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[edit] Discussion of Osteopetrosis

  • AKA Albers-Schonberg disease
  • Heterogeneous group of osteosclerotic bone dysplasias
  • The entire skeleton is unusually dense.
  • Impaired bone resorption results in abundant osteoid and narrow, fibrotic medullary spaces.
  • Hematologic derangements are due to the diminished hematopoietic compartment.
  • Bones are fragile and prone to fracture and show a high susceptibility to osteomyelitis.
  • Neural and vascular foramina are narrow, causing cranial nerve palsies by neural compression.

  • Two modes of inheritance are known.
    • Dominant osteopetrosis, which has a benign course and late manifestation
    • Autsomal recessive:
      • Malignant form manifests at birth
      • Intermediate autosomal recessive form manifests in the first decade of life.

[edit] Imaging Findings for Osteopetrosis

[edit] Images

Patient #1: Plain films and MRI images from a patient with osteopetrosis

Patient #2

[edit] See also

[edit] External Links

[edit] References for Osteopetrosis