A successful treatment of positive cross-match by treatment with postoperative repeated donor whole blood administration via portal vein in adult, living related partial liver transplantation: 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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Living-related liver transplant to treat epithelioid hemangioendothelioma – a case report

Brazilian Journal of Transplantation ◽

10.53855/bjt.v8i4.383 ◽

2005 ◽

Vol 8 (4) ◽

pp. 449-451

Author(s):

Marcelo A. F. Ribeiro Jr ◽

Christian Evangelista Garcia ◽

Telma Eugênio dos Santos ◽

Adavio de Oliveira e Silva ◽

Regina Leitão ◽

...

Keyword(s):

Liver Transplantation ◽

Case Report ◽

Liver Transplant ◽

Soft Tissues ◽

Liver Tumors ◽

Epithelioid Hemangioendothelioma ◽

Low Grade ◽

Living Related Liver Transplant ◽

Transplantation Technique ◽

Living Related

Introduction: Epithelioid hemangioendothelioma of liver is a rare, low-grade neoplasm of vascular origin that has an unpredictable malignant potential. It preferentially arises in soft tissues and bones, and seldom in the liver. Biologically, it trends to be multi-focal and often unresectable. Objective: The outcome of a patient submitted to a living-related liver transplant to treat epithelioid hemangioendothelioma of the liver. Case report: The reported case involves a 39-year old female patient with an immense epithelioid hemangioendothelioma of the liver, involving segments III, IV, V, VII, and VIIl. The prognostic to any kind of treatment was considered unfavorable, and the decision of submitting her to a liver transplant was made. It was suggested a living-related liver transplant. The donor was the patient’s 36-year old brother, who donated his right liver lobe (segments V, VI, VII, VIII). The recipient´s original liver was large, presenting very solid consistence and no node evidence. The transplant itself was performed using the conventional living-related liver transplantation technique. The recovery was good, and she was discharged from hospital on the 15th postoperative day using micophenolate mofetil, tacrolimus, and corticoid. Conclusion: Liver transplantation for epithelioid hemangioendothelioma of the liver can be performed with acceptable survival rate. The living related liver transplant opens a great perspective to that group of patients presenting liver tumors and who are unfeasible to wait for an organ on a waiting list.

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