. Treatment None or mild symblepharon Moni oring or progression is impor an . Signif can symblepharon may cause richiasis and cica ricial en ropion ha hen may require rea men . Prognosis Variable depending on he cause o he symblepharon

Symblepharon

A

B FIGURE 5-8. Symblepharon. A. Scarring is seen be ween he eyelid and he in erior cornea. B.E arly symblepharon may be no ed only as shor ening o he ornix.

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5 EYELID MALPO SITIO NS

TRICHIASIS richiasis is an acquired misdirec ion o eyelashes. richiasis may be ocal, as is seen a er eyelid rauma in he area o he lacera ion. T e process may be di use wi h eyelid scarring and lashes along he en ire eyelid margin. Epidemiology and Etiology Age: Any age. Non rauma ic causes are rare in childhood. More common wi h increasing age. Gender: More common in emales E iology: Lash ollicles are dis or ed and become misdirec ed wi h scarring o he eyelid. Chronic eyelid in amma ion may resul in grow h o misdirec ed lashes. Chronic blephari is, eyelid rauma, and conjunc ival scarring diseases can all cause richiasis. History Pa ien s will o en have a his ory o chronic eye irri a ion and in amma ion. T ey may also have a long his ory o eyelash problems. T ere may be a his ory o eyelid rauma or surgery. Examination Eyelashes are seen rubbing on he eyelid sur ace ( Fig. 5-9). T e amoun o corneal changes depends on he number o lashes and dura ion. T ere may be jus SPK or here may be corneal scarring. Special Considerations Impor an o di eren ia e richiasis rom abnormal eyelid posi ions, such as en ropions, which secondarily resul in eyelashes rubbing on he cornea.

Dif erential Diagnosis Spas ic en ropion Involu ional en ropion Cica ricial en ropion Congeni al dis ichiasis Laboratory Tests Conjunc ival scarring o unknown e iology requires a conjunc ival biopsy wi h immuno uorescence es ing o rule ou ocular cica ricial pemphigoid. Treatment Lashes can be epila ed or emporary relie bu hey always grow back. Elec rolysis or cryo herapy will abla e lashes on a more “permanen ” basis. A bes , 50% o he lashes will no regrow so mul iple rea men s are required. In cases o severe scarring, eyelid surgery will be needed o correc he problem. T is may include marginal ro a ion, excision o he abnormal lashes, and a buccal mucosal gra . Prognosis Dependen on he cause o he richiasis Chronic progressive in amma ory diseases, such as ocular cica ricial pemphigoid, will o en have recurren lashes ha can be very di cul o comple ely eradica e. richiasis rela ed o rauma or o her nonprogressive scarring usually responds well o rea men .

Trichiasis

73

A

B FIGURE 5-9. richiasis. A. Eyelashes are growing pos eriorly, con ac ing he cornea rom he upper eyelid. In rue richiasis, he eyelid margin is normal. Of en, wi h conjunc ival scarring disease, here will be some accompanied in- urning o he eyelid margin. B. Lower eyelid richiasis rom conjunc ival scarring.

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5 EYELID MALPO SITIO NS

PTOSIS CONGENITAL MYOGENIC PTOSIS

C

ongeni al myogenic p osis is he mos common congeni al p osis and resul s rom a dysgenesis o he leva or muscle. I may be unila eral or bila eral and can vary in severi y rom very mild p osis o very severe.

Epidemiology and Etiology Age: Bir h Gender: Equally a ec s males and emales E iology: T e leva or muscle developmen is abnormal, resul ing in f brosis and at y inf l ra ion o he leva or muscle. History P osis no ed a bir h or soon a er. Child may have chin up head posi ion, especially i bila eral. Paren s may no e he child’s eyes are open while asleep. Examination P osis is no ed o be ei her unila eral or bila eral. T ere is lit le or no leva or unc ion, resul ing in a f bro ic, s i eyelid wi h a airly f xed posi ion as he eye moves rom up o down gaze. T e lid crease is o en poorly ormed. Depending on he severi y o he p osis, here may be amblyopia wi h unila eral p osis ( Fig. 5-10). Special Considerations T ere will be abnormal superior rec us unc ion in 16% o pa ien s; his makes exposure problems a er repair and s rabismus a concern.

Dif erential Diagnosis I he p osis is congeni al wi h poor leva or unc ion, here is lit le else in he di erenial. Bir h rauma can resul in a p osis, bu here is usually good leva or unc ion and he leva or muscle is no f bro ic. Marcus Gunn jaw wink needs o be considered in all cases o congeni al p osis (see sec ion “Marcus Gunn Jaw Wink”). T ere are o her orms o myogenic p osis ha are acquired, no congeni al, such as in muscular dys rophy, chronic progressive ex ernal oph halmoplegia (CPEO), myas henia gravis, or oculopharyngeal dys rophy. Laboratory Tests Skele al muscle biopsy and elec rophysiologic es ing may be needed. An elec rocardiogram should be done i CPEO is suspec ed. Treatment Fron alis suspension surgery using su ure, a silicone rod, or ascia la a T e age o do surgery depends on he severi y o he p osis and any underlying amblyopia. Amblyopia may need rea men wi h pa ching a er he eyelid is li ed. T ere is con roversy in rea ing unila eral congeni al p osis regarding whe her o do a ron alis suspension only on he p o ic eye or i he normal eye should have excision o he leva or and a ron alis suspension as well o provide symme ry. Prognosis Surgery is very success ul in li ing he lid above he pupillary axis. T e eyelid will someimes all wi h ime and repea surgery may be needed la er.

Ptosis

75

FIGURE 5-10. Congenital myogenic ptosis. Modera e congeni al p osis in a child. No e he ex reme use o he eyebrows o lif he eyelids. T ere is a prominen eyelid crease in his child, bu i is usually poorly de ned in congeni al p osis. Leva or unc ion was 3 mm.

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5 EYELID MALPO SITIO NS

ACQUI ED MYOGENIC PTOSIS

A

cquired myogenic p osis is an unusual cause o p osis rela ed o developmen o a muscular disease ha can be localized or may be sys emic. Epidemiology and Etiology Age: Acquired bu can presen in children or adul s E iology: Sys emic muscular diseases ha can cause acquired myogenic p osis include muscular dys rophy, CPEO, myas henia gravis, and oculopharyngeal dys rophy. History Progressive p osis ha is o en associa ed wi h o her muscular dys unc ion Examination P osis is no ed wi h decreased leva or unc ion. T ere may be abnormal eye movemen s and abnormal acial one. Myas henia gravis may have double vision as par o he presen a ion. Chronic progressive ex ernal oph halmoplegia has decreased eye movemen s, bu here is no diplopia. Care ul evalua ion o he abili y o close he eyes is needed, as poor closure will increase he risk o pos opera ive corneal exposure ( Fig. 5-11). Special Considerations Chronic progressive ex ernal oph halmoplegia is a gradual, bila eral p osis ha begins in childhood or young adul hood and is progressive wi h involvemen o ex raocular muscles.

I is heredi ary in 50% o he cases. I is progressive un il he eyes are f xed in a sligh ly downward direc ion wi h a severe p osis. Hear block, re ini is pigmen osa, abnormal re inal pigmen a ion, and various neurologic signs have been associa ed wi h his syndrome. Dif erential Diagnosis Congeni al p osis Neurogenic p osis Laboratory Tests Skele al muscle biopsy and elec rophysiologic es ing may be needed. An ECG should be done i CPEO is suspec ed. Treatment Surgery is needed or correc ion. Evalua ion and rea men o any sys emic abnormali ies mus be addressed f rs . Depending on he severi y o he p osis and he amoun o leva or unc ion, ei her leva or resec ion or ron alis suspension is indica ed. Fron alis suspension wi h a silicone rod will work well in many o hese pa ien s. Prognosis Mos pa ien s can achieve an eyelid level ha is unc ional. Mos will no be able o achieve an eyelid level or unc ion ha is considered normal.

Ptosis

77

A

B FIGURE 5-11. Acquired myogenic ptosis. A. Pa ien wi h muscular dys rophy and severe p osis. T ere is very lit le leva or unc ion, and he pa ien is barely able o keep his eyelids above he pupil wi h ex reme eyebrow eleva ion. B.T e same pa ien af er ron alis suspension surgery. He can now e ec ively lif his eyelids by eleva ing his eyebrows.

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5 EYELID MALPO SITIO NS

APONEU OTIC PTOSIS

A

poneuro ic p osis is he mos common ype o p osis and is he resul o disinser ion o he leva or aponeurosis. T is ype o p osis may be caused by normal aging changes, swelling, or repe i ive s re ching o he upper eyelid. T e onse o p osis is gradual. Epidemiology and Etiology Age: arely congeni al. Mos common in older pa ien s. Gender: Equal E iology: Aponeuro ic p osis is due o an abnormali y o he leva or aponeurosis or i s inser ion. T is resul s rom normal involu ional changes and/ or repe i ive rac ion such as eyelid rubbing, eyelid swelling, and eye surgery. History Gradual, progressive droopiness o he eyelids is he mos common his ory. ecen eye surgery or eyelid swelling can exacerba e he p osis. Examination Mild o severe p osis wi h normal leva or unc ion and o en a high eyelid crease or less commonly a poor eyelid crease.

T e p osis may be worse in downgaze ( Fig. 5-12). Special Considerations Myas henia gravis mus be considered in all cases. Dif erential Diagnosis Congeni al p osis (di eren ia e by poor leva or unc ion) Myas henia gravis rauma ic p osis Laboratory Tests None Treatment Ex ernal leva or resec ion and müllerecomy are bo h good surgical approaches or success ul repair. Dry eyes, poor eye closure, and poor Bell’s phenomenon mus all be recognized preopera ively. T ese condi ions make pos opera ive corneal exposure more likely. Prognosis Excellen prognosis or success ul surgical correc ion

Ptosis

79

FIGURE 5-12. Aponeurotic ptosis. Bila eral p osis rom dehiscence o he leva or aponeurosis. No e he high, very de ned upper eyelid crease. Leva or unc ion was 18 mm.

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NEUROGENIC PTOSIS THI D NE VE PALSY hird cranial nerve palsy is usually mani es as sudden or progressive onse o p osis wi h an underlying s rabismus. De ermining he e iology is he f rs priori y, as some causes o a hird cranial nerve palsy are li e- hrea ening. rea men is di cul . Epidemiology and Etiology Age: Any age. are in children. Gender: No emale versus male di erence no ed. Etiology Ischemic microvascular disease Compressive: aneurysm, umor rauma Oph halmoplegic migraine: children History Acu e onse o p osis wi h double vision when he eyelid is li ed. May or may no be associa ed wi h pain. Examination Comple e p osis wi h he eye posi ioned down and ou ( Fig. 5-13). T ere is an inabili y o eleva e, depress, or adduc he eye. T e pupil may or may no be dila ed. Aberran regenera ion o he hird nerve should be ruled ou . Special Considerations I he pupil is dila ed, he pa ien needs emergen neuroimaging o rule ou a pos erior communica ing ar ery aneurysm. Nonresolving hird nerve palsies, incomple e hird nerve palsies, and any hird nerve palsy wi h aberran regenera ion requires neuroimaging.

Pa ien s younger han 50 years o age need neuroimaging unless hey have signif can vascular disease. Vasculopa hic causes o hird nerve palsies should resolve wi hin 3 mon hs. Dif erential Diagnosis Myas henia gravis Chronic progressive ex ernal oph halmoplegia Laboratory Tests M I wi h MR , or an angiogram i he hird nerve palsy involves he pupil Pathophysiology In errup ion o he hird nerve may be caused by compression o he nerve or ischemia. Ischemia will no cause pupillary dilaion and will resolve wi hin 3 mon hs. Treatment T e majori y o pupil-sparing hird nerve palsies will resolve in 3 mon hs. T ese pa ien s should be given adequa e ime or spon aneous resolu ion be ore surgical correc ion is per ormed. T e underlying s rabismus mus be rea ed be ore at emp ing o li he eyelid. P osis surgery requires ron alis suspension, bu here is risk o corneal exposure. Fron alis suspension wi h a silicone rod is a sa er surgical approach. Prognosis Many hird nerve palsies will resolve in 3 o 6 mon hs. T ose ha do no resolve are di cul o ge in o normal eyelid posi ion wi hou causing an unaccep able amoun o corneal exposure. Pa ien s will o en have residual diplopia rom he mo ili y problems when he eyelid is raised.

Neurogenic Ptosis

81

A

B FIGURE 5-13. T ird nerve palsy. A. Comple e p osis o he upper eyelid wi h no leva or unc ion. B.E leva ion o he p o ic eyelid reveals ocular misalignmen consis en wi h a hird nerve palsy.

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MYASTHENIA GR VIS

M

yas henia gravis is an au oimmune disorder in which au oan ibodies at ack he recep ors o he neuromuscular juncion. T e resul is usually a sys emic muscular weakness ha can be li e- hrea ening due o respira ory compromise. P osis wi h or wi hou diplopia can o en be he ini ial presena ion. rea men o he sys emic disease is maximized be ore any surgery is done on he eyelids. Epidemiology and Etiology Age: Any age Gender: More common in emales E iology: Au oimmune disease; may be associa ed wi h hymoma or an eceden in ec ion. History Insidious onse o droopy eyelids, double vision, or bo h, which are o en worse a he end o he day. Pa ien s may have acial weakness, proximal limb weakness, or di cul y swallowing and brea hing. Symp oms are ypically in ermit en . Examination T e disease is mos o en generalized and sys emic bu may presen ini ially wi h p osis and/ or double vision. P osis is worsened wi h sus ained upgaze and diplopia is worse wi h con inual eye movemen s ( a igue). Weakness o he orbicularis muscles is usually ound. T ere may also be acial and proximal limb weakness. Diagnosis may be made wi h he ice es , ace ylcholine recep or an ibody es ing, single-f ber EMG, or ensilon es ing.

T e ice es involves placing an ice pack on he eyelids or 2 minu es, i he p osis is secondary o myas henia gravis, i will improve. ensilon es ing involves IV adminis raion o edrophonium chloride ( ensilon). Improvemen o he p osis or diplopia indica es he e iology is myas henia gravis. T e use ulness o ensilon es ing is limi ed because o he po en ial adverse e ec s including bradycardia and even respira ory arres ( Fig. 5-14). A combina ion o single-f ber EMG and an ibody es ing is mos commonly used o make he diagnosis. Special Considerations Pa ien s wi h newly diagnosed myas henia gravis need a C or M I o he ches o rule ou hymoma. Pa ien s wi h any signs or symp oms o respira ory compromise need immedia e neurologic evalua ion or possible hospi al admission and rea men . Dif erential Diagnosis Ea on–Lamber syndrome Chronic progressive ex ernal oph halmoplegia T ird nerve palsy Laboratory Tests Ace ylcholine recep or an ibody assay and single-f ber EMG Pathophysiology An au oimmune disorder in which au oanibodies at ack he recep ors o he neuromuscular junc ion Treatment rea men o he disease is sys emic and may include pyridos igmine bromide (Mes inon), prednisone, and possible hymec omy.

Neurogenic Ptosis

rea men should be coordina ed by a neuro-oph halmologis or neurologis . Once maximum medical improvemen has been achieved, surgical correc ion o he p osis can be at emp ed.

83

Fron alis suspension is usually required. Prognosis Variable depending on he severi y o he disease

FIGURE 5-14. Myasthenia gravis. Bila eral p osis wi h inabili y o keep he lids rom covering he pupils. T ere is also decreased one o he acial muscles.

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MA CUS GUNN JAW-WINKING SYND OME his syndrome may be very mild or very drama ic wi h eleva ion o he eyelid wi h each jaw movemen when chewing ood. rea men is required i he p osis or he eyelid movemen is signif can . Epidemiology and Etiology Age: Presen a bir h E iology: A congeni al synkine ic syndrome caused by a congeni al aberran connec ion o he oculomo or nerve f bers ha innerva e he leva or muscle and he rigeminal nerve f bers o he muscles o mas ica ion.

Movemen o he mandible la erally, o he con rala eral side mos commonly, resul s in eleva ion o he eyelid ( Fig. 5-15). Special Considerations T e amoun o p osis and he amoun o synkine ic movemen will de ermine he rea men . Dif erential Diagnosis Congeni al p osis Laboratory Tests None

History A care aker o en f rs no ices his condi ion when eeding he baby. T e a ec ed eyelid will move up and down wi h he jaw movemen during eeding.

Treatment I he amoun o synkine ic movemen is small, hen a ron alis suspension is done. I he synkine ic movemen is large, hen he leva or muscle mus be disinser ed and excised be ore he ron alis suspension can be done.

Examination A unila eral p osis wi h poor leva or uncion. T e unila erally p o ic eyelid eleva es wi h movemen o he jaw.

Prognosis I may be di cul o ge good symme ry in he eyelids unless bo h eyes are opera ed on.

Neurogenic Ptosis

85

A FIGURE 5-15. Marcus Gunn jaw winking syndrome. A. Pa ien wi h severe p osis wi h ull eyebrow eleva ion and chin up posi ion o keep his eyelids above he pupil. ( continued)

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B

C FIGURE 5-15. (Continued) Marcus Gunn jaw winking syndrome. B. Wi h opening o his mou h, he eyelids go up and he is able o normalize his head posi ion. C and D. More common unila eral jaw winking where lef upper lid open wi h opening o he mou h. ( continued)

Neurogenic Ptosis

D FIGURE 5-15. (Continued)

87

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5 EYELID MALPO SITIO NS

HO NE ’S SYND OME

H

orner’s syndrome classically presen s wi h mild p osis (2 mm) and pupillary miosis. A er any serious e iology has been ruled ou , hese cases respond very well o surgical correc ion wi h a müllerec omy.

Epidemiology and Etiology Age: May be congeni al or acquired E iology: E iology o acquired cases includes rauma, surgical procedures in he neck area, apical lung malignancies, aneurysm, dissec ion o he caro id ar ery, and idiopa hic. History Mild p osis is no ed. T e pupillary miosis may or may no be no ed un il he examina ion. Examination P osis, pupillary miosis, and anhidrosis are he hree f ndings. T e p osis is usually mild (1 o 2 mm). In congeni al Horner’s syndrome, here is also decreased pigmen a ion o he iris on he involved side ( Fig. 5-16). Special Considerations Cocaine es ing is used o conf rm he diagnosis. A 4% o 10% cocaine solu ion will dila e a normal pupil bu will ail o dila e a pupil a ec ed by Horner’s syndrome.

O her pharmacologic es ing will di erenia e f rs - and second-order neuron in errupion rom hird-order neuron. Hydroxyamphe amine drops will no dila e he pupil o a hird-order neuron Horner’s syndrome. A hird-order neuron Horner’s syndrome is generally o benign e iology. Dif erential Diagnosis Aponeuro ic p osis wi h pupillary anisocoria Laboratory Tests Ches C and M I/ MR wi h gadolinium o he neck and brain in all pa ien s wi h f rs - or second-order neuron involvemen Many neurologis s will image all pa ien s wi h Horner’s syndrome. Pathophysiology In errup ion o he sympa he ic innervaion o Müller’s muscle resul s in he p osis, whereas he dila or muscle o he iris resul s in he miosis. Treatment Once de ermined o be o benign e iology, a müllerec omy is he procedure o choice. Prognosis T e p osis responds well o surgical correc ion.

Neurogenic Ptosis

89

A

B FIGURE 5-16. Horner’s syndrome. A. P osis wi h miosis on he lef side. T e amoun o p osis is more han is of en seen in Horner’s syndrome and here may be some aponeuro ic dehiscence as well. B. Congeni al Horner’s syndrome wi h p osis, miosis, and iris hypopigmen a ion.

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MECH ANICAL PTOSIS

M

echanical p osis is drooping o he eyelid rela ed o res ric ion o he eyelid rom ei her scar issue or he weigh o a mass or swelling. Epidemiology and Etiology Age: Any age Gender: Equal E iology: T e increased weigh o any mass will weigh he eyelid down. T is can include chalazion, skin carcinoma, gian papillary conjunc ivi is, hemangiomas, neurof bromas, and so or h. es ric ion o eyelid movemen by scar issue will also produce his orm o p osis. History apidi y o onse varies according o he process Chalazion will have rapid onse , whereas a large basal cell carcinoma may slowly worsen over years. Examination P osis wi h an eyelid mass or evidence o scar issue ( Fig. 5-17) T e cause can be ex ernal or under he eyelid and di cul o see such as in severe gian papillary conjunc ivi is.

Eyelid scarring in ernally requires palpaion and s re ching and eversion o he eyelid o iden i y i . Special Considerations Imaging, such as C , may be needed o de ermine he ex en o he mass or o rule ou a oreign body or a rac ure a er rauma. Dif erential Diagnosis rauma ic p osis Aponeuro ic p osis Laboratory Tests None Treatment Addressing he cause is he primary rea men . Managemen can include medical rea men or surgical excision o a lesion or scar issue. Some pa ien s may require a second surgery o correc he p osis i removing he mechanical cause is no cura ive. Prognosis ela ed o prognosis o he mass or swelling ha is causing he p osis. I i is he resul o a recurren process, he prognosis is poor.

Mechanical Ptosis

91

FIGURE 5-17. Mechanical ptosis.N euro broma o he lef upper lid weighing down he eyelid and causing a p osis.

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TRAUMATIC PTOSIS rauma ic p osis has mul iple causes; however, exac ly which causes are presen in each case can be di cul o de ermine. Allowing 6 mon hs or spon aneous resoluion be ore correc ion is he rule in all rauma ic cases. Epidemiology and Etiology Age: All ages Gender: Males more common E iology: May include mul iple causes including myogenic, neurogenic, and aponeuro ic injuries History rauma o he eyelid wi h residual p osis a er he swelling resolves Examination Documen a ion o he severi y o p osis will allow moni oring or improvemen . Assessmen o leva or unc ion also helps de ermine e iology.

Any residual swelling, scarring, and lagoph halmos should also be documen ed ( Fig. 5-18). Special Considerations Mus wai 6 mon hs or possible resolu ion o he p osis be ore repairing. A number o cases will improve or resolve during his ime. Any injury ha sugges s direc cut ing o he leva or should have explora ion o he leva or a he ime o rauma repair i possible. Treatment Moni or or improvemen over he 6 mon hs ollowing he rauma. I he lid is s ill p o ic a er 6 mon hs, surgical correc ion wi h ex ernal leva or resec ion or ron alis suspension depending on leva or unc ion is indica ed. Prognosis Good i here is good leva or unc ion. I here is poor leva or unc ion wi h scarring, he prognosis is no as good.

Traumatic Ptosis

93

FIGURE 5-18. raumatic ptosis. Eyelid and eyebrow lacera ions resul ing in a rauma ic p osis. T is p osis did no improve over 6 mon hs and required surgical repair.

