Mesoportal bypass surgery with congenital portocaval shunt in a child aged 1 year and 3 months

Abstract

Introduction. Abernethy malformation (CEPS) is a rare condition in which portomesenteric blood bypasses the liver through a partial or complete shunt into the inferior vena cava. This pathology is extremely rare in children. Clinically, it can occur almost asymptomatically or under the guise of other diseases. It is manifested by signs of hepatic encephalopathy, pulmonary hypertension or hepatopulmonary syndrome. Mesoportal bypass surgery is an option for correcting portocaval shunts with the restoration of physiological blood flow in the liver.Since 2016, a center for treating developmental anomalies in the hepato-pancreato-biliary system in children has been operating in Filatov Municipal Children's Hospital (Moscow). To date, the center has performed more than 1,500 surgical interventions on the organs of biliopancreato-duodenal zone. For the period from 2016 to 2022, an endovascular occluder was used in 13 children with congenital portocaval shunts; surgical dressing – in 6 children.Description of clinical observation. This article describes a clinical case of a child with a congenital portocaval shunt. A comprehensive instrumental examination revealed a complete absence of portal blood flow in the liver of a newborn baby . The spleen vein and superior mesenteric vein flowed into the inferior vena cava as a single trunk. It is believed that liver transplantation is the only effective treatment for this type of pathology. Despite the ambiguous anatomy of liver vessels, the child was operated on for the mesoportal bypass. The architectonics of child's liver was restored, and he returned to an absolutely normal life without any life-threatening factors.Conclusion. This clinical case demonstrates the only described case of complete surgical cure of a complex liver vascular defect.