Definition
Cleidocranial dysostosis, a development defect
characterized by absence or rudimentary development of the
clavicles, abnormal shape of the skull with depression of the
sagittal suture, frontal bosses, many wormian bones, and aplasia or
hypoplasia of teeth.
|
Radiographic Appearance
One or more of the following appearences may be seen
Skull
worian bones
calvarial thickening
late mineralization of the sutures
small sphenoid bones
accessory sinuses and mastoids late or hypoplastic in developing
nonunion of mandibular symphysis
Others
absent clavicles
vertebral malformations
small scapulae
delayed ossification of the pubic bone
wide symphysis pubis
flat acetabula
hypoplasia of the iliac wings
broad femoral head with short femoral neck
slow rate of carpal ossification
metacarpal pseudoepiphyses
osteosclerosis |
Pathology
A congenital disorder of bone formation with clavicular hypoplasia
or agenesis with a narrow thorax, allowing approximation the
shoulders in front of the chest occurring with delayed ossification
of the skull, excessively large fontanelles, and delayed closing of
the sutures. The fontanelles may remain open until adulthood, but
the sutures often close with interposition of wormian bones. Bosses
of the frontal, parietal, and occipital regions give the skull a
large globular shape with small face. The characteristic skull
abnormalities are sometimes referred to as the "Arnold head" named
after the descendants of a Chinese who settled in South Africa and
changed his name to Arnold. More than 100 additional anomalies may
be associated, including wide pubic symphysis, dental abnormalities,
short middle phalanges of the fifth fingers, delayed skeletal
maturation, hearing deficiency, and mild mental retardation in some
cases.
|
Treatment:
There is no specific treatment for the bony
abnormalities. An oral surgeon should monitor teeth regularly, with
particular attention to both decay and cosmetic appearance. An
otologist should check for hearing defects. |
Image
 |
|
Useful Link
|
|
 |
