Pediatric oral and dental manifestations of biliary atresia and Alagille syndrome: a comparative retrospective study.

BACKGROUND

Case reports, small case series, and studies not specifically focused on a single type of cholestatic disorder suggest the presence of oral and dental manifestations in Alagille syndrome (AGS) and Biliary Atresia (BA). While some may be directly due to cholestasis, others may be phenotypic of these conditions.

OBJECTIVES

The aim of this study was to describe the oral characteristics of children with AGS and BA and to distinguish features specific to each condition from those attributable to cholestasis.

METHODS AND

SETTING

Oral characteristics where retrospectively compared between children with cholestasis, including patients with AGS ("AGS group") and BA ("BA group"), and control children matched for age, sex and dentition, who had consulted in the Pediatric Dentistry and Rare Diseases Unit at Timone University Hospital (Marseille, France) between September 2021 and December 2023.

RESULTS

Congenital cholestasis patients (10 with AGS and 16 with BA) were more likely to have tooth shape anomalies (14 vs 6 patients, p = 0.01), tooth number anomalies (10 vs 2 patients, p = 0.02), tooth structure anomalies (13 vs 7 patients, p = 0.09), tooth discoloration (18 vs 0 patients, p < 0.001), and poor oral hygiene (19 vs 13 patients, p = 0.09) than matched controls, but not malocclusion (20 vs 17 patients, p = 0.36) or periodontal disease (15 vs 11 patients, p = 0.27). Carious lesions were more common in control patients (20 vs 14 patients, p = 0.08). Malocclusion was more frequent among AGS patients (10/10) than among BA patients (10/16, p = 0.053).

CONCLUSION

In this group of pediatric dental patients, congenital cholestasis was associated with a higher rate of dental abnormalities, particularly discoloration, tooth shape, and number anomalies. Our results provide a more detailed description of these features. Multicenter studies are required to confirm these findings on larger samples.