Congenital intrahepatic portohepatic shunt managed by interventional radiologic occlusion: a case report and literature review☆

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Abstract

Congenital intrahepatic portosystemic shunts are rare hepatic vascular anomalies that often lead to severe secondary conditions. A 6-year-old boy was referred for assessment of severe hypoxia, and a large liver mass was diagnosed with such a malformation and was managed by direct closure of the venous fistula by interventional radiology. Follow-up assessment shows normalization of the respiratory condition and a progressive reduction of the vascular liver lesion. Review of literature suggests that radiologic interventions are currently the criterion standard for managing these conditions, with surgery reserved for patients who are not eligible for radiologic procedure and those requiring liver transplantation.

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Case report

A 6-year-old white boy with a history of recurrent mild bronchopulmonary infections was seen at a local hospital for progressive dyspnea and fatigue. Physical examination showed digital clubbing and cyanosis with an oxygen saturation (SaO2) of 72% on room air. Thoracic auscultation, lung function tests, bronchoscopy, chest radiograph, and computed tomographic scan of thorax and lungs were unremarkable, with only mention of a slightly enlarged heart. A provisional diagnosis of hypoxia caused by

Discussion

Congenital portosystemic shunts are rare vascular malformations first described by Abernethy in 1793 and subsequently classified by Morgan and Superina [3] in 2 major variants: intrahepatic or extrahepatic types. Intrahepatic CPSS is defined as an anatomically aberrant veno-venous communication between a portal and an hepatic vein or the vena cava with a diameter larger than 1 mm and completely or partially located inside the liver [4]. Recently, Lautz et al [5] classified intrahepatic

Conclusion

Congenital portosystemic shunt is a rare but pathologic condition, with possible severe related complications. The latter conditions and the related clinical symptoms are likely reversible after occlusion of the fistula. Interventional radiology has become the criterion standard with surgery reserved for selected patients not eligible for percutaneous management. Occlusion of the shunt should be recommended when radiologic intervention can be performed at low risk and before complications arise

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Note: Amplatzer: see http://international.amplatzer.com.

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