Brief Reports

BILATERAL PREPAPILLARY LOOPS WITH UNILATERAL BRANCH RETINAL ARTERY OCCLUSION FOLLOWING THROMBUS AT THE LOOP APEX KAMIAR MIRESKANDARI, FRCSED, FRCOPHTH, CHRISTOPHER BENTLEY, FRCOPHTH, WAGIH A. ACLIMANDOS, FRCS, FRCOPHTH From the Department of Ophthalmology, Kings College Hospital, London, England. Liebrich rst described a prepapillary arterial loop in 1871. This is a congenital vascular anomaly thought to originate from a main branch of the central retinal artery.1,2 These loops are usually unilateral and more common than venous loops. Most are asymptomatic but rare cases of retinal artery obstruction have been seen, most commonly affecting the inferior retina. We report a case of unilateral branch artery occlusion in a patient with bilateral loops demonstrating a thrombus at its apex with a follow-up period of 18 months. Case Report
A 24-year-old African man presented with sudden loss of vision in his right eye while reading in bed. Shortly after, the inferior eld of vision started to return and became normal but the superior visual eld loss remained. There were no other symptoms or relevant medical or ocular history. On examination the visual acuity was 6/6 in both eyes. There was a mild relative afferent pupillary defect and a marked superior altitudinal eld defect in the right eye. Examination of the right fundus revealed a twisted prepapillary loop (PPL) and a pale and edematous inferior retina (Figure 1A). The left eye showed a similar but smaller loop. Laboratory tests including hemoglobin electrophoresis were normal. The uorescein angiogram conrmed the inferior hemispheric arterial occlusion and the PPL did not ll at any stage (Figure 1B). The vascular pattern at the disk was unusual with more than one cilioretinal artery present. Presented at the Royal Society of Medicine; London; October 6, 1999. The authors have no proprietary interests. Reprint requests: Kamiar Mireskandari, FRCSEd, FRCOphth, Flat 2, 109 Sutherland Avenue, Maida Vale, London W9 2QH, England. Three months later, the PPL was empty of blood and a small thrombus could be seen at the apex of the loop (Figure 2A). The inferior retinal arteries were attenuated and the pallor had resolved. Eighteen months later, the blood supply to the inferior retina had improved. The uorescein angiogram demonstrated slow lling of the inferior retinal arteries possibly via cilioretinal collaterals (Figure 2B) whereas the PPL remained occluded and gliosed.

Discussion There has been some debate over the origin of PPL, principally concerning whether they are derived from the hyaloid or retinal arterial system. Mann1 suggested that the loops originate at about the 100 mm embryonic stage at the time when retinal vascularization begins. It is postulated that the retinal artery rst extends forward within Bergmeisters papilla (glial tissue anterior to the optic nerve) instead of turning 90 degrees to grow radially onto the retina. When the Bergmeisters papilla atrophies, the vessel loop is left protruding into the vitreous cavity. Two anatomic studies have supported the view of retinal arterial connection.2,3 There have been 10 cases of retinal arterial occlusion associated with unilateral loops but none with bilateral loops or where the thrombus was demonstrated.4 7 Although generally considered to be benign, PPL are associated with arterial occlusions, amaurosis fugax, vitreous hemorrhage, and hyphema. As most loops are asymptomatic and therefore remain either unseen or merely documented as a chance nding, the true incidence of these rare complications remains unknown. In this case as with most others reported, there was an abnormal peripapillary vascular pattern with unusual cilioretinal circulation. Furthermore, the inferior retinal arteries were those affected, the patient was young, and there were no associated systemic ndings. The mechanisms of occlusion of the PPL are not clear. Some authors have postulated a mechanical effect secondary to the spiral makeup of the loop leading to increased turbulence or torsion especially in association with posterior vitreous detachment. The size of the loop is thought to be important as well as

RETINA, The Journal of Retinal and Vitreous Diseases, encourages authors to submit Brief Reports describing unusual ndings, new techniques, and new instruments. Material submitted for consideration in this section of the journal is done so with the assumption that the data provided do not duplicate previously published material and that the material has not been submitted for consideration elsewhere. Each author must sign a disclosure to this effect (see Instructions to Authors for complete wording of transfer letter). Brief Reports submitted for this section of the journal may be subjected to the standard review process that is applied to other material submitted to RETINA. Brief Reports should follow the requirements listed in the Instructions to Authors, with the following caveats: Brief Reports should not exceed 4 pages in length; no more than 5 references should be cited; and each Brief Report should include no more than 4 gures.

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Fig. 1. A, Right inferior hemispheric retinal artery occlusion and prepapillary loop. B, Acute uorescein angiogram shows arterial nonperfusion and empty prepapillary loop.

Fig. 2. A, Magnied view of empty loop with thrombus at the apex. B, Angiogram 18 months later shows delayed perfusion inferiorly.

any focal narrowing that may trap chance emboli. Careful observation and monitoring of patients with this rare complication might reveal more specic predisposing factors. References
1. Mann I. Developmental Abnormalities of the Eye, 2nd Ed. Philadelphia: JB Lippincott, 1957;133136. 2. Goldstein I, Wexler D. The preretinal artery. An anatomical study. Arch Ophthalmol 1929;11:324 334. 3. Shakin EP, Shields JA, Augsburger JJ, Brown GC. Clinicopathological correlation of a pre-papillary vascular loop. Retina 1988;8:5558.

4. Harcourt R, Locket N. Occlusion of a pre-retinal arterial loop. Br J Ophthalmol 1967;51:562565. 5. Khoda N, Nakan Ishi Y. A case of pre-papillary arterial loops with retinal artery obstruction. Jpn J Clin Ophthalmol 1977;31: 744 745. 6. Brown GC, Margaral LE, Augsburger JJ, Shields JA. Pre-retinal arterial loops and retinal arterial occlusion. Am J Ophthalmol 1979;87:646 651. 7. Limaye SR, Tang RA, Pilkerton AR. Cilio-retinal circulation and branch arterial occlusion associated with pre-retinal arterial loops. Am J Ophthalmol 1980;89:834 839.

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PURTSCHERS RETINOPATHY SECONDARY TO AIRBAG INJURY GAURAV K. SHAH, MD,* ROBERT PENNE, MD, M. GILBERT GRAND, MD* From *Barnes Retina Institute and the Department of Ophthalmology and Visual Sciences, Washington University School of Medicine, St. Louis, Missouri; and Wills Eye Hospital, Philadelphia, Pennsylvania. Airbags have become a popular means of reducing injury and death in motor vehicle accidents. A recent National Highway and Trafc Safety Administration study estimated that airbags had saved the lives of approximately 3500 Americans as of September 1998.1 Airbags cushion occupants from the rigid components in a vehicle and need to be fully inated before an occupant moves forward to provide the most adequate protection. Unbelted passengers move forward further than belted passengers, which increases the risk of striking an expanding bag that is propelled out from its storage compartment at typically more than 100 miles per hour. Theoretically, unrestrained passengers may strike the airbag not only with their head but also with their thoracic region due to the absence of a shoulder harness. Airbags have been known to cause a multitude of ocular injuries, including corneal abrasions, alkali burns, traumatic cataracts, retinal and vitreous hemorrhages, retinal tear and detachment, macular holes, and choroidal ruptures.2,3 We present a case of Purtschers retinopathy following deployment of an airbag. Case Report
A 33-year-old man presented with decreased vision in both eyes after being involved in a motor vehicle accident. He was an unrestrained driver involved in an accident that occurred at less than 20 miles per hour. Although he was not wearing a seat belt, his car was equipped with an airbag, which inated at the time of the accident. His visual acuity was 20/30 in the right eye and count ngers in the left eye. He had a 3 afferent pupillary defect in the left eye. Intraocular pressures were within normal limits. The anterior segment examination was remarkable for 1 cell and are in the left eye; the right eye was quiet. The posterior segment examination of both eyes revealed multiple areas of yellowwhite patches at the inner retina level along with supercial intraretinal hemorrhages (Figure 1, A and B). The peripapillary changes were more pronounced in the left compared to the right eye. The left eye also revealed several blot hemorrhages in the posterior pole. A uorescein angiogram showed early blockage of the choroidal

uorescence in the area of the yellowwhite patches, whereas the late frames revealed hyperuorescence (Figure 2, A and B). Along with loss of vision, the patient had periorbital ecchymoses, but no fractures or systemic injuries. No chest contusion or trauma were suspected owing to lack of subjective chest pain or radiologic evidence of fractures of ribs or sternum. Given the constellation of clinical ndings, he was diagnosed with Purtschers retinopathy with a component of traumatic optic neuropathy. The patients follow-up after the injury was sporadic, but his vision did not improve in the left eye and remained at count ngers 3 months after the injury. The right eye stayed at 20/30.

Discussion Purtschers retinopathy was rst described by Purtscher in 1912, with ndings of white retinal patches and supercial retinal hemorrhages in patients with head trauma. Since the original description, other conditions associated with Purtschers have been reported, including acute pancreatitis; fat, air, or amniotic embolization; lymphoproliferative disorders; chest compression; bone marrow transplantation; and most recently pancreatic adenocarcinoma.4 We are unaware of any previous report of Purtschers retinopathy resulting from airbag injuries. Either direct blunt trauma to the face or a compressive effect in the thoracic region may have led to the development of a Purtscher-type retinopathy in our patient. The pathophysiology of Purtscher-like retinopathy is uncertain. An increase in thoracic pressure may lead to a reux in the venous system. Subsequent endothelial damage can cause incompetence of the microvascular system, resulting in occlusion and ischemia. Complement derived C5a has also been associated with the initiation of intravascular aggregation in some of the conditions associated with Purtscher-like retinopathy.5 In clinicopathologic studies, occluded retinal arterioles and choroidal vessels along with damage to the photoreceptors have been described. In our patient, the facial contusion may have led to the Purtschers and the traumatic optic neuropathy. The tractional forces at the time of injury may have disrupted the posterior ciliary arteries, causing a secondary ischemic optic neuropathy from the trauma. There may also have been contusion necrosis, compression, axonal disruption, and ischemia, all accounting for the traumatic optic neuropathy associated with the airbag injury. The same mechanism responsible for traumatic optic neuropathy also leads to a Purtscher-type response, with retinal edema and hemorrhage in the posterior pole. In this case there may have been a combined mechanism for the Purtschers, with both direct trauma to the face and a compressive effect in the thoracic region. Owing to lack of injury to the thoracic region, direct facial trauma may be the

Reprint requests: Gaurav K. Shah, MD, Barnes Retina Institute, One Barnes Hospital Plaza, Suite 17413, St. Louis, MO 63110; e-mail: shah_G@vision.wustl.edu

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Fig. 1. A, Color photograph of the right eye shows supercial intraretinal hemorrhages along with yellowwhite patches in the peripapillary region. B, Extensive peripapillary yellowwhite patches are noted around the optic nerve. Note multiple retinal hemorrhages in the posterior pole of the left eye.

Fig. 2. A, Early phase of the uorescein angiogram of the left eye reveals blockage of the choroidal circulation secondary to yellowwhite patches in the peripapillary region. B, Late frame of the uorescein angiogram reveals peripapillary hyperuorescence with staining of the optic nerve.

most likely cause for both the Purtschers and the optic neuropathy. This report describes a case of Purtscher-like retinopathy in association with an airbag-related injury in an unrestrained passenger. The mechanism, although unknown, is most likely due to a compressive-type injury. Despite this injury, our patient most likely avoided serious head injury due to the protective effects of the airbag. It is conceivable that by using his seat belt he may have been able to avoid his retinal injuries. Purtschers retinopathy should be added to the list of complications from airbag-related injuries. The future development of more sophisticated airbags can only enhance the safety features of the system and decrease the risk of secondary injuries due to the airbags themselves. References
1. Insurance Institute for Highway Safety. Airbags [online]. Available at: http://www.highwaysafety.org/airbags/airbag.htm. Accessed March 20, 1999. Kuhn F, Morris R, Witherspoon CD. Eye injuries and the airbag. Curr Opin Ophthalmol 1995;111:38 44. Stein JD, Jaeger EA, Jeffers JB. Airbags and ocular injuries. Trans Am Ophthalmol Soc 1999;97:59 82. Tabandeh H, Rosenfeld PJ, Alexandrakis G, et al. Purtscher-

5.

like retinopathy associated with pancreatic adenocarcinoma. Am J Ophthalmol 1999;128:650 655. Snady JL, Morse PH. Retinopathy associated with acute pancreatitis. Am J Ophthalmol 1985;100:246 251.

