Sickle Cell Vaso-Occlusive Crisis Leading to Uteroplacental Malperfusion: A Case Report.

Abstract

Background Sickle cell disease (SCD) in pregnancy is associated with substantially increased risks of stillbirth and neonatal death due to vaso-occlusive crises, chronic anemia, and impaired placental perfusion. Case We present the case of a 25-year-old primigravida with HbSS disease who presented at 29 weeks' gestation with a vaso-occlusive pain crisis. Although she was initially clinically stable with reassuring fetal testing, she developed acute fetal distress on hospital day 3, prompting an emergent cesarean delivery. Intraoperatively, the uterus appeared profoundly hypoperfused with minimal bleeding despite uterine atony. The neonate was delivered in asystole and required prolonged resuscitation. Postresuscitation examination raised concern for severe hypoxic ischemic encephalopathy, and the parents elected to transition to comfort care. The neonate died shortly after withdrawal of life-sustaining interventions. Placental pathology demonstrated prominent sickled erythrocytes and increased fetal nucleated red blood cells, consistent with prolonged impaired oxygen delivery. Conclusion This case highlights the unpredictable and acute nature of fetal compromise in pregnancies complicated by SCD, which may occur prior to guideline-recommended antenatal surveillance. Although prophylactic transfusion remains controversial, emerging evidence suggests a potential benefit in selecting high-risk patients. Early multidisciplinary management, individualized transfusion strategies, and heightened vigilance for sudden fetal decompensation are essential to improving perinatal outcomes in SCD.