Parafoveal Telangiectasia in Association with CREST Syndrome

CASE REPORTS AND SMALL CASE SERIES

ized by flattening of the anterior after cataract extraction; a periph- Pars Plana Tube Insertion chamber without pupillary block or eral iridectomy had not helped con- of Aqueous Shunt With choroidal effusion or suprachoroi- trol the IOP and the vision in the left Vitrectomy in Malignant dal hemorrhage, usually with an ac- eye had been lost. companying rise in IOP. To ex- On presentation, the visual acu- Glaucoma plain the pathogenesis of this ity was 20/100 OD and no light per- condition, Shaffer1 proposed that ception in the left eye. The IOP was Medical treatment, laser surgery, and aqueous is diverted posteriorly to- 42 mm Hg in the right eye and 12 vitreous surgery have been useful ward the vitreous cavity. Medical mm Hg in the left. Treatment for the options to treat aqueous misdirec- treatment,2 laser surgery,3,4 and vit- right eye was 2% dorzolamide hy- tion (malignant glaucoma). Two pa- reous surgery5-9 have all been use- drochloride thrice daily, 0.5% timo- tients with pseudophakic malig- ful options to treat this condition. lol maleate twice daily, 0.005% la- nant glaucoma unresponsive to We report a new method of sur- tanoprost at bedtime, and 50 mg of medical treatment underwent pars gical treatment for malignant glau- methazolamide orally twice daily, and plana vitrectomy and tube shunt im- coma, consisting of vitrectomy and treatment for the left eye was 1% plantation through the pars plana. tube shunt implantation through the prednisolone acetate twice daily and The outcome was favorable in both pars plana. 1% atropine sulfate twice daily. The patients. The implantation of the right eye had a shallow anterior cham- Report of Cases. Case 1. An 86- ber, 0.9 mm in depth (Figure 1). For editorial comment year-old white woman was first seen There were 2 patent peripheral iri- see page 799 in our service in September 1996 be- dotomies. The left eye had a deep cause of a shallow anterior cham- anterior chamber. The anterior cham- tube shunt through pars plana can ber associated with episodes of high ber angle was closed by peripheral help prevent recurrence of this con- IOP in the right eye. The patient had anterior synechiae, 360° in both eyes. dition by avoiding reaccumulation had 2 Nd:YAG laser peripheral iri- The optic disc of the right eye showed of fluid in the posterior segment of dotomies after cataract extraction, advanced glaucomatous cupping; the the eye, and can help in long-term which had been performed 6 years cupping was total in the left eye. control of intraocular pressure (IOP) before coming to us. The IOP was Visual field examination of the right in these glaucomatous patients. quite labile despite medicinal treat- eye with a computerized perimeter “Aqueous misdirection” or ment. The left eye was amblyopic (threshold strategy) revealed a severe “malignant glaucoma” is character- and had developed “pupillary block” constriction of the visual field, and with a marked depression of the retinal sensitivity in the arcuate areas. Ultrasound biomicroscopy ruled out ciliary or choroidal effusion or supra- choroidal hemorrhage. Additional medical treatment was prescribed: 1% tropicamide 4 times daily, 1% atropine sulfate 4 times daily, and 2.5% phenylephrine hydrochloride 4 times daily. Topical and systemic aqueous suppressants were continued, but 24 hours later the anterior chamber depth and IOP were unchanged in the right eye. Neodymium:YAG laser capsulotomy was considered, but

This article is also available on our Web site: Figure 1. Slitlamp photograph of the right eye in case 1. The anterior chamber is very shallow. Two www.ama-assn.org/ophth. (patent) peripheral iridotomies are barely seen at the 10- and 1-o’clock positions.

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/23/2021 believed unlikely to be of adequate immediate benefit. The severity of the glaucomatous damage and high IOP, which prompted an urgent and effective intervention, and the presence of peripheral anterior synechiae in the anterior chamber angle, which would probably impede a sat- isfactory long-term control of IOP, were the factors considered in the surgical planning. The patient underwent a standard 3-port pars plana vitrectomy, removing the anterior vitreous and adhesions around the peripheral iridectomies; a Baerveldt (350 mm2) tube shunt was im- planted through the pars plana. This surgical technique has been described elsewhere.5-9 During the procedure, the anterior chamber deepened. The postoperative outcome was Figure 2. Slitlamp photograph of the same eye as in Figure 1 four weeks after surgery. The anterior uncomplicated (Figure 2). Six chamber is deep. The temporal iridotomy has been enlarged. months later, the visual acuity in the right eye was 20/80, IOP was 13 mm The patient was treated with in- Chronic angle closure glaucoma Hg with no medical treatment, the an- travenous mannitol solution and oral and/or failure of previous filtering terior chamber was deep, and there acetazolamide; the current topical surgery can occur in cases of malig- was no apparent complication. treatment was supplemented with nant glaucoma after successful pars Case 2. A 72-year-old white 2.5% phenylephrine hydrochloride plana vitrectomy: all 5 cases re- woman developed pain and blurred 4 times daily. The following day the ported by Momoeda et al5 required vision in the left eye. She had pre- corneal edema in the left eye per- medicinal treatment, and 2 of 5 pa- viously had Nd:YAG laser periph- sisted, the anterior chamber depth tients required further filtration sur- eral iridotomies in both eyes and 2 was unchanged, and the IOP was 29 gery after successful reversal of ma- weeks before coming to us had un- mm Hg. Neodymium:YAG laser was lignant glaucoma by pars plana dergone an uncomplicated phaco- not performed because of the lack vitrectomy, associated with intra- emulsification with posterior cham- of corneal clarity. The patient un- capsular cataract extraction. The fil- ber intraocular lens implantation in derwent a pars plana vitrectomy and tering bleb failed in 1 of the 2 pa- the left eye. The visual acuity was a Baerveldt (250 mm2) tube shunt tients with pseudophakic malignant 20/30 OD and counting fingers at implantation. The tube tip was glaucoma, which was treated by vi- 120 cm OS. The IOP was 15 mm Hg placed in the posterior segment trectomy, as described by Lynch et in the right eye and 48 mm Hg in the through the pars plana. The ante- al.6 Byrnes et al7 observed failure of left. Treatment in the left eye con- rior chamber deepened during the the bleb after vitrectomy for ciliary sisted of 0.5% timolol maleate twice procedure. The postoperative pe- block glaucoma in 4 of 19 cases. Har- daily, 0.2% brimonidine tartrate thrice riod was uneventful. Four months bour et al8 recently described 21 pa- daily, 2% dorzolamide thrice daily, after surgery in the left eye, the best- tients with malignant glaucoma and 1% cyclopentolate hydrochlo- corrected visual acuity was 20/25, treated with vitrectomy; 4 had post- ride twice daily. The right eye had a the anterior chamber was deep, and operative visual loss due to progres- clear cornea, a moderately shallow an- IOP was 10 mm Hg. sive glaucoma.8 terior chamber, 2 patent iridoto- Although pars plana tube in- mies, and a moderately dense nuclear Comment. Malignant glaucoma or sertion with vitrectomy has been cataract. The cornea of the left eye had aqueous misdirection is initially used to treat neovascular glauco- diffuse corneal edema and a very shal- managed with mydriatic-cyclople- mas and glaucomas associated with low central anterior chamber but gic drops, aqueous suppressants, and pseudophakia and aphakia,10-12 to without lens-corneal touch. Two hyperosmotics.2 If medicinal therapy our knowledge, the use of this tech- patent iridotomies were present at the is unsuccessful, laser or surgical in- nique to treat malignant glaucomas 12- and 2-o’clock positions. Gonios- tervention is usually advised. Pars has not been reported previously. copy with indentation revealed a plana vitrectomy can be effective In the 2 patients described closed anterior chamber angle. The when other therapies fail, espe- herein, prompt surgical interven- retina was attached, without periph- cially in pseudophakic and aphakic tion was indicated owing to the lack eral choroidal or retinal abnormali- eyes.5-9 However, after resolving the of response to the initial medicinal ties. The optic disc had moderate attack of aqueous misdirection it- treatment, the level of IOP, the ad- glaucomatous damage. Visual field ex- self, the IOP can remain elevated or vanced glaucomatous damage (case amination was not performed. even increase due to other factors. 1), and the presence of corneal