SURGICAL REMOVAL OF A LASERINDUCED CHOROIDAL NEOVASCULAR MEMBRANE ANDRS KYCHENTHAL, MD,* ALEJANDRO SIEBERT, MD,* PAOLA DORTA, MD,* BILL AYLWARD, MD From *Clnica Alemana, Santiago, Chile; and Moorelds Eye Hospital, London, United Kingdom. Choroidal neovascular membranes (CNVM) can be secondary to numerous causes that allow neovascular ingrowth from the choroid through a defect in Bruchs
Reprint requests: Andrs Kychenthal, MD, Clnica Alemana de Santiago, Vitacura 5951, Santiago, Chile; e-mail: akychenthal@ yahoo.com

2. 3. 4.

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Fig. 1. A, Preoperative color fundus photograph shows laser photocoagulation scars and the subfoveal laser-induced choroidal neovascular membrane. B, Preoperative uorescein angiogram shows uorescein leakage from the subfoveal choroidal neovascular membrane.

membrane. Iatrogenic CNVM after laser photocoagulation for different conditions have been reported.1 The development of an iatrogenic CNVM after laser photocoagulation in diabetic patients represents an uncommon but serious complication.2,3 We present a case of subfoveal iatrogenic CNVM treated by surgical removal with a good visual outcome. Case Report
In April 1997, a 34-year-old woman with a 20-year history of insulin-dependent diabetes presented with blurred vision in both eyes for 2 days. Visual acuity was 20/30 in both eyes. Clinically signicant macular edema was present in the right eye. Clinically signicant macular edema and new vessels located at the disk and along the superior arcade were present in the left eye. Four days later the patient underwent laser photocoagulation in both eyes. Focal laser of the right eye was performed with a 532-nm laser (Ophthalas 532, Alcon, Fort Worth, TX), 11 applications to the posterior pole (100 m spot size, 0.1 second duration, 70 to 100 mW power). One month after photocoagulation vision in the right eye was 20/100. Two months later vision dropped to 20/200 and fundus examination showed a CNVM with foveal involvement. Five months after laser treatment vision was 20/400 and uorescein angiography showed a CNVM with a classic angiographic pattern. In April 1999, 2 years after focal laser to the right eye, vision was still 20/400 with signicant distortion. Fundus examination revealed clinical and angiographic signs of an active CNVM involving the fovea (Figure 1).

In May 1999, the patient underwent pars plana vitrectomy and removal of the CNVM. At the rst month follow-up vision improved to 20/80. Five months after surgery vision was 20/70 and no evidence of recurrence was noted during the follow-up period (Figure 2).

Discussion Surgical removal of subfoveal CNVM is now well described. The difference in visual outcome after surgery for subfoveal CNVM in different clinical conditions was explained by Gass.4 With the fundus appearance and the histologic pattern, Gass suggested two distinct types of choroidal neovascular growth. In Type 1 CNVM, such as in age-related macular degeneration, diffuse changes in Bruchs membrane are associated with multiple ingrowth sites and the CNVM develops beneath the retinal pigment epithelium (RPE). In Type 2 CNVM, such as seen in multifocal choroiditis and presumed ocular histoplasmosis syndrome, there tends to be a single ingrowth site that proliferates anterior to the RPE in the subsensory retinal space. When Type 2 membranes are removed, preservation of native RPE could lead to good visual function. We report a case of a surgically treated iatrogenic subfoveal CNVM secondary to laser pho-

Fig. 2. A, Color fundus photograph 3 months after surgical removal shows no clinical sings of the choroidal neovascular membranes. Some pigmentary atrophy in the macular area is present. B, Postoperative uorescein angiogram shows complete excision of the choroidal neovascular membrane.

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tocoagulation in a diabetic patient with macular edema. Current surgical techniques allow CNVM removal with visual stabilization or improvement in a number of clinical conditions. Our patient improved in Snellen visual acuity from 20/400 to 20/80. This patient also observed a great decrease in distortion. These good results could be explained by the localized action of laser, which is more compatible with the formation of a Type 2 CNVM and thus preservation of native RPE after surgery. Timing of the surgical removal of CNVM is unclear. In this case we report good visual function obtained after pars plana vitrectomy and CNVM removal for a iatrogenic laser-induced CNVM in a diabetic patient, 2 years after the CNVM developed. References
1. Ching JC, Urban LL, Nelson NC, Fratkin JD. Surgical removal of iatrogenic choroidal neovascular membranes. Ophthalmology 1998;105:1606 1611. Chandra SR, Bresnick GH, Davis MD. Choroidovitreal neovascular ingrowth after photocoagulation for proliferative diabetic retinopathy. Arch Ophthalmol 1980;98:15931599. Wallow I, Johns K, Barry P, et al. Chorioretinal and choriovitreal neovascularization after photocoagulation for proliferative diabetic retinopathy. A clinicopathologic correlation. Ophthalmology 1985;92:523532. Gass JD. Biomicroscopic and histopathologic considerations regarding the feasibility of surgical excision of subfoveal neovascular membranes. Am J Ophthalmol 1994;118:285298.

chamber intraocular lens implantation.1 Since then, these lesions have been described in cases with anterior chamber lens placement and in cases without any lens placement.2,3 Of the reported cases of phototoxicity following cataract extraction, almost all had extrafoveal lesions with mild to moderate loss of visual acuity (VA) followed by spontaneous recovery within a few months postoperatively. We describe a patient who had profound visual loss secondary to macular phototoxicity following cataract extraction. Visual improvement did not occur until 8 months postoperatively.

Case Report
A 63-year-old man was struck in the left eye by a golf ball and was treated medically for a traumatic hyphema and uncontrolled intraocular pressure (IOP). Three months later he was referred for management of progressive shallowing of the anterior chamber, a traumatic cataract, and glaucoma. Examination revealed VA of 20/20 in the right eye and 20/70 in the left, improving with pinhole to 20/40. The results of anterior segment and funduscopic examination of the right eye were normal. The left pupil was 9 mm and minimally reactive with multiple sphincter tears. There was no afferent pupillary defect. Slit-lamp examination showed a traumatic cataract with mild phacodonesis and a shallow anterior chamber. With compression gonioscopy, anterior trabecular meshwork was visible inferiorly with scattered peripheral anterior synechiae. The IOP was marginally controlled at 28 mmHg on latanoprost 0.005%, dorzolamide 2%/timolol 0.5%, and brimonidine 0.2%. Fundus examination showed pigment cells in the vitreous, but healthy disk, macula, and vessels. B-scan ultrasonography conrmed the forward displacement of the normal thickness crystalline lens. Because of the progressive forward displacement of the lens and marginal IOP control, it was decided to remove the traumatic cataract via an anterior approach. The patient was taken to the operating room for planned phacoemulsication using the Zeiss OPMI (West Germany) operating microscope. During the capsulorhexis, the lens was noted to spin freely and the case was converted to an intracapsular procedure. An anterior vitrectomy was performed. An attempt to sew in a posterior chamber intraocular lens was aborted because of poor centration within the dilated, traumatized pupil. On the rst postoperative day, the patient noted a central scotoma with best-corrected vision of hand motions (HM). There was mild corneal edema and a moderate amount of inammation in the anterior chamber. The IOP was normal, and there was no afferent pupil defect. Dilated fundus examination revealed a 4 disk diameter elliptical area of retinal whitening in the macula involving the fovea. The lesion was highly suggestive of a macular phototoxicity burn (Figures 1 and 2). Best-corrected VA improved to 20/400 at 10 weeks and remained at that level until 6 1/2 months, where it improved to 20/200 with an aphakic contact lens. At 8 months follow up, the vision had improved to 20/40 with manifest refraction and 20/25 with an aphakic contact lens. The macular whitening gradually resolved, leaving retinal pigment epithelial changes with areas of atrophy and hyperplasia (Figure 3).

2.

3.

4.

VISUAL RECOVERY FROM MACULAR PHOTOTOXIC INJURY FOLLOWING CATARACT SURGERY MICHAEL S. LEE, MD, STEPHEN E. ORLIN, MD, ALEXANDER J. BRUCKER, MD From the Scheie Eye Institute, Department of Ophthalmology, University of Pennsylvania School of Medicine, Philadelphia. McDonald and Irvine rst described retinal phototoxicity from an operating microscope in 1983 following uncomplicated cataract extraction with posterior
The authors have no proprietary interest in the outcome of this report. Reprint requests: Alexander J. Brucker, MD, Scheie Eye Institute, 51 North 39th Street, Philadelphia, PA 19104; e-mail: ajbrucke@mail.med.upenn.edu

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Fig. 1. Appearance of the phototoxic lesion 1 week postoperatively. Blurred fundus appearance is secondary to corneal edema. Visual acuity is hand movements.

Fig. 3. Lesion from Figures 1 and 2, 8 months postoperatively. Visual acuity is 20/25 with aphakic contact lens.

Discussion Retinal phototoxicity following anterior segment surgery does not involve the fovea in most cases. Two mechanisms for foveal sparing have been proposed. It has been postulated that there is a protective effect of higher levels of xanthophyll pigment in the fovea, which absorbs the blue light most responsible for phototoxic injury. Additionally, light from operating microscopes is not coaxial with the visual axis in most cases of cataract surgery, thus avoiding the fovea.15 However, we found four cases of phototoxicity involving the fovea following cataract extraction described in the literature. Reported VA in these patients ranged from 20/100 to 20/200 postoperatively with visual recovery to better than 20/30 in three over the course of 3 months; the fourth patient remained at 20/200.2,4,5

Postel et al4 reported four cases of foveal phototoxicity after vitreoretinal surgery. The macula was subjected to intense light from endoilluminators without the protective effect of the phakic lens for longer periods of time. These patients VA ranged from 20/ 400 to HM postoperatively. Two of these patients experienced only minimal improvement and two had worsening of their VA. Initial postoperative VA in cases of macular phototoxicity involving the fovea has been reported to be only moderately affected following cataract surgery (20/100 20/200). Good recovery occurred within a few months in all cases. Foveal injury after vitrectomy, however, is typically more profound with poor outcome. As in the current case, despite profound visual loss (HM), excellent visual recovery can occur as late as 8 months postoperatively, which may be useful in patient counseling.

Fig. 2. Early (A) and late (B) frames of the uorescein angiogram of the lesion depicted in Figure 1.

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References
1. McDonald HR, Irvine AR. Light-induced maculopathy from the operating microscope in extracapsular cataract extraction and intraocular lens implantation. Ophthalmology 1983;90: 945951. Boldrey EE, Ho BT, Grifth RD. Retinal burns occurring at cataract extraction. Ophthalmology 1984;91:12971302. Lindquist TD, Grutzmacher RD, Gofman JD. Light-induced maculopathy: potential for recovery. Arch Ophthalmol 1986; 104:16411647. Postel EA, Pulido JS, Byrnes GA, et al. Long-term follow-up of iatrogenic phototoxicity. Arch Ophthalmol 1998;116:753 757. Ross WH. Light-induced maculopathy. Am J Ophthalmol 1984;98:488 498.

2. 3.

4.

5.

SECTOR RETINITIS PIGMENTOSA WITH BITEMPORAL VISUAL FIELD DEFECTS AND MACULAR HOLE DAVID A. SAPERSTEIN, MD From Emory Eye Center, Emory University School of Medicine, Atlanta, Georgia. This case report describes a patient who presented with sector retinitis pigmentosa with bitemporal visual eld defects complicated by a macular hole. Case Report
A 63-year-old woman sought a second opinion concerning gradual decreased central vision in the left eye diagnosed as cystoid macular edema (CME). She has a history of retinitis pigmentosa (RP). She reported that her mother had very poor vision as a result of RP. Her only other visual complaint was nyctalopia. In 1985 her Goldmann visual elds (GVF) were full (using V4eI4e targets). In August 1997, she noted decreased vision. At that time she was seen by another physician and her visual acuity (VA) was 20/20 in the right eye and 20/80 in the left. Dilated fundus examination revealed RP-like changes and an abnormality in the macula of the left eye that was diagnosed as CME. Goldmann visual eld examination revealed bitemporal scotomata. Funded in part by NEI K1100347, P30EY06360 (a NIH Department Core Grant), The Foundation Fighting Blindness Wayne T. Robertson Young Investigator Award, and Research to Prevent Blindness. The author has no proprietary interest. Reprint requests: David A. Saperstein, MD, Department of Ophthalmology, Box 356485, The University of Washington, Seattle, WA 98195; e-mail: dsapers@u.washington.edu

This unexpected visual eld nding closely resembled that produced by a central chiasmal lesion. Magnetic resonance imaging of the brain revealed no pathology. The CME was thought to be related to RP and the patient was treated with oral acetazolamide, retroseptal steroids, and topical 1% prednisolone acetate drops. Over several months her VA dropped to 20/200 in the left eye. In January 1998, the patient was examined in our clinic. Her VA was 20/20 in the right eye and 20/200 in the left eye. Intraocular pressures were 13 mmHg in the right eye and 11 mmHg in the left. The external examination was unremarkable. The anterior segment showed mild nuclear sclerotic cataracts. The vitreous was clear in both eyes. The cup-to-disk ratio was 0.4 in both eyes. Funduscopy revealed that arteries nasal to the foveae in both fundi were attenuated. There were typical bone spicule like pigmented lesions nasal to the foveae in both eyes (Figure 1, A and B). There were no pigment abnormalities temporal to the foveae in either eye. There was a normal foveal depression in the right eye. In the left eye there was no sign of CME; rather, there was a stage II macular hole with a surrounding cuff of subretinal uid (Figure 1C). A positive WatzkeAllen sign was present. Repeat GVF examinations revealed eld defects suggestive of bitemporal defects that obey the vertical midline and are consistent with the fundus ndings (Figure 2). Fluorescein angiography revealed no active CME; however, there was a retinal pigment epithelial window defect in the left foveal region (Figure 1D). Electroretinogram responses to scotopic and photopic stimuli were attenuated 40 to 60% in amplitude and the implicit times were within the normal range. Single stranded conformational polymorphism DNA testing revealed no defects in the rhodopsin or peripherin genes. Typical vitrectomy/uid gas exchange (10% C3F8 gas) technique with stripping of the internal limiting membrane, without the use of adjuvants,1 was performed to treat the macular hole. The patient maintained face-down positioning for 1 week. The hole was noted to be closed (Figure 1E). Her VA after 3 months improved to 20/30 and has remained at that level for 18 months.

