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March 1951

CLEIDOCRANIAL DYSOSTOSIS SYNDROME: Report of a Case in a Negro Child Exhibiting Retarded Growth

Author Affiliations

From the Department of Pediatrics of Howard University School of Medicine and the Pediatric Service of Freedmen's Hospital.

AMA Am J Dis Child. 1951;81(3):394-402. doi:10.1001/archpedi.1951.02040030404006

Cleidocranial dysostosis is a rare congenital syndrome which was originally described by Marie and Sainton1 in 1897. According to the classic description, the syndrome consists in aplasia of one or both clavicles, exaggerated development of the transverse diameter of the cranium, delay in the ossification of the fontanels and hereditary transmission. At present the requirements for the diagnosis of the condition are less rigid than those originally set down by Marie and Sainton. Various workers2 have reported atypical cases which did not fulfil the four requisites listed. To help to clear some of the confusion that surrounds this subject Fitchet3 has suggested that use of the term "hereditary cleidocranial dysostosis" be discontinued and the condition be recognized for what it is, "a congenital cleidal dysostosis, either hereditary or nonhereditary, with or without other anomalies."

Clinically the most prominent sign is the defect of the clavicles and extreme

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