Primary prophylaxis of variceal bleeding in children and the role of MesoRex Bypass: Summary of the Baveno VI Pediatric Satellite Symposium

Abstract

Approaches to the management of portal hypertension and variceal hemorrhage in pediatrics remain controversial, in large part because they are not well informed by rigorous clinical studies. Fundamental biological and clinical differences preclude automatic application of approaches used for adults to children. On April 11-12, 2015, experts in the field convened at the first Baveno Pediatric Satellite Meeting to discuss and explore current available evidence regarding indications for MesoRex bypass (MRB) in extrahepatic portal vein obstruction and the role of primary prophylaxis of variceal hemorrhage in children. Consensus was reached regarding MRB. The vast majority of children with extrahepatic portal vein obstruction will experience complications that can be prevented by successful MRB surgery. Therefore, children with extrahepatic portal vein obstruction should be offered MRB for primary and secondary prophylaxis of variceal bleeding and other complications, if appropriate surgical expertise is available, if preoperative and intraoperative evaluation demonstrates favorable anatomy, and if appropriate multidisciplinary care is available for postoperative evaluation and management of shunt thrombosis or stenosis. In contrast, consensus was not achieved regarding primary prophylaxis of varices. Although variceal hemorrhage is a concerning complication of portal hypertension in children, the first bleed appears to be only rarely fatal and the associated morbidity has not been well characterized

Diagnosis and treatment guidelines for aberrant portal hemodynamics

“…When symptoms associated with splenomegaly (abdominal pain and compression) or a tendency to bleed and severely decreased blood cells (at least one of the following: platelets, ≤5 × 10 4 ; white blood cells, ≤3000; and red blood cells, ≤300 × 10 4 ) associated with splenomegaly, partial splenic embolization or splenectomy should be considered …”

Novel insights into the assessment of risk of upper gastrointestinal bleeding in decompensated cirrhotic children

“…The risk stratification of bleeding is important to identify those who may benefit from a prophylactic treatment with band ligation or sclerotherapy. Up to now, there is no pediatric recommendation for primary prophylaxis with endoscopic ligation, sclerotherapy, or nonspecific beta‐blockers in children …”

Portal hypertension and its management in children

“…According to recent recommendations for children with PHT based on the Baveno VI consensus, children ‘would be considered’ for surveillance oesophagogastroduodenoscopy (OGD) on the basis of splenomegaly and thrombocytopenia with intention to treat depending on the grade of varices 11. There are two options for endoscopic treatment: endoscopic sclerotherapy (EST) or endoscopic variceal ligation (EVL).…”

“…However, a recent international survey of specialised paediatric liver units showed that 70% of paediatric centres would perform surveillance OGDs and instigate primary prophylaxis 44. According to current recommendations for children with PHT, based on the Baveno VI consensus, children ‘would be considered’ for surveillance OGD on the basis of hypersplenism with intention to treat depending on endoscopy findings 11. To our knowledge, the decision for a surveillance OGD in children is based on either evidence of splenomegaly on the background of CLD or PVT or radiological evidence of PHT with limited data on the use of predictor models for bleeding risk or presence of varices 45…”

“…PHT is the the most common cause of severe GI bleeding in children and may be the first presenting feature of liver disease 11. Families of children with varices (or suspected varices) should be provided with a written plan for this scenario and all paediatric GI units should have an established local pathway for its management.…”