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Transient Open-Angle Glaucoma Associated with Sickle Cell Trait: Report of 4 Cases ALAN H

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Transient Open-Angle Glaucoma Associated with Sickle Cell Trait: Report of 4 Cases ALAN H Br J Ophthalmol: first published as 10.1136/bjo.63.12.832 on 1 December 1979. Downloaded from British Journal of Ophthalmology, 1979, 63, 832-836 Transient open-angle glaucoma associated with sickle cell trait: report of 4 cases ALAN H. FRIEDMAN,'2 BARTON L. HALPERN,' DOROTHY N. FRIEDBERG,2 FREDERICK M. WANG,2 AND STEVEN M. PODOS From the Departments of Ophthalmology of the 'Mount Sinai School of Medicine of the City University of New York and the 2Albert Einstein College ofMedicine, Bronx, New York SUMMARY Four black patients, all with sickle trait (SA), developed transient open-angle glaucoma with blood in Schlemm's canal. In 3 patients the condition followed blunt trauma, while in the fourth no antecedent trauma was described. The intraocular pressure became normal in all 4 cases with the resolution of the haemorrhage from the trabecular meshwork and Schlemm's canal. Sickle cell trait, the commonest of the sickle haemo- compound aspirin, phenacetin and caffeine tablets. globinopathies, affects approximately 9 % of the There was no history of diabetes mellitus, glaucoma, black population of North America.' A variety of ocular trauma, or other medical or ocular diseases. ocular abnormalities2-'2 have been reported to occur An ophthalmological examination 1 1 years pre- in patients with sickle cell trait. These have included viously showed a corrected visual acuity of 20/25 in proliferative retinopathy, chorioretinal scarring, each eye, applanation tension of 22 mmHg OU and copyright. spontaneous vitreous haemorrhages, central retinal a cup/disc ratio of025 in each eye. artery occlusion, angioid streaks, conjunctival Ophthalmological examination during the present vascular abnormalities, and transient open-angle illness revealed a corrected visual acuity of 20/30 glaucoma. We herewith report 4 additional cases of OU. Applanation pressures were 42 mmHg OD and transient open-angle glaucoma which occurred in 10 mmHg OS. Slit-lamp examination of the anterior patients with sickle trait. In 3 patients the glaucoma segment was normal in both eyes. Gonioscopic followed blunt trauma with microscopic hyphaema, examination of the anterior chamber angle showed a while in the fourth patient a microscopic haemor- grade III-IV open angle in both eyes, without rhage appeared in Schlemm's canal spontaneously. recession or neovascularisation. However, the right http://bjo.bmj.com/ All patients had blood in the trabecular meshwork eye showed blood present in Schlemm's canal or the canal of Schlemm on gonioscopy, and the (7.00 to 11.00 o'clock), and a haemorrhage localised glaucoma resolved concomitantly with clearing of to the trabecular meshwork from 7.00 to 8.00 blood from these areas. o'clock (Fig. 1). On ophthalmoscopic examination cup/disc ratios of 0 45 OD and 0-15 OS were seen on Case reports stereoscopic examination at the slit-lamp. Outflow facilities were 0-14 OD and 0-21 OS. Goldmann on October 2, 2021 by guest. Protected CASE 1 kinetic perimetry was normal in both eyes. Systemic A 53-year-old black woman had a history of 3 blood pressure was 190 mmHg systolic and 110 episodes of blurred vision in the right eye associated mmHg diastolic. with halos around lights for a 4-week period. These A complete blood count and sedimentation rate episodes lasted several hours each and were not were normal. Antinuclear antibody and latex accompanied by pain, injection, or other ocular fixation tests were negative. Haemoglobin electro- symptoms. The patient had hypertension of 8 years' phoresis revealed an AS pattern. The 3-hour oral duration, treated with methyldopa and a diuretic, and glucose tolerance test was abnormal: fasting blood rheumatoid arthritis for several years, treated with sugar 117 mg/100 ml (6-5 mmol/l); at 1 hour 231 mg/100 ml (12-8 mmol/l); at 2 hours 166 Requests for reprints to Sam Gartner, Library, Albert ml 88 ml Einstein College of Medicine/Montefiore Hospital and mg/100 (9-2 mmol/l); at 3 hours mg/100 Medical Center, IlI East 210th Street, Bronx, New York (4T9 mmol/p). 10467, USA. The patient was treated with acetazolamide 832 Br J Ophthalmol: first published as 10.1136/bjo.63.12.832 on 1 December 1979. Downloaded from Transient open-angle glaucoma associated with sickle cell trait: report of4 cases 833 Fig. 1 Goniophotograph ofcase 1 showing blood in the canal of Schlemm. copyright. Fig. 2 Goniophotograph ofcase 3 showing blood in the trabecular meshwork. http://bjo.bmj.com/ on October 2, 2021 by guest. Protected 250 mg orally 4 times a day and Epitrate (adrenaline immediately developed a reduction in vision followed acid tartrate) drops 2% OD twice a day. Over a several hours later by pain in the eye and vomiting. 