“ Rare inherited disorder due to a defect in type-1 collagen formation . The result is poor bone density and brittle bones prone to fracture...
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Rare inherited disorder due to a defect in type-1 collagen formation. The result is poor bone density and brittle bones prone to fracture. There are four subtypes of varying severity; type II is the most severe and is frequently fatal in utero. Type I is mild, type IV moderate and type III severe (manifest at birth).
APPENDICULAR X-RAY
• Multiple, repeated, unexplained fractures with minor/no trauma.
• Exuberant callus.
• Fractures typically affect the long bones, spine and apophyses.
• Pseudarthroses.
• Poor bone density.
• Bone deformity—bending and thinning of the long bone diaphysis; may be marked and cause e.g. ‘shepherd’s crook’ deformity of the femur
SKULL X-RAY
• Deformity of the skull (e.g. platybasia, prominent occiput, ‘Darth Vader skull’)
• Wormian bones (>10)
• Basilar invagination
• Enlarged sinuses and abnormal teeth
• Compression fractures at multiple levels
• Spondylolysis
• Lumbar hyperlordosis
Osteogenesis imperfecta. Frontal knee radiograph demonstrating osteopenia, femoral fracture with callus formation and bone deformity with bending and thinning.
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