This blog entry has abbreviated/summarized this presentation. Though not intentional, some important material is likely to have been omitted; in addition, transcription errors are possible as well.
One of the topics debated at this year’s meeting was the issue of whether it is worthwhile for patients with esophageal varices to undergo primary prophylaxis.
Here’s a summary:
Esophageal Banding: Proactive vs Expectant Waiting Maureen Jonas (Boston Children’s) and Karen Murray (Seattle Children’s)
Reviewed definitions of portal hypertension. Hepatic venous portal gradient (HVPG) >12 associated with variceal bleeding is the standard in adult medicine.
Management issues: primary prophylaxis, treatment of acute bleeding, and secondary prophylaxis.
- 1-year rate of first bleeding 5% for small varices and 15% for large varices
- 1-year recurrent variceal bleeding ~60%
- Compensated cirrhotics with small high-risk varices (or mod-large varices): consider treatment with beta-blocker (and/or EVL for mod-large varices).
- Beta-blockers and EVL –similar efficacy and survival in adults.
- Lowering HVPG by 20% lowers risk of complications
- Beta-blockers stopped in ~20% of adults due to side effects like fatigue or shortness of breath.
Pediatrics and Beta-Blockers:
- Beta-blockers have good safety in children in a wide range of conditions –cardiomyopathy, migraines, others. HVPG is used in adults but is very invasive.
- Pediatric HVPG correlation to variceal development is not yet established.
- Bleeding from varices –17-29% in biliary atresia (BA) patients over 10 years. Yearly rates: 2-9%.
- Mortality in pediatrics from bleeding varices: 2-5% with BA, 0-2% with portal vein thrombosis.
- There are adverse effects: hypotension, bronchospasm, hypoglycemia. Am Gastroenterol 2014; 27: 20-6. In infants/pediatric patients with shock, tachycardia is the primary response. Beta-blockers interfere with this.
- In pediatric studies, bleeding risk has not been proven to be reduced with non-selective Beta-Blockers.
Risks of primary prophylaxis with banding or sclerotherapy:
- Adverse effects: could convert a child not prone to bleeding into one prone to bleeding. Stricture possible.
- Efficacy? Limited data. Study on prophylactic sclerotherapy if grade 2/3. Median followup was only 1.7 years. JPGN 2012; 55:574
- Sometimes cannot eradicate varices and/or recur quickly. Gastroenterol 2013; 145: 801.
- Sometimes we have to extrapolate from adult data
- Currently about half of pediatric GIs use primary prophylaxis in these cases (JPGN 2011; 52: 751)
Take-home message: insufficient data to demonstrate efficacy of primary prophylaxis as well as to demonstrate adverse effects of primary prophylaxis.
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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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