Contractile Peripapillary Staphyloma with Light Stimulus to The

CLINICOPATHOLOGIC REPORTS, CASE REPORTS, AND SMALL CASE SERIES

SECTION EDITOR: W. RICHARD GREEN, MD

ing the supraorbital notch revealed High-resolution MRI scans of the Lymphoepitheliomalike lymphoepithelioma with perineural orbits were obtained with conven- Carcinoma of the Orbit invasion. tional pulse sequences. They re- The ophthalmologic exam- vealed a multilocular cystlike mass in Lymphoepitheliomalike carcinoma ination results revealed a best- the medial aspect of the right orbit (LELC) of the skin is an uncommon corrected visual acuity of 20/70 OD (Figure 1). Other imaging features cutaneous malignancy with the po- and 20/15 OS; the visual acuity had included fluid-fluid layers within the tential for distant metastasis.1 We de- been stable in the right eye since a lesion and peripheral enhancement. scribe a patient with LELC of the mid scleral buckle procedure for retinal A computed tomography scan of the forehead and an asymptomatic or- detachment was performed approxi- head and neck area that was ob- bital mass, which when biopsied mately 18 years prior to this presen- tained 6 weeks prior to the MRI scan proved to be a lymphoepithelioma- tation. The external examination re- did not show an orbital mass. like carcinoma (LELC). vealed quiet globes; the Hertel An orbital biopsy of the mass exophthalmometry measurement was performed through a modified Report of a Case. A 45-year-old man was 19 mm in each eye. Results of Lynch incision (superonasal orbi- was referred to the Ophthalmology the extraocular motility examina- totomy).2 The cystic mass was iden- Clinic at the University of Texas M. tion were normal. The pupils were tified in the superonasal orbit, ap- D. Anderson Cancer Center, Hous- equal, round, and reactive to light proximately 25 mm posterior to the ton, for the evaluation of an asymp- with no afferent pupillary defect. The anterior lacrimal crest. The mass ex- tomatic right-sided orbital mass that confrontation visual fields were nor- tended posteriorly toward the supe- had been identified on recent mag- mal in the left eye, but superior and rior orbital fissure, and there was no netic resonance imaging (MRI) scans. temporal field defects were found in clearly defined capsule. Partial exci- The patient denied any orbital symp- the right eye; it was not clear whether sion of the mass was performed, and toms or signs except for numbness the field deficit was new or due to the frozen-section diagnosis was con- and paresthesia in the area of the the previous retinal detachment in sistent with poorly differentiated right-sided supraorbital notch, which the right eye. The slitlamp exami- LELC. The patient underwent con- had been present for at least 2 years. nation and applanation tonometry current chemoradiotherapy, consist- He had a history of a skin nodule on measurements were normal in both ing of 2 cycles of cisplatin followed his mid forehead, of which a biopsy eyes. A dilated fundus examination by 3 cycles of cisplatin and 5-fluoro- specimen had been taken at another revealed a 0.4 cup-disc ratio in the uracil; external beam radiotherapy institution and diagnosed as LELC of right eye and a 0.2 cup-disc ratio in was administered in 33 fractions, at the skin. Subsequently, 2 wide-local the left eye. There was evidence of a total dose of 6600 rad (66 Gy). excisions with positive margins were a scleral buckle band with an at- The material from the fore- performed on the nodule, and bi- tached retina in the right eye; there head lesion that was provided by opsy specimens of the skin overly- were no choroidal striae. another institution consisted of

A B C

Figure 1. A, An axial T1-weighted magnetic resonance imaging (MRI) scan, tailored to the orbits, reveals a multilobular, slightly hyperintense lesion in the upper medial aspect of the right orbit (arrows). B, An axial T2-weighted MRI scan demonstrates a cystlike appearance, with a fluid-fluid layer (arrows) best seen in the anterior component of the tumor. C, An axial postcontrast fat-suppressed T1-weighted image shows peripheral enhancement and a lack of central enhancement.

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Figure 2. A, Deep nodular infiltrate (hematoxylin-eosin, original magnification ϫ4). B, Island of atypical cells (pleomorphic nuclei with prominent nucleoli) surrounded by small lymphocytes (original magnification ϫ40). C, Immunohistochemical study performed in the orbital lesion, which shows positive expression of cytokeratin (anti-cytokeratin stains: AE1/AE3, CAM 5.2, Zym 5.2, MNF 116; amino-ethylcarbazole, original magnification ϫ40).

a skin ellipse containing a dense, Swanson et al11 in 1988, at least 21 tures of the orbital mass were sug- nodular infiltrate of the deep der- cases have been described. The pa- gestive of a benign cystic lesion. Al- mis (Figure 2A). Islands of large tient described here is slightly though there were minimal to no cytologically malignant cells with younger than most previously re- orbital signs or visual symptoms of polygonal, pleomorphic nuclei and ported patients (older than 50 years) a mass in this patient, and the ra- prominent nucleoli were identified with LELC of the skin. diologic features suggested a be- (Figure 2B). These islands were sur- Lymphoepitheliomalike carci- nign process, the orbital biopsy was rounded by a very dense lympho- noma of the skin is microscopically undertaken to rule out metastasis. cytic infiltrate. distinct from other primary cutane- Performing the orbital biopsy was a The surgical specimen from the ous neoplasms. Although poorly crucial step, and it changed the stag- orbital biopsy performed at M. D. differentiated squamous cell carci- ing and treatment of this disease. Anderson Cancer Center consisted of noma could conceivably be associ- This case underscores the im- multiple fragments of pink, firm tis- ated with a prominent lymphoid portance of a thorough systemic sue with a combined measurement of infiltration, the latter is usually con- workup for patients with LELC of the 1ϫ0.6ϫ0.4 cm. The specimen was centrated in the superficial dermis skin. The orbital mass was identi- routinely processed, and additional and has connections with the epi- fied during an MRI scan of the head slides were analyzed by a standard dermis. Furthermore, squamous cell and neck, which was obtained to rule immunohistochemical method. His- carcinoma is usually associated with out involvement of the paranasal si- tologically, there was an infiltrate very at least focal evidence of cytoplas- nuses or the oropharynx. In gen- similar to that seen in the outside ma- mic keratinization. eral, because of the close histologi- terial. An immunohistochemical Cases of LELC of the skin asso- cal similarity to nasopharyngeal study showed expression of cyto- ciated with a metastatic or satellite le- lymphoepithelioma, patients with keratin (Figure 2C), which con- sion in the orbit are extremely rare. suspected LELC of the skin should firmed the epithelial nature of the ma- The only mention in the literature is have a thorough otolaryngological ex- lignant cells. This constellation of in a study of 1422 orbital tumors ana- amination, including indirect laryn- histologic features is indicative of lyzed at the eye pathology labora- goscopy, to rule out metastasis to the LELC. Standard in situ hybridiza- tory at the Shanghai Medical Univer- skin. The differential diagnosis also tion studies failed to reveal evidence sity, Shanghai, China.12 The authors includes Merkel cell tumor; lym- of infection by Epstein-Barr virus. list an LELC of the skin metastatic to phoma; pseudolymphoma; and meta- the orbit in one of the 1422 cases re- static lymphoepithelioma from the Comment. Lymphoepitheliomas are viewed in their article, but no fur- salivary gland, thymus, cervix, lung, malignant tumors of epithelial ori- ther description of the histologic find- vulva, stomach, or tonsil. These may gin with various amounts of reac- ings or the clinical findings is be excluded by history, physical ex- tive lymphocytic infiltrate. Al- provided. amination findings, and the results of though initially described in the In the case described here, the histological studies, including im- nasopharynx (World Health Orga- orbital mass was an incidental find- munohistochemical analysis. Lym- nization type 3 nasopharyngeal car- ing on an MRI scan after the diag- phoma and pseudolymphoma can be cinoma),3 these tumors have been nosis of LELC of the forehead skin excluded because of the expression identified in various locations with perineural invasion. It is likely of lymphoid markers and the lack of throughout the body.4-8 that the orbital mass represents a cytokeratin expression. Merkel cell Lymphoepitheliomalike carci- metastatic lesion secondary to the carcinoma is characterized by malig- noma of the skin is a rare cutane- forehead lesion, though the possi- nant epithelial cells with a lesser de- ous malignancy that is probably of bility of a direct extension into the gree of pleomorphism than is seen in adnexal origin.9,10 It is usually a flesh- orbit or a primary orbital tumor with LELC of the skin; also, the nuclei in colored or red, firm nodule or plaque subsequent cutaneous metastasis Merkel cell carcinoma characteristi- that appears most often on the face. cannot be completely ruled out. It cally have finely dispersed (“salt-and- Since the original description by is interesting that the radiologic fea- pepper”) chromatin.

