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Dental findings and management in cockayne syndrome: a case and a review

Author: 
Bourguiba Emna, Jazi Imene, Kraoua Ichraf, Ben Youssef-Turki Ilhem and Chemli Mohamed Ali
Subject Area: 
Health Sciences
Abstract: 

Cockayne syndrome is a rare autosomal recessive disease clinically characterized by growth failure, neurological and sensory dysfunction, photosensitivity and visual abnormalities. Dental and cranio-facial abnormalities are common but rarely studied. Methods: We performed a review of literature about oro-dental findings and dental management in Cockayne syndrome infants. We reported a 7-year-old girl with genetically confirmed Cockayne syndrome. We focused on oro-dental phenotype and management. Results: Sixteen articles were reviewed. Oro-dental findings reviewed were bad oral hygiene (N=5) (33.33%) , dental caries (N=16) (100%) , enamel hypoplasia (N=6) (37.5%) , white opacities(N=3) (18.75%) , narrow dental arches (N=4) (25%) , teeth malposition(N=4) (25%) ,agenesis (N=5) (31.25%), anomalies of form(N=4) (25%) , hyposialia (N=5) (31.25%), temporo-mandibular joint defect (N=1) (6.25 %) and other non-detailed features(N=4) (25%) . Dental management in CS patients was detailed in (N=7) (43.75%) of retained articles. Conclusion: Early dental check-up and management of CS is very important as preventive option. More oro-dental signs need to be explored as skeletal diagnosis, temporal-mandibular joints, and salivary and pulp tests.

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