Congenital Portosystemic Shunts: Current Diagnosis and Management

J Pediatr Gastroenterol Nutr. 2019 May;68(5):615-622. doi: 10.1097/MPG.0000000000002263.


Congenital portosystemic shunts are increasingly recognized in several settings and at any age. The following are some of the most common presentations: prenatal ultrasound, neonatal cholestasis, incidental finding on abdominal imaging, or systemic complications such as unexplained cardiopulmonary or neurological disease, or the presence of liver nodules in a noncirrhotic liver. The purpose of the present review is to summarize clinical presentation and current recommendations for management, and highlight areas of future research. Illustrative examples from the veterinary literature complement our current lack of knowledge of this rare malformation often masquerading as a multisystem disease.

Publication types

  • Review

MeSH terms

  • Animals
  • Diagnosis, Differential
  • Disease Management
  • Female
  • Humans
  • Infant
  • Infant, Newborn
  • Liver / abnormalities
  • Liver / blood supply
  • Male
  • Portal Vein / abnormalities*
  • Vascular Malformations*

Supplementary concepts

  • Patent Ductus Venosus