A consensus report on portal hypertension has helpful advice on a broad range of management issues and should be kept in mind as a handy reference (Pediatr Transplantation 2012; 16: 426-37). The report is concise and full of bullet points. It is based on a meeting of pediatric experts to modify adult guidelines (Baveno V) for pediatrics.
In many instances, the experts indicate that there is not enough pediatric data. Specific subjects include the following (along with some points):
- Treatment options for portal hypertension -consider screening for varices if thrombocytopenia and splenomegaly. ‘No indication to use beta-blockers to prevent varices.’
- Prevention of first bleeding episode -in the presence of varices (grade II or III), variceal ligation reasonable in selected children and/or within context of defined research protocols. Grade I varices can be flattened with insufflation, and grade III varices are confluent around circumference of esophagus (per Japanese Research Society for Portal HTN analysis)
- Role of hepatic venous pressure gradient measurement (HVPG) -‘panel was undecided as to whether HVPG measurements in children’ should be ‘part of specialized clinical practice or’ a research tool.
- Blood volume restitution -suggests use of platelets in cases of bleeding with profound thrombocytopenia (<20,000).
- Antibiotic prophylaxis -unclear whether empiric antibiotics in children are needed in the presence of variceal bleeding.
- Management of treatment failures -can retry endoscopy and if fails, consider transjugular intrahepatic portosystemic shunting (TIPS)
- Management of gastric varices -only case reports in children, thus no evidence-based recommendations.
- Prevention of rebleeding -variceal ligation (EVL) preferred in patients with cirrhosis. EVL should be performed every 2-4 weeks up to five sessions to eradicate varices after 1st bleed.
- Treatment of portal vein obstruction -diagnosis, natural history, anticoagulation, use of MesoRex bypass procedure, associated portal biliopathy -diagnosis and treatment. With regard to MesoRex, ‘controversy exists as to the appropriateness of ..this procedure in an asymptomatic child.’ Surveillance endoscopy may assist in decision-making.
- Hypersplenism with portal vein obstruction-in the presence of platelet count <50,000 and portal vein obstruction, strong consideration should be given to MesoRex procedure.
- Portopulmonary hypertension and hepatopulmonary syndrome -important to monitor oxygen saturation in patients with portal vein obstruction/other causes of portal hypertension. If <97%, additional investigation may be needed. Portopulmonary hypertension is best characterized with cardiac catheterization and hepatopulmonary syndrome with saline echocardiography.
- Other topics: Prevention of hepatic encephalopathy, managing bleeding episodes, endoscopic treatment
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