CONGENITAL PORTOSYSTEMIC VENOUS SHUNTS
| Autor: | Paola Trupac, Petja Fister |
|---|---|
| Jazyk: | slovinština |
| Rok vydání: | 2021 |
| Předmět: | |
| Zdroj: | Slovenska pediatrija, Vol 28, Iss 1, Pp 10-15 (2021) |
| Druh dokumentu: | article |
| ISSN: | 1318-4423 2712-3960 |
| DOI: | 10.38031/slovpediatr-2021-1-02en |
| Popis: | 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. |
| Databáze: | Directory of Open Access Journals |
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