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Journal of Indian Society of Pedodontics and Preventive Dentistry Official publication of Indian Society of Pedodontics and Preventive Dentistry
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CASE REPORT
Year : 2008  |  Volume : 26  |  Issue : 4  |  Page : 177-181
 

Prosthetic rehabilitation of an adolescent with hypohidrotic ectodermal dysplasia with partial anodontia: Case report


1 MDS (Prosthodontics), ITS-CDSR, Ghaziabad, Uttar Pradesh, India
2 MDS (Pedodontics), Panineeya Mahavidyalaya Institute for Dental Science and Research Centre, Dilsukhnagar, Ranga Reddy District, Andhra Pradesh, India

Correspondence Address:
S Kaul
91, Block I, First Floor, Eros Garden, Charmwood Village, Surajkund, Faridabad, Haryana-121 009
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0970-4388.44041

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   Abstract 

Ectodermal dysplasia is a hereditary syndrome characterized by dysplasia of tissues of ectodermal origin (hair, skin, nails, and teeth) and occasionally, dysplasia of mesodermally derived tissues. The triad of nail dystrophy (onychodysplasia), alopecia, or hypotrichosis (scanty, fine, light hair on the scalp and eyebrows) and palmoplantar hypohidrosis is usually accompanied by lack of sweat glands and partial or complete absence of primary and permanent dentition. Hypohidrotic ectodermal dysplasia usually has an X-linked inheritance and affects only males severely, while female heterozygotes show only minor defects. The clinical management of children with ectodermal dysplasia provides a unique opportunity for cooperative effort between the pedodontist and the prosthodontist. The following case report discusses the management of a young boy with hypohidrotic ectodermal dysplasia. Removable prostheses were employed in the treatment. The aim was to rehabilitate the adolescent prosthodontically and boost him psychologically.


Keywords: Hypohidrotic ectodermal dysplasia, partial anodontia, prosthetic therapy


How to cite this article:
Kaul S, Reddy R. Prosthetic rehabilitation of an adolescent with hypohidrotic ectodermal dysplasia with partial anodontia: Case report. J Indian Soc Pedod Prev Dent 2008;26:177-81

How to cite this URL:
Kaul S, Reddy R. Prosthetic rehabilitation of an adolescent with hypohidrotic ectodermal dysplasia with partial anodontia: Case report. J Indian Soc Pedod Prev Dent [serial online] 2008 [cited 2019 Nov 19];26:177-81. Available from: http://www.jisppd.com/text.asp?2008/26/4/177/44041



   Introduction Top


Ectodermal dysplasias represent a large and complex nosological group of congenital diseases which were first described by Thurnam in 1948 and later in the 19 th century by Darwin. Ectodermal dysplasia was assigned to the X chromosome in 1921 by Thadani, who later reported that carrier females could manifest signs of the condition. Their incidence is relatively rare (1/100000 births) and is characterized by the abnormal development of the ectodermal derivaties. [1]


   Case Report Top


A 14-year-old boy presented with a complaint of missing teeth to the Department of Pedodontics and preventive dentistry, SudhaRustagi College of Dental Sciences and Research, Faridabad. He was accompanied by his mother who gave a history of the boy's missing teeth since infancy, except for two teeth in the upper jaw which erupted when the boy was 6-years old. She also gave a history of frequent bouts of fever throughout infancy and childhood, and mentioned that he was intolerant to heat and takes frequent dips in summer to keep cool. There was no family history of missing teeth. Neither his siblings (a brother and a sister) nor his parents or his grandparents had any missing teeth.

The boy was moderately built and poorly nourished. His skin was dry and the body hair was scanty. He had the typical face of a ectodermal dysplasia patient with features like: frontal bossing, periorbital pigmentation, depressed nasalbridge, protuberant lips, low-set ears, and scanty scalp hair. [2],[3] The nails were not very brittle [Figure 1],[Figure 2],[Figure 3],[Figure 4].

Intraoral examination revealed a relatively dry muscosa. Mandibular arch was completely edentulous with poorly developed alveolus and the maxillary arch had two standing molars [Figure 5] and [Figure 6]. Radiographic investigations (OPG and lateral cephalogram) showed malformed incisors impacted in the maxillary arch [Figure 7] and [Figure 8]. Since hypohidrosis, hypotrichosis, and hypodontia were very evident on physical examination, the boy was diagnosed with Hypohidrotic Ectodermal Dysplasia (HED) with partial anodontia.

