Osteopetrosis is a developmental bone abnormality
radiologically characterised by generalised increase in bone density. Bone
appears chalky white and is brittle. Paradox it may seem but they are more
prone for fractures like osteoporotic bones.
Heinrich Ernst Albers-Schonberg of Hamburg (Germany) first
described this condition. He was one of the pioneer radiologist and many
radiographic techniques used in day to day practice were developed by him. He
is well known for is early reports of radiation induced damage of the rabbit
ovaries in 1903.
Excessive resorption of the bone causes osteoporosis,
similarly defective bone resorption leads to osteopetrosis. At least eight
subtypes of osteopetrosis are described. Tarda variant of osteopetrosis is encountered
in adults and have reasonably good prognosis. Mode of inheritance is autosomal
dominant. More malignant varieties are encountered is early age group and show
autosomal recessive inheritance.
Long bones show generalised increased density and undertubulation
of the bones resulting in the Erlenmeyer flask deformity. Joint is spared.
Sclerosis of the vertebral endplates results in sandwich vertebra. Failure of
the osteoclasts to remodel the bone result in endobone formation or bone with
in bone appearance.
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