Ann Liver Transplant.  2023 May;3(1):35-43. 10.52604/alt.23.0004.

Living donor liver transplantation with graft dextro-rotation and vein interposition in a pediatric patient with congenital absence of the portal vein

• Namgoong JM
• Hwang S
• Park GC
• Kim SH
• Kim KM
• Oh SH

• KMID: 2560920
• DOI: http://doi.org/10.52604/alt.23.0004

Abstract

Congenital absence of the portal vein (CAPV) is a rare venous malformation in which the mesenteric venous blood drains directly into the systemic circulation. We report a case of pediatric living donor liver transplantation (LDLT) for CAPV with a large portocaval shunt (PCS). A 10-year-old boy was diagnosed with CAPV at the age of 9 years. Multiple liver nodules and hepatopulmonary syndrome were progressed, therefore, we decided to perform LDLT. The graft was a left liver graft from the 45-year-old father of the patient. The recipient hepatectomy was performed according to the standard procedures of pediatric LDLT with isolation of a PCS vein. Unification venoplasty for graft outflow vein orifice was performed because of segment VIII vein at the donor liver. Iliac vein interposition was performed for portal vein extension. Graft dextro-rotation and portal vein interposition were used for anatomy-compliant vascular reconstruction. The patient recovered from the LDLT operation. The reconstructed graft hepatic vein and portal vein were maintained well without hemodynamic abnormality. This patient has been doing well for 2 months after the LDLT. In conclusion, since CAPV patients show various types of PCS, individualized portal vein reconstruction should be performed after thorough anatomical assessment before and during the liver transplantation operation.

Keyword

Portal vein agenesis; Portocaval shunt; Interposition graft; Iliac vein homograft; Dextro-rotation