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©2001 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/24/2021 We recommend an imaging 1. Dozier SE, Jones TR, Nelson-Adesokan P, Hruza magnetic resonance (MR) imaging GJ. Dermatol Surg. 1995;21:690-694. study of the head and neck in all pa- 2. Dortzbach RK. Ophthalmic Plastic Surgery: Pre- findings of central cavitation, simu- tients with lymphoepithelioma of the vention and Management of Complications. New lating an orbital lymphangioma. periocular skin not only to rule out York, NY: Raven Press; 1994:317-320. 3. Shanmurgaratnam K, Sobin LH. Histological Typ- nasopharyngeal carcinoma, but also ingofUpperRespiratoryTractTumors:International Report of a Case. An otherwise to rule out the possibility of orbital Histological Classification of Tumors. New York, healthy 4-year-old girl was referred NY: World Health Organization; 1978. No. 19. metastasis, as was the case in this 4. Holmang S, Borghede G, Johansson SL. Blad- to us because of abrupt-onset prop- patient. der carcinoma with lymphoepithelioma-like dif- tosis of her right eye (Figure 1). The association of LELC and ferentiation: report of 9 cases. J Urol. 1998;159: The parents had noted mild pain- 779-782. the Epstein-Barr virus varies in dif- 5. Kuo T, Hsueh C. Lymphoepithelioma-like sali- less swelling of the right upper eye- ferent organs and in different geo- vary gland carcinoma in Taiwan: a clinico- lid during the previous 3 weeks. graphical regions. The Epstein- pathological study of nine cases demonstrating a strong association with Epstein-Barr virus. The visual acuity was 20/20 in Barr virus is definitively associated Histopathology. 1997;31:75-82. each eye. There was 6 mm of right with LELC from the stomach, sali- 6. Frank DK, Cheron F, Cho H, DiCostanzo D, proptosis. Motility was restricted in 13 Sclafani AP. Nonnasopharyngeal lymphoepi- vary gland, lung, and thymus. The thelioma (undifferentiated carcinomas) of the all gazes, and the right eye was dis- association of Epstein-Barr virus upper aerodigestive tract. Ann Otol Rhinol Laryn- placed inferiorly and temporally. with LELC is restricted to Asian pa- gol. 1995;104:305-310. Subtle subcutaneous ecchymosis was 7. Dubey P, Ha CS, Ang KK, et al. Nonnasopha- tients with tumors of the salivary ryngeal lymphoepithelioma of the head and present at the inferotemporal or- gland and lung; whereas associa- neck. Cancer. 1998;82:1556-1662. bital rim. There was a dilated clear tion of Epstein-Barr virus with gas- 8. Sashiyama H, Nozawa A, Kimura M, et al. Case report: a case of lymphoepithelioma-like carci- lymphatic channel on the conjunc- tric and thymic LELC is indepen- noma of the esophagus and review of the litera- tiva superonasally. dent of race. As was the case in our ture. J Gastroenterol Hepatol. 1999;14:534-539. An MR image showed a supero- 9. Ko T, Muramatsu T, Shirai T. Lymphoepithe- patient, in the skin, there is no ap- lioma-like carcinoma of the skin. J Dermatol. nasal heterogeneous soft tissue mass parent association between LELC 1997;24:104-109. withnoboneerosion.OnT1-weighted and Epstein-Barr virus.14 10. Ortiz-Frutos FJ, Zarco C, Gil R, Ballestin C, gadolinium-enhanced images, the Iglesias L. Lymphoepithelioma-like carcinoma The treatment of choice for of the skin. Clin Exp Dermatol. 1993;18:83-86. central area of the mass was hypoin- LELC of the skin is complete surgi- 11. Swanson SA, Cooper PH, Mills SE, Wick MR. tense,suggestiveofproteinaceousma- cal excision whenever possible.15 Lymphoepithelioma-like carcinoma of the skin. Mod Pathol. 1988;1:359-365. terial. A peripheral hyperintense rim Radiotherapy is also an effective mo- 12. Ni C. Histopathologic classification of 1422 or- indicated vascularized tissue. There dality for the treatment of lympho- bital tumors. Chin J Ophthalmol. 1991;27:71-73. was a thin septum dividing the cen- 13. Iezzoni JC, Gaffey MJ, Weiss LM. The role of Ep- epitheliomas, particularly in pa- stein-Barr virus in lymphoepithelioma-like car- tral “cystic” area, but fluid-fluid lev- tients in whom complete surgical cinomas. Am J Clin Pathol. 1995;103:308-315. els were not seen (Figure 2A). T2- excision of the cancer is not pos- 14. Gillum PS, Morgan MB, Naylor MF, et al. Ab- weighted images disclosed an irregu- 16 sence of Epstein-Barr virus in lymphoepithe- sible. In the patient whose case we lioma-like carcinoma of the skin: polymerase lar isointense rim and central area of describe, the forehead lesion had re- chain reaction evidence and review of five cases. extreme hyperintense signal (Figure curred twice and was found to be as- Am J Dermatopathol. 1996;18:478-482. 15. Dozier SE, Jones TR, Nelson-Adesokan P, Hruza 2B). These findings were suggestive sociated with perineural invasion. GJ. Lymphoepithelioma-like carcinoma of the of lymphangioma with proteinaceous The orbital mass could not be com- skin treated by Mohs micrographic surgery. Der- fluid and hemorrhage. matol Surg. 1995;21:690-694. pletely excised without total sacri- 16. Ortiz-Frutos FJ, Zarco C, Gil R, Ballestin C, A diagnosis of lymphangioma fice of the intraorbital contents be- Iglesias L. Lymphoepithelioma-like carcinoma was favored in light of the rapid de- cause it extended posteriorly almost of the skin. Clin Exp Dermatol. 1993;18:83-86. velopment of proptosis, dilated con- to the superior orbital fissure. Ex- junctival lymphatics, subcutaneous ternal beam radiotherapy was the ecchymosis, and the presence of pre- best treatment option for this pa- sumed cystic, rather than solid, struc- tient with extensive skin, perineu- tures on MR imaging. Cautious ob- ral, and orbital lymphoepithe- Orbital Cavitary servation was advised, but 10 days lioma. Rhabdomyosarcoma later, worsening of the proptosis and Masquerading as eyelid swelling prompted a biopsy. M. Amir Ahmadi, MD Lymphangioma Intraoperatively, a multicystic blue Victor G. Prieto, MD, PhD mass was found and aspiration of 1 Gary L. Clayman, MD Rhabdomyosarcoma is the most com- mL of internal serosanguineous fluid Lawrence E. Ginsberg, MD mon primary orbital malignant neo- collapsed the mass. Near-total exci- Bita Esmaeli, MD plasm of childhood. However, it con- sion of the remaining mass was per- Houston, Tex stitutes only 4% of all orbital lesions formed. These findings continued to studied by biopsy in children.1 As it suggest the diagnosis of hemor- Corresponding author and reprints: is a highly malignant tumor, prompt rhagic lymphangioma. Bita Esmaeli, MD, Ophthalmology Sec- diagnosis and treatment are impera- Histopathologic examination tion, Department of Plastic Surgery, tive. Current therapeutic regimens of disclosed undifferentiated small University of Texas M. D. Anderson radiation and chemotherapy have round cells with hyperchromatic nu- Cancer Center, Box 443, 1515 Hol- provided 93% 3-year survival.2 We clei (Figure 3), high nuclear-to- combe Blvd, Houston, TX 77030 hereinreport a rare variant of orbital cytoplasmic ratio, and brisk mitotic (e-mail: [email protected]). rhabdomyosarcoma that showed activity. Immunohistochemistry

