title: Successful surgical ligation under intraoperative portal vein pressure monitoring of a large portosystemic shunt presenting as an intrapulmonary shunt: report of a case.
abstract: We report a rare case of patent ductus venosus (PDV) with collapsed intrahepatic portal branches and an intrapulmonary shunt. Excellent improvement of the intrahepatic portal vein flow was achieved by ligating the large ductus venosus under intraoperative portal vein pressure (PVP) monitoring. A 3-year-old boy being followed up for hypergalactosemia at a local hospital was found to have mild lip cyanosis, exertional dyspnea, clubbed fingers, and mild liver dysfunction with high levels of transaminase and ammonia. Cardiac catheterization indicated an intrapulmonary shunt with a ratio of 33%. Abdominal ultrasonography and computed tomography showed remarkable communication between the portal vein and the inferior vena cava. We performed laparotomy and successfully ligated the PDV under PVP monitoring. The PVP did not increase until the catheter was removed 7 days postoperatively. The patient's liver function test results returned to normal within 2 weeks. His serum galactose level was 0 mg/dl after drinking milk, and his SpO2 in room air and exertional dyspnea also improved. He was discharged 18 days after his operation, without any complications. We propose that ligation of a PDV under PVP monitoring could be a treatment of choice, bearing in mind that PDV is associated with collapsed intrahepatic portal branches.
authors.affiliation: Department of Transplantation and Immunology, Faculty of Medicine, Kyoto University, Kyoto, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Yagi
authors.forename: Yasutsugu
authors.initials: Y
authors.lastname: Takada
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Fujimoto
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Ogura
authors.forename: Koichi
authors.initials: K
authors.lastname: Kozaki
authors.forename: Mikiko
authors.initials: M
authors.lastname: Ueda
authors.forename: Koichi
authors.initials: K
authors.lastname: Tanaka
mesh_terms: D000015:Abnormalities, Multiple / Q000175:diagnosis / Q000601:surgery*; D000792:Angiography; D001165:Arteriovenous Malformations / Q000175:diagnosis* / Q000601:surgery*; D002675:Child, Preschool; D004374:Ductus Arteriosus, Patent / Q000175:diagnosis / Q000601:surgery; D005500:Follow-Up Studies; D006801:Humans; D008026:Ligation / Q000379:methods; D008297:Male; D016343:Monitoring, Intraoperative / Q000379:methods; D017082:Portal Pressure; D011168:Portal System / Q000002:abnormalities* / Q000601:surgery*; D011169:Portal Vein / Q000601:surgery; D011652:Pulmonary Circulation / Q000502:physiology; D018570:Risk Assessment; D016896:Treatment Outcome; D018608:Ultrasonography, Doppler; D014656:Vascular Surgical Procedures / Q000379:methods; D014682:Vena Cava, Inferior / Q000601:surgery