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Case report: Early prosthetic treatment in children with ectodermal dysplasia
M A Derbanne1, M C Sitbon, M M Landru
1Department of Dentistry, Albert Chenevier-Henri Mondor Hospital, APHP, Creteil, Service d'Odontologie, 40 avenue de Mesly, 94010 Créteil Cedex, France.
Summary
Early prosthetic rehabilitation for children with severe hypohidrotic ectodermal dysplasia (XHED) is crucial. This approach helps correct oral functions, prevent growth anomalies, and improve social integration for affected individuals.
Area of Science:
- Pediatric Dentistry
- Craniofacial Anomalies
- Genetic Disorders
Background:
- Hypohidrotic ectodermal dysplasia (XHED) is a genetic disorder affecting ectodermal structures, including teeth, leading to oligodontia or anodontia.
- Current consensus on the optimal age for prosthetic rehabilitation in XHED patients is lacking.
- XHED presents significant challenges in dental development and oral function from an early age.
Observation:
- A 2-year-old male with severe oligodontia and classic XHED features (sparse hair, hypohidrosis) presented with functional prognathism and mandibular deviation.
- Radiographic examination revealed only intra-osseous maxillary primary canines.
- The patient exhibited significant challenges with oral function due to severe dental anomalies.
Findings:
- Prosthetic rehabilitation, adapted for a young child, successfully restored oral functions, including mastication and speech.
- Within three months, the patient's prognathic tendency resolved, and he could consume hard foods.
- Six-month follow-up showed near-complete normalization of oral functions, with subsequent prostheses fabrication being straightforward due to patient adaptation.
Implications:
- Early prosthetic rehabilitation in XHED patients is vital for maintaining oral function and preventing craniofacial growth anomalies.
- Timely intervention facilitates subsequent dental treatments and improves the child's ability to integrate socially.
- This case highlights the potential for successful prosthetic management in very young children with XHED, emphasizing early intervention and consistent follow-up.
