hydrophilic acrylic intraocular lens was implanted in the capsular bag. The early postoperative period was uneventful, and the patient achieved a best-corrected vistal acuity of 20/ 30, Within the next 2 months, there developed an excessive capsular bag librosis with mild upward decentra- tion of the intraocular lens, Comment. Although hyphemais one fof the most common eaely postop: ‘erative complications following ts beculectomy, to our knowledge, talenticula collection of blood has not been previously reported. B ‘cause ofthe tse of an operating mi ‘eroscope and the refinement of sir ical techniques, lens injury during trabeculectomy has been infre- ‘quently reported.’ We hypothesized that theze had been anterior capsule injury while performing peripheral inidectomy’ inthis eas, with seep- age of blood into the capsular bag The development of a fibrous \ype of posterior capsule opaciica- tion in relation to the presence of blood inthe eapsularbag, as was ev dent from the exaggerated postop- ‘erative capsular bag libros i this ‘ease, hasbeen previously noted “This ‘ase highlights the possibility of lens injury during trabeculectomy and provides an insight into the prob- lems encountered while performing phacoetmulsiiation when there isin- tralenticula blood collection G.S. Brar, MS Jeagat Ream, MS JJaspreet Singh, MS Ravinder Kaur, MS “Amod Gupta, MS The authors have no relevant finan- cial interest in this article Correspondence: Dr Brar, Depart- ment of Ophthalmology, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India Cevepgi@satyam.net.in). 1 Ends Tampon Sa ‘Watson PG Jakeman 0 culestony (220 yer flown) Ee 195 4 Ailey, Seton RD. Tete MR. Pot ‘spent Sere Opti 193 (©2004 American Medi Downloaded From: on 11/20/2018 Episodic Elevations Elevated intraocular pressure (JOP) associated with blood in the Schlemm canal may be associated with many different conditions." To our knowledge, we describe a pre- viously unreported case of a pa- tient with recurrent, intermittent spikes in IOP associated with epi- sodic and transient blood in the Schlemm canal. Whenever the pa- Uient was examined because of el evaied LOP in one or both eyes, go- nioscopy disclosed the presence of blood in varying quadrants of the Schlemm canal inthe affected eye(s) Whenever lOP was normalized, with, or without medical therapy, the blood was no longer visualized. Report of a Case. A 38-year-old woman of Latin American descent souight medical care because of right ocular pain, redness, and de- creased visual acuity of approx mately 12 hours’ duration. The pain was not relieved by ibuprofen. The patient reported a history of simi- lar ocular episodes that had oc- curred intermittently during the pre ceding. was significant for systemic hype tension and left elbow surgery; her blood pressure was 160/00 mm Hg, a the time of examination. There Goniscopc vw ote angles of the righty. Blood can besten in th Shem canal. was no significant ocular, family, oF social history On initial examination, visual culties were 20/40 OD and 20/25 (0, witha midl-dilated, mitimallyre- active pupilin the left eye. We found no proptosis, orbital bruits, or pul- ssating exophthalmos. The right con- juunetival region had a mild red ap- pearance, and mild corneal edema for haze was noted in the right eye. There was no apparent dilation ofthe cepiscleal veins. The lefLanteriorseg- ment appeared normal, Goldmann applanation tonometry measur ments were 55 mm Hg OD and 17 mm Hg OS, In the right eye, goni- ‘oscopy showed open angles with blood in the Schlemm eatal infer orly, temporally, and superiorly (Figure). Gonioseopic findings of the left angle structures were unre- markable. Dilated funduscopic ex- amination of the right eye showed mild vascular tortuosity without leakage on fluorescein angiogra- phy. Ophthalimodynomometry mes surements were 70 mm Hg OD and 60 mm Hg OS (both within normal limits). The elevated 1OP was ini- tilly treated with an intensive course of topical medications and oral agents (eg, acetazolamide and alycerin). Once the IOP was nor- malized, the patient was prescribed fan antiglaucoma regimen of timo- lol maleate, latanoprost, and dor- zolamide hydrochloride. During the next 5 months, the patient experienced recurrent, in- Association, Al rights reserved.termittent episodes of increased 1OP in either or both eyes. These 1OP spikes were always associated with the gonioscopic presence of blood in varying quadrants of the Schlemm ‘canal. Whenever 1OP was normal- ized in the affected eye(s), with or ‘without medical therapy, the blood ‘was no longer visualized. The pa tient also experienced 2 mild epi- sodes of white-colored cells and flare noted in the anterior chamber, both resolving within a few