Experiences in clinical management of Abernethy malformation combined with focal nodular hyperplasia / 中华肝胆外科杂志

ABSTRACT

Objective:To summarize clinical experience in the management of Abernethy malformation combined with focal nodular hyperplasia (FNH).Methods:Three patients with Abernethy malformation combined with FNH who were managed in the Department of Hepatobiliary Surgery, the First Medical Center of Chinese PLA General Hospital from January 2010 to December 2018 entered into this study. There were one male aged 35 years and two females aged 21 years and three years and nine months, respectively. Patients underwent digital subtraction angiography (DSA) with balloon dilation, shunt vasulature ligation, portal vein (PV) pressure measurement and other investigations were performed to study the changes of PV pressure before and after treatment.Results:DSA indicated that the congenital portal shunt of the patients was Abernethy deformity Ⅱ and Ⅰb, respectively, and with combined FNH diagnosed preoperatively. One patient was admitted to hospital for partial hepatectomy 5 years ago for the treatment of FNH, which presented with recurrent abdominal pain. The PV pressure increased from 8.5, 9.0, 20.0 cmH 2O(1 cmH 2O=0.098 kPa) to 15.0, 21.0, 25.0 cmH 2O after portal cavity shunt vasculature/portal vein ligation and splenic artery ligation/portal body shunt ligation, respectively. The PV blood flow into the liver increased, and DSA suggested shunt vessel occlusion. On follow-up, all patients survived well. Conclusion:Ligation or constriction of shunt vessels significantly improved the flow of PV into liver in Abernethy malformation patients with FNH. The treatment benefited long-term survival.