Fax +41 61 306 12 34 E-Mail karger@karger.ch www.karger.com Case Report Horm Res 2005;63:86–89 DOI: 10.1159/000083879 Severe Hypothyroidism Caused by Hepatic Hemangioendothelioma in an Infant of a Diabetic Mother Ayla Güven a Canan Aygun b Hülya I ˙ nce b Murat Aydın a F. Güçlü Pınarlı c Kemal Baysal d Sükrü Küçüködük b Divisions of a Endocrinology, b Neonatology, c Oncology, and d Cardiology, Department of Pediatrics, Medical Faculty, Ondokuz Mayıs University, Samsun, Turkey tion and severe hypothyroidism [1–4]. Production of type 3 iodothyronine deiodinase by HAEs has been reported [4–6]. Increased degradation of thyroxine by this enzyme within the tumor tissue, exceeding the synthetic capacity of the thyroid gland, causes hypothyroidism. The marked increases in serum reverse triiodothyronine (r-T 3 ) con- centrations during treatment with L -thyroxine therapy could be explained by the increase in the activity of type 3 iodothyronine deiodinase [4]. The risk of congenital malformations in infants of di- abetic mothers is higher than newborns of non-diabetics [7]. Sacral agenesis, vertebral fusion defects and DiGeorge anomaly have been reported in newborns of diabetic mothers [8–10]. Severe cardiac abnormalities such as ventricular and atrial septal defect, interrupted aortic arch and hypertropic cardiomyopathy have also been shown in babies of diabetic women [11, 12] . Here we report an infant of a diabetic mother with hy- pothyroidism and liver HAE, in whom hypothyroidism was thought to be associated with type 3 iodothyronine deiodinase expression by the tumor tissue. Case Report A 7-hour-old girl was admitted to the Neonatal Intensive Care Unit for respiratory distress and multiple congenital abnormalities. Her gestational age was 34 weeks. Her mother had been diabetic Key Words Hemangioendothelioma  Hypothyroidism  Newborn and infant  Diabetic mother Abstract Hemangioendotheliomas can express type 3 iodothyro- nine deiodinase and cause severe hypothyroidism. The risk of congenital malformations such as vertebral and cardiac abnormalities in infants of diabetic mothers is higher than babies of healthy women. Here we report an infant of a diabetic mother with hypothyroidism caused by liver hemangioendothelioma. Consumptive hypothy- roidism should be an indicator to search for a vascular tumor in infants. Supranormal doses of L -thyroxine might be required for normalization of thyroid function until the tumor involutes or is resected. Copyright © 2005 S. Karger AG, Basel Introduction Hemangioendotheliomas (HAEs) are the most com- mon vascular tumors of infancy. Infantile hepatic HAE is a benign hepatic tumor that can be associated with life- threatening complications such as cardiac failure, ulcer- ations, Kasabach-Merritt phenomenon, airway obstruc- Received: November 1, 2004 Accepted: December 14, 2004 Published online: February 7, 2005 HORMONE RESEARCH Dr. Ayla Güven Ondokuz Mayıs Universitesi Tıp Fakültesi Pediatrik Endokrinoloji Bilim Dalı, Kurupelit TR–55200 Samsun (Turkey) Tel. +90 362 4576000, Fax +90 362 4576041, E-Mail aylaguven@yahoo.com © 2005 S. Karger AG, Basel 0301–0163/05/0632–0086$22.00/0 Accessible online at: www.karger.com/hre