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/23/2021 edema (case 2). In patients without kic malignant glaucoma. Am J Ophthalmol. 1986; wear.1 They have been noted less fre- 102:149-153. advanced glaucomatous damage, 7. Byrnes GA, Leen MM, Wong TP, Benson WE. quently after daily wear soft or rigid medical treatment should be tried for Vitrectomy for ciliary block (malignant) glau- contact lens wear. Patients using ex- 2 to 4 days before surgical interven- coma. Ophthalmology. 1995;102:1308-1311. tended wear soft lenses typically de- 8. Harbour JW, Rubsamen PE, Palmberg P. Pars tion, and, when possible, Nd:YAG la- plana vitrectomy in the management of pha- velop microcysts after 4 to 8 weeks. ser treatment can be tried. The sat- kic and pseudophakic malignant glaucoma. The number of cysts that develop is Arch Ophthalmol. 1996;114:1073-1078. isfactory postoperative outcome 9. Weiss H, Shin DH, Kollarits CR. Vitrectomy for related to the oxygen permeability probably reflects the efficacy of pars malignant (ciliary block) glaucomas. Int Oph- of the lens and the duration of wear, plana vitrectomy in treating the thalmol Clin. 1981;21:113-119. and they take several months to dis- 10. Lloyd MA, Heuer DK, Baerveldt G, et al. Com- aqueous misdirection and tube shunt bined Molteno implantation and pars plana vi- appear after discontinuatiuon of lens implantation in controlling the IOP. trectomy for neovascular glaucoma. Ophthal- wear. They typically occur in small It is also likely that the tube in- mology. 1991;98:1401-1405. 11. Gandham SB, Costa VP, Katz LJ, et al. Aque- numbers in the center or the mid- serted through the pars plana will ous tube-shunt implantation and pars plana vi- periphery of the cornea and rarely prevent recurrence of this condi- trectomy in eyes with refractory glaucoma. Am produce symptoms. In contrast, we J Ophthalmol. 1993;116:189-195. tion. However, it is not known 12. Varma R, Heuer DK, Lundy DC, Baerveldt G, describe 4 patients who wore daily whether more prolonged medical Lee PP, Minckler DS. Pars plana Baerveldt tube wear soft contact lenses who devel- therapy or pars plana vitrectomy insertion with vitrectomy in glaucomas asso- oped an acute reduction in vision as- ciated with pseudophakia and aphakia. Am J without tube shunt implantation Ophthalmol. 1995;119:401-407. sociated with a gross microcystic epi- would have been sufficient. theliopathy. The clinical features and We suggest that pars plana tube the possible origin of this condi- insertion with vitrectomy can be a Acute Microcystic Corneal tion are discussed. useful technique to treat patients Epitheliopathy After Daily with malignant glaucoma and a Soft Contact Lens Wear Methods. Four patients who had closed anterior chamber angle. This worn daily wear soft contact lenses technique can effectively relieve the We describe 4 patients who devel- and who presented with an acute bi- aqueous misdirection. The implan- oped an acute bilateral microcystic lateral corneal microcystic epitheli- tation of the tube shunt through pars corneal epitheliopathy with a opathy were identified (Table). plana can help prevent recurrence of marked reduction in visual acuity When first examined, all were ad- this condition, and can help in long- after using daily wear soft contact vised not to wear their contact lenses; term control of IOP in glaucoma- lenses. The cysts cleared over a pe- they were then given a topical anti- tous patients. This technique ap- riod of 4 weeks with complete vi- biotic (0.5% chloramphenicol 4 pears to be especially appropriate for sual recovery, although 1 patient de- times a day for 1 week) and re- patients with moderate or severe veloped central corneal flattening. viewed until resolution of the mi- glaucomatous damage. Examination of an epithelial bi- crocysts was complete. The first pa- opsy specimen from 1 patient was Augusto Azuara-Blanco, MD, PhD tient had an epithelial biopsy negative for Acanthamoeba. How- L. Jay Katz, MD performed to exclude Acantha- ever, apoptosis was demonstrated Sai B. Gandham, MD moeba keratitis as the cause; part of by staining with TUNEL (TdT- George L. Spaeth, MD the biopsy specimen was incubated mediated dUTP nick-end labeling) Philadelphia, Pa on nonnutrient agar seeded with assay . We believe that this phenom- killed Escherichia coli, while the re- Dr Azuara-Blanco is now with Queen’s enon is the result of acute corneal mainder was examined using a rab- Medical Centre, University of Not- hypoxia. bit anti–Acanthamoeba monoclonal tingham, Nottingham, England. Epithelial microcysts are small antibody. A TUNEL (TdT-medi- Corresponding author: L. Jay (15- to 50-µm) inclusions previ- ated dUTP nick-end labeling) as- Katz, MD, Wills Eye Hospital, 900 ously described as a delayed re- say (Gibco BRL, Paisley, United Walnut St, Philadelphia, PA 19107 (e- sponse to extended contact lens Kingdom), which identifies dying mail: [email protected]).

