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Lecture Craniofacial Limoudei Hemsech

Lecture Craniofacial Limoudei Hemsech

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16<br />

Crouzon (<strong>Craniofacial</strong><br />

dysostosis)<br />

(Acrocephalosyndactyly, type<br />

II)<br />

• 1:25,000<br />

• Autosomal dominant (Most cases sporadic)<br />

Chromosome 10<br />

• Craniosynostosis, midfacial hypoplasia,<br />

exopthalmos.<br />

Crouzon cont<br />

• Various dformations of skull- usually brachy.<br />

• Midfacial retrusion – class III bite and shallow<br />

orbits. May cause damage to eyes. Beaked<br />

nose, High arched palate<br />

• Conductive hearing loss is common.<br />

• Synostosis develops during 1 st year of life<br />

usually complete by 3 rd .<br />

• Increased ICP common.

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