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©2001 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/24/2021 Figure 3. Photomicrograph showing poorly differentiated, malignant “small blue cell tumor” with occasional strap cells consistent with embryonal rhabdomyosarcoma (hematoxylin-eosin, original magnification ϫ250).

Figure 1. Ptosis, proptosis, and inferotemporal displacement of the right eye in a 4-year-old girl.

A B

Figure 2. A, Coronal magnetic resonance image (T1-weighted with fat suppression and gadolinium enhancement) showing a nonenhancing low-signal central area compatible with proteinaceous fluid or blood. The rim shows moderate enhancement. Note the septation within the central area. B, Coronal magnetic resonance image (T2-weighted) showing the central cavity with bright signal, again suggestive of proteinaceous fluid or blood.

demonstrated intense immunoreac- Rhabdomyosarcoma can usu- Our case presented a diagnos- tivity for smooth muscle actin and ally be differentiated from lymphan- tic dilemma. Rhabdomyosarcoma desmin. These findings confirmed the gioma by MR imaging. Rhabdomyo- and lymphangioma occur in similar diagnosis of rhabdomyosarcoma. sarcoma characteristically shows a age groups and are seen with simi- Radiotherapy (4500 rad [45 solid homogeneous mass that is iso- lar clinical findings. The malignant Gy]) and chemotherapy with cyclo- intense to vitreous on T1-weighted nature of rhabdomyosarcoma vs the phosphamide, dactinomycin, and images.3 Lymphangioma demon- benign course of lymphangioma un- vincristine sulfate were instituted by strates a multicystic mass with lob- derscores the importance of correct means of the Intergroup Rhabdo- ulated margins, showing hypoin- diagnosis. Although conservative myosarcoma Study Committee pro- tensity to vitreous on T1-weighted management of lymphangioma is tocol.2 The patient continued to images and hyperintensity to vitre- generally advised, proper follow-up demonstrate complete tumor regres- ous on T2-weighted images. The rim should be performed.6 If the sus- sion at 20 months’ follow-up. of lymphangioma can enhance mini- pected lymphangioma fails to dem- mally.4 Our case had MR imaging onstrate resolution or shows progres- Comment. Orbital rhabdomyosar- characteristics most consistent with sion, biopsy should be considered to coma typically occurs as a unilateral lymphangioma. rule out rhabdomyosarcoma. solid mass in the superonasal part of Cavitation can occur within the orbit. The average age at diagno- other ocular tumors secondary to sis is 8 years. Proptosis and displace- hemorrhage, necrosis, and mucoid Douglas R. Fetkenhour, BS ment of the globe usually develop degeneration.5 However, cavitation Carol L. Shields, MD during a period of weeks.3 Lymph- within rhabdomyosarcoma is dis- An N. Chao, MD angioma also occurs in a similar age tinctly unusual. To our knowledge, Jerry A. Shields, MD group, about 4 to 10 years of age. this finding has not been previ- Philadelphia, Pa Bleeding into lymphangioma can pro- ously reported. In a review of 250 or- Carl B. Guterman, MD duce sudden proptosis and ecchy- bital childhood tumors by Shields Hackensack, NJ mosis. Frequently, conjunctival in- and associates,1 there were no rhab- Ralph C. Eagle, Jr, MD volvement is found.4 domyosarcomas with cavitation. Philadelphia

