Case of the Month I June 2019

Case of the Month

June 25, 2019

The Case

The patient was a previously healthy 13-year-old girl who presented with a history of decreasing vision for one week associated with blind spots in each eye. She had no other ocular or systemic complaints. The visual acuity was 20/400 OD and count fingers at 4 feet OS. The anterior segment examination was unremarkable. The vitreous was clear, there was mild disc edema, and there were cotton-wool spots, several intraretinal hemorrhages, and cystoid edema in each macula. There was deep yellowish discoloration in the temporal mid-periphery of the left eye, as can be seen on the fundus photo. Extensive laboratory work-up included an unremarkable CT of the orbits and CBC. ANA and FTA-Abs were negative. Chemistry studies included an elevated creatinine of 2.06 (normal 0.50-1.20), normal BUN, low protein at 4.9 (normal 6.0-8.5), normal liver function tests, and normal amylase, lipase, angiotensin converting enzyme, and lysozyme. The OCTs revealed dramatic cystoid edema. The fluorescein angiogram showed extensive capillary leakage in both maculas as well as capillary nonperfusion in the posterior poles and choroidal nonperfusion in the temporal mid-periphery of the left eye. What are diagnostic considerations? What crucial piece of information has not been yet provided?

The patient had a clinical presentation that resembled Purtcher's retinopathy, which features multiple cotton-wool spots. Purtcher's retinopathy is most commonly seen in pancreatitis, long-bone fractures, or severe blunt chest trauma. Disc edema and cystoid macular edema are atypical for this condition. Inflammatory retinitis, such as lupus erythematosus, can result in vascular occlusions with cotton-wool spots and edema.

The missing piece of information was the blood pressure, which was 200/110. The patient had hypertensive retinopathy, and underlying renal disease was suspected. She was started on prednisone and anti-hypertensive therapy. A kidney biopsy showed IgA nephropathy, also known as Berger's disease. The blood pressure quickly normalized and the vision improved rapidly. When last seen, the visual acuity was 20/30-1 OD and 20/25-1 OS. Unfortunately, the acute insult resulted in renal failure, and she had a kidney transplant 7 months after her initial presentation. When last seen, she was on CellCept and prednisone 10 mg every other day.

Berger's disease is an autoimmune condition and is the most common cause of glomerulonephritis in the world and results in mild or severe renal disease. Treatment can include immunosuppression, plasmapheresis, and hemodialysis.

Reference

Taban A, Chand D, Sears JE. Ocular findings in IgA nephropathy with renal failure and hypertension. J Petiatr Ophthalmol Strabismus 2006;43:378-380.