Document Detail


Etiology and long-term outcome of extrahepatic portal vein obstruction in children.
MedLine Citation:
PMID:  20954284     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Batia Weiss; Eyal Shteyer; Asaf Vivante; Drora Berkowitz; Shimon Reif; Zvi Weizman; Yoram Bujanover; Rivka Shapiro
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Publication Detail:
Type:  Journal Article; Multicenter Study    
Journal Detail:
Title:  World journal of gastroenterology : WJG     Volume:  16     ISSN:  2219-2840     ISO Abbreviation:  World J. Gastroenterol.     Publication Date:  2010 Oct 
Date Detail:
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    
Other Details:
Languages:  eng     Pagination:  4968-72     Citation Subset:  IM    
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Child
Child, Preschool
Endoscopy
Esophageal and Gastric Varices / etiology
Female
Gastrointestinal Hemorrhage / etiology
Humans
Hypertension, Portal / etiology
Incidence
Infant
Infant, Newborn
Israel
Male
Portal Vein*
Portasystemic Shunt, Surgical
Retrospective Studies
Risk Factors
Sclerotherapy
Splenectomy
Time Factors
Treatment Outcome
Vascular Diseases / epidemiology,  etiology*,  therapy
Comments/Corrections

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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