Related Experiment Videos
Seckel syndrome: report of a case
Figen Seymen1, Bahar Tuna, Hulya Kayserili
1Department of Pedodontics, Faculty of Dentistry, University of Istanbul, Turkey. figenseymen@superonline.com
The Journal of Clinical Pediatric Dentistry
|May 7, 2002
Summary
This case report details a child with Seckel syndrome, highlighting unique dental anomalies. Early dental intervention is crucial for managing this rare genetic disorder.
Area of Science:
- Genetics
- Pediatrics
- Dentistry
Background:
- Seckel syndrome is a rare autosomal recessive disorder characterized by primordial dwarfism, microcephaly, and facial dysmorphisms.
- Dental anomalies are frequently associated with Seckel syndrome, but comprehensive documentation remains limited.
Observation:
- A 7-year-old boy presented with characteristic Seckel syndrome features including microcephaly, midfacial hypoplasia, and stunted stature.
- Clinical and radiographic dental examination revealed a Class II dentoskeletal pattern, open bite, microdontia, enamel hypoplasia, taurodontism, dentinal dysplasia, and congenitally missing teeth.
Findings:
- The patient exhibited borderline intellectual disability with normal motor development.
- A unique constellation of dental abnormalities was observed, underscoring the systemic impact of Seckel syndrome.
Implications:
- This case emphasizes the critical role of dentists in identifying and managing rare genetic syndromes like Seckel syndrome.
- Routine dental surveillance is essential for patients with Seckel syndrome to address complex oral health issues and improve overall patient care.