Discussion This case illustrates three important issues. First, sector RP usually presents in the nasal quadrant of the fundus.2 Sector RP nasal to the foveae resulting in bitemporal visual eld defects is rare.3 In this case the GVF ndings led the referring physician to obtain magnetic resonance imaging to look for a central chiasmal lesion. Second, macular holes in patients with RP respond to standard surgical correction. This hole may be a result of previous CME, but more likely represents an idiopathic macular hole misdiagnosed as CME. Finally, this case illustrates that patients with RP are susceptible to non-RPrelated eye disease and shows the importance of these patients receiving routine eye examinations to rule out problems that might be masked by the RP.

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Fig. 1. A, B, Digital composite photographs of right and left fundi, respectively. Note that the pigment spicules are only present nasal to the foveae in each eye. These ndings are consistent with the Goldmann visual eld examinations in Figure 2. C, Red-free photograph of the left fovea. Note the macular hole (arrows) and the surrounding subretinal uid (fat arrows). D, Late uorescein angiogram of the left macula. There is an area in the fovea of hyperuorescence at the level of the retinal pigment epithelium (RPE). This area most likely represents an RPE window defect due to the macular hole. E, Postoperative fundus photograph of the left eye 3 months after surgery. The macular hole is closed with resolution of subretinal uid.

Fig. 2. Goldmann visual eld of both the right (A) and left (B) eyes. The temporal eld defects obey the vertical midline and are consistent with the pigmentary changes in the fundus in Figure 1, A and B.

References
1. Olsen TW, Sternberg P Jr, Capone A Jr, et al. Macular hole surgery using thrombin-activated brinogen and selective removal of the internal limiting membrane. Retina 1998;18:322 329.

2. 3.

Krill AE, Archer D, Martin D. Sector retinitis pigmentosa. Am J Ophthalmol 1970;69:977987. Abraham FA. Sector retinitis pigmentosa. Electrophysiological and pyschophysiological study of the visual system. Doc Ophthalmol 1975;39:1328.

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BILATERAL OCULAR ISCHEMIC SYNDROME IN TAKAYASU DISEASE MARTIN WORRALL, MD,* NEAL ATEBARA, MD, TRAVIS MEREDITH, MD, ERIC S. MANN, MD, PHD From the *Baylor College of Medicine, Department of Ophthalmology, Houston, Texas; Retina Associates of Hawaii, Honolulu; and Barnes Retina Institute and Department of Ophthalmology, Saint Louis University Health Sciences Center, St. Louis, Missouri. Takayasu or pulseless disease is a rare, idiopathic, chronic granulomatous large-vessel vasculitis involving the aorta and its primary branches. Clinical manifestations relate to the anatomic site of vascular obstruction and may be life threatening. Takayasu disease occurs predominantly in young Japanese women, but also rarely in black patients.1 Ocular manifestations occur as a consequence of attenuation or obliteration of the carotids with ocular ischemia. The retinal vascular changes progress from generalized vasodilation and microaneurysmal formation to arteriovenous shunts, vaso-obliteration, and proliferative retinopathy.2 Irreversible visual loss also occurs from anterior ischemic optic neuropathy3 and neovascular glaucoma.4 We report a case of bilateral ocular ischemic syndrome in an black patient with Takayasu disease. Case Report
A 49-year-old black woman was referred for loss of vision with ocular pain in the left eye for 1 week. Fever, weight loss, malaise, and amaurosis fugax in the right eye occurred 12 years earlier. The patient appeared cachectic with absent carotid, brachial, and radial Reprint requests: Eric S. Mann, MD, PhD, Saint Louis University Eye Institute, 1755 S. Grand Boulevard, St. Louis, MO 63104; e-mail: manne4@slu.edu

pulse. Visual acuity measured 20/20 in the right eye and 20/50 in the left with no relative afferent pupillary defect. Anterior segment examination was unremarkable. Posterior pole ndings showed cotton-wool spots, venous dilation, and beading with moderate arterial narrowing (Figure 1A) in both eyes. Multiple intraretinal hemorrhages, microaneurysms, and telangiectasis extended to the midperiphery. Fluorescein angiography showed delayed arterial lling and arteriovenous transit time with retinal vascular staining particularly the arterioles (Figure 1B) in both eyes. Panretinal photocoagulation was not performed because there was no neovascularization of the iris, optic disk, or retina. Cardiac, carotid, and vascular evaluation was recommended. Six days later the patient awoke with confusion, aphasia, and right-sided weakness. A cranial MRI revealed an acute left temporoparietal stroke. Carotid Doppler and thoracic angiogram demonstrated total occlusion of the left common carotid artery and right and left subclavian arteries with near complete occlusion of the right common carotid artery, consistent with Takayasu disease. She was lost to follow-up but returned 3 years later with visual acuity 3/200 in the right eye and light perception in the left with normal intraocular pressures. The left cornea revealed 360 of vascular ingrowth with no view of the fundus and complete angle closure. The right eye showed 1 anterior chamber cell and are, rubeosis iridis with 270 of angle closure, and progressive cataract. There was optic nerve pallor with collateral vessels on the disk and arteriovenous shunts within the macula. Arterial pulsations on the disk were easily elicited with minimal pressure on the globe. Fluorescein angiography (Figure 2) revealed peripheral capillary nonperfusion. Panretinal photocoagulation and cataract extraction without intraocular lens placement was performed with regression of rubeosis iridis. Visual acuity remains 20/320 in the right eye through 12 months of postoperative follow-up.

Discussion The American College of Rheumatology criteria for Takayasu disease require three of the following six features of the disease: 1) onset at age 40 years or younger, 2) claudication of the extremities, 3) decreased pulsation in one or both brachial arteries, 4) systolic blood pressure difference of greater than 10 mmHg between arms, 5) audible bruit over the aorta or at least one subclavian artery, and 6) arteriographic narrowing or occlusion of the aorta, its primary

Fig. 1. A, Fundus photograph of the left eye shows dilated, beaded retinal veins, cottonwool spots, and microaneurysms. B, Fluorescein angiogram of the left eye shows microaneurysms and late retinal vascular staining, primarily arterioles.

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EPIPAPILLARY ADENOMA OF RETINAL PIGMENT EPITHELIUM JERRY A. SHIELDS, MD,* TOUFIC MELKI, MD, CAROL L. SHIELDS, MD,* RALPH C. EAGLE, JR, MD, ARUN D. SINGH, MD* From the *Oncology Service and the Department of Pathology, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania; and the Department of Ophthalmology, Georgetown University, Washington, DC. Adenoma of retinal pigmented epithelium (RPE) is a rare tumor that can simulate choroidal melanoma.1 Features that help differentiate RPE adenoma from melanoma have been elucidated.2 Most RPE adenomas are located in peripheral retina and it is extremely rare for an adenoma of the RPE to arise posteriorly and grow to obscure the optic disk. We report a clinicopathologic correlation of a juxtapapillary RPE adenoma that enlarged and covered the optic disk, suggesting malignant transformation of a melanocytoma into a malignant melanoma. Case Report
In 1976, an asymptomatic 22-year-old man was found to have a nevus on the margin of his left optic disk. He was examined over the next 23 years by several ophthalmologists, each of whom assured him that the lesion was benign and stable. In August 1999, he experienced sudden visual loss in the left eye. His visual acuity was 6/6 in the right and 6/7.5 in the left eye. A deeply pigmented mass, much larger than noted previously, obscured the left optic disk. It measured 6.5 5.5 mm in diameter and 4.5 mm in thickness, conrmed with ultrasonography. Yellow circinate intraretinal exudation and subretinal uid were present on the inferior and nasal borders of the lesion (Figure 1). Pigmented cells emanated from the mass to inltrate the vitreous inferiorly. There was a tractional retinal hole between the equator and ora serrata superonasally. Fluorescein angiography showed the mass to be relatively hypouorescent in the early angiograms and to have slight overlying leakage of dye in the late angiograms. There was intense late staining of the surrounding subretinal uid and exudation (Figure 2). A- and B-scan ultrasonography revealed the mass to have medium internal reectivity. We could not obtain prior photographs but the patient recalled that the lesion was described as small and benign.

Fig. 2. Fluorescein angiogram of the right eye (3 years after previous uorescein angiogram; Figures 1) shows collaterals on the optic disk and retinal arteriovenous shunts at arteriovenous crossings (arrows) with vaso-obliteration of peripheral neighboring capillaries.

branches, or large vessels of the proximal upper or lower extremities. Our patient demonstrated all six features with ocular ndings in both eyes consistent with ocular ischemic syndrome. Systemic evaluation failed to reveal any other known etiology. Takayasu arteritis should be considered in the case of retinal arteriovenous shunts2 or ocular ischemic syndrome, particularly when accompanied by symptoms of claudication, coldness, or weakness of the extremities, even in young, non-Asian patients. Although systemic steroids and immunosuppressive agents may be used early in the disease, once retinal or anterior segment neovascularization occurs, panretinal photocoagulation, cryoablation, or glaucoma surgical procedures are often necessary but may not halt disease progression. This case shows the ocular manifestations of Takayasu disease and the important role of the ophthalmologist in disclosing a life-threatening systemic vascular disease.

References
1. 2. 3. Human GP. Takayasus arteritis case report of an African female. S Afr Med J 1967;41:445 447. Tanaka T, Shimizu K. Retinal arteriovenous shunts in Takayasus arteritis. Ophthalmology 1987;94:1380 1388. Schmidt MH. Bilateral anterior ischemic optic neuropathy as a presentation of Takayasus disease. J Neuro-ophthalmol 1997; 17:156 161. Karwatowski WSS, Jampol LM, Mani H, Weinreb RN. Neovascular glaucoma in Takayasus disease: a case report. Retina 1995;15:353354.

4.

Supported by the Eye Tumor Research Foundation, Philadelphia, PA, the Award of Merit in Retina Research, Houston, TX (Dr. J. Shields), the Macula Foundation, New York, NY (Dr. C. Shields), and the Noel T. Sara L. Simmonds Endowment for Ophthalmic Pathology, Wills Eye Hospital, Philadelphia, PA (Dr. Eagle). Presented at the annual Wills Eye Hospital Retina Conference; Philadelphia, PA; November 12, 1999 (Dr. Melki). Reprint requests: Jerry A. Shields, MD, Director, Oncology Service, Wills Eye Hospital, 900 Walnut Street, Philadelphia, PA 19107.

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Fig. 3. Elevated, solid, pigmented mass lying directly over the optic disk (hematoxylin-eosin, original magnication 10). Fig. 1. Fundus photograph shows deeply pigmented epipapillary mass with surrounding circinate exudation.