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©2001 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/24/2021 This study was supported by the Paul listat, fluvoxamine maleate (a selec- also started to decrease the inflam- Kayser International Award of Merit tive serotonin reuptake inhibitor matory response within the supra- in Retina Research, Houston, Tex (Dr [SSRI], which she started 2 days choroidal space. After consultation J. Shields); the Eye Tumor Research prior to initial examination), and with her psychiatrists, she stopped Foundation, Philadelphia, Pa (Dr topiramate (which she started 2 receiving fluvoxamine maleate and C. Shields); Noel T. and Sara L. Sim- weeks prior to initial examination). the topiramate was tapered over 2 mons Endowment for Ophthalmic On examination her visual acu- weeks. She was seen every 2 to 3 Pathology, Wills Eye Hospital, Phila- ity was 20/250 OU. Slitlamp exami- days. She reported alleviation of her delphia (Dr Eagle); and the Depart- nation revealed trace conjunctival in- symptoms by the sixth day after ini- ment of Ophthalmology, Chang Gung jection and chemosis, relatively clear tial examination. By then, her my- Memorial Hospital, Taipei, Taiwan corneas, and shallow anterior cham- opic shift had resolved and her (Dr Chao). bers (approximately 2 corneal thick- chambers were deep. On gonios- ness deep centrally). The pupils were copy, she was open to ciliary body 1. Shields JA, Bakewell B, Augsburger JJ, Donoso LA, Bernardino V. Space-occupying orbital widely dilated, and the lenses were band without synechiae in both eyes. masses in children: a review of 250 consecutive clear. Intraocular pressures mea- Her intraocular pressures were 11 to biopsies. Ophthalmology. 1986;93:379-384. 2. Wharam M, Beltangady M, Hays D, et al. Local- sured 51 mm Hg OD and 45 mm Hg 14 mm Hg OU. She finished her ized orbital rhabdomyosarcoma: an interim re- OS. Funduscopic examination find- prednisone taper and stopped report of the Intergroup Rhabdomyosarcoma Study ings were normal with a cup-disc ceiving the antiglaucoma medica- Committee. Ophthalmology. 1987;94:251-254. 3. Shields JA, Shields CL. Myogenic tumors. In: ratio of 0.3 OU. No choroidal effu- tions within 1 week. Subsequently, Shields JA, Shields CL, eds. Atlas of Orbital Tu- sions were seen by indirect ophthal- the scopolamine was stopped, and mors. Philadelphia, Pa: Lippincott Williams & moscopy. On gonioscopy there was her pupils returned to normal size. Wilkins; 1999:106-111. 4. Shields JA, Shields CL. Vascular and hemor- a steep iris convexity with apposi- Her vision returned to 20/25 OU rhagic lesions. In: Shields JA, Shields CL, eds. tional angle closure. With compres- without correction. Repeated B scans Atlas of Orbital Tumors. Philadelphia, Pa: Lip- pincott Williams & Wilkins; 1999:60-63. sion, trabecular meshwork was seen and ultrasound biomicroscopy were 5. Lois N, Shields CL, Shields JA, Eagle RC, De- in both eyes without peripheral an- performed 3 weeks after initial ex- Potter P. Cavitary melanoma of the ciliary body: terior synechiae. amination, which revealed a reso- a study of eight cases. Ophthalmology. 1998;105: 1091-1098. The diagnosis of bilateral angle- lution of the effusion (Figure, C and 6. Muallem MS, Garzozi HJ. Conservative man- closure glaucoma was made. The pa- E). Her axial lengths were 23.54 mm agement of orbital lymphangioma. J Pediatr Oph- tient was treated with 0.5% timolol OD and 23.74 mm OS. thalmol Strabismus. 2000;37:41-43. maleate, dorzolamide hydrochlo- Case 2. A 53-year-old white ride, brimonidine tartrate, oral ac- woman with blurry vision in both etazolamide (500 mg), and latano- eyes on awakening was seen at an- prost. Her pressures eventually other clinic. Her medical history was Uveal Effusion and decreased to 28 mm Hg OD and 27 notable for depression and high cho- Secondary Angle-Closure mm Hg OS. 1% Pilocarpine was lesterol levels. Her medications in- Glaucoma Associated added to alleviate the pupillary my- cluded premarin, venlafaxine hy- With Topiramate Use driasis. drochloride (an SSRI), atorvastatin She was seen the following day calcium, and topiramate (which she Rarely, drugs, mostly sulfa-related with examination findings rela- started 10 days prior to the onset of compounds, have produced uveal ef- tively unchanged, except that her pu- her symptoms). fusions, forward rotation of the iris- pils were now mid-dilated and ten- Her vision was recorded as lens diaphragm, transient myopia, sions were 29 mm Hg OD and 32 counting fingers OD and 20/160 OS. and secondary angle closure.1 We mm Hg OS. The anterior chambers Slitlamp examination revealed che- have recently encountered 2 cases in were still shallow in both eyes. A mosis, diffuse corneal edema, and which uveal effusions have oc- manifest refraction revealed the for- diffusely shallow anterior cham- curred after administration of topi- merly emmetropic patient now had bers. Her intraocular pressures were ramate (Topamax; Ortho-McNeil measurements of −8.75 sphere OD recorded as 72 mm Hg OD and 74 Pharmaceutical, Raritan, NJ), a new and −7.25 sphere OS. A B scan was mm Hg OS. Funduscopic examina- anticonvulsant medication. performed and demonstrated a sepa- tion findings were reported as nor- ration between the choroidal layer mal with normal-appearing optic Report of Cases. Case 1. A 34-year- and the sclera 360° with the crys- nerves. A diagnosis of bilateral angle old white woman was seen in our talline lens shifted anteriorly closure was made. emergency department with severe (Figure, D). Ultrasound biomicros- Peripheral iridotomies were headaches and progressively blurry copy was also performed and dem- performed that same day in both vision in both eyes. Her medical his- onstrated a closed angle with a for- eyes, and medications were admin- tory was notable for depression. Her ward shift of the ciliary body (Figure, istered without reduction of her in- ocular history was unremarkable, A and B). traocular pressures. One hour post- she had never worn glasses, and she A diagnosis of bilateral uveal ef- laser, paracenteses were made to denied ocular disease within the fusion was made. Pilocarpine hy- both eyes to relieve the pressure and family. Her medications included drochloride was discontinued and then repeated several hours later clonazepam, buspirone hydrochlo- scopolamine hydrochloride was ad- with reduction of her intraocular ride, citalopram hydrobromide, or- ministered. An oral steroid taper was pressure to 45 mm Hg OD and 48

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S AC

C D E

AC S

Case 1. A, Ultrasound biomicroscopy (UBM) demonstrating uveal effusion (asterisk) at the time of initial presentation. B, UBM obtained at initial examination showing a shallow anterior chamber. C, UBM after treatment for the uveal effusion demonstrating deep anterior chamber and resolution of the uveal effusion. D, B scan at the time of initial examination with arrow pointing to the uveal effusion. E, Repeated B scan after treatment without the uveal effusion. S indicates sclera; CB, ciliary body; and AC, anterior chamber.

mm Hg OS. The patient continued als with microphthalmic eyes or with To our knowledge, these are to receive topical and oral antiglau- abnormal sclera.2 Drug-induced the first reported cases of choroidal coma medications. She discontin- uveal effusions have been cited, al- effusions associated with topira- ued only the topiramate since this though they also occur rarely. Both mate. It is therefore our suggestion was the only recent change to her of our patients received topiramate that if a patient is seen with bilat- medications. She was seen the next as adjunctive therapy for depres- eral angle-closure glaucoma, a his- day. Her anterior chambers were sion approximately 2 weeks prior to tory of topiramate usage should be deeper centrally but still shallow presentation. We feel that topira- sought. peripherally. Her intraocular pres- mate has some relation to the cause sure was 25 mm Hg OU. Gonios- of the patients’ bilateral uveal effu- Prithvi S. Sankar, MD copy revealed no angle structures, sions, though it is unclear whether Louis Robert Pasquale, MD and funduscopic examination did it is topiramate alone or in conjunc- Cynthia L. Grosskreutz, MD, PhD not show clinically evident choroi- tion with an SSRI. Boston, Mass dal effusions. Tropicamide was ad- Topiramate is a sulfamate- ministered. By the next day, her in- substituted monosaccharide, used We thank Herbert Knauf, MD, for his traocular pressure was 12 mm Hg primarily as an antiepileptic medi- clinical information and Danny OU, and her anterior chambers cation. Topiramate is thought to Gauthier, MD, and Lois Hart, RD, MS, were deep. Repeated gonioscopy possess a state-dependent sodium for their technical assistance. revealed angles open to scleral channel–blocking action. It also Corresponding author: Cynthia spur. potentiates the activity of GABA L. Grosskreutz, MD, PhD, Depart- She was seen at our service 1 (␥-aminobutyric acid) and antago- ment of Ophthalmology, Glaucoma month after her initial presenta- nizes the ability of kainate to Consultation Service, Massachusetts tion. Her vision was 20/25 OU un- activate the kainate/AMPA (␣- Eye and Ear Infirmary, 243 Charles corrected. Slitlamp examination re- amino-3-hydroxy-5-methylisoxa- St, Boston, MA 02114-3096. vealed clear corneas with deep, quiet zole-4-propionic acid) subtype of anterior chambers in both eyes. In- excitatory amino acid receptor. 1. Grant WM, Schuman JS. Introductory Outline of traocular pressure was 14 mm Hg Topiramate also has a weak car- Toxic Effects on the Eye and Vision: Toxicology of OU, and gonioscopy revealed grade bonic anhydrase inhibition.3 These the Eye. 4th ed. Springfield, Ill: Charles C Tho- mas; 1993:22-24. III open angles without synechiae in mechanisms of action help explain 2. Uyama M, Takahashi K, Kozaki J, et al. Uveal both eyes. Funduscopic examina- the antiepileptic nature of the effusion syndrome. Ophthalmology. 2000;107: 441-449. tion revealed a cup-disc ratio of 0.6 drug, though the mechanism of 3. Arky R. Product information [topiramate]. In: with temporal pallor. choroidal effusions remains un- Physician Desk Reference. Montvale, NJ: Medi- clear. Topiramate does cross the cal Economics Co; 1999:2249-2252. 4. Mozayani A, Carter J, Nix R. Distribution of topi- Comment. Spontaneous uveal effu- blood-brain barrier and has also ramate in a medical examiner’s case. J Anal Toxi- sions are most common in individu- been detected in the vitreous.4 col. 1999;23:556-558.

