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October 1969

Oculodentodigital Dysplasia

Author Affiliations

From the departments of pediatrics, neurology, radiology, and plastic surgery, Beilinson Hospital, Petach Tikvah, Tel-Aviv University Medical School, Israel, and the Division of Oral Pathology, University of Minnesota School of Dentistry, Minneapolis (Dr. Gorlin).

Am J Dis Child. 1969;118(4):600-607. doi:10.1001/archpedi.1969.02100040602013

ALTHOUGH recognized as early as 1920 by Lohmann1 and by several other observers during the intervening years,2-4 it was not until 1957 that Meyer-Schwickerath et al5 suggested the term oculodentodigital dysplasia (ODD) to describe a syndrome consisting of: (1) characteristic facies exhibiting a thin nose with hypoplastic alae and narrow nostrils, (2) microphthalmos with anomalies of the iris, (3) syndactyly and camptodactyly of the fourth and fifth fingers, and (4) hypoplasia of the enamel. Subsequently, several cases were reported with similar clinical findings, under various titles.6-10 After Gorlin et al11 published a case and review of the condition in 1963, the term ODD has persisted in use.12-17

The purpose of this paper is to present a family in which a mother and three of her four children have clinical findings compatible with the diagnosis of ODD.

Report of Cases  Case 1.—A 14-year-old boy was born