The Natural History of Gastroesophageal Varices in Children With Portal Hypertension.

BACKGROUND & AIMS: In children with portal hypertension, the natural history of gastroesophageal varices remains unclear. We report our experience.

METHODS: From 1990 to 2021, 1586 children with portal hypertension underwent ≥1 diagnostic upper gastrointestinal endoscopies before any treatment, including 590 with ≥2 endoscopies. High-risk varices (HRV) were defined as grade 3 esophageal varices as well as grade 2 esophageal varices with red color signs or gastroesophageal varices type 1 or both.

RESULTS: Worsening of the endoscopic pattern occurred in 58% of children over a mean 4-year interval. The 5- and 10-year probabilities of HRV emergence in initially HRV-negative children were 36% and 54%, respectively, and were associated with the severity of the initial endoscopic findings. In children with biliary atresia, a younger age and higher serum bilirubin levels were linked to increased HRV risk. At study end, HRV were present in 639 of 1586 children; 161 of 639 were aged <12 months old, 62% of whom had platelet count of ≥150,000/mm3. The probabilities of spontaneous bleeding after HRV detection on screening endoscopy were 63% at 5 years and 75% at 10 years. Among children with cirrhosis and no HRV at baseline, the Baveno VI criteria and spleen stiffness ≤30 kPa were associated with a low 3-year probability of HRV emergence.

CONCLUSIONS: Worsening of the endoscopic pattern is common in children with portal hypertension. HRV can be expected to emerge in one-third and one-half of initially HRV-negative children after 5 and 10 years, respectively. Infants with biliary atresia are at particularly high risk. These findings support the rationale for considering primary prophylaxis of bleeding.