Atypical haemolytic uremic syndrome presenting with posterior lenticonus like opacities: A rare association

Abstract

Hemolytic uremic syndrome (HUS) is one of the thrombotic microangiopathies characterized by intravascular hemolysis, thrombocytopenia, and end-organ damage. HUS is classified as typical (Shiga toxin producing Escherichia coli), atypical (uncontrolled complement activation), and secondary associated with other diseases. Ocular involvement is rare in atypical HUS (aHUS). Literature search using PubMed, Google scholar, Embase, etc., revealed very few case reports on ocular manifestations such as retinal detachment, putscher such as retinopathy, vitreous, and intraretinal hemorrhages, and optic atrophy that have been reported. Here, we report an atypical finding of posterior lenticonus and Grade I hypertensive retinopathy in a 13 years' boy diagnosed as aHUS with refractory hypertension who maintained stable vision after treatment with plasma exchange, mycophenolate mofetil, hemodialysis, and newer antihypertensive drugs (hydralazine).

Keywords

• atypical hemolytic uremic syndrome
• hypertensive retinopathy
• posterior lenticonus