Evaluation of Ten Pediatric Patients With Congenital Extrahepatic Portosystemic Shunt Surgically Treated

Abstract

Purpose: A congenital portosystemic shunt (CPSS) may cause hyperammonemia, pulmonary vascular problems, and hepatic nodules such as focal nodular hyperplasia (FNH). We evaluated our CPSS patients treated surgically.

Methods: Between January 2004 and October 2018, ten CPSS patients were surgically treated. Age and episode for diagnosis, type of shunt, clinical manifestations, indication, age at surgery, mode of surgery, and prognosis were evaluated.

Results: There were three boys and seven girls with the median age of 0 months ranging from 0 to 5 years old. The most frequent reason for diagnosis was a positive result in neonatal mass screening for galactosemia. The types of shunts were port-caval shunt in five and patent ductus venosus in four. Regarding clinical manifestations, portopulmonary hypertension (PPHTN) was observed in five, hepatopulmonary syndrome (HPS) in two, and rapid growth of FNH in two. In patients with PPHTN, liver transplantation was performed in three and shunt ligation in two. In those with HPS, liver transplantation was performed in one, shunt ligation in one, and left lateral segmentectomy in two with rapid growth of FNH. One patient with HPS treated by shunt ligation showed recurrence of the intrahepatic portosystemic shunt after the ligation and PPHTN occurred. Liver transplantation was required four years after ligation. After surgical treatment, all the patients with pulmonary vascular problems improved, and four patients who received liver transplantation became free from medication. Two with FNH treated surgically have been doing well without recurrence.

Conclusions: Surgical treatments were effective for CPSS patients. The indication and method of operation should be determined individually on the basis of the clinical status of patients and the morphology of the shunt.