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Overview of Osteopetroses

(Marble Bones)

By Frank Pessler, MD, PhD, Helmholtz Centre for Infection Research;Hannover Medical School, Braunschweig, Germany;Hannover, Germany

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Osteopetroses are familial disorders characterized by increased bone density and skeletal modeling abnormalities.

Osteopetroses can be categorized based on whether sclerosis or defective skeletal modeling predominates. There are a number of different types, including

They are all familial but have different inheritance patterns. Some types are comparatively benign; others are progressive and fatal.

Bony overgrowth sometimes severely distorts the face. Malocclusion of the teeth may require specialized orthodontic measures. Plain x-rays typically are diagnostic.

Surgical decompression may be required to relieve elevated intracranial pressure or to release a trapped facial or auditory nerve.