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©2001 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/24/2021 Ghost Cell Glaucoma Related to Snake Poisoning The presence of blood or blood de- bris in the anterior chamber can increase intraocular pressure (IOP). Vitreous hemorrhage can also lead to secondary glaucoma, producing a “ghost cell glaucoma” (GCG).1,2 Ghost cells (GCs) are degenerated spherical erythrocytes that partially lose their hemoglobin content by ag- ing for a long period in the vitreous. Changes begin after a few days and are usually completed within 3 weeks. Hemoglobin abandons the red blood cell and forms clumps that ad- here to vitreous bands. Hemoglobin Figure 1. Left inferior subconjunctival hemorrhage as part of hemorrhagic syndrome. that remains in the red blood cell be- comes denatured and binds to the internal surface of the cell membrane, forming granules (Heinz bodies).3 Once formed, GCs may remain intact for months, moving freely within the vitreous. Neither fresh erythrocytes nor GCs are able to pass through an intact anterior hyaloid membrane; thus, a hyaloid injury must be present for these cells to be found in the anterior segment.2,3 Since GCs are rigid, they have difficulty passing through the trabecular meshwork. They tend to accumulate in its middle and external portions, whereas fresh erythrocytes pass 3 times more eas- ily to the external portion and from there to the Schlemm canal.1,3 Increased IOP usually occurs about 2 to 4 weeks after the injury, Figure 2. Left slitlamp photograph showing corneal edema and elevated number of ghost cells but it may also take from 1 week to in anterior chamber. many months to develop.2,3 It is a complication that often requires surgical intervention with profuse and pital and treated for respiratory dis- lated indirect ophthalmoscopy repeated lavage of the anterior cham- tress syndrome and hemorrhagic showed a dense vitreous hemor- ber or vitrectomy to remove the syndrome with renal and cerebral in- rhage in both eyes. Results of B- hemorrhagic tissue.2-4 volvement. Two days later, after re- scan ultrasonography confirmed bi- We describe a patient who de- covery from respiratory distress and lateral vitreous hemorrhage with veloped vitreous hemorrhage and renal failure, he complained of bi- incomplete posterior vitreous de- GCG after a snake bite. There was lateral visual loss. tachment. no evidence of anatomic alteration On examination, his visual acu- With an initial diagnosis of GCG of the anterior hyaloids. ity was hand motions in the right eye in the left eye, a paracentesis and and light perception in the left eye. aqueous sampling were performed in Report of a Case. A 44-year-old male The left eye showed inferior and the left eye. Cytologic examination of farmer was seen in the emergency temporal subconjunctival hemor- the aqueous humor disclosed the department of our institution (Hos- rhage (Figure 1), stromal and epi- presence of GCs. Meanwhile, the IOP pital San Juan de Dios, National Uni- thelial corneal edema, ++++ cells and in the right eye rose to 28 mm Hg, versity of Colombia, Bogota´) 72 flare in the anterior chamber, and and treatment was begun with 0.4% hours after sustaining a snake bite dense anterior vitreous hemor- apraclonidine hydrochloride twice (Bothrops athrox) in his right foot. rhage (Figure 2). The IOP was 17 daily, 0.4% timolol maleate twice The patient was admitted to the hos- mm Hg OD and 40 mm Hg OS. Di- daily, and a prostaglandin deriva-

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©2001 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/24/2021 Corresponding author: Ledy Rojas, MD, Departamento de Oftalmologı´a, Hospital San Juan de Dios, Carrera 10 Calle 1, Bogota´, Colombia (e-mail: [email protected]).

