Lipemia retinalis as a presenting feature of hypertriglyceridemia associated with protease inhibitors in human immunodeficiency virus-infected patients.

PURPOSE: To report two cases of lipemia retinalis in patients infected with human immunodeficiency virus on protease inhibitors with increased triglyceride levels. METHODS: Retrospective review of medical records. Two patients infected with human immunodeficiency virus using protease inhibitors who were diagnosed with severe hypertriglyceridemia based on eye findings of lipemia retinalis. RESULTS: One patient developed triglyceride level of >5,300 mg/dL after adding an integrase inhibitor to a regimen that included protease inhibitors. Hypertriglyceridemia was diagnosed after routine ophthalmic screening for cytomegalovirus retinitis revealed lipemia retinalis. Triglyceride levels improved significantly after both discontinuation of HIV medications and adjustment of cholesterol-lowering medications. Another patient developed triglyceride levels of >9,000 mg/dL while on protease inhibitors, which was also detected on routine retinal examination. Shortly after the diagnosis, he experienced acute coronary syndrome and cardiac arrest requiring plasmapheresis and emergent bypass surgery. CONCLUSION: Lipemia retinalis is rare but may be a presenting sign of severe hypertriglyceridemia in patients infected with human immunodeficiency virus. Recognition of this condition during the ophthalmic examination can lead to diagnosis and may allow for treatment before the development of life-threatening complications, such as pancreatitis and acute coronary syndrome.

Rebound of cystoid macular edema with continued use of acetazolamide in patients with retinitis pigmentosa.

PURPOSE: To demonstrate the presence of a rebound effect with the use of acetazolamide for the treatment of cystoid macular edema (CME) in patients with retinitis pigmentosa (RP). METHODS: Six patients with RP and cystic-appearing lesions in the macula demonstrated by fluorescein angiography and/or optical coherence tomography (OCT) were treated with an oral form of carbonic anhydrase inhibitor (acetazolamide [500 mg]) as a single daily dose. RESULTS: All patients, treated with acetazolamide for a period of 3 weeks to 5 weeks, had initial improvement of macular edema demonstrated by OCT. However, extended use of acetazolamide, for at least 8 weeks to 12 weeks, resulted in recurrence (rebound) of CME in 3 of the 6 patients. CONCLUSIONS: Results from our study suggest that rebound of CME with the continued use of acetazolamide observed by OCT may occur more frequently than previously appreciated.

Correlation of optical coherence tomography findings with visual acuity and macular lesions in patients with X-linked retinoschisis.

PURPOSE: To evaluate and correlate findings obtained by optical coherence tomography (OCT) imaging with visual acuity (VA) and macular lesions in patients with X-linked retinoschisis (XLRS). DESIGN: Prospective comparative case series. PARTICIPANTS: Thirty-one patients with confirmed XLRS. METHODS: Best-corrected VA was determined using Early Treatment Diabetic Retinopathy Study charts, and a dilated funduscopic examination was performed on all patients. For all patients, the macula in each eye was OCT imaged. A correlation between VA, macular presentation, and OCT images was determined. MAIN OUTCOME MEASURES: Analysis of findings on OCT images, including foveal thickness and the area of macular cystic-appearing lesions, and their correlation with VA. RESULTS: Twenty-five patients with funduscopically evident cystic-appearing macular lesions demonstrated cysticlike spaces on OCT images. No statistically significant correlation was observed between the macular area of the cysticlike spaces, foveal thickness, and VA. Three patients with no funduscopic or OCT evidence of foveal cysts demonstrated thinning of the fovea on OCT images and more extensive reduction of their VA. Retinal papillomacular bundle nerve fiber layer thickness on OCT images showed no significant difference between control and patient groups. CONCLUSIONS: In XLRS patients with cystic-appearing macular lesions, there was a lack of correlation between VA, foveal thickness, and cystic area. The anatomical appearance of smaller perifoveal cysts on OCT imaging was most consistent with their location being primarily within the inner nuclear layer of the retina. In older patients, macular cysts were no longer apparent clinically or by OCT imaging, and foveal thickness was reduced. The findings on OCT images are consistent with the hypothesis of a primary Muller cell defect in patients with XLRS.

Monitoring cystoid macular edema by optical coherence tomography in patients with retinitis pigmentosa.

PURPOSE: To determine the value of optical coherence tomography (OCT) imaging in the diagnosis and monitoring of cystoid macular edema (CME) in patients with retinitis pigmentosa (RP). DESIGN: Prospective, noncomparative, small case series. PARTICIPANTS: Three patients with RP and cystic-appearing spaces in the macula on OCT images. INTERVENTION: All 3 patients were treated with a carbonic anhydrase inhibitor, and 1 also received topical and systemic steroids. MAIN OUTCOME MEASURES: Changes in OCT images, fluorescein angiography, and best-corrected visual acuity (VA). RESULTS: Although foveal cysticlike spaces were evident on OCT images in all 3 patients, only 1 patient showed CME on fluorescein angiography at baseline. Two of the 3 patients showed funduscopic evidence of macular cystic lesions, whereas a third showed no clinically evident fundus changes in the macula. Optical coherence tomography images documented improvement in the cystic-appearing spaces after treatment with the carbonic anhydrase inhibitor. Changes on fluorescein angiography were either not apparent or considerably less apparent. An improvement of > or =1 line on a Snellen acuity chart was recorded in 2 patients, whereas a third showed no change of VA in either eye. CONCLUSIONS: Optical coherence tomography is a potential method for the diagnosis and monitoring of CME in patients with RP. It was more sensitive in this regard than either fluorescein angiography or funduscopic examination.