1. Shaffer R. Role of vitreous detachment in apha- Characteristics of Patients With Acute Microcystic Corneal kic and malignant glaucoma. Trans Am Acad Ophthalmol Otolaryngol. 1954;58:217-231. Epitheliopathy From Daily Wear Soft Contact Lenses 2. Chandler PA, Simmons RJ, Grant EM. Malig- nant glaucoma: medical and surgical treat- Patient No./ Duration of Wear Worst Final ment. Am J Ophthalmol. 1968;66:495-502. Age, y/Sex Prior to Onset, y Cleaning System Visual Acuity* Visual Acuity* 3. Herschler J. Laser shrinkage of the ciliary pro- cesses: a treatment for malignant (ciliary block) 1/31/F 5 Isopropyl alcohol, polidronium −6/18+2 OD −6/5 OD glaucoma. Ophthalmology. 1980;87:1155- chloride −6/12+2 OS −6/5 OS 1159. 2/25/F 2 Hydrogen peroxide −1/60 OD −6/6 OD 4. Epstein DL, Steinert RF, Puliafito CA. Neo- dymium:YAG laser therapy to the anterior hya- −1/60 OS −6/6 OS loid in aphakic malignant (ciliary block) glau- 3/28/F 1 Polyquad −2/60 OD −6/6 OD coma. Ophthalmology. 1980;87:1155-1159. −3/36 OS −6/6 OS 5. Momoeda S, Hayashi H, Oshima K. Anterior 4/29/F 4 Hydrogen peroxide −6/18 OS −6/6 OD pars plana vitrectomy for phakic malignant glau- −6/36 OS −6/6 OS coma. Jpn J Ophthalmol. 1983;27:73-79. 6. Lynch MG, Brown RH, Michels RG, Pollack IP, Stark WJ. Surgical vitrectomy for pseudopha- *With spectacle correction.

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/23/2021 Figure 1. Left, Clinical photograph of case 1 taken on the morning of the onset of symptoms. Note the large number of cysts that are almost confluent in the epithelium. Right, Clinical photograph of case 2 taken 3 days after the onset of symptoms. The peripheral cornea (inferior of photograph) is clear of cysts.

(apoptotic) cells by labeling fragmented DNA, was also performed.

Results. Two patients wore lenses on a regular basis and 2 only wore lenses for social occasions. All patients denied previous lens prob- lems, overnight wear, or contact lens abuse. All had worn their lenses on the day before the onset of symptoms and, on waking, had noted a marked reduction in vision with only a mild foreign body discomfort. First examinations were between 1 and 3 days of the onset of symptoms. Vision was reduced in all eyes and deteriorated further in 2 patients despite the discontinuation of contact lens wear (Table). All had Figure 2. Photomicrograph of biopsy specimen taken from the corneal epithelium from case 1 that has mild conjunctival hyperemia, slight folded such that the superficial surface is concave. Note the microcysts containing shrunken epithelial cells with condensed nuclear material (hematoxylin-eosin, original magnification ϫ 60). Insert, A single chemosis, but no anterior uveitis. In TUNEL (TdT-mediated dUTP nick-end labeling) assay–positive cell (original magnification ϫ 75). 2 patients the microcysts extended to the limbus, but in the 2 who were seen mm at 180/7.60 at 090 OD; 7.60 mm wear soft contact lenses with a rec- later, there was a peripheral ring of at 180/7.75 at 090 OS) when she was ommendation that the daily wear- normal epithelium approximately 2 first fitted with Permaflex (Wesley ing time does not exceed 8 hours. mm in diameter (Figure 1). There Jesson, PBH Ltd, Southampton, There has been no recurrence of was only scattered punctate uptake of United Kingdom) lenses (8.70:14.40: symptoms after a minimum fol- fluorescein by the epithelium. Cen- −9.50 OD, 8.70:14.40:−8.50 OS) that low-up of 20 months. tral corneal thickness was normal, she wore uneventfully for 5 years. Findings from examination of there were no stromal striae, and, af- Four weeks after we saw her, her the corneal epithelial biopsy speci- ter resolution, all patients had a nor- myopia had reduced from −9.50/ men were negative for Acantha- mal endothelial cell structure with no −0.75 at 015 OD; −8.75/−1.25 at 180 moeba. Histologic investigation signs of epithelial basement mem- OS to −5.25/−1.00 at 050 OD; −3.75/ showed superficial cysts contain- brane disease. Peripheral corneal neo- −1.00 at 145 OS, and this refrac- ing shrunken epithelial cells with vascularization was not present. tion has been stable for 24 months. small condensed nuclei or frag- Resolution of the microcysts Her keratometry readings have not ments of nuclear material, often with progressed centrally and all eyes changed, but videokeratoscopy dem- dark homogeneous eosinophilic cy- were free of cysts within 4 weeks. Pa- onstrated approximately 4 diopters toplasm (Figure 2). The TUNEL as- tient 3 developed a 2-mm-diameter of apical corneal flattening. say revealed that a few of these cells epithelial erosion in one eye that All patients have subse- had nuclei with cleaved DNA (Fig- healed without further symptoms. quently returned to contact lens ure 2, insert). Patient 1 developed a bilateral faint wear. Patient 1 changed to daily wear subepithelial haze that had cleared rigid gas permeable contact lenses af- Comment. The formation of cor- by 4 weeks. This patient had kera- ter 9 months without lenses, while neal epithelial microcysts follow- tometry readings in 1988 of (7.55 the remainder have returned to daily ing extended contact lens wear is

