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2/2018
vol. 93 abstract:
Review paper
Congenital portosystemic shunts – diagnosis and treatment
Mateusz Ciopiński
,
Weronika Jaroń
,
Marek Szymczak
,
Grzegorz Kowalewski
,
Piotr Kaliciński
Pediatr Pol 2018; 93 (2): 180–185
Online publish date: 2018/06/07
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Congenital portosystemic shunt is a very rare congenital defect of the splanchnic venous system, in which blood from the portal vein completely or partially bypasses the liver. The most common symptoms are: hyperammonaemia (which may lead to encephalopathy), liver tumours, and hepatopulmonary syndrome. Due to its rarity and complex symptomatology it remains a great diagnostic challenge. Currently, endovascular closure of the shunt is a primary therapy. However, in cases where it is impossible, surgical ligation is an alternative. In selected cases liver transplantation may be indicated.
keywords:
congenital portosystemic shunt, Abernethy malformation, liver tumour, hepatopulmonary syndrome, hepatic encephalopathy |
POLSKI
