NM-356
Decompensating Infantile Hemangioendothelioma: A Multidisciplinary Approach to Optimize a Three Month Patient for Liver Transplantation.
Jivan K, Nguyen V, Nguyen V
Georgetown University Hospital, Washington, DC, USA
A three month girl, former-38 week with IUGR, presented to Georgetown University Hospital (GUH) for liver transplant due to infantile hepatic hemangioendothelioma. She was noted to have an enlarging abdomen at her 6-week wellness visit by her pediatrician. A CT scan at that time showed near complete replacement of the liver with a presumed vascular mass. She was subsequently diagnosed with a hepatic hemangioendothelioma and started on steroids at that time. Due to the lack of steroid response, she was started on a course of vincristine and sirolimus to shrink the mass. As well, propranolol is known to treat hemangioma due to its vasocontrictive properties, this was started one month prior to liver transplantation. Prior to arrival to GUH, she was noticed to have increasing respiratory effort and hypoxic episodes. She was intubated and had difficulties weaning from the ventilator. Her thyroid panel demonstrated a type 3 deiodinase and consumptive hypothyroidism. This massive hypothyroidism was secondary to a consumptive hypothyroidism by her tumor. At that time, her echocardiogram presented with a hyperdynamic biventricular function with increase flow over the ascending aorta and pulmonary outflow tract. Left ventricle was enlarged as well. As a result, her respiratory distress was believed to be cardiogenic in nature due to her hypothyroid state. The patient was automatically placed on the transplant list as a status 1b, while a multidisciplinary approach was initiated to optimize the patient for liver transplantation. Since her admission, she was extubated after the tumor shrunk and thyroid function was normalized. Her repeat echocardiogram showed significant improvement with no heart failure or pulmonary hypertension. Couple of weeks later she was given a left lateral segment orthotopic liver transplant via left hepatic artery to recipient left hepatic. Due to her large hemagioma, significant blood loss was anticipated. Daily ultrasounds post-transplant for 5 days showed stable graft function with good flow. Propanolol and synthroid were eventually discontinued and successfully extubated on postoperative day 2.
References:
Congenital infantile hepatic hemangioendothelioma type II treated with orthotopic liver transplantation. J Pediatr Hematol Oncol. 2004 Feb;26(2):121-3. Walsh R1, Harrington J, Beneck D, Ozkaynak MF
Propranolol as first-line treatment for rapidly proliferating Infantile Haemangiomas. J Plast Reconstr Aesthet Surg. 2011 Apr;64(4):445-51. Holmes WJ, Mishra A, Gorst C, Liew SH.
Monosegmental living-donor liver transplantation for infantile hepatic hemangioendothelioma. J Pediatr Surg. 2003 Jul;38(7):1108-11. Kasahara M1, Kiuchi T, Haga H, Uemoto S, Uryuhara K, Fujimoto Y, Ogura Y, Oike F, Yokoi A, Kaihara S, Egawa H, Tanaka K.
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