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5 EYELID MALPO SITIO NS

PSEUDOPTOSIS

P

seudop osis is he alse illusion o p osis caused by malposi ion o he eye, conrala eral eyelid, or lack o orbi al volume. I is very impor an o recognize a pseudop osis o avoid doing unnecessary or he wrong surgery. Epidemiology and Etiology Age: Any age Gender: Equal E iology: T e eyelid posi ion is normal bu o her ac ors resul in he appearance o p osis. T e mos common is an abnormal posi ion o he eye. Eleva ion o he eye or he eye sinking in will bo h give a alse p osis. Derma ochalasis (see “Derma ochalasis”) is also a common cause o pseudop osis. History Pa ien presen s wi h he complain o a droopy eyelid or a variable period. Examination In all pa ien s wi h p osis, evalua ion mus include no ing he posi ion o he eye. Enoph halmos, hyper ropia, and a globe pushed superiorly all cause pseudop osis.

Evalua ion o he eyelid skin rela ive o he rue eyelid margin posi ion is impor an ( Fig. 5-19A). Eyelid re rac ion on he con rala eral side mus also be considered. Special Considerations C scanning may be needed i a mass pushing he globe up is suspec ed. Dif erential Diagnosis Enoph halmos ( Fig. 5-19B) Hyper ropia Globe malposi ion Lid re rac ion o he con rala eral eyelid Derma ochalasis Treatment Depends on he cause o he pseudop osis Enoph halmos, microph halmos, ph hisis bulbi: build up he orbi Hyper ropia: consider s rabismus surgery Globe eleva ion: remove mass Con rala eral eyelid re rac ion: correc eyelid re rac ion Derma ochalasis: blepharoplas y Prognosis Good

Pseudoptosis

95

A

B FIGURE 5-19. Pseudoptosis. A. Derma ochalasis is he mos common cause o pseudop osis. I he eyelid skin is eleva ed, he eyelid is in a normal posi ion under he skin. B. Ano her orm o pseudop osis is enoph halmos resul ing in drooping o he eyelid. T is pa ien may also have some componen o leva or aponeurosis disinser ion.

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BROW PTOSIS

B

row p osis is drooping o he eyebrows. I is o en a par o derma ochalasis bu mus be recognized and rea ed separa ely or he bes resul s. Many pa ien s will have some degree o brow p osis and no have symp oms. Symp oms can include loss o superior visual f eld, brow ache and a igue, and rhy ids o he orehead and brow. T ose wi h signif can brow p osis may require eyebrow surgery wi h or wi hou eyelid surgery.

Epidemiology and Etiology Age: More common as pa ien s age Gender: Equal. Females are more likely o be symp oma ic. Epidemiology: Wi h aging, gravi y, involu ional changes, and loss o elas ici y resul in drooping o he eyebrows. Depending on mul iple ac ors, his drooping will become symp oma ic in hose 50 years o age or older.

T is may give he appearance o excess upper eyelid skin. Pa ien s will also develop urrows o he orehead rom chronically eleva ing heir eyebrows ( Fig. 5-20). Special Considerations Brow droop will add o redundan skin o he upper eyelids. I is impor an o recognize how much o he excess skin on he upper eyelids will go away i he eyebrows are eleva ed. T is excess rom he brow droop should no be excised during blepharoplas y or he eyebrows will appear oo close o he eyelids. Ideally, he brow should be li ed and hen he blepharoplas y per ormed.

History Pa ien s will at emp o li heir eyebrows resul ing in deep urrows o he orehead, brow ache, and even headaches.

Treatment Brow li using one o he ollowing echniques; each has i s own indica ions and advan ages. Endoscopic eyebrow li Coronal eyebrow li Mid orehead eyebrow li Direc eyebrow li

Examination Eyebrows si below he superior orbi al rim.

Prognosis Good

BrowPtosis

97

FIGURE 5-20. Brow ptosis.T e eyebrows are well below he orbi al rim in his pa ien , adding o he apparen amoun o derma ochalasis.

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5 EYELID MALPO SITIO NS

DERMATO CH ALASIS

D

erma ochalasis is a very common condi ion which increases as people age. Loss o elas ici y rom ul raviole exposure and aging resul s in excess skin o he upper and lower lids. I severe enough, he upper eyelid skin may cause unc ional blockage o he superior visual f eld. More commonly, he excess skin and he associa ed an erior prolapse o orbi al a is a cosme ic de ormi y.

Epidemiology and Etiology Age: Older pa ien s Gender: Equal male- o- emale ra io E iology: E iology is loss o eyelid skin elas ici y rom aging and ul raviole ligh exposure. T ere may be a heredi ary componen o derma ochalasis, especially when i occurs a a younger age.

History Symp oms are brow ache, heaviness around he eyes, and loss o superior visual f eld. T ese have a slow, insidious onse . Examination Excess skin o he upper eyelids o varying degrees I becomes especially bo hersome a er he skin con ac s he eyelashes. Derma ochalasis is o en associa ed wi h an erior prolapse o orbi al a . Underlying he excess skin, he possibili y o a rue p osis mus be evalua ed ( Fig. 5-21). Treatment Blepharoplas y, which may be unc ional or cosme ic in na ure. Prognosis Good

Dermatochalasis

99

FIGURE 5-21. Dermatochalasis.T ere is a large amoun o overhanging skin o bo h upper eyelids. T e underlying eyelid posi ion is normal. T ere is also some brow droop, which was addressed surgically a he same ime as he blepharoplas y.

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BLEPH ARO CH ALASIS

B

lepharochalasis is a rare, amilial varian o angioneuro ic edema ha occurs in younger individuals. I is charac erized by recurren episodes o in amma ory edema o he eyelids ha resul s in s re ching o he issues; over ime, he eyelids ake on he appearance o derma ochalasis commonly seen in much older pa ien s.

Epidemiology and Etiology Age: Onse in eens o 20s Gender: More common in emales E iology: Unknown; a varian o angioneuro ic edema

T ere may also be rue p osis, lacrimal gland prolapse, and prominen vessels o he lids. Mos commonly, hese f nding are unila eral. Pa ien s may also be seen a he ime o swelling wi h a swollen, uid-f lled lid wi h very lit le in amma ory signs. Dif erential Diagnosis Derma ochalasis T yroid-rela ed oph halmopa hy Orbi al in amma ory disease Pathophysiology Unknown

History ecurren episodes o eyelid swelling, usually unila eral

Treatment rea men is surgical excision o he skin and correc ion o p osis. Surgical repair can be complica ed by recurren edema, which may resul in recurrence o he problem.

Examination Excess skin o he eyelids ha is very hin and paper-like ( Fig. 5-22)

Prognosis Variable, depending on whe her he edema con inues o recur or burns ou

Blepharochalasis

101

FIGURE 5-22. Blepharochalasis.T is is a 25-year-old pa ien wi h recurren swelling o he righ eyelids resul ing in he hin, s re ched, redundan skin o he upper and lower eyelid.

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EYELID RETRACTION

E

yelid re rac ion is displacemen o he eyelid oward he respec ive superior or in erior orbi al rim, resul ing in scleral show. T is condi ion can be mild and wi hou symp oms or can resul in corneal exposure. T yroid oph halmopa hy is he mos common cause and eyelid re rac ion is o en he ini ial sign o his disease. Epidemiology and Etiology Age: Adul hood. are in children. Age o onse dependen on e iology. Gender: Females more common E iology: Eyelid re rac ion is caused by hyroid oph halmopa hy mos commonly, ollowed by overaggressive eyelid surgery and ver ical rec us muscle surgery ( Fig. 5-23). Upper eyelid re rac ion may be he resul o con rala eral p osis. Lower lid re rac ion can be a normal ana omic varian . Parinaud syndrome is a cen ral nervous sys em cause o upper eyelid re rac ion. History In hyroid oph halmopa hy, he pa ien no es slow onse o one or bo h eyes appearing oo wide open or having he “hyper hyroid s are” O en, redness and irri a ion o he a ec ed eye accompany hese symp oms. Pa ien s may have a his ory o sys emic hyroid abnormali ies. T ose pa ien s wi h a surgical cause will give he his ory o eyelid or eye muscle surgery. Examination Documen he amoun o eyelid re rac ion and whe her here is re rac ion or p osis on he o her side. Upper eyelid lag on down gaze, prop osis, and dysmo ili y all go along wi h hyroid ophhalmopa hy. No e signs o corneal exposure and previous eye or eyelid surgery.

Dif erential Diagnosis Malposi ion o he globe Hypo ropia or hyper ropia Con rala eral p osis Laboratory Tests T yroid unc ion es s unless pa ien has known, con rolled hyroid abnormali ies or here is a known surgical cause o he eyelid re rac ion. Pathophysiology T yroid oph halmopa hy resul s in chronic in amma ion o he lid re rac ors leading o scar issue orma ion and re rac ion o he eyelids. Treatment In hyroid oph halmopa hy, he disease mus be rea ed so i is inac ive be ore any surgical correc ion. rea corneal exposure wi h lubrica ion while wai ing or he disease o become inac ive. Mos causes o eyelid re rac ion will require surgical rea men i hey are signif can . ecession o he re rac ors is he mos common procedure used. T is works or mild o modera e re rac ion. More severe re rac ion o he lower eyelids requires eyelid spacer ma erial o be implan ed. Excess excision o skin o he upper or lower lids during blepharoplas y may require in ernal spacers or rarely skin gra s. Prognosis Generally, re rac ion can be rea ed success ully wi h surgery. Corneal exposure is o en an ongoing problem ha is improved wi h rea men bu no comple ely cured.

Eyelid Retraction

103

A

B FIGURE 5-23. Eyelid retraction. A. Lef , lower eyelid re rac ion rom scarring o he eyelid o he orbi al rim and a i anium pla e af er rauma and orbi al rac ure repair. B.T yroid-rela ed oph halmopa hy wi h upper eyelid re rac ion.

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EYELID DYSKINESIS BENIGN ESSENTIAL BLEPHA OSPASM

B

enign essen ial blepharospasm is a bila eral condi ion charac erized by involunary spasms o he orbicularis oculi, procerus, and corruga or muscles. T is condi ion may s ar as mild wi ching o he eyelids and can progress so ha hese con rac ions leave he pa ien s unc ionally blind during he con racion. Pa ien s canno predic when he spasms will occur and he orced closure o he eyelids can be li e- hrea ening i hey occur during driving, crossing s ree s, and so or h. Epidemiology and Etiology Age: Onse is when pa ien s are 40 years o age or older. Gender: Women more commonly a ec ed han men E iology: Unknown, bu probably o cenral nervous sys em origin, mos likely in he basal ganglia. T e process causes involun ary spasms o he orbicularis oculi, procerus, and corruga or muscles. History T e spasms s ar as mild wi ches and progressively worsen wi h ime. Pa ien s o en do no presen un il he spasms are severe enough o in er ere wi h ac ivi ies o daily living. Examination Pa ien s have in ermit en episodes o orced eyelid closure ha usually las or minu es ( Fig. 5-24). Be ween spasms, he examina ion may be normal. T us, he diagnosis is o en based on his ory.

T e spasms are bila eral, al hough hey can some imes be more severe on one side han he o her. Spasms can involve he lower ace and neck wi h ime. Spasms do no occur during sleep, unlike in hemi acial spasm. Special Considerations Mus rea any condi ion ha may cause ocular irri a ion and hus worsen he blepharospasm such as dry eyes. Blepharospasm can have a al consequences i no con rolled and he pa ien drives. Dif erential Diagnosis Hemi acial spasm Severe dry eyes or o her ocular irri a ion Treatment Bo ulinum oxin injec ion. T ese injec ions are e ec ive or mos pa ien s and las 3 o 4 mon hs be ore reinjec ion is required. Wi h ime, in some pa ien s hese injecions may become less e ec ive and par ial surgical excision o orbicularis muscle and o her pro rac ors will be required. Bo ulinum oxin injec ion may s ill be needed bu will be more e ec ive a er surgery. Muscle relaxan s and seda ives are occasionally used in his disease bu are o lit le benef . Prognosis Good wi h bo ulinum oxin injec ion are cases may no respond o rea men .

Eyelid Dyskinesis

105

FIGURE 5-24. Benign essential blepharospasm.T is pa ien would develop blepharospasm wi h any at emp o ouch he eyes. T e spasm can go on o involve o her acial muscles.

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HEMIFACIAL SPASM Epidemiology and Etiology Age: Adul hood Gender: Equal male o emale ra io E iology: Vascular compression o he acial nerve a he level o he brains em History Unila eral spasm o one side o he ace Examination T e pa ien may have mild acial palsy on he a ec ed side. T e spasm may be seen on examina ion or no ed by he pa ien ’s his ory ( Fig. 5-25). Special Considerations Spasms are presen during sleep, unlike benign essen ial blepharospasm where hey are absen .

Dif erential Diagnosis Benign essen ial blepharospasm Myokymia Treatment T e pa ien needs M I o he cerebellar– pon ine angle o rule ou a mass lesion. Bo ulinum oxin is hen generally he rea men o choice. Neurosurgical decompression o he acial nerve is some imes considered. Prognosis Bo ulinum A oxin con rols he spasm bu requires repea injec ion every 3 o 6 mon hs.

Eyelid Dyskinesis

107

FIGURE 5-25. Hemifacial spasm. Hemi acial spasm o he lef side o he ace. T e spasm is usually in ermit en .

C H AP T ER

Congeni al Eyelid Anomalies BLEPH AROPHIMOSIS

B

lepharophimosis is a congeni al eyelid syndrome ha has a charac eris ic eyelid appearance and includes elecan hus, epicanhus inversus, and severe myogenic p osis. Epidemiology and Etiology Age: Congeni al Gender: Equal Inheri ance: Au osomal dominan E iology: Unknown

History O en have amily members wi h he same syndrome Examination Charac eris ic eyelid f ndings include elecan hus, epican hus inversus, and severe p osis. O her f ndings, which may or may no be presen , include lower eyelid ec ropion, a poorly developed nasal bridge, hypoplasia o he superior orbi al rims, hyper elorism, 108

mo ili y disorders, and various degrees o men al def ciency ( Fig. 6-1). Dif erential Diagnosis No o her syndrome gives hese characeris ic changes. Mus di eren ia e rom simple epican hus (see Fig. 6-2) and elecan hus. Treatment Mul iple s ages o recons ruc ion are required. Ini ial surgery is aimed a he elecan hus and epican hus inversus. T is may require a simple Z-plas y, Y–V plas y, or ransnasal wiring. T e second s age is correc ion o he p osis, which usually requires ron alis suspension. Finally, o her eyelid abnormali ies are addressed. Prognosis Signif can improvemen can be made wi h surgery. Depending on he severi y, here will always be some eyelid changes ha remain.

Blepharophimosis

109

FIGURE 6-1. Blepharophimosis. T is child has classic changes o blepharophimosis with ptosis, telecanthus, and epicanthus inversus. T is must be dif erentiated rom simple epicanthus ( see Fig. 6-2) .

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6 CO NGENITAL EYELID ANO MALIES

EPICANTHUS

E

pican hus is a medial can hal old ha is usually caused by imma ure mid acial bones. T e condi ion is usually bila eral and will resolve as he child’s ace ma ures. Epican hus is associa ed wi h cer ain eyelid syndromes.

Epidemiology and Etiology Age: Congeni al Gender: Equal E iology: Imma ure mid acial bones are considered o be he cause. History No ed a bir h Examination T ere is an ex ra old o skin and subcu aneous issue medially involving he eyelids. T is may make he child appear eso ropic. Four ypes o epican hus have been described: Epican hus arsalis: Fold is more prominen on he upper eyelid. Epican hus inversus: Fold is more prominen on he lower eyelid.

Epican hus palpebralis: Fold is equally on he upper and lower eyelids ( Fig. 6-2). Epican hus superciliaris: Fold runs rom he eyebrow region o he lacrimal sac. Special Consideration Epican hus arsalis can be a normal varian o he Asian eyelid. Epican hus inversus is par o blepharophimosis syndrome. Dif erential Diagnosis Blepharophimosis Treatment Mos cases o epican hus resolve wi h normal acial ma ura ion, so any po en ial rea men should be delayed un il he child is ma ure. T e excep ion is epican hus inversus, which rarely disappears wi h acial ma ura ion. I surgical rea men is required, hese cases respond well o a Y–V-plas y or Z-plas y. Prognosis Excellen . Mos cases resolve as he child grows. I surgery is required, he resul s are good.

Epicanthus

111

FIGURE 6-2. Epicanthal folds. T ese olds can o en be seen as an isolated nding in young children. Unless severe, these epicanthal olds will lessen and even disappear as the child’s ace matures.

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6 CO NGENITAL EYELID ANO MALIES

EPIBLEPH ARON

E

piblepharon is override o he pre arsal muscle and skin, which causes he cilia o assume a ver ical posi ion al hough he eyelid margin is in a normal posi ion. T is disorder is usually asymp oma ic wi h no corneal s aining and requires no rea men .

Epidemiology and Etiology Age: Congeni al Gender: Equal E iology: Imma ure acial bones are el o allow or his excess skin and muscle. History T ere are usually no symp oms. Examination T ere is an excess o skin overriding he eyelid margin, which may even come in con ac wi h he eye. I his skin can be pulled back, he eyelid margin under i is in a normal posi ion. T ere is rarely any corneal s aining.

I here are corneal changes, considera ion mus be given o surgical correc ion ( Fig. 6-3). Special Considerations I is o en very di cul o di eren ia e epiblepharon rom congeni al en ropion, especially in an awake child who is squeezing his or her eyes shu . I here are signif can corneal changes, hen i is likely he eyelid is en ropic and surgery is required. Dif erential Diagnosis Congeni al en ropion Treatment No rea men is needed in mos cases. I here are corneal changes, hen excision o he excess skin and muscle is he rea men o choice. Prognosis Excellen . Mos cases resolve as he acial bones ma ure. T e rare case ha requires surgery will respond well.

Epiblepharon

113

A

B FIGURE 6-3. Epiblepharon (A&B). Excess skin o the lower eyelid rolls in and touches the cornea. T e eyelid is in a normal position and the cornea is normal. T is condition must be dif erentiated rom congenital entropion (see Fig. 6-4).

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6 CO NGENITAL EYELID ANO MALIES

CONGENITAL ENTROPION

C

ongeni al en ropion is rare bu can also be di cul o diagnose in an in an . An eye ha is always irri a ed and ha he child does no wan o open is a clue o look or an epi helial de ec or corneal scar. Epidemiology and Etiology

Age: A bir h Gender: Equal E iology: Usually rela ed o an abnormali y o he eyelid re rac ors or arsus. Very rarely, here can be conjunc ival scarring causing he en ropion. History T e child’s eye is always irri a ed, and he child does no wan o open he eye. Examination I is di cul o examine he eyelid o an in an unless hey are asleep. When he child

is awake and an at emp is made o examine he eyelid, he child squeezes he eye shu and a normal eyelid may urn in. Evidence o corneal scarring or an epi helial de ec on he in erior cornea is enough o suspec an en ropion ( Fig. 6-4). Dif erential Diagnosis Epiblepharon Treatment Surgical correc ion is required. Excision o pre arsal skin and orbicularis wi h igh ening o he re rac ors is he rea men o choice. Prognosis Good. Some chance o corneal scarring i no diagnosed early.

Congenital Entropion

115

A

B

C FIGURE 6-4. Congenital entropion. A. It is di cult to examine a child’s eyelid to determine whether it is entropic, especially when the eye is already irritated. In this child, there is corneal scarring rom a congenital entropion. B. Child immediately a er placing rotating sutures in the lower eyelid. C. Postoperative picture 4 weeks a er entropion surgery. T e eyelid is in a normal position and the corneal opacity is resolving.

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6 CO NGENITAL EYELID ANO MALIES

CONGENITAL COLOBOMA

C

ongeni al colobomas are ull hickness de ec s in he eyelid. Even larger de ec s are usually well olera ed over he shor erm un il he de ec can be repaired. Upper eyelid colobomas are usually no associa ed wi h o her sys emic abnormali ies, whereas lower eyelid colobomas are more commonly associa ed wi h acial cle syndromes.

Epidemiology and Etiology Age: Apparen a bir h Gender: Equal E iology: Abnormal embryonic developmen resul s in hese eyelid de ec s. History Eyelid de ec is usually no ed a bir h or soon a er. T ere are ew symp oms. Examination T e ull- hickness de ec is mos commonly medially on he upper eyelid.

Colobomas in his loca ion are no usually associa ed wi h any o her abnormali y. A coloboma o he lower eyelid is more likely o be par o a acial cle syndrome and may have o her acial de ec s and lacrimal abnormali ies. At en ion mus be given o he cornea or signs o exposure, al hough exposure is rare ( Fig. 6-5). Dif erential Diagnosis Bir h rauma o he eyelid Treatment Surgical repair o he coloboma is usually s raigh orward and can be done wi hou any aps ha would occlude he eye and cause amblyopia. Prognosis Colobomas do very well wi h surgical repair. O her acial de ec s may no be as easy o repair.

Congenital Coloboma

117

A

B FIGURE 6-5. Congenital coloboma. A. Child born with a coloboma o the upper eyelid. T is may be a totally isolated nding, but coloboma and a preauricular skin tag (B) are consistent with Goldenhar’s syndrome.

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6 CO NGENITAL EYELID ANO MALIES

CONGENITAL DISTICHIASIS

D

is ichiasis is a rare condi ion where an ex ra row o eyelashes replaces he meibomian gland openings on he eyelids. Epidemiology and Etiology Age: Presen a bir h

Gender: Equal E iology: Embryonic pilosebaceous uni s improperly di eren ia e in o hair ollicles. History T e ex ra row o lashes may be no ed or eye irri a ion may promp oph halmic evaluaion a which ime he problem is discovered. Examination A second row o eyelashes is no ed growing pos erior o he normal eyelash posi ion ( Fig. 6-6). T ese lashes may be in con ac wi h he cornea causing symp oms o eye irria ion, corneal punc a e s aining, and scarring. A good corneal evalua ion is impor an .

Dif erential Diagnosis Congeni al en ropion Conjunc ival scarring causing richiasis Treatment rea men is based on he symp oms and individualized. I rea men is required, op ions are variable. Conserva ive rea men wi h lubrica ion and con ac lenses is o en no success ul. Eyelash abla ion wi h cryo herapy or elec rolysis o en resul s in recurrence o eyelashes bu will be adequa e in some pa ien s. Surgical excision o he eyelashes and recons ruc ion—some imes using buccal mucosal gra s—works in he mos severe cases. Prognosis Usually good, bu mul iple procedures may be required and here may be undesirable cosme ic de ec s a er surgery.

Congenital Distichiasis

119

FIGURE 6-6. Congenital distichiasis. All our eyelids have these extra rows o eyelashes growing out o the position where the meibomian glands should be.

