Pycnodysostosis Unveiled: Craniofacial and Dental Manifestations in Focus.

Pycnodysostosis refers to an uncommon autosomal recessive developmental anomaly, which results in decreased bone remodeling, manifesting itself in a variety of clinical and radiographic displays. We report two cases involving two sisters aged 12.5 and 8 years old from a consanguineous marriage. Both presented with distinctive manifestations of the syndrome and were observed at the Dental Consultation and Treatment Center of Ibn Sina Hospital in Rabat. They presented pathognomonic features of the disorder, including short stature, beaked nose, brachydactyly, obtuse mandibular gonial angle and acro-osteolysis of the distal phalanges. This case report is aimed at highlighting these features, in particular the craniofacial and dental characteristics that help in diagnosing this genetic disorder and to emphasize the role of the dentist in this process.