|Etiology and long-term outcome of extrahepatic portal vein obstruction in children.|
|PMID: 20954284 Owner: NLM Status: MEDLINE|
|AIM: To study the management and outcome of children with extrahepatic portal vein obstruction (EHPVO) in a whole country population.
METHODS: A nationwide multicenter retrospective case series of children with EHPVO was conducted. Data on demographics, radiographic studies, laboratory workup, endoscopic and surgical procedures, growth and development, were extracted from the patients' charts. Characteristics of clinical presentation, etiology of EHPVO, management and outcome were analyzed.
RESULTS: Thirty patients, 13 males and 17 females, 19 (63.3%) Israeli and 11 (36.7%) Palestinians, were included in the analysis. Age at presentation was 4.8 ± 4.6 years, and mean follow-up was 4.9 ± 4.3 years. Associated anomalies were found in 4 patients. The incidence of EHPVO in Israeli children aged 0-14 years was 0.72/million. Risk factors for EHPVO were detected in 13 (43.3%) patients, including 9 patients (30%) with perinatal risk factors, and 4 patients (13.3%) with prothrombotic states: two had low levels of protein S and C, one had lupus anticoagulant, and one was homozygous for methyltetrahydrofolate reductase mutations. In 56.6% of patients, no predisposing factors were found. The most common presenting symptoms were an incidental finding of splenomegaly (43.3%), and upper gastrointestinal bleeding (40%). No differences were found between Israeli and Palestinian children with regard to age at presentation, etiology and clinical symptoms. Bleeding occurred in 18 patients (60%), at a median age of 3 years. Sclerotherapy or esophageal banding was performed in 20 patients. No sclerotherapy complications were reported. Portosystemic shunts were performed in 11 patients (36.6%), at a median age of 11 (range 3-17) years: splenorenal in 9, mesocaval in 1, and a meso-Rex shunt in 1 patient. One patient underwent splenectomy due to severe pancytopenia. Patients were followed up for a median of 3 (range 0.5-15) years. One patient died aged 3 years due to mucopolysaccharidase deficiency type III. None of the patients died due to gastrointestinal bleeding.
CONCLUSION: EHPVO is a rare disorder. The etiological factors are still mostly unknown, and the endoscopic and surgical treatment options ensure a good long-term prognosis.
|Batia Weiss; Eyal Shteyer; Asaf Vivante; Drora Berkowitz; Shimon Reif; Zvi Weizman; Yoram Bujanover; Rivka Shapiro|
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|Type: Journal Article; Multicenter Study|
|Title: World journal of gastroenterology : WJG Volume: 16 ISSN: 2219-2840 ISO Abbreviation: World J. Gastroenterol. Publication Date: 2010 Oct|
|Created Date: 2010-10-18 Completed Date: 2011-02-01 Revised Date: 2014-05-20|
Medline Journal Info:
|Nlm Unique ID: 100883448 Medline TA: World J Gastroenterol Country: China|
|Languages: eng Pagination: 4968-72 Citation Subset: IM|
|APA/MLA Format Download EndNote Download BibTex|
Esophageal and Gastric Varices / etiology
Gastrointestinal Hemorrhage / etiology
Hypertension, Portal / etiology
Portasystemic Shunt, Surgical
Vascular Diseases / epidemiology, etiology*, therapy
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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