days. At any of the visits, no signs of hyphema, cither on sitlamp or gonioscapic ex- amination, were noted, ‘A computed tomographic sean of the head (with contrast) showed that both superior ophthalmic veins were normal; no mass effect was noted. An open magnetic resonance imaging sean (with gadolinium en- hancement) was also read as within normal limits; the cavernous sinus and ophthalmic veins were unre- markable. Automated visual fields (created with the 24-2 SITA pro- ‘gram; Humphrey Instruments, Ine, Dublin, Calif) were normal in both ‘eyes. Measurement of the episcleral venous pressure was within normal limits in both eyes, Doppler imaging of the jugular, subclavian, and up- per extremity Venoussystem showed normal values for compression and velocity. Extensive laboratory evalu- ation results (including a full hy- percoagulable workup), as well as Huorescent treponemal antibody absorption test results, erythrocyte sedimentation rate, and beta human ‘chorionic gonadotropin, rapid plasma reagin, antinuclear antibody, serum angiotensin-converting enzyme,and Lymeantibody titers, wereall within normal limits, The chest x-ray flan ‘was also within normal limits. OF note, the patient was found to have a positive sickle cell trait on hemoglo- bin electrophoresis (HbAS trait; such individuals have 1 copy of the nor- mal hemoglobin A gene and 1 copy ofthe hemoglobin S gene) Comment. In our patient, the dil ferential diagnosis included in- cereased episcleral venous pressure duc to Sturge-Weber syndrome, du ral cavernous sinus fistula, carotid- cavernous sinus fistula, superior vena cava syndrome, orbital arterio- venous fistula, jugular vein obstruc- (©2004 American Med Downloaded From: on 11/20/2018 tion, thyroid ophthalmopathy, and ‘idiopathic familial entities.” Other causes of blood in the Schlemm ca- nal include artifact due to the com- pression of epscleral veins withthe Goldmann or Allen-Thorpe gonio- Jens, ocular hypotony (following in- jury, inflammation, oF eyclodial sis), and pathologie deep arterial anastomosis from a deep limbal artery tothe Schlemm canal. 2 Dur- ing our extensive diagnostic eval. ation (including normal values on episcleral venous pressure testing), these diagnoses were eventually excluded ‘Asystematic review of the It crature demonstrated one previous case report’ of a patient with HbAS trait who was fist seen becatse of a single episode of elevated IOP in fone eye associated with blood in the Schlemm canal. However, ou pa- tient had multiple intermittent epi sodes of elevated 1OP associated with transient episodes of blood in the Schlemm canal, which appeared in either eye. Another recent case re- port! described a patient with a 5-year history of uvetis-glaucoma- hyphema syndrome who was diag- nosed as having HbAS trait on the basis of eytopathologic examina- tion findings ofthe submitted aq ous fluid during posterior-cham- ber intraocula-lens explanation and anterior-chamber washoit ‘While patients with sickle cell disease (either hemoglobin SS [Hbss} individuals having 2 cop- {es of the hemoglobin S gene] oF HSC [individuals having 1 copy of the hemoglobin S gene and | copy ofthe hemoglobin C gene]) are well known to have systemic and ocular vaso-oechusive episodes, little is known about the extent of in- czeased coagulation activity in indie Viluals with HBAS teait Although HbAS trait disorders are usually asymptomatic in nature, we theo- nize that in our patient, the HbAS trait caused localized sickling of the red blood cells (with subsequent sludging) in the Seblemm canal. In support of this theory, 4 recent study reports that individtals with HbAS trait have increased coagula- tion activity although itis lower than in patients with HBSC oF HBSS disease). Moreover, Goldberg” has reported that patents with sickle cell hhemoglobinopathies (including AS) have a higher percentage of sickled ‘erythrocytes in theiranterior cham- bers than in their circulating nous blood. Injection of sickle cell erythrocytes (AS, $8, SC, and Sthal [consisting of 1 copy of the hemo- alobin S gene and I copy ofthe hi moglobin B-thalassemia gene]) into the anterior chambers of living hu- man, monkey, or guinea pig eyes re- ‘sultsin sickling of the red blood cells ‘with resultant 1OP elevation.