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Wiedemann-Steiner syndrome: A case report.
1Dental and Maxillofacial Department Great Ormond Street Hospital London UK.
Clinical Case Reports
|March 26, 2021
Summary
Wiedemann-Steiner syndrome, a rare genetic disorder, can affect dental development. This case highlights a 7-year-old girl experiencing premature loss of baby teeth and early growth of adult teeth.
Area of Science:
- Genetics
- Pediatrics
- Dentistry
Background:
- Wiedemann-Steiner syndrome (WDSTS) is an autosomal dominant disorder characterized by significant phenotypic variability.
- Key features include dysmorphic facial and skeletal features, growth deficiency, developmental delay, hypertrichosis cubiti, and diverse dental anomalies.
Observation:
- A 7-year-old female patient presented with unusual dental development.
- The patient exhibited premature exfoliation of primary teeth (baby teeth).
- Simultaneously, there was premature eruption of permanent teeth.
Findings:
- This case illustrates a specific dental manifestation within the spectrum of Wiedemann-Steiner syndrome.
- The observed premature dental development (both loss and eruption) is a notable feature.
- This presentation adds to the understanding of WDSTS phenotypical variation.
Implications:
- Early recognition of dental anomalies in WDSTS is crucial for timely intervention.
- Further research is needed to elucidate the mechanisms behind dental abnormalities in WDSTS.
- This case underscores the importance of comprehensive dental evaluations in patients with rare genetic syndromes.