Our differential diagnosis included 1) melanoma arising from optic disk melanocytoma, 2) necrotic melanocytoma, and 3) RPE adenoma. The patient was informed of the diagnostic possibilities and was offered observation, needle biopsy, or enucleation. He elected to undergo enucleation because of concern about malignancy. Gross and microscopic examination disclosed an intensely pigmented tumor that measured 5 5 4 mm and appeared to arise from and cover the optic disk (Figure 3). Microscopically, it was composed of cords and tubules of RPE cells (Figure 4), separated by periodic acid-Schiffpositive basement membrane. The tumor was continuous with the RPE and focally invaded the choroid, sensory retina, and vitreous. There was localized juxtapapillary retinal detachment with lipoproteinaceous intraretinal exudation. The diagnosis was adenoma of the RPE, presumably arising adjacent to the optic disk.

viously documented to grow entirely over and obscure the optic disk. Such a nding is more consistent with an optic nerve melanocytoma or juxtapapillary melanoma. In retrospect, the circinate exudation is more suggestive of adenoma of the RPE2 but is rare with melanocytoma or choroidal melanoma. However, in our case, a melanoma could not be absolutely excluded based on clinical ndings. In summary, this case demonstrates that adenoma of the RPE can occur on the optic disk and produce vitreous seeding, suggesting malignant transformation of a melanocytoma. Although these pigmented lesions can be quite similar, the presence of exudative retinopathy should suggest adenoma of the RPE, rather than melanocytoma or melanoma. .

Discussion The features that help differentiate adenoma of the RPE from choroidal melanoma have been elucidated.2 RPE adenoma usually occurs in the peripheral retina and rarely arises in a juxtapapillary location.35 To our knowledge, however, RPE adenoma has not been preReferences
1. Shields JA, Shields CL. Tumors of the retinal pigment epithelium. In: Shields JA, Shields CL. Atlas of Intraocular Tumors. Philadelphia: Lippincott Williams & Wilkins, 1999:288 292. Shields JA, Shields CL, Gunduz K, Eagle RC Jr. Neoplasms of

2.

Fig. 2. Fluorescein angiography. A, Full venous phase shows hypouorescence of the mass. B, Late angiogram shows minimal leakage of dye into the center of the mass and more intense staining around the mass corresponding to the exudation and subretinal uid.

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Fig. 4. Bleached section shows cords of epithelial cells, consistent with adenoma of the retinal pigment epithelium (bleached sections, hematoxylin-eosin, original magnication 200).

3.

4.

5.

the retinal pigment epithelium. The 1998 Albert Ruedemann Sr. Memorial Lecture. Part 2. Arch Ophthalmol 1999;117:601 608. Shields JA, Eagle RC Jr, Shields CL, De Potter P. Pigmented adenoma of the optic nerve head simulating a melanocytoma. Ophthalmology 1992;99:17051708. Loose IA, Jampol LM, OGrady R. Pigmented adenoma mimicking a juxtapapillary melanoma. Arch Ophthalmol 1999;117: 120 122. Shields JA, Eagle RC Jr, Barr CC, Shields CL, Jones DE. Adenocarcinoma of the retinal pigment epithelium arising from a juxtapapillary histoplasmosis scar. Arch Ophthalmol 1994;112:650 653.

OPTICAL COHERENCE TOMOGRAPHY AND ELECTROPHYSIOLOGY IN X-LINKED JUVENILE RETINOSCHISIS ASSOCIATED WITH A NOVEL MUTATION IN THE XLRS1 GENE PAULO E. STANGA, MD,* N.H. VICTOR CHONG, FRCOPHTH,* ANNE C. RECK, FRCOPHTH,* ALISON J. HARDCASTLE, PHD, GRAHAM E. HOLDER, PHD From the *Department of Clinical Ophthalmology, Moorelds Eye Hospital and Institute of Ophthalmology; the Department of Molecular Genetics, Instituteof Ophthalmology; and Department of Electrophysiology, Moorelds Eye Hospital, London, United Kingdom.
None of the authors has a proprietary interest. P.E. Stanga is supported by the Lady Anne Allerton Fund, London, UK. Reprint requests: G.E. Holder, PhD, Department of Electrophysiology, Moorelds Eye Hospital, 162 City Road, London EC1V 2PD, UK; e-mail: graham.holder@moorelds.nthames.nhs.uk

We describe optical coherence tomography (OCT) and electrophysiology ndings in X-linked juvenile retinoschisis (XLRS) associated with a novel mutation in the XLRS1 gene. A 39-year-old man had been diagnosed with macular dystrophy in early childhood. There was no family history. Right visual acuity had been 20/120 from childhood, and left acuity dropped from 20/40 to 20/ 200 over 20 years. The fundal appearance is shown in Figure 1, A and B. Optical coherence tomography single line bilaminar scans of the posterior pole region were performed using the OCT 2000 scanner (Zeiss Humphrey Instruments, San Leandro, CA). Electroretinogram (ERG), pattern ERG (PERG), and ONOFF responses were recorded using standardized methods (International Society for Clinical Electrophysiology of Vision [ISCEV]). Sequence analysis of the XLRS1 gene was carried out to obtain genetic conrmation of the disease. All six exon fragments of the XLRS1 gene were amplied with intronic primers.1 Polymerase chain reaction was carried out in 25-L reactions in the presence of 0.5 U Taq polymerase (Biotaq; Bioline, London, UK); 200 mol/L each of dATP, dCTP, dGTP, and dTTP; 200 pmol of each primer; and 1 KCl reaction buffer (Bioline) including 1.5 mmol/L MgCl2. Polymerase chain reaction conditions were as described by Sauer et al.1 Amplication products were puried with centricon concentrators (Amicon Limited, Gloucestershire, UK) following manufacturers instructions. The puried DNA sample (5 L) was cycle sequenced in both directions (with primers used for amplication) using ABI prism Ready Reaction dye termination cycle sequencing kit (FS kit, Perkin Elmer Applied Biosystems, Warrington, UK) following manufacturers instructions. Reactions were then electrophoresed on an ABI 373A sequencer. Electrophysiology (Figure 2) showed marked generalized postreceptor dysfunction with macular involvement, compatible with XLRS. Note the absent rod ERG, the electronegative maximal ERG, the amplitude and implicit time changes in photopic and icker ERG, and the marked PERG reduction, the latter indicating the macular dysfunction. The reduction in both ON- b-wave and OFF- d-wave, with sparing of the ON- a-wave, suggested involvement of both depolarizing and hyperpolarizing photopic pathways. Optical coherence tomography demonstrates a cleavage plane in neural retina (Figure 3). Direct sequencing of the XLRS1 gene revealed a splice donor mutation in intron 1 (52 2 t 3 c) (Figure 4). This t-to-c substitution at the splice donor site of intron 1 disrupts a nucleotide that is 100% conserved and is predicted to result in a lack of protein product.

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Fig. 1. A, Color fundus photograph of the right eye shows nonspecic foveal changes (solid black line indicates the optical coherence tomography scan plane in Figure 4). B, Color fundus photograph of the left eye shows classic peripheral schitic changes.

Discussion X-linked juvenile retinoschisis is a recessively inherited vitreoretinal degeneration characterized by macular pathology and intraretinal splitting of the retina that may be associated with alterations in the XLRS1 gene.2 The characteristic foveal cystic changes in XLRS may disappear in adulthood with the development of nonspecic atrophic lesions. Only 50% have peripheral schitic changes.3 Histopathologically, the splitting occurs in the nerve ber layer, and may reect Mller cell abnormality.4 The cleavage plane location may be determined by OCT,5 enabling in vivo imaging of schisis even when it is not evident on biomicroscopy. Typical electrophysiology conrms generalized postreceptor retinal dysfunction.

This brief report illustrates the combined value of OCT and electrophysiology in suspected retinoschisis, and describes a novel mutation. References
1. Sauer CG, Gehrig A, Warneke-Wittstock R, et al. Positional cloning of the gene associated with X-linked juvenile retinoschisis. Nat Genet 1997;17:164 170. The Retinoschisis Consortium. Functional implications of the spectrum of mutations found in 234 cases with X-linked juvenile retinoschisis (XLRS). Hum Mol Genet 1998;7:1185 1192. Gass JDM. Stereoscopic Atlas of Macular Diseases, 3rd ed. St Louis: CV Mosby, 1987;1:292. Manschot WA. Pathology of hereditary juvenile retinoschisis. Arch Ophthalmol 1972;88:131. Rutledge BK, Puliato CA, Duker JS, et al. Optical coherence

2.

3. 4. 5.

Fig. 2. Electrophysiologic ndings in the patient show an electronegative electroretinogram with a bright white ash, typical of X-linked retinoschisis. See text for further details.

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tomography of macular lesions associated with optic nervehead pits. Ophthalmology 1996;103:10471053.

DIABETIC PAPILLOPATHY WITH MACULAR STAR MIMICKING CLINICALLY SIGNIFICANT DIABETIC MACULAR EDEMA YOLANDA FRIEDRICH, MD, MICHAEL FEINER, MD, HAYTHUM GAWI, MD, ZVI FRIEDMAN, MD From The Annette and Aron Rozin Department of Ophthalmology, BnaiZion Medical Center, Faculty of Medicine, Haifa, Israel. Optic disk edema occurring in young people with type I diabetes mellitus (DM) has been described by several investigators.1-3 The usual presentation is transient unilateral or bilateral optic disk swelling associated with minimal or no permanent visual decit. Similar cases have been reported later in older patients, most of them with type II DM. Regillo et al4 described 19 patients (27 eyes) with disk edema,13 of whom had type II DM. Of the 27 eyes, 19 had diabetic macular edema (DME), 6 of which required laser treatment. In two eyes the macular edema seemed to be an extension of the disk edema. We present three cases one bilateral, two unilateral (four eyes) of diabetic optic disk swelling and marked decrease of visual acuity associated with macular edema presenting as macular star, originating from the disk capillaries, mimicking clinically signicant DME (CSDME). Materials and Methods Three patients with DM, background diabetic retinopathy (BDR), and optic disk swelling one bilateral, two unilateralwere seen in whom the disk swelling was associated with contiguous macular edema and a macular star. Detailed clinical information was obtained on each patient including complete eye examination, uorescein angiography, and Goldmann perimetry. Systemic evaluation, blood tests, and brain computerized tomography (CT) scan were performed to rule out other causes of disk swelling.
Fig. 4. Electropherogram depicts patient mutation at position (52 2 t 3 c) in intron 1 of the XLRS1 gene.

Fig. 3. A, Single line optical coherence tomography (OCT) scan of the right eye, which demonstrates a cleavage plane in the neural retina not evident on biomicroscopy. B, Higher magnication OCT of the left eye, which demonstrates a cleavage plane in the neural retina with bridging retinal elements.

Reprint requests: Zvi Friedman, MD, The Annette & Aron Rozin Department of Ophthalmology, BnaiZion Medical Center, Faculty of Medicine, The Technion, 47, Golomb St., Haifa 31048, Israel.

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Fig. 1. Left eye of Case 1, 2 days after presentation. A, Fundus photograph shows disk edema with multiple ame shaped hemorrhages and macular edema with star conguration of hard exudates. B, Fluorescein angiography, arteriovenous phase. Multiple ame shaped hemorrhages mask background uorescence. Scattered leaking capillaries due to background diabetic retinopathy. Capillary bed is intact. C, Fluorescein angiography, late phase. Dye leakage extends from disk.

