ABSTRACT
PURPOSE: To report a patient with retinal and choroidal vascular occlusion as a presenting sign of sickle cell trait following the development of aqueous misdirection syndrome. METHODS: Retrospective chart review. RESULTS: A patient treated for bilateral chronic angle-closure glaucoma with sequential EX-PRESS glaucoma filtration device surgery developed sequential bilateral aqueous misdirection syndrome. The left eye developed retinal arterial and localized choroidal vascular occlusions subsequent to an acute elevation in intraocular pressure and possibly the use of oral acetazolamide. The patient was subsequently found to have sickle cell trait. The right eye developed aqueous misdirection with acute elevation of intraocular pressure as well, but the patient was not treated with oral acetazolamide and did not develop vascular occlusion. CONCLUSION: Retinal and choroidal vascular occlusions can be the presenting sign of a patient with sickle cell trait. Sickle cell screening may be beneficial in African American or Middle Eastern patients after an acute rise in intraocular pressure, particularly before initiation of treatment with oral carbonic anhydrase inhibitors.
Subject(s)
Aqueous Humor/metabolism , Choroid Diseases/etiology , Choroid/blood supply , Filtering Surgery/adverse effects , Intraocular Pressure/physiology , Retinal Artery Occlusion/etiology , Sickle Cell Trait/complications , Adolescent , Choroid/diagnostic imaging , Choroid Diseases/diagnosis , Female , Fluorescein Angiography/methods , Fundus Oculi , Glaucoma Drainage Implants/adverse effects , Glaucoma, Angle-Closure/physiopathology , Glaucoma, Angle-Closure/surgery , Humans , Postoperative Complications , Retinal Artery Occlusion/diagnosis , Retinal Vessels/pathology , Syndrome , Tomography, Optical Coherence/methods , Visual AcuityABSTRACT
Objective To assess the frequency of negative waveform electroretinograms (ERGs) in a tertiary referral center. Design Retrospective chart review. Participants All patients who had an ERG performed at the electrophysiology clinic at Emory University from January 1999 through March 2008 were included in the study. Methods Patients with b-wave amplitude ≤ a-wave amplitude during the dark-adapted bright flash recording, in at least one eye, were identified as having a "negative ERG". Clinical information, such as age, gender, symptoms, best corrected visual acuity, and diagnoses were recorded for these patients when available. Results A total of 1,837 patients underwent ERG testing during the study period. Of those, 73 patients had a negative ERG, for a frequency of 4.0%. Within the adult (≥ 18 years of age) and pediatric populations, the frequencies of a negative ERG were 2.5 and 7.2%, respectively. Among the 73 cases, negative ERGs were more common among male than female patients, 6.7% versus 1.8% (P < 0.0001). Negative ERGs were most common among male children and least common among female adults, 9.6% versus 1.1%, respectively, (P < 0.0001). Overall in this group of patients, the most common diagnoses associated with a negative ERG were congenital stationary night blindness (CSNB, n = 29) and X-linked retinoschisis (XLRS, n = 7). Conclusions The overall frequency of negative ERGs in this large retrospective review was 4.0%. Negative ERGs were most common among male children and least common among female adults. Despite the growing number of new diagnoses associated with negative ERGs, CSNB, and XLRS appear to be the most likely diagnoses for a pediatric patient who presents with a negative ERG.
Subject(s)
Dark Adaptation , Electroretinography/methods , Myopia/diagnosis , Night Blindness/diagnosis , Retina/physiopathology , Retinoschisis/diagnosis , Adult , Child , Child, Preschool , Diagnosis, Differential , Eye Diseases, Hereditary , Female , Follow-Up Studies , Genetic Diseases, X-Linked , Humans , Male , Myopia/physiopathology , Night Blindness/physiopathology , Retinoschisis/physiopathology , Retrospective Studies , Visual AcuityABSTRACT
A 73-year-old man presented with acute visual loss in his left eye associated with transient left upper extremity numbness. Diagnosis was made in the emergency room, where treatment strategies were discussed.
