Clinical Characteristics, Management, and Outcomes of Pediatric Noncirrhotic Portal Hypertension: A 25-Year Single-Center Cohort

Background/Objectives: To describe the clinical characteristics and outcomes of pediatric noncirrhotic portal hypertension (NCPH), focusing on postoperative growth, hematologic, and biochemical changes. Methods: This retrospective study included children diagnosed with NCPH between 2000 and 2024. Clinical characteristics and treatments were reviewed. Paired preoperative and one-year postoperative measurements were compared using the Wilcoxon signed-rank test. Results: Ninety-four children were included (median diagnostic age, 4 years). Prehepatic disease accounted for 75.5%, predominantly portal vein thrombosis (69.1%). Splenomegaly and thrombocytopenia occurred in 88.3% and 62.8%, respectively. Varices were identified in 76/90 patients (84.4%), and 60/87 (69.0%) experienced gastrointestinal bleeding. Shunt surgery was performed in 53 patients (56.4%), primarily for variceal bleeding (47/53); postoperative bleeding occurred in 3/53 (5.7%). Among 37 patients with paired anthropometric data, median weight SDS increased from −0.56 to 0.00 (p = 0.001) and height SDS from −0.63 to 0.40 (p = 0.002), without a significant change in BMI SDS. Among 48 patients with paired laboratory data, hemoglobin increased from 10.15 to 12.00 g/dL (p < 0.001) and platelets from 83.3 to 112.7 × 103/µL (p = 0.009); white blood cell counts remained unchanged. Total bilirubin increased from 0.71 to 0.91 mg/dL (p = 0.003), without significant changes in albumin or INR. Bilirubin changes did not differ significantly between distal splenorenal shunt and Meso-Rex bypass. Conclusions: Shunt surgery was associated with postoperative bleeding in 5.7% of patients and significant improvements in weight, height, hemoglobin, and platelet counts at one year. Bilirubin increased without evidence of deteriorating hepatic synthetic function; its clinical significance remains uncertain.