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DIAGNOSIS: PYCNODYSOSTOSIS
Discussion
- Autosomal recessive condition
- Usually present in early childhood
as delayed growth and rarely fracture
RADIOGRAPHIC
FINDINGS:
Common :
- Generalised and
uniform osteosclerosis
- Narrow medullary
cavities in tubular bones
- No clubbing or
transverse striations seen ( as against osteopetrosis )
- Osteolysis of
the distal phalanges, sometimes progressive but self limiting
- Delayed closure
of sutures, anterior fontanelle and persistent wormian bones
- Thick and sclerotic
skull base with thin vault
- Hypoplastic mandible
with increased angle of the mandible
Other
features :
- Poorly developed mastoids
- Radiodense orbits
- Small maxilla with malformed, malpositioned teeth
- Sclerotic vertebral bodies and segmentation defects in upper cervical
spine
- Spondylolisthesis of the lower lumbar vertebrae
Differentials
:
- Osteopetrosis
- Cleido Cranial Dysostosis
Contribution
Dr. Nagachandar K.
Email:
naga@doctor.com
Reference
:
- Resnick diagnosis of bone
and joint disorders - 3rd edition WB SAUNDERS company
- Caffey’s Paediatric
x-ray diagnosis – 9th edition MOSBY
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