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A Case of Cleidocranial Dysostosis

1937; Wiley; Volume: 10; Issue: 116 Linguagem: Inglês

10.1259/0007-1285-10-116-613

1748-880X

John P. Steel, Patrick Whitaker,

Osteomyelitis and Bone Disorders Research

A Woman, aged 24, was admitted to this hospital some seven years ago complaining of epileptic fits nocturnally, and was kept under observation and was given Luminal in small doses. She was discharged temporarily free from epilepsy, and it was noted that the head was a peculiar shape, and the limbs were stunted. In 1935 she was again admitted as an epileptic, with a discharging sinus on the face, and X ray showed an unerupted, infected molar, and a similar condition in the front teeth of the lower jaw. She was removed from hospital by her relatives shortly after the abscess had been opened, but was re-admitted with a chronic sinus, and on this occasion opportunity was taken to X ray more extensively, and it was found that the skeletal deformities associated with cleidocranial dysostosis were present in a very marked degree. It will be seen from the reproductions that there is a normal degree of density of the bones, but very incomplete development. The sutures are gaping widely, and although the margins are well developed and show the normal serrations, in no part is there any attempt at fusion. The vertex is large (Fig. 1) compared to the base and maxillæ, and gives the skull a hydrocephalic appearance (Fig. 2). In both upper and lower jaws several unerupted milk and supernumary teeth are present.