Results Four eyes (three patients, all with type II DM) presented with diabetic papillopathy associated with contiguous macular edema during the years 1996 to 1998. Age ranged between 65 and 67 years. In all eyes there was moderate BDR, swelling of the optic nerve head, ame shaped hemorrhages, and macular edema with hard exudates mimicking CSDME. The main distinguishing feature from CSDME was the star conguration of these exudates rather than a circinate pattern (Figure 1A). All affected eyes had severe visual loss with visual acuity at presentation ranging from 1/60 to 5/60. There was a relative afferent pupillary defect (RAPD) in the two unilateral cases. Goldmann perimetry revealed cecocentral scotoma. Fluorescein angiography (Figure 1, B and C) conrmed the presence of BDR and demonstrated moderate to marked late staining extending from the disk. There was no or only mild dye leakage from perifoveal capillaries. Neither capillary nonperfusion nor proliferative diabetic retinopathy was detected in any of the eyes. In all patients, blood pressure measurement, brain CT scan, chest X rays, puried protein derivative skin test, blood sedimentation rate, angiotensin converting enzyme, and blood serology revealed no abnormal nding. There was no history of recent tightening of the glycemic state by intensive insulin treatment. Because diabetic papillopathy was the assumed diagnosis no treatment was applied. In three of the eyes the disk edema and ame shaped hemorrhages as well as the macular edema and macular star resolved spontaneously after 1 to 8 months and visual acuity improved, ranging from 6/6 to 6/12 with disappearance of the RAPD and central scotomata. The remaining patient was lost to follow-up. A year later three eyes (two patients) developed

CSDME and underwent modied grid laser photocoagulation. Discussion A syndrome of idiopathic transient optic disk edema with minimal or no clinical evidence of permanent visual loss in young, insulin-dependent diabetic patients was described in 1971 by Lubow and Makley.1 Since then, a similar clinical picture has been described in patients with type II DM.4,5 To our knowledge, no reference to a macular star conguration of hard exudates was made in these cases, although Regillo et al4 noted macular edema extending from the disk in a few patients. The incidence of diabetic papillopathy is difcult to estimate. Barr et al3 reported a frequency of 0.4% among all diabetic patients seen by an ophthalmologist. In the clinical context of DM, disk swelling, decreased visual acuity, normal systemic blood pressure, the absence of symptoms of increased intracranial pressure, and a normal brain CT scan, an intrinsic optic nerve head vasculopathy (diabetic papillopathy) should be considered. Visual eld defect, which in early papilledema presents as an enlarged blind spot, manifests as a central scotoma, arcuate scotoma, or sector scotoma in diabetic papillopathy3patterns similar to those found in our series. Anterior ischemic optic neuropathy (AION) is the most common cause of optic disk edema in subjects over the age of 55 and is believed to be the result of occlusion of branches of the short posterior ciliary arteries supplying the optic nerve head.6 Twenty percent of patients with AION, especially below the age of 64, also have DM.7 Visual loss in patients with AION is usually sudden and frequently permanent, and disk pallor usually results after the swelling resolves. In the current series, three of the four eyes

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regained vision of 6/6 to 6/12 within 1 to 8 months; the remaining eye was lost to follow-up. The recovery of nal visual acuity in two of our patients is in contrast to the optic atrophy and permanent visual loss usually seen in patients with AION. Macular star formation of hard exudates indicates marked permeability of the disk capillaries. To our knowledge it has never been seen in AION. Gass8 suggested that the star-shaped pattern of hard exudates is due to leakage from prelaminar disk capillaries. Fluid spreads from the disk through the outer plexiform layer of the retina and accumulates in Henles layer. The unique anatomy of this layer accounts for the formation of the radially oriented macular star following the absorption of the edematous uid and the precipitation of lipoproteins. It is common in neuroretinitis and malignant hypertension,9 which were not present in our cases. We describe four eyes of three patients with type II DM who developed disk edema associated with macular edema and a star formation of hard exudates. According to the Early Treatment Diabetic Retinopathy Study,10 the presence of hard exudates within 500 m from the center of the fovea associated with retinal thickening (CSDME) is an indication for laser treatment; however, in cases where such exudates assume a star conguration rather than a circinate pattern and are associated with disk edema, uorescein angiography determines the origin of these exudates to be from the disk and not from the perifoveal capillaries, obviating the need for laser treatment. Repeat blood pressure measurement is important to exclude malignant hypertension as the causative factor. These exudates often disappear without treatment as soon as the disk edema subsides, leading to recovery of visual acuity. Further follow-up is needed to detect CSDME that can develop later, as happened in two of our patients and required laser treatment. References
1. 2. Lubow M, Makley TA. Pseudopapilledema of juvenile diabetes mellitus. Arch Ophthalmol 1971;85:417 422. Pavan PR, Aiello LM, Wafai MZ, Briones JC, Sebestyen JB, Bradbury MJ. Optic disc edema in juvenile-onset diabetes. Arch Ophthalmol 1980;98:21932195. Barr CC, Glaser JS, Blankenship G. Acute disc swelling in juvenile diabetes. Clinical prole and natural history of 12 cases. Arch Ophthalmol 1980;98:21852192. Regillo CD, Brown GC, Savino PJ, Byrnes GA, Benson WE, Tasman WS. Diabetic papillopathy. Patient characteristics and fundus ndings. Arch Ophthalmol 1995;113:889 895. Barrett K. Disc swelling in an adult diabetic patient. Surv Ophthalmol 1990;35:158 163. Miller NR. Anterior ischemic optic neuropathy: diagnosis and management. Bull NY Acad Med 1980;56:643 654. Repka MX, Savino PJ, Schatz NJ, Sergott RC. Clinical prole and long term implication of anterior ischemic optic neuropathy. Am J Ophthalmol 1983;96:478 483.

9. 10.

Gass JDM. Disease of the optic nerve that may simulate macular disease. Trans Am Acad Ophthalmol Otolaryngol 1977;83:763770. Tso MOM, Jampol LM. Pathophysiology of hypertensive retinopathy. Ophthalmology 1982;89:11321145. The Early Treatment of Diabetic Retinopathy Study Research Group. Photocoagulation for diabetic macular edema. Arch Ophthalmol 1985;103:1796 1806.

SURGICAL MANAGEMENT OF COMBINED TRACTION AND RHEGMATOGENOUS RETINAL DETACHMENT ASSOCIATED WITH PERSISTENT FETAL VASCULATURE JOHN O. MASON III, MD From the University of Alabama at Birmingham School of Medicine. Persistent hyperplastic primary vitreous (PHPV), a congenital anomaly in which the embryonic hyaloid vasculature fails to regress normally, was rst described by Reese in 1955.1 Goldberg, in 1997, renamed PHPV persistent fetal vasculature (PFV).2 Persistent fetal vasculature has been separated into anterior, posterior, and combined anterior/posterior, depending on which ocular structures are involved. This report describes an infant who underwent a scleral buckle procedure for combination traction and rhegmatogenous retinal detachment (RD) secondary to PFV. Case Report
A 3-month-old, full-term girl was noted to have right facial and lid capillary hemangiomas. The referring ophthalmologist discovered a retinal abnormality and referred the patient for further evaluation. On examination, the patient had right facial and lid capillary hemangiomas. The anterior segment examination revealed a posterior polar lenticular density with a small retrolental membrane. The nasal ciliary processes were elongated (Figure 1). The vitreous was clear and the optic nerve was normal. A thin vitreous stalk was present from the posterior lens to the optic nerve. The retina was totally detached and traction folds were noted in the far nasal periphery. The left eye was normal. Examination under anesthesia of the right eye revealed the elongated nasal ciliary processes with taut vitreous bands extending from the ciliary processes to the nasal retina near the ora serrata. The retina appeared to attach at the pars plicata and a small stretch retinal break was noted between retinal folds and taut vitreous bands nasally approximately 2 mm from the anterior retinal edge. As the posterior polar cataract and the vitreous stalk were mild and the vitreous was relatively clear, vitrectomy and lensectomy Supported by Research to Prevent Blindness departmental grant. Reprint requests: John O. Mason, III, MD, Assistant Professor of Ophthalmology and Vitreoretinal Surgery, University of Alabama at Birmingham School of Medicine, 700 South 18th Street, Suite 505, Birmingham, AL 35233; e-mail: lisa_mason@msn.com

3.

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5. 6. 7.

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used a scleral buckle procedure on only two cases. One case failed and the other case preoperatively had a traction RD and did not have a total RD or a rhegmatogenous component. Persistent fetal vasculature has a spectrum of presentation anteriorly and posteriorly. Elongation of the ciliary processes in combination with an abnormal vitreoretinal adhesion (taut bands) can lead not only to traction RD, but also to rhegmatogenous RD. Patients should be carefully examined for a retinal break. When a rhegmatogenous component is present in addition to vitreous traction, the rhegmatogenous component may be the primary cause of the detachment. In this type of scenario, a scleral buckle procedure alone can result in a surgical success by closing the retinal break and limiting the tractional forces on the retina in the far periphery.
Fig. 1. External photograph of the right eye shows lid capillary hemangiomas, very elongated nasal ciliary processes, posterior iris synechiae, and a posterior polar lens opacity; a light reex is present nasal to the lens opacity.

References
1. 2. Reese AB. Persistent hyperplastic primary vitreous. Am J Ophthalmol 1955;40:317331. Goldberg MF. Persistent fetal vasculature (PFV): an integrated interpretation of signs and symptoms associated with persistent hyperplastic primary vitreous (PHPV). Am J Ophthalmol 1997;124:587. Pollard ZF. Results of treatment of persistent hyperplastic primary vitreous. Ophthalmic Surg 1991;22:48 52. Mittra RA, Huynh LT, Ruttum MS, et al. Visual outcomes following lensectomy and vitrectomy for combined anterior and posterior persistent hyperplastic primary vitreous. Arch Ophthalmol 1998;116:1190 1194. Dass DA, Trese MT. Surgical results of persistent hyperplastic primary vitreous. Ophthalmology 1999;106:280 284. Pruett RC, Schepens CL. Posterior hyperplastic primary vitreous. Am J Ophthalmol 1970;69:534 543.

surgery was not performed. Instead, an encircling scleral buckle procedure using a wide band nasally was performed without complication and the retina was attached on postoperative day 1. Amblyopia therapy was initiated. Nine months postoperatively, the encircling band was segmented. One year postoperatively, the retina remains attached and the patient xes and follows.

3. 4.

Discussion Multiple surgical procedures have been advocated for management of PFV. Most earlier papers reported dismal results for surgical management of posterior PFV.1,3 Recently, improved vitrectomy techniques have improved the surgical success rates for posterior PFV or combined anterior/posterior PFV.4,5 This case differs from most cases of PFV by virtue of having a combined traction and rhegmatogenous RD without any posterior fundus vitreoretinal adhesions being present. It appears as though the elongation of the nasal ciliary processes (causing tractional folds of the nasal retina) and abnormal vitreoretinal attachments (taut bands) in the nasal periphery caused a stretch hole to develop, leading to a combined traction and rhegmatogenous RD. Pruett and Schepens have suggested that congenital vitreoretinal adhesions may cause rhegmatogenous RD.6 This case also differs by its surgical management. The majority of posterior segment PFV is managed via vitrectomy. This is primarily due to the prevalence of either posterior pole vitreoretinal membranes or stalk, dense cataract, or RD. Dass and Trese5 described surgical management of 27 cases of PFV and

5. 6.

CENTRAL RETINAL VEIN OCCLUSION ASSOCIATED WITH PRIMARY PULMONARY HYPERTENSION A. BHAN, FRCOPHTH, I.G. RENNIE, FRCOPHTH, T.W. HIGENBOTTAM, FRCP From Royal Hallamshire Hospital, Shefeld, United Kingdom. Primary pulmonary hypertension (PPH) is an unusual condition that has rarely been associated with ocular abnormalities.
Reprint requests: A. Bhan, FRCOphth, Department of Ophthalmology, Queens Medical Centre, Nottingham, NG7 2UH, UK; e-mail: abhan9@hotmail.com

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Fig. 1. Red-free anterior segment photographs of both eyes demonstrate episcleral injection.

Case Report
A 37-year-old woman presented to us with blurred vision in her left eye. She had developed this symptom 12 months previously. The onset was sudden and the resolution gradual. Following resolution she experienced uctuation of vision in that eye. Her medical history included right-sided refractive amblyopia and PPH. The latter had been present for over 18 months before this presentation, and her mean pulmonary arterial pressure was found to be 48 mmHg. The patient had undergone tests to exclude causes of secondary pulmonary hypertension including liver function tests, autoantibodies, human immunodeciency virus1 screening, electrocardiography, chest x-ray, pulmonary function tests, and a ventilation-perfusion scan. At the time of presentation, she was taking isosorbide mononitrate, diuretics, and warfarin. She was not taking oral contraceptives and there was no family history of note. Examination revealed her vision to be 6/60 in the right eye and 6/18 in the left, with best spectacle correction. Anterior segment

examination revealed marked episcleral vessel dilation in both eyes, normal and symmetric intraocular pressures, and no relative afferent pupillary defect (Figure 1). Fundal examination revealed signs in keeping with a left-sided central retinal vein occlusion: unilateral disk swelling, venous tortuosity, blot retinal hemorrhages, and macular edema (Figure 2). Her clotting factors, including protein C, protein S, factor V Leiden, and anticardiolipin antibodies, were normal.

Discussion Pulmonary hypertension is dened as a mean pulmonary arterial pressure of more than 25 mmHg at rest or more than 30 mmHg during exercise. Primary pulmonary hypertension is a rare condition, with an

Fig. 2. Red-free fundus photographs of the left eye show a central retinal vein occlusion (A), with the normal right eye shown for comparison (B). Fluorescein angiography of the left eye shows early phase masking by the hemorrhages of the central retinal vein occlusion (C) and the late phase shows dilation and tortuosity with staining of the venous walls and peripapillary leakage (D).

