Extensive Thrombosis of the Portal Vein and Vena Cava After Orthotopic Liver Transplantation With Cavoportal Hemitransposition: A Case Report

Abernethy malformation or congenital agenesis of the portal vein (CAPV) is a rare malformation of the abdominal splanchnic venous system. This malformation is commonly found in children and is often associated with other malformations such as congenital cardiac anomalies and skeletal system disorders, as well as liver tumors. There are two types of Abernethy malformation. In type I, portal blood bypasses the liver completely, with the superior mesenteric vein and the splenic vein draining into the inferior vena cava separately (type Ia), or together (type Ib). There are no intrahepatic portal vein branches in the liver. Type II is a partial portocaval shunt in which portal blood partially supplies the liver. There is no unified therapeutic approach for all patients with Abernethy malformation, however, liver transplantation is recommended in patients with liver disease (encephalopathy, poor liver function) and those with liver tumors. In this case report we present a case of Abernethy type Ib malformation in a 17-year-old patient with chronic malaise and uper abdominal pain. During diagnostic work-up, an unresectable liver tumor was found and the patient was successfully treated with orthotopic liver transplantation.

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Cadaveric Liver Transplantation in Biliary Atresia Splenic Malformation Syndrome With the Absence of Retrohepatic Inferior Vena Cava, Preduodenal Portal Vein, and Intestinal Malrotation: A Case Report

Transplantation Proceedings ◽

10.1016/j.transproceed.2007.11.027 ◽

2008 ◽

Vol 40 (1) ◽

pp. 313-315 ◽

Cited By ~ 3

Author(s):

E. Sen-Oran ◽

Y. Yankol ◽

B. Tuzun ◽

B. Kocak ◽

T. Kanmaz ◽

...

Keyword(s):

Liver Transplantation ◽

Case Report ◽

Inferior Vena Cava ◽

Portal Vein ◽

Biliary Atresia ◽

Inferior Vena ◽

Vena Cava ◽

Malformation Syndrome ◽

Preduodenal Portal Vein ◽

Retrohepatic Inferior Vena Cava

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Prolonged Anhepatic Phase after Liver Transplantation Failure Followed by ABO Incompatible Liver Transplantation: A Case Report and Review of the Literature

10.31487/j.jcmcr.2020.01.08 ◽

2020 ◽

pp. 1-5

Author(s):

Olivier Zerbib ◽

Benjamin Zribi ◽

David Dahan ◽

Eviatar Nesher ◽

Jonathan Cohen ◽

...

Keyword(s):

Liver Transplantation ◽

Intensive Care ◽

Case Report ◽

Portal Vein ◽

Liver Failure ◽

Orthotopic Liver Transplantation ◽

Supportive Therapy ◽

Successful Outcome ◽

Biliary Cirrhosis ◽

Fulminant Liver Failure

Objective: To describe the experience with a multimodal therapeutic approach in a patient who developed toxic liver syndrome and fulminant hepatic failure following orthotopic liver transplantation (OLT) as a result of occlusion of the portal vein. Setting: Department of Intensive Care. Patient: A patient with liver cirrhosis secondary to autoimmune hepatitis and primary biliary cirrhosis who underwent orthotopic liver transplantation (OLT). Interventions: Transplant hepatectomy, plasmapheresis and retransplantation. Case Report: A 39-year-old man underwent an elective OLT. A routine postoperative doppler ultrasound examination a few hours after surgery revealed portal vein thrombosis. Attempts at recanalization failed, and the patient developed acute fulminant liver failure, which remained resistant to supportive therapy. A transplant hepatectomy was performed 9 hours later and plasmapheresis started. Following a 10-hour anhepatic period, the patient received a second liver, from an ABO-incompatible donor. The patient underwent column plasmapheresis and subsequent splenectomy to remove anti-B antibody to preserve the incompatible transplanted liver from immunogenic complications. The patient spent a total of 21 days in the Intensive Care Unit (ICU) before being discharged to a step-down ward. Conclusion: Our experience suggests that multimodal therapy, including transplant hepatectomy, plasmapheresis and retransplantation of an even non-ABO compatible liver may result in the successful outcome in patients with acute fulminant liver failure complicating OLT.

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