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Congenital portosystemic venous shunt.

M Papamichail1, M Pizanias2, N Heaton3

  • 1Department of Transplantation and Hepato-Pancreato-Biliary Surgery, Lahey Hospital and Medical Center, Burlington, Boston, MA, 01805, USA.

European Journal of Pediatrics
|December 16, 2017
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Summary

Congenital portosystemic venous shunts are rare liver vascular anomalies. Early diagnosis and treatment are crucial to prevent serious complications like encephalopathy and pulmonary hypertension.

Keywords:
CongenitalPortosystemicShunt

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Area of Science:

  • Vascular Surgery
  • Pediatric Surgery
  • Medical Genetics

Background:

  • Congenital portosystemic venous shunts are rare developmental anomalies.
  • These shunts involve abnormal connections between the portal and systemic circulation.
  • They are associated with other congenital abnormalities and increased risk of liver tumors.

Purpose of the Study:

  • To provide a comprehensive review of congenital portosystemic shunts.
  • To highlight key aspects of pathogenesis, clinical presentation, and treatment options.
  • To propose a management algorithm for pediatricians.

Main Methods:

  • Literature review of congenital portosystemic shunts.
  • Analysis of anatomical variations, embryogenesis, and clinical manifestations.
  • Discussion of treatment timing, options, and long-term outcomes.

Main Results:

  • Congenital portosystemic shunts can be extrahepatic or intrahepatic.
  • Untreated shunts pose risks of hepatic encephalopathy and pulmonary hypertension.
  • Early intervention is emphasized for pediatric cases to prevent adult complications.

Conclusions:

  • This review synthesizes current knowledge on congenital portosystemic shunts.
  • It emphasizes the importance of timely diagnosis and management in children.
  • A proposed algorithm aims to guide pediatricians in managing these rare anomalies.