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Etiology, presenting features and outcome of children with non-cirrhotic portal vein thrombosis: A multicentre national study
- Publication Year :
- 2019
-
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 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.
- Subjects :
- Male
Diseases
Infant, Newborn, Diseases
0302 clinical medicine
Child
Children
Venous Thrombosis
medicine.diagnostic_test
Portal Vein
Gastroenterology
Portal vein thrombosis
Venous thrombosis
Italy
030220 oncology & carcinogenesis
Child, Preschool
Hypertension
Portal hypertension
030211 gastroenterology & hepatology
Female
Gastrointestinal Hemorrhage
Infant, Premature
medicine.medical_specialty
Gastrointestinal bleeding
Adolescent
Socio-culturale
Esophageal and Gastric Varices
Hypersplenism
Catheterization
03 medical and health sciences
Hypertension, Portal
medicine
Humans
Transjugular Intrahepatic
Portasystemic Shunt
Extra-hepatic portal vein obstruction
Non-cirrhotic portal hypertension
Infant
Infant, Newborn
Portasystemic Shunt, Transjugular Intrahepatic
Splenomegaly
Preschool
Survival rate
Premature
Children, Extra-hepatic portal vein obstruction, Non-cirrhotic portal hypertension, Portal vein thrombosis
Hepatology
business.industry
medicine.disease
Newborn
Surgery
Endoscopy
Etiology
Portal
business
Varices
Subjects
Details
- Language :
- English
- Database :
- OpenAIRE
- Accession number :
- edsair.doi.dedup.....c80390fb9f8f02c8b89df7f1b9deb2cd