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incidence of 1 to 2 cases per million per year and a prevalence of 1300 per million. Its etiology is unknown although 10% of cases are familial and it has a predilection for young to middle-aged females. Current theories on pathogenesis focus on abnormalities in the interaction between endothelial and smooth muscle cells. Clinical features include those resulting from hypoxia and cor pulmonale. Typically, patients initially develop dyspnea, which they attribute to being unt. This worsens and they may develop chest pain from right heart ischemia and syncopal episodes before signs of right heart failure. The mainstay in the current treatment of severe PPH is chronic intravenous infusion of prostacyclin, which improves survival and quality of life.1 Earlier stages of the disease can be treated with warfarin anticoagulation. Some patients are given vasodilators. Such treatments have been shown to enhance survival. Right ventricular dysfunction is thought to be reversible if normal pulmonary artery pressures are restored. Heartlung transplantation is now undertaken for patients in whom medical treatments are unsuccessful. The prognosis for untreated PPH is poormean survival rates are 2 to 3 years from diagnosis. Primary pulmonary hypertension is infrequently associated with ocular abnormalities. Single case reports of patients with bilateral uveal effusions2 and exudative retinal detachment3 have been reported, as well as one case of a central retinal vein occlusion in conjunction with purpura and livedo reticularis.4 Eisenmengers syndrome (secondary pulmonary hypertension) has been associated with chronic ocular ischemia.5 We hypothesize that right-sided heart failure and raised venous pressure contribute to this condition. To our knowledge, this is the second report of this association.

NEOVASCULARIZATION ASSOCIATED WITH CYTOMEGALOVIRUS RETINITIS GEMINI J. BOGIE, MD, SUMIT K. NANDA, MD From the Department of Ophthalmology, Dean A. McGee Eye Institute, University of Oklahoma College of Medicine, Oklahoma City. Cytomegalovirus (CMV) retinitis is the most common cause of blindness in patients with acquired immunodeciency syndrome (AIDS). Retinal vascular inammation and attenuation are components of the retinitis.1 We report two cases of neovascularization occurring in patients with human immunodeciency virus (HIV) and CMV retinitis without other causes of retinal ischemia. A case of neovascularization associated with both CMV retinitis and cryptococcal choroiditis was reported in 1996.2 A case of optic disk neovascularization after branch retinal vein occlusion and branch retinal artery occlusion in association with HIV and CMV retinitis was also reported in 1996.3 Optic disk neovascularization was reported in a patient with inactive CMV retinitis without capillary nonperfusion and with immune recovery vitritis.4 Case 1
A 43-year-old man with AIDS was diagnosed with CMV retinitis when he experienced bilateral blurred vision and oaters. His medical history included HIV positivity for 6 years, oral candidiasis, Pneumocystis carinii pneumonia, AIDS-related anemia, and disseminated CMV infection. He had previously been treated with intravenous (IV) ganciclovir with a neutropenic response. Current medications included zidovudine, IV foscarnet, and trimethoprimsulfamethoxazole. His visual acuity (VA) was 20/80 in the right eye and 20/40 in the left eye. Anterior segment examinations were normal. Vitreous cell was present in both eyes. Fundus ndings included scattered intraretinal inltrates and retinal hemorrhages, as well as vascular attenuation (Figure 1A). At that time, therapy for CMV infection was changed from IV foscarnet to IV ganciclovir. One year after initial diagnosis of CMV retinitis, VA was hand motion in the right eye and 20/60 in the left eye. His CD4 count at that time was 10. His medical regimen included zidovudine, lamivudine, and maintenance IV ganciclovir. On the right, he had a prominent afferent pupillary defect and very hazy vitreous. Retinal pigmentary changes were present bilaterally without active retinitis. Four months later the patient returned with no light perception in the right eye and 20/70 VA in the left eye. His CD4 count had increased to 184. He was receiving both ganciclovir and foscarnet IV. Examination ndings included hazy vitreous on the right with optic nerve pallor and retinal pigmentary changes. Cellophane maculopathy, cystoid macular edema, retinal pigmentary changes, and neovascularReprint requests: Gemini J. Bogie, MD, 608 Stanton L. Young Blvd., Oklahoma City, OK 73104.

References
1. 2. Higenbottam T, Stenmark K, Simmoneau G. Treatments for severe pulmonary hypertension. Lancet 1999;353:338 340. Akduman L, Del Priore LV, Kaplan HJ, Meredith T. Uveal effusion syndrome associated with primary pulmonary hypertension and vomiting. Am J Ophthalmol 1996;121:578 580. Gislason I, Jonasson F, Stefansson E. Exudative retinal detachment in familial pulmonary hypertension. Acta Ophthalmol 1991;69:805 809. Berliner S, Shoenfeld Y, Dean H, et al. Primary pulmonary hypertension: a facet of a diffuse angiopathic process? Respiration 1982;43:76 79. Harino S, Motokura M, Nishikawa N, et al. Chronic ocular ischaemia associated with the Eisenmengers syndrome. Am J Ophthalmol 1994;117:302307.

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Fig. 1. Case 1. A, Hazy vitreous, scattered retinal inltrates, hemorrhages, cotton wool spots, and vascular attenuation at the time of diagnosis of cytomegalovirus (CMV) retinitis. B, Retinal pigmentary changes and vascular attenuation located inferotemporally, with a tuft of neovasculature along the inferotemporal arcade approximately 14 months after diagnosis of CMV retinitis. C, Fluorescein angiogram shows focal areas of capillary nonperfusion with early hyperuorescence along the inferotemporal arcade. D, Fluorescein angiogram shows progressive leakage along inferotemporal arcade in area corresponding to tuft of neovascularization seen clinically.

ization along the inferotemporal arcade (Figure 1B) were present in the left eye. Fluorescein angiography of the left eye conrmed focal areas of nonperfusion inferiorly, with early hyperuorescence and progressive leakage consistent with neovascularization (Figure 1, C and D). The patient was subsequently lost to follow-up.

Case 2
A 50-year-old man was diagnosed with CMV retinitis when he experienced blurred vision, oaters, redness, and photophobia of the right eye. His medical history included HIV positivity for 12 years and treated syphilis with a currently negative rapid plasma reagin status. His CD4 count was approximately 200. His medications included indinavir, lamivudine, dapsone, and stavudine. His VA was 20/250 in the right eye and 20/20 in the left eye. Intraocular pressure (IOP) was 31 on the right and 18 on the left. Signicant anterior and posterior cell was present in the right eye only. Right fundus ndings included frosted branch angiitis along the major vascular arcades and white intraretinal inltrates with retinal hemorrhages inferiorly (Figure 2A). The left fundus was

normal. The patients elevated IOP and uveitis were treated with topical medications. After induction therapy with IV ganciclovir, he continued to have IOP elevation, but resolving uveitis and retinitis. Retinal arterioles had sclerosed. A moderate amount of neovascularization of the iris was present with involvement of the trabecular meshwork inferiorly (Figure 2B). Panretinal photocoagulation was performed after uorescein angiography, which revealed delayed venous lling, diffuse capillary nonperfusion, and no early hyperuorescence or late leakage (Figure 2C). YAG cyclophotoablation was performed for continued elevated IOP despite maximal topical and oral medication. These therapies were successful in lowering the IOP and causing regression of the iris neovascularization. Approximately 5 months after diagnosis of rubeotic glaucoma, neovascularization of the disk was noted. Panretinal photocoagulation was repeated, with regression of the neovascularization within 3 weeks. The patient was eventually tapered off of the topical steroid, and his secondary cataract was extracted, resulting in a best-corrected VA of 20/80.

Fig. 2. Case 2. A, Hazy vitreous, frosted branch angiitis along major vascular arcades, and intraretinal inltrates and hemorrhages located inferiorly at the time of diagnosis of cytomegalovirus (CMV) retinitis. B, Neovascularization of the iris approximately 1 month after diagnosis of CMV retinitis. C, Diffuse capillary nonperfusion in areas of regressing retinitis approximately 1 month after diagnosis of CMV retinitis.

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Nine months after cataract extraction, the patient returned with profoundly reduced VA due to dense vitreous hemorrhage and recurrent rubeosis. He underwent pars plana vitrectomy with posterior membranectomy and endolaser. The postoperative course was complicated by hypotony, nonclearing vitreous hemorrhage, and posterior tractional retinal detachment by ultrasound examination. He underwent repeat vitrectomy with membranectomy and endolaser. His VA increased to his baseline of 20/80, and he is currently stable with an attached retina.

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References
1. 2. Hugh HL, Scott AA, Apple DJ. Cytomegalovirus retinitis. Surv Ophthalmol 1989;34:193203. Saran BR, Pomilla PV. Retinal vascular nonperfusion and retinal neovascularization as a consequence of cytomegalovirus retinitis and cryptococcal choroiditis. Retina 1996;16:510 512. Conway MD, Tong P, Olk RJ. Branch retinal artery occlusion combined with branch retinal vein occlusion and optic disk neovascularization associated with HIV and CMV retinitis. Int Ophthalmol 1996;19:249 252. Sanislo SR, Lowder CY, Kaiser PK. Optic nerve head neovascularization in a patient with inactive cytomegalovirus retinitis and immune recovery. Am J Ophthalmol 1998;126:318 320. Regan CD, Foster CS. Retinal vascular diseases: clinical presentation and diagnosis. Int Ophthalmol Clin 1986;26:2553. Newsome DA. Microvascular aspects of acquired immune deciency syndrome retinopathy. Am J Ophthalmol 1984;98: 590 601. Friedman SM, Margo CE. Bilateral central retinal vein occlusions in a patient with acquired immunodeciency syndrome. Arch Ophthalmol 1995;113:1184 1188.

3.

Discussion These cases show the development of retinal nonperfusion and neovascularization in two patients with CMV retinitis, HIV infection, and diminished immune status. It is presumed that the CMV retinitis caused the retinal nonperfusion and neovascularization because evidence of other possible causes was not found in previous laboratory studies and physical examinations. The retinitis resolved with anti-CMV therapy, providing additional evidence that CMV was the causative agent. Optic nerve head neovascularization has been reported in a patient with inactive CMV retinitis and immune recovery vitritis, without evidence of capillary nonperfusion on angiography.4 Our Case 1 did have a rise in CD4 count after combination antiretroviral therapy, but also had evidence of capillary nonperfusion. The immune recovery may have contributed to, but may not have been the sole cause of, the development of neovascularization. In Case 2, we cannot rule out the possibility of syphilitic chorioretinitis, which has been shown to cause vascular compromise.5 As in our cases, as the CMV retinitis resolves, atrophy of the retinal vasculature develops. This commonly occurs as a result of progressive occlusion from mononuclear cellular inltrates,1 and likely led to ischemia and subsequent neovascularization of the iris and retina in these two cases. Atrophic retinal vessels have been documented in prior cases of CMV retinitis with both uorescein angiography and direct histopathologic examination.6 It is possible that the obliterative effect on arterioles by CMV infection is potentiated by background HIV microangiopathy. Neovascularization has not been reported to result solely from the microvascular abnormalities seen in background HIV retinopathy.6 Central and branch retinal vein occlusion have been reported in patients with AIDS and no other known risk factors for venous occlusive disease.7 The etiology of these vasoocclusive changes in patients with HIV is unclear. These cases demonstrate that neovascularization and the complications associated with neovascularization are possible in the setting of CMV retinitis and HIV infection. Practitioners should be on guard for this nding.
4.

5. 6.

7.

RETINAL AND CHOROIDAL VASCULAR CHANGES IN HETEROZYGOUS FABRY DISEASE MARCOS A. DANTAS, MD, ROBERTO A. FONSECA, MD, TATSUSHI KAGA, MD, LAWRENCE A. YANNUZZI, MD, RICHARD F. SPAIDE, MD From the LuEsther T. Mertz Retina Research Center, Manhattan Eye, Ear, and Throat Hospital, and VitreousRetina-Macula Consultants of New York, New York. Fabry disease (angiokeratoma corporis diffusum universale) is an X-linked lysosomal storage disease resulting in defective activity of the enzyme lysosomal hydrolase -galactosidase A.1 This defect leads to a progressive accumulation of the glycosphingolipid trihexosylceramide, especially in the liver, heart, spleen, kidney, cornea, plasma, polymorphonuclear cells, vascular endothelial cells, and smooth muscle cells.1,2 The -galactosidase A locus has been mapped to Xq21.33. The diagnosis of the condition can be conrmed by the nding of decreased levels of -galactosidase A in the plasma or in polymorphonuclear leukocytes.2
The authors have no proprietary interest in any aspect of this article. Reprint requests: Richard F Spaide, MD, 519 East 72nd Street, Suite 203, New York, NY 10021.

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Fig. 1. Right eye. A, Fluorescein angiography shows areas of capillary nonperfusion (large dark arrow), capillary telangiectasias (small dark arrow), and microaneurysms (white arrow). B, Early phase uorescein angiography shows leakage of dye in the temporal macula. C, Late phase uorescein angiogram shows diffuse leakage of dye in the temporal macula. D, Indocyanine green angiography shows a moth-eaten appearance with areas of choroidal nonperfusion (large dark arrow), gaps in the continuity of vessels (small dark arrow), and looping of choroidal vessels (white arrow).