1. Campbell DG, Simmons RJ, Grant WM. Ghost cells as a cause of glaucoma. Am J Ophthalmol. 1976;81:441-440. 2. Montenegro MH, Simmons RJ. Ghost cell glaucoma. Int Ophthalmol. 1994;34:111-114. 3. Campbell D, Schertzer RM. Ghost cell glaucoma. In: Ritch R, Shields MB, Krupin T, eds. The Glaucomas. St Louis, Mo: CV Mosby Co; 1996:1277-1284. 4. Campbell DG, Essigmann EM. Hemolytic ghost cell glaucoma: further studies. Arch Ophthal- mol. 1979;97:2141-2146. 5. Mansour AM, Chess J, Starita R. Nontraumatic ghost cell glaucoma: a case report. Ophthalmic Surg. 1986;17:34-36. 6. Rodriguez FJ, Foos RY, Lewis H. Age-related macular degeneration and ghost cell glaucoma. Figure 3. Spherical erythrocytes with vacuoles and partial loss of hemoglobin (ghost cells) in vitreous Arch Ophthalmol. 1991;109:1305-1305. (Papanicolaou, original magnification ϫ100). 7. Russell FE, Dart RC. Toxic effects of animal tox- ins. In: Amdur MO, Doul J, Klaassen CD, eds. Casarett and Doull’s Toxicology: The Basic Sci- ence of Poison. 4th ed. New York, NY: Per- tive 3 times daily in both eyes. A di- and thrombinoid enzymes with fi- gamon Press; 1991:743-803. agnostic paracentesis in the right eye brinolytic action have also been de- 8. Bolan˜osR.Serpientes, Venenos y Ofidismo en Cen- confirmed the presence of GCs. tected. Viper’s venom alters vascu- troame´rica. San Jose, Costa Rica: Editorial Uni- versidad de Costa Rica; 1984:48-49. Results of B-scan ultrasonog- lar resistance and, often, vascular 9. Rosenthal DL, Mandell DB, Glasgow BJ. Eye. In: raphy performed 2 weeks later integrity. It produces changes in Bibbo M, ed. Comprehensive Cytopathology. 2nd showed little or no resolution of the blood cells and coagulation mecha- ed. Philadelphia, Pa: WB Saunders Co; 1997: 493-509. vitreous hemorrhage. Both eyes were nisms as well as alterations in cen- treated with standard 3-port poste- tral nervous system, cardiovascular, rior vitrectomy. Cytologic examina- and pulmonary dynamics.7 Hyal- tion of the vitreous showed the pres- uronidase is also present in all Ameri- Recurrent Transient ence of GCs (Figure 3). Bilateral can viperous poisons studied to date.8 Visual Loss After indirect ophthalmoscopy showed Hyaluronidase, collagenase, and other Deep Sclerectomy optic nerve pallor with a cup-disc ra- proteolytic enzymes present in the tio of 0.2 and attenuated retinal vas- Bothrops venom may decrease the Recurrent transient visual loss in the cular tree and fovea reflex loss. The vitreous viscosity and alter the ante- elderly is mostly associated with car- IOP normalized in the right eye and rior hyaloid permeability. This physi- diovascular disorders. Other causes visual acuity improved to 20/70. The ologic disruption may allow migra- include giant cell arteritis, mi- IOP normalized in the left eye, but tion of the GCs in the aqueous, graine, increased intracranial pres- the visual acuity was counting fin- causing the secondary glaucoma sure, orbital mass, and idiopathy. We gers at 40 cm because of a rheg- affecting both eyes. describe a patient with unusual re- matogenous retinal detachment with Ghost cells were identified by current transient visual loss after macular involvement. cytologic examination of vitreous deep sclerectomy with collagen im- and aqueous humor, centrifuged and plant (DSCI). Comment. Bilateral GCG second- stained with Papanicolaou stain9 ary to snake poisoning has not yet (Figure 3). Sometimes, the vitre- Report of a Case. A 75-year-old been described in the literature, ous has “hemolytic cells” that are ac- woman was referred for investiga- based on our MEDLINE search of tually macrophages with hemosid- tion of possible amaurosis fugax. She the medical literature since the ini- erin and erythrocyte fragments. complained of recurrent painless tial description of GCG in 1976 to Ophthalmologists should be blurred vision in her left eye for the the present. aware that snake bite can cause vi- past 6 months. Her medical history Ghost cell glaucoma has been sual loss. Early diagnosis and prompt was relevant for common migraine associated with diabetic vitreous treatment to reduce the number of and systemic hypertension. Sys- hemorrhage in a phakic eye without blood cells and GCs may increase the temic medications included losar- previous trauma or surgery as well as potential for recovery. tan potassium, hydrochlorothia- in other rare cases.5,6 Snake venoms, zide, lorazepam, carvedilol, and especially those from crotalids, as in Ledy Rojas, MD aspirin. Severe bilateral glaucoma ne- the case of Bothrops, contain proteo- Gabriel Ortiz, MD cessitated trabeculectomy in the right lytic enzymes capable of breaking tis- Myrian Gutie´rrez, MD eye in 1994 and DSCI in the left eye sue proteins, thereby acting as hem- Sonia Corredor, MD in 1996. Both procedures were un- orrhagic factors. Thrombinogenic Bogota´, Colombia eventful.

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©2001 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/24/2021 vealed active bleeding through a microperforation in the trabeculo- Descemet membrane at the site of surgery (Figure 2). Because of repeated bleeding episodes and increased IOP in the left eye, the site of DSCI underwent reoperation. Many actively bleeding blood vessels surrounding the Schlemm canal orifice were found and coagulated. No further bleeding has occurred since, and the IOP remained less than 15 mm Hg without therapy.

Comment. Transient visual loss secondary to recurrent hyphema has been reported after trabeculec- tomy1 and after cataract surgery with either an iris suture2 or an anterior 3 Figure 1. Hyphema (2 mm) in the left eye. chamber implant but not after nonpenetrating filtering surgery. Now- adays, DSCI is becoming a common technique to safely lower IOP and is believed to be less traumatic than trabeculectomy because of the nonpenetrating technique.4 More than 1500 patients have undergone DSCI in our department (unpub- lished data); however, recurrent hyphema occurred only in the present case. During surgery, no obvi- ous perforation was noted, but we cannot rule out the possibility of a microperforation of the trabeculo- Descemet membrane. Recurrent hyphema after DSCI could result from spontaneous bleeding of anomalous scleral blood vessels at the site of surgery (such as in the present case), venous hy- Figure 2. Gonioscopy revealed actively bleeding blood vessels at the site of the trabeculo-Descemet pertension (Valsalva phenom- membrane. enon) with blood reflux in the Schlemm canal, or a combination of The episodes of visual loss in disc ratio, 0.9) and moderate in the both. Aspirin therapy might have the left eye occurred on average once left eye (cup-disc ratio, 0.5). Visual also contributed to the bleeding. a week, lasted up to 24 hours, and field defect was severe in the right History of previous filtering affected her ability to read. Some epi- eye and moderate in the left. surgery in the setting of transient sodes occurred after performing Results of investigations, includ- visual loss should then prompt go- gymnastic exercises, after bending ing a complete blood cell count, eryth- nioscopy and a careful anterior forward, and once after sneezing. She rocyte sedimentation rate, cardio- chamber examination for the pres- had no symptoms or signs suggest- vascular examination, precerebral ence of hyphema or blood reflux ing giant cell arteritis. Doppler and cerebral magnetic reso- through the trabeculo-Descemet Best-corrected visual acuity was nance imaging, and angiography, membrane. 20/40 OD and 20/25 OS. Pupil ex- were normal. amination revealed a 2+ right rela- Several similar episodes have Aude Ambresin, MD tive afferent defect. Intraocular pres- occurred since she was examined. A Franc¸ois-Xavier Borruat, MD sure (IOP) was 11 mm Hg in the few hours after onset of the latest epi- Andre´ Mermoud, MD right eye and 14 mm Hg in the left. sode, examination of the left eye Lausanne, Switzerland Fundus examination revealed bilat- showed visual acuity of 20/50−2,2 eral glaucomatous disc atrophy that mm of hyphema (Figure 1), and Corresponding author: Franc¸ois- was severe in the right eye (cup- IOP of 38 mm Hg; gonioscopy re- Xavier Borruat, MD, Hoˆpital Oph-

