Portal hypertension in children: High-risk varices, primary prophylaxis and consequences of bleeding.
Primary prophylaxis of bleeding is debated for children with portal hypertension because of the limited number of studies on its safety and efficacy, the lack of a known endoscopic pattern carrying a high-risk of bleeding for all causes, and the assumption that the mortality of a first bleed is low. We report our experience with these issues.



From 1989 to 2014, we managed 1300 children with portal hypertension. Endoscopic features were recorded; high-risk varices were defined as: grade 3 esophageal varices, grade 2 varices with red wale markings, or gastric varices. Two hundred forty-six children bled spontaneously and 182 underwent primary prophylaxis. The results of primary prophylaxis were reviewed as well as bleed-free survival, overall survival and life-threatening complications of bleeding.



High-risk varices were found in 96% of children who bled spontaneously and in 11% of children who did not bleed without primary prophylaxis (p<0.001), regardless of the cause of portal hypertension. Life-threatening complications of bleeding were recorded in 19% of children with cirrhosis and high-risk varices who bled spontaneously. Ten-year probabilities of bleed-free survival after primary prophylaxis in children with high-risk varices were 96% and 72% for non-cirrhotic causes and cirrhosis respectively. Ten-year probabilities of overall survival after primary prophylaxis were 100% and 93% in children with non-cirrhotic causes and cirrhosis respectively.



In children with portal hypertension, bleeding is linked to the high-risk endoscopic pattern reported here. Primary prophylaxis of bleeding based on this pattern is fairly effective and safe.



In children with liver disease, the risk of bleeding from varices in the esophagus is linked to their large size, the presence of congestion on their surface and their expansion into the stomach but not to the child's age nor to the cause of portal hypertension. Prevention of the first bleed in children with high-risk varices can be achieved by surgery or endoscopic treatment, and decreases mortality and morbidity.
Hépatologie Pédiatrique and Centre de Référence National de l'Atrésie des Voies Biliaires, Hôpital Bicêtre, AP-HP and Université Paris-Sud 11, Le Kremlin-Bicêtre 94275, France; Radiologie Pédiatrique, Hôpital Bicêtre, AP-HP, Le Kremlin-Bicêtre 94275, France. Electronic address: mathieu.duche@aphp.fr.
Mathieu
M
Duché
Santé Publique et Èpidémiologie, Hôpital Bicêtre, AP-HP, Le Kremlin-Bicêtre 94275, France; Inserm, CESP Centre for Research in Epidemiology and Population Health, U1018, Epidemiology of Reproduction and Children Development Team, 94276 Le Kremlin-Bicêtre, France; Université Paris-Sud 11, UMRS 1018, 94276 Le Kremlin-Bicêtre, France.
Béatrice
B
Ducot
Hépatologie Pédiatrique and Centre de Référence National de l'Atrésie des Voies Biliaires, Hôpital Bicêtre, AP-HP and Université Paris-Sud 11, Le Kremlin-Bicêtre 94275, France.
Oanez
O
Ackermann
Chirurgie Pédiatrique Hôpital Bicêtre, AP-HP and Université Paris-Sud 11, Le Kremlin-Bicêtre 94275, France.
Florent
F
Guérin
Hépatologie Pédiatrique and Centre de Référence National de l'Atrésie des Voies Biliaires, Hôpital Bicêtre, AP-HP and Université Paris-Sud 11, Le Kremlin-Bicêtre 94275, France; Inserm U 1174, Hepatinov, Université Paris-Sud 11, Orsay 91405, France.
Emmanuel
E
Jacquemin
Hépatologie Pédiatrique and Centre de Référence National de l'Atrésie des Voies Biliaires, Hôpital Bicêtre, AP-HP and Université Paris-Sud 11, Le Kremlin-Bicêtre 94275, France.
Olivier
O
Bernard
D002648:Child; D016145:Endoscopy, Digestive System / Q000379:methods / Q000706:statistics & numerical data; D004932:Esophageal and Gastric Varices / Q000175:diagnosis / Q000209:etiology; D005260:Female; D005602:France / Q000453:epidemiology; D006471:Gastrointestinal Hemorrhage / Q000209:etiology / Q000401:mortality / Q000517:prevention & control; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications*; D008103:Liver Cirrhosis / Q000175:diagnosis / Q000209:etiology; D008297:Male; D011322:Primary Prevention / Q000379:methods; D018570:Risk Assessment / Q000379:methods; D016019:Survival Analysis
Biliary atresia; Children; Cirrhosis; Endoscopy; Gastrointestinal bleeding; Liver transplantation; Portal hypertension; Portal vein obstruction; Primary prophylaxis