title: Living donor liver transplantation for congenital absence of the portal vein.
abstract: The congenital absence of the portal vein (CAPV) is a rare venous malformation in which mesenteric venous blood drains directly into the systemic circulation. Liver transplantation (OLT) may be indicated for patients with symptomatic CAPV refractory to medical treatment, especially due to hyperammonemia, portosystemic encephalopathy, hepatopulmonary syndrome, or hepatic tumors. Because portal hypertension and collateral circulation do not occur with CAPV, significant splanchnic congestion may occur when the portocaval shunt is totally clamped during portal vein (PV) reconstruction in OLT. This phenomenon results in severe bowel edema and hemodynamic instability, which negatively impact the patient's condition and postoperative recovery. We have successfully reconstructed the PV in living donor liver transplantation (LDLT) using a venous interposition graft, which was anastomosed end-to-side to the portocaval shunt by a partial side-clamp, using a patent round ligament of the liver, which was anastomosed end-to-end to the graft PV with preservation of both the portal and caval blood flows. Owing to the differences in anatomy among patients, at LDLT for CAPV liver transplant surgeons should seek to preserve both portal and caval blood flows.
authors.affiliation: Department of Transplant Surgery, Jichi Medical University, 3311-1, Yakushiji, Shimotsuke City, Tochigi, 329-0498, Japan. yuki371@jichi.ac.jp
authors.forename: Y
authors.initials: Y
authors.lastname: Sanada
authors.forename: K
authors.initials: K
authors.lastname: Mizuta
authors.forename: Y
authors.initials: Y
authors.lastname: Kawano
authors.forename: S
authors.initials: S
authors.lastname: Egami
authors.forename: M
authors.initials: M
authors.lastname: Hayashida
authors.forename: T
authors.initials: T
authors.lastname: Wakiya
authors.forename: M
authors.initials: M
authors.lastname: Mori
authors.forename: S
authors.initials: S
authors.lastname: Hishikawa
authors.forename: K
authors.initials: K
authors.lastname: Morishima
authors.forename: T
authors.initials: T
authors.lastname: Fujiwara
authors.forename: Y
authors.initials: Y
authors.lastname: Sakuma
authors.forename: M
authors.initials: M
authors.lastname: Hyodo
authors.forename: Y
authors.initials: Y
authors.lastname: Yasuda
authors.forename: E
authors.initials: E
authors.lastname: Kobayashi
authors.forename: H
authors.initials: H
authors.lastname: Kawarasaki
mesh_terms: D000714:Anastomosis, Surgical; D002675:Child, Preschool; D005260:Female; D006498:Hepatectomy; D006801:Humans; D022124:Hyperammonemia / Q000209:etiology; D007805:Language Development Disorders / Q000209:etiology / Q000601:surgery; D016031:Liver Transplantation / Q000379:methods*; D019520:Living Donors; D008297:Male; D011169:Portal Vein / Q000002:abnormalities* / Q000601:surgery*; D013152:Splanchnic Circulation; D016896:Treatment Outcome; D014682:Vena Cava, Inferior / Q000601:surgery