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6 CO NGENITAL EYELID ANO MALIES

ANKYLOBLEPH ARON

C

ongeni al ankyloblepharon is a ailure o he eyelids o separa e during embryonic developmen . Ankyloblepharon may also be acquired due o scarring, which resul s in adherence o he eyelids o each o her and o he globe. Epidemiology and Etiology Age: Congeni al. Acquired cases can occur a any age depending on he cause. Gender: Equal E iology: Congeni al ailure o he eyelids o separa e during embryonic developmen . Acquired ankyloblepharon is mos commonly he resul o progressive conjunc ival scarring resul ing in usion o he eyelids. Some causes include ocular cica ricial pemphigoid, S evens–Johnson syndrome, chemical burns, and herpes zos er. History Congeni al cases no ed a bir h Acquired cases will usually have a his ory o progressive scarring rela ed o he primary disease. Examination Congeni al ankyloblepharon may have comple e usion o he eyelids or jus a ew bands holding he eyelids oge her.

T e eye and orbi may be normal or have associa ed abnormali ies ( Fig. 6-7A). Acquired ankyloblepharon shows usion o he eyelids rom scar issue. T e eye i sel is no able o be seen ( Fig. 6-7B). Dif erential Diagnosis Cryp oph halmos Microph halmos Treatment Congeni al ankyloblepharon: lysis o he bands holding he eyelids oge her O her recons ruc ive procedures may be needed depending on severi y. Acquired ankyloblepharon: de ermining he e iology o he scarring is done f rs . I his process needs rea men o quie any in amma ion, hen ha mus be done f rs . At emp s o recons ruc he eyelids and resur ace he cornea can hen be at emp ed. T is requires coordina ion be ween oculoplas ic and corneal surgeons. Prognosis Congeni al ankyloblepharon: good Acquired ankyloblepharon: poor in mos cases. T e process causing he scarring will o en hamper he healing a er eyelid and corneal recons ruc ive surgery.

Ankyloblepharon

121

A

B FIGURE 6-7. Congenital ankyloblepharon. A. Unilateral combined ankyloblepharon and cryptophthalmos. (Courtesy Richard W. Hertle, MD.) Ankyloblepharon. B. Acquired ankyloblepharon as the result o scarring rom ocular cicatricial pemphigoid. T e eyelids are used, and there is likely scarring o the eyelids to the globe as well.

C H AP T ER

Miscellaneous Eyelid Condi ions OCULAR CICATRICIAL PEMPHIGOID

O

cular cica ricial pemphigoid (OCP) is a conjunc ival scarring disease ha occurs in older adul s. I can be mild or can be progressive and lead o corneal scarring and blindness. OCP con inues o be a con using, poorly unders ood condi ion ha can be very di cul o rea in some pa ien s. Epidemiology and Etiology Age: Older adul s Gender: More common in emales E iology: An au oimmune process in which an ibodies bind o he conjunc ival basemen membranes, resul ing in in ammaion and scarring. History T ere may be a long his ory o ocular irri a ion and epila ion o eyelashes over many years. T e o her ex reme is rapidly progressive conjunc ival and even corneal scarring wi h very red in amed eyes. 122

Some pa ien s will have ulcera ion o o her mucosal sur aces such as oral, esophageal, or geni al lesions. Skin lesions may also be par o he presen a ion. A signif can number o hese pa ien s have used or are curren ly using an iglaucoma drops. Examination Findings range rom mild, sub le conjunc ival scarring in he early s ages o severe scarring where he eyelid is s uck o he cornea. Cica ricial en ropion, richiasis, and severe dryness all add o he poor ocular sur ace. T e condi ion o he cornea is impor an in guiding rea men . Evalua ion o he mou h and skin or o her lesions is impor an ( Fig. 7-1). Special Considerations Some pa ien s on an iglaucoma medicaions will ge conjunc ival scarring ha is no progressive i he medica ion is s opped. T is f nding was more common in pa ien s using mio ics, such as pilocarpine, bu also seems o be associa ed wi h some o he modern an iglaucoma drops.

Ocular Cicatricial Pemphigoid

I is no clear whe her hese pa ien s have OCP or i he scarring is en irely rela ed o he drops. Dif erential Diagnosis S evens–Johnson syndrome Acid and alkali burns Previous eyelid surgery rachoma A opic disease Laboratory Tests Immuno uorescence es ing o he conjunc iva will reveal immunoglobulins a he basemen membrane in OCP. A posi ive biopsy is diagnos ic, bu a negaive biopsy does no rule ou OCP because here are a signif can number o biopsynega ive OCP cases. Pathophysiology An au oimmune process in which immune complexes bind a he conjunc ival basemen

123

membrane ha resul s in in amma ion and even ual scarring. T is des roys he ear glands o he conjunc iva and causes in- urned eyelids, lashes and corneal scarring. Treatment T e in amma ion mus be quie ed f rs . T is may require only doxycycline in very mild cases or s rong medica ions such as cyclophosphamide, mycophenola e mo e il or aza hioprine in re rac ory cases. A er he in amma ion is quie , eyelid problems, such as richiasis or en ropions, can be addressed surgically. All pa ien s will require aggressive lubrica ion and/ or punc al occlusion. Prognosis Variable. Some pa ien s’ disease will burn ou or respond o rea men wi hou signif can ocular injury. In o her pa ien s, he disease can progress no mat er wha rea men is used.

124

7 MISCELLANEO US EYELID CO NDITIO NS

A

B FIGURE 7-1. Ocular cicatricial pemphigoid. A. Scarring o the eyelid to the cornea in this advanced case. B. Earlier in the disease, the conjunctival scarring is less obvious and may even be overlooked i the conjunctiva in the ornices is not care ully examined. ( continued)

Ocular Cicatricial Pemphigoid

125

C

D FIGURE 7-1. ( Continued) Ocular cicatricial pemphigoid. C.T e lower eyelid is entropic secondary to conjunctival scarring rom pemphigoid. D. Oral ulcerations are of en ound in active ocular cicatricial pemphigoid and help solidi y the diagnosis.

C H AP T ER

Lacrimal Obs ruc ions CONGENITAL OBSTRUCTIONS

Mos obs ruc ions will spon aneously resolve by he age o 6 o 12 mon hs.

CONGENITAL NASOLACRIMAL DUCT OBSTRUCTION ongeni al nasolacrimal duc obs ruc ion is seen in 2% o 6% o newborns bu resolves in he f rs 3 o 4 weeks in mos in an s. T e chronic purulen discharge is he main problem or caregivers, bu , wi h addi ional ime, a large percen age o hese obs ruc ions will resolve on heir own.

Examination Diagnosis is based mainly on he his ory. Examina ion may reveal increased ear f lm and some crus ing o he eyelashes. Mucus re ux wi h pressure over he lacrimal sac conf rms he diagnosis, bu is no always presen . Examina ion mus rule ou a dacryocys ocele or any sign o in ec ion ( Fig. 8-1).

Epidemiology and Etiology Age: Congeni al Gender: Equal in males and emales E iology: Incomple e developmen o he dis al lacrimal passage wi h a membranous block a he valve o Hasner

Dif erential Diagnosis Chronic conjunc ivi is Punc al dysgenesis En ropion richiasis

History Paren s will no e a chronic mucous discharge wi h mat ing o he eyelashes a 3 o 4 weeks o age in 2% o 6% o ull- erm in an s in one or bo h eyes.

Treatment iming o he rea men is con roversial. Nine y percen o all congeni al nasolacrimal duc obs ruc ions will resolve by age 12 mon hs.

C

126

Congenital Obstructions

Many physicians will use conserva ive rea men un il his ime. T is managemen consis s o massage wi h opical an ibio ics as needed o con rol he mucus discharge. Probing and irriga ion under general aneshesia will success ully rea 90% o pa ien s. T ose pa ien s no responsive o probing and irriga ion may require in uba ion wi h silicone ubes wi h or wi hou a balloon dacryoplas y.

127

T e rare pa ien will require a dacryocys orhinos omy. Prognosis rea men is very success ul. Wai ing or spon aneous resolu ion while he child has chronic discharge is o en di cul or he caregivers.

FIGURE 8-1. Congenital nasolacrimal duct obstruction. T ere is redness, crusting, and irritation o the right eyelids rom the chronic discharge. T e tear f lm is also increased. In many patients with congenital nasolacrimal duct obstruction, there will be no external signs and the diagnosis is based on the history the caregivers report.

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8 LACRIMAL O BSTRUCTIO NS

DACRYOCYSTOCELE

A

dacryocys ocele is a rare lesion no ed a bir h in he medial can hal area. I represen s uid and mucus rapped in he lacrimal sac. Dacryocys oceles will resolve bu mus be observed care ully because hey can become in ec ed. Epidemiology and Etiology Age: Congeni al Gender: Equal in males and emales E iology: Blockage o he lacrimal sys em dis ally, a he valve o Hasner, and proximally, a he valve o Rosenmüller, resul ing in rapped amnio ic uid and/ or mucus produced by he lacrimal sac goble cells.

I a mass is no ed above he medial can hal endon, ano her e iology mus be considered. I bila eral consider nasal exam and chance o respira ory obs ruc ion. Dif erential Diagnosis Hemangioma Meningoencephalocele Dacryocys i is

History Cys ic swelling o he medial can hus below he endon no ed a bir h

Treatment Observa ion or he f rs 1 o 2 weeks wi h massage Many will resolve on heir own. Probing is required i here is any sign o in ec ion or i here is no resolu ion a er 2 weeks.

Examination Prominen cys ic mass below he medial can hal endon ( Fig. 8-2)

Prognosis Excellen

FIGURE 8-2. Dacryocystocele. T e large, distended right lacrimal sac is easily seen and is f rm to palpation. T is child underwent probing and irrigation, which resolved the obstruction.

Congenital Obstructions

LACRIMAL FISTULA

A

lacrimal f s ula is an ex ra opening o he lacrimal sys em on o he skin usually loca ed in erior-nasal o he lacrimal punc um. One hird o f s ulas will have an associa ed lacrimal obs ruc ion wi h chronic mucous discharge. T e o her pa ien s are o en asymp oma ic. Epidemiology and Etiology Age: ypically congeni al, bu acquired f sulas can occur a an age. Gender: Equal in males and emales E iology: Abnormal embryonic developmen o he lacrimal sys em. Cases o acquired f s ulas are rela ed o dacryos enosis wi h dacryocys i is. History O en asymp oma ic unless here is an associa ed dacryos enosis

129

Examination Small cu aneous opening in erior and nasal o he medial can hal angle May or may no have ears exi ing rom i ( Fig. 8-3) Dif erential Diagnosis Mus de ermine i here is associa ed dacryos enosis Treatment I symp oma ic, he epi helial lined is ula can be excised. I here is also an associa ed dacryos enosis, a dacryocys orhinos omy and excision o he is ula is indica ed. Acquired f s ulas will disappear when he dacryocys i is resolves. Prognosis Excellen

FIGURE 8-3. Congenital lacrimal f stula. Note the very small opening in erior-nasal to the puncta, which is connected to the lacrimal system.

130

8 LACRIMAL O BSTRUCTIO NS

ACQUIRED OBSTRUCTIONS ACQUIRED NASOLACRIMAL DUCT OBSTRUCTION

A

cquired nasolacrimal duc obs ruc ion becomes more common as pa ien s age. T e obs ruc ion mos commonly occurs in he nasolacrimal duc . Pa ien s may presen wi h earing or an in ec ion. Many pa ien s may have an obs ruc ion and be wi hou symp oms. Epidemiology and Etiology Age: Older pa ien s Gender: Females mos commonly E iology: Involu ional changes in he lacrimal duc / sac is he mos common cause. Naso-orbi al rauma or surgery, sinusi is, and dacryocys i is are also causes. History Con inual earing, which may have been preceded by in ermit en episodes o earing. T e process is mos commonly unila eral bu may be bila eral. Examination Increased ear f lm on sli lamp examinaion wi h abnormal dye disappearance es

Def ni ive diagnosis made wi h irriga ion o he lacrimal sys em, which will demons ra e obs ruc ion o ow ( Fig. 8-4). Dif erential Diagnosis O her causes o earing such as: Kera i is sicca Blephari is Ec ropion Punc al abnormali ies Special Tests Dacryocys ography may help def ne lacrimal s enosis in di cul cases and in par ial obs ruc ions. Treatment Symp oma ic comple e obs ruc ion requires a dacryocys orhinos omy. Par ial obs ruc ions can be rea ed wi h balloon dacryoplas y. Prognosis Dacryocys orhinos omy is success ul 90% o he ime or more o en. Balloon dacryoplas y is 70% o 80% success ul bu is less invasive.

Acquired Obstructions

131

FIGURE 8-4. Acquired nasolacrimal duct obstruction. T ere are o en no external signs o acquired nasolacrimal duct obstruction. T ere are excess tears running down the cheek and slight injection o the right eye. I there is some dacryocystitis associated with the blockage, the eye may be red.

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8 LACRIMAL O BSTRUCTIO NS

CANALICULAR OBSTRUCTION Epidemiology and Etiology Age: Any Gender: More common in emales E iology: rauma, ex ernal conjunc ival in ec ions (Epidemic Kera oconjunc ivi is, herpes), canaliculi is, sys emic chemo herapy History Onse o earing may be gradual or acu e. Examination Increased ear f lm, normal eyelid posi ion, and evidence o canalicular obs ruc ion on probing o he canaliculi ( Fig. 8-5)

Special Considerations T ere may be an addi ional, more dis al, obs ruc ion in he lacrimal sac and duc in some o hese cases. Dif erential Diagnosis Kera i is sicca Blephari is Ec ropion O her lacrimal sys em abnormali ies Treatment Silicone in uba ion wi h or wi hou a dacryocys orhinos omy Prognosis Canalicular obs ruc ions have a poorer prognosis han more dis al obs ruc ions. Success is in he range o 50%, depending on he e iology.

Acquired Obstructions

133

FIGURE 8-5. Canalicular obstruction. External signs o herpes simplex are the only sign o the canalicular obstruction. Probing demonstrates canalicular scarring as a result o herpes simplex.

C H AP T ER

Lacrimal In ec ions DACRYOCYSTITIS Epidemiology and Etiology Age: Mos common in older adul s bu can be seen a any age. Gender: More common in emales E iology: Nasolacrimal obs ruc ion rom various causes wi h s asis o f uid in he lacrimal sac and even ual in ec ion. History May have acu e onse o pain and swelling over he lacrimal sac. O hers may give he hisory o chronic earing wi h chronic mucous discharge and a ender lump over he lacrimal sac. T ere may be a prolonged his ory o a chronic conjunc ivi is. Examination enderness over he lacrimal sac is he mos common nding. T e lacrimal sac may be enlarged wi h signi can swelling, or i may be rela ively small ( Fig. 9-1A). Similarly, he amoun o periorbi al swelling varies wi h he severi y o he in ec ion. 134

Orbi al celluli is mus be considered i he in ec ion is severe ( Fig. 9-1B). Pa ien s wi h a low-grade chronic in ec ion may have mucus/ pus expressible hrough he canaliculi wi h pressure over he lacrimal sac. T e conjunc iva may be injec ed. Probing and irriga ion should no be done in he set ing o an in ec ion. Special Considerations I a pa ien complains o blood expressed rom he lacrimal sys em, a lacrimal sac umor mus be considered and imaging done. In ec ions can give bloody discharge as well. Dif erential Diagnosis Lacrimal sac umor Laboratory Tests Cul ure and sensi ivi y o any ma erial expressed or drained rom he lacrimal sac Imaging C or MRI scanning may be needed i a lacrimal sac umor is suspec ed.

Dacryocystitis

135

I here is a ormed abscess o he sac, incision and drainage is indica ed.

Rare pa ien s will have an open lacrimal sys em a er he in ec ion is gone and will no require a dacryocys orhinos omy. Pa ien s who have had a dacryocys i is and have an obs ruc ed lacrimal sys em have an increased risk o recurren dacryocys i is.

Ul ima ely, when he in ec ion has resolved, mos pa ien s will require a dacryocys orhinos omy.

Prognosis Excellen unless he pa ien is immunocompromised

Treatment rea men o he acu e in ec ion is he rs priori y. Sys emic an ibio ics and warm compresses are he rea men o choice.

136

9 LACRIMAL INFECTIO NS

A

B FIGURE 9-1. Dacryocystitis. A.T is 68-year-old man has a ormed lacrimal sac mass that is tender with a surrounding mild cellulitis. B. A more severe dacryocystitis with surrounding cellulitis.

Canaliculitis

CANALICULITIS

C

analiculi is is a rare in ec ion involving he proximal lacrimal sys em. T e in ecion can be bac erial or ungal and is usually indolen . Diagnosising canaliculi is can be di cul because i o en presen s as a chronic conjunc ivi is and no un il la e does he lacrimal in ec ion become apparen . Epidemiology and Etiology Age: Usually older adul s E iology: Some abnormali y o he lacrimal sys em leads o concre ion orma ion and a chronic in ec ion. In ra-canalicular plugs have become a more common cause o canaliculi is. History Chronic mucous discharge, earing, and conjunc ivi is unresponsive o opical an ibio ics Ask abou any his ory o punc al plug placemen and he ype o plug placed. Examination T e diagnosis can be di cul o con rm unless i is suspec ed. An ery hema ous, pou ing, dila ed puncum, which is o en ender o palpa ion and very ender o probing, is o en presen .

137

T ere may be a ollicular conjunc ivi is and a chronic mucous discharge. Pressure over he canaliculus may express pus or concre ions ( Fig. 9-2A and B). T ere is more likely o be enderness over he canaliculus wi h an in ec ious e iology and less likely when rela ed o re ained puncal plugs ( Fig. 9-2C). Dif erential Diagnosis Chronic conjunc ivi is Migra ed punc al plug Laboratory Tests Cul ure and sensi ivi y o ma erial in canaliculus is help ul in de ermining rea men . Treatment Warm compresses and opical and sysemic an ibio ics are he ini ial rea men . Mos pa ien s will have concre ions or a plug in he canaliculus and he process will recur un il hese are removed wi h incision and drainage o he canaliculus. Prognosis Good once recognized. A second obs rucion lower in he lacrimal sys em may resul in a recurrence.

138

9 LACRIMAL INFECTIO NS

A

B FIGURE 9-2. Canaliculitis. A. A red, tender upper canaliculus with expression o pus with pressure over the canaliculus. B.T e lacrimal stones ound on opening the canaliculus. ( continued)

Canaliculitis

C FIGURE 9-2. (Continued) Canaliculitis. C. Intra-canalicular plug removed af er causing canaliculitis.

139

C H AP T ER

Lacrimal Sac umors

L

acrimal sac umors are rare and he e iology is widely varied rom benign o malignan . Any dacryos enosis or dacryocys i is has he po en ial o be a lacrimal sac umor. When

Classically, he mass may be above he medial can hal endon bu early in he course may presen like a dacryocys i is. Examination Findings vary rom being iden ical o dacryocys i is o a palpable mass in he lacrimal sac area. T e umor may be ound during dacryocys orhinos omy when here was no evidence o a umor preopera ively. I a umor is suspec ed, nasal examina ion by an o olaryngologis may help def ne i s ex en , along wi h C and/ or MRI scanning ( Fig. 10-1). Dif erential Diagnosis Dacryocys i is Laboratory Tests Biopsy o he lacrimal sac or any abnormal appearing lacrimal sac. Dacryocys ogram may be help ul. Imaging C or MRI scanning is needed i a lacrimal sac umor is suspec ed. I may no be able o di eren ia e a umor rom an enlarged sac

140

Lacrimal Sac Tumors

141

secondary o in ec ion bu will show a large erosive mass.

Care ul long- erm ollow-up is impor an or any lacrimal sac umor.

Treatment Comple e excision o any benign or malignan umor is impor an . Frozen sec ion con rol is required o ry o assure comple e excision. Benign papillomas may recur wi h malignan rans orma ion. Lymphomas are sensiive o irradia ion.

Prognosis Recurrence is no uncommon. Fi y percen o ransi ional and squamous cell carcinomas will recur, and 50% o hese recurrences will be a al.

A

B FIGURE10. 1 Lacrimal sac tumor. A.T e patient has ullness o the lef lacrimal sac area and bloody discharge. B. An axial C scan showing a mass in the lacrimal sac ossa, which was a lymphoma on biopsy.

C H AP T ER

Orbi al In ec ions ORBITAL CELLULITIS

O

rbi al celluli is is a real oph halmic emergency ha needs promp recogni ion and rea men . T e in ec ion can progress rapidly over a ew hours in severe cases wi h po en ial li e- hrea ening complica ions.

Epidemiology and Etiology Age: All ages Gender: Equal incidence in males and emales E iology: Sinusi is is he mos common cause bu o her causes include skin in ecions or skin wounds, den al in ec ions, and dacryocys i is. History One o 3 days o progressive swelling around he eye T e process may be preceded by an upper respira ory in ec ion. T e pa ien may have a his ory o sinus in ec ions. 142

Examination Ery hema, swelling, chemosis, res ric ed mo ili y, pain on eye movemen , and prop osis charac erize orbi al celluli is. T ese symp oms are progressive over 24 o 48 hours. As he in ec ion advances, vision can be af ec ed. Pa ien s may or may no have a ever and leukocy osis. I is very impor an o make he dis incion be ween he signs o orbi al celluli is and presep al celluli is where here is jus swelling and redness o he eyelids ( Fig. 11-1). Imaging C scanning is no required o make he diagnosis o orbi al celluli is bu is needed o look or he source o in ec ion (e.g., sinusi is, orbi al abscess) and o rule ou o her processes such as an orbi al umor. A C scan will show sinusi is, which can require drainage. Orbi al oreign bodies or an orbi al abscess can require addi ional surgery.

Orbital Cellulitis

Special Considerations Aggressive and promp rea men o orbi al celluli is is required o preven poserior ex ension o he in ec ion, which can resul in cavernous sinus hrombosis, which is li e- hrea ening. Dif erential Diagnosis Presep al celluli is Orbi al pseudo umor Orbi al abscess Phycomycosis Me as a ic orbi al umor

143

Laboratory Tests Comple e blood coun : Whi e coun may be normal. Blood cul ures are o ques ionable value. Treatment Immedia e broad-spec rum IV an ibio ics, orbi al imaging, and care ul moni oring or improvemen in he rs 24 o 48 hours. Prognosis Good. Rare complica ions rom developmen o an abscess or cavernous sinus hrombosis.

A FIGURE 11-1. Preseptal cellulitis. A. Child with a scratch on the lateral lef upper eyelid that resulted in preseptal cellulitis 2 days later. Ocular motility is normal. T e patient responded to antibiotics within 48 hours. ( continued)

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B

C

D FIGURE 11-1. (Continued) Preseptal cellulitis. B. Early cellulitis related to a subconjunctival abscess that required drainage and oral and topical antibiotics. C–F. Patient with 2 days o swelling o the lef eye with orbital cellulitis. T e eye is swollen shut but, with lif ing, the eyelid ocular motility is limited and there is chemosis. T e patient responded with improvement in 48 hours on IV antibiotics. ( continued)

Orbital Cellulitis

145

E

F

G FIGURE 11-1. (Continued) Preseptal cellulitis. G. C scan shows proptosis and sinusitis and is consistent with the clinical diagnosis o orbital cellulitis.

