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KMID : 0358920160430030313
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Journal of the Korean Academy of Pedodontics
2016 Volume.43 No. 3 p.313 ~ p.319
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Dental Treatment of a Wolf-Hirschhorn Syndrome Patient: A Case Report
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Kim Mi-Ae
Park Ji-Hyun
Mah Yon-Joo
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Abstract
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Wolf-Hirschhorn syndrome (WHS), associated with the deletion of the short arm of chromosome 4, causes multiple congenital malformations. Patients suffer from various deformities, including mental and growth disorders, epilepsy, hypotonia, congenital heart defects, and atypical craniofacial features. The “Greek warrior helmet appearance”is the most characteristic feature, with a prominent glabella, high arched eyebrow, broad nasal bridge, and hypertelorism.
Cleft lip with or without cleft palate is observed in 30% of patients. Dental structure anomalies also exist including multiple tooth agenesis and over-retained primary molars caused by MSX1 gene impairment, and cone-shaped and taurodontic teeth. This case, a 9-year-old girl with WHS, showed intellectual disability, delayed growth development, previous occurrence of seizures, otitis media, and the typical facial features of WHS. Dental findings included multiple congenital missing teeth, over-retained primary teeth, and severe caries on the primary molars. Dental treatments were performed under general anesthesia. This report documents the characteristics of WHS, including general and oral features, and discusses the importance of oral hygiene and preventive dental management.
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KEYWORD
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Wolf-Hirschhorn syndrome, Dental findings, Preventive dental management
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