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©2001 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/24/2021 talmique Jules Gonin, Avenue de France was maintained on timolol and la- bility to the optic nerve from the 15, CH-1004 Lausanne, Switzerland tanoprost after the third day. Visual marked rise and subsequent stabi- (e-mail: [email protected]). acuity was noted to be 20/40 after 11 lization of intraocular pressure. Al- days. Ophthalmoscopy showed a though permanent vision loss, which 1. Glaser JS. In: Glaser JS, ed. Neuro-ophthal- healthy left optic nerve with a 0.1 occurs at the time of angle-closure mology. 2nd ed. Philadelphia, Pa: JB Lippin- cup-disc ratio. At this time, the pa- glaucoma, is thought to be caused cott; 1990:92. tient was advised, but refused, to have by an ischemic event to the optic 2. Kosmorsky GS, Rosenfeld SI, Burde RM. Tran- sient monocular obscuration—amaurosis fu- prophylactic iridotomy in the fel- nerve, NAION following such a bout gax: a case report. Br J Ophthalmol. 1985;69:688- low eye. One week later, vision loss has not (to our knowledge) previ- 690. 3. Anton A, Weinreb N. Recurrent hyphema sec- in the left eye recurred. There was no ously been reported. The explana- ondary to anterior chamber lens implant. Surv associated eye pain, headache, jaw tion for the duration between the Ophthalmol. 1997;41:414-416. claudication, or polymyalgia rheu- angle-closure episode and the on- 4. Mermoud A, Schnyder CC. Nonpenetrating filtering surgery. Curr Opin Ophthalmol. 2000;11: matica. On examination, visual acu- set of NAION is unknown. We pos- 151-157. ity was hand movements, and a re- tulate that in our case, subclinical maining inferotemporal island of low-grade optic nerve ischemia oc- vision was detected on confronta- curred at the time of the pressure rise tion visual field testing. Slitlamp ex- with subsequent progressive ische- amination results were unremark- mia until frank vision loss ensued. Anterior Ischemic Optic able, and intraocular pressure was Perhaps a vicious cycle consisting of Neuropathy Following normal. A left afferent pupil defect ischemia, optic disc swelling, and ad- Acute Angle-Closure was present. The left optic nerve was ditional ischemia occurred. Hay- Glaucoma swollen with overlying hemor- reh3 has described several cases of rhages. Westergren erythrocyte sedi- subclinical optic disc edema result- Nonarteritic anterior ischemic optic mentation rate was normal. Left ing in frank bouts of symptomatic neuropathy (NAION) is believed to NAION was diagnosed. NAION months later. Perhaps our be caused by acute occlusion of small One month after the bout of case and those of Hayreh shed some vessels to the optic nerve, resulting angle-closure glaucoma in the left light on the possible mechanism as in lacunar infarction. Most in- eye, the patient experienced sud- to why some cases of NAION fol- volved optic nerves are anatomi- den vision loss in the right eye due lowing cataract extraction have a de- cally crowded with a cup-disc ratio to angle-closure glaucoma. The in- monstrable interval between sur- that is small and usually less than traocular tension was 56 mm Hg. gery and vision loss.4 In any event, 30%.1 Nonarteritic anterior ische- Medications (the same used as in fel- acute rise in intraocular pressure is mic optic neuropathy may also follow eye) and laser iridotomy again to be considered a risk factor in low other episodes of hemody- were successfully employed, and NAION. Whenever possible, pre- namic instability, such as cerebral 10 days later, visual acuity recov- cautions should be taken to avoid hypoperfusion (shock optic neu- ered to 20/50. One week later, how- pressure rise, especially in eyes with ropathy) or blood loss. It has been ever, there was marked worsening optic discs that are developmen- described after uncomplicated cata- of vision in the right eye. Neuro- tally small, with small cup-disk ract extraction, in which it is pre- ophthalmologic consultation was ratios. sumed to be due to perioperative el- sought, and examination revealed a evation of intraocular pressure.2 We best-corrected visual acuity of 20/ Michael L. Slavin, MD report a case of NAION developing 200 OD and 2/200 OS. Each pupil Michael Margulis, MD in each eye of a man with sequen- was dilated and unresponsive to Great Neck, NY tial acute angle-closure glaucoma. In light. Glaucomflecken was noted on each eye, the vision loss followed the the left lens. Goldmann visual fields Corresponding author and reprints: bout of glaucoma by approximately showed bilateral central scotomas Michael L. Slavin, MD, Division of 21⁄2 weeks. with peripheral inferior extension. Neuro-ophthalmology, Department of Ophthalmoscopy revealed pale Ophthalmology, Long Island Jewish Report of a Case. A 70-year-old man swelling of the right optic disc and Medical Center, the Albert Einstein with angina pectoris and hypercho- resolving optic disc edema in the left College of Medicine, 600 Northern lesterolemia developed acute pain eye, with marked optic atrophy. Reti- Blvd, Great Neck, NY 11021 (e-mail: and markedly diminished vision in nal arterioles were attenuated bilat- [email protected]). his left eye due to angle-closure glau- erally. Sequential NAION was diag- coma. Visual acuity was noted to be nosed. On subsequent follow-up 1. Beck RW, Savino PJ, Repka MX, et al. Optic disc 5/200, corneal edema was present, there was no improvement of vi- structure in anterior ischemic optic neuropathy. Ophthalmology. 1984;91:1334-1337. and intraocular pressure was 50 mm sual acuity or visual fields in either 2. Hayreh SS. Anterior ischemic optic neuropa- Hg. Medications ( timolol maleate, eye, and bilateral optic atrophy was thy, IV: occurrence after cataract extraction. Arch Ophthalmol. 1980;98:1410-1416. brimonidine tartrate, latanoprost, 1% noted. 3. Hayreh SS. Anterior ischemic optic neuropa- pilocarpine hydrochloride, predniso- thy, V: optic disc edema as early sign. Arch Oph- lone, and oral acetazolamide), and Comment. In our case, vision loss thalmol. 1981;99:1030-1040. 4. Carroll FD. Optic nerve complications of cata- subsequent laser iridotomy were suc- due to NAION was most likely ract extraction. Trans Am Acad Ophthalmol Oto- cessful in reversing the process. He precipitated by hemodynamic insta- laryngol. 1973;77:623-629.

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©2001 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/24/2021 sia. The best-corrected visual acu- from biomicroscopy and scanning Contractile Peripapillary ity was 20/25 OD and 20/30 OS. The laser ophthalmoscopy (Figure 1 pupillary reflexes were normal. The and Figure 2). Staphyloma With Light left eye had a peripapillary staphy- Stimulus to the loma showing circular-type contrac- Comment. The condition described Contralateral Eye tile movements characterized by a herein must be distinguished from short time delay, following the light coloboma of the optic disc, in which A peripapillary staphyloma is a spo- stimulus to the contralateral eye. The the defect is within the nerve head, radic, unilateral, congenital defect correlations were negative with the or myopic conus, in which the defect characterized by an excavation sur- Valsalva maneuver, neck venous is usually secondary to an abnormal 2 rounding a usually normal optic disc compression, forced lid closure, in- disc. In this case report, an error dur- and often accompanied by decrease of ocular pressure with con- ingembryologicaldevelopmentseems creased vision1,2 or by enlargement tact lens, respiratory movements, ac- likely. An area that should have be- of the blind spot.3 A less common oc- commodation, and illumination of come sclera may have become a cir- 4 currence is when the wall of the peri- the affected eye. cular muscle instead, using concen- papillary staphyloma is contractile, The visual field showed en- trically oriented smooth strands and which is caused by 1 of 2 possible largement of the blind spot. Fluo- forming an incomplete ring around 2,5 mechanisms, “pressure balance” or rescein angiography revealed a the nerve. “muscular contraction.”2 This ar- window defect in the peripapillary We favor a neuromuscular con- ticle provides the first documenta- region. Optical coherence tomogra- traction mechanism as the basis for 4 tion of a contractile peripapillary phy, B-scan ultrasonography, com- the observed phenomenon. A cir- staphyloma through sequential pic- puted tomography, and nuclear cular, heterotopic smooth muscle tures and discusses the main differ- magnetic resonance in combina- situated at the posterior pole of the ential diagnosis and the possible tion revealed a parietal ectasia. The eye, associated with an autonomic pathophysiological mechanism. orbital Doppler was normal. No ret- cholinergic reflex, and innervated by robulbar tumors, inflammation, ab- a ciliary nerve is, in our estimation, Report of a Case. A 23-year-old normal vessels, or other congenital the most likely cause for these in- white woman was referred for evalu- anomalies were identified. Changes traocular motions.2 The contrac- ation after undergoing a vitrec- in size and shape affecting the disc tion was noticeably changed by ret- tomy on the left eye for removal of and the peripapillary zone were robulbar anesthesia. The relevant a peripapillary cisticercus, which dis- documented by serial photographs mechanism may be rudimentary and appeared after retrobulbar anesthe- and video documentation obtained may explain the nonsynchronous re-