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/23/2021 thought to result from chronic hy- Corresponding author: Stephen J. roids, severe temporal scleral thin- poxia that leads to cell death and an Tuft, MD, FRCOphth, Moorfields Eye ning developed (Figure 1). There accumulation of debris in intercel- Hospital, London EC1V 2PD, England were no signs of external inflamma- lular pockets.1 Increased metabolic (e-mail: [email protected]). tion or active scleritis, and there was activity after discontinuation of lens no clinical or laboratory evidence of 1. Holden BA, Sweeney DF. The significance of the wear may then result in an increase microcystic response: a review. Optom Vis Sci. collagen vascular disease. On ex- in the number of microcysts that are 1991;68:703-707. amination, visual acuity was hand then cleared by anterior migration 2. Kenyon E, Polse KA, Seger RG. Influence of wear- motions and results of funduscopic 1 ing schedule on extended-wear complications. from the epithelium. Associated ab- Ophthalmology. 1986;93:231-236. examination revealed marked vit- normalities, such as faint axial stro- 3. Ren H, Wilson G. Apoptosis in the corneal epi- reous debris and a total RD with 2 thelium. Invest Ophthalmol Vis Sci. 1996;37: mal infiltrates, are uncommon. 1017-1025. grade C proliferative vitreoreti- We propose that acute corneal 4. Shimizu S, Eguchi Y, Kamiike W, et al. Induc- nopathy. microcystic epitheliopathy is also a tion of apoptosis as well as necrosis by hypoxia At surgery, placement of the and predominant prevention of apoptosis by response to hypoxia. The reason for Bcl-2 and Bcl-XL. Cancer Res. 1996;56:2161- eyelid speculum alone provoked ec- the sudden onset after a period of 2166 tasia of the temporal sclera with im- trouble-free lens wear is unclear and 5. Bron AJ, Tripathi RC. Cystic disorders of the cor- ϫ neal epithelium, I: clinical aspects. Br J Ophthal- pending rupture. An 18 10-mm the mechanism of the apical cor- mol. 1973;57:361-375. full-thickness donor scleral patch neal flattening in one patient is un- graft was placed over the thinned explained. A recent study of cor- area and sutured to healthy sclera neal epithelial cell shedding has Repair of a Complex along its edges (Figure 2). Imme- shown that a few surface cells nor- diately, a 3-port pars plana vitrec- Retinal Detachment mally have fragmented DNA and that tomy was performed. Following vit- cell death, as defined by a calcein- With Proliferative reous removal, extensive preretinal ethidium viability assay, is a nor- Vitreoretinopathy and subretinal fibrous proliferation mal feature of corneal epithelial cell in an Eye With Extreme surrounding an inferotemporal pe- turnover.3 Hypoxia is a recognized Scleral Thinning ripheral retinal break was identi- cause of apoptosis,4 and it is pos- fied. The retina was reattached by sible that premature cell death be- The repair of complicated retinal de- membrane peeling, relaxing reti- neath the epithelial surface contrib- tachments (RDs) entails substan- notomy for 3 clock hours, and per- uted to the formation of microcysts tial mechanical stresses on the globe fluorocarbon liquid (Perfluoron, in our patients. due to ocular rotation for expo- Infinitech, Chesterfield, Mo). En- Other causes of epithelial mi- sure, elevated intraocular pressure dolaser retinopexy was performed, crocystic keratopathy were consid- with infusion fluids and tampon- the perfluorocarbon liquid ex- ered.5 However, none of our pa- ades, and scleral alterations with changed for air, and the air ex- tients had features of an associated buckling. We successfully repaired changed for 16% perfluoropropane corneal dystrophy or endothelial fail- a total RD with grade C prolifera- gas. It was not possible to com- ure, history of infection, surgery, or tive vitreoretinopathy in an eye with pletely cover the scleral graft by mo- abrasion, and associated external eye extreme scleral attenuation by bilizing conjunctiva; 1 week later, a disease. No chemical component of strengthening the sclera prior to vi- free conjunctival autograft from the the contact lens care systems was trectomy without the use of scleral contralateral eye was placed over the common to all cases, although a non- buckling. exposed sclera. specific toxic side effect cannot be At the last examination, 6 weeks excluded as a possible mechanism. Report of a Case. A 60-year-old man after vitrectomy, the visual acuity was Findings from the culturing and im- with a phthisical left eye due to an 20/500. While necrosis of the con- munohistochemistry for Acantha- unrepaired RD after cataract sur- junctival autograft had necessitated moeba, a further cause of epitheli- gery 10 years earlier was referred its excision 2 weeks earlier, vascu- opathy in contact lens wearers, were with a total RD in his right eye. Three larization of the healthy-appearing also negative. We therefore con- years prior to being referred to us, scleral patch was evident (Figure 3). sider that hypoxia is the most likely the patient had undergone unevent- The retina was completely reat- cause and that the phenomenon may ful extracapsular cataract surgery tached and there were no signs of be a step on the way to contact lens– with posterior chamber intraocular recurrent proliferative vitreoretinopa- induced epithelial necrosis. lens implantion in the right eye. Nine thy. At no time during the postop- months later, a rhegmatogenous RD erative period was control of intra- Stephen J. Tuft, MD, FRCOphth was successfully repaired using an ocular pressure a problem. Philip Luthert, FRCPath encircling silicone sponge exo- London, England plant with visual recovery to 20/70. Comment. Donor sclera is com- Six months prior to examination, a monly used to cover ectatic areas of Staphylococcus epidermidis buckle in- sclera in patients with inflamma- The rabbit anti–Acanthamoeba mono- fection necessitated removal of the tory diseases, wrap orbital im- clonal antibody was a gift from Simon exoplant, with redetachment 6 days plants, cover glaucoma seton hard- Kilvington, PhD, Public Health Labo- later. Despite successful treatment ware, support extremely thin eye ratory, Bath, England. with systemic antibiotics and ste- walls prior to scleral buckling,1 and

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/23/2021 Figure 1. Severe superotemporal scleral thinning present 1 month after removal of an infected scleral buckle.

