Primary prophylaxis of bleeding in children with portal hypertension is safe for treatment of cirrhosis associated with high-risk varices, according to Mathieu Duché, MD, of Hôpital Bicêtre in France, and his associates.
In a study from July 1989 to June 2014, researchers examined 1,300 children with various causes of liver disease, based on the presence of palpable splenomegaly and/or ultrasonographic signs of portal hypertension. During the study, a high-risk pattern – including grade 3 esophageal varices, grade 2 varices with red markings and/or gastric varices along the cardia, or gastric varices with or without esophageal varices – was present in 96% of the 246 children who bled spontaneously and in 11% of the 872 children who did not bleed. Of the 246 children who bled spontaneously, 170 children with high-risk varices underwent primary prophylaxis of bleeding with portal surgery, endoscopic banding/sclerotherapy of varices, or interventional radiology.
In 50 children with noncirrhotic causes of portal hypertension and the high risk varices, those with portal vein obstruction underwent portal surgery (Rex bypass or portosystemic shunt) as primary prophylaxis. Endoscopic banding or sclerotherapy was performed instead in younger children or when angiograms did not favor the surgery. One child who had a severe stenosis of the portal trunk underwent successful interventional radiology. After a mean follow-up of 5.5 years after primary prophylaxis, all these patients are alive.
Of the 120 children with cirrhosis with high risk varices, 10 children with well compensated cirrhosis received a portosystemic shunt; thrombosis of the shunt occurred in 1 child who later underwent liver transplantation, and 1 child with a patent shunt developed portosystemic encephalopathy and underwent liver transplantation. No gastrointestinal bleeding was recorded in these 10 children who were alive at the last follow-up.
Of 110 children who underwent endoscopic banding or sclerotherapy, in 76 children there was eradication of varices; there was a relapse of high-risk varices in 20 children, so further endoscopic was needed. In two children who initially underwent endoscopic treatment and achieved eradication of varices, a protein-losing enteropathy and bleeding from ectopic varices, respectively, required subsequent treatment by a surgical portosystemic shunt. Of the 34 children in whom no eradication was obtained with this primary prophylaxis, 3 died and 29 underwent liver transplantation before eradication could be obtained; 1 was lost to follow-up and 1 received a transjugular intrahepatic portosystemic shunt. After a mean follow-up of 5 years, 8 of the 120 children with cirrhosis who underwent primary prophylaxis have died: 4 children died before transplantation, 2 of septic shock unrelated to endoscopic treatment, and 1 of atrioventricular block during variceal injection of aethoxysklerol. Four other children died after transplantation.
It was noted that no esophageal or gastric perforation was observed as a consequence of endoscopic primary prophylaxis in the pre- or posttransplantation period.
“Although no statistical analysis was performed because this was not a controlled study, the results suggest that, compared with liver transplantation performed directly or with care after a spontaneous bleed, primary prophylaxis of bleeding has a fairly good safety record for the management of children with cirrhosis and high-risk varices,” researchers concluded.
Read the full study in the Journal of Hepatology (doi: 10.1016/j.jhep.2016.09.006).