PMID: 9430147Jan 16, 1998Paper

Cleidocranial dysplasia: a case report

No shinkei geka. Neurological surgery
M KandaY Hasegawa

Abstract

A 3-month-old female infant who presented with patent sagittal suture and loss of weight is described. Physical examination revealed a large sagittal and metopic suture showing delayed closure, a high-arched palate, saddle nose, hypertelorism and nonpalpable edges of the bilateral clavicles. The clavicles also showed undue mobility. Radiological investigations of the cranial skeletal abnormalities revealed enlargement of the sagittal and metopic sutures, and the anterior and posterior fontanelles. A chest radiograph showed a small, bell-shaped thoracic rib cage with partial aplasia of both clavicles. On the basis of the clinical findings, cleidocranial dysplasia was diagnosed. Cleidocranial dysplasia is an uncommon generalized skeletal disorder which, as its name implies, shows striking involvement of the cranial vault and clavicles. The clinical features reflect a generalized defect of both membranous and endochondral bone formation. It is characterized by delayed ossification of the skull, aplastic or hypoplastic clavicles, delayed deciduous dentition, and hereditary characteristics. The amount of calvarial growth is generally small, and the shape remains nearly unaltered. In all cases, calvarial bone thickness increases with...Continue Reading

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