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To ensure eruption of the dentition in patients with cleidocranial dysostosis, close collatoration between the oral surgeon and the orthodontist is essential to determine the sequence of extraction, the teeth to be ligated, the preferred technique of ligation, and the approach for surgical exposure.
After presenting the aetiopathogenetic, clinical, radiological and therapeutic aspects of cleidocranial dysostosis syndrome, a case is reported. The patient, a boy of 14, was the subject of examination since birth.
Cleidocranial dysostosis is a rare embryopathy affecting the whole osseous system. Anomalies predominate on skull, chest and dentition but the whole skeleton can be affected especially pelvic girdle and spine. An autosomal transmission is often demonstrable; isolated forms are very uncommon. We present two cases fortuitously diagnosed in our ward: a familial cleidocraniopelvic major form and an incomplete isolated form. The treatment must be especially oriented towards dental malformations.
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Using computerized tomography (5 patients) and lateral cephalograms (17 patients) for analysing craniofacial pathology in patients with cleidocranial dysostosis, a number of specific morphologic disorders could be demonstrated in great detail. Furthermore, new characteristics of cranial base dysplasia and disproportion of the facial skeleton could be clearly demonstrated. These findings also explain the typical concave profile of these patients.
Developmental deformities of the permanent dentition are of primary concern in the treatment of patients suffering from cleidocranial dysostosis. The therapeutic approach should be based on interdisciplinary cooperation between orthodontists as well as oral and maxillofacial surgeons. Therapy may include surgical exposure of impacted teeth in combination with removal of supernumerary teeth. Already during the surgical procedure orthodontic bands and brackets may be attached. The next step in treatment should consist of orthodontic alignment of the teeth and correction of the jaw relations. In most cases, midfacial hypoplasia and corresponding abnormal jaw relations are also present. This deformity is best treated with midfacial osteotomy. Following this second surgical intervention, orthodontic treatment is completed in order to establish an optimal result regarding function and esthetics.
The morphology of the dentition and facial parts of the skull in a 17-year-old girl with cleidocranial dysostosis are described. The opening movement of the mandible was limited to 18 mm. Radiological examination demonstrated that this limitation was due to deformities of the temporomandibular joints. To our knowledge, such deformities have not been described in the literature.
A case report is presented in which the management of impacted teeth and cystic lesions is described in a patient with cleidocranial dysostosis. A review of the literature with emphasis on the incidence, clinical features, etiology, diagnosis, and treatment is also discussed.
Five patients from three families have a new genetic syndrome. The features include cleidocranial dysostosis, bilateral absence of the thumbs and of the distal phalanges of the fingers, hypoplasia of the first metatarsus, absence of the distal phalanx and hypoplasia of the proximal phalanx of the big toe, pelvic dysplasia, bilateral hip dislocation, a constant facial dysmorphism with sparse hair, peculiar ears, severe micrognathism, and retracted and poorly delineated lips. The existence of consanguinity in two of the families, both with two affected siblings, as well as the lack of sex predilection, allow us to postulate that this syndrome is inherited in a recessive manner.
CONTEXT: Upper limb ischemia is not as common as lower limb ischemia but may cause severe impairment or disability if it is misdiagnosed. CASE REPORT: A case of a woman with cleidocranial dysostosis resulting in upper right limb ischemia is presented. This uncommon condition is an exceedingly rare cause of vascular compression that gives rise to thrombosis of the axillary-subclavian arteries. Only two cases have previously been reported.
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Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.