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Presinusoidal extrahepatic portal hypertension: a review of thirty-five cases variously treated
Insights
Extrahepatic portal hypertension in children often causes gastrointestinal bleeding. Surgical interventions, including portosystemic shunts and esophagectomy with gastrectomy, offer effective management for affected pediatric patients.
Area of Science:
- Pediatric Surgery
- Hepatology
- Vascular Surgery
Background:
- Extrahepatic portal hypertension (EHPH) is a rare but serious condition in children.
- It often presents with significant gastrointestinal bleeding due to varices.
- Understanding the etiology, clinical course, and treatment outcomes is crucial for effective management.
Observation:
- This review analyzed 35 children with proven pre-sinusoidal EHPH.
- Gastrointestinal bleeding was a predominant symptom, affecting 33 patients.
- Surgical intervention was performed in 31 cases, with no operative mortality.
Findings:
- Portosystemic venous shunts were successful in 12 out of 21 patients.
- For "unshuntable" patients, esophagectomy and proximal partial gastrectomy with colon substitution showed effectiveness in controlling hemorrhage.
- Recurrent bleeding is common, necessitating definitive treatment in older children.
Implications:
- Early diagnosis and appropriate surgical strategy are vital for managing pediatric EHPH.
- The study highlights effective surgical options for both shuntable and unshuntable cases.
- Long-term follow-up is essential to monitor outcomes and prevent recurrent bleeding in children with EHPH.
Abstract:
Thirty-five cases of proven preinusoidal extrahepatic portal hypertension in children have been reviewed with particular reference to etiology, clinical course, therapy, and long-term follow up results. Thirty-three patients has significant gastrointestinal bleeding and 31 were operated upon. There were no operative deaths. Portosystemic venous shunts were successful in 12 of 21 patients. A variety of procedures were used for 15 of the 17 "unshuntable" individuals. Some form of esophagectomy and proximal partial gastrectomy with colon substitution appears to be the most effective operation for control of hemorrhage in the "unshuntable" patients. Expectant treatment is preferred in the infant and younger child, but the older child should have a definitive shunt or direct attack on the varices since recurrent bleeding is usually the rule.