Dysostosis cleidocranialis - klinické zkušenosti
| Title in English | Cleidocranial dysostosis - clinical experiences |
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| Authors | |
| Year of publication | 2015 |
| Type | Conference abstract |
| MU Faculty or unit | |
| Citation | |
| Description | Cleidocranial dysostosis is a rare autosomal dominant inherited condition. The prevalence is estimated one per million, without sex or ethnic group predilection. The most obvious characteristics are hypoplasia or aplasia of the clavicles, a brachycephalic skull, a delayed closure of sutures and fontanelles. The arms and legs are abnormally short and the patients have short stature. The face appears small in relation to the cranium with underdeveloped maxilla, causing a relative mandibular protrusion. The maxillary sinuses may be small or missing. The palate may be abnormally high. The dental features include lack of exfoliation or resorption of deciduous teeth, delayed eruption of permanent teeth, failure of permanent teeth to erupt, multiple supernumerary teeth and tendency for cyst formation of the unerupted teeth. Early diagnosis is very important. The treatment includes extraction of the deciduous and supernumerary teeth, surgical exposure and orthodontic traction of permanent teeth. This stage of the treatment is slow and requires a team approach and good cooperation from the patient. Many surgeries might be required. The problems of cleidocranial dysostosis are documented and discused on case reports. |
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