Cleidocranial Dysostosis

Jarvis, Jeffrey L.; Te, Keats

doi:10.2214/ajr.121.1.5

Cited by 91 publications

(2 citation statements)

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“…Differential diagnosis is commonly achieved when cleidocranial dysplasia (CCD) is confirmed. Cleidocranial dysplasia is a rare genetic bone abnormality characterized by hypoplastic or anaplastic clavicles, delayed closure of cranial sutures and multiple dental anomalies (15). Other conditions and diseases associated with hypo-or anaplasia of the clavicle, such as mandibuloacral dysplasia (MAD), should also be considered as a differential diagnosis (16).…”

Section: Discussionmentioning

confidence: 99%

A clinical case report of hypodontia and impacted supernumerary teeth in a patient diagnosed with Yunis-Varon syndrome

Dzhongova,

Edreva

2022

Scripta Scientifica Medicinae Dentalis

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INTRODUCTION: Yunis-Varon syndrome (YVS) is an extremely rare autosomal recessive congenital disorder affecting many organs and systems (bones, ectodermal structures, the cardiorespiratory system, etc.). Abnormalities include aplasia or hypoplasia of the clavicles, facial dysmorphism, sparse hair, absence or hypoplasia of the thumbs, toes and distal phalanges, prominent auricles, shortened upper lip and other skeletal deformities, such as pelvic dysplasia, kyphosis, scoliosis, etc. Tooth structures and jaws are characterized by marked micrognathia, a narrow high-arched palate, hypodontia, impacted permanent teeth as well as supernumerary teeth. AIM: The present case report aims to describe the clinical and radiographic changes in the masticatory apparatus of a patient diagnosed with Yunis-Varon syndrome. CASE PRESENTATION: The subject of this study was а patient, suffering from pain in tooth #38. The clinical intraoral examination revealed hypodontia, but the radiographic image revealed many supernumerary teeth in the anterior and premolar region of both the maxilla and the mandible. RESULTS: In addition to skeletal anomalies and impaired ectodermal structures, this rare syndrome can cause disturbances in the teeth and jaws. For this reason, dental specialists play an important role in the diagnosis and treatment of patients suffering from Yunis-Varon syndrome. CONCLUSION: Early intervention is important in Yunis-Varon syndrome. Future investigation will permit identification of thеsе skeletal and dental abnormalities.

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Section: Discussionmentioning

confidence: 99%

A clinical case report of hypodontia and impacted supernumerary teeth in a patient diagnosed with Yunis-Varon syndrome

Dzhongova,

Edreva

2022

Scripta Scientifica Medicinae Dentalis

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“…The latter sporadically produce even a full set of additional dentition [7]. Due to the delayed eruption of permanent teeth and the limited space in the bone resulted from the existence of supernumerary teeth, the development of permanent teeth might be affected, reflecting ectopia and aberrant morphology especially in the roots, including taurodontism [9]. Thus, dental treatment of CCD positive patients is challenging and compels an interdisciplinary approach including surgery, orthodontics, conservative dentistry, and prosthodontics.…”

Section: Introductionmentioning

confidence: 99%

A Prosthodontic Approach as a Complementary Solution for a Complicated Orthodontic Treatment of a Patient with Cleidocranial Dysplasia

Jasnoch

Zielke

Maciejewska

2021

Case Reports in Dentistry

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This clinical report describes a prosthodontic rehabilitation of a 29-year-old patient with cleidocranial dysplasia (CCD), who, after completing an orthodontic treatment, was not satisfied with the aesthetic outcome. Besides aesthetics, the patient complained about mastication muscles pain, and clicking while eating but was not aware about her unilateral open bite on the right side. The aim of this treatment was to improve smile appearance and patient’s well-being, as well as to restore the proper occlusal vertical dimension (OVD) along with complete intercuspation and to establish masticatory function. The first phase of the treatment concentrated on eliminating the muscle pain and temporomandibular joint (TMJ) clicking with a repositioning splint. During the second phase, the functional and aesthetic rehabilitation was obtained using adhesive prosthesis overlays and veneers.

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