title: Successful living donor liver transplantation for giant extensive venous malformation.
abstract: We report our success in employing LDLT as a course of treatment for extensive hepatic VM. A 14-yr-old pediatric patient presented at our hospital with nosebleed, fatigability, orthopnea, and abdominal distension. He had a history of right hemicolectomy with primary anastomosis due to VM of the transverse colon at age seven. Coagulation abnormalities were apparent, characterized by high international normalized ratio of prothrombin time, decreased fibrinogen level, increased FDPs, and D-dimer. T2-weighted magnetic resonance imaging revealed numerous, variable-sized high signal intensity nodules. Abdominal ultrasonography and CT scan showed hepatomegaly with multiple hypo-echogenic lesions and arteriovenous shunting in the liver. Doppler ultrasound showed hypokinetic flow in the hypo-echogenic lesions of liver. Immediate LDLT was performed to avoid spontaneous rupture and DIC. The right lobe of the liver was implanted with temporary portocaval shunt to prevent intestinal congestion and bleeding. Pathologic examination of the explanted liver confirmed the presence of an extensive hepatic VM. The postoperative course was uneventful, and the patient remained symptom-free with normal liver function throughout the 12-month follow-up period.
authors.affiliation: Department of Pathology, National Center for Child Health and Development, Tokyo, Japan; Department of Pediatric Surgery, Dokkyo Medical University Koshigaya Hospital, Koshigaya, Saitama, Japan.
authors.forename: Masahiro
authors.initials: M
authors.lastname: Hatanaka
authors.forename: Atsuko
authors.initials: A
authors.lastname: Nakazawa
authors.forename: Natsuko
authors.initials: N
authors.lastname: Nakano
authors.forename: Kentaro
authors.initials: K
authors.lastname: Matsuoka
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Ikeda
authors.forename: Ikumi
authors.initials: I
authors.lastname: Hamano
authors.forename: Seisuke
authors.initials: S
authors.lastname: Sakamoto
authors.forename: Mureo
authors.initials: M
authors.lastname: Kasahara
mesh_terms: D000293:Adolescent; D000328:Adult; D005260:Female; D005338:Fibrin Fibrinogen Degradation Products / Q000378:metabolism; D006503:Hepatic Veins / Q000601:surgery; D006801:Humans; D008099:Liver / Q000000981:diagnostic imaging / Q000502:physiology; D016031:Liver Transplantation / Q000379:methods*; D019520:Living Donors; D008297:Male; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D014463:Ultrasonography; D054079:Vascular Malformations / Q000097:blood / Q000628:therapy*
chemical_list: D005338:Fibrin Fibrinogen Degradation Products; C036309:fibrin fragment D
keywords: Kasabach-Meritt syndrome; hepatic venous malformation; living donor liver transplantation; pediatric liver transplantation