CONGENITAL PORTOSYSTEMIC VENOUS SHUNTS
Congenital portosystemic venous shunts (CPSS) are a rare group of heterogeneous developmental malformations of the portal and systemic circulation causing blood from the portal system to bypass liver metabolism through various vascular junctions and flow directly into the systemic circulation. They c...
| Main Authors: | , |
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| Format: | Article |
| Language: | Slovenian |
| Published: |
The Society for Children with Metabolic Disorders
2021-03-01
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| Series: | Slovenska pediatrija |
| Subjects: | |
| Online Access: |
http://www.slovenskapediatrija.si/Portals/0/Clanki/2021/Slovpediatr-2021-1-02en.pdf
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| Summary: | Congenital portosystemic venous shunts (CPSS) are a rare group of heterogeneous developmental malformations of the portal and systemic circulation causing blood from the portal system to bypass liver metabolism through various vascular junctions and flow directly into the systemic circulation. They can be divided into two groups comprising intrahepatic shunts, with persistent ductus venosus, and extrahepatic shunts. The anomalous flow of splanchnic blood with undegraded metabolites directly into the systemic circulation may lead to severe complications: neonatal cholestasis, benign and malignant liver tumours, hepato-pulmonary syndrome, porto-pulmonary hypertension and encephalopathy. Small intrahepatic shunts may regress spontaneously in the first two years of life. Other permanent intrahepatic and extrahepatic shunts should be closed in one or two steps by minimally invasive interventional radiological closure methods or by surgical procedure. Extrahepatic type 1 shunts always require liver transplantation. |
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| ISSN: | 1318-4423 2712-3960 |