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ORBITAL ABSCESS

A

n orbi al abscess is a rare complica ion o sinusi is and orbi al celluli is. Orbi al celluli is ha does no improve on broadspec rum IV an ibio ics needs care ul imaging o look or an orbi al abscess. Epidemiology and Etiology Age: Any Gender: Equal E iology: Sinus disease is he mos common source o a subperios eal abscess. Rarely, an orbi al oreign body can be he cause and mus be suspec ed i he abscess is in raorbi al (in raconal). History Orbi al celluli is wi h no sign o improvemen on appropria e an ibio ics Examination Signs are hose o orbi al celluli is ha do no improve on appropria e IV an ibio ics. T e globe may be displaced away rom he abscess. T e abscess is diagnosed on C scanning ( Fig. 11-2).

Imaging C scanning will demons ra e a subperios eal opaci y usually adjacen o an in ec ed sinus. Rarely, he abscess may be in raconal. Dif erential Diagnosis Orbi al celluli is Phycomycosis Cavernous sinus hrombosis Orbi al pseudo umor Laboratory Tests Comple e blood coun ; cul uring o he abscess con en s. Treatment Mos pa ien s will require immedia e surgical drainage o he abscess and rea men wi h broad-spec rum IV an ibio ics. Some abscesses have been rea ed wi h IV an ibio ics alone and close observa ion in children younger han age 9 years. Prognosis Promp and aggressive rea men usually allows success ul rea men . An orbi al abscess does have he po en ial o resul in visual loss, mo ili y problems, or even severe CNS morbidi y.

Orbital Abscess

147

A

B FIGURE 11-2. Orbital abscess. A. A patient with a 2- to 3-day history o swelling o the lef eye. B.T ere is 5 mm o proptosis and limited motility. ( continued)

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C FIGURE 11-2. (Continued) Orbital abscess. C. C scan shows pan sinusitis with a medial orbital abscess that required surgical drainage. ( continued)

Orbital Abscess

149

D

E FIGURE 11-2. (Continued) Orbital abscess. D. A patient with weeks o a red irritated eye. E. C scan shows an abscess around an old orbital oor implant.

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PHYCOMYCOSIS ( MUCORMYCOSIS)

P

hycomycosis is a rare, o en a al ungal in ec ion ha occurs in very sick, immunocompromised pa ien s, mos commonly in poorly con rolled diabe ics. T is in ec ion s ar s in he nasopharynx or sinuses and secondarily invades he orbi . Aggressive rea men has improved he survival in his o en a al condi ion.

Epidemiology and Etiology Age: Adul s Gender: Equal male and emale occurrence E iology: Fungi invade he orbi rom he sinuses or nose. T e ungi invade blood vessel walls and produce hrombosis, ischemia, and allow spread o he ungi. History Pa ien s o en have a his ory o severe sinus pain and progressive orbi al swelling. T e pa ien s who develop his in ec ion are immunocompromised in some way. T e mos common underlying condi ion is severe diabe es wi h poor con rol bu o hers include malignancy, chemo herapy, and chronic s eroid use. Examination Prop osis is he mos common nding wi h an orbi al apex syndrome. Black eschar in he nasal cavi y is a la e nding and is no a reliable diagnos ic sign. Pa ien s are very sick sys emically ( Fig. 11-3A).

Imaging C scanning will show evidence o sinus disease, which a imes can be very mild ( Fig. 11-3B). MRI wi h gadolinium should be done o look or evidence o ex ension in o he cavernous sinus. Pathology Diagnosis is made on biopsy. Nonsep a e, large branching hyphae ha s ain on hema oxylin and eosin s aining, unlike mos ungi, are ound. Dif erential Diagnosis Orbi al celluli is Orbi al pseudo umor Cavernous sinus hrombosis Laboratory Tests Evalua ion or diabe ic con rol, leukocy e coun Pathophysiology Oppor unis ic ungal in ec ion ha grows in an immunocompromised hos . Treatment Con rol sys emic disease, IV ampho ericin B, and surgical debridemen o necro ic issue, which can involve orbi al exen era ion. Prognosis Poor Depending on s a e o he pa ien ’s sys emic disease, his condi ion can o en be a al. Even i he disease is con rolled, vision is o en los in he af ec ed eye.

Phycomycosis (Mucormycosis)

151

A

B FIGURE 11-3. Phycomycosis. A. A patient with poorly controlled diabetes with a 1-week history o sinusitis. T e patient has a rozen globe and a central retinal artery occlusion. T ere is a dusky erythema o the cheek. B. C scan shows di use sinus disease with orbital involvement. Biopsy o the sinus revealed ungus consistent with phycomycosis.

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ASPERGILLOSIS

A

spergillosis occurs in wo orms. One orm, very similar o phycomycosis, occurs in immunocompromised pa ien s and has a poor prognosis. T e second orm occurs in heal hy pa ien s wi h chronic sinus disease and allergies. T is orm has a good prognosis. Epidemiology and Etiology Age: Adul s Gender: Equal male and emale incidence E iology: An oppor unis ic in ec ion ha grows in he sinuses and secondarily invades he orbi . I can occur in wo orms. One orm ac s like phycomycosis and hus occurs in immunocompromised hos s. T e second, “allergic” orm occurs in immune compe en hos s wi h chronic sinus disease and allergies. T e sinus is lled wi h mucin and ungus and may have bone erosion. History Aspergillosis occurring in immunocompromised hos s presen s similar o phycomycosis. T e allergic orm will presen as chronic sinus problems bu will invade he orbi wi h ime in 17% o pa ien s, resul ing in orbi al signs depending on he sinus involved. Examination Findings on examina ion are dependen on he orm o in ec ion. T e presen a ion o aspergillosis in he immunocompromised hos is he same as ha o phycomycosis and is only dif eren ia ed on biopsy. T e allergic orm will only presen wi h orbi al ndings in he minori y o cases. T e signs vary rom displacemen o he globe o an orbi al apex syndrome, depending on he loca ion o he in ec ion and exac direc ion o invasion ( Fig. 11-4). Imaging C scan will show sinus disease wi h secondary orbi al invasion.

MRI can be help ul in de ning ex en o he disease in he orbi and looking or possible CNS ex ension. T e allergic orm shows he sinus lled wi h mot led area o increased at enua ion on nonenhanced C scan. T ere may be areas o bone remodeling and even erosion. MRI imaging shows a signal void on 2 images. Pathology Diagnosis is made by biopsy. Sep a e branching hyphae o uni orm wid h are seen on Gomori’s me henamine silver s aining. Dif erential Diagnosis Sinusi is wi h mucocele Phycomycosis Me as a ic orbi al umor Laboratory Tests Immunocompromised pa ien s will have associa ed blood nding such as ke oacidosis, leukopenia, and so or h, depending on he e iology o he immunode ciency. Pa ien s wi h allergic orm may have a peripheral blood eosinophilia, eleva ed o al immunoglobulin E, and posi ive allergy skin es ing or ungus. Treatment Immunocompromised pa ien s wi h aspergillosis are rea ed he same as phycomycosis (see previous discussion). Cleaning ou he mucin and ungus rom he af ec ed sinus and orbi de ni ively rea s he allergic orm. Prognosis Poor in he immunocompromised orm Good in he allergic orm wi h appropria e rea men

Aspergillosis

153

A

B

C FIGURE 11-4. Aspergillosis. A. A 45-year-old patient with loss o vision in the lef eye and very mild proptosis on the lef . T ere are no other orbital signs. B. C scan shows a large mass o the sphenoid sinus with erosion into the cavernous sinus. C. On MRI, the central area o signal void is classic or aspergillosis. T is mass was simply cleaned out via transnasal sinus surgery and the vision returned to normal.

C H AP

ER

Orbi al Inf amma ion THYROID-RELATED OPHTH ALMOPATHY hyroid-rela ed oph halmopa hy ( RO) is he mos common cause o prop osis in adul s. T e disease can range rom mild eyelid re rac ion o severe prop osis wi h op ic nerve compression and corneal exposure. Early in he disease course, RO can be di cul o diagnose bu la er he ocular signs become classic. Epidemiology and Etiology Age: Rare in children, mainly adul s Gender: Women a ec ed ve o eigh imes more o en han men E iology: Poorly unders ood au oimmune inf amma ory process ha a ec s he eyelid and orbi al issues History Ini ial onse o nonspeci c ocular irri a ion ollowed by eyelid re rac ion, lid lag, eyelid swelling, and bulging o he eyes. Pa ien s will no e symp oms o be worse in he morning and improve over he day. Many pa ien s will have he his ory o a sys emic 154

hyroid imbalance, bu up o 30% may be eu hyroid a he onse o symp oms. Examination T e earlies signs o RO are very nonspeci c and i can be di cul o make he diagnosis early in he disease. Eyelid re rac ion and eyelid lag are also early signs ha will help con rm he diagnosis. As he disease progresses, chemosis, prop osis, and mo ili y res ric ion wi h diplopia will become apparen . La e signs are decreased vision rom op ic nerve compression and severe corneal exposure ( Fig. 12-1A–F). Imaging C scan will show enlargemen o he rec us muscles wi h endon sparing. T e in erior rec us is he mos commonly involved muscle ollowed by medial rec us and superior rec us. T e la eral rec us is rarely involved. C scan is no needed o make he diagnosis o RO, as his is a clinical diagnosis.

T yroid-Related Ophthalmopathy

C scanning is help ul o con rm unusual cases, evalua e op ic nerve compression, and be ore surgery or irradia ion ( Fig. 12-1G, H). Special Considerations T e course and severi y o disease is widely variable. Pa ien s may have a ew mon hs o mild inf amma ion wi hou any sequelae, whereas o hers can have severe inf amma ion ha can lead o severe prop osis, double vision, and visual loss over a ew mon hs or years. Pa ien s who smoke have a longer and more severe course. Dif erential Diagnosis Orbi al pseudo umor Orbi al celluli is Orbi al lymphoma Laboratory Tests T yroid-s imula ing hormone Pathophysiology Chronic inf amma ory process leads o deposi ion o glycosaminoglycans in he muscles and orbi al a wi h even ual scarring and dys unc ion o hese issues. Treatment Limi ing he inf amma ion will limi he scarring and severi y o he disease. Sys emic s eroids will decrease inf amma ion, bu because o he side e ec s rom

155

long- erm use, hey are usually limi ed o use as a emporary, shor - erm rea men . Orbi al irradia ion in some pa ien s is e ec ive a s opping he progression o he disease bu no e ec ive a reversing any o he changes ha have occurred. Any pa ien wi h signi can , ac ive disease is a po en ial candida e or irradia ion (excep pa ien s wi h diabe ic re inopa hy). T e use o orbi al irradia ion is con roversial. S eroid injec ions in o he orbi and a shor course o high-dose IV s eroids are o her rea men s. Immune modula ors such as ri uximab are also being explored o s op his au oimmune process. A er he inf amma ory phase is over, surgical correc ion o residual prop osis, diplopia, and eyelid de ormi ies can be considered. T is is done via a combina ion o orbi al decompression and eye muscle and eyelid surgery. Pa ien s presen ing wi h severe inf amma ion and an op ic neuropa hy or corneal decompensa ion can require an urgen orbi al decompression. Prognosis Good, bu some pa ien s may require mul iple surgical procedures over years as par o he rea men . Pa ien s wi h signi can disease o en have a prolonged course o rea men .

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12 O RBI AL INFLAMMA IO N

A

B

C FIGURE 12-1. T yroid-related ophthalmopathy. A. A patient with very early thyroid-related ophthalmopathy with slight lid retraction on the lef . B. In down gaze, there is eyelid lag. C. A 20-year-old patient with severe proptosis, eyelid retraction, and corneal exposure. ( continued)

T yroid-Related Ophthalmopathy

157

D

E

F FIGURE12- 1. (Continued) T yroid-related ophthalmopathy. D–F. A 45-year-old patient with progressive swelling o the eyes with double vision and recent decreased vision. T ere is proptosis, chemosis, and limitation o motility. Vision was 20/ 80 rom optic nerve compression. ( continued)

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12 O RBI AL INFLAMMA IO N

G

H FIGURE12- 1. (Continued) T yroid-related ophthalmopathy. G and H. C scans show enlargement o all rectus muscles with crowding at the orbital apex. T e patient required an orbital decompression and her vision returned to normal. ( continued)

T yroid-Related Ophthalmopathy

I

J FIGURE12- 1. (Continued) T yroid-related ophthalmopathy. I. A patient with severe thyroid-related ophthalmopathy. J.A f er 3 years and multiple surgeries, there is signi cant improvement.

159

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IDIOPATHIC ORBITAL INFLAMMATION (ORBITAL PSEUDOTUMOR) Epidemiology and Etiology Age: Children and adul s Gender: Equal incidence in males and emales E iology: T is inf amma ory process is by de ni ion unrela ed o any sysemic abnormali y and he cause remains unknown. History Acu e onse o orbi al pain o en associa ed wi h prop osis, ery hema, swelling, and res ric ed eye movemen s. T e symp oms depend on he exac locaion o he process bu pain is common o all presen a ions. Adul s more commonly have unila eral disease bu in children his can be a bila eral process. Examination T e acu e inf amma ory process can occur an eriorly and presen wi h acu e ery hema and swelling o he lids and globe. I may presen as a myosi is wi h res ric ed mo ili y and pain wi h eye movemen , as a scleri is, a dacryoadeni is, or in he orbi al apex wi h ew ex ernal signs bu signi can pain, dysmo ili y, and decreased vision. T e presen a ion is variable depending on he issues a ec ed. Pa ien s wi h orbi al pseudo umor can have a ever and a leukocyosis ( Fig. 12-2). Imaging C scanning will show hickening o he a ec ed issues such as enlarged muscles, hickened sclera, enlarged lacrimal gland, or an in l ra e in he orbi al a .

Special Considerations Rarely, here may be very ew inf ammaory signs and a more chronic bro ic process ha is ermed sclerosing inf amma ory orbi al pseudo umor. T is condi ion is no very responsive o rea men as rea men is geared oward elimina ing inf amma ion and here is very lit le inf amma ion in his process. Sys emic condi ions, such as sarcoidosis, may cause a very similar pic ure. Dif erential Diagnosis Orbi al celluli is T yroid-rela ed oph halmopa hy Lymphoma Sarcoidosis Wegener’s Granuloma osis Rup ured dermoid cys Me as a ic disease Laboratory Tests Pa ien s may have a leukocy osis, peripheral blood eosinophilia, eleva ed ESR, and a posi ive ANA. None o hese are diagnos ic. Pathophysiology A pleomorphic cellular inf amma ory response occurs and i no rea ed or no responsive o rea men here will be a resulan bro ic response ha will progress wi h ime and resul in chronic scarring. Treatment Sys emic s eroids are he mains ay o rea men . T ere should be an improvemen in symp oms in 24 o 48 hours. T e longer he process has been presen , he longer i can ake or a clinical response. A er here is a good clinical response, he s eroids are apered over 4 o 6 weeks. Pa ien s wi hou response o s eroids or wi h mul iple recurrences o he inf amma ion

Idiopathic Orbital Inf ammation (Orbital Pseudotumor)

require an orbi al biopsy o con rm he diagnosis. I con rmed, hey are hen candida es or orbi al irradia ion. Immune suppressive agen s may also be used in some pa ien s wi h recurrences.

161

Prognosis Excellen prognosis in mos acu e cases. T ere may be recurrences. Cases ha are chronic wi h less inf ammaory response are less responsive o rea men and can be progressive.

A

B FIGURE 12-2. Orbital pseudotumor. A. A 33-year-old man with a 5-day history o swelling, erythema, and pain that is worse with eye movement. T e eye is red with orbital swelling and tenderness to palpation. Eye movements are limited by pain. B.D i use in ltration o the orbit and slight enlargement o the medial rectus. T e clinical presentation along with the C scan are consistent with orbital pseudotumor. T e patient responded within 24 hours to oral prednisone. ( continued)

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C

D FIGURE 12-2. (Continued) Orbital pseudotumor. C.T is is a scleritis: Scleritis with some anterior orbital swelling. D.D i use scleral thickening on lef side. ( continued)

Idiopathic Orbital Inf ammation (Orbital Pseudotumor)

163

E

F FIGURE 12-2. (Continued) Orbital pseudotumor. E. C shows a di use enlargement o the lateral rectus muscle consistent with a myositis. T e patient had limited adduction and abduction as well as pain with eye movement. F. C scan showing in ammation at the orbital apex. On examination, the eye may be white and quiet with minimal proptosis. T ere is of en decreased vision and motility dys unction consistent with an orbital apex syndrome.

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SARCOIDOSIS

S

arcoidosis can occur in he orbi in mul iple orms and wi h varying amoun s o inf amma ion.Mos commonly,i presen s wi h lacrimal gland enlargemen wi h very mild inf amma ory signs. Sarcoidosis may have a much more acu e swelling and can a ec he sclera, ex raocular muscles, or o her orbi al issues. Epidemiology and Etiology Age: Any age bu mos common in adul hood Gender: Equal E iology: Mul isys em inf amma ory disease ha occurs primarily in individuals o A rican and Scandinavian descen . History Mos commonly presen s wi h lacrimal gland enlargemen wi h varying amoun o inf amma ory signs Examination Bila eral lacrimal gland enlargemen is he mos common presen a ion. Ex raocular muscles, he op ic nerve, and eyelid skin are less commonly a ec ed. Inf amma ion in adjacen sinuses may secondarily a ec he orbi . T e en ire eye mus be evalua ed or signs o sarcoidosis causing uvei is (an erior or pos erior), iris nodules, or re inal vascular changes. Conjunc ival granulomas as well as sarcoid skin lesions can help con rm he diagnosis ( Fig. 12-3).

Imaging C scanning will show enlargemen o he lacrimal gland, muscle, or o her a ec ed s ruc ure.

Ches x-ray or a ches C is needed o evalua e he lungs or possible pulmonary sarcoidosis. Special Considerations Mos pa ien s wi h sarcoidosis will have sys emic disease wi h pulmonary ndings. Some pa ien s will have he disease isola ed o he orbi wi hou sys emic indings. Dif erential Diagnosis Idiopa hic orbi al pseudo umor Dacryoadeni i is Laboratory Tests Angio ensin-conver ing enzyme may be helpul in es ablishing he diagnosis. Treatment A biopsy o he a ec ed issue is usually required o con rm he diagnosis o sarcoidosis. When presen , a conjunc ival nodule is simple o biopsy. O herwise, he a ec ed issue is biopsied. A er he diagnosis is es ablished, careul sys emic evalua ion is needed o look or sarcoid. rea men is mos commonly sys emic prednisone al hough o her immunosuppressive agen s have been used. rea men is usually aimed a disease conrol whe her i is or con rol o orbi al inf amma ion or o con rol pulmonary disease. Prognosis Mos pa ien s do well, bu rare pa ien s can have signi can sys emic mani es a ions. T e orbi al disease can be chronic and recurren .

Sarcoidosis

165

A

B FIGURE 12-3. Orbital sarcoidosis. A and B. A patient with proptosis and double vision with some mild aching o the right eye. Motility shows poor adduction o the right eye. ( continued)

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C

D FIGURE 12-3. (Continued) Orbital sarcoidosis. C. C scan shows an enlarged medial rectus muscle. T is myositis responded to oral prednisone but recurred. Biopsy showed sarcoidosis. D. Bilateral lacrimal gland enlargement on the external photograph. ( continued)

Sarcoidosis

167

E

F

G FIGURE 12-3. (Continued) Orbital sarcoidosis. E and F. Axial and coronal C scans show enlarged lacrimal glands. Biopsy showed sarcoidosis. G.I n ltration o the eyelid and anterior lacrimal tissue by sarcoidosis. Under the eyelid, the in ltration is yellow-brown with prominent blood vessels.

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WEGENER’S GRANULOMATOSIS

W

egener’s granuloma osis can involve he eye and orbi as a secondary ex ension rom he sinuses or i can presen involving he eye i sel wi h scleri is, kera i is, uvei is, and so or h. T e sys emic disease can be li ehrea ening and he ocular involvemen can cause blindness and loss o he eye. Epidemiology and Etiology Age: Mainly adul s Gender: Equal occurrence in males and emales E iology: A sys emic necro izing granuloma ous vasculi is ha classically a ec s he upper and lower respira ory rac and can a ec he small vessels o any major organ sys em. History Diagnosis may or may no already be made when he pa ien presen s wi h eye ndings. Mos commonly, here is bony erosion via ex ension o he disease in o he orbi rom he sinus cavi y. Pa ien s can also have a necro izing scleriis, which can be severe.

Examination Findings include scleri is, which may be an erior or pos erior and is o en necro izing. Prop osis wi h or wi hou orbi al inf amma ion may be presen ( Fig 12-4A). Imaging C scans show bone erosion rom sinus ex ension o he disease ( Fig 12-4B and C). Dif erential Diagnosis Malignan umor o he sinus Laboratory Tests An ineu rophil cy oplasmic an ibodies (speci cally c-ANCA) are o en presen wi h Wegener’s disease. Pathology Vasculi is, granuloma ous inf amma ion, and issue necrosis are ound on pa hologic evalua ion. Treatment Immunosuppressive medica ion, speci cally cor icos eroids and cyclophosphamide, is he rea men o choice. Prognosis Variable. T e disease can be progressive and a al.

A FIGURE 12-4. Wegener’s granulomatosis. A. A patient with orbital pseudotumor-like picture. ( continued)

Wegener’s Granulomatosis

169

B

C FIGURE 12-4. (Continued) Wegener’s granulomatosis. B and C. C scans show in ltration along the in erior– medial orbit and into the sinus. Poor response to prednisone and a positive ANCA led to a biopsy, which was consistent with Wegener’s granulomatosis.

C H AP T ER

Congenital Orbital Anomalies MICROPHTH ALMOS

M

icrophthalmos is a de ect in the eye development. T e eye is small and there are usually structural de ects. T e microphthalmos can be mild or the eye can be so small that it cannot be seen at all. Epidemiology and Etiology Age: Congenital Gender: Equal occurrence in males and emales Etiology: Developmental de ect with ailure o the choroidal ssure to close as an embryo. T is results in a small eye with structural abnormalities ( Fig. 13-1) Examination T e eye may be small to virtually nonexistent. T ere are structural abnormalities within the eye and the eye usually has poor or no vision.

