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Review
. 2018 Mar;177(3):285-294.
doi: 10.1007/s00431-017-3058-x. Epub 2017 Dec 14.

Congenital portosystemic venous shunt

M Papamichail  1 , M Pizanias  2 , N Heaton  3
Affiliations
Review

Congenital portosystemic venous shunt

M Papamichail et al. Eur J Pediatr. 2018 Mar.

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.

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.

Keywords: Congenital; Portosystemic; Shunt.

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Conflict of interest statement

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

This article does not contain any studies with human participants or animals performed by any of the authors.

Figures

Fig. 1
Fig. 1
Diagram showing development of the portal and hepatic veins. The two vitelline veins communicate inside the liver and around the duodenum to form intrahepatic portal and hepatic veins: the left vitelline vein disappears, and the cranial part of the right vitelline vein and the segment that lies inferior to the liver give rise to the terminal branch of the IVC and portal and superior mesenteric veins. Incomplete involution and persistent communication of the vitelline venous system during the development of newly formed hepatic sinusoids results in various types of portosystemic shunts [11]
Fig. 2
Fig. 2
Therapeutic algorithm for CPSS
Fig. 3
Fig. 3
Intervention protocol for CPSS
See this image and copyright information in PMC

References

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Supplementary concepts

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