Angelo Di Giorgio1, Paola De Angelis2, Maurizio Cheli3, Pietro Vajro4, Raffaele Iorio5, Mara Cananzi6, Silvia Riva7, Giuseppe Maggiore8, Giuseppe Indolfi9, Pier Luigi Calvo10, Emanuele Nicastro1, Lorenzo D'Antiga11. 1. Paediatric Liver, GI and Transplantation, Hospital Papa Giovanni XXIII Bergamo, Bergamo, Italy. 2. Paediatric Surgery and Endoscopy, Ospedale Pediatrico Bambino Gesù, Roma, Italy. 3. Paediatric Surgery, Hospital Papa Giovanni XXIII Bergamo, Bergamo, Italy. 4. Department of Medicine, Surgery and Dentistry "Scuola Medica Salernitana" Section of Pediatrics, University of Salerno, Baronissi (Salerno), Italy. 5. Paediatric Liver Unit, Department of Translational Medical Science, University of Naples Federico II, Naples, Italy. 6. Unit of Pediatric Gastroenterology and Hepatology, Dpt. of Women's and Children's Health, University Hospital of Padova, Italy. 7. Paediatric department and transplantation, Ismett, Palermo, Italy. 8. Paediatric Section of the Department of Medical Sciences, University of Ferrara, Ferrara, Italy. 9. Paediatric Liver, Ospedale Pediatrico Meyer, Firenze, Italy. 10. Paediatric Gastroenterology, Department of Pediatrics, Azienda Ospedaliera-Universitaria Città della Salute e della Scienza di Torino, University of Torino, Torino, Italy. 11. Paediatric Liver, GI and Transplantation, Hospital Papa Giovanni XXIII Bergamo, Bergamo, Italy. Electronic address: ldantiga@asst-pg23.it.
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.
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.
Authors: Al-Faraaz Kassam; Gillian R Goddard; Michael E Johnston; Alexander R Cortez; Andrew T Trout; Todd M Jenkins; Alexander G Miethke; Kathleen M Campbell; Jorge A Bezerra; William F Balistreri; Jaimie D Nathan; Maria H Alonso; Gregory M Tiao; Alexander J Bondoc Journal: Hepatol Commun Date: 2020-07-16