Yonsei Med J.  2009 Apr;50(2):289-292.

Dental Findings in Cornelia De Lange Syndrome

• Toker AS
• Ay S
• Yeler H
• Sezgin I

Abstract

Cornelia de Lange syndrome is a congenital disease, basically characterized by psychomotor retardation associated with a series of malformations, including mainly skeletal, craniofacial deformities together with gastrointestinal and cardiac malformations. There is no definitive biochemical or chromosomal marker for the prenatal diagnosis of this syndrome. We actually want to present the case of a 10-year-old patient, who was admitted to our clinic for dental pain. The patient had the symptoms of Cornelia de Lange syndrome. During the oral examination of this patient, the patient was found to have the typical symptoms of Cornelia de Lange syndrome, such as micrognathia and delayed eruption in conjunction with the symptoms of the Hutchinson's syndrome, which had never been reported before.

Keyword

Cornelia de Lange; Brachmann de Lange; syndrome; Hutchinson's teeth; dental caries

MeSH Terms

Child
De Lange Syndrome/*pathology/*physiopathology
Humans
Male
Tooth/*pathology

Figure

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  Fig. 1 Facial appearance of the patient.

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  Fig. 2 Radiological appearance of the hands.

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  Fig. 3 Radiological appearance of the feet.

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  Fig. 4 Hirsutism on the back.

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  Fig. 5 Intraoral appearance of the patient.

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