Surgical shunt ligation for a congenital extrahepatic portosystemic shunt with pulmonary hypertension: A case report

Congenital extrahepatic portosystemic shunt (CEPS) presents with various symptoms due to abnormal communication between the portal venous system and inferior vena cava. And Klippel-Trenaunay-Weber syndrome is another rare congenital disorder characterized by vascular malformations. A 16-year-old mal...

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Bibliographic Details
Published in:International journal of surgery case reports 2022-04, Vol.93, p.107024-107024, Article 107024
Main Authors: Yamada, Kensuke, Matsukuma, Satoshi, Tokumitsu, Yukio, Shindo, Yoshitaro, Ikeda, Yasuhiro, Nagano, Hiroaki
Format: Article
Language:English
Subjects:
Case Report
Congenital extrahepatic portosystemic shunt
Klippel-Trenaunay-Weber syndrome
Portopulmonary hypertension
Shunt ligation
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Summary:Congenital extrahepatic portosystemic shunt (CEPS) presents with various symptoms due to abnormal communication between the portal venous system and inferior vena cava. And Klippel-Trenaunay-Weber syndrome is another rare congenital disorder characterized by vascular malformations. A 16-year-old male with Klippel-Trenaunay-Weber syndrome was referred to our hospital for surgical treatment of pulmonary hypertension due to CEPS since childhood. Dyspnea had developed about two years before presentation at our hospital and gradually worsened. Right heart catheterization had revealed pulmonary hypertension and a high cardiac output state associated with a portosystemic shunt. Although pulmonary vasodilators improved the dyspnea, pulmonary hypertension remained. The patient was then referred to our hospital for surgical shunt occlusion. The results of the preoperative and intraoperative shunt occlusion tests were within acceptable limits. Therefore, primary shunt ligation was performed. There were no postoperative complications. Continuous intravenous vasodilator was tapered off four months after discharge. No additional or increased doses of medications were required for four years after surgery. Surgical shunt ligation for CEPS is effective but can cause acute portal hypertension. Primary shunt ligation could be performed without complications according to the criteria for safe shunt occlusion. Surgical portosystemic shunt ligation could be performed safely and ameliorated pulmonary hypertension. Patients with CEPS, especially young patients, should be referred to a tertiary center while asymptomatic, and early therapeutic intervention is necessary. •Congenital extrahepatic portosystemic shunt (CEPS) is a rare vascular malformation.•Our patient had pulmonary hypertension due to CEPS.•Pre- and intraoperative shunt occlusion tests were within acceptable limits.•He underwent primary surgical portosystemic shunt ligation.•No postoperative complications including acute portal hypertension occurred.
ISSN:2210-2612
2210-2612
DOI:10.1016/j.ijscr.2022.107024