The Natural History of Gastroesophageal Varices in Children With Portal Hypertension

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

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.

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.

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.