® Fur. thermore, a vicious cycle of hy- poxiaand acidosisare known to con- tribute to increased sickling of the erythrocytes.” In our patient, we sug ‘gest that intermittent episodes of sickled red blood cells in the trabec- ular meshwork (as a result of the HBAS trait) contributed to her tran- sient elevations in LOP. Robyn E. Horowitz, MD. ‘Max Forbes, MD Steven M, Podos, MD James C. Tsai, MD The authors have no relevant finan- ial interest in this article This study was supported in part by the Homer McK, Rees Scholar Award, New York, NY (Dr Tsai); the Eye Surgery Fund (Dr Tsai), New York; and unrestricted departmental _grants from Research to Prevent Blind ness, Inc, New York This study was presented in part ‘at the Annual Meeting of the Associa tion for Research in Vision and Oph- thalmology; May §, 2002; Fort Law derdale, Fla Correspondence: Dr Tsai, Hark ness Eye Institute, Columbia Univer- sity College of Physicians and Sur- _geons, 635 W 163th St, New York, NY 10032 (jet2002@columbia edu). 1. Phelps, Anll F,Weisman RL Poo SM, ST pin 3 ‘Sted epee venous prure) Claacona mucened wih seca eper lee Brjophhaiet 179 e3ee ‘Shana ar Pie Semeur Syed A Amun eof vee facoma bps peta ata 20715 55. alle Am Honatel 200299388. Association, Al rights reserved.Coats Disease and VATER Coats disease is an uncommon ext dative retinopathy of unknown ort- ‘gin that may cause blindness, We re- port the unusual occurrence of Coats disease in a5-yeat-old boy with mul- tiple congenital abnormalities die to VATER association. The possibility ofa genetic basis for some eases of Coats disease ts discussed, The VATER association com- prises congenital defects including vertebral defects, imperforate anus, tracheoesophageal fistula, and ra. dial and renal dysplasia. The unde lying cause of VATER association may bea severe embryonic insult dur- ing the simultaneous development of the organ systems.‘ Patients with VATER association often require multiple surgical procedures and ex- tensive rehabilitation. We report a unique case of exudative retinopa- thy (Coats disease) in a case of VATER association, A Scout fi ofa computed tomo photograph shoving severe exudate (©2004 American Medi Downloaded From: on 11/20/2018 Reportof'aCase. A child with anor- ‘mal prenatal ultrasound at 20 weeks gestation and a negative TORCH (toxoplasmosis, other infections, ru- bella, cytomegalovirus infection, and herpes simplex) panel was born af- teran otherwise uncomplicated pre nancy by normal spontaneous vagi- nal delivery at 33 weeks gestational age. The results of physical exami ‘nation at birth revealed an imperfo- rateanusand polydactyly withan ex tra digit lateral to the right thumb, The results of a radiological examai- nation showed communicating by drocephalus with grade It bilateral renal reflux and hemivertebra with, severe scoliosis (Figure 1A). Cor- rective surgical procedures in- cluded digit amputation, colos- tomy, and anoplasty, There was no family history of eye or genetic de- fects, and karyotyping was normal {in the child and his parents. ALS years of age, the patient was referred for left eye leukocoria, Visual acuity was 20/20 OD and 1/60 OS, There was total exudat nal detachment with associated tor- twious vessels with multiple aneu- rysinal dilatations compatible with, Coats disease, stage 3B (Figure 1B) The lower 2 quadrants of the retina were preferentially affected. There was no evidence of associated ante- rior chamber or vitreal inflamins lion. The findings of the retinal ex- amination of the fellow eye including the periphery area, were normal, Further examination of old photographs showed the patient had. had leukocoria since the age of 6 months, The results of B-scan ultra- sonography revealed retinal detach- ment with no caleification of mass. This was confirmed by a computed tomography scan of the orbits. In- traocular pressure was normal with no evidence of neovascularization of the iis. Cryotherapy was offered to the patient to preserve vision and prevent further complications, in- cluding neovascular glaucoma, painful blind eye, anda possible need for enucleation, The parents finally ‘opted for conservative treatment. Comment, A number of patients with VATER association have been described ashaving eye defects." The ‘most common associations are colo- rnd microphthalmos.” How- cever, the retina and posterior seg- mentareseldom involved, and Coats disease was hitherto unreported. The term Coats disease refers to idio- pathic retinal telangiectasia with in- Uraretinal orsubretinal exudation and without appreciable signs of retinal ‘or viteal traction. The diagnosis of Coatsdisease isoneof exclusion alt careful workup and examin pecially with regatd to retinoblas- oma, retinopathy of prematurity retinal capillary angiomatosis, and toxocara, all of which were not im- plicated in our case. Reported asso- ‘ations include renal-retinal abnor- oma malities and retinal disorders." Familial forms exist, but no spe- cific gene defect is known. In the largest reported series, the median hy san shoving absethemivarbras(aows) and severe scolass typi of VATE association. , Inder unduscopy inopathy wah inal detachment Association, Al rights reserved.