Bilateral tractional retinal detachments complicating hemolytic-uremic syndrome

Purpose

To present a case of bilateral retinal thrombotic microangiopathy and tractional retinal detachments in a pediatric patient with typical hemolytic uremic syndrome (HUS) weeks after resolution of systemic complications.

Observations

A 23-month-old female with a history of typical HUS requiring treatment with temporary peritoneal dialysis and eculizumab transfusions presents three months later with bilateral fovea involving tractional retinal detachments. Sequential surgical repair of both eyes with pars plana vitrectomy was only successful in reattaching the superior retina of the right eye despite careful bimanual dissection. Her vision remained bare light perception in both eyes. Given similarities to advanced familial exudative vitreoretinopathy (FEVR), the trio was examined further. Genetic testing did not identify a definitive cause. Clinically, her mother had zone 3 angiographic microvascular alterations and capillary dropout consistent with stage 1 FEVR.

Conclusions and importance

We report an unusual case of a pediatric patient with typical HUS who developed bilateral blindness from tractional retinal detachments despite treatment with eculizumab and complete resolution of systemic disease. FEVR may have contributed to the severity of her retinal disease in the setting of existing thrombotic microangiopathy secondary to HUS.