170

T ere may be an accompanying cyst, which may be quite large. T is condition is usually unilateral, rarely bilateral. Dif erential Diagnosis Anophthalmos Treatment reatment is aimed at stimulating the orbit to grow and mature normally. I the eye is only slightly small or i there is a large cyst, orbital growth may continue as normal. However, i the eye is very small, expanding con ormers should be used to try to stimulate orbital growth. Dermis at graf s are used sometimes. Prognosis T ese patients of en have some orbital asymmetry even with aggressive treatment. T e cosmetic result is generally acceptable.

Microphthalmos

171

A FIGURE 13-1. Microphthalmos. A. A child wi h microph halmos wi h cys . ( continued)

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13 CO NGENITAL O RBITAL ANO MALIES

B FIGURE 13-1. (Continued) Microphthalmos. B. CT scan shows a small eye and at ached cys . ( continued)

Microphthalmos

173

C FIGURE 13-1. (Continued) Microphthalmos. C. Pa hologic specimen shows he cys ic ou pouching coming from he abnormally developed eye.

C H AP T ER

Orbital Neoplasms CONGENITAL ORBITAL TUMORS DERMOID CYSTS

D

ermoid cysts are relatively common, benign, orbital tumors in children. Classically, they are present at birth, are located superior temporally at the orbital rim, and enlarge with time. Epidemiology and Etiology Age: Congenital and enlarge with age Gender: Equal occurrence in males and emales Etiology: Epidermal elements are le during embryonic development in deeper tissues. T ese epidermal elements then orm a cyst that enlarges with time.

History More superf cial dermoids are o en noted in the f rst 1 to 2 years o li e as they grow and become more noticeable (Fig. 14-1A, B). T e dermoids that are deeper, such as in the orbit, may not become symptomatic until 174

adulthood when they have become large, start to leak, or rupture rom trauma. Examination T e classic location or a superf cial dermoid cyst is at the lateral brow over the rontozygomatic suture. Less commonly, they can be superior medial or even in the lower lid. T ey are smooth, painless masses that slowly enlarge. T ey can be reely mobile or f xed to the bony suture. Deeper dermoids can be in the superior and/ or lateral orbit. “Dumbbell” dermoids occur in the temporal ossa and have a component in the orbit and a part in the temporal ossa. Deeper dermoids present with proptosis or with symptoms o orbital in ammation as the dermoid cyst either leaks or ruptures ( Fig. 14-1C). Imaging C scan: nonenhancing cystic mass (Fig. 14-1D).

Congenital Orbital Tumors

MRI: hypoin ense on 2(Fig. 14-1E).

; hyperin ense on

1

Dif erential Diagnosis When loca ed super icially and emporally, here are very ew lesions his can be con used wi h. Imaging usually helps make he diagnosis i loca ed deep in he orbi . Pathology T e cys is lined by kera inizing epidermis wi h dermal appendages such as hair ollicles and sebaceous glands. T e cys is f lled wi h kera in and oil.

175

Treatment Comple e surgical excision wi h an in ac capsule is he surgery o choice. T is procedure should be done when he po en ial or cys rup ure becomes a risk. T is mos o en occurs when he child begins o walk and be more ac ive. Prognosis Excellen or superf cial dermoids. Good or deep dermoids as long as he en ire cys is removed

A FIGURE 14-1. Dermoid cyst. A. Sof , mobile mass along he superior emporal rim in a 1-year-old pa ien . T is has been presen since bir h. ( con inued)

176

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B

C FIGURE 14-1. (Con inued) Dermoid cyst. B. Excision o he dermoid hrough a lid crease incision. Ca nd D. Prop osis and globe displacemen caused by a deep orbi al dermoid, which was no ed a age 5 years. T e ossa orma ion caused by hese lesions is seen on he C scan. T e deep orbi al loca ion means hey are of en no no iced un il he child is older. T is was comple ely excised and he pa ien did well wi hou any ur her problems. ( con inued)

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177

D

E FIGURE 14-1. ( Con inued) Dermoid cyst. D. C scan show cys ic lesion wi h bony ossa orma ion. E. MRI o a dermoid cys . On a 2-weigh ed image, he cys is hyperin ense o a and muscle.

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LIPODERMOIDS

L

ipodermoids are congeni al solid umors loca ed emporally below he conjunc iva. T ese are some imes no no ed un il la er in li e. T ey should be le alone in almos all cases.

Dif erential Diagnosis Fa prolapse Lymphoma Prolapsed lacrimal gland

Epidemiology and Etiology Age: Congeni al Gender: Equal occurrence in males and emales E iology: Developmen al anomaly

Pathology Kera inizing squamous epi helium wi h adnexal s ruc ures T e underlying dermis usually con ains a and connec ive issue.

History Presen a bir h and generally does no change wi h ime Examination Yellowish, pink lesion over he la eral sur ace o he globe deep o he conjunc iva ( Fig. 14-2) T ey vary in size and o en have hairs on he sur ace.

Treatment No rea men At emp ed excision can damage he adjacen lacrimal duc s and rec us or leva or muscles. In rare cases when he lipodermoid is very large, he an erior por ion can be debulked leaving he conjunc iva unresec ed.

Imaging I large, C scan will show a mass wi h a densi y.

Prognosis Excellen i le alone

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179

A

B FIGURE 14-2. Lipodermoid. A. Classic loca ion or a lipodermoid, which has been presen since bir h. B. Close inspec ion of en shows hairs on he lesion. Despi e he cosme ic appearance, hese are bes lef alone.

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VASCULAR ORBITAL TUMORS CAPILLARYHEMANGIOMAS

C

apillary hemangiomas are benign umors o he orbi ha appear in he f rs ew weeks o li e and enlarge over he f rs 6 o 12 mon hs. T ey hen end o shrink over ime bu he ini ial presen a ion can be drama ic.

Epidemiology and Etiology Age: No ed in he f rs year o li e Gender: Equal occurrence in males and emales E iology: Abnormal grow h o blood vessels wi h varying degrees o endo helial cell proli era ion History Lesions are o en no ed in f rs ew weeks o li e and hey grow, some imes rapidly, over weeks o mon hs. T ey can presen deeper in he orbi wi h prop osis or more superf cially as an expanding mass. T e hemangioma will hen involu e over mon hs o years. Seven y-f ve percen o lesions will resolve over 4 years. Examination T e lesion appearance is dependen on he loca ion (Fig. 14-3A-C, F, G). T e more common superf cial lesions produce an eleva ed, dimpled, s rawberrycolored lesion. Deeper lesions may give a bluish discolora ion. Deep orbi al lesions may only give sympoms o an expanding orbi al mass.

Di eren ia ion be ween rhabdomyosarcoma and deep capillary hemangioma can only be made wi h biopsy. Imaging C scan reveals a mass ha can be well or poorly margina ed wi h enhancemen wi h con ras ( Fig. 14-3D, E). MRI is hypoin ense on 1 and hyperinense on 2. T e lesion enhances wi h gadolinium. Dif erential Diagnosis Rhabdomyosarcoma Pathology Proli era ion o endo helial cells organized in o a ne work o basemen membrane–lined vascular channels. Treatment T ese lesions will regress so hemangiomas are observed or regression unless hey cause visual obs ruc ion or as igma ism leading o amblyopia. In his case, rea men is required. Orbi al lesions causing severe prop osis may also require rea men . Orbi al biopsy is required i he lesion canno be di eren ia ed rom a rhabdomyosarcoma. rea men op ions include in ralesional s eroid injec ion, sys emic s eroids, or, in selec cases, surgical excision. Recen s udies sugges sys emic propranolol may become he rea men o choice. Care ul moni oring o hese babies on propranolol mus be done by pedia ric cardiology. Prognosis Good

Vascular Orbital Tumors

181

A

B FIGURE 14-3. Capillary hemangioma. A. Subcu aneous capillary hemangioma o he righ eyebrow ha increased in size over 6 mon hs. T e lesion becomes more prominen and red wi h crying. T is lesion resolved over 3 years. B. A small hemangioma on he child’s arm. ( con inued)

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C

D

E FIGURE 14-3. (Con inued) Capillary hemangioma. C.S uper cial orbi al hemangioma ha had increased in size and was causing amblyopia rom 7 diop ers o induced as igma ism. D and E. C scans show his an erior orbi al mass, which is well circumscribed and enhances wi h con ras . T is was excised because o he as igma ism and amblyopia. ( con inued)

Vascular Orbital Tumors

F

183

G

FIGURE 14-3. (Con inued) Capillary hemangioma. F. Large cu aneous capillary hemangioma wi h visual obs ruc ion. G.T is lesion responded well o a series o in ralesional s eroid injec ions.

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CAVERNOUS HEMANGIOMAS

C

avernous hemangiomas can presen as asymp oma ic, very insidious onse prop osis. More commonly, hese lesions presen wi hou any symp oms and are ound on imaging done or unrela ed reasons. T ey are slowly growing masses ha are generally easy o remove depending on heir loca ion.

Epidemiology and Etiology Age: Adul s Gender: Mos commonly middle-aged women E iology: Unknown History Very slow grow h usually means he pa ien is unsure o he onse or dura ion o he lesion. Mos commonly, he presen a ion is prop osis bu rarely here can be symp oms o visual loss. Examination Axial prop osis is he common presen a ion. I he lesion is a he apex or is very large, i can cause op ic nerve compromise ( Fig. 14-4A-C) or s rabismus. Lesions can rarely cause orbi al pain or he appearance o a choroidal mass.

Imaging C scan shows an encapsula ed, homogeneous, round mass wi h variable enhancemen . MRI: isoin ense on 1 and hyperin ense on 2(Fig. 14-4D, E). Marked enhancemen wi h gadolinium Special Considerations Rarely, lesions may grow rapidly during pregnancy. Dif erential Diagnosis Hemangiopericy oma Schwannoma Fibrous his iocy oma Pathology Encapsula ed umor consis ing o large endo helial lined channels wi h abundan , loosely dis ribu ed smoo h muscle in he vascular walls and smoo h muscle. Treatment Surgical excision is he rea men o choice. T ese lesions are easily removed once exposed. T ey do no regress and slowly enlarge so observa ion only delays surgery. Prognosis Excellen

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185

A

B FIGURE 14-4. Cavernous hemangioma. A. A pa ien wi h prop osis o he righ eye o unknown dura ion and no o her visual or orbi al complain s. B. C scan shows a well-circumscribed in raconal orbi al mass. ( con inued)

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C

D FIGURE 14-4. (Con inued) Cavernous hemangioma. C.T e mass was excised and was a cavernous hemangioma. D. MRI o a cavernous hemangioma. T e 1-weigh ed image shows he lesion isoin ense o muscle and hypoin ense o a . ( con inued)

Vascular Orbital Tumors

187

E FIGURE 14-4. ( Continued) Cavernous hemangioma. E. On the T 2-weighted image, the lesion is hyperintense to fat and muscle.

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LYMPHANGIOMAS

L

ymphangiomas are rare vascular hamar omas ha can behave in many di eren ways depending on loca ion and grow h pat erns. T is condi ion can vary rom mild ra her asymp oma ic lesions, o progressively growing, inf l ra ive lesions, o acu e prop osis and visual loss rom bleeding in o hese lesions. Epidemiology and Etiology Age: Usually no ed in he f rs decade o li e Gender: More common in emales E iology: Congeni al lesion History T ese lesions are o en no ed associa ed wi h a spon aneous bleed o he lesion al hough hey likely were presen or years prior. T ey can grow slowly and hen suddenly hemorrhage. Lymphangiomas can mani es as pain, subconjunc ival hemorrhage, or as prop osis. Less commonly, he cys s o hese lesions are no ed subconjunc ivally. T ese lesions enlarge wi h upper respiraory in ec ions. Examination T e f ndings on examina ion are dependen on he loca ion o he lesion. T e mos common presen a ion is associa ed wi h sudden bleeding in o he lymphangioma. I he bleed is superf cial, hen a subconjunc ival bleed is seen and he cys s o he lymphangioma are o en ound ( Fig 14-5A, B). I he hemorrhage is in he orbi , he f ndings may only be prop osis ( Fig 14-5C).

Care ul evalua ion or evidence o a lymphangioma superf cially should be done in hese cases. Imaging will aid in he diagnosis i he lesion is en irely orbi al. Imaging C scan: Poorly circumscribed, he erogeneous mass MRI: Hyperin ense on 1; very hyperinense on 2, wi h possible area o uid and blood ( Fig. 14-5D, E) Special Considerations Surgery per ormed on a lymphangioma increases he chances o spon aneous bleeds wi hin he lesion. Surgery should only be done i absolu ely necessary. Dif erential Diagnosis Diagnosis can usually be made wi h MRI. Pathology Nonencapsula ed mass wi h large serum- illed spaces lined by la endo helial cells T e in ers i ium has scat ered lymphoid ollicles. Treatment Observa ion unless he spon aneous bleeding causes visual loss, corneal exposure, or severe cosme ic disf guremen . Generally, wi h ime he blood will resorb. When an orbi al hemorrhage causes visual loss, drainage o he hemorrhage should be per ormed. Debulking o he lesion or orbi al decompression are o her rea men op ions.

Vascular Orbital Tumors

Lymphangiomas are inf l ra ive so excision is very di cul and here is usually signif can bleeding associa ed wi h excision, which is only per ormed as a las resor .

189

Prognosis Variable depending on he grow h o he lymphangioma Progressive lesions have a high incidence o visual disabili y and poor cosme ic resul .

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A

B FIGURE 14-5. Lymphangioma. A. Pa ien wi h sudden onse o orbi al discom or and prop osis. Medially, a small area o hemorrhage is no ed wi h subconjunc ival cys s consis en wi h a lymphangioma. B. More obvious hemorrhage was no ed along wi h he onse o deep orbi al pain. Mul iple cys s can be seen in he hemorrhage. Imaging was consis en wi h a lymphangioma. ( con inued)

Vascular Orbital Tumors

191

C

D

E FIGURE 14-5. (Con inued) Lymphangioma. C. Prop osis o he lef eye wi h recurren episodes o orbi al pain. T e pain was usually associa ed wi h an increase in he prop osis. D and E. MRIs show a superior orbi al mass wi h area o resh and old blood consis en wi h a lymphangioma.

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HEMANGIOPERICYTOMA

H

emangiopericy oma is a rare lesion ha can mimic a cavernous hemangioma bu has more rapid grow h and is more likely o cause symp oms. T ese can recur and have a chance o me as asis. Epidemiology and Etiology Age: Middle age Gender: Equal occurrence in males and emales E iology: umor origina es rom he pericy e. T is is a rare orbi al umor. History Insidious onse o prop osis and mass e ec bu usually more rapid onse han a cavernous hemangioma Examination Prop osis is o en he only f nding. T ese lesions appear more o en in he superior orbi bu in raconal loca ion is also common ( Fig. 14-6A, B). Imaging C scan: well-circumscribed, encapsula ed mass

MRI: isoin ense on 1; hyperin ense on (Fig. 14-6C, D). Enhances wi h gadolinium

2

Special Considerations T ese lesions have he po en ial or recurrence locally whe her he pa hology is benign or malignan . Malignan lesions can recur and can also me as asize. Dif erential Diagnosis Cavernous hemangioma Fibrous his iocy oma Schwannoma Pathology Uni orm spindle-cell umor wi h a sinusoidal vascular pat ern; can be divided in o benign, in ermedia e, and malignan orms. Treatment Comple e excision in he capsule is he bes rea men . Recurrence, i malignan , may require exen era ion. Prognosis Variable. Pa ien s mus be ollowed or a leas 10 years or local recurrence or me as asis.

Vascular Orbital Tumors

193

A

B FIGURE 14-6. Hemangiopericytoma. A and B. A 55-year-old man presen ed wi h increasing prop osis over 6 mon hs and diplopia. T ere is axial prop osis on he lef and a well-circumscribed mass on C scan. Pa hologic examina ion revealed a hemangiopericy oma. ( con inued)

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C FIGURE 14-6. ( Con inued) Hemangiopericytoma. C. 1-weigh ed coronal MRI wi h con ras and a suppression shows a hemangiopericy oma nex o he righ lacrimal gland. ( con inued)

Vascular Orbital Tumors

195

D FIGURE 14-6. (Con inued) Hemangiopericytoma. D. nex o he righ lacrimal gland.

-weigh ed coronal MRI o he same hemangiopericy oma

2

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ORBITAL VARICES

O

rbi al varices will presen in he 20s and 30s wi h a his ory o years o in ermi en prop osis. T ese lesions can be superf cial and no iceable or deep wi h only prop osis as a sign o he lesion. Mos lesions should be le alone unless here is ex reme orbi al pressure wi h unc ional def ci or a severe cosme ic disf guremen .

Epidemiology and Etiology Age: Usually no ed in he f rs hrough hird decades o li e Gender: Equal occurrence in males and emales E iology: Dila a ion o pre-exis ing venous channels History Pa ien s wi h nondis ensible varices presen wi h recurren episodes o hrombosis and hemorrhage in he lesion ( Fig. 14-7E, F). T is leads o prop osis, pain, mo ili y res ric ion, and even decreased vision. T ese symp oms resolve as he hemorrhage resolves. T e dis ensible varices presen wi h pain, prop osis, and pressure symp oms associa ed wi h s raining, bending orward, or Valsalva ( Fig. 14-7A, B). T e changes in he orbi and lids associa ed wi h his venous dis ension are also no ed. Examination Dis ensible varices are diagnosed easily by having he pa ien s bring heir head in o a dependen posi ion and no e he f lling o he varix.

Nondis ensible varices are more di cul o diagnose. T e pa ien will presen wi h symp oms o an acu e hemorrhage in o he lesion as no ed previously. T ere is generally no ex ernal hemorrhage presen or any sign o a varix in his ype. Imaging C scan may appear rela ively normal or wi h jus a small di use mass on axial cu s. In he dependen posi ion (coronal cu s), he mass will enlarge as he varix f lls wi h blood ( Fig. 14-7C, D). Nondis ensible varices will show a di use mass ha enhances wi h con ras . Dif erential Diagnosis Lymphangioma is he main di eren ial and di eren ia ion rom he nondis ensible varix is no always possible. Pathology Well-def ned venous channels Treatment Conserva ive observa ion in mos cases I a nondis ensible varix bleeds and visual or exposure symp oms require in erven ion, drainage o he blood clo is usually he rea men o choice. Prognosis Variable. Progressive lesions can be disf guring and success ul rea men is di cul .

Vascular Orbital Tumors

197

A

B FIGURE 14-7. Distensible orbital varix. A. A 55-year-old woman wi h a dis ensible varix in her superior medial orbi . B. Valsalva resul s in massive enlargemen and closure o he eye. ( con inued)

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C

D FIGURE 14-7. (Con inued) Distensible orbital varix. C. C scan showing he medial orbi al varix. D.W hen he head is placed in a dependen posi ion or he coronal C , he varix lls wi h blood, accoun ing or he enlargemen o he lesion on he coronal cu s. ( con inued)

Vascular Orbital Tumors

199

E

F FIGURE 14-7. ( Con inued) Distensible orbital varix. E. Nondis ensible varix may be deep in he orbi wi h only a small an erior componen . F.D i use orbi al involvemen wi h mul iple varices.

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ARTERIOVENOUS MALFORMATIONS

A

r eriovenous mal orma ions (AVM) presen wi h variable severi y. All cases involve he connec ion o an ar erial ow in o a venous drainage area such as he cavernous sinus. T ere may be sub le swelling and redness o he eye and orbi or he presen a ion may be severe prop osis, exposure, and in raocular vascular conges ion. Epidemiology and Etiology Age: Older adul s, excep a er rauma, which can occur a any age Gender: Equal occurrence in males and emales E iology: rauma (basal skull rac ure) resul s in high- ow f s ulas Degenera ive vascular process in pa ien s wi h hyper ension and a herosclerosis resul s in a low- ow f s ula. History Abrup onse o prop osis, chemosis, ar erializa ion o he conjunc ival vessels in one eye. T is occurs in a high- ow AVM. High- ow AVMs will have more severe sympoms bu o en have he his ory o head rauma. Low- ow AVMs are in older pa ien s, are slower in onse , and he symp oms are less drama ic. Examination Prop osis, chemosis, dysmo ili y, ar erializa ion o he conjunc ival vessels (corkscrew

pat ern) ( Fig. 14-8A, B), and eleva ed in raocular pressure are seen in AVMs. In high- ow s a es, he re inal vessels are a ec ed wi h venous conges ion. Imaging C scan and MRI show an enlarged superior oph halmic vein and here may be enlargemen o he ex raocular muscles ( Fig. 14-8D and E). Orbi al Doppler shows reversal o ow in he superior oph halmic vein and is diagnosic o an AVM ( Fig. 14-8C). Dif erential Diagnosis Orbi al pseudo umor Orbi al celluli is T yroid-rela ed oph halmopa hy Chronic conjunc ivi is Treatment Low- ow AVMs will o en resolve spon aneously. T e signs may worsen as he f s ula closes o . High- ow lesions o en require at emp ed selec ive emboliza ion o close he f s ula. T is may also be needed in low- ow lesions ha resul in uncon rolled glaucoma, diplopia, or vascular occlusion. Prognosis Variable. Many low- ow AVMs will close on heir own. rea men or AVMs is successul bu does have a risk o visual loss.

Vascular Orbital Tumors

201

A

B FIGURE 14-8. Arteriovenous mal ormation. A and B. A pa ien wi h a 3- o 4-week his ory o swelling and redness o he lef eye. Mo ili y is limi ed as no ed in at emp ed upgaze. (con inued)

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C

D FIGURE 14-8. ( Con inued) Arteriovenous mal ormation. C. Color Doppler imaging shows ar erializa ion o he superior oph halmic vein, which is diagnos ic o an ar eriovenous mal orma ion. D and E. C scan shows enlarged superior oph halmic vein and engorged rec us muscles, which is usually seen wi h an AVM. ( con inued)

Vascular Orbital Tumors

E FIGURE 14-8. ( Con inued)

203

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NEURAL TUMORS OPTIC NERVE GLIOMAS

O

p ic nerve glioma is a glial umor ha mos commonly presen s in children and includes painless prop osis and visual loss. T ese can ini ially involve he op ic chiasm or grow o involve i . rea men remains con roversial.

Epidemiology and Etiology Age: Predominan ly in children during he f rs decade o li e. Malignan gliomas occur in middle-aged males. Gender: Equal occurrence in males and emales E iology: Unknown History In children, gliomas presen wi h gradual, painless, unila eral, axial, prop osis wi h loss o vision and an a eren pupillary de ec . T e malignan orm in adul s presen s wi h symp oms o op ic neuri is bu rapidly progress o blindness and dea h. Examination Axial prop osis wi h visual loss, a eren pupillary de ec , op ic a rophy, or nerve swelling are all f ndings ( Fig. 14-9). T ere are no in amma ory signs or pain. Diagnosis is usually made on he basis o orbi al imaging.

