Adult Liver Transplantation Utilizing a Long-Term Mesocaval Shunt for Portal Blood Flow in Congenital Absence of the Portal Vein with Unusual Variceal Hemorrhage and Persistent Shunt Encephalopathy

Category	Detail
Condition	Congenital Absence of the Portal Vein (CAPV)
Key Mechanisms	Failure of the native portal vein to develop, leading to direct drainage of mesenteric and splenic venous blood into systemic circulation.
Target Population	Patients with congenital portosystemic vascular anomalies, particularly CAPV.
Care Setting	Surgical intervention for liver transplantation.

CAPV is characterized by metabolic and vascular complications rather than classical portal hypertension.
The case involved a 36-year-old female with atypical variceal hemorrhage and persistent shunt encephalopathy.
An H-type mesocaval shunt was utilized for over thirty years before liver transplantation.
Postoperative complications included biliary anastomotic stricture and late stenosis of the shunt-to-portal anastomosis.
The mesocaval shunt provided a viable source of portal inflow during transplantation.

Imaging studies to confirm CAPV and rule out extrahepatic portal vein obstruction.

Consider liver transplantation for patients with refractory encephalopathy or other severe complications.

Monitor for complications such as hyperammonemic encephalopathy and gastrointestinal dysmotility.

Potential for severe hyperammonemic encephalopathy related to long-term shunting.

Patients with congenital absence of the portal vein and related complications.

Liver transplantation may be necessary for definitive treatment in cases of refractory symptoms.

Tailor management strategies to the unique anatomy of the patient.
Utilize established shunts for portal inflow during liver transplantation when native portal vein is absent.

Clinical Scorecard: Adult Liver Transplantation Utilizing a Long-Term Mesocaval Shunt for Portal Blood Flow in Congenital Absence of the Portal Vein with Unusual Variceal Hemorrhage and Persistent Shunt Encephalopathy