Figure 2. Left, Intraoperative exposure of a large region of drastically attenuated sclera prior to patching. Right, An 18 ϫ 10-mm donor scleral graft being sized to cover the area of missing eye wall.

Figure 3. Left, Well-positioned scleral patch graft, 6 weeks after vitrectomy. The conjunctival autograft had necrosed and been removed by this time. Right, Early vascularization of the scleral patch has begun.

cover exoplants that have become scleral graft appears to be surviving of globe rupture in this one-eyed pa- exposed.2,3 Although the conjunc- and the importance of conjunctival tient with a defect of almost one third tival autograft became necrotic de- covering remains unclear.4 of the eye wall. Reinforcement by spite aggressive management with Intraoperative trauma and post- placement of a scleral patch permit- 1% topical progesterone drops, the operative activity posed a serious risk ted successful repair of a complex

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/23/2021 RD using vitrectomy techniques that the telangiectatic vessels, presence staining involving the temporal hemi- did not cause scleral buckling. of retinal exudation, and degree of fovea of both eyes, while the right capillary occlusion.1 We present a macula displayed evidence of marked Panagiotis G. Theodossiadis, MD case that, to the best of our knowl- enlargement of the foveal avascular Donald J. D’Amico, MD edge, is the first description of para- zone with an irregular “moth-eaten” Boston, Mass foveal telangiectasia seen in associa- appearance (Figure 2). No intrareti- David J. Weissgold, MD tion with the syndrome of calcinosis nal crystals were noted. On general Burlington, Vt cutis, Raynaud phenomenon, esoph- examination, dermal characteristics ageal dysmotility, sclerodactyly, and of scleroderma were noted on her Reprints: Donald J. D’Amico, MD, telangiectasia (CREST syndrome). face, extremities, and chest; sausage- Retina Service, Massachusetts Eye & like thickening of her fingers was also Ear Infirmary, 243 Charles St, Bos- Report of a Case. In 1986, a 61- observed. ton, MA 02114-3096. year-old white woman was seen by her general practitioner with symp- Comment. Although relatively un- 1. Wilson RS, Parker JC. Scleral patch for exposed silicone buckles. Ophthalmic Surg. 1975;6:83- toms indicative of the Raynaud phe- common, CREST syndrome con- 85. nomenon affecting both hands. Cu- sists of easily identifiable symp- 2. Regenbogen L, Romano A, Zuckerman M, Stein taneous erythema and telangiectatic toms. Systemic vascular changes are R. Histoacryl tissue adhesive in some types of retinal detachment surgery. Br J Ophthalmol. 1976; vessels were observed and the fa- responsible for the telangiectatic ves- 60:561-564. cial skin had a foreshortened ap- sels, which seem to affect several sys- 3. Watzke RC. Scleral patch graft for exposed epi- pearance with resulting tightness. tems within the body. These vascu- scleral implants. Arch Ophthalmol. 1984;102: 114-115. From these features a diagnosis of lar abnormalities have been noted 4. de la Maza MS, Tauber J, Foster CS. Scleral graft- scleroderma was established. Ap- in the skin and gastric mucosa,3 ing for necrotizing scleritis. Ophthalmology. 1989; 96:306-310. proximately 4 years after her initial but to date have not been identified diagnosis, she developed gastric mo- with retinal findings. However, it is tility symptoms consisting of dys- reasonable to suggest that such wide- pepsia and dysphagia. In January spread vascular change could pre- 1997, she complained of decreas- sent itself in the eye. Our examina- Parafoveal Telangiectasia ing vision in her right eye. Exami- tion of the patient’s retina and the in Association With nation by her ophthalmologist re- discovery of parafoveal telangiec- CREST Syndrome vealed macular edema and a retinal tasia, in the absence of diabetic reti- consultation was obtained. Exami- nopathy or other causative factors, Parafoveal telangiectasia has been nation at that time disclosed a vi- supports this argument and is im- described as a developmental or ac- sual acuity of 20/200 OD and 20/20 portant in identifying a potential sys- quired vascular anomaly of the fo- OS. Slitlamp examination results and temic association. This finding is an veal avascular zone characterized by intraocular pressure were unremark- important consideration for pa- irregular capillary dilatation, se- able. Dilated fundus examination re- tients with scleroderma, who may rous exudation, and macular edema.1 vealed telangiectatic vessels in both also be seen with unexplained loss The acquired form of this anomaly maculae with mild retinal thicken- of vision. has been termed idiopathic juxtafo- ing seen in the temporal parafoveal According to the Gass classifi- veal retinal telangiectasia and its eti- region (Figure 1). No evidence of cation,1 our patient would be cat- ology has not been established.2 Gass diabetic retinopathy was seen. Fluo- egorized as group 3A. Similar cases has divided this disorder into 3 rescein angiography disclosed a have been described in the litera- groups based on ease of visibility of characteristic appearance of late ture with systemic associations dif-

Figure 1. Demonstration of parafoveal telangiectatic changes in the right (left) and left (right) eye.

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/23/2021 Figure 2. Left, Midframe fluorescein angiography of right macula. Note evidence of leakage from telangiectatic vessels in parafoveal location in addition to enlargement of foveal avascular zone. Right, Midframe fluorescein angiography of left macula demonstrating staining in the temporal hemifovea.