Patients with typical Fabry disease usually present with severe manifestations such as painful acroparesthesia, neurologic abnormalities, cardiovascular disease, heavy proteinuria, and characteristic cutaneous angiokeratomas.1,2 They demonstrate progressive impairment of the kidneys, heart, and central nervous system, and usually die by the fth decade of life.2 The ocular ndings include whorl-like corneal opacities (cornea verticillata), dilated aneurysmal conjunctival vessels (corkscrew-like appearance), retinal vessel tortuosity, granular anterior capsular or subcapsular lens deposits, posterior capsular cataracts, anterior ischemic optic neuropathy, and optic disk, retinal, and periorbital edema.1 The retinal involvement includes dilated veins, but arterial and venous occlusions have also been observed.2,3 We describe a female carrier with Fabry disease with a history of central retinal venous occlusion (CRVO) in one eye and unsuspected retinal microvascular and choroidal vascular abnormalities in the fellow eye. Case Report
A 66-year-old white woman was an enzymatically documented Fabry disease carrier with a history of mild episodes of pain and discomfort in the limbs (acroparesthesia) during childhood. Her medical history was negative for systemic arterial hypertension,

diabetes mellitus, systemic lupus erythematosus, past radiation treatment, or other diseases. Recent cardiac and renal evaluations were normal. She was asymptomatic in the right eye and had a history of a CRVO in the left eye 8 years previously, treated with panretinal photocoagulation. Her visual acuity was 20/25 in the right eye and 20/800 in the left eye. She was myopic, with 1.0 diopter in the right eye and 1.25 diopters in the left eye. Intraocular pressure was 13 mmHg in the right eye and 12 mmHg in the left. Slit-lamp examination revealed characteristic bilateral whorl-like corneal deposits. Tortuosity (corkscrew-like) was seen in the conjunctival vessels in both eyes. Lens opacities were not seen. Fundus examination of the right eye revealed telangiectatic vessels, occasional microaneurysms, and edema at the temporal macula. The left eye showed panretinal photocoagulation scars and evidence of old vitreous hemorrhages with resolving devitalized blood in the inferior vitreous. Fluorescein fundus angiography (FA) showed areas of nonperfusion, capillary telangiectasias, and occasional microaneurysms in the right eye (Figure 1A) and ndings of a past central vein occlusion in the left eye. Later in the course of the angiographic evaluation, there was diffuse leakage evident in the temporal macula of the right eye (Figure 1, B and C). An indocyanine green angiogram (ICG) was performed, and the choroid had a moth-eaten appearance with areas of nonperfusion, gaps in the continuity of the vessels, and looping of choroidal vessels (Figure 1D).

Discussion We describe a 66-year-old female carrier with Fabry disease who had a CRVO in one eye and

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unsuspected vascular abnormalities in the fellow eye: regions of capillary nonperfusion and telangiectasias, a few microaneurysms, and leakage of dye during FA, all signs of microvascular damage. During ICG, there was a moth-eaten appearance and suggestion of choroidal vascular perfusion abnormalities. Males with Fabry disease are at risk for vascular occlusion because they are hemizygous gene carriers.3 Of interest is that females, although they carry a normal X-chromosome, are also at risk for vascular occlusion, especially in the kidneys, central nervous system, heart, and extremities.2,3 The reason lies in the Lyon hypothesis: there is an inactivation of one of the X chromosomes in each cell of the female heterozygote.3 Inactivation of the normal X chromosome would leave the cell with only the production of abnormal -galactosidase with the eventual expression of the gene.3 Studies have been conducted to try to establish the molecular basis of the alterations that are responsible for the higher incidence of thrombotic events in patients with Fabry disease.4,5 The concentrations of soluble intercellular adhesion molecule1, vascular cell adhesion molecule1, P-selectin, plasminogen activator inhibitor, and platelet aggregation were significantly higher, and thrombomodulin was signicantly lower, in Fabry patients.4,5 Expression of the integrin CD11b on monocytes was also signicantly higher.4 All of these changes are consistent with a prothrombotic state and are indicative of endothelial cell leukocyte and platelet activation in these patients. Accumulation of glycosphingolipids in the vascular endothelial cells also may reduce the luminal diameter of the vessels, which may predispose to vascular occlusions as well.4 Although most patients with CRVO have no recognizable cause for developing venous thrombosis, the existence of a prothrombotic state in Fabry disease, and the absence of established CRVO risk factors in our patient (her age was 58 years when the event occurred), suggest the possibility that Fabry disease may be implicated in the occlusive event in her left eye.6 Whereas most reports on retinal changes in Fabry disease highlight the involvement of the larger vasculature, we show a case of retinal microvascular and choroidal vascular changes in an otherwise healthy woman with Fabry disease. The lack of choroidal and retinal pigment epithelial pigmentary changes, such as seen in Elschnig spots, may be due to the small size of the affected vessels, as well as compensatory remodeling of the surrounding vessels in the choroid. We could not attribute the choroidal changes to any other specic condition, such as past radiation therapy, and believe they may be related to Fabry disease. Ruling out other diseases that could be responsible

for some of the clinical ndings in this patient suggests that Fabry disease may affect the choroidal vasculature and the retinal microcirculation, in addition to the damage to the larger vessels described elsewhere.13 These ndings might occur in carriers who may present signs and symptoms usually expected to be found in hemizygotes. Further investigation of other patients with Fabry disease is needed to investigate the prevalence of microvascular disease. However, it is possible that Fabry disease may be considered in the differential diagnosis of diseases causing both large and small vessel disease in the fundus. References
1. 2. Sher NA, Letson RD, Desnick RJ. The ocular manifestations in Fabrys disease. Arch Ophthalmol 1979;97:671 676. Oto S, Kart H, Kadayifilar S, et al. Retinal vein occlusion in a woman with heterozygous Fabrys disease. Eur J Ophthalmol 1998;8:265267. Utsumi K, Yamamoto N, Kase R, et al. High incidence of thrombosis in Fabrys disease. Intern Med 1997;36:327329. Degraba T, Azhar S, Dignat-George F, et al. Prole of endothelial and leukocyte activation in Fabry patients. Ann Neurol 2000;47:229 233. Igarashi T, Sakuraba H, Suzuki Y. Activation of platelet function in Fabrys disease. Am J Hematol 1986;22:63 67. The Central Retinal Vein Occlusion Study Group. Natural history and clinical management of central retinal vein occlusion. Arch Ophthalmol 1997;115:486 491.

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A CASE OF ALPORTS SYNDROME AND RETINAL DEGENERATION EKATERINI TSILOU, MD, BENJAMIN I. RUBIN, MD, RAFAEL C. CARUSO, MD, MURIEL KAISERKUPFER, MD From the Ophthalmic Genetics and Visual Function Branch, National Eye Institute, National Institutes of Health, Bethesda, Maryland. Alports syndrome is the prototype of basement membrane disease characterized by a structural defect of the noncollagenous portion of type IV collagen. Eighty-ve percent of patients show X-linked inheritance but autosomal recessive and dominant forms also have been described. The X-linked and autosomal
The authors have no proprietary interests. Reprint requests: Ekaterini Tsilou, MD, Ophthalmic Genetics and Visual Function Branch, National Eye Institute, National Institutes of Health, 10 Center Drive, MSC-1860, Building 10, Room 10N226, Bethesda, MD 20892; e-mail: tsiloue@intra.nei.nih.gov

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Fig. 1. The posterior poles of the right fundus (A, B) and the left fundus (C, D) show evidence of punctate retinal pigment epithelial changes (arrows). A depigmented lesion below the macula (A) and an area of pigment accumulation below the inferior arcade (B) are evident in the right eye.

recessive forms have in common renal, audiologic, and ophthalmologic manifestations. Fleck and dot retinopathy, anterior lenticonus, and posterior polymorphous-like dystrophy of the cornea are the most common eye manifestations. Several other ocular manifestations have been described but not seen uniformly. Ocular abnormalities have not clearly been described in the rare autosomal dominant form. The purpose of this report is to present a patient with Alports syndrome and evidence of moderately severe retinal degeneration.

changed. Fluorescein angiography (FA) demonstrated bilateral multiple RPE window defects. Electroretinography (ERG) revealed a moderately severe reduction of rod responses (their amplitude was 20% and 22% of the median normal value in the right and left eye, respectively) and cone responses (35% and 31% of the median normal amplitude in the right and left eye, respectively) (Figure 2). Both a-wave and b-wave amplitudes were reduced to a similar degree, suggesting that the ERG abnormalities were due to a compromise of photoreceptor function. Electro-oculography (EOG) showed a decline in light peak amplitude with a similar degree of severity as that seen in the ERG (32% and 38% of the median normal amplitude in the right and left eye, respectively). In consequence, the Arden ratio was reduced (1.49 and 1.45 in the right and left eye, respectively).

Case Report
A 35-year-old man with Alports syndrome was initially seen in our eye clinic in April 1997. Family history included a sister who had died at age 33 years from renal failure secondary to Alports syndrome. Ophthalmic history revealed an amblyopic left eye since childhood and anterior lenticonus diagnosed at the age of 26 years, followed by bilateral cataract extraction. On examination, bestcorrected visual acuity was 20/25 in the right eye, 20/50 in the left eye. Slit-lamp biomicroscopy revealed multiple endothelial corneal opacities. Ophthalmoscopy revealed yellow punctate lesions in the retinal pigment epithelium (RPE) level of both eyes that spared the macula. Pigment accumulation was seen in both retinas inferiorly to the vascular arcades. Decreased foveal reex was noted in both maculae as well as patchy hypopigmentation of the RPE in the inferior portion of the right macula (Figure 1). The patient underwent a kidney transplant in July 1997. On follow-up examination approximately 2 years later, the ocular examination was un-

Discussion Electroretinography and EOG in Alports syndrome have not been studied extensively, with most of the reported cases being normal. In 1974, Hochgesand et al1 reported three patients with Alports syndrome with normal ERG and abnormal EOG. This led to the assumption that the origin of the retinopathy was in the pigment epithelium. In 1980, Perrin et al2 reported one patient with an abnormal ERG and EOG and normal FA without any further information. In 1980, Zylbermann et al3 reported two siblings with Alports syndrome having pigment epithelial changes, decreased ERG, and normal EOG. In 1988, Gelisken et

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Fig. 2. Rod responses (A), maximal responses (B), cone responses (C), and icker electroretinogram (D) of our patient compared with a normal control.

al4 reported one patient with Alports syndrome, salt and pepperlike retinopathy, diminished scotopic ERG, and an elevated threshold on dark adaptation, raising the question of a possible associated retinal degeneration. The EOG was normal. Setala et al5 reported a mother and a son with macular degeneration and decreased ERG. To our knowledge, our case is the rst reporting the details of an abnormal ERG and EOG. In addition, an abnormal FA demonstrated window defects in the RPE. In the remainder of the cited cases it was either the ERG or the EOG that was abnormal and the other parameter was either normal or not reported. Perrin et al2 briey mentioned that one of their patients had abnormal ERG and EOG without giving any further information or reaching any conclusions. Our patient showed equally diminished cone and rod responses as well as EOG changes, pointing to a retinal degeneration with generalized involvement of the photoreceptors. The photoreceptor degeneration

could be primary, but is more likely secondary to RPE degeneration as suggested by the FA. Alports syndrome is caused by a structural defect of collagen type IV that only manifests itself in the kidney, ear, and eye. Although the pathogenesis of Alports retinopathy remains speculative, collagen type IV is a constituent of all basement membranes, including Bruchs membrane, the basement membrane of the RPE. Long-term follow-up of our patient and other similar cases will be required to further investigate the etiology and natural history of this retinal degeneration.

References
1. Hochgesand P, Steinbach PO, Straub E. Augenveranderungen bei Alport-Syndrome. Klin Monatsbl Augenheilkd 1974;165: 447 452. Perrin D, Jungers P, Grunfeld JP, et al. Perimacular changes in Alports syndrome. Clin Nephrol 1980;13:163167. Zylbermann R, Silverstone BZ, Brandes E, Drukker A. Retinal

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lesions in Alports syndrome. J Pediatr Ophthalmol Strabismus 1980;17:255260. Gelisken O, Hendrikse F, Schroder C, Berden JHM. Retinal abnormalities in Alports syndrome. Acta Ophthalmol 1988; 66:713717. Setala K, Ruusuvaara P. Alport syndrome with hereditary macular degeneration. Acta Ophthalmol 1989;67:409 414.

