Childhood pheochromocytoma crisis complicated with brain stem infarction: A case report.

Abstract

Pheochromocytoma crises are very rarely seen in children. In this report, we present a case of the death related to occult pheochromocytoma crisis combined cerebral infarction. A 5-year-old boy has a 1-month history of polydipsia, polyuria, sweating, and weight loss of 2.5 kg. He was admitted to our hospital because of 1 week of anorexia, 2 days of vomiting, and 12 hours of convulsions and confusion. Magnetic resonance imaging of the brain and cervical spinal cord showed abnormal signals in the left parie-occipital lobe, medulla oblongata till C7 cervical vertebrae. Based on patient's complaints and clinical appearance, provisional diagnosis of pheochromocytoma crisis complicated brainstem infarction was considered. Tracheal intubation, volume expansion, continuous infusion of dobutamine, and sedation reduce intracranial pressure. Chest compression was performed when the child suddenly developed sobbing respiration. The patient was dead. Congenital metabolic defects screening suggested mild ketonuria. Trio whole exon sequencing revealed a synonymous mutation of von Hippel-Lindau syndrome c.414 A > G in the decedent. Autopsy revealed pheochromocytoma, acute myocarditis, liquefaction necrosis of the medulla oblongata cerebral edema, and congestion. Early clinical symptoms of pheochromocytoma in children are not typical. It may induce serious complications and develop into a pheochromocytoma crisis and cause death without proper treatment.