Early prosthetic treatment in children with ectodermal dysplasia
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BACKGROUND: Ectodermal dysplasia affects at least two ectoderm-derived structures such as hair, nails, skin, sweat glands and teeth. The dentition in hypohidrotic ectodermal dysplasia (XHED) is altered with many phenotypes ranging from oligodontia to anodontia. No consensus exists on the ideal age for beginning of prosthetic rehabilitation. CASE REPORT: A 2-year-old male patient with severe oligodontia in the primary dentition was referred for examination, evaluation and treatment. The child exhibited classical XLHED features with sparse hair, absence of eyelashes and eyebrows, severe hypohidrosis associated with heat intolerance and skin dryness. Radiographic examination revealed only intra-osseous maxillary primary canines. He presented an apparent prognathism of partially functional cause, retrusion of the maxillary and lateral deviation of the mandible. TREATMENT: The rehabilitation protocol was similar to an edentulous adult but extra care was taken to avoid discomfort to this very young patient. For example, alginate was used for the impressions for its neutral taste, ease of use, and quick setting speed. An hour was given for the child to adapt to the maxillary prosthesis and then the mandibular one was fitted. Denture adhesive was used to maximize retention. The child succeeded in eating some dry biscuits and drinking some water, demonstrating the exceptional potential for adaptation children usually present with at this very young age. FOLLOW-UP: Three months later, the prognathic tendency had vanished and he could eat hard food. Periodic recall visits were scheduled and at six months and normalization of oral functions was almost complete. Three years later, a new set of prostheses was made very easily as the child knew the protocol and was used to dentures wearing. CONCLUSIONS: Prosthetic rehabilitation must be done at the earliest age possible in order to maintain and correct the oral functions and prevent growth anomalies. It makes the subsequent treatment steps easier. Early rehabilitation and follow-up seems to be one of the keys to a successful treatment that help these children overcome their handicap and integrate them into society.
- Bal E, Baala L, Cluzeau C, et al. Autosomal dominant anhidrotic ectodermal dysplasias at the EDARADD locus. Hum Mutat 2007;28:703–9. CrossRef
- Bergendal B, Dahl N. Consensus conference of ectodermal dyoplasia with special reference to dental treatment. In Gothia F(Ed) University of Stockholme, 1998.
- Bergendal B. The role of prosthodontists in habilitation and rehabilitation in rare disorders: the ectodermal dysplasia experience. Int J Prosthodont 2001;14:466–70.
- Bergendal B. Children with ectodermal dysplasia need early treatment. Spec Care Dentist 2002;22:212–3. CrossRef
- Bondarets N, Jones RM, McDonald F. Analysis of facial growth in subjects with syndromic ectodermal dysplasia: a longitudinal analysis. Orthod Craniofac Res 2002;5:71–84. CrossRef
- Cluzeau C, Hadj-Rabia S, Jambou M, et al. Only four genes (EDA1, EDAR, EDARADD and WNT10A) account for 90 % of hypohidrotic/anhidrotic ectodermal dysplasia cases. Human Mutation 2010;In Press.
- Glavina D, Majstorovic M, Lulic-Dukic O, Juric H. Hypohidrotic ectodermal dysplasia: dental features and carriers detection. Coll Antropol 2001;25:303–10.
- Headon DJ, Emmal SA, Ferguson BM, et al. Gene defect in ectodermal dysplasia implicates a death domain adapter in development. Nature 2001;414:913–6. CrossRef
- Huth K, Sagner T, Hickel R. Interdisciplinary rehabilitation and prevention in a case with early and extensive loss of primary teeth. J Clin Pediatr Dent 2002;26:125–30.
- Irvine AD. Towards a unified classification of the ectodermal dysplasias: opportunities outweigh challenges. Am J Med Genet A 2009;149A:1970–2. CrossRef
- Itin PH. Rationale and background as basis for a new classification of the ectodermal dysplasias. Am J Med Genet A 2009;149A:1973–6. CrossRef
- Johnson EL, Roberts MW, Guckes AD, et al. Analysis of craniofacial development in children with hypohidrotic ectodermal dysplasia. Am J Med Genet 2002;112:327–34. CrossRef
- Kere J, Srivastava AK, Montonen O, et al. X-linked anhidrotic (hypohidrotic) ectodermal dysplasia is caused by mutation in a novel transmembrane protein. Nat Genet 1996;13:409–16. CrossRef
- Kotsiomiti E, Arapostathis K, Kapari D, Konstantinidis A. Removable prosthodontic treatment for the primary and mixed dentition. J Clin Pediatr Dent 2000;24:83–9.
- Lesot H, Clauss F, Maniere MC, Schmittbuhl M. Consequences of X-linked hypohidrotic ectodermal dysplasia for the human jaw bone. Front Oral Biol 2009;13:93–9. CrossRef
- Levin LS. Dental and oral abnormalities in selected ectodermal dysplasia syndromes. Birth Defects Orig Artic Ser 1988;24:205–27.
- Lexner MD, Bardow A, Hertz JM, et al. Anomalies of tooth formation in hypotidrotic ectodermal dysplasia. Int J Paediatr Dent 2007;17:10–18. CrossRef
- Monreal AW, Ferguson BM, Headon DJ, et al. Mutations in the human homologue of mouse dl cause autosomal recessive and dominant hypohidrotic ectodermal dysplasia. Nat Genet 1999;22:366–9. CrossRef
- Pinheiro M, Freire-Maia N. Ectodermal dysplasias: a clinical classification and a causal review. Am J Med Genet 1994;53:153–62. CrossRef
- Sforza C, Dellavia C, Goffredi M, Ferrario VF. Soft tissue facial angles in individuals with ectodermal dysplasia: A three-dimensional noninvasive study. Cleft Palate Craniofac J 2006;43:339–49.
- Vierucci S, Baccetti T, Tollaro I. Dental and craniofacial findings in hypohidrotic ectodermal dysplasia during the primary dentition phase. J Clin Pediatr Dent 1994;18:291–7.
- Early prosthetic treatment in children with ectodermal dysplasia
European Archives of Paediatric Dentistry
Volume 11, Issue 6 , pp 301-305
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- ectodermal dysplasia
- case report
- dental prosthesis
- paediatric dentistry