Etiology, presenting features and outcome of children with non-cirrhotic portal vein thrombosis: A multicentre national study

Angelo Di Giorgio, Paola De Angelis, Maurizio Cheli, Pietro Vajro, Raffaele Iorio, Mara Cananzi, Silvia Riva, Giuseppe Maggiore, Giuseppe Indolfi, Pier Luigi Calvo, Emanuele Nicastro, Lorenzo D'Antiga

Research output: Contribution to journalArticlepeer-review

Abstract

OBJECTIVES: Non-cirrhotic portal vein thrombosis (PVT) is a main cause of portal hypertension in children. We describe the characteristics at presentation and outcome of a cohort of patients with PVT to determine clinical features and predictors of outcome.

METHODS: We recorded: (1) Associated factors: prematurity, congenital malformations, neonatal illnesses, umbilical vein catheterization (UVC), deep infections, surgery; (2) congenital and acquired prothrombotic disorders; (3) features at last follow up including survival rate and need for surgery.

RESULTS: 187 patients, mean age at diagnosis 4 ± 3.7 years, had a history of prematurity (61%); UVC (65%); neonatal illnesses (79%). The diagnosis followed the detection of splenomegaly (40%), gastrointestinal bleeding (36%), hypersplenism (6%), or was incidental (18%). Of 71 patients who had endoscopy at presentation 62 (87%) had oesophageal varices. After 11.3 years' follow up 63 (34%) required surgery or TIPS. Ten-year survival rate was 98%, with 90% shunt patency. Spleen size, variceal bleeding and hypersplenism at presentation were predictors of surgery or TIPS (p < 0.05).

CONCLUSION: PVT is associated with congenital and acquired co-morbidities. History of prematurity, neonatal illnesses and UVC should lead to rule out PVT. Large spleen, variceal bleeding and hypersplenism at presentation predict the need for eventual surgery in a third of cases.

Original languageEnglish
JournalDigestive and Liver Disease
DOIs
Publication statusE-pub ahead of print - Mar 6 2019

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