Children with Alagille syndrome (ALGS) and biliary atresia (BA) have considerable oral manifestations that may require extensive dental input, especially when presenting with congenital cholestatic disease, according to a study published in Archives de Pédiatrie. 

Among the major causes of congenital neonatal cholestasis in infancy are ALGS and BA. They impact oral health by lowering immunity and protein metabolism, and are aggravated by bleeding disorders and insufficient therapeutics. Malnutrition is also common among these groups of patients, which can further aggravate oral issues. 

The chief danger is in the development of infectious dental foci, which can negatively impact liver transplantation outcomes. The authors of the study conducted a systematic review of case reports and case series investigating the impact of ALGS, BA, and progressive familial intrahepatic cholestasis (PFIC) on the oral health of children. 

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Their review, conducted on academic search engines, included 19 studies, 16 case reports, and 3 case series. Despite the intention of the authors to include ALGS, BA, and PFIC, only studies on the first two were found.

Read more about ALGS etiology 

The results may be broadly divided into 3 categories: teeth impact, periodontal issues, and jaw morphology. Regarding teeth impact, coloration was detected affecting one or both dentitions. In BA, for example, deciduous teeth demonstrated scalable coloration, with nonuniformity of the involvement of the tooth surface. Coloration most often affects the incisors or the molars. 

Over 50% of patients with ALGS were found to have enamel hypoplasia and hypomineralization. In addition, morphological abnormalities were a recurrent theme, with some authors describing micro/macrodontia and talon cases on incisors. These abnormalities, however, were not detected in patients with BA. 

When orthodontic surgical procedures were performed, physicians often prescribed antibiotic prophylaxis. This was mostly the case regardless of a patient’s liver transplant status. With regard to dental treatment, oral hygiene motivation and prophylactic treatments (fissure sealant and fluoride application in ALGS; topical fluoride and gentle rubber cup prophylaxis in BA) were often implemented. 

“The dentist must therefore be a key player in the care pathway of these patients to provide preventive care or to offer expeditious solutions to the oral problems that typically arise in association with these conditions,” the authors concluded. 


Reynal F, Camoin A, Tardieu C, Fabre A, Blanchet I. Oral findings in children with congenital cholestatic disease: a systematic review of case reports and case seriesArch Pediatr. Published online June 30, 2023. doi:10.1016/j.arcped.2023.06.003