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

摘要: Background Hemolytic Uremic Syndrome (HUS) is a rare disorder characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure, considered within group thrombocytic microangiopathies. Ocular complications in HUS are very rare. Here, we report an adult patient who suffered from onset paracentral scotoma, caused branch retinal artery occlusion (BRAO), as leading symptom atypical HUS. Case presentation A 39-year-old healthy male was lately diagnosed with essential hypertension mild impairment. He complained about central scotoma his left eye. Fundus examination revealed marked narrowing vessels, cotton wool spots few hemorrhages both eyes. The bilateral ischemic vasculopathy macular BRAO Workup worsening failure. Renal biopsy showed signs chronic thrombotic microangiopathy. (aHUS) started on plasmapheresis, together eculizumab. As condition continued to worsen, he put replacement therapy. Due persistent monoclone IgG1, underwent bone marrow which Monoclonal Gammopathy significance, triggering treatment initiated accordingly. Two months after initial presentation, developed neovascularization optic disc (NVD) eye, treated 3 monthly intravitreal bevacizumab injections complete regression NVD. myocardial infarction later course lost for follow-up. returned 11 last injection because sudden loss vision eye dense vitreous hemorrhage. Biomicroscopy new NVD right panretinal photocoagulation eyes neovascularization. Vision improved remained 20/20 Conclusion We present case showing ischemia can be linked aHUS. clinal diagnosis might challenging, physicians should aware ocular manifestations this devastating multi-organ disease. In ischemia, soon avoid blinding complications.