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Clinical and radiographic characteristics of pycnodysostosis: A systematic review.
- Source :
-
Imaging science in dentistry [Imaging Sci Dent] 2024 Mar; Vol. 54 (1), pp. 13-24. Date of Electronic Publication: 2024 Jan 04. - Publication Year :
- 2024
-
Abstract
- Purpose: Pycnodysostosis (PYCD), an autosomal recessive syndrome, is characterized by an imbalance in bone remodeling that produces various clinical and radiographic craniofacial manifestations. This review represents a systematic examination of these manifestations, as well as oral features associated with PYCD.<br />Materials and Methods: A systematic review was conducted across 8 databases from February to March 2023. The search strategy focused on studies reporting cases of PYCD that examined the clinical and radiographic craniofacial and oral characteristics associated with this syndrome.<br />Results: The review included 84 studies, encompassing a total of 179 cases of PYCD. More than half of the patients were female (55.3%), and the mean age was 14.7 years. Parental consanguinity was reported in 51.4% of the cases. The most common craniofacial clinical manifestation was a prominent nose, observed in 57.5% of cases. Radiographically, the most frequently reported craniofacial characteristics included the presence of an obtuse mandibular angle (84.3%) and frontal cranial bosses (82.1%). Clinical and radiographic examinations revealed oral alterations, with micrognathia present in 62.6% of patients and malocclusion in 59.2%. Among dental anomalies, tooth agenesis was the most commonly reported, affecting 15.6% of patients.<br />Conclusion: Understanding the clinical and radiographic craniofacial features of PYCD is crucial for dental professionals. This knowledge enables these clinicians to devise effective treatment plans and improve patient quality of life.<br />Competing Interests: Conflicts of Interest: None<br /> (Copyright © 2024 by Korean Academy of Oral and Maxillofacial Radiology.)
Details
- Language :
- English
- ISSN :
- 2233-7822
- Volume :
- 54
- Issue :
- 1
- Database :
- MEDLINE
- Journal :
- Imaging science in dentistry
- Publication Type :
- Academic Journal
- Accession number :
- 38571780
- Full Text :
- https://doi.org/10.5624/isd.20230191