https://www.ncbi.nlm.nih.gov/pubmed/31990868 Portal hypertension

J Pediatr Gastroenterol Nutr. 2020 Jan 27. doi: 10.1097/MPG.0000000000002643. [Epub ahead of print]
Long-Term Outcomes and Health Perceptions in Pediatric-Onset Portal Hypertension Complicated by Varices.
Luoto T1, Koivusalo A, Pakarinen M.

Abstract

OBJECTIVES:
Outcomes of pediatric-onset portal hypertension are poorly defined. We aimed assess population-based long-term outcomes of pediatric-onset portal hypertension complicated by varices.

METHODS:
All children with esophageal varices (n = 126) were identified from 14144 single nationwide referral center endoscopy reports during 1987-2013, and followed up through national health care and death registers. A questionnaire was sent to survivors (n = 94) of whom 65 (69%) responded.

RESULTS:
Nineteen underlying disorders included biliary atresia (35%), extrahepatic portal vein obstruction (35%), autosomal recessive polycystic kidney disease (7%), and other disorders (23%). During median follow-up of 15.2 (range 0.5-43.1) years patients underwent median 9 (1-74) upper gastrointestinal endoscopies. Esophageal varices were first observed at median age 4.0 (0.3-18.2) years, 112 (89%) patients underwent median 6 (1-56) sclerotherapy/banding sessions, and 61 (48%) experienced median 2 (range 1-20) variceal bleeding episodes. Forty-eight surgical shunt procedures were performed to 41 (36%) patients and 38% underwent liver transplantation. Portal hypertensive biliopathy was diagnosed in 4 patients. Hepatopulmonary syndrome necessitated liver transplantation in 2 patients, hepatic encephalopathy in 2 and hepatorenal syndrome in 1. No patient died of variceal bleeding. Patient reported perception of health on a scale of 1-10 was 9 (range 4-10), and 86% reported no current symptoms attributable to esophageal varices.

CONCLUSIONS:
Pediatric-onset portal hypertension is a heterogeneous disease with significant long-term morbidity, requiring multimodal approach with considerable resources and continuation of follow-up in adulthood. Although mortality to variceal bleeding was avoided, bleeding episodes recurred also in adulthood, while patient reported health of long-term survivors was encouraging.