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

Nozomu Takahashi, MD; Koji Nishijima, MD, PhD; Makoto Orisaka, MD, PhD; Hideaki Tsuyoshi, MD; Tetsuji Kurokawa, MD, PhD; Kana Kato, MD; Aya Shirafuji, MD; Kenichiro Arakawa, MD, PhD; Kaori Hisazaki, MD; Hiroshi Tada, MD, PhD; Yoshio Yoshida, MD, PhD

AACE Clinical Case Reports (Jan 2015)

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

Nozomu Takahashi, MD,
Koji Nishijima, MD, PhD,
Makoto Orisaka, MD, PhD,
Hideaki Tsuyoshi, MD,
Tetsuji Kurokawa, MD, PhD,
Kana Kato, MD,
Aya Shirafuji, MD,
Kenichiro Arakawa, MD, PhD,
Kaori Hisazaki, MD,
Hiroshi Tada, MD, PhD,
Yoshio Yoshida, MD, PhD

Affiliations

Nozomu Takahashi, MD: From the Departments of Obstetrics and Gynecology, Faculty of Medical Sciences, University of Fukui, Japan.
Koji Nishijima, MD, PhD: From the Departments of Obstetrics and Gynecology, Faculty of Medical Sciences, University of Fukui, Japan.; Address correspondence to Dr. Koji Nishijima; Department of Obstetrics and Gynecology, Faculty of Medical Sciences, University of Fukui, Matsuoka, Eiheiji-cho, Yoshida-gun, Fukui 910-1193, Japan.
Makoto Orisaka, MD, PhD: From the Departments of Obstetrics and Gynecology, Faculty of Medical Sciences, University of Fukui, Japan.
Hideaki Tsuyoshi, MD: From the Departments of Obstetrics and Gynecology, Faculty of Medical Sciences, University of Fukui, Japan.
Tetsuji Kurokawa, MD, PhD: From the Departments of Obstetrics and Gynecology, Faculty of Medical Sciences, University of Fukui, Japan.
Kana Kato, MD: From the Departments of Obstetrics and Gynecology, Faculty of Medical Sciences, University of Fukui, Japan.
Aya Shirafuji, MD: From the Departments of Obstetrics and Gynecology, Faculty of Medical Sciences, University of Fukui, Japan.
Kenichiro Arakawa, MD, PhD: Cardiovascular Medicine, Faculty of Medical Sciences, University of Fukui, Japan.
Kaori Hisazaki, MD: Cardiovascular Medicine, Faculty of Medical Sciences, University of Fukui, Japan.
Hiroshi Tada, MD, PhD: Cardiovascular Medicine, Faculty of Medical Sciences, University of Fukui, Japan.
Yoshio Yoshida, MD, PhD: From the Departments of Obstetrics and Gynecology, Faculty of Medical Sciences, University of Fukui, Japan.

Journal volume & issue: Vol. 1, no. 3
pp. e178 – e181

Abstract

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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

Published in AACE Clinical Case Reports

ISSN: 2376-0605 (Online)
Publisher: Elsevier
Country of publisher: United States
LCC subjects: Medicine: Internal medicine: Specialties of internal medicine: Diseases of the endocrine glands. Clinical endocrinology
Website: https://www.journals.elsevier.com/aace-clinical-case-reports

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