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.

Di Giorgio, A., De Angelis, P., Cheli, M., Vajro, P., Iorio, R., Cananzi, M., et al. (2019). Etiology, presenting features and outcome of children with non-cirrhotic portal vein thrombosis: A multicentre national study. DIGESTIVE AND LIVER DISEASE, 51(8), 1179-1184 [10.1016/j.dld.2019.02.014].

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

D'Antiga L.
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 < 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.
Articolo in rivista - Articolo scientifico
Children; Extra-hepatic portal vein obstruction; Non-cirrhotic portal hypertension; Portal vein thrombosis;
English
2019
51
8
1179
1184
none
Di Giorgio, A., De Angelis, P., Cheli, M., Vajro, P., Iorio, R., Cananzi, M., et al. (2019). Etiology, presenting features and outcome of children with non-cirrhotic portal vein thrombosis: A multicentre national study. DIGESTIVE AND LIVER DISEASE, 51(8), 1179-1184 [10.1016/j.dld.2019.02.014].
File in questo prodotto:
Non ci sono file associati a questo prodotto.

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/10281/479679
Citazioni
  • Scopus 29
  • ???jsp.display-item.citation.isi??? 24
Social impact