REVIEW  NEW TRENDS IN PEDIATRIC SURGERY 

Minerva Pediatrica 2018 June;70(3):303-7

DOI: 10.23736/S0026-4946.18.05207-6

Copyright © 2018 EDIZIONI MINERVA MEDICA

language: English

New therapies for vascular anomalies of the gastrointestinal tract

Victor L. FOX ✉

Gastrointestinal Procedures and Endoscopy Unit, Division of Gastroenterology, Hepatology, and Nutrition, Boston Children’s Hospital, Harvard Medical School, Boston, MA, USA

Vascular anomalies are a morphologically and biologically diverse group of vascular channel abnormalities that are often congenital but may evolve or change over time in the developing child. Classification is based on a combination of physical and biological properties and clinical behavior that differentiate primarily between tumors and malformations and includes a few provisionally unclassified lesions. Anomalies of the gastrointestinal (GI) tract may present clinically with GI bleeding, abdominal pain, high-output cardiac failure, and malabsorption. This review focuses on new therapies for the treatment of GI bleeding. Important new pharmacological therapies include treatment of hemangioma with non-selective and selective beta-antagonist agents, propranolol and atenolol, and treatment of blue rubber bleb nevus syndrome and cutaneo-visceral angiomatosis with thrombocytopenia (also known as multifocal lymphangioendotheliomatosis with thrombocytopenia) with sirolimus, an inhibitor of the mammalian target of rapamycin. Therapeutic endoscopy may offer an effective alternative to bowel resection for colonic varices and other focal vascular anomalies of the GI tract that fail to respond to pharmacological therapy.

KEY WORDS: Vascular malformations - Blue rubber bleb nevus syndrome - Hemangioma - Angiogenesis inhibitors - Colonic varices without portal hypertension - Sirolimus