Extracorporeal membrane oxygenation (ECMO) in the treatment of acute cardiac failure and pulmonary edema secondary to pheochromocytoma (PHEO) crisis in a Pediatric patient with MEN-2B

Program: Abstracts - Orals, Featured Poster Presentations, and Posters
Session: MON 37-82-Pheochromocytoma & Paraganglioma
Monday, June 17, 2013: 1:45 PM-3:45 PM
Expo Halls ABC (Moscone Center)

Poster Board MON-77
Kanthi Bangalore Krishna*1, Michelle Yarelis Rivera-Vega2 and Luigi R Garibaldi3
1Children's Hospital of Pittsburgh, Pittsburgh, PA, 2Childrens Hospital of Pittsburgh, Pittsburgh, PA, 3Children's Hospital of UPMC, Pittsburgh, PA
Background:Patients with MEN- 2B (especially codon 634 or 918 mutations) have up to a 50% lifetime risk of developing a PHEO. Catecholamine induced adrenergic cardiomyopathy, cardiac failure and pulmonary edema (PE), reported in adults with PHEO, are mostly reversible following surgical removal of the tumor. Short term ECMO support, as a bridge between medical management and therapeutic surgery, and on-ECMO bilateral adrenalectomy (BA) has never been reported in the Pediatric age group.

Case:17 year old male with MEN-2B (M918T mutation) and a past history of medullary thyroid carcinoma, s/p total thyroidectomy at age 11 presented to an outside ED with intense headache, emesis and hypertension. Following intravenous Metoclopramide and Ketorolac for presumed migraine, he became severely hypertensive (BP up to 200/ 120 mm Hg), tachycardic (HR of 180/min), diaphoretic and dyspneic. After transfer to our hospital, he continued to have tachycardia, and hypertension intermixed with hypotension. A chest radiograph, showed PE and an Echocardiogram showed severe left ventricular (LV) dysfunction and hypokinesia. Worsening PE, poor perfusion and metabolic acidosis lead to refractory hypotension and cardiorespiratory arrest. He was successfully resuscitated, intubated and placed on veno-arterial  ECMO. Abdominal CT scan showed bilateral suprarenal masses, supporting the diagnosis of PHEO. Lab studies showed elevated plasma metanephrine of 500 pg/ml (57) and normetanephrine of 4090 pg/ml (<148).

He underwent BA via open laparotomy while on ECMO. Following surgery, LV function and ejection fraction improved, PE resolved and he was successfully weaned off the ECMO in 2 days. His cardiac function gradually improved and returned to normal approximately 1 month after discharge.

Discussion:To our knowledge, this is the first pediatric patient undergoing BA while on ECMO for treatment of acute cardiac failure and PE secondary to PHEO crisis, worsened by Metoclopramide. Stabilization of cardio-respiratory parameters while on ECMO minimized the risk of complications from intraoperative catecholamine discharge.

This case highlights that 1-ECMO can be used effectively to stabilize pediatric patients with PHEO and severe cardiomyopathy and 2- Adrenalectomy can be safely performed while the patient is on ECMO.  Despite the successful outcome, prevention of severe decompensation via regular screening and early detection of PHEOs in patients with MEN 2B is of utmost importance.

Nothing to Disclose: KB, MYR, LRG

*Please take note of The Endocrine Society's News Embargo Policy at http://www.endo-society.org/endo2013/media.cfm