Delayed visual recovery in pregnancy-associated thrombotic thrombocytopenic purpura with bilateral serous retinal detachment

Abstract

To present a pregnancy-associated thrombotic thrombocytopenic purpura (TTP) patient with bilateral serous retinal detachment (SRD). Case report. A 28-year-old nulliparous woman with 31 weeks gestation was presented to the local hospital with preeclampsia, hemolytic anemia, thrombocytopenia and bilateral blurry vision. Funduscopic examination showed bilateral macular SRD. Within the first month after delivery of a live female baby via cesarean section (at 32 weeks gestation), the patient experienced a recurrent course of hemolytic anemia and thrombocytopenia, and was then transferred to our hospital. On admission, her best corrected visual acuity (BCVA) was 0.1 OU; optical coherence tomography (OCT) confirmed the presence of bilateral macular SRD; electroretinography (ERG) examination showed diminished rod responses with reduced a and b waves in cone and mixed rod-cone responses. She was ultimately diagnosed with TTP and was treated systemically with fresh frozen plasma, rituximab, prednisone and cyclophosphamide. Despite persistent visual disturbances, she was discharged 1 month after admission with stabilization of systemic manifestations. At her first follow-up visit 6 months after discharge, surprisingly, her BCVA had improved to 1.0 OU; fundus examination and OCT confirmed the complete resolution of bilateral macular SRD and ERG revealed subnormal (right) and normal (left) electrophysiological responses. We believe that in this case, the clinical context (pregnancy) in which TTP developed, the unreported ERG characteristics and the unexpected delayed visual recovery are worth reporting. TTP should be considered as a potential differential diagnosis in patients with pregnancy-associated SRD. Appropriate systemic treatment might be mandatory for visual recovery.