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Propositus' uncle at 7 years. X-Ray images showing signs of severe dysostosis multiplex in the hand (A) - proximal pointing of metacarpals, shortened metacarpals and carpal hypoplasia - and spine (B) - abnormal modeling and inferiorly beaked vertebrae. Cranial computed tomography (C) and (D) showing enlargement of the supratentorial ventricles and cortical atrophy, even after shunting.
