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

Eduardo Bent Robinson; Gregory Jordan; Danielle Katz; Shikha S Sundaram; Julia Boster; Dania Brigham; Patricia Ladd; Christine M Chan; Rebecca L Shay; Emily Ochmanek; Aparna Annam

doi:10.1097/PG9.0000000000000279

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

JPGN Rep. 2023 Jan 12;4(1):e279. doi: 10.1097/PG9.0000000000000279. eCollection 2023 Feb.

Authors

Affiliations

¹ From the Department of Radiology, University of Colorado School of Medicine, Aurora, CO, USA.
² Division of Pediatric Radiology, Department of Radiology, University of Colorado, Aurora, CO, USA.
³ Division of Pediatric Gastroenterology, Hepatology and Nutrition, Digestive Health Institute, Department of Pediatrics, University of Colorado, Aurora, CO, USA.
⁴ Division of Pediatric Endocrinology, Department of Pediatrics, University of Colorado, Aurora, CO, USA.
⁵ Division of Neonatology, Department of Pediatrics, University of Colorado, Aurora, CO, USA.
⁶ Division of Interventional Radiology, Department of Radiology, University of New Mexico, Albuquerque, NM, USA.

Abstract

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.

Keywords: endovascular closure; hepatic tumors; hyperinsulinism; interventional radiology; portosystemic shunt.

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.

Publication types

Review