Dental Management of a Pediatric Patient With Progressive Familial Intrahepatic Cholestasis Having Dental Anomalies: A Case Report and Brief Review of the Literature Publisher Pubmed



Yazdizadeh M¹ ; Sharifi M² ; Torabi Parizi A³ ; Alipour F⁴ ; Ghasempuor M⁵ ; Zanguei E⁶ ; Yazdizadeh M¹
Source: BMC Oral Health Published:2023

Abstract

Background: Progressive familial intrahepatic cholestasis is a heterogeneous group of disorders, leading to intrahepatic cholestasis, with the possibility of chronic liver failure and biliary cirrhosis. Oligodontia is either the manifestation of a specific syndrome or is non-syndromic. To the best of our knowledge, this is the first case report of type 3 progressive familial intrahepatic cholestasis and concurrent oligodontia, craniosynostosis, dens in dente, taurodontism, and delayed permanent dentition in the medical and dental literature. Case presentation: We present the dental and medical histories and comprehensive dental management of a girl with type 3 progressive familial intrahepatic cholestasis and several dental anomalies, who was referred to a dental clinic due to severe dental caries and pain. Conclusion: Our findings suggest that PFIC with manifestations as oligodontia, craniosynostosis, dens in dente, taurodontism, and delayed permanent dentition, might indicate an unknown syndrome; otherwise, the craniofacial anomalies are the manifestations of an independent disease coinciding with PFIC. Moreover, our case is a good example of the importance of timely medical and dental care in confining further health-related complications. The patient was able to ingest without any pain or discomfort after receiving proper dental management. © 2023, The Author(s).