OCULODENTODIGITAL SYNDROME : A CASE REPORT

,; ,; ,

Journal of the Korean Academy of Pediatric Dentistry 1999;26(3):492-498.
Published online August 31, 1999.

OCULODENTODIGITAL SYNDROME : A CASE REPORT

Oculodentodigital syndrome의 1 증례

강호승, 정태성, 김신

부산대학교 치과대학 소아치과학교실

Abstract

Oculodentodigital syndrome(ODD) was first reported by Lohmann in 1920 and termed by Meyer Schwicketath, which they called "dysplasia oculo-dento-digitalis" in 1957. It is somewhat rare heritable disease. ODD is generally inherited in an autosomal dominant pattern with a complex phenotype. The characteristic features are : (1) unique facial features, (2) microphthalmos, (3) syndactyly and camptodactyly of 4th and 5th fingers, (4) osseous anomalies of the middle phalanges of 5th fingers and toes, (5) enamel hypoplasia, (6) dry lusterless hair. We found several occlusal wearing and yellow discoloration of succedaneous teeth, multiple caries lesions, premature loss and pulpal involvement of primary teeth with associated enamel abnormalities caused by generalized enamel hypoplasia in a fairly constant oral finding. Occasionally partial anodontia, microdontia and cleft lip and palate can be manifested. This case, a 9-year-old female with repaired bilateral syndactyly was referred to pediatric dental clinic, Pusan National University Hospital for evaluation of severe attrition of teeth and caries lesions. She had most of the above mentioned typical manifestations of the syndrome. Dental treatment including caries control, stainless steel crown were performed.

Key Words: Genetic disease, Oculodentodigital syndrome, Enamel hypoplasia