Cleidocranial Dysplasia
What is cleidocranial dysplasia?
Cleidocranial dysplasia is a condition that primarily affects the development
of the bones and teeth.
What are the symptoms and signs of cleidocranial dysplasia?
Signs and symptoms of cleidocranial dysplasia can vary
widely in severity, even within the same family.
Individuals with cleidocranial dysplasia usually have underdeveloped or
absent collarbones (clavicles). As a result, their shoulders are narrow and
sloping, can be brought unusually close together in front of the body, and in
some cases the shoulders can be made to meet in the middle of the body. Delayed
closing of the spaces between the bones of the skull (fontanels) is also
characteristic of this condition. The fontanels usually close in early
childhood, but may remain open into adulthood in people with this disorder.
Affected individuals may be 3 to 6 inches shorter than other members of their
family, and may have:
- short, tapered fingers and broad thumbs;
- short forearms;
- flat feet;
- knock knees; and
- an abnormal curvature of
the spine (scoliosis).
Characteristic facial features may include:
- a wide, short skull (brachycephaly);
- a
prominent forehead;
- wide-set eyes (hypertelorism); a
- flat nose; and a
- small
upper jaw.
Individuals with cleidocranial dysplasia may have
decreased bone density
(osteopenia) and may
develop osteoporosis, a condition that makes bones progressively more brittle
and prone to fracture, at a relatively early age.
Women with cleidocranial dysplasia have an increased risk of requiring a
cesarean section when delivering a baby, due to a narrow pelvis preventing
passage of the infant's head.
Dental abnormalities seen in cleidocranial dysplasia may
include:
- delayed loss of the primary (baby) teeth;
- delayed appearance of the
secondary (adult) teeth;
- unusually shaped, peg-like teeth;
- misalignment of the
teeth and jaws (malocclusion); and
- extra teeth, sometimes accompanied by
cysts
in the gums.
In addition to skeletal and dental abnormalities, people
with cleidocranial dysplasia may have hearing loss and be prone to
sinus and
ear
infections. Some young children with this condition are mildly delayed in the
development of motor skills such as crawling and walking, but intelligence is unaffected.
Next: How common is cleidocranial dysplasia? »
- Sinus Infection - Learn about sinus infections and inflammation of the sinuses (also known as sinusitis) with information written by doctors for patients. Includes causes, symptoms, diagnosis, and complications of sinus infection.
- Scoliosis - Scoliosis causes abnormal curvature of the spine. Get the facts on scoliosis types and symptoms. Treatment may involve exercises, surgery, physical therapy, yoga and massage.
- Otitis Media - Learn more about otitis media and how this acute or chronic middle ear infection, that mostly affects children, is caused, diagnosed and treated on MedicineNet.com
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