Clinical Report: Adult Liver Transplantation Utilizing a Long-Term Mesocaval Shunt

Background

Congenital absence of the portal vein (CAPV) is a rare vascular anomaly that leads to systemic circulation bypassing the liver, often resulting in metabolic complications rather than classical portal hypertension. This case highlights the complexities involved in treating CAPV.

Data Highlights

No numerical data or trial data presented in the article.

Key Findings

• The patient experienced esophageal variceal hemorrhage, an atypical presentation of CAPV.
• An H-type mesocaval shunt was performed at age three, allowing for clinical compensation for over thirty years.
• Refractory hyperammonemic encephalopathy developed, necessitating liver transplantation.
• Portal inflow was restored using an end-to-end shunt-to-portal anastomosis due to the absence of the native portal vein.
• Postoperative complications included biliary anastomotic stricture and late stenosis of the shunt-to-portal anastomosis, both of which were managed effectively.
• At follow-up, the patient showed clinical stability with no recurrence of encephalopathy.

Clinical Implications

Clinicians should be aware of the atypical presentations of CAPV.

Conclusion

This case demonstrates the use of a mesocaval shunt for portal inflow in liver transplantation in the absence of the native portal vein.

Related Resources & Content

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10. Frontiers | Case Report: Adult Liver Transplantation Using a Long-Standing Mesocaval Shunt as Portal Inflow in Congenital Absence of the Portal Vein With Atypical Variceal Bleeding and Refractory Shunt Encephalopathy