A Rare Case of Sudden Death Due to Hypotension during Cesarean Section in a Woman Suffering from Pheochromocytoma and Neurofibromatosis

Sudden death following acute hypotension due to an undiagnosed pheochromocytoma (PHEO) is a rare event. Moreover, histopathology of the myocardium in such cases is rarely reported. We present a case of a woman who died during delivery. A 37-year-old parturient, who was 38 weeks pregnant, suffering from neurofibromatosis underwent a cesarean section following peridural anesthesia. Acute hypotension, acute intra-operative pulmonary edema and supraventricular paroxysmal tachyarrhythmia occurred during delivery, followed by death. The autopsy revealed the presence of a PHEO, confirmed immunohistochemically with chromogranin-A (CgA), CD20 antibody (L26), anti-Keratocan antibody (KER-1) and neuron-specific enolase (NSE), and a PHEO-induced cardiomyopathy. The physiopathology of both stress-induced cardiomyopathy and PHEO-induced cardiomyopathy, as well as the role of anesthesia in provoking the death, are discussed. The association of an undiagnosed PHEO with neurofibromatosis as the cause of sudden death in pregnancy is an obstetric urgency that raises forensic pathology issues. © 2013 American Academy of Forensic Sciences