2-week period the trabecular haemorrhage and Ocular examination showed the uncorrected visual blood in Schlemm's canal resolved. The intraocular acuity to be 20/20 OD and 20/200 OS. Applanation pressure remained normal after medication was pressures were 10 mmHg OD and 40 mmHg OS. discontinued and remained normal when the patient A superficial abrasion was present on the left upper was last examined 18 months later. eyelid. The left eye displayed moderateconjunctival hyperaemia and mild microcystic corneal epithelial CASE 2 oedema. The anterior chamber of the right eye was A 9-year-old boy was struck in the left eye with a clear while the left eye had 3 + flare and cells (many stick 24 hours before admission to hospital. He erythrocytes). No gross hyphaema was seen at the Br J Ophthalmol: first published as 10.1136/bjo.63.12.832 on 1 December 1979. Downloaded from 834 Alan H. Friedman, Barton L. Halpern, Dorothy N. Friedberg, Frederick M. Wang, and Steven M. Podos slit-lamp. The pupillary reflex was brisk OD and CASE 4 sluggish OS. The vitreous and fundus were unre- The patient was a 34-year-old black man who was markable. Gonioscopy of the left eye revealed blood punched in the left eye. When seen 2 days later the in Schlemm's canal. corrected visual acuity was 20/20 OD and 20/100 OS The patient was placed on bed rest, sedation, and Intraocular pressure was 16 mmHg OD and 36 acetazolamide 125 mg by mouth 4 times daily. mmHg OS. The right eye was entirely normal. The Examination next day showed an uncorrected visual left eye displayed 2 + bulbar hyperaemia and acuity in the left eye of 20/20 with intraocular moderate microcystic corneal oedema. The cornea tension 36 mmHg. Gonioscopy showed blood layered was cleared with topical glycerin. No keratic precipi- in the inferior portion of the anterior chamber angle tates were present. The anterior chamber revealed and prominently in Schlemm's canal in the remainder 2+ aqueous flare and cells, many of which were of the angle. A sickle preparation was positive, and erythrocytes. Gonioscopy showed a grade 3 open haemoglobin electrophoresis revealed an AS pattern angle, both eyes, and in the left eye a dense column (Hgb A 56 %, Hgb S 44 %). All other laboratory tests of dark red blood in Schlemm's canal. The pupillary including complete blood count, urine analysis, reactions were brisk OD and sluggish OS. The sequential multiple analyser (SMA) 6 and 12 were lens, vitreous, and fundus appeared normal. The normal. The haemorrhage cleared from the anterior patient was placed on acetazolamide 250 mg by chamber over the next 5 days with return of intra- mouth 4 times daily and prednisolone acetate 1 % ocular pressure to normal levels. The acetazolamide drops 4 times daily. A complete laboratory examina- was discontinued without sequelae. The patient was tion and haemoglobin electrophoresis revealed asymptomatic when examined 24 months later. AS pattern (Hgb A 59%, Hgb S 41 %). The intra- ocular tension was unchanged over the next 24 CASE 3 hours, and consequently the patient was given A 23-year-old black man was struck in the right eye. oral glycerol 1-5 mg per kg. After 2 hours, the He noticed a mild decrease in vision with pain in the intraocular pressure was 22 mgHg in the left eye. eye. He was in excellent health otherwise. Ophthal- Acetazolamide and glycerol were both required to mological evaluation showed a best visual acuity of control the intraocular tension for the ensuing 6 copyright. 20/50 OD and 20/20 OS. The left eye was completely days. Examination at that time showed an intra- normal. Applanation pressures were 30 mmHg ocular pressure of 24 mmHg and blood in Schlemm's OD and 14 mmHg OS. The right eye had 2 + bulbar canal. Glycerol was discontinued. Visual fields hyperaemia, clear cornea with very fine cellular utilising a tangent screen at 1 m with a 2 mm white deposits on the endothelium, 3 + aqueous flare, and target were full in both eyes. Tonography revealed a cells (mostly erythrocytes). Gonioscopy revealed a facility of outflow of 0-22 OD and 0 10 OS. During grade 3 open angle OU with a dark red band of the next 10 days the blood in Schlemm's canal was blood in the trabecular meshwork OD (Fig. 2). No gradually absorbed and the intraocular pressure was peripheral anterior synechiae or recession of the 16 mmHg. Tonography at that time was 0-20 OD and http://bjo.bmj.com/ anterior chamber angle were present. The pupillary 0-20 OS. Ocular examination 18 months later was reactions, lens, vitreous, and fundus were normal. entirely within normal limits. Tonography at this time revealed a facility of out- flow of 0-08 OD and 0-26 OS. The patient was put on Discussion acetazolamide 250 mg by mouth 4 times daily and prednisolone acetate 1 % eye drops 4 times daily.
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