Splenic-Z-score predicts severe portal hypertensive changes on colonoscopy in infants and children with biliary atresia.

Abstract

Objectives: Biliary atresia (BA) is one of the common causes of progressive portal hypertension (PHT) in children. Rectal varices and colopathy are seen in 38%-92% and 40%-54% of adults with PHT, and 94% and 75% of children with extrahepatic portal venous obstruction. The present work aimed to study the prevalence of rectal varices and colopathy in children with BA.

Methods: From March 2020 to February 2022, all consecutive children with BA with clinically evident PHT were enrolled. Colonoscopy was performed after overnight bowel preparation under monitored anesthesia care. The association of colonoscopic findings with esophagogastroduodenoscopy findings, clinical and laboratory parameters was analyzed. Presence of rectal varices, friability or ulcerations was defined as severe PHT changes on coloscopy.

Results: Twenty-nine BA children (21 males) were enrolled with a median age of 24 (9, 48) months. Majority (21, 72.4%) were post-Kasai portoenterostomy (KPE)-20 were successful. Median PELD score was 1(-6, 16). Clinically significant varices were present in 12 (41.4%); 5 (17.2%) had variceal bleeding at a median age of 24 months. Hemorrhoids, rectal varices and colopathy were present in 37.9%, 48.3%, and 75.9%. Colitis-like and vascular changes were present in 75.9% and 37.9%. Severe PHT changes were seen in 65.5%. On binary logistic regression analysis, severe changes on colonoscopy were independently associated with splenic-Z score [Exp(B) = 3.03, 95% CI 1.07-8.58, p = 0.037] and using the model improved prediction from 65.5% to 82.8%.

Conclusion: Children with BA have rectal varices and colopathy in 48% and 76%. Severe PHT changes, present in two-thirds, are independently associated with higher splenic-Z score.