Amniotic Fluid Embolism Triggered By Hypertensive Crisis Due to Undiagnosed Pheochromocytoma in a Pregnant Subject With Neurofibromatosis Type 1

ABSTRACT Objective: To report an unusual case of amniotic fluid embolism (AFE) caused by undiagnosed pheochromocytoma in a pregnant subject with neurofibromatosis type 1 (NF-1). Methods: We describe the clinical course and discuss the management of AFE in a patient with pheochromocytoma complicating NF-1 and present a review of relevant literature. Results: A 29-year-old female with a history of NF-1 presented with hypertension but no neurologic abnormalities. Pre-eclampsia and fetal dysfunction were diagnosed, and a cesarean section was performed. After it was completed, her oxygen saturation suddenly dropped, and an endotracheal intubation was performed. A watery discharge gushed out of the endotracheal tube. Whole-body computed tomography showed a left adrenal tumor (5-cm diameter). Two hours after admission to the intensive care unit, she developed ventricular fibrillation with cardiac shock. Direct current cardioversion, percutaneous cardiopulmonary support, and intra-aortic balloon pumping were immediately applied. Disseminated intravascular coagulation (DIC) persisted; around postpartum day 15, elevated levels of zinc-coproporphyrin I, a serum marker of AFE were observed. After the patient’s general condition and cardiac function stabilized, adrenalectomy was performed. Conclusion: The hypertensive crisis, subsequent AFE, and long hospital stay may have been prevented had the pheochromocytoma complicating NF-1 been diagnosed early. Genetic preconception counseling and screening is recommended for females with NF-1 with urine and/or serum fractionated metanephrines and catecholamines and imaging studies; further, whole-body magnetic resonance imaging should be performed in early pregnancy, even in the absence of pheochromocytoma symptoms, including hypertension, to prevent AFE. Abbreviations: AFE amniotic fluid embolism CT computed tomography DIC disseminated intravascular coagulation MIBG meta-iodobenzylguanidine MRI magnetic resonance imaging NF-1 neurofibromatosis type 1 PCPS percutaneous cardiopulmonary support Zn-CPI zinc-coproporphyrin I