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Correspondence
Early Versus Late Removal of Retained Intraocular Foreign Bodies Dear Editor: We appreciated the article on early versus late removal of retained intraocular foreign bodies (IOFB),1 especially concerning the suggested timing for surgical management of retained IOFB. Early removal by pars plana vitrectomy (PPV) reduces the risks of endophthalmitis and proliferative vitreoretinopathy (PVR) whereas late removal, despite the risk of endophthalmitis and PVR, offers a clean operating eld because of the developing posterior vitreous detachment and reabsorption of hemorrhages. Consequently, Jonas and Budde suggest operating as soon as possible. We found fault with their article because they did not focus their analysis on a single type of IOFB. In fact, nonmetallic IOFB are associated with a higher risk of endophthalmitis. Moreover, nonmetallic IOFB were found only in the group that underwent deferred PPV (two patients). Authors found a higher risk of endophthalmitis in patients with deferred PPV (P 0.05, chi square test) but did not consider this anomalous distribution of IOFB. Ozdamar et al2 show that silicone oil can reduce the risk of endophthalmitis, but no patient with this complication underwent silicone oil tamponade. The better visual outcomes found in the early removal group did not consider the central corneal laceration found especially in the deferred removal group. Lens cataract, although well distributed in the groups, had to be considered based on the presence of nuclear fragments in the posterior camber. Those fragments could inuence the higher incidence of PVR found in the deferred removal group. Following the study of Jonas and Budde, we studied the correct timing for removal of retained IOFB. From January 1996 to December 1998, we treated 273 patients with retained IOFB in our department. Retrospectively, we selected 29 patients (29 eyes) with scleral open-globe injuries due to metallic and magnetic IOFB embedded on the retina. In the rst group (17 eyes), IOFB were removed within 24 hours (mean

7 hours [SD 1.41]); in 5 eyes, IOFB were found within the temporal vascular branches (central IOFB). In the second group, composed of 12 eyes (4 with central IOFB), IOFB were removed after 24 hours (mean 5.75 days [SD 5.68]). There was no signicative difference between the study groups. All eyes underwent threeway PPV and were endotamponaded with silicone oil. We considered anatomic and functional ndings at admission and at 1 week; 1, 2, and 6 months; and 1 and 2 years postoperation. We considered the number of surgical procedures. All patients had retinal reattachment. Visual acuity did not vary signicantly between the groups during the follow-up (unpaired ttest, P 0.05).3 The number of operations was not signicantly distributed in the groups. Because of retinal side impact, visual function was seriously compromised.4 Proliferative vitreoretinopathy was wellcontrolled with silicone oil and PPV. We found no functional or anatomic differences among the groups, showing that early removal has the same prognostic results as deferred surgery. Tommaso Micelli Ferrari, MD Nicola Cardascia, MD Ilaria Di Gesu ` , MD Natalia Catella, MD Nicola Recchimurzo, MD Francesco Boscia, MD Department of Ophthalmology and Otorhinolaryngology University of Bari Bari, Italy

Reply Dear Editor: We appreciate the letter of Dr. Ferrari and colleagues, in which they describe their clinical experience with the timing of PPV to remove retained IOFB. We agree that the composition of the foreign body is one of the main risk factors for the development of posttraumatic infectious endophthalmitis. In our study,1 the IOFB was magnetic in all patients in the early intervention group and in 17 of the 19 patients (89.5%) in the late intervention group. This difference was not statistically signicant. Frequencies of postoperative infectious endophthalmitis and PVR were signicantly (P 0.05) higher in the late intervention group than in the early intervention group. In view of the limitations of the study pointed by Dr. Ferrari and colleagues, we continued the investigation. In a larger scaled follow-up study,5 the results concerning the risk factors for the development of postoperative infectious endophthalmitis were con-

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rmed. A total of 130 patients presenting consecutively with penetrating ocular injuries due to lacerations by foreign bodies were operated on by one of two surgeons between 1989 and 1997. Follow-up time was an average of 20.84 20.76 months. All foreign bodies were located posterior to the lens. Occurrence of posttraumatic infectious endophthalmitis, developing in seven patients, was signicantly (P 0.026) associated with removal of the foreign body later than 24 hours after the accident and with the type of foreign body (P 0.01). Size of the foreign body (P 0.37), preoperative visual acuity (P 0.62), presence of traumatic cataract (P 0.75) or a retinal lesion by the foreign body (P 0.16), age (P 0.39), and gender (P 0.46) did not show a statistically signicant inuence on the occurrence of endophthalmitis. Statistically signicant risk factors for the development of PVR, occurring in 27 patients (27/99 [27.6%] patients with a minimal follow-up of 3 months), were size of the foreign body (P 0.001), preoperative visual acuity (P 0.02), presence of a retinal lesion (P 0.002), and traumatic cataract (P 0.03). The time between foreign body removal and the accident was marginally associated with the development of PVR (P 0.07). It was concluded that prognosis in open-globe injuries with intraocular or retrobulbar foreign bodies depends on the size and type of the foreign body, presence and location of retinal lacerations, additional involvement of other intraocular structures, preoperative visual acuity, and timing of surgery. In conclusion, the larger-scaled follow-up study involving 130 patients conrms the previous studys nding that, in special clinical situations, early removal of a foreign body can be benecial in terms of reducing the risk of postoperative infectious endophthalmitis. The question remains whether early versus late removal of IOFB also reduces the risk of PVR. Jost B. Jonas Department of Ophthalmology Friedrich-Alexander-University Erlangen, Germany

4. Chiquet C, Zech JC, Denis P, Adeleine P, Trepsat C. Intraocular foreign bodies. Factors inuencing nal visual outcome. Acta Ophthalmol Scand 1999;77:321325. 5. Jonas JB, Knorr HLJ, Budde WM. Prognostic factors in ocular injuries due to intraocular or retrobulbar foreign bodies. Ophthalmology 2000;107:823 828.

Fungal Endophthalmitis After a Single Intravenous Administration of Presumably Contaminated Dextrose Infusion Fluid Dear Editor: We read Fungal Endophthalmitis After a Single Intravenous Administration of Presumably Contaminated Dextrose Infusion Fluid, by Gupta and coworkers, with interrest and concern.1 It is alarming that 11 of 72 factory-sealed bottles of 5% dextrose available to physicians in pharmacies in similar settings showed growth of fungus. We have also encountered three patients who had endogenous endophthalmitis following a history of intravenous (IV) infusions in a series of 14 patients with endogenous endophthalmitis (unpublished study). We have observed that such contamination occurs if the infusion bottle has a minor crack during transport. Such cracks may not be visible and may not leak. Thus, they can be overlooked by the practitioner during administration of IV infusion. Close inspection can identify such cracks in the bottle. Such cracks can lead to the loss of vacuum maintained in the bottle allowing the entry of organisms. Due to the threat of contaminated IV infusion, we have adopted three simple precautionary measures. 1) Each infusion bottle should be shaken and then viewed against a pencil of light e.g., a table lampto look for suspended impurities. We have seen such particles that subsequently microbiologically tested positive for fungus. This test appears to be relatively simple, but can detect gross contamination with fungus. 2) We give a rm thud on the bottom of the glass bottle containing IV infusion uid with the palm of the hand. In an intact bottle in which vacuum has been maintained, a dull thump sound is heard. Such sound is absent in bottles that have lost vacuum due to invisible cracks. 3) While introducing the needle into the bottle as an air vent, a gurgling sound is heard as air bubbles rush inside the bottle of infusion uid where vacuum is maintained. Cracks or leaks can lead to the loss of vacuum and such sound will be absent. This indicates that vacuum is lost and may indicate possible entry of organism. Such bottles should be discarded. We also believe that glass bottles, which are com-

References
1. Jonas JB, Budde WM. Early versus late removal of retained intraocular foreign bodies. Retina 1999;19:193197. 2. Ozdamar A, Aras C, Ozturk R, Akin E, Karacorlu M, Ercikan C. In vitro antimicrobial activity of silicone oil against endophthalmitis-causing agents. Retina 1999;19:122126. 3. Pavlovic S, Schmidt KG, Tomic Z, Dzinic M. Management of intraocular foreign bodies impacting or embedded in the retina. Aust NZJ Ophthalmol 1998;26:241246.

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monly used for IV infusion, even though of medical grade quality, have more risk of developing cracks. Plastic bottles for infusion can have a much lesser risk of development of such cracks or leaks leading to contamination and should be preferred. Such simple precautionary measures can greatly reduce the risk of infusion-related fungal infections including endogenous fungal endophthalmitis as reported by Gupta and coworkers in rural settings of developing countries.1 Jyotirmay Biswas, MD Medical and Research Vision Foundation Chennai, India

maximum value of 1 in 2500. These approximations suggest that the average prevalence of uveal melanoma among all living Americans is half of the rate at age 76; i.e., 1 in 5000. Given the additional assumption that the prevalence of each tumor is independent, the likelihood that a random American has had both retinoblastoma and uveal melanoma is: 1/15,000 1/5000 1.33 10 8 0.0000000133 Conversely, the likelihood that a random American has not suffered these tumors is 1 0.0000000133 0.9999999867. To determine the likelihood that no living American has had these tumors, we raise this fraction to the appropriate power (the approximate current population of the United States): 0.9999999867 263,000,000 0.03026 Thus, these calculations suggest that there is only approximately a 3% chance that no single living American has had both retinoblastoma and uveal melanoma. Even if our assumptions have introduced a 10-fold error, there is still greater than a 50% chance that both retinoblastoma and uveal melanoma have occurred in at least one American. Furthermore, such an American would almost certainly end up eventually at the Oncology Service of Wills Eye Hospital. We conclude that the occurrence of a single American with both retinoblastoma and uveal melanoma does not imply a biologic association between these tumors. John Gamel Professor of Ophthalmology University of Louisville Louisville, Kentucky

Reference
1. Gupta A, Gupta V, Dogra MR, et al. Fungal endophthalmitis after a single intravenous administration of presumably contaminated dextrose infusion uid. Retina 2000;20:262268.

Occurrence of Retinoblastoma and Uveal Melanoma in the Same Patient Dear Editor: We read with interest the article from Wills Eye Hospital in which the authors reported a patient who underwent enucleation of her right eye for retinoblastoma in childhood and then was diagnosed at the age of 47 years with uveal melanoma in her left eye.1 The authors stated that The probability of occurrence of unilateral retinoblastoma and then uveal melanoma in the remaining eye is estimated to be 1 in 165 billion. We submit an alternative approach to the likelihood of such an event. Our goal is to estimate the probability that a single person in America has experienced both retinoblastoma and uveal melanoma. To simplify our computation, we will ignore the impact of bilateral disease and of mortality from ocular tumors. First, the prevalence of retinoblastoma is approximately 1 in 15,000 live births. Second, and of greater complexity, is the prevalence of uveal melanoma. The lifetime prevalence of this disease is approximately 1 in 2500. Thus, if all living Americans were 76 years old (the average life expectancy at birth), approximately 1 in 2500 would have had uveal melanoma. To simplify, we will assume that the lifetimes of Americans are linearly distributed between age 0 and 76 years, and that over this range of years the prevalence increases linearly from a minimum value of 0 to a

Reply Dear Editor: We are grateful to Dr. Gamel for his interest and response to our article.1 Dr. Gamel has used different methodology, prevalence estimates, and assumptions in calculating the odds of retinoblastoma and uveal melanoma occurring in the same patient. Fortunately, he arrived at a similar conclusion that the occurrence of retinoblastoma and uveal melanoma in the same

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patient is a rare event with no suggestion for biologic association between these tumors. Arun D. Singh, MD Carol L. Shields, MD Jerry A. Shields, MD Oncology Service Wills Eye Hospital Thomas Jefferson University Philadelphia, Pennsylvania Paul Sternberg, Jr., MD Emory University Eye Center Atlanta, Georgia

Errata
Coats Response in Lebers Congenital Amaurosis Bhagat, Caputo, Pignato, and Zarbin reported a case of Coats response in Lebers congenital amaurosis (Retina 1999;19:356 359). Although the authors and reviewers believed that this was an original observation, a similar case has been reported by J. Donald M. Gass in Stereoscopic Atlas of Macular Diseases, 4th Edition. St. Louis: Mosby, 1997:362363. Multiple Recurrences of Exudative Retinal Detachment in a Patient with VogtKoyanagiHarada Disease In the article Multiple Recurrences of Exudative Retinal Detachment in a Patient with VogtKoyanagi Harada Disease (Retina 2000;20:672 674), the corresponding authors name was misspelled. The correct spelling is Debra A. Goldstein.

Reference
1. Singh AD, Shields CL, Shields JA, Sternberg Jr P. Occurrence of retinoblastoma and uveal melanoma in the same patient. Retina 2000;20:305306.