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[Case report on the Ehlers-Danlos syndrome]
Shoni Shikagaku Zasshi. the Japanese Journal of Pedodontics
|January 1, 1989
Summary
Ehlers-Danlos syndrome (EDS) Type III in a child presented with numerous complications, including skeletal abnormalities and dental issues. Histopathological analysis revealed collagen and elastic fiber hypoplasia, confirming connective tissue abnormalities.
Area of Science:
- Genetics
- Connective Tissue Diseases
- Pediatric Dentistry
Background:
- Ehlers-Danlos syndrome (EDS) is a group of inherited connective tissue disorders.
- Key features include skin hyperextensibility, joint hypermobility, and tissue fragility.
- Type III EDS is characterized by moderate joint hypermobility.
Observation:
- A 4-year-old boy diagnosed with Ehlers-Danlos syndrome Type III was studied.
- The case presented with multiple additional complications, including exotropia and hip dislocation.
- Histopathological examination showed hypoplasia of collagen and elastic fibers.
Findings:
- Dental findings included an edge-to-edge occlusion, high-arched palate, and contracted dental arches.
- Reduced mesiodistal tooth lengths and dental arch dimensions were noted compared to standards.
- Radiographic analysis indicated delayed tooth calcification, root canal strictures, and retarded craniofacial growth.
Implications:
- This case highlights the diverse and complex clinical manifestations of Ehlers-Danlos syndrome Type III in children.
- The findings underscore the systemic impact of connective tissue abnormalities on skeletal and dental development.
- Further research is needed to understand the long-term consequences and management strategies for pediatric EDS cases.