From Discovery to Delivery: Anesthetic Strategy in a Bernard–Soulier Variant 

Coagulopathy in pregnancy increases maternal and fetal morbidity and mortality¹. Affected patients are at higher risk for postpartum hemorrhage, intrauterine bleeding, and transfusion². From an anesthetic perspective, coagulopathy limits neuraxial techniques, complicates labor analgesia and operative planning, and requires multidisciplinary coordination. Rare inherited platelet disorders, such as Bernard–Soulier variants, present unique peripartum challenges due to limited evidence guiding anesthetic management. 

A 31-year-old primigravida with suspected Bernard–Soulier–variant platelet disorder presented at 39 weeks and 3 days for delivery planning. A platelet abnormality had been identified years earlier before lumbar surgery, but the diagnosis was incompletely characterized. During the second trimester, hematology evaluation demonstrated reduced ristocetin-induced platelet aggregation and decreased CD42a/CD42b expression on flow cytometry, consistent with a Bernard–Soulier–like phenotype. She reported heavy menstrual bleeding, and IVF-related preconception testing ruled out common BSS mutations. 

Care was coordinated by maternal–fetal medicine, obstetrics, anesthesiology, and hematology. A peripartum plan included prophylactic platelet transfusion and perioperative tranexamic acid, with postoperative duration guided by clinical course. Neuraxial anesthesia was contraindicated; labor analgesia included nitrous oxide and remifentanil patient-controlled analgesia. Labor arrest of descent necessitated cesarean under general anesthesia. Induction was performed with a modified rapid sequence, and anesthesia was maintained with total intravenous anesthesia using propofol and adjuncts to minimize uterine atony. Intraoperative hemorrhage (~1500 mL) from uterine atony and arterial extension was managed with multimodal uterotonics, tranexamic acid, and mechanical tamponade. Postoperatively, she received two units of red blood cells and was discharged on postoperative day four without complications. 

Inherited platelet disorders challenge obstetric anesthesia because functional defects may not correlate with platelet count. Hematology testing guided the avoidance of neuraxial anesthesia, alternative analgesia selection, and preparation for general anesthesia. Prophylactic platelet transfusion and tranexamic acid were administered. Despite these measures, significant hemorrhage occurred, highlighting the need for anesthesiologists to remain vigilant and adaptable to evolving perioperative conditions inherent to both surgical procedures and the unpredictable phenotype of Bernard–Soulier variants. This case shows how diagnostic insights can guide real-time anesthetic planning and optimize outcomes in high-risk parturients.