Supernumeraries in Nicolaides-Baraitser Syndrome
Bouthayna Al-Tamimi1, Stefan Abela2, Huw G Jeremiah3
1Dental and Maxillofacial Department, Great Ormond Street Hospital for Children, London, UK.
International Journal of Paediatric Dentistry
|June 22, 2017
Summary
Nicolaides-Baraitser Syndrome (NCBRS) is a rare genetic disorder. This case highlights dental impactions and supernumerary teeth as potential NCBRS features requiring assessment.
Area of Science:
- Genetics
- Pediatrics
- Dentistry
Background:
- Nicolaides-Baraitser Syndrome (NCBRS) is an extremely rare genetic disorder.
- Characterized by global developmental delay, speech impairment, seizures, and distinct dysmorphic features.
Observation:
- This report details the 28th fully documented case of NCBRS.
- The patient presented with multiple dental impactions, a previously unreported feature in NCBRS literature.
Findings:
- NCBRS diagnosis confirmed through clinical features.
- Dental assessment revealed multiple supernumerary teeth, impacting occlusion.
Implications:
- Highlights the importance of comprehensive dental evaluation in NCBRS patients.
- Early identification of dental anomalies can prevent future complications and guide management.
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