en POLSKI
ISSN: 1734-1558
Forum Ortodontyczne / Orthodontic Forum
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SCImago Journal & Country Rank
4/2020
vol. 16
 
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abstract:
Review paper

Orthodontic treatment of patients with cleidocranial dysplasia Systematic review of the literature

Barbara Lizończyk
1
,
Kinga Pielech
1
,
Maria Panaś
2
,
Wojciech Stós
1

1.
Department of Orthodontics, Dental Institute, Faculty of Medicine, Jagiellonian University, Medical College, Cracow
2.
Department of Oral Surgery, Dental Institute, Faculty of Medicine, Jagiellonian University, Medical College, Cracow
Forum Ortod 2020; 16 (4): 307-17
Online publish date: 2020/12/30
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Introduction
The cleidocranial dysplasia (CCD) is a rare autosomal dominantly inherited genetic disease that does not have a gender predisposition. It occurs in a frequency of 1:1,000,000. Patients with cleidocranial dysplasia are characterized by pathognomonic appearance and a variety of phenotypes (isolated dental disorders, mild form, severe form) are associated with the type of mutation (deletions, insertions, nonsense, missense) of the RUNX2 gene, which is located on the short arm of chromosome 6 (6p21). A triad of symptoms is distinguished: clavicular hypoplasia, multiple impacted teeth and supernumerary teeth and delayed cranial suture healing. Due to the rarity of the disease only a small number of cases were described.

Aim
The aim of the article is to present a systematic review of the literature of patients with cleidocranial dysplasia treated orthodontically.

Material and methods
A systematic review of the literature of PubMed and Scopus databases as well as Polish periodicals such as Journal of Stomatology, Dental and Medical Problems and Forum Ortodontyczne until December 2019 was conducted. Fourteen articles met the inclusion criteria.

Results
The collected data indicate the need for interdisciplinary treatment of patients with cleidocranial dysplasia, orthodontic surgery, in selected cases supported by maxillofacial surgery, prosthetic or implantoprosthetic, endodontic or periodontological treatment. There are four protocols for interdisciplinary treatment of the disease in the literature. Due to the rarity of the disease only a small number of cases were described. Structured treatment protocols should be carefully adapted to each individual case.

keywords:

cleidocranial dysplasia, cleidocranial dysostosis, surgical-orthodontic treatment, systematic review