The outcomes of pediatric-onset portal hypertension are unknown. The goal of this study was to look at the long-term outcomes of pediatric-onset portal hypertension complicated by varices in a population. From 1987 to 2013, all children with esophageal varices were identified from 14,144 single countrywide referral center endoscopic reports and tracked down using national health care and mortality records. A questionnaire was distributed to survivors, and 65 answered. Among the 19 underlying conditions were biliary atresia, extrahepatic portal vein blockage, autosomal recessive polycystic kidney disease, and others. During a median follow-up of 15.2 years, patients had 9 upper gastrointestinal endoscopies. At a median age of 4.0 years, 112 patients received a median of 6 sclerotherapy/banding procedures, and 61 experienced a median of 2 variceal bleeding episodes. A total of 48 surgical shunt operations were done on 41 patients, with 38 percent requiring a liver transplant. Four individuals were diagnosed with portal hypertensive biliopathy. Two patients required liver transplants due to hepatopulmonary syndrome, two due to hepatic encephalopathy, and one due to hepatorenal syndrome. On a scale of 1 to 10, patients’ perceived health was a 9, and 86% reported no ongoing symptoms related to esophageal varices.

Pediatric-onset portal hypertension is a diverse illness with high long-term morbidity, necessitating a multimodal strategy with large resources and continued follow-up in adulthood. Although death from variceal bleeding was averted, bleeding episodes recurred in maturity, although the patient-reported health of long-term survivors was positive.