Cohen syndrome: case report.

A report of a child with Cohen syndrome is presented. Of particular interest to dentists are the relatively consistent findings of open mouth, short philtrum, micrognathia, and the prominent maxillary central incisors. A combination of these findings in addition to other traits such as hypotonicity, variable degrees of mental retardation, narrow hands and feet, childhood obesity, and delayed puberty, should raise the dentist’s suspicion of this or related syndromes. Cohen syndro~ne first was.described as a unique clinical entity in 3 patients in 1973.1 That report included histories of 3 Caucasian children of full-term gestation with decreased fetal activity and with apparently normal parents. Two of the 3 children were siblings, and the third case was an isolated occurrence in an unrelated family. In reviewing the ]iterature, 28 other reports 2-12 were found. The most consistent findings include obesity, mental retardation, hypotonia, maxillary hypoplasia, short philtrum, open mouth, micrognathia, and narrow hands and feet. Less frequently observed characteristics include short stature, microcephaly, antimongoloid slanting, and prominent central incisors. Although the syndrome originally was suggested to involve an autosomal recessive pattern, 1 recent analysis strongly supports an autosomal dominance process. 13 In this report, the authors describe an individual with documented Cohen syndrome.