title: Pycnodysostosis with Special Emphasis on Dentofacial Characteristics.
abstract: Pycnodysostosis is an autosomal recessive disorder that manifests as osteosclerosis of the skeleton due to the defective osteoclasts mediated bone turnover. The diagnosis of this disorder is established on the basis of its characteristic features and must be differentially diagnosed with other bone disorders. Dental surgeons should be aware of the limitations and possible adverse oral complications such as osteomyelitis of bone in these patients. This will guide them in planning realistic treatment goals. This paper reports the clinical and radiographic features of pycnodysostosis with the great emphasis on its dentofacial characteristics. The aim of this case report is to give an insight into the etiology, pathogenesis, and differential diagnosis of this disorder and to prepare the dentists and maxillofacial surgeons to overcome the challenges in treating these patients.
authors.affiliation: Section of Dentistry, Department of Surgery, The Aga Khan University Hospital, Stadium Road, P.O. Box 3500, Karachi 74800, Pakistan.
authors.forename: Aisha
authors.initials: A
authors.lastname: Khoja
authors.affiliation: Section of Dentistry, Department of Surgery, The Aga Khan University Hospital, Stadium Road, P.O. Box 3500, Karachi 74800, Pakistan.
authors.forename: Mubassar
authors.initials: M
authors.lastname: Fida
authors.affiliation: Section of Dentistry, Department of Surgery, The Aga Khan University Hospital, Stadium Road, P.O. Box 3500, Karachi 74800, Pakistan.
authors.forename: Attiya
authors.initials: A
authors.lastname: Shaikh