T e malignan orm in adul s may show in amma ory signs along wi h signs o an op ic neuropa hy and prop osis. Imaging C scan demons ra es usi orm enlargemen o he op ic nerve ( Fig. 14-9B). MRI is he imaging o choice o evalua e he ex en and grow h o an op ic nerve glioma. 1 imaging is iso- o hypoin ense, whereas 2 imaging shows prolonged relaxa ion imes ( Fig. 14-9C, D). Special Considerations Neurof broma osis ype 1 is associa ed wi h 25% o 50% o op ic nerve gliomas. Dif erential Diagnosis Op ic nerve meningioma; he di eren ial diagnosis is more rela ed o how ex ensive he umor is and no wha i is. Pathology T ese are in radural lesions ha are juvenile pilocy ic as rocy omas. Treatment Con roversial and mus be individualized. Mos gliomas can be observed because hey are usually very slow-growing lesions. I here is signif can grow h, surgical excision is he bes rea men . I he umor is unresec able, radia ion is considered. Prognosis Variable. Some gliomas grow aggressively; o hers can remain s able or years.

Neural Tumors

205

A

B FIGURE 14-9. Optic nerve glioma. A. A 6-year-old girl wi h painless prop osis and visual loss. B. C scan shows usi orm enlargemen o he op ic nerve consis en wi h an op ic nerve glioma. ( con inued)

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C FIGURE 14-9. (Con inued) Optic nerve glioma. C. enlargemen .

-weigh ed axial MRI shows again shows usi orm

1

( con inued)

Neural Tumors

207

D FIGURE 14-9. (Con inued) Optic nerve glioma. D. cour esy o Ka e Lane, MD.)

-weigh ed axial MRI o he same pa ien . ( C and D

2

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NEUROFIBROMAS

N

eurof bromas are composed o proli era ing Schwann cells wi hin heir nerve shea h. T ere are mul iple orms o neurof bromas. Plexi orm neurof bromas are o en associa ed wi h neurof broma osis ype 1. Epidemiology and Etiology Age: Plexi orm neurof bromas are usually seen in he f rs decade. Isola ed lesions occur in he hird hrough f h decades. Gender: Equal occurrence in males and emales E iology: Plexi orm neurof bromas are he mos common neurof broma o involve he orbi and are associa ed wi h neurof bromaosis ype 1. History Pa ien s will o en already have he diagnosis o neurof broma osis and will develop hickening and hyper rophy o he a ec ed nerve. T ey may presen wi h hickening o eyelid or periorbi al skin, or wi h prop osis. Isola ed neurof bromas are no usually associa ed wi h neurof broma osis. Examination Findings will vary depending on he nerve or nerves ha are involved. T e involved nerves grow as a or uous, ropy angle o nerves. T is grow h is usually slow bu progressive and resul s in hickening o involved periorbi al and orbi al issues, prop osis, and orbi al bony abnormali ies ( Fig. 14-10A). T ese bony changes include orbi al enlargemen , abnormali ies o he sphenoid wing, and hypoplasia o he e hmoid and maxillary sinuses ( Fig. 14-10B). Isola ed neurof bromas presen wi h mass e ec . Prop osis, diplopia, and decreased vision may occur.

Imaging C and MRI show a di use inf l ra ing lesion in plexi orm neurof bromas. An isola ed neurof broma will be wellcircumscribed wi h charac eris ics similar o a schwannoma. Special Considerations Any pa ien wi h a plexi orm neurof broma mus be care ully evalua ed or neurof broma osis i hey are no known o have he disease. Soli ary neurof bromas are rare orbi al umors ha can be excised and are unlikely o be associa ed wi h neurof broma osis. T ese end o occur in middle age. Dif erential Diagnosis Lymphangioma Orbi al pseudo umor Isola ed lesions mus consider schwannoma, cavernous hemangioma, f brous his iocy oma Pathology Proli era ing, in er wining bundles o Schwann cells, axons, and endoneural f broblas s wi hin he nerve shea hs. Treatment Observa ion wi h surgical debulking only as a las resor . T ese umors canno be comple ely excised and recur and regrow wi h ime. Rare, isola ed lesions can be comple ely excised. T ese umors are o en very vascular and bleed during excision. Prognosis Generally poor cosme ic and unc ional resul s because o he progressive na ure o hese inf l ra ive umors. Isola ed lesions have a good prognosis.

Neural Tumors

209

A

B FIGURE 14-10. Neurof broma. A. Severe prop osis, comple e p osis, and orbi al in l ra ion by a plexi orm neuro broma. B. C scan shows orbi al in l ra ion as well as absence o par o he sphenoid bone o he orbi . All is consis en wi h neuro broma osis.

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MENINGIOMAS

M

eningiomas are invasive umors ha arise in racranially and secondarily invade he orbi . T ey are usually slowly progressive umors ha are very di cul o comple ely excise because o heir inf l ra ive na ure. Epidemiology and Etiology Age: Bimodal peak in he second and f h decades Gender: More common in women E iology: T ese umors arise rom arachnoid villi. Mos commonly, hese umors s ar as in racranial umors and ex end in o he orbi secondarily. A primary orbi al orm arises rom he op ic nerve shea h arachnoid issue. History Meningiomas will have a gradual onse o symp oms as hey slowly ex end rom heir in racranial origin in o he orbi . Oph halmic mani es a ions are dependen on he loca ion o he umor. Mos common orbi al presen a ion is umors arising near he p erion ha presen as a emporal ossa mass and prop osis ha can o en be suddenly no iced bu have been presen or years ( Fig. 14-11A–D). Op ic nerve meningiomas presen wi h slow, painless, progressive visual loss and prop osis. Examination Findings on examina ion depend on he loca ion o he meningioma. I loca ed in he emporal ossa, f ndings include emporal ossa ullness, prop osis, eyelid edema, and chemosis. I he meningioma arises near he sella and op ic nerve, early f ndings will be visual loss wi h op ic nerve edema or a rophy.

Op ic nerve meningiomas presen wi h decreased vision, an a eren pupillary de ec , prop osis, and possible oph halmoplegia ( Fig. 14-11E–J). T e op ic nerve may be normal, swollen, a rophic, or have shun vessels. Imaging C scan: Hyperos osis, calcif ca ion, wi h adjacen so issue ullness MRI: Use ul o de ec grow h along he dura Gadolinium enhancemen and a suppression echniques help def ne hese lesions in he orbi . Dif erential Diagnosis Op ic nerve glioma Lymphangioma Pathology hese umors are composed o cells ha can be round, polygonal, or spindle shaped. In addi ion, here are varying admix ures o blood vessels, f broblas s, and psammoma bodies. T e di eren pat erns o meningiomas show varying mix ures o hese componen s. Treatment In racranial meningiomas ha ex end in o he orbi are usually rea ed surgically. I well encapsula ed, hese can be comple ely excised wi h a neurosurgical and orbi al approach. Meningiomas can be inf l ra ive and involvemen o vi al s ruc ures may preven comple e excision and allow or debulking only. rea men o op ic nerve shea h meningiomas is required i here is aggressive

Neural Tumors

grow h, hrea o in racranial spread, or visual loss. Radia ion is he rea men o choice in progressive umors. Radia ion has been shown o slow grow h o hese umors. Surgical excision or biopsy is rarely needed.

211

Prognosis umors are generally progressive bu very slowly. Radia ion will slow or some imes s op grow h. More rapid grow h is unusual and he diagnosis should be ques ioned.

A

B FIGURE 14-11. Sphenoid wing meningioma. A. A pa ien wi h a lef -sided prop osis o gradual progressive onse . No e he emporal ossa ullness. B. C scan shows hyperos osis and an associa ed sof issue mass, all consis en wi h a sphenoid wing meningioma. ( con inued)

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C FIGURE 14-11. (Con inued) Sphenoid wing meningioma. C. suppression shows lef sphenoid wing meningioma.

1

-weigh ed coronal pos con ras MRI wi h a ( con inued)

Neural Tumors

213

D

E FIGURE 14-11. ( Con inued) Sphenoid wing meningioma. D. 2-weigh ed coronal MRI shows lef sphenoid wing meningioma. E. A pa ien wi h axial prop osis and visual loss. (con inued)

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F

G FIGURE 14-11. ( Continued) Sphenoid wing meningioma. F. MRI scan shows a usi orm enlargement o the optic nerve and is consistent with a meningioma. G. More commonly, there is dif use thickening o the optic nerve. T e thickened right optic nerve has a central lucency, termed the “railroad track” sign. ( continued)

Neural Tumors

215

H FIGURE 14-11. ( Continued) Sphenoid wing meningioma. H.T e hyperintense to at and muscle as seen on the lef side.

-weighted image can be hypointense to

2

( continued)

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I FIGURE 14-11. ( Con inued) Sphenoid wing meningioma. I. MRI 1-weigh ed coronal image wi h con ras and a suppression shows a more globular op ic nerve meningioma on he lef . ( con inued)

Neural Tumors

217

J FIGURE 14-11. ( Con inued) Sphenoid wing meningioma. J. MRI globular op ic nerve meningioma on he lef .

2

-weigh ed coronal image shows a more

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SCHWANNOMAS

S

chwannomas presen as well-encapsula ed, slowly growing masses ha ac very much like a cavernous hemangioma. T ese masses are usually easily excised and cause no subsequen problems.

Epidemiology and Etiology Age: 20 o 50 years o age Gender: Equal occurrence in males and emales E iology: Eccen ric grow hs rom peripheral nerves History Slow, insidious onse o prop osis over years Examination Prop osis wi h he direc ion dependen on he umor posi ion (mos commonly in raconal) ( Fig. 14-12A). Less commonly, here may be eyelid swelling, diplopia, visual dis or ion. Imaging C and MRI scan show a well-circumscribed, round lesion ( Fig. 14-12B–D).

Special Considerations Eigh een percen o pa ien s wi h schwannomas have neurof broma osis. Dif erential Diagnosis Capillary hemangioma Hemangiopericy oma Fibrous his iocy oma Pathology Proli era ion o Schwann cells in a perineural capsule is seen. T ese may be in a igh ly ordered arrangemen (An oni A) or loose arrangemen (An oni B). Treatment Surgical excision is he rea men o choice. Because hey are ou pouchings o a nerve, hey can o en be s ripped o he nerve. Recurrence o he umor, even i here is only par ial resec ion, is very rare. Prognosis Excellen

Neural Tumors

219

A

B FIGURE 14-12. Schwannoma. A and B. A 45-year-old woman wi h gradual onse o righ eye prop osis. C scan shows a well-circumscribed mass in he superior orbi . T ere is some bony ossa orma ion. On excision, his was a schwannoma. Schwannomas are mos commonly in raconal. ( con inued)

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C FIGURE 14-12. ( Con inued) Schwannoma. C. MRI shows a well-circumscribed lesion. T is shows he lesion isoin ense o muscle and hypoin ense o a .

-weigh edi mage

1

( con inued)

Neural Tumors

221

D FIGURE 14-12. ( Con inued) Schwannoma. D. On he muscle.

-weigh ed image, he lesion is hyperin ense o a and

2

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MESENCHYMAL TUMORS RHABDOMYOSARCOMA

R

habdomyosarcoma is he mos common primary orbi al malignancy o childhood. Classically, he child presen s wi h sudden onse o prop osis over days o weeks wi h an orbi al mass ound on imaging. Once suspec ed, he lesion should be immedia ely biopsied so rea men can be s ar ed as quickly as possible. Epidemiology and Etiology Age: Average age 7 o 8 years Gender: Equal occurrence in males and emales E iology: Rhabdomyosarcomas develop rom undi eren ia ed pluripo en ial mesenchymal cells. History Classically, rapid onse o unila eral prop osis is seen over days o a week, bu some pa ien s may presen less rapidly over many weeks. Examination Prop osis is seen wi h variable amoun s o adnexal response, such as edema, ery hema, and globe displacemen , and some imes a palpable mass ( Fig. 14-13A, C, D). T e superior nasal orbi is he mos common loca ion. Imaging C scan: Homogenous mass ha may have bony erosion ( Fig. 14-13B, E). MRI: Hypoin ense on 1 and hyperinense on 2 ( Fig. 14-13F). Variable enhancemen wi h gadolinium

Special Considerations Acu e prop osis in a child is an emergency. A er a rhabdomyosarcoma is suspec ed, he lesion needs o be biopsied wi hin 24 hours ollowed by promp ini ia ion o rea men . Dif erential Diagnosis Capillary hemangioma Orbi al pseudo umor Orbi al celluli is Rup ured dermoid cys Me as a ic umor Pathology T ere are our dis inc orms o rhabdomyosarcoma. Embryonic: poorly di eren ia ed spindle cells Alveolar: shows rounded rhabdomyoblas s Pleomorphic: rounded or s rap-like cells wi h cross-s ria ions Bo ryoid: rare orm wi h grape-like clus ers Treatment Once suspec ed, a C scan is done o iden i y he lesion. Urgen orbi al biopsy wi h pa hologic evalua ion is hen done o conf rm he diagnosis and classi y he ype o rhabdomyosarcoma. Sys emic evalua ion by a pedia ric oncologis is hen done. Radia ion and sys emic chemo herapy are he mains ays o rea men . Prognosis Survival ra es are 90% wi h his rea men .

Mesenchymal Tumors

223

A

B FIGURE 14-13. Rhabdomyosarcoma. A. An 8-year-old girl wi h 3-week his ory o progressive swelling o he righ eye. B. C scan shows large mass o he orbi , which on biopsy was a rhabdomyosarcoma. ( con inued)

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C

D FIGURE 14-13. ( Con inued) Rhabdomyosarcoma. C. and D. A 3-mon h-old girl wi h 1- o 2-week his ory o swelling o he righ eye. ( con inued)

Mesenchymal Tumors

225

E

F FIGURE 14-13. (Con inued) Rhabdomyosarcoma. E. C scan shows well-circumscribed mass wi h inden a ion o he globe. Biopsy o he mass revealed a rhabdomyosarcoma. T e ac ha his pa ien is younger han he ypical rhabdomyosarcoma pa ien shows ha rhabdomyosarcoma can presen a various ages. F. MRI o a rhabdomyosarcoma. T e 2-weigh ed image shows he lesion is hyperin ense o muscle and a .

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FIBROUS HISTIOCYTOMA

F

ibrous his iocy oma is a umor ha can be benign, locally aggressive, or malignan . I no comple ely excised, he benign orms can become malignan . T e umor is usually inf l ra ing and no encapsula ed. I usually presen s wi h prop osis and is o en accompanied by various orms o orbi al dys unc ion depending on he umor loca ion.

Epidemiology and Etiology Age: Middle age Gender: Equal occurrence in males and emales E iology: Arises de novo rom mesenchymal issue. T e umor can be benign, in ermedia e, or malignan . History Usually slow onse o prop osis wi h no def ni ive onse in he leas aggressive orm. T e malignan orm can presen more rapidly accompanied by diplopia, pain, swelling, and res ric ed eye movemen s. Examination Prop osis wi h ew o her orbi al signs in he benign orm T e more aggressive orms show signs o in amma ion, res ric ed eye movemen s, chemosis, and swelling ( Fig. 14-14A). Imaging C scan: Well-circumscribed orbi al mass bu he in ermedia e and malignan orms can be more inf l ra ive ( Fig. 14-14B). MRI: isoin ense on 1 and hyperin ense on 2 ( Fig. 14-14C) Enhances wi h gadolinium

Special Considerations Incomple e excision can lead o recurrence and he recurrence can be malignan . T e malignan , mos aggressive orm can me as asize. Dif erential Diagnosis Hemangiopericy oma Capillary hemangioma Schwannoma Pathology Fibrous-appearing his iocy ic cells ha orm a charac eris ic car wheel or s ori orm pat ern T e availabili y o immunohis ochemical s udies has allowed or more accura e classif ca ion o mesenchymal umors. Many o hese umors in he pas may have been incorrec ly classif ed. T is brings in o ques ion some o he prognos ic predic ions or hese umors. For example, some hemangiopericy omas ha are aggressive and even po en ially malignan umors may have classif ed as f brous his iocy omas. Treatment Comple e surgical excision. T e his ology will reveal he prognosis. Prognosis Depends on his ologic ype. I he benign or locally aggressive orms are comple ely excised, he prognosis is usually good.

Mesenchymal Tumors

227

A

B

C FIGURE 14-14. Fibrous histiocytoma. A. A 42-year-old man wi h progressive prop osis o he lef eye over 2 o 3 mon hs, increasing diplopia, and blurred vision. B. C scan shows an in raconal mass wi h possible in l ra ion o he op ic nerve. C. On MRI, he mass abu s bu does no in l ra e he op ic nerve. T e mass was removed and was a benign brous his iocy oma.

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LYMPHOPROLIFERATIVE TUMORS LYMPHOID HYPERPLASIA AND LYMPHOMAS

L

ymphoid lesions include a spec rum o lesions rom benign o malignan . Presence o even he benign lymphoid hyperplasia implies a risk in he u ure or he developmen o a lymphoma somewhere in he body. T ese lesions can occur in he orbi or subconjunc ival area and end o mold around s ruc ures ra her han displacing s ruc ures.

Epidemiology and Etiology Age: Older adul s Gender: Equal occurrence in males and emales E iology: Clonal expansion o abnormal precursor cells. T ere is a con inuum o disease rom he benign, localized lymphoid hyperplasia hrough malignan lymphoma. History A his ory o a painless, progressive mass T e exac his ory depends on he loca ion o he mass. An eriorly, i can presen as a visible, palpable mass. More pos eriorly, he symp oms will be more o prop osis or globe displacemen depending on he loca ion. Examination An eriorly, a visible, subconjunc ival salmonpa ch mass can be observed ( Fig. 14-15A, B). A so , di use mass can be palpa ed i he mass is in he an erior orbi bu no visible ( Fig. 14-15C). More pos erior umors will cause prop osis wi h symp oms depending on he umor loca ion ( Fig. 14-15E). T ese umors end o mold around orbi al s ruc ures and rarely displace or inf l ra e s rucures, so mo ili y or visual dis urbances are rare.

Imaging C scan: A mass ha molds around orbi al s ruc ures ra her han displacing or inf l ra ing ( Fig. 14-15D). MRI: Shows ex en o umor bu does no di eren ia e orbi al in amma ion and canno separa e lymphoid hyperplasia rom lymphoma ( Fig. 14-15F). PE scanning is used o look or lymphoid lesions elsewhere in he body. Special Considerations T ere are mul iple ways o evalua e a lymphoid lesion o de ermine whe her i is a benign or a malignan lesion. No all lesions can be clearly de ermined o be benign or malignan . Even he pa ien wi h benign lymphoid hyperplasia mus be observed sys emically or he developmen o a lymphoma elsewhere in he body over u ure years. Dif erential Diagnosis Orbi al pseudo umor Me as a ic orbi al umor Lymphangioma Pathology A collec ion o lymphocy es is seen, which iden if es he lesion as lymphoid. Microscopic appearance will give some evidence o he lesion as benign or malignan . Fresh issue is evalua ed o iden i y cell sur ace markers. Polyclonal lymphocy ic popula ions are less likely o develop sys emic disease; monoclonal lesions are more likely o accompany lymphoma elsewhere in he body. T e at emp o separa e lymphoid lesions in o benign and malignan is no exac and is bo h con roversial and con using; however, a his ime, i is he bes sys em available. Treatment T e lesions require biopsy o iden i y whe her hey are benign or malignan .

Lymphoproliferative Tumors

A sys emic workup is done o look or evidence o lymphoid lesions elsewhere in he body. rea men or localized benign orbi al disease is low-dose radia ion. More malignan lesions or sys emic disease usually requires radia ion and sys emic chemo herapy.

229

Prognosis T e prognosis is dependen on he ype o lymphoma. Many lymphomas are very responsive o rea men bu a high-grade lymphoma can be rapidly a al, even wi h rea men .

A

B FIGURE 14-15. Lymphoid hyperplasia and lymphoma. A and B. Subconjunc ival lymphoid in l ra es. T ese may be isola ed or here may be orbi al ex ension. T ey may be a reac ive process or a lymphoma. Where hese lesions all on he spec rum o benign versus malignan can only be di eren ia ed on biopsy. A was benign reac ive lymphoid hyperplasia, and B was a low-grade lymphoma. ( con inued)

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C

D FIGURE 14-15. ( Con inued) Lymphoid hyperplasia and lymphoma. C. A 65-year-old woman wi h swelling and redness o he lef eye. D. C scan shows a di use orbi al process, which on biopsy was a lymphoma. Pa ien was rea ed wi h radia ion and chemo herapy. ( con inued)

Lymphoproliferative Tumors

231

E

F FIGURE 14-15. ( Con inued) Lymphoid hyperplasia and lymphoma. E. Massive lid and orbi al in l ra ion by a lymphoma on he righ . F. MRI o a lymphoma. T e 2-weigh ed image shows he lesion o be hyperin ense o muscle and a .

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PLASMACYTOMA

lasmacy oma is an isola ed mass o plasma cells ha occurs in he bone. T is lesion can ex end rom he bone in o he orbi al so issue. Progression o a sys emic plasma cell umor is ermed mul iple myeloma.

Special Considerations As wi h lymphoma, plasma cell umors can be benign or malignan . T ey are di eren ia ed rom mul iple myeloma on he basis o sys emic involvemen in mul iple myeloma.

Epidemiology and Etiology Age: Six h and seven h decades Gender: Males more commonly a ec ed E iology: Rare proli era ion o plasma cells in so issues or bone o he orbi

Dif erential Diagnosis Mul iple myeloma Me as a ic disease His iocy ic disorders Malignan umor o he sinus

History Pa ien s presen wi h slow onse o a mass e ec wi h some in amma ory signs bu very rarely pain. Symp oms depend on he loca ion o he umor.

Pathology Classic plasma cells make up he umor. Vary rom ma ure o larger, imma ure cells depending on he umor. Di eren ia ion rom mul iple myeloma is on he basis o sys emic workup; mul iple myeloma having o her sys emic mani es a ions.

P

Examination I loca ed an eriorly, here is a palpable mass over or adjacen o an orbi al bone. T ere may be prop osis or globe displacemen depending on he loca ion ( Fig. 14-16A). Imaging C scan shows a lesion in or adjacen o bone wi h bony des ruc ion ( Fig. 14-16B, C).

Treatment Biopsy o he lesion, and hen a comple e sys emic workup. I he lesion is isola ed, higher dose irradia ion is indica ed. Chemo herapy may be indica ed. Prognosis Variable depending on he aggressiveness o he umor.

Lymphoproliferative Tumors

233

A FIGURE 14-16. Plasma cell tumor. A. A 70-year-old woman was no ed o have swelling around he lef eye. Examina ion shows prop osis wi h downward displacemen o he lef eye. ( con inued)

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B FIGURE 14-16. (Con inued) Plasma cell tumor. B. C scan shows a superior emporal lesion ha has eroded bone and may even be cen ered in bone. Biopsy revealed a plasmacy oma. ( con inued)

Lymphoproliferative Tumors

C FIGURE 14-16. ( Con inued) Plasma cell tumor. C. Axial C scan o plasmacy oma.

