Case 17 :
Diagnosis please:
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  • Autosomal recessive condition
  • Usually present in early childhood as delayed growth and rarely fracture

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


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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