FRASER SYNDROME - A CASE-REPORT AND REVIEW OF THE OTOLARYNGOLOGIC MANIFESTATIONS

Fraser syndrome is a rare autosomal recessive disorder whose major man ifestations are cryptophthalmos, syndactyly and genital abnormalities. These patients also frequently have malformations of the ears, nose a nd/or larynx. The diagnosis is usually made at birth from the obvious malformations, although occasionally made on prenatal ultrasound. Trea tment is dependent on which malformations are present and genetic coun seling is indicated. Prognosis is dependent on the severity of renal a nd laryngeal malformations.