Cranio-Maxillofacial and Dental Findings in Albright's Hereditary Osteodystrophy and Pseudohypoparathyroidism

Cleft Palate Craniofac J. 2019 Jul;56(6):831-836. doi: 10.1177/1055665618814661. Epub 2018 Nov 29.

Abstract

Introduction: The clinical phenotype of pseudohypoparathyroidism (PHP) is caused by Albright's Hereditary Osteodystrophy (AHO). Often, "round face" the only facial clinical sign reported in the literature. The aim of this study was to highlight various cranio-maxillofacial clinical findings associated with AHO.

Results: Four patients presented with PHP type 1a. Only one patient exhibited the classical round face. All patients exhibited dental anomalies, class III malocclusion with maxillary retrusion, and a copper beaten appearance of the skull. One suffered from craniosynostosis.

Conclusion: The frequency of craniofacial and dental features associated with malocclusion should prompt careful follow-up, particularly during facial growth, in patients with AHO.

Keywords: craniofacial growth; craniofacial morphology; dental anomalies; dental arch; dental occlusion; facial growth; facial morphology; maxilla; midfacial growth; tooth agenesis; tooth development.

MeSH terms

  • Craniosynostoses
  • Humans
  • Phenotype
  • Pseudohypoparathyroidism* / complications