235

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HISTIOCYTIC DISORDERS

H

is iocy ic disorders are a rare group o abnormali ies o he mononuclear phagocy ic sys em. In he orbi , hey mos commonly presen as a uni ocal lesion o he superior bone o he orbi wi h secondary progressive prop osis. Epidemiology and Etiology Age: Children. Children younger han age 2 years are more likely o have sys emic disease, which is up o 50% a al. Over he age 2 years, he disease involves he bone wi hou sys emic involvemen bu is o en mul i ocal. T e older he child, he more likely he disease will be uni ocal and less severe. Gender: Males more commonly a ec ed E iology: Abnormal immune regula ion resul ing in an accumula ion o proli era ing dendri ic his iocy es History Orbi al swelling mos commonly superiorly over days o weeks. Examination Superior orbi al swelling wi h a variable amoun o mass e ec is he mos common presen a ion ( Fig. 14-17A). Younger children are more likely o have more swelling, mul i ocal bony involvemen , and sys emic involvemen . Imaging C scan will show a lesion adjacen o bone wi h bone erosion ( Fig. 14-17B, C).

Mos commonly in he superior, emporal orbi . Special Considerations T e older erm or hese disorders was hisiocy osis X wi h specif c mani es a ions ermed Let erer–Siwe disease, Hand–Schüller–Chris ian disease, and eosinophilic granuloma o bone. T ese erms are replaced by dif use so - issue his iocy osis, mul iple eosinophilic granuloma o bone, and uni ocal granuloma o bone. Dif erential Diagnosis Choles ea oma Repara ive granuloma Pathology Proli era ion o dendri ic his iocy es along wi h granulocy es and lymphocy es Treatment Bony lesions require a conf rma ory biopsy and hen debulking. T is rea men is o en cura ive bu in younger children, evidence o sys emic disease mus be sough . Rarely, a s eroid or low-dose radia ion is needed. rea men or sys emic disease in younger children may include s eroids, irradia ion, or cy o oxic agen s. In some cases, he disease may no respond o any hing. Prognosis Excellen in uni ocal disease in older children. Very young children wi h sys emic disease have a 50% mor ali y ra e.

Lymphoproliferative Tumors

237

A FIGURE 14-17. Histiocytic disorder. A. An 8-year-old boy wi h a 1- o 2-week his ory o swelling o his righ eye. Mild ery hema and swelling superiorly and downward displacemen o he globe on he righ is shown. ( con inued)

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B FIGURE 14-17. ( Con inued) Histiocytic disorder. B and C. C scans shows a superior in l ra e wi h bony erosion. Biopsy revealed a uni ocal granuloma o bone, which was rea ed wi h curet age. ( con inued)

Lymphoproliferative Tumors

C FIGURE 14-17. ( Con inued)

239

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LACRIMAL GLAND TUMORS EPITHELIAL TUMORS OF THE LACRIMAL GLAND

T

he lacrimal gland can harbor mul iple disease processes. T e mos common is in amma ory disease. Benign and malignan processes inheren o he lacrimal gland also occur. Biopsy is o en required o iden i y hese processes. Epidemiology and Etiology T is group includes a number o en i ies ha involve he lacrimal gland. T is does no include idiopa hic in amma ion or lymphoid inf l ra ion o he lacrimal gland. Age: Pleomorphic adenoma occurs in he our h and i h decades. Malignan mixed umors occur a an older age. Adenoid cys ic carcinoma has a peak incidence in he second and our h decades. Gender: Equal occurrence in males and emales E iology: Proli era ion o epi helial cells History Pleomorphic adenomas presen wi h progressive, downward, and inward displacemen o he globe, some imes wi h axial prop osis( Fig. 14-18A). T e process is painless, unlike he malignan umors o he lacrimal gland. Malignan mixed umors usually arise rom exis ing pleomorphic adenomas. Adenoid cys ic carcinoma will presen wi h more rapid grow h associa ed wi h signif can pain( Fig. 14-18E). T is pain is wha easily separa es many malignan lacrimal gland umors rom a benign pleomorphic adenoma. Examination Palpable mass in he superior, emporal quadran wi h displacemen o he globe down and in.

T e presence o in amma ion and he amoun o globe displacemen is variable depending on he e iology o he lacrimal gland mass. Imaging C scan: Pleomorphic adenomas show a globular, circumscribed mass. T e mass at ens and de orms he globe. T ere can be pressure expansion o he lacrimal ossa bu no erosion. Malignan lesions are no globular, are less well def ned, and may have bony erosions and calcif ca ions ( Fig. 14-18B, C, E, I). MRI: Valuable o def ne he ex en o in racranial ex ension in aggressive, malignan umors ( Fig. 14-18D, G). Special Considerations Mus comple ely excise a pleomorphic adenoma or i can recur as a malignan umor. Dif erential Diagnosis Idiopa hic in amma ion o he lacrimal gland Lymphoid inf l ra ion o he lacrimal gland ( Fig. 14-18H). Sarcoidosis Pathophysiology Pleomorphic adenoma: Proli era ion o epihelial cells wi h duc al and secre ory elemen s. Adenoid cys ic carcinoma: Small, benignappearing cells arranged in nes s, ubules, or in a cribri orm, Swiss-cheese pat ern Treatment Pleomorphic adenoma: Comple e excision wi hin he capsule Malignan umors: Individualize rea men . Generally ex ensive excision, especially wi h adenoid cys ic carcinoma and high-dose radia ion. Some umors will require orbi al exen era ion. Prognosis Pleomorphic adenoma: Excellen i comple ely excised Malignan umors: High ra e o recurrence over ime

Lacrimal Gland Tumors

241

A

B FIGURE 14-18. Lacrimal gland tumor. A. A 33-year-old man wi h slowly progressive, painless prop osis over a ew years. B and C. C scans show a round, well-circumscribed mass replacing he lacrimal gland. Comple e excision showed a pleomorphic adenoma. ( con inued)

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C

D FIGURE 14-18. (Con inued) Lacrimal gland tumor. D. MRI o a pleomorphic adenoma. T e shows he lesion is hyperin ense o a and muscle.

2

-weigh edi mage ( con inued)

Lacrimal Gland Tumors

243

E

F FIGURE 14-18. ( Con inued) Lacrimal gland tumor. E. A 58-year-old man wi h 6-mon h his ory o swelling and pain around he lef eye. Massive prop osis and swelling wi h downward displacemen o he eye are seen. F.C scan shows a large mass in he lacrimal gland area wi h bony des ruc ion. T is was adenoid cys ic carcinoma o he lacrimal gland. ( con inued)

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G

H FIGURE 14-18. (Con inued) Lacrimal gland tumor. G. MRI o an adenoid cys ic carcinoma. T e 2-weigh ed image shows he lesion is hyperin ense o muscle and a . H.T is 75-year-old man had swelling and downward displacemen o he lef eye or 2 o 3 mon hs. ( con inued)

Lacrimal Gland Tumors

245

I FIGURE 14-18. ( Con inued) Lacrimal gland tumor. I. C scan shows an enlarged lacrimal gland, which was a lymphoma on biopsy. Con ras he shape o his mass wi h he very round con our in B and C.

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MISCELLANEOUS ORBITAL TUMORS SECONDARYORBITAL TUMORS

S

econdary orbi al umors are umors ha invade he orbi rom adjacen s ruc ures including sinus umors, eyelid umors, and umors ha ex end in o he orbi rom wi hin he globe.

Epidemiology and Etiology E iology: Includes umors ha invade he orbi rom he sinus, eyelid, or globe. Sinus processes include mucoceles and squamous cell carcinoma. Eyelid umors include basal cell carcinoma ( Fig. 14-19A–C), sebaceous adenocarcinoma, and squamous cell carcinoma ( Fig. 14-19E–G). Re inoblas oma and choroidal melanoma ( Fig. 14-19D) can ex end rom he globe in o he orbi . Age and gender: Variable based on primary umor History O en, here is a his ory o ei her a neglec ed primary malignancy or a his ory o previous rea men o he primary malignancy. T e ime course o symp oms and grow h is dependen on he primary malignancy. Examination T ere may be obvious ex ernal signs o he primary umor such as in a neglec ed basal cell carcinoma. Likewise, in raocular umors ha ex end ou side he globe usually have ex ernal signs o in amma ion.

Sinus umors ha ex end in o he orbi may only show prop osis, o en wi h some direc ional displacemen o he globe. Imaging C scan: Dependen on he primary source. A umor rom he sinuses will show sinus changes. MRI: May help o def ne ex raocular ex ension o a primary ocular umor. Dif erential Diagnosis I he primary umor is known, he secondary process is easily suspec ed. Pathology T e pa hology is specif c or each o he individual processes. Treatment rea men is aimed a comple e excision i possible. I he umor is resec able and here is no evidence o dis an me as asis, hen ha is he rea men o choice. Depending on he umor, irradia ion a er excision can be used. For hose umors unresec able, he use o irradia ion and chemo herapy can be considered. rea men mus be individualized. Prognosis Generally poor. Even i he umor is hough o be removed, here is o en recurrence.

Miscellaneous Orbital Tumors

247

A

B FIGURE 14-19. Basal cell carcinoma with orbital invasion. A. A pa ien wi h a neglec ed medial can hal basal cell carcinoma presen s wi h prop osis. B and C. Axial and coronal C scans show a large medial orbi al mass ha was basal cell carcinoma on biopsy. T e pa ien required an orbi al exen era ion. ( con inued)

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C

D FIGURE 14-19. (Con inued) Basal cell carcinoma with orbital invasion. D. A pa ien wi h a large limbal mass. On undus examina ion, here was a large choroidal melanoma ha had ex ended ex rasclerally. ( con inued)

Miscellaneous Orbital Tumors

249

E FIGURE 14-19. ( Con inued) Squamous cell carcinoma with perineural spread. E. A 77-year-old man wi h an orbi al apex syndrome. ( con inued)

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F FIGURE 14-19. ( Con inued) Squamous cell carcinoma with perineural spread. F. C scan shows an in l ra ing mass a he apex wi h hickening along he superior orbi . Biopsy showed squamous cell carcinoma ha had spread in o he apex along he ron al nerve. T e pa ien had he his ory o a squamous cell carcinoma o he orehead excised 2 years prior. ( con inued)

Miscellaneous Orbital Tumors

251

G FIGURE 14-19. ( Con inued) Squamous cell carcinoma with perineural spread. G. Coronal C showing lesion a apex.

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METASTATIC ORBITAL TUMORS

M

e as a ic orbi al umors usually presen wi h orbi al in amma ion, pain, prop osis, and bony des ruc ion. Mos cases will have a known primary malignancy bu in up o 25%, he primary si e is unknown ( Fig. 14-20A–G). Epidemiology and Etiology Age: Mos commonly in he f h, six h, and seven h decades E iology and gender: Breas me as asis is he mos common in women. Lung me as asis is he mos common in men and second in women. O her e iologies include pros a e in men, gas roin es inal, and many have an unknown primary si e. History T e majori y o pa ien s will have a known primary malignancy a he ime o orbi al occurrence. T e onse o symp oms ends o be more rapid han in mos orbi al umors and can be accompanied by pain. Examination Prop osis is he mos common f nding. T is can be axial or displace he globe. A palpable mass may be presen ha is usually f rm. P osis, mo ili y dis urbances, and decreased vision may be presen because o he inf l ra ive na ure o he me as asis.

Imaging C scan: Highly variable appearance o hese lesions. May be discre e or invasive, cause bony erosion or hyperos osis (pros a e), and may only show muscle enlargemen . MRI: No diagnos ic and does no de ine bone well. May show ex en in o so issues. Dif erential Diagnosis Lymphoma Wegener’s granuloma osis Orbi al pseudo umor Pathology Special s ains and marker s udies can help iden i y he issue o origin in hose cases ha do no have a primary malignancy already iden if ed. Treatment Biopsy o iden i y he umor as me as a ic Sys emic rea men o he carcinoma is hen ha o he primary malignancy. Orbi al irradia ion will o en shrink he orbi al umor and diminish symp oms, ei her alone or in combina ion wi h chemo herapy. Prognosis T e prognosis is ha o he primary umor wi h me as asis. In mos cases, he prognosis is poor.

Miscellaneous Orbital Tumors

253

A

B FIGURE 14-20. Metastatic breast carcinoma. A and B. A 65-year-old woman wi h known his ory o breas cancer presen s wi h swollen, pain ul righ eye. T e pa ien has prop osis wi h a rozen globe and a corneal ulcer. C scan shows di use in l ra ion o he orbi wi h me as a ic breas carcinoma. Fur her workup revealed o her areas o me as a ic disease. ( con inued)

254

14 O RBITAL NEO PLASMS

C FIGURE 14-20. ( Con inued) Metastatic breast carcinoma. C. MRI hypoin ense o a and muscle.

1

-weigh ed image shows he lesion ( con inued)

Miscellaneous Orbital Tumors

255

D FIGURE 14-20. ( Con inued) Metastatic breast carcinoma. D.T e hyperin ense o a and muscle.

2

-weigh ed image shows he lesion ( con inued)

256

14 O RBITAL NEO PLASMS

E

F FIGURE 14-20. ( Con inued) Metastatic breast carcinoma. E and F. A 68-year-old man wi h known me as a ic lung adenocarcinoma presen s wi h prop osis and pain. No e he la eral mass on he righ . C scan shows a la eral orbi al mass wi h bone des ruc ion. ( con inued)

Miscellaneous Orbital Tumors

257

G FIGURE 14-20. ( Con inued) Metastatic breast carcinoma. G. An 8-year-old child wi h known neuroblas oma wi h orbi al me as asis. T e pho ograph shows he classic orbi al ecchymosis along wi h prop osis.

C H AP T ER

Orbi al rauma ORBITAL FRACTURES O BITAL FLOO FR CTU E

O

rbi al oor rac ures are he mos common ype o orbi al rac ure. T is is he resul o a blow o he eye i sel or o he bony rim. Many rac ures only resul in swelling and ecchymosis o he orbi al issues. T ose wi h en rapped issue and persis en diplopia, or wi h a large rac ure and enoph halmos, will require repair. Epidemiology and Etiology Age: Mos common in second o our h decades Gender: More common in males E iology: Direc orce o he in erior orbi al rim wi h buckling and rac ure o he oor is one mechanism. T e second mechanism consis s o orces ha raise he in raorbi al pressure and hen “blow-ou ” he hin orbi al oor. History rauma such as s , ngers, elbow, hi wi h a ball, and so or h 258

T e pa ien will of en have double vision af er he injury. Less commonly, he pa ien may no e orbi al swelling af er he rauma rom orbi al emphysema af er blowing he nose. Examination Orbi al swelling and ecchymosis are variable. Some rac ures have very lit le. In raorbi al hypes hesia and res ric ed mo ili y wi h diplopia are he mos speci c signs. As he orbi al swelling decreases, large rac ures will develop enoph halmos. Variable degrees o crepi ance may be presen as an indica ion o he rac ure. Imaging C scanning shows a rac ure o he orbi al loor o en wi h blood in he sinuses. A rac ure ha is very small is more likely o have en rapmen o orbi al issue han a very large rac ure. T e in erior rec us is almos never in he rac ure i sel bu issues around he muscle are en rapped.

Orbital Fractures

MRI does no image bone well and should no be used ini ially af er rauma. T e excep ion is he Whi e-Eyed Blowou Frac ure(WEBOF) where he muscle may be in he rac ure(below). Special Considerations Children and eenagers may sus ain an orbi al oor rac ure wi h no ecchymosis, bu wi h severe en rapmen o he in erior rec us muscle and associa ed pain, nausea, and vomi ing. T is is called a whi e-eyed blowou rac ure (WEBOF) ( Fig. 15-1). T ese pa ien s are very uncom or able and di cul o examine. T e en rapmen needs o be released wi hin 24 o 48 hours, as he muscle is severely en rapped and will become ischemic i no released. T is requires emergen surgery.

259

Treatment Open repair is required or pa ien s wi h unc ional diplopia ha does no improve as he swelling resolves. Frac ures involving more han 50% o he oor will resul in signi can enoph halmos and also should be considered or repair. Frac ures should be repaired wi hin 2 weeks o rauma wi h he excep ion o a WEBOF, which is an emergency. Mos rac ures ha are repaired will require an implan o some ype. Prognosis Good i repaired wi hin 2 weeks Some pa ien s will have direc muscle or nerve injury and ei her will no improve or may ake mon hs o improve.

A FIGURE 15-1. White-eyed blowout racture. A and B. A 9-year-old boy wi h a his ory o being hi wi h an elbow 1 day prior. He has pain, worse wi h eye movemen ; double vision; and has had nausea and some vomi ing. T e pho ograph shows his lack o ecchymosis and swelling as well as severe res ric ion o upgaze. ( continued)

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15 O RBITAL TRAUMA

B

C FIGURE 15-1. (Continued) White-eyed blowout racture. C. C scan shows a small rap-door f oor rac ure wi h issue en rapmen . T is rac ure needs o be repaired promp ly as he en rapped muscle may become ischemic. ( continued)

Orbital Fractures

261

D

E FIGURE 15-1. (Continued) Orbital f oor racture. D. T is 72-year-old woman ell and hi her eye on her bedpos . She has ull mo ili y bu signi can swelling, ecchymosis, and in raorbi al hypes hesia. E. C scan shows a large orbi al f oor rac ure. T e pa ien is a risk or developmen o enoph halmos rom he large rac ure.

262

15 O RBITAL TRAUMA

MEDIAL WALL FR CTU E

M

edial wall rac ures can be isola ed racures o he medial wall only or hey can be a par o larger rac ures involving he nose and sinuses. Isola ed rac ures are rea ed much like orbi al oor rac ures ( Fig. 15-2). Larger rac ures usually involve a mul idiscipline approach o he repair o he rac ures. Epidemiology and Etiology Age: Mos common in second hrough our h decades Gender: More common in males E iology: Direc rac ures occur rom s riking a solid objec . Indirec (blow-ou ) racures occur in associa ion wi h and by similar mechanisms as orbi al oor rac ures. History rauma his ory is variable. Symp oms include diplopia and cosme ic de ormi ies depending on he ex en o he nasal rac ures. Examination Medial rec us en rapmen wi h diplopia and even ual enoph halmos are he wo ocular mani es a ions ha may occur. Direc rac ures of en have signi can damage o he nasal bridge and medial orbi . T e nasal bridge may be depressed wi h elecan hus. O her ndings ha can occur include epis axis, orbi al hema oma, cerebral spinal

uid rhinorrhea, and damage o he lacrimal drainage sys em. Imaging C scanning will show he ex en o he rac ure and assis wi h po en ial planning o he repair. MRI does no image bone well and should no be used ini ially af er rauma. Special Considerations Medial wall rac ures wi h en rapmen o he medial rec us need o be repaired sooner han oor rac ures (wi hin 1 week) i possible. Treatment I isola ed, medial wall rac ures of en do no need repair. Medial rec us en rapmen wi h diplopia is one indica ion or repair. I he rac ure is large, enoph halmos can develop and require surgery o build up he orbi . Implan s are some imes placed. Larger rac ures involving he nasal bridge and medial orbi require repair and pla ing, usually in conjunc ion wi h an o olaryngology specialis . Prognosis Good. Larger rac ures may require muliple surgeries and revisions.

Orbital Fractures

263

A

B

C FIGURE 15-2. Medial wall racture. A o C. A 55-year-old man s ruck in he ace wi h an unknown objec presen ed wi h horizon al diplopia. Mo ili y is res ric ed in he righ eye in bo h adduc ion and abduc ion. ( continued)

264

15 O RBITAL TRAUMA

D FIGURE 15-2. (Continued) Medial wall racture. D and E. C scans show a medial wall orbi al rac ure wi h he medial rec us muscle pulled in o he rac ure. ( continued)

Orbital Fractures

E FIGURE 15-2. (Continued)

265

266

15 O RBITAL TRAUMA

O BITAL OOF FR CTU E

O

rbi al roo rac ures ( Fig. 15-3) are rare rac ures ha need o be recognized because o he po en ial or li e- hrea ening neurologic sequelae. T ere may jus be a small rac ure wi h no neurologic problems or here may be signi can in racranial air and bleeding. rea men is in conjunc ion wi h neurosurgery. Epidemiology and Etiology Age: Mos common in second hrough our h decades Gender: More common in males E iology: Blun rauma or direc injury by a hin objec ha goes above he globe under he superior orbi al rim. An isola ed roo racure is rare. History rauma his ory will of en sugges highenergy orces ha caused he injury. T ese include hydraulic air hoses, a blun objec wi h high veloci y, and so or h. Examination Poor upgaze, supraorbi al hypes hesia, and more swelling superiorly han in eriorly sugges an orbi al roo rac ure.

En rapmen o he superior rec us or superior oblique muscle is ex remely rare. Imaging C scanning will show he rac ure usually jus inside he orbi al rim. MRI does no image bone well and should no be used ini ially af er rauma. MRI can be o value o evalua e in racranial injury. Special Considerations Impor an o consul neurosurgery or he po en ial o CNS complica ions wi h a roo rac ure Treatment Repair o a roo rac ure is usually done or neurologic reasons ra her han ocular. Any pla ing and repair is done via cranio omy. Nondisplaced rac ures do no require repair. Prognosis Variable depending on he ex en o associa ed CNS injuries

Orbital Fractures

267

A

B FIGURE 15-3. Orbital roo racture. A.T e pa ien was s ruck in he eye wi h a hydraulic air hose. Examina ion shows signi can swelling, wi h decreased upgaze and supraorbi al hypes hesia. B. C scan shows an orbi al roo rac ure wi h in racranial hemorrhage.

268

15 O RBITAL TRAUMA

ZYGOMATIC FR CTU E

Z

ygoma ic rac ures are he resul o signi can rauma ic orce o he zygoma ic area ( Fig. 15-4A–C). T e resul injury and symp om depend on he direc ion and amoun o displacemen o he bone. Repair should be done wi hin he rs week when needed. Epidemiology and Etiology Age: Young adul s Gender: Males mos common E iology: rauma wi h orce direc ed a he zygoma History rauma wi h signi can orce Pa ien s of en complain o pain and di cul y opening heir mou h and chewing. Examination Ini ial ndings may be minimal i here is signi can swelling and ecchymosis o he orbi and cheek. Depressed cheek, orbi al rim s ep o , and inabili y o open he mou h wide are common ndings.

