Crouzon Syndrome with Acanthosis Nigricans

Abstract

Crouzon syndrome with acanthosis nigrican is a rare autosomal dominant disorder with the estimated prevalence of 1:1,000,000. The characteristics of this syndrome were early closing of skull sutures which may interfere with the growth of the brain, maxillary hypoplasia, wide-set eye, exophthalmos, and a specific skin disorder of hyperkeratosis and hyperpigmentation. In particular, management should address cranial suture release, midfacial advancement and create normal facial skeletal relationship, evaluation for eye and hearing deficits, obstructive sleep apnea and skin problem. The review articles and a case of adult with Crouzon Syndrome with acanthosis nigrican are presented.