Bilateral proliferative retinopathy and ischemic optic neuropathy in a patient with atypical hemolytic-uremic syndrome

Abstract

Rationale:To report a rare case of severe atypical hemolytic-uremic syndrome (HUS) in a patient who presented with vitreous hemorrhage and tractional retinal detachment (TRD) in both eyes. To our knowledge, this is the first reported case of atypical HUS complicated with bilateral TRD in the literature.Patient concerns:A 20-year-old man with atypical HUS demonstrated bilateral visual acuity of hand motion at 30 cm.Diagnoses:Dilated fundus examination revealed diffuse intraretinal hemorrhage with vascular engorgement, neovascularization of the disc, and neovascularization elsewhere bilaterally. Fluorescein angiography revealed bilateral proliferative retinopathy, retinal hemorrhage, and a large nonperfusion area with extensive neovascularization. Intravitreal antivascular endothelial growth factor (ranibizumab) injection was administered in both eyes, but his ophthalmic condition did not improve, and TRD developed bilaterally. Therefore atypical HUS complicated with bilateral TRD was diagnosed.Interventions:Pars plana vitrectomy was performed with panretinal photocoagulation and silicone oil tamponade in the right eye.Outcomes:After the pars plana vitrectomy of right eye, the retina was well-attached after surgery, but visual acuity remained poor. Visual evoked potential examination showed poor waveforms bilaterally, which suggested ischemic optic neuropathy.Lessons:Atypical HUS can cause systemic thrombotic microangiopathy, resulting in ischemic retinal changes. These ischemic retinal changes can then cause hypoxia, which triggers production of angiogenic factors and subsequently causes retinal vascular hyperpermeability, retinal and vitreous neovascularization, fibrovascular proliferation, vitreous hemorrhage, and TRD, in a manner similar to that of other ischemia-induced proliferative retinopathies. Despite successful surgery in the right eye, our patient's visual acuity did not improve, possibly because of severe and generalized ischemia of intraocular tissue, which resulted in ischemic optic neuropathy.

Occlusive retinal vasculopathy with macular branch retinal artery occlusion as a leading sign of atypical hemolytic uremic syndrome – a case report

“…In the left eye, NVD was noted only 4 months after the diagnosis of aHUS was established. Similarly, Lin et al described a case of aHUS with severe capillary nonperfusion who developed bilateral proliferative retinopathy and ischemic optic neuropathy as soon as 2 months after diagnosis of aHUS [12]. As in our case, the patient was initially treated with an intravitreal anti-VEGF injection.…”

“…The different retinal manifestations described in the literature all result from occlusive (i.e. thrombotic) microangiopathy [ 10 – 12 , 16 ]. In our case, FA showed macular and peripheral ischemia with mild leakage from small retinal vessels.…”

“…Ocular complications in typical HUS are very rare and have been described in 4% of pediatric cases [7,8]. The prevalence is lower with aHUS, with only few cases reported in the literature, including Purtscher-like retinopathy [9][10][11], occlusive retinopathy [12] and hypertensive choroidopathy [13].…”

Thrombocytopenia and Retina

“…Literatürde daha çok olgu sunumu şeklinde yayınlar mevcuttur. [26][27][28][29] Retina tutulumu bazı olgularda hafif, bazı olgularda ciddi seyredebilir. Retina bulguları olarak iskemi, neovaskülarizasyon, kanama, TRD, seröz RD ve Purtscher benzeri lezyonlar dikkati çekmektedir.…”

“…Retina bulguları olarak iskemi, neovaskülarizasyon, kanama, TRD, seröz RD ve Purtscher benzeri lezyonlar dikkati çekmektedir. [26][27][28][29] Sistemik tedaviler ve retina patolojisine yönelik girişimler bulguların düzelmesine katkı sağlamaktadır.…”