ferent from ours. Grand et al2 have crossing to the nose and upper eye- identified a hereditary syndrome in Low-Dose External Beam lid of the left side. Visual acuity mea- which retinal telangiectasia with cap- Irradiation for Bilateral sured 20/400 OD and could not be illary nonperfusion is associated improved with refraction. Visual Diffuse Choroidal with frontoparietal lobe pseudotu- acuity was 20/80 OS and improved mor. The histopathologic features Hemangioma to 20/60 with +2.50+0.50ϫ70°. The of the central nervous system le- anterior segments and intraocular sions included unusual vasculopa- The diffuse choroidal hemangioma pressures were normal. Fundu- 2 thy and necrosis of white matter. is an uncommon congenital vascu- scopic examination of the right eye 4 Lim and Bressler have reported a lar tumor associated with the Sturge- revealed a diffuse reddish hue and case of group 3A parafoveal telan- Weber syndrome.1 Visual loss is massive choroidal thickening that giectasia associated with marked often secondary to nonrhegmatog- was most prominent in the poste- peripheral ischemia. Our patient enous retinal detachments, cystoid rior pole. A retinal fold was present did not display central nervous degeneration, photoreceptor loss, through the papillomacular bundle system dysfunction or peripheral and fibrous transformation of the with hyperplastic and metaplastic retinal ischemia. proliferated retinal pigment epithe- changes in the retinal pigment epi- The prescence of both juxtafo- lium.1 The use of low-dose external thelium surrounding the optic nerve veal telangiectasia and CREST syn- beam irradiation has been reported and in the macula. A serous detach- drome in our patient suggests a pos- as a possible treatment for nonrheg- ment of the retina was present infe- sible common pathophysiologic matogenous retinal detachments as- riorly with a pocket of fluid overly- basis for the 2 diseases. Investiga- sociated with diffuse choroidal he- ing the macula. In the left eye, an tion of more patients will assist in mangioma.2 We describe a patient even greater degree of choroidal establishing this association. with Sturge-Weber syndrome, bilat- thickening was associated with a bul- eral diffuse choroidal hemangio- lous detachment of the inferior retina Brad Proctor, BSc mas, and nonrhegmatogenous se- and a shallow detachment of the Tom Chang, MD, FRCSC rous retinal detachments treated macular region. No significant reti- Dawn Hay, RN with low-dose external beam irra- nal pigment epithelial changes were Vancouver, British Columbia diation. noted ophthalmoscopically. Corresponding author: Tom Chang, B-scan ultrasonography mea- MD, FRCSC, Section D, 2550 Willow Report of a Case. An 8-year-old girl sured the choroidal thickness as 8 St, Vancouver, British Columbia, with bilateral facial nevus flam- mm in the macula of the right eye Canada V5Z 3N9. meus was examined at the Bascom and 6 mm in the left eye. A simul- Palmer Eye Institute, Miami, Fla, for taneous A-scan revealed the tumor 1. Gass JD, Oyakawa RT. Idiopathic juxtafoveal reti- progressive visual loss in her left eye to be of high reflectivity. Fluores- nal telangiectasis. Arch Ophthalmol. 1982;100: of 8 days’ duration. Six months pre- cein angiography showed rapid fill- 769-780. 2. Grand MG, Kaine J, Fulling K, et al. Cerebro- viously, she had developed an exu- ing and marked extravasation of retinal vasculopathy; a new hereditary syn- dative detachment in her right eye fluorescein dye from the retinal and drome. Ophthalmology. 1988;95:649-659. 3. Binford R. CRST syndrome with gastrointesti- with decreased visual acuity to the choroidal vessels. Staining of the nal bleeding. Arch Dermatol. 1968;97:603-604. 20/400 level. peripapillary retinal pigment epi- 4. Lim JI, Bressler NM. Atypical parafoveal telan- Examination revealed a facial thelial hyperplastic changes was giectasis with subsequent anterior and posterior segment neovascularization. Retina. 1992; angioma involving the right fore- noted in the right eye, while in the 12:351-354. head and eyelid, cheek, and nose and left eye there was a C-shaped area of

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/23/2021 A, Horizontal axial B-scan echogram of the right eye shows a diffuse, highly elevated choroidal lesion with bullous retinal detachment. B, Horizontal axial B-scan echogram of the left eye shows a diffuse, highly elevated choroidal lesion with very shallow retinal detachment. C and D, Follow-up B-scan echograms of the right (C) and left (D) eyes show markedly decreased elevation of choroidal hemangiomas with resolution of retinal detachments.

fluorescein staining at the level of the sual field in the left eye was full with multiple occasions with xenon arc retinal pigment epithelium extend- enlargement of the blind spot tem- photocoagulation prior to undergo- ing temporally from the inferior por- porally, while in the right eye there ing radiation therapy when his section of the macula. was superior constriction. There was ond eye became affected. Because of the extensive dif- no evidence of irradiation compli- Since the initial report of Scott fuse nature of the choroidal hem- cations. There was no recurrence of et al, 2 additional reports have de- angiomas, low-dose external beam serous exudation in either eye. The scribed successful treatment of both radiation therapy was recommen- choroid measured less than 2 mm in unilateral diffuse choroidal heman- ded. The patient underwent an un- each eye by ultrasonographic ex- giomas associated with the Sturge- complicated course of external beam amination. Weber syndrome and circum- radiation therapy to both eyes to a to- Twenty months after radiation scribed choroidal hemangiomas with tal dose of 20 Gy (10 fractions of 200 therapy, visual acuity was 20/200 OD the use of lens-sparing low-dose ex- Gy over 10 days). and 20/30+1 OS. Lenses remained ternal beam irradiation.3,4 Schilling Follow-up 3 months after radia- clear and there was no evidence of re- et al3 treated 15 unilateral cases of tion therapy revealed complete reso- currence of the choroidal thicken- diffuse choroidal hemangiomas with lution of the exudative detachments ing or serous exudation. complete resolution of subretinal in each eye. The choroidal thicken- fluid in all cases, tumor mass shrink- ing appeared decreased with moder- Comment. To our knowledge, this age in 5 cases, and the improve- ate retinal pigment epithelial mot- is only the second case of bilateral dif- ment of vision in 7 eyes. Madre- tling of the posterior pole in the right fuse choroidal hemangiomas associ- perla et al4 report their experience eye. Visual acuity had improved to ated with bullous serous retinal de- with 5 unilateral cases in which sub- 20/50 OS and remained stable in the tachments to be treated by low-dose retinal fluid was resorbed in all pa- right eye. B-scan ultrasonography re- external beam irradiation. Among the tients by 1 year after radiation vealed a choroidal thickening of 3.0 4 patients desribed by Scott et al2 therapy. They report improvement mm OU (Figure). treated with low-dose ocular irradia- of the intraocular pressure after ir- At 11 months after radiation tion for diffuse choroidal heman- radiation in 1 patient who had an el- therapy, visual acuity measured 20/ gioma was 1 patient who had evi- evated intraocular pressure.4 300 OD and 20/40+2 OS. Intraocu- dence of bilateral disease. However, The occurence of bilateral cho- lar pressures were normal. The vi- he had been previously treated on roidal hemangiomas associated with

