Related Experiment Videos
Cornelia de Lange syndrome with cleft palate
K Yamamoto1, K Horiuchi, K Uemura
1Department of Oral and Maxillofacial Surgery, Nara Medical University, Japan.
International Journal of Oral and Maxillofacial Surgery
|August 1, 1987
Summary
Cornelia de Lange syndrome (CdLS) cases with cleft palate present characteristic features and oral abnormalities like high palatal arches and partial anodontia. This study details these findings in four patients, referencing existing literature.
Area of Science:
- Medical Genetics
- Pediatric Dentistry
- Craniofacial Biology
Background:
- Cornelia de Lange syndrome (CdLS) is a rare genetic disorder affecting multiple organ systems.
- Cleft palate is a known, though not universal, manifestation in CdLS.
- Understanding oral manifestations is crucial for comprehensive patient management.
Observation:
- Four pediatric cases of CdLS exhibiting cleft palate were analyzed.
- Oral findings included high palatal arches, cleft of hard and soft palate, and submucous cleft palate.
- Associated dental anomalies such as partial anodontia and microdontia were observed.
Findings:
- All four cases presented with hallmark features of Cornelia de Lange syndrome.
- Specific oral manifestations varied, with cleft palate and related anomalies being prominent.
- Dental abnormalities like partial anodontia and microdontia were noted in conjunction with CdLS.
Implications:
- Highlights the importance of thorough oral examinations in CdLS patients.
- Contributes to the understanding of the spectrum of oral and dental anomalies in CdLS.
- Informs clinical practice regarding the diagnosis and management of CdLS-associated craniofacial differences.