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Congenital portosystemic venous shunt.
- Source :
-
European journal of pediatrics [Eur J Pediatr] 2018 Mar; Vol. 177 (3), pp. 285-294. Date of Electronic Publication: 2017 Dec 14. - Publication Year :
- 2018
-
Abstract
- Congenital portosystemic venous shunts are rare developmental anomalies resulting in diversion of portal flow to the systemic circulation and have been divided into extra- and intrahepatic shunts. They occur during liver and systemic venous vascular embryogenesis and are associated with other congenital abnormalities. They carry a higher risk of benign and malignant liver tumors and, if left untreated, can result in significant medical complications including systemic encephalopathy and pulmonary hypertension.<br />Conclusion: This article reviews the various types of congenital portosystemic shunts and their anatomy, pathogenesis, symptomatology, and timing and options of treatment. What is Known: • The natural history and basic management of this rare congenital anomaly are presented. What is New: • This paper is a comprehensive review; highlights important topics in pathogenesis, clinical symptomatology, and treatment options; and proposes an algorithm in the management of congenital portosystemic shunt disease in order to provide a clear idea to a pediatrician. An effort has been made to emphasize the indications for treatment in the children population and link to the adult group by discussing the consequences of lack of treatment or delayed diagnosis.
- Subjects :
- Abnormalities, Multiple diagnosis
Abnormalities, Multiple embryology
Abnormalities, Multiple therapy
Endovascular Procedures
Hepatectomy
Humans
Ligation
Liver Transplantation
Portal Vein embryology
Portal Vein abnormalities
Vascular Malformations diagnosis
Vascular Malformations embryology
Vascular Malformations therapy
Subjects
Details
- Language :
- English
- ISSN :
- 1432-1076
- Volume :
- 177
- Issue :
- 3
- Database :
- MEDLINE
- Journal :
- European journal of pediatrics
- Publication Type :
- Academic Journal
- Accession number :
- 29243189
- Full Text :
- https://doi.org/10.1007/s00431-017-3058-x