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/23/2021 bilateral facial nevus flammeus is Reprints: Justin L.Gottlieb, MD, Report of a Case. A 17-year-old boy rare.5 The treatment of eyes with dif- Department of Ophthalmology, Uni- suffered from focal epilepsy of un- fuse choroidal hemangioma that de- versity of Wisconsin, 2870 Univer- known origin since the age of 13. velop bullous nonrhegmatogenous sity Ave, Suite 206, Madison, WI Following unsuccessful treatment detachments is very difficult. These 53705 (e-mail: [email protected] with carbamazepine and valproate as lesions generally do not respond well .wisc.edu). monotherapy and polytherapy, he to the modalities commonly used to was put on a combination regimen 1. Witschel H, Font RL. Hemangioma of the cho- treat circumscribed choroidal hem- roid: a clinicopathologic study of 71 cases and of valproate and vigabatrin 18 angiomas.6 Another less common a review of the literature. Surv Ophthalmolol. months prior to our first examina- form of treatment that appears ef- 1976;20:415-431. 2. Scott TA, Augsburger JJ, Brady LW, Hernandez tion (2 g of vigabatrin and 2.4 g of fective in the treatment of choroi- C, Woodleigh R. Low-dose ocular irradiation for valproate per day). One year later, dal hemangiomas associated with diffuse choroidal hemangiomas associated with the patient became aware of a con- bullous nonrhegmatogenous retinal detach- exudative detachment is brachy- ment. Retina. 1991;11:389-393. striction of his visual field. 4,7 7 therapy. Zografos et al report the 3. Schilling H, Sauerwein W, Limmatzsch A, et al. Figure 1, A, shows the visual successful treatment of 2 diffuse cho- Long-term results after low-dose ocular irradia- field constriction (Goldmann ki- tion for choroidal haemangiomas. Br J Ophthal- roidal hemangiomas in which a mol. 1997;81:267-273. netic perimetry). The results of the 15-mm radioactive plaque was 4. Madreperla SA, Hungerford JL, Plowman PN, static perimetry were comparable placed “in the presumed center of Laganowski HC, Gregory PTS. Choroidal hemangiomas: visual and anatomic results of treat- (Figure 1, C). Findings for visual acu- the tumor.” Although the authors re- ment by photocoagulation or radiation therapy. ity (20/20 OU), motility, eye posi- port successful resolution of asso- Ophthalmology. 1997;104:1773-1778. tion, anterior segments, and fundus 5. Lindsey PS, Shields JA, Goldberg RE, Augsburger ciated serous detachment in each JJ, Frank PE. Bilateral choroidal hemangiomas and were completely normal. Color vi- case, they conclude that this tech- facial nevus flammeus. Retina. 1981;1:88-95. sion tested by the desaturated Panel nique cannot be recommended in 6. Shields JA, Shields CL. Intraocular Tumors: A Text D-15 test, absolute rod threshold af- and Atlas. Philadelphia, Pa: WB Saunders Co; patients with diffuse choroidal he- 1992:254-255. ter 30 minutes of dark adaptation mangiomas.7 7. Zografos L, Bercher L, Chamot L, Gailloud C, (Hartinger dark adaptometer, Carl The treatment of exudative de- Raimondi S, Egger E. Cobalt-60 treatment of choroidal hemangiomas. Am J Ophthalmol. 1996; Zeiss, Jena, Germany), and pattern tachments secondary to diffuse cho- 121:190-199. evoked visual potentials were also un- roidal hemangiomas with low-dose 8. Sagerman RH, Chung CT, Alberti WE. Radio- remarkable. Baseline and light peak sensitivity of ocular and orbital structures. In: fractionated ocular irradiation ap- Alberti WE, Sagerman RH, eds. Radiotherapy of of the electro-oculogram (ISCEV pears promising. In this case, such Intraocular and Orbital Tumors. Berlin, Ger- standard) also rendered normal re- treatment was safe and effective for many: Springer-Verlag; 1993:375-385. sults (Arden ratio, 2.1 OD, 1.9 OS). the short term. The potential com- The Ganzfeld electroretino- plications include cataract forma- gram (ERG) performed according to tion, keratitis sicca, radiation pap- the International Society for Clini- Electrophysiologic illopathy, and radiation retinopathy.8 cal Electrophysiology of Vision With follow-up of 20 months, our Evaluation of a Patient (ISCEV) standard was basically nor- patient showed an impressive reso- With Peripheral Visual mal. The b/a ratio was low (1.2; nor- lution of the massive thickening of Field Contraction mal ratio, 1.39 (SD, 0.12) and the the choroid (8 mm to 3 mm), reso- Associated With Vigabatrin amplitude of the second oscillatory lution of the associated serous de- potential was 31 µV compared with tachments without evidence of re- Anticonvulsants are known to cause a normal value of 76 µV (SD, 18). currence, and an improvement of the different kinds of visual distur- While the rod and maximal b-wave vision in both eyes. We can only bances.1 Recently, vigabatrin has re- amplitudes fell within the center of speculate whether earlier treat- ceived attention because of consid- the normal range, the cone and ment of the right eye may have re- erable bilateral concentric field 30-Hz flicker responses were at the sulted in preservation of better vi- defects observed in a few patients re- lower border. In the multifocal ERG sion in that eye. At this time she ceiving this drug as add-on therapy.2 there were local responses of nor- shows no complications attribut- These field constrictions appear to mal amplitude (ie, the difference be- able to the radiation therapy, al- be irreversible in most cases. Sur- tween positive peak and negative though longer term follow-up may prisingly, only subtle changes of reti- trough) and implicit time even in ar- reveal such complications. nal function have been reported, eas of missing perception for the most often alterations of the oscil- Goldmann target III/4 (Figure 2, Justin L. Gottlieb, MD latory potentials in the electroreti- C). In contrast, the multifocal ERG Timothy G. Murray, MD nogram (ERG). Findings from his- of the patient with retinitis pigmen- J. Donald M. Gass, MD topathological studies in rats, mice, tosa who had a comparable visual Miami, Fla and dogs but not in monkeys and field constriction shows the typical This research was supported in part humans show a microvacuolation in decrease of amplitude and increase by an American Cancer Society award, myelin sheaths of the white matter of implicit time in the affected re- Tampa, Fla (Dr Murray), Fight for when exposed to vigabatrin.3 How- gions (Figure 2, B and D).4 How- Sight, Schaumburg, Ill (Dr Murray), ever, all these alterations cannot suf- ever, the wave form in the affected and a Stanley Glaser Award, Miami, ficiently explain a severe concen- areas of the patient receiving treat- Fla (Dr Murray). tric visual field constriction. ment with vigabatrin was different