Prosthodontic management

The prosthodontic management in children suffering from HED depends on the degree of anodontia/hypodontia. In complete anodontia, the treatment would comprise of complete dentures, either conventional or implant supported ones. [4] In patients with partial anodontia, removable/fixed partial dentures and over dentures may be considered. In the present case, a removable partial denture in the maxillary arch and a conventional complete denture in the mandibular arch were planned.

The right maxillary molar was restored with GIC (Fuji II LC,GC Corp, Tokyo, Japan) prior to impression making. Preliminary impressions were made using alginate (Zelgan 2002, Dentsply, India). Special trays were fabricated using autopolymerizing acrylic resin (DPI-RRCold cure acrylic resin, DPI, Mumbai, India) after applying spacers (Modelling wax, MDM Corp, India) to relieve the midpalatine raphe, incisive papilla, and the standing molars in the maxillary arch and the crest of the ridge in the mandibular arches. Border molding was done using green stick compound (Pinnacle Tracing sticks, DPI, Mumbai, India). Secondary impressions were made using Zinc Oxide Eugenol Impression (ZnOE) paste (Impression Paste, DPI, Mumbai, India) for the mandibular arch and light viscosity addition silicone (Reprosil, Dentsply, India) for the maxillary arch [Figure 9] and [Figure 10]. Record bases (Cold cure acrylic resin, DPI and Modelling wax, MDM Corp,India) were made and jaw relations registered.

Selection of teeth for children and adolescents requires more attention as the commercially available moulds do not satisfy esthetic requirements in most cases; obviously because these moulds mimic adult dentitions. [5] Anterior mould was chosen (Premadent, Dr. Jagdishlal Sethi, India) using intercanine distance. A vigorous-looking mould was chosen to provide a masculine appearance. But considering the age of the boy and the soft facial features, we tried to tone down the masculine-looking mould by arranging the maxillary anteriors to mimic a C1 II, div ii case [Figure 11]. Mild crowding was replicated in the mandibular anteriors to enhance the natural appearance [Figure 12]. Posterior teeth were arranged according to the boy's natural teeth. Trial dentures were checked for retention, phonetics, occlusion, and esthetics. Acrylic dentures were processed (DPI Heat Cure Acrylic Resin, DPI) through compression molding and the polished dentures were inserted [Figure 13] and [Figure 16]. The boy was taught the insertion and removal of dentures and was given postinsertion instructions on denture wear, functions of speech, mastication, hygiene, and maintenance. He was instructed to speak and read aloud daily for sometime in order to improve phonation. Regarding diet, he was restricted to mashed potatoes, rotis softened in gravy, biscuits, and liquids like milk, tea, etc. We asked him to continue the liquid/semi-solid diet till he was completely at ease while eating with the dentures. We explained and emphasized the importance of the existing maxillary molars and the need to maintain them. He was recalled 24 hours after denture insertion for the first postinsertion checkup. Later, he was kept on monthly recall visits. He reported inconvenience in mastication for more than two weaks even with a semisolid diet. But the esthetic satisfaction was so immense that he wore the denture and willingly adapted to masticate and speak with them.

Psychological aspect

During his first visit, the boy was visibly shy and not very articulate. His mother would reply most of the queries. We started with close ended questions initially (to which the boy had to give a simple "yes" or "no") and later led him through small conversations by posing open ended queries about his school, friends, diet, etc. and finally could establish a comfortable rapport. We gathered that he was an intelligent boy with a good academic record who turned introvert due to occasional mockery at school. He was definitely more cheerful and interactive at the subsequent recalls and impressively meticulous with denture and oral hygiene. It was extremely gratifying to know that the dentures made a significant difference in his overall personality.