Imaging C scanning shows rac ure o he zygoma ic arch a he ron al-zygoma ic su ure and a he maxillary–zygoma ic su ure. T e zygoma ic arch is displaced in various direc ions depending on he direc ion o rauma. T ere is usually an associa ed orbi al oor rac ure ( Fig. 15-4D–E). MRI does no image bone well and should no be used ini ially af er rauma. Treatment Repair is required or mos rac ures wi h any signi can displacemen . T is should be done as soon as he swelling has lessened. T is is done wi h open reduc ion and pla ing as needed. Nondisplaced rac ures do no require repair. Prognosis Excellen i repaired promp ly

Orbital Fractures

269

A

B FIGURE 15-4. Zygomatic racture. A. A 43-year-old man s ruck on he righ cheek and eye wi h a ba . T ere is f at ening o he cheek wi h rismus. B and C. C scans show a zygoma ic rac ure. ( continued)

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15 O RBITAL TRAUMA

C

D FIGURE 15-4. (Continued) Zygomatic racture. D and E. C scans show a smaller minimally displaced zygoma ic rac ure wi h associa ed orbi al f oor rac ure. ( continued)

Orbital Fractures

E FIGURE 15-4. (Continued)

271

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15 O RBITAL TRAUMA

MISCELLANEOUS TRAUMA O BITAL HEMO

O

HAGE

rbi al hemorrhage as he resul o orbi al rauma is common and rarely requires any speci c rea men ( Fig. 15-5A&B). Spon aneous orbi al hemorrhage is rare and requires evalua ion o he orbi or a source o bleeding, al hough none may be ound. Orbi al or eyelid surgery is also a cause. T e need o drain an orbi al bleed is very rare. Epidemiology and Etiology Age: Any Gender: Males more common because o he higher incidence o rauma E iology: rauma , surgery ( Fig. 15-5E) or orbi al vascular lesion such as lymphangioma or vascular mal orma ion

History His ory is ha o rauma. Wi h vascular mal orma ions, here is sudden onse o orbi al pain, pressure, prop osis, and some imes ecchymosis. Examination Examina ion reveals prop osis wi h variable symp oms depending on he severi y o he hemorrhage. T ere can be o her ocular and orbi al injuries i he cause is rauma. Mild hemorrhage may only reveal prop osis. Severe hemorrhage can resul in no ligh percep ion vision, wi h severe prop osis, corneal exposure, rozen globe, eleva ed in raocular pressure, and inabili y o close he eye because o he severe prop osis ( Fig. 15-5C–D). Imaging C scan: May show a discre e mass or more in l ra ive lesion. Mos commonly

he hemorrhage is di use wi hin he issues MRI: Acu e hemorrhage is hypoin ense on 1 and hyperin ense in 2. When blood is more han 7 days old, i will become hyperinense on 1 and variable on 2. Special Considerations In spon aneous hemorrhage, an orbi al vascular mal orma ion needs o be looked or. I no hing is seen on imaging af er he acu e hemorrhage, a ollow-up MRI wi h gadolinium may iden i y a lesion. Dif erential Diagnosis Spon aneous hemorrhage (no rauma) includes he ollowing. Lymphangioma Venous mal orma ion and varix Ar eriovenous mal orma ion Treatment Observa ion, unless here is visual loss Mild visual loss needs moni oring wi h in ravenous s eroids, ace azolamide, and possible la eral can ho omy. I visual loss is more severe, immedia e la eral can holysis is indica ed along wi h highdose in ravenous s eroids. Orbi al imaging is done o look or locula ed blood. T e blood is usually wi hin he orbi al issues and orbi al drainage or even decompression is rarely o value. T e excep ion would be a locula ed hemorrhage such as in a lymphangioma. Prognosis Chance o permanen visual loss is presen wi h severe hemorrhage. Less severe hemorrhages resolve wi hou sequelae.

Miscellaneous Trauma

273

A

B FIGURE 15-5. Orbital hemorrhage. A. A 17-year-old girl wi h orbi al hemorrhage a er being poked in he eye wi h a eld hockey s ick. T ere was no o her injury no ed on C scan. Vision was normal bu he orbi was modera ely igh . She was observed or progressive hemorrhage bu he ullness o he orbi resolved overnigh . B.T ere was a small corneal delle ha resolved wi h lubrica ion and resolu ion o he hemorrhage. ( continued)

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15 O RBITAL TRAUMA

C

D FIGURE 15-5. (Continued) Orbital hemorrhage. C. Severe orbi al hemorrhage a er re robulbar injec ion in pa ien on Coumadin. D. C scan shows di use hemorrhage wi hin he issue and no locula ed blood. No e he s re ching and s raigh ening o he op ic nerve. ( continued)

Miscellaneous Trauma

E FIGURE 15-5. (Continued) Orbital hemorrhage. E. Bila eral orbi al hemorrhage a er blepharoplas y.

275

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15 O RBITAL TRAUMA

O BITAL FO EIGN BODIES

O

rbi al oreign bodies mus always be suspec ed in any kind o orbi al rauma ( Fig. 15-6A–J). Mos oreign bodies are removed surgically wi h he excep ion o cer ain iner ma erials ha are deep in he orbi .

Epidemiology and Etiology Age: Any age Gender: More common in males E iology: Foreign bodies can en er he orbi be ween he globe and he orbi al wall or by double per ora ion o he globe. History T e his ory may be o a speci c oreign body en ering he orbi . he more di icul si ua ion is where here is rauma wi h a poor his ory bu wi h wounds ha could sugges a oreign body. Examination I he oreign body is an erior, i may be palpable or even visible. I i is deeper, here may very ew signs excep an en rance wound, or here may be signi can hemorrhage and swelling .

Imaging Imaging is key o iden i ying and localizing an orbi al oreign body and C scanning is he imaging modali y o choice. MRI should never be done af er rauma unless a me allic oreign body has been ruled ou . Glass, plas ic, and organic oreign bodies may no show up well wi h C scanning. T ese can be bet er visualized wi h MRI scanning, bu even MRI may no show hese oreign bodies. Treatment Orbi al oreign bodies should be removed i hey are organic, cause symp oms, or i hey have sharp edges so ha migra ion could cause damage. T e posi ion o he oreign body can a ec he decision o remove a oreign body. T e more pos erior i is, he more di cul i will be o remove i . Any oreign body lef in place requires counseling o he pa ien abou he po en ial or u ure ex rusion or in ec ion. I a oreign body is suspec ed, wound and orbi al explora ion is required even i imaging is nega ive. Prognosis Good. Organic oreign bodies can someimes be re ained and lead o chronic in amma ion or in ec ion.

Miscellaneous Trauma

277

A

B FIGURE 15-6. Orbital oreign body. A. A 12-year-old sho wi h a BB gun 2 weeks prior. T e child has a longs anding eso ropia. No e he mild ery hema o he la eral righ globe. B. C scan shows he BB in he an erior la eral orbi . BBs can be le in he orbi wi hou a problem. In his case, because o he an erior loca ion and rela ive ease o removal, he BB was removed. ( continued)

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15 O RBITAL TRAUMA

C

D FIGURE 15-6. (Continued) Orbital oreign body. C and D. Mul iple eyelid lacera ions rom being s ruck wi h a wineglass ha broke. A re ained oreign body mus always be suspec ed wi h broken glass. On C scan, a oreign body is no ed. ( continued)

Miscellaneous Trauma

279

E

F FIGURE 15-6. ( Continued) Orbital oreign body. E.T e glass oreign body ha was removed. T e wineglass was leaded crys al, which is why i showed up so well on C scan. F.T e pa ien was s ruck in he eye wi h a pencil 4 mon hs prior. He presen ed wi h mild irri a ion o he le orbi . No e he lump in he le medial can hus. ( continued)

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15 O RBITAL TRAUMA

G

H FIGURE 15-6. (Continued) Orbital oreign body. G. C scan shows a medial orbi al opaci y. H.T e medical orbi al opaci y urned ou o be par o a pencil. ( continued)

Miscellaneous Trauma

281

I

J FIGURE 15-6. ( Continued) Orbital oreign body. I and J.T e pa ien ran in o a bush and an eyelid lacera ion was revealed on examina ion. I. C scan showed no oreign body. J. Explora ion showed mul iple wood ragmen s.

282

15 O RBITAL TRAUMA

MUCOCELE

D

es ruc ion o he sinus os ium rom rauma or sinus disease can resul in a mucous- lled sinus ha can hen expand in o he orbi ( Fig. 15-7). Symp oms depend on he loca ion o he mucocele. rea men is usually aimed a excision o he cys and obli era ion o he sinus. Epidemiology and Etiology Age: Any age bu mos common age is 40 o 70 years Gender: More common in males E iology: Blockage o he sinus os ium resul s in a mucous- lled cys ha can expand wi h ime. History T ere is usually a his ory o rauma o he sinuses or a long his ory o sinus disease. T e orbi al process shows very slow insidious onse o prop osis or globe displacemen . More pos erior mucoceles can presen wi h slow visual loss. Rarely, i he mucocele becomes in ec ed, he symp oms can have a rapid progression.

Examination Findings will depend on he loca ion o he mucocele. T e mass expands slowly and will displace he globe, cause prop osis, and, i pos erior, may cause op ic nerve compression and/ or an orbi al apex syndrome. Special Considerations Fron al and e hmoidal sinus mucoceles are he mos common, wi h sphenoid sinus mucoceles being less common. Dif erential Diagnosis Orbi al abscess Primary sinus umor wi h orbi al ex ension Treatment Surgical excision wi h obli era ion o he a ec ed sinus Prognosis T ere can be recurrences. Mos mucoceles are easily rea ed surgically unless ex remely large.

Miscellaneous Trauma

283

A

B FIGURE 15-7. Orbital mucocele. A. A pa ien wi h a his ory o prior acial rac ures presen s wi h he complain ha his le eye is “ou o place.” T e dura ion o his condi ion is unknown. B. C scan shows a large ron al sinus mucocele displacing he globe downward.

Index NO E: Locators ollowed by ‘ ’ re er to f gures.

A Acid and alkali burns, 123 Acquired myogenic ptosis, 76–77 Acquired nasolacrimal duct obstruction, 130–131 Actinic keratosis, 32–33 , 34, 40 pigmented, 4 Acute spastic entropion, 56–57 Ankyloblepharon, 120–121 Anophthalmos, 170 Antibiotic ointment, 54 Apocrine hydrocystoma, 16–17 Aponeurotic ptosis, 78–79 , 90 Arteriovenous mal ormations (AVM), 200–203 , 272 Aspergillosis, 152–153 Atopic disease, 123 AVM. see Arteriovenous mal ormations (AVM)

B Balloon dacryoplasty, 130 Basal cell carcinoma, 18, 20, 24, 30, 36–39 , 40, 42 cystic, 16 pigmented, 4, 44 Bell’s palsy, 64 Benign adenoma, 14 Benign essential blepharospasm, 104–105 , 106 Benign eyelid lesions apocrine hydrocystoma, 16–17 cutaneous horn, 6–7 epidermal inclusion cyst, 8–9 hemangioma, 22–23 molluscum contagiosum, 10–11 nevocellular nevi, 20–21 papilloma, 2–3 seborrheic keratosis, 4–5 syringoma, 14–15 trichoepithelioma, 18–19 xanthelasma, 12–13 Birth trauma, 116 Blepharitis, 130, 132 Blepharophimosis, 108–109 , 110 Botulinum toxin injection, 104

Brow ptosis, 96–97 Burns, 54–55

C Canalicular eyelid laceration, 50–51 Canalicular obstruction, 132–133 Canaliculitis, 137–138, 139 Capillary hemangiomas, 180–183 , 218, 222, 226 Cavernous hemangiomas, 184–187 , 192, 208 Cavernous sinus thrombosis, 146, 150 Central eyelid laceration, 49 Chalazion, 8, 24–25 , 46 Cherry angioma. see Hemangioma Cholesteatoma, 236 Chronic blepharitis, 42 Chronic chalazion, 42 Chronic conjunctivitis, 126, 137 Chronic progressive external ophthalmoplegia (CPEO), 74, 82 Cicatricial ectropion, 62, 66–67 , 68 Coloboma, 116–117 Congenital distichiasis, 72 Congenital entropion, 112, 114–115 Congenital eyelid anomalies ankyloblepharon, 120–121 blepharophimosis, 108–109 coloboma, 116–117 distichiasis, 118–119 entropion, 114–115 epiblepharon, 112–113 epicanthus, 110–111 Congenital myogenic ptosis, 74–75 Congenital nasolacrimal duct obstruction, 126–127 Congenital orbital anomalies microphthalmos, 170–173 Congenital orbital tumors dermoid cysts, 174–177 lipodermoids, 178–179 Congenital ptosis, 76, 78 Conjunctival granulomas, 164 Conjunctival papillomas, 2 Contralateral ptosis, 102 Copious corneal lubrication, 54

Corneal abrasion, 57 CPEO, see chronic progressive external ophthalmoplegia (CPEO) Cryptophthalmos, 120 Cutaneous horn, 6–7

D Dacryoadenititis, 164 Dacryocystitis, 134–135, 136 , 140 Dacryocystocele, 128 Dacryocystorhinostomy, 130 Dermal nevus, 2 Dermatochalasis, 98–99 , 100 Dermatof broma, 20 Dermoid cysts, 174–177 Discoid lupus, 32 Distichiasis, 118–119 Dog bites, 48, 52 with eyelid lacerations, 53

E Eaton–Lambert syndrome, 82 Eccrine hydrocystoma, 16 Ectropion cicatricial, 66–67 involutional, 62–63 mechanical, 68–69 paralytic, 64–65 symblepharon, 70–71 trichiasis, 72–73 Electrical burn with necrosis, 55 Entropion, 58, 60, 114–115 acute spastic, 56–57 , 58 cicatricial, 56, 58, 60–61 , 72, 122 congenital, 112, 114–115 involutional, 56 spastic, 72 Epiblepharon, 112–113 , 114 Epicanthus, 110–111 Epidermal inclusion cyst, 8–9 , 10, 18 Epithelial tumors, 240–245 Eyelid abscess, 26 burns, 54–55 in ammation

285

286

INDEX

Eyelid (cont.) chalazion, 24–25 oppy eyelid syndrome, 28–29 hordeolum, 26–27 neoplasms actinic keratosis, 32–33 basal cell carcinoma, 36–39 Kaposi’s sarcoma, 46–47 keratoacanthoma, 30–31 lentigo maligna, 34–35 malignant melanoma, 44–45 sebaceous adenocarcinoma, 42–43 squamous cell carcinoma, 40–41 retraction, 102–103 trauma canalicular eyelid laceration, 50–51 dog bites, 52–53 eyelid burns, 54–55 marginal eyelid laceration, 48–49 Eyelid malpositions Brow ptosis, 96–97 dermatochalasis, 98–99 dyskinesis benign essential blepharospasm, 104–105 hemi acial spasm, 106–107 ectropion cicatricial, 66–67 involutional, 62–63 mechanical, 68–69 paralytic, 64–65 symblepharon, 70–71 trichiasis, 72–73 entropion acute spastic entropion, 56–57 cicatricial entropion, 60–61 involutional entropion, 58–59 mechanical ptosis, 90–91 neurogenic ptosis Horner’s syndrome, 88–89 marcus gunn jaw winking syndrome, 84–87 myasthenia gravis, 82–83 third nerve palsy, 80–81 pseudoptosis, 94–95 ptosis acquired myogenic, 76–77 aponeurotic, 78–79

congenital myogenic, 74–75 traumatic, 92–93 retraction, 102–103

F Fat prolapse, 178 Fibrous histiocytoma, 184, 192, 208, 218, 226–227 Floor racture, 258–261 Floppy eyelid syndrome, 28–29 Fractures, orbital trauma oor, 258–261 medial wall, 262–265 orbital roo , 266–267 zygomatic, 268–271

H Hemangioma, 22–23 , 46 Hemangiopericytoma, 184, 192– 195 , 218, 226 Hemi acial spasm, 104, 106–107 Histiocytic disorders, 232, 236–239 Hordeolum, 26–27 Horner’s syndrome, 88–89 Hyperkeratotic actinic keratosis, 30 Hypotropia, 102

I Idiopathic orbital in ammation. see Orbital pseudotumor Idiopathic orbital pseudotumor, 164 Intracranial meningiomas, 210 Involutional ectropion, 62–63 , 68 Involutional entropion, 56, 58–59 , 60, 72

K Kaposi’s sarcoma, 46–47 Keratitis sicca, 130, 132 Keratoacanthoma, 10, 30–31 , 40

L Lacrimal f stula, 129 Lacrimal gland tumors epithelial tumors, 240–245 Lacrimal in ections canaliculitis, 137–138, 139 dacryocystitis, 134–135, 136

Lacrimal obstructions acquired nasolacrimal duct, 130–131 canalicular, 132–133 congenital nasolacrimal duct, 126–127 dacryocystocele, 128 lacrimal f stula, 129 Lacrimal sac tumor, 134, 140–141 Lentigo maligna, 34–35 Lipodermoids, 178–179 Lymphangiomas, 188–189, 190 –191 , 196, 208, 210, 228, 272 Lymphoid hyperplasia and lymphomas, 228–231 Lymphoid inf ltration o lacrimal gland, 240 Lymphoma, 160, 178, 252 Lymphoproli erative tumors histiocytic disorders, 236–239 lymphoid hyperplasia and lymphomas, 228–231 plasmacytoma, 232–235

M Malignant melanoma, 20, 34, 44–45 survival in relation to tumor depth, 44t Marcus gunn jaw winking syndrome, 84–87 Marginal eyelid laceration, 48–49 Mechanical ectropion, 68–69 Mechanical ptosis, 90–91 Medial wall racture, 262–265 Melanocytic nevus, 46 Melanoma, 22 Meningiomas, 210–217 Mesenchymal tumors f brous histiocytoma, 226–227 rhabdomyosarcoma, 222–225 Metastatic breast carcinoma, 253 Metastatic disease, 160, 232 Metastatic orbital tumors, 143, 152, 228, 252–257 Metastatic tumor, 222 Microphthalmos, 120, 170–173 Migrated punctal plug, 137 Molluscum contagiosum, 2, 8, 10 eyelid margin, lesions o , 11 Mucocele, 282–283 rontal and ethmoidal sinus, 282 sinusitis with, 152

INDEX Mucormycosis. see Phycomycosis Multiple myeloma, 232 Myasthenia gravis, 74, 78, 82–83 Myokymia, 106

N Neural tumors meningiomas, 210–217 neurof bromas, 208–209 optic nerve gliomas, 204–207 schwannomas, 218–221 Neurof bromas, 208–209 Neurof bromatosis, 204 Neurogenic ptosis, 76 Nevi. see Nevocellular nevi Nevocellular nevi, 20–21 Nevus, 44

O OCP. see Ocular cicatricial pemphigoid (OCP) Ocular cicatricial pemphigoid (OCP), 122–123, 124 –125 Oculopharyngeal dystrophy, 74 Optic nerve gliomas, 204–207 , 210 Optic nerve meningioma, 204 Orbital abscess, 143, 146–149 , 282 Orbital cellulitis, 142–143, 143 –145 , 146, 150, 155, 160, 222 Orbital oreign bodies, 276–281 Orbital hemorrhage, 272–275 Orbital in ections abscess, 146–149 aspergillosis, 152–153 cellulitis, 142–143, 143 –145 phycomycosis, 150–151 Orbital in ammation pseudotumor, 160–163 sarcoidosis, 164–167 RO, 154–155, 156 –159 Wegener’s granulomatosis, 168–169 Orbital in ammatory disease, 100 Orbital lymphoma, 155 Orbital neoplasms congenital orbital tumors dermoid cysts, 174–177 lipodermoids, 178–179

lacrimal gland tumors epithelial tumors, 240–245 lymphoproli erative tumors histiocytic disorders, 236– 239 lymphoid hyperplasia and lymphomas, 228–231 plasmacytoma, 232–235 mesenchymal tumors f brous histiocytoma, 226–227 rhabdomyosarcoma, 222–225 metastatic orbital tumors, 252–257 neural tumors meningiomas, 210–217 neurof bromas, 208–209 optic nerve gliomas, 204–207 schwannomas, 218–221 secondary orbital tumors, 246–251 vascular orbital tumors AVM, 200–203 capillary hemangiomas, 180–183 cavernous hemangiomas, 184–187 hemangiopericytoma, 192–195 lymphangiomas, 188–189, 190 –191 orbital varices, 196–199 Orbital pseudotumor, 143, 146, 150, 155, 160–163 , 208, 222, 228, 252 Orbital roo racture, 266–267 Orbital trauma oreign bodies, 276–281 ractures oor, 258–261 medial wall, 262–265 orbital roo , 266–267 zygomatic, 268–271 hemorrhage, 272–275 mucocele, 282–283 Orbital varices, 196–199

P Papilloma, 2–3 Paralytic ectropion, 62, 64–65 , 68 Parinaud syndrome, 102

287

Phycomycosis, 143, 146, 150–151 , 152 Pigmented actinic keratosis, 4 Plasmacytoma, 232–235 Pleomorphic adenoma, 240 Polyclonal lymphocytic populations, 228 Preseptal cellulitis, 26, 143 Prolapsed lacrimal gland, 178 Pseudoptosis, 94–95 Pseudotumor, 160–163 Punctal abnormalities, 130 Punctal dysgenesis, 126 Pyogenic granuloma, 22, 46

R Rhabdomyosarcoma, 180, 222–225 Ruptured dermoid cyst, 160, 222

S Sarcoidosis, 164–167 , 240 Schwannomas, 184, 192, 218–221 , 226 Sebaceous adenocarcinoma, 24, 42–43 Seborrheic keratosis, 4–5 , 20, 34 pedunculated, 2 Secondary orbital tumors, 246–251 Sinus tumor, 282 Solitary neurof broma, 2 Spastic entropion, 72 SPK. see Superf cial punctate keratitis (SPK) Squamous cell carcinoma, 24, 32, 36, 40–41 , 42 Stevens-Johnson syndrome, 123 Superf cial punctate keratitis (SPK), 58 Symblepharon, 70–71 Syringoma, 8, 10, 14–15 , 18

T etanus immunization, 48, 52 T ird nerve palsy, 80–81 , 82 T yroid-related ophthalmopathy ( RO), 100, 154–155, 156 –159 , 160 rachoma, 123 raumatic ptosis, 90, 92–93

288

INDEX

richiasis, 72–73 , 122, 126 richoepithelioma, 18–19 , 36

V Vascular orbital tumors AVM, 200–203 capillary hemangiomas, 180–183 cavernous hemangiomas, 184–187 hemangiopericytoma, 192–195

lymphangiomas, 188–189, 190 –191 orbital varices, 196–199 Venous mal ormation and varix, 272 Verruca vulgaris, 4

W WEBOF. see White-eyed blowout racture (WEBOF) Wegener’s granulomatosis, 168–169 , 252

White-eyed blowout racture (WEBOF), 259

X Xanthelasma, 12–13

Z Zygomatic racture, 268–271