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/23/2021 Treatment with vigabatrin was immediately discontinued when the visual field defect was diagnosed. At the follow-up examinations the patient sometimes indicated a sub- jective improvement, but even 6 months after the first examination, the visual field contraction tested by kinetic and static perimetry remained the same.

Comment. In the case reported herein, the concentric visual field loss is probably due to the intake of vigabatrin because (1) the intake of carbamazepine which is known to interact with retinal function was cessated 12 months prior to the first symptoms of visual field constriction; (2) we are unaware of retinal toxic side effects associated with valproate treatment; and (3) the fre- quency and severity of seizures were low and therefore probably not affecting visual function. Comparing the multifocal ERG to that of a patient with retinitis pigmentosa shows that the outer retina is probably not the site of disease action. As in other reports, the oscillatory potentials of the Ganzfeld ERG were attenuated in our patient. Together with the se- lective reduction of the N95 component in the pattern ERG, there is evidence of an alteration of inner retinal function. Vigabatrin is an aminobutyrate- aminotransferase antagonist and acts as a GABA (␥-aminobutyric acid) analog.5 GABA is a transmitter at different sites of the postreceptoral retina.6 It plays a role in the regula- tion of horizontal cell coupling, and an accumulation of GABA can be Figure 1. Goldmann kinetic visual fields of the patient taking vigabatrin (A) and a patient with retinitis found in amacrine cells. The visual pigmentosa (B). In part B, an additional isopter (V-4e) is displayed to illustrate that the difference between the sensitivity for bigger (V-4e) and smaller (I-4e) stimuli is rather small in the patient receiving field constriction might result from treatment with vigabatrin. C, A 30° static perimetry of the patient receiving vigabatrin (Octopus perimeter a block of transmission between bi- 500 EZ, Interzeag, Schlieren, Switzerland), program 36; the missing blind spot in the visual field of the polar cells and amacrine and/or gan- right eye is due to the rather coarse pattern of test stimuli (6°). glion cells. Another possibility might be a loss of ganglion cell function. in that the positive peak was low tern ERGs according to the ISCEV If so, the high redundancy of compared with the preceding nega- guidelines were normal for the information-processing in the cen- tive trough (Figure 2, A, arrows). central visual field. In contrast, tral retina may be the reason for The ERG-off response (red stimulating the temporal retina sparing of the central visual field. light, 200-millisecond stimulus du- where 50% of the pattern stimulus Another explanation could be that ration) was present, but the im- was displayed in the visual field vigabatrin is eventually more toxic plicit time was late (230 millisec- loss area, the N95 component of to peripheral than to central gan- onds; normal response time, 225.6 the transient pattern ERG became glion cells. In any case, ganglion milliseconds (SD,1.95 millisec- smaller than the P50 component cell damage may lead to optic atro- onds). The scotopic threshold re- (about 70% of the P50 component) phy in later stages of the disease sponse was normal. Readings from that never occurred in a normal which has already been reported in the transient and steady state pat- observer. other cases of visual field constric-

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/23/2021 Figure 2. The multifocal electroretinogram of the patient receiving treatment with vigabatrin and the patient with retinitis pigmentosa (left eyes) shown in Figure 1. A, Trace array of the patient receiving treatment with vigabatrin showing the 61 local responses representing the cone activity within the central 50° of visual angle. The change of wave form is marked by arrows (see “Report of a Case” section for further details). B, Trace array of the patient with retinitis pigmentosa showing the typical decrease of amplitude and increase of implicit time in the affected regions. C, A 3-D representation (scalar product) of the data from part A. The central peak corresponds to the high cone density of the fovea; the blind spot is visible on the left. D, A 3-D representation of the data for part B. Note the peripheral loss of response density.

tion associated with vigabatrin Corresponding author: Klaus Rue- abatrin [with letters of correspondence appearing in BMJ. 1997;314:1693-1695]. BMJ. 1997; medication. ther, MD, Charite´-Virchow Augen- 314:180-181. klinik, Augustenburger Platz 1, 13353 3. Graham D. Neuropathology of vigabatrin. Br J Klaus Ruether, MD Berlin, Germany (e-mail: ruether@ Clin Pharmacol. 1979;27(suppl 1):43-53. 4. Seeliger M, Kretschmann U, Apfelstedt-Sylla E, Tamara Pung, MD ukrv.de). Ruether K, Zrenner E. Multifocal electroretinog- Ulrich Kellner, MD raphy in Retinitis pigmentosa. Am J Ophthal- Bettina Schmitz 1. Bayer AU, Thiel H-J, Zrenner E, Paulus W, Ried mol. 1997;125:214-226. S, Schmidt D: Disorders of color perception and 5. Perucca E. The clinical pharmacology of the new Christian Hartmann, MD, PhD increased glare sensitivity in phenytoin and car- antiepileptic drugs. Pharmacol Res. 1993,28:89- Berlin, Germany bamazepine therapy: ocular side effects of anti- 106. convulsants. Nervenarzt. 1995; 66:89-96. 6. Kalloniatis M. Amino acids in neurotransmis- Mathias Seeliger, MD 2. Eke T, Talbot JF, Lawden MC: Severe persis- sion and disease. J Am Optom Assoc. 1995, 66: Tuebingen, Germany tent visual field constriction associated with vig- 750-757.

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