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

Abstract

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

Transient vision loss associated with paracentral acute middle maculopathy detected on multi-modal imaging

“…PAMM is reported in association with hematologic diseases, such as hemolytic uremic syndrome and sickle cell disease which inherently yield an increased risk of systemic thrombosis. 18 , 19 We believe that this represents the first reported case of PAMM associated with iron deficiency anemia. It is possible that additional risk factors not elicited upon history were also present as a ‘second hit’.…”

Monoclonal gammopathies of clinical significance (MGCS): In pursuit of optimal treatment

“…(A-C) MGCS-associated kerathopathy with visible deposits on slit-lamp examination ( A , B (Image courtesy: Koo, et al., 2011) ( 182 ) and C (Image courtesy: Kocabeyoglu et al., 2014) ( 183 )); (D-G) MGC-associated maculopathy. (D) Neovascularization of the disc on the fundal examination (Image courtesy: Gonzales et al, 2021) ( 184 ); (E) Fluoroscopic angiography demonstrating telangiectasia of vessels and leakage from retinal capillaries (Image courtesy: Gonzales et al., 2021) ( 184 ); (F) Colored fundus examination showing optic disc pallor, attenuation of retinal vessels, and peripheral pigmentation (Image courtesy: Eton et al., 2020) ( 185 ).…”