Review of the dental implications of X-linked hypophosphataemic rickets (XLHR).

OBJECTIVES: The aim of this article was to review the dental implications of X-linked hypophosphataemic rickets (XLHR) and to provide suggestions regarding the dental treatment of these patients. MATERIALS AND METHODS: The following search items "x-linked hypophosphataemia, hypophosphataemic rickets, vitamin D-resistant rickets" were used for literature search. Only full-text articles were analysed and summarized to get an overview of the different treatments and outcomes of hypophosphataemic patients. RESULTS: Radiographically, very large pulp chambers with an abnormally high pulp volume/tooth volume ratio, suggesting taurodontism, are often evident. The affected teeth are characterised by a thin enamel layer and dentinal defects. The gender distribution of hypophosphataemic patients is almost equal, but postpubertary males seem to show a trend to develop more severe dental symptoms of the disease. Abscesses without any signs of dental caries or trauma are frequent findings. The most often affected teeth are incisors followed by molars and premolars. CONCLUSIONS: Treatment options include frequent dental examination, application of topical fluoride varnish and sealing of pits and fissures to prevent microbial invasion that may result in pulpitis and further endodontic complications. CLINICAL RELEVANCE: X-linked hypophosphataemic rickets is associated with marked structural alterations of dental hard tissues and the development of multiple abscesses and sinus tracts of dental origin. Therefore, profound knowledge of the various dental implications of XLHR is required to provide these patients with the best possible treatment options.

Hallermann-Streiff syndrome: case report and literature review.

Hallermann-Streiff syndrome is a rare genetic disorder characterized primarily by head and face abnormalities. Patients show birdlike faces; hypotrichosis; various ophthalmic disorders; and dental abnormalities including absence of teeth, natal and neonatal teeth, enamel hypoplasia, and supernumerary teeth. In addition, delayed eruption of existing teeth and severe agenesis of permanent teeth are frequent findings. Dental and hereditary disorders can be associated with disturbances during tooth development and cause shortened roots. Short roots are a rare developmental anomaly in the permanent dentition, and the etiology is not well established. The generalized form is extremely rare. Generalized diminished root formation can lead to early loss of teeth. This article provides a case report of a 9-year-old boy with Hallermann-Streiff syndrome. Extraoral examination revealed a brachycephalic head, proportionate short stature, sparse hair, and atrophic skin. His face was characterized by a thin beak-shaped nose and retrognathia, resulting in a characteristic birdlike appearance. Radiographically, all teeth of the permanent dentition showed severely underdeveloped roots and partially underdeveloped crowns. The predisposition to severe dental caries and dental malformations makes it imperative to schedule effective prevention measures, especially since root canal treatment to preserve teeth can be hampered due to underdeveloped roots.