Extra-hepatic portal vein thrombosis in children: single center experience

Tawhida Yassin Abdel-Ghaffar; Haidy Zakaria; Suzan El Naghi; Solaf M Elsayed; Alaa Haseeb; Gihan Ahmed Sobhy

doi:10.5114/ceh.2023.125840

eISSN: 2449-8238
ISSN: 2392-1099

Clinical and Experimental Hepatology

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abstract:

Original paper

Extra-hepatic portal vein thrombosis in children: single center experience

Tawhida Yassin Abdel-Ghaffar

^{1, 2}

,

Haidy Mohammed Zakaria

³

,

Suzan El Naghi

^{1, 4}

,

Solaf M Elsayed

^{1, 5}

,

Alaa Haseeb

^{1, 6}

,

Gihan Ahmed Sobhy

^{1, 7}

Dr. Yassin Abdel Ghaffar Charity Center for Liver Disease and Research, 6 Emarat El Tasnieea St. from Makram Ebeid, 11566, Nasr city, Cairo, Egypt
Department of Pediatrics, Pediatric Hepatology, Faculty of Medicine, Ain Shams University, 11591 Cairo, Egypt
Department of Clinical Research and Health Development, Menoufia Directorate of Health Affairs, Ministry of Health and Population, 32511 Shebin El-Kom, Menoufia, Egypt
Department of Pediatrics, National Hepatology and Tropical Medicine Research Institute (NHTMRI), Cairo, Egypt
Medical Genetics Department, Faculty of Medicine, Ain Shams University, 11591 Cairo, Egypt
Department of Gastroenterology, Hepatology and Endemic Medicine, Faculty of Medicine, Beni Suef University, 62511 Beni Suef, Egypt
Department of Pediatric Hepatology, Gastroenterology and Nutrition, National Liver Institute, Menoufia University, 32511 Shebin El-koom, Menoufia, Egypt

Clin Exp HEPATOL 2023; 9, 1: 37-45

DOI: https://doi.org/10.5114/ceh.2023.125840

Online publish date: 2023/03/15

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Aim of the study:
We aimed to discuss our experience in management of children with extra-hepatic portal vein thrombosis (EHPVT).

Material and methods:
This retrospective cohort study included 62 children with EHPVT. All patients’ records were reviewed. The patients’ socio-demographic data, post-natal history, disease presentation and clinical examination were collected. Data from laboratory investigations – complete blood count, liver function tests, renal function tests, abdominal ultrasound/Doppler studies, upper endoscopic findings and treatment regimens – were collected whenever available.

Results:
Of the 62 patients, 62.9% were male and 37.1% were female. The mean age at disease presentation was 3.5 ±2.7 years. The main initial clinical presentation of the disease was hematemesis and/or melena (30 cases; 48.4%). History of umbilical catheterization (UVC) was present in 60% of cases. The thrombophilia profile was assessed in 17 patients, of whom 12 (70.6%) were found to have a coagulation disorder. Splenomegaly was present in 91.7% of the patients. Hematological abnormalities in the form of cytopenias were present in most cases. Ultrasound revealed the presence of collaterals in 76.2%. Upper endoscopy showed the presence of varices in 45 cases, all of which needed endoscopic intervention, while in 11 cases the varices were either low grade or absent and thus were subjected only to medical treatment with propranolol and 6 cases were lost to follow-up. Splenectomy was done in only one case and 2 cases underwent the Rex operation.

Conclusions:
Variceal bleeding is the most common clinical presentation of EHPVT in children. UVC is still the main etiological factor of EHPVT in our cohort especially with presence of thrombophilic disorder.

keywords:

Extra-hepatic portal vein thrombosis in children: single center experience

Tawhida Yassin Abdel-Ghaffar 1, 2 , Haidy Mohammed Zakaria 3 , Suzan El Naghi 1, 4 , Solaf M Elsayed 1, 5 , Alaa Haseeb 1, 6 , Gihan Ahmed Sobhy 1, 7

propranolol, varices, portal vein thrombosis, endoscopic management

Tawhida Yassin Abdel-Ghaffar

^{1, 2}

,

Haidy Mohammed Zakaria

³

,

Suzan El Naghi

^{1, 4}

,

Solaf M Elsayed

^{1, 5}

,

Alaa Haseeb

^{1, 6}

,

Gihan Ahmed Sobhy

^{1, 7}