Congenital Portosystemic Shunts: Variable Clinical Presentations Requiring a Tailored Endovascular or Surgical Approach.

Autor: Robinson EB; From the Department of Radiology, University of Colorado School of Medicine, Aurora, CO, USA., Jordan G; From the Department of Radiology, University of Colorado School of Medicine, Aurora, CO, USA., Katz D; Division of Pediatric Radiology, Department of Radiology, University of Colorado, Aurora, CO, USA., Sundaram SS; Division of Pediatric Gastroenterology, Hepatology and Nutrition, Digestive Health Institute, Department of Pediatrics, University of Colorado, Aurora, CO, USA., Boster J; Division of Pediatric Gastroenterology, Hepatology and Nutrition, Digestive Health Institute, Department of Pediatrics, University of Colorado, Aurora, CO, USA., Brigham D; Division of Pediatric Gastroenterology, Hepatology and Nutrition, Digestive Health Institute, Department of Pediatrics, University of Colorado, Aurora, CO, USA., Ladd P; Division of Pediatric Radiology, Department of Radiology, University of Colorado, Aurora, CO, USA., Chan CM; Division of Pediatric Endocrinology, Department of Pediatrics, University of Colorado, Aurora, CO, USA., Shay RL; Division of Neonatology, Department of Pediatrics, University of Colorado, Aurora, CO, USA., Ochmanek E; Division of Interventional Radiology, Department of Radiology, University of New Mexico, Albuquerque, NM, USA., Annam A; Division of Pediatric Radiology, Department of Radiology, University of Colorado, Aurora, CO, USA.
Jazyk: angličtina
Zdroj: JPGN reports [JPGN Rep] 2023 Jan 12; Vol. 4 (1), pp. e279. Date of Electronic Publication: 2023 Jan 12 (Print Publication: 2023).
DOI: 10.1097/PG9.0000000000000279
Abstrakt: Congenital portosystemic shunts (CPSS) are rare developmental anomalies resulting in diversion of portal flow to the systemic circulation. These shunts allow intestinal blood to reach the systemic circulation directly, and if persistent or large, may lead to long-term complications. CPSS can have a variety of clinical presentations that depend on the substrate that is bypassing hepatic metabolism or the degree of hypoperfusion of the liver. Many intrahepatic shunts spontaneously close by 1 year of age, but extrahepatic and persistent intrahepatic shunts require intervention by a single session or staged closure with a multidisciplinary approach. Early detection and appropriate management are important for a good prognosis. The aim of this case series is to describe the varied clinical presentations, treatment approaches, and outcomes of 5 children with CPSS at our institution. Management of these patients should involve a multidisciplinary team, including interventional radiology, surgery, hepatology, and other medical services as the patient's clinical presentation warrants. Regardless of clinical presentation, if a CPSS persists past 1-2 years of age, closure is recommended.
Competing Interests: The authors report no conflicts of interest. At least one author is a member of NASPGHAN.
(Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)
Databáze: MEDLINE
Externí odkaz: