Judith Flores-Calderón1, Segundo Morán-Villota2, Solange-Heller Rouassant3, Jesús Nares-Cisneros4, Flora Zárate-Mondragón5, Beatriz González-Ortiz6, José Antonio Chávez-Barrera7, Rodrigo Vázquez-Frías8, Elsa Janeth Martínez-Marín9, Nora Marín-Rentería10, Maria Del Carmen Bojórquez-Ramos11, Yolanda Alicia Castillo De León11, Roberto Carlos Ortiz-Galván12, Gustavo Varela-Fascinetto13. 1. Department of Gastroenterology, UMAE, Hospital de Pediatria, Centro Médico Nacional Siglo XXI, IMSS. Mexico City, Mexico.. Electronic address: judithflores1@hotmail.com. 2. Gastroenterology and Hepatology Research Laboratory, Hospital de Pediatría, Centro Médico Nacional Siglo XXI, IMSS. Mexico City, Mexico. 3. Centro Nacional para la Salud de la Infancia y la Adolescencia, Secretaría de Salud. Mexico City, Mexico. 4. Division of Pediatric Gastroenterology and Nutrition, UMAE Hospital de Especialidades No. 71, IMSS, Torreón, Coahuila, Mexico. 5. Department of Gastroenterology, Instituto Nacional de Pediatría, SSA. Mexico City, Mexico. 6. Department of Gastroenterology, UMAE, Hospital de Pediatria, Centro Médico Nacional Siglo XXI, IMSS. Mexico City, Mexico. 7. Department of Gastroenterology, UMAE Dr. Gaudencio González Garza, Hospital de Pediatría, IMSS. Mexico City, Mexico. 8. Department of Gastroenterology, Hospital Infantil de México Dr. Federico Gómez, SSA. Mexico City, Mexico. 9. Endoscopy Department, Hospital Infantil de México Dr. Federico Gómez, SSA. Mexico City, Mexico. 10. Pediatric Department, Hospital para el Niño Poblano. Puebla, Mexico. 11. Department of Gastroenterology, UMAE Hospital de Pediatria, Centro Médico de Occidente, IMSS, Guadalajara, Jalisco, México. 12. Department of Transplant Surgery, UMAE Hospital de Pediatría, Centro Médico Nacional Siglo XXI, IMSS. Mexico City, Mexico. 13. Department of Transplant Surgery, Hospital Infantil de México Dr. Federico Gómez, SSA. Mexico City, Mexico.
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.
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.