   Discussion Top


The treatment of all patients with craniofacial anomalies includes not only the management of the defect or anomaly itself, but also the psychological management of the person as a whole. [6],[7] So, the treatment plan for a child suffering from HED should always include a prosthodontic aspect and a psychological aspect. [8] Though removable complete/partial dentures still remain the main stay of treatment in many cases, FPDs and implant-supported prostheses are to be considered when deemed viable. Implants seem to be particularly indicated in cases with complete anodontia.In children where severe midface hypoplasia is anticipated, early growth modification and implants may be helpful. Although dentures are poor alternatives to healthy dentition, they create conditions for maintenance of a normal, satisfactory daily diet, thus helping to establish a lifelong dietary pattern at an early age. Also, in the absence of occlusal stops (or dentures), the anterorotation of the mandible causes an upward and forward displacement of the chin, with a reduction in the height of the lower-third of the face; a tendency to C1 III malocclusion. Dentures help positioning of the chin in place. [9]

Periodic recalls are often necessary in young adults as a result of continuing growth and development that mandates adjustments in the prostheses. [10]


   Conclusion Top


Early prosthetic therapy for children with ectodermal dysplasia provides a unique opportunity for cooperative effort between the pedodontist and the prosthodontist, as pedodontists are better trained in the psychological management of children. Prosthodontic treatment may commence at an early age of 3-4 years as it enhances conditions for growth and development of orofacial structures. It helps normalize the function of masticatory and perioral muscles and consequently the growth pattern of basal bones and gives a psychological boost to the self-image of the child. [11]

The ultimate long-term goal is a young adult who is functionally habilitated and psychologically adjusted because the scientific fact that the disease is rare is of no consolation to its victim.


   Acknowledgment Top


Dr. Rahul Munjal, (BDS, Sudha Rustagi College of Dental Sciences and Research, Faridabad) for technical assistance during laboratory procedures.[Figure 14],[Figure 15]



 
   References Top

1.Clarke A. Hypohidrotic ectodermal dysplasia. J Med Genet 1987;24:659-63.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]
2.Crawford PJ, Aldred MJ, Clarke A. Clinical and radiographic dental findings in X linked Hypohidrotic ectodermal dysplasia. J Med Genet 1991;28:181-5.  Back to cited text no. 2  [PUBMED]  [FULLTEXT]
3.Neville, Damm, Allen, Bouquot. Oral and maxillofacial pathology. 2 nd ed. Elsevier; p. 644-5.  Back to cited text no. 3    
4.Johnson EL, Roberts MW, Guckes AD, Bailey LJ, Phillips CL, Wright JT. Analysis of craniofacial development in children with Hypohidrotic ectodermal dysplasia. Am J Med Genet 2002;112:327-34.  Back to cited text no. 4    
5.Vieira KA, Teixeira MS, Guirado CG, Gaviγo MB. Prosthodontic treatment of hypohidrotic ectodermal dysplasia with complete anodontia: Case report. Quintessence Int 2007;38:75-80.  Back to cited text no. 5    
6.Endriga MC, Kapp-Simon KA. Psychological issues in craniofacial care: State of the art. Cleft palate Craniofac J 1999;36:3-11.  Back to cited text no. 6    
7.Hickey AJ, Salter M. Prosthodontic and psychological factors in treating patients with congenital and craniofacial defects. J Prosthet Dent 2006;95:392-6.   Back to cited text no. 7    
8.Bolender CL, Law DB, Austin LB. Prosthodontic treatment of ectodermal dysplasia: A case report. J Prosthet Dent 1995;19:167-72.  Back to cited text no. 8    
9.Tarjan I, Katalin G, Noemi R. Early prosthetic treatment of patients with ectodermal dysplasia: A clinical report. J Prosthet Dent 2005;93:419-24.  Back to cited text no. 9    
10.Shaw RM. Prosthetic management of hypohydrotic ectodermal dysplasia with anodontia: Case report. Aust Dent J 1990;35:113-6.  Back to cited text no. 10    
11.Franchi L, Branchi R, Tollaro I. Craniofacial changes following early prosthetic treatment in a case of hypohidrotic ectodermal dysplasia with complete anodontia. J Dent Child 1998;65:116-21.  Back to cited text no. 11    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11], [Figure 12], [Figure 13], [Figure 14], [Figure 15], [Figure 16]


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    Abstract
    Introduction
    Case Report
    Discussion
    Conclusion
    Acknowledgment
    References
    Article Figures

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