Ambati Naveen R, Hassan Muhammad, Sisk Robert A
University of Cincinnati, Department of Ophthalmology, 5110 231 Albert Sabin Way, 5th Floor, Cincinnati, OH, 45267-0527, USA.
Retina Division, Cincinnati Eye Institute, 9997 Carver Rd Level 2, Cincinnati, OH, 45242, USA.
Am J Ophthalmol Case Rep. 2025 Jul 22;39:102402. doi: 10.1016/j.ajoc.2025.102402. eCollection 2025 Sep.
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.
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.
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.
报告一例小儿患者，在全身性并发症消退数周后，出现双侧视网膜血栓性微血管病和牵拉性视网膜脱离，该患者患有典型的溶血尿毒综合征（HUS）。
一名23个月大的女性，有典型HUS病史，曾接受临时腹膜透析和依库珠单抗输血治疗，三个月后出现双侧累及黄斑的牵拉性视网膜脱离。尽管进行了仔细的双手剥离，但双眼先后采用玻璃体视网膜手术修复，仅成功复位了右眼的上方视网膜。她双眼的视力仍仅为光感。鉴于与晚期家族性渗出性玻璃体视网膜病变（FEVR）相似，对这一家三口进行了进一步检查。基因检测未确定明确病因。临床上，她的母亲有3区血管造影微血管改变和毛细血管缺失，符合1期FEVR。
我们报告了一例不寻常的小儿典型HUS患者，尽管接受了依库珠单抗治疗且全身性疾病已完全消退，但仍因牵拉性视网膜脱离而导致双眼失明。在继发于HUS的现有血栓性微血管病背景下，FEVR可能加剧了她视网膜疾病的严重程度。
