Abstract: Objective To analyze the clinical characteristics, surgical intervention strategies,, and prognostic outcomes of 40 pediatric patients diagnosed with portal vein cavernous transformation. Methods Between June 2017 and June 2022, 40 pediatric patients diagnosed with portal vein cavernous transformation admitted to our hospital were subjected to a comprehensive analysis of their clinical manifestations, a summary of the surgical treatments administered, and an evaluation of their subsequent outcomes. Results In children, cavernous degeneration of the portal vein typically manifests with clinical symptoms such as abdominal pain, bloating, and splenomegaly, often accompanied by alterations in liver function indicators and esophageal varices as revealed through imaging examination. Forty pediatric cases treated surgically for portal vein cavernous transformation at our institution were retrospectively analysed. The cohort comprised 28 cases (70.0%) undergoing Rex shunt prodedure, 8 cases (20.0%) receiving splenorenal shunt surgery, and 4 cases (10.0%) treated with liver transplantation. Postoperatie survival was 100% among the 40 children. Follow-up assessments indicated that of the children who received Rex shunt, 25 (62.5%) exhibited patent bypass vessels with normal blood flow velocity and no stenosis, while 3 (7.5%) experienced postoperative vascular occlusion. In the splenorenal shunt cohort, 3 (7.5%) did not show a reduction in splenic size or alleviation of esophageal varices, though no cases of hematemesis or melena were reported. Among the liver transplantation recipients, 1 child(2.5%) developed early postoperative hepatic artery thrombosis, which was successfully treated with anticoagulant therapy. Conclusion The clinical manifestations of cavernous transformation of the portal vein in pediatric patients exhibit distinct characteristics. Surgical interventions are tailored based on the developmental status of the portal vein both intra- and extrahepatically. Among the availaboe surgical options, the Rex shunt procedure is notable for its ability to restore the normal anatomy and physiological function of the portal vein system, enhace liver perfusion, and mitigate adverse postoperative outcomes.

Key words: Children, Cavernous trasformation of portal vein, Clinical manifestations, Surgical treatment, Reversion