Copyright ©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Nov 14, 2020; 26(42): 6582-6598
Published online Nov 14, 2020. doi: 10.3748/wjg.v26.i42.6582
Vascular anomalies associated with hepatic shunting
Michael J Schmalz, Kadakkal Radhakrishnan
Michael J Schmalz, Department of Pediatric Gastroenterology, Cleveland Clinic, Cleveland, OH 44106, United States
Kadakkal Radhakrishnan, Department of Pediatric Gastroenterology, Children's Hospital, Cleveland Clinic, Cleveland, OH 44106, United States
Author contributions: Schmalz MJ and Radhakrishnan K contributed equally to this work, both authors have read and approved the final manuscript.
Conflict-of-interest statement: The authors have no conflicts of interest with regards the topic of this review.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Corresponding author: Michael J Schmalz, MD, Academic Fellow, Department of Pediatric Gastroenterology, Cleveland Clinic, 8950 Euclid Ave, Cleveland, OH 44106, United States.
Received: August 12, 2020
Peer-review started: August 12, 2020
First decision: September 30, 2020
Revised: October 14, 2020
Accepted: October 27, 2020
Article in press: October 27, 2020
Published online: November 14, 2020
Processing time: 93 Days and 4 Hours

Congenital vascular anomalies affecting the liver have been described in the scientific literature for decades. Understanding these malformations begins with knowledge of hepatic vascular embryology. Surgeons have applied numerous classification systems to describe both intrahepatic and extrahepatic shunts, which can confuse the reader and clinician. In our experience, focusing on one classification system for extrahepatic shunts and one for intrahepatic shunts is better. Today many patients with these shunts carry good long-term prognosis thanks to advances in imaging to better detect shunts earlier and classify them. Timely intervention by skilled radiologists and surgeons have also limited complications arising from dynamic shunts and can avoid a liver transplant. Congenital hepatic shunts are not the only vascular condition affecting the liver. Hereditary hemorrhagic telangiectasia, also known as Osler Weber Rendu syndrome, particularly type 2, may have varying severity of hepatic involvement which warrants longitudinal care from an experienced hepatologist. Lastly, congenital hemangiomas, often first identified on the skin and oral mucosa, also can affect the liver. While most will resolve in infancy and childhood, the pediatric hepatologist must understand how and when to treat persistent lesions and their complications. This article serves as a concise reference to help clinicians better care for patients with these rare conditions.

Keywords: Hepatic, Shunt, Pediatric, Hemangioma, Congenital, Vascular

Core Tip: Hepatic shunts present from birth, hepatic hemangiomas, and hereditary hemorrhagic telangiectasia have all been described in the scientific literature over the decades. Most reviews were written by radiologists or surgeons, but none have adequately covered all these topics from the gastroenterologist’s perspective. Our review serves as a reference for most congenital vascular anomalies that present in the liver. Our goal is to provide knowledge to help clinicians understand the burden of disease of these conditions and guide management decisions.