Cleidocranial Dysplasia: Case Report of Three Siblings

Abstract

Background: A family case report of cleidocranial dysplasia (CCD) with varied manifestations from father to three siblings is presented. CCD ( MIM # 119600) is a rare autosomal dominant skeletal dysplasia caused by CBAF1 gene ( OMIM 600211) with a wide range of variability. In all the cases generalized dysplasia in bone, prolonged retention of primary teeth and delayed eruption of permanent teeth were evident. Interestingly, there were no supernumerary teeth present. There was mandibular prognathism which was intercepted by occipital chin cup therapy.Aims and objective: To present the clinical manifestations, diagnostic imaging and treatment modalities along with dermatoglyphics in CCD patients. Conclusion: Cleidocranial dysplasia is an uncommon disorder however its clinical and radiological features are characteristic. In addition the CCD patients may be distinguished by specific dermatoglyphic markers. It carries with it several implications in terms of complications like skeletal malocclusion, dental caries, etc. Medical treatment is mainly directed at orthopedic and dental correction. A team approach to the management of dental abnormalities on a long-term basis with the overall goal to provide an esthetic facial appearance and functioning occlusion by late adolescence or early adulthood should be focused.

Oral Rehabilitation with Removable Partial Denture of a Patient with Cleidocranial Dysplasia

“…There is no consistent protocol for patients with CCD who seek treatment at different ages [1]. In some cases [1,10,18], the orthodontic treatment was suggested after the extraction of retained deciduous teeth. In these cases, subsequent orthodontic alignment resulted in a functional and esthetic outcome, and a good facial profile.…”

“…The treatment plan largely depends on the age [9] and a patient's medical condition [10]. On the other hand, esthetic and masticatory functions are two essential needs, which should be provided by therapists [5].…”

Prediction of clinical height gain from surgical posterior correction of idiopathic scoliosis

“…1B) clinical heights were determined before surgery and within 15 days and at 3 months postoperatively. To limit measurement bias, clinical height was measured by the same operator under similar conditions: the patient was barefoot in the Frankfort horizontal plane, 11,14 and the same stadiometer fixed to the wall was used for all patients.…”

A case report of cleidocranial dysplasia: A noninvasive approach

“…Effective communication between patient and paediatric dentist is highly important in deciding the most appropriate treatment plan for the patient. In other studies, the most common dental treatment for young patients with CCD has been to remove dental anomalies, achieve optimal occlusion, and improve aesthetics, which requires a multidisciplinary approach involving orthodontist, prosthodontist, and oral maxillofacial surgeons 14–18 . However, in our case, a surgical intervention was refused by the patient, as she was not mentally prepared for the long‐term postsurgical management that would be required.…”