A B A B

C D C D

Figure 1. Serial fundus photographs of the peripapillary staphyloma, Figure 2. Serial ultrasonographic pictures, revealing changes of ectasia showing its contractile movements and shape and size modifications in deepness at the nasal aspect of the peripapillary staphyloma. A, Initial response to light stimulation to the contralateral eye. A, Normal appearance appearance after the light stimulus in the contralateral eye. B-D, Progressive of the ectasia with an indefinite nasal margin of the disc. B, Initial contraction contraction following the provocative test. of the anomaly, allowing a partial identification of the nasal margin of the disc. C, Progressive contraction revealing a normal shape of the disc. D, Final appearance of the region after a circular contraction pattern.

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©2001 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/24/2021 sponse with a latency period after the tor” in the Journal of the American and 6 were measured within 12 hours pupillary reflex and the negative cor- Medical Association, the issue has of fixation. No statistical difference relation to direct illumination. been raised as to whether this is a was detected between these 2 groups. prefixation or postfixation measure- Michel E. Farah, MD ment guideline and whether forma- Comment. Our study shows that Fausto Uno, MD lin fixation reduces the effective shrinkage of temporal artery bi- Pedro P. Bonomo, MD length of a temporal artery biopsy opsy specimens does occur follow- Mario No´brega, MD specimen.4 This is a potentially im- ing formalin fixation. This observa- Ana L. Ho¨fling-Lima portant question since the accu- tion suggests that clinicians may Sa˜o Paulo, Brazil racy of the histological diagnosis of need to take slightly longer tem- GCA is believed to be strongly cor- poral artery biopsy specimens than We thank Tercio Guia for the serial related to the length of the fixed ar- previously recommended to insure pictures and video documentation. tery specimen that is available for that shrinkage due to chemical fixa- Corresponding author: Michel E. pathological assessment. We re- tives does not reduce diagnostic Farah, MD, Avenida Ibijau´, 331, 4° port the first available data that di- accuracy in the investigation of andar, CEP 04524-020, Sa˜o Paulo– rectly address this question. this treatable but potentially sight- SP, Brazil (e-mail: michelfarah@ threatening condition. While a uol.com.br). Report of a Case. All patients un- 20-mm sample of temporal artery is dergoing temporal artery biopsy at generally considered to be an ad- 1. Caldwell J, Sears M, Gilman M. Bilateral peri- equate biopsy specimen, because of papillary staphyloma with normal vision. Am J Wills Eye Hospital (Philadelphia, Pa) Ophthalmol. 1971;71:423-425. between January 15 and July 1, 1999, the presence of skip lesions, the 2. Kral K, Svarc D. Contractile peripapillary were prospectively enrolled. Imme- shorter the biopsy specimen, the staphyloma. Am J Ophthalmol. 1971;71:1090- more important the shrinkage fac- 1092. diately upon excision of the tempo- 3. Konstas P, Katikos G, Vatakas L. Contractile peri- tor is to diagnosis. papillary staphyloma. Ophthalmologica. 1976; ral artery segment and prior to the placement of the specimen in 10% 172:379-381. Helen V. Danesh-Meyer, FRACO 4. Willis R, Zimmerman L, O’Grady R, et al. Het- neutral buffered formalin fixative, the erotopic adipose tissue and smooth muscle in Auckland, New Zealand length of the specimen was mea- the optic disc: associations with isolated colo- Philadelphia, Pa bomas. Arch Ophthalmol. 1972;88:139-146. sured in the operating room by the 5. Wise J, Maclean A, Gass D. Contractile peripap- surgeon. It was later remeasured af- Peter J. Savino, MD illary staphyloma. Arch Ophthalmol. 1966;75: Jurij R. Bilyk, MD 626-630. ter fixation before sectioning by the pathologist, who was masked as to Ralph C. Eagle, MD the initial measurement. Both sets of Robert C. Sergott, MD Philadelphia, Pa Shrinkage: Fact or Fiction? measurements were done using the same standardized millimeter rulers with measurements being made to The diagnosis of giant cell arteritis Corresponding author: Helen V. the nearest tenth of a millimeter. (GCA) is confirmed by finding char- Danesh-Meyer, MD, Discipline of acteristic histological changes in the Twenty-eight temporal artery Ophthalmology, University of Auck- disease in an arterial segment, such biopsies were performed. Fifteen land, Private Bag 92019, Auckland, as the superficial temporal artery. were positive and 13 were negative New Zealand (e-mail: h.daneshmeyer The length of the arterial specimen for shrinkage. The average prefix- @auckland.ac.nz). is crucial because of the presence of ation length was 28.4 mm (SD, 6.0 “skip lesions”1 (areas of no inflam- mm), whereas the average postfix- 1. Klein RG, Cambell RJ, Hunder GG, Carney JA. mation) in GCA. Recently, there has ation length was 26.0 mm (SD, 5.5 Skip lesions in temporal arteritis. Mayo Clin Proc. 1976;51:504-510. been discussion concerning the re- mm), representing a mean shrink- 2. Boyev LR, Miller NR, Green WR. Efficacy of uni- quired length of an arterial speci- age of 2.4 mm (95% confidence in- lateral versus bilateral temporal artery biopsies Ͻ for the diagnosis of giant cell arteritis. Am J Oph- men to securely confirm or ex- terval, 1.6-3.1 mm; P .001, 2-tailed thalmol. 1999;128:211-215. 2,3 clude the diagnosis of GCA. It is test) or a mean reduction of about 8% 3. Gordon LK, Levin LA. Visual loss in giant cell generally accepted that 20 mm is an (6%-13%). Twenty-two biopsy speci- arteritis. JAMA. 1998;280:385-386. 4. Caroe A. Temporal artery biopsy to diagnose adequate specimen length for a uni- mens were measured between 3 and temporal arteritis [letter]. JAMA. 1998;280: lateral biopsy. In a “Letter to the Edi- 6 hours of being placed in fixation, 1992.

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