Hajime Uchida, Masato Shinkai, Hiroomi Okuyama, Takehisa Ueno, Mikihiro Inoue, Toshihiro Yasui, Eiso Hiyama, Sho Kurihara, Yasunasru, Sakuma, Yukihiro Sanada, Akinobu, Taketomi, Shohei Honda, Motoshi Wada, Ryo Ando, Jun Fujishiro, Mariko Yoshida, Yohei Yamada, Hiroo Uchida, Takahisa Tainaka, Mureo Kasahara, Japanese Society of Pediatric Splenology Portalvenology
National Center for Child Health and Development. Kanagawa Children’s Medical Center. Osaka University Graduate School of Medicine. Fujita Health University. Hiroshima University Hospital. Jichi Medical University. Hokkaido University Graduate School of Medicine. Tohoku University Graduate School of Medicine. University of Tokyo. Keio University School of Medicine. Nagoya University Graduate School of Medicine.
Japan
Journal of Pediatric Surgery
J Pediatr Surg 2024;
DOI: 10.1016/j.jpedsurg.2024.05.008
Abstract
Background: Although congenital portosystemic shunts (CPSSs) are increasingly being recognized, the optimal treatment strategies and natural prognosis remain unclear, as individual CPSSs show different phenotypes.
Methods: The medical records of 122 patients who were diagnosed with CPSSs at 15 participating hospitals in Japan between 2000 and 2019 were collected for a retrospective analysis based on the state of portal vein (PV) visualization on imaging.
Results: Among the 122 patients, 75 (61.5%) showed PV on imaging. The median age at the diagnosis was 5 months. The main complications related to CPSS were hyperammonemia (85.2%), liver masses (25.4%), hepatopulmonary shunts (13.9%), and pulmonary hypertension (11.5%). The prevalence of complications was significantly higher in patients without PV visualization than in those with PV visualization (P < 0.001). Overall, 91 patients (74.6%) received treatment, including shunt closure by surgery or interventional radiology (n = 82) and liver transplantation (LT) or liver resection (n = 9). Over the past 20 years, there has been a decrease in the number of patients undergoing LT. Although most patients showed improvement or reduced progression of symptoms, liver masses and pulmonary hypertension were less likely to improve after shunt closure. Complications related to shunt closure were more likely to occur in patients without PV visualization (P = 0.001). In 25 patients (20.5%) without treatment, those without PV visualization were significantly more likely to develop complications related to CPSS than those with PV visualization (P = 0.011).
Conclusion: Patients without PV visualization develop CPSS-related complications and, early treatment using prophylactic approaches should be considered, even if they are asymptomatic.
Category
Class I. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease
Class I. Pulmonary Hypertension Associated with Liver Disease
Symptoms and Findings Associated with Pulmonary Vascular Disease
Surgical and Catheter-mediated Interventions for Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: No