Prosthetic rehabilitation of an adolescent with ...

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Prosthetic rehabilitation of an adolescent with hypohidrotic ectodermal dysplasia with partial anodontia: Case report KAUL S.1, REDDY R.2

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

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 [Figures 1–4].

Introduction Ectodermal dysplasias represent a large and complex nosological group of congenital diseases which were first described by Thurnam in 1948 and later in the 19th 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]

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

Case Report 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.

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

The boy was moderately built and poorly nourished. His skin was dry and the body hair was scanty. He had the typical face

1

MDS (Prosthodontics), ITS-CDSR, Ghaziabad, Uttar Pradesh, India. 2MDS (Pedodontics), Panineeya Mahavidyalaya Institute for Dental Science and Research Centre, Dilsukhnagar, Ranga Reddy District, Andhra Pradesh, India 177

J Indian Soc Pedod Prevent Dent - December 2008


Hypohidrotic ectodermal dysplasia with partial anodontia

Figure 1: Typical features of ectodermal dysplasia: depressed nasal bridge, reduced vertical dimension of face, protuberant lips, and low-set ears

Figure 2: Scanty scalp hair

Figure 3: Periocular pigmentation

Figure 4: Nail defects

Figure 5: Mandibular arch – completely edentulous

Figure 6: Maxillary arch with two standing molars.(severe hypodontia)

Figure 7: OPG showing malformed and impacted incisors

Figure 8: Lateral cephalogram depicting impacted malformed incisors, standing maxillary molars, and poor alveolar ridges


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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 [Figures 9 and 10]. Record bases (Cold cure acrylic resin, DPI and Modelling wax, MDM Corp,India) were made and jaw relations registered.

meticulous with denture and oral hygiene. It was extremely gratifying to know that the dentures made a significant difference in his overall personality.

Discussion 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]

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 [Figures 13 and 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.

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

Conclusion 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]

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

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 Dr. Rahul Munjal, (BDS, Sudha Rustagi College of Dental Sciences and Research, Faridabad) for technical assistance during laboratory procedures. 179




Figure 9: Secondary impression of maxillary arch (using light viscosity elastomer)

Figure 10: Secondary impression of mandibular arch (using ZnOE impression paste)

Figure 11: Maxillary denture showing the anterior teeth in closer view

Figure 12: Mandibular denture showing anterior teeth in closer view

Figure 13: Acrylic dentures inserted

Figure 14: Right intraoral view of dentures in occlusion

Figure 15: Left lateral intraoral view of dentures in occlusion

Figure 16: A confident smile with dentures in place


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Hypohidrotic ectodermal dysplasia with partial anodontia Prosthet Dent 2006;95:392-6. Bolender CL, Law DB, Austin LB. Prosthodontic treatment of ectodermal dysplasia: A case report. J Prosthet Dent 1995;19:16772. 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. 10. Shaw RM. Prosthetic management of hypohydrotic ectodermal dysplasia with anodontia: Case report. Aust Dent J 1990;35:113-6. 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.

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There is no data on follow-up of these patients. Authors’ Reply: The follow up of patients have been included in the results section [Page 3, para 2] Authors should highlight the relation of complication to duration of diabetes. Authors’ Reply: The complications as seen in our study group has been included in the results section [Page 4, Table]

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