2013, Number S1
Ann Hepatol 2013; 12 (S1)
Guidelines for the diagnosis and treatment of extrahepatic portal vein obstruction (EHPVO) in children
Flores-Calderón J, Morán-Villota S, Solange-Heller R, Nares-Cisneros J, Zárate-Mondragón F, González-Ortiz B, Chávez-Barrera JA, Vázquez-Frías R, Martínez-Marín EJ, Marín-Rentería N, Bojórquez-Ramos MC, Castillo-De León YA, Ortiz-Galván RC, Varela-Fascinetto G
Language: English
References: 140
Page: 3-24
PDF size: 210.80 Kb.
ABSTRACT
Introduction. Extrahepatic portal vein obstruction is an important cause of portal hypertension among
children. The etiology is heterogeneous and there are few evidences related to the optimal treatment.
Aim and methods. To establish guidelines for the diagnosis and treatment of EHPVO in children, a group of
gastroenterologists and pediatric surgery experts reviewed and analyzed data reported in the literature
and issued evidence-based recommendations.
Results. Pediatric EHPVO is idiopathic in most of the cases.
Digestive hemorrhage and/or hypersplenism are the main symptoms. Doppler ultrasound is a non-invasive
technique with a high degree of accuracy for the diagnosis. Morbidity is related to variceal bleeding, recurrent
thrombosis, portal biliopathy and hypersplenism. Endoscopic therapy is effective in controlling
acute variceal hemorrhage and it seems that vasoactive drug therapy can be helpful. For primary prophylaxis
of variceal bleeding, there are insufficient data for the use of beta blockers or endoscopic therapy.
For secondary prophylaxis, sclerotherapy or variceal band ligation is effective; there is scare evidence
to recommend beta-blockers. Surgery shunt is indicated in children with variceal bleeding who fail endoscopic
therapy and for symptomatic hypersplenism; spleno-renal or meso-ilio-cava shunting is the alternative
when Mesorex bypass is not feasible due to anatomic problems or in centers with no experience.
Conclusions.
Prospective control studies are required for a better knowledge of the natural history of EHPVO, etiology
identification including prothrombotic states, efficacy of beta-blockers and comparison with endoscopic
therapy on primary and secondary prophylaxis.
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