Customized Prosthetic Oral Rehabilitation of a Child with Hypohidrotic Ectodermal Dysplasia: A Case Report

Hitesh Chander Mittal¹ https://orcid.org/0000-0002-5610-8171, Sudhir Bhandari², Ashima Goyal³, Aditi Kapur⁴, Krishan Gauba⁵

¹Department of Dentistry, Pandit Deen Dayal Upadhyay Medical College, Churu, Rajasthan, India

²Unit of Prosthodontics, Department of Oral Health Sciences Center, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India

^3–5Unit of Pedodontics and Preventive Dentistry, Department of Oral Health Sciences Center, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India

Corresponding Author: Hitesh Chander Mittal, Department of Dentistry, Pandit Deen Dayal Upadhyay Medical College, Churu, Rajasthan, India, Phone: +91 8607707755, e-mail: dr.hiteshmittal@gmail.com

Received: 18 December 2023; Accepted: 29 January 2024; Published on: 27 April 2024

ABSTRACT

The present case report describes interdisciplinary prosthetic oral rehabilitation of a 7-year-old male child presenting classical features of hypohidrotic ectodermal dysplasia (HED) and subtotal anodontia. The treatment, in this case, was simplified and customized to address various challenges viz. behavioral management, limited hope, low education, and socioeconomic status as well as preserving the erupted two molars. The removable denture was designed to utilize erupted molars for retention with the help of thermoplastic material. The simplification of fabrication steps leads to the successful restoration of a young HED child’s masticatory function and esthetics, as well as increased hope and cooperation for future definitive treatment.

How to cite this article: Mittal HC, Bhandari S, Goyal A, et al. Customized Prosthetic Oral Rehabilitation of a Child with Hypohidrotic Ectodermal Dysplasia: A Case Report. J South Asian Assoc Pediatr Dent 2024;7(1):38–41.

Source of support: Nil

Conflict of interest: None

Patient consent statement: The author(s) have obtained written informed consent from the patient’s parents/legal guardians for publication of the case report details and related images.

Keywords: Anodontia, Case report, Child, Dentures, Ectodermal dysplasia, Esthetics, Hypohidrotic

INTRODUCTION

Hypohidrotic ectodermal dysplasia (HED) occurs with a frequency between 1:10,000 and 1:100,000 live births and manifests hypodontia as a classical feature.¹^-⁴ There is no general cure for HED, but oral rehabilitation (replacing missing teeth, restoring normal vertical dimension, and providing support for facial soft tissues) is recommended at the earliest age possible.⁵ Oral rehabilitation is fundamental to the normal appearance, functional needs, optimized social integration, and thus, overall patient’s quality of life.²^,⁴^-⁶

The conventional treatment options for oral rehabilitation of patients with subtotal anodontia include overdenture, complete denture, or implant-supported denture. In general, clinicians encounter case-specific unique challenges besides limited experience in prosthetic rehabilitation of children with genetic syndrome.⁴^,⁷ Thus, oral rehabilitation in such cases needs to be simplified³^,⁶ and customized⁶^,⁷ to address various challenges posed by individual cases viz variable number, irregular distribution and abnormal shape and size of teeth as well as limited hope, low education and poor socioeconomic status as well as preserving natural teeth. The present case report discusses simplified and customized treatment for successful prosthetic oral rehabilitation of a child diagnosed with HED using thermoplastic removable dentures.

CASE DESCRIPTION

A 7-year-old boy diagnosed with HED was referred for oral rehabilitation for subtotal anodontia. The patient presented classical features (Fig. 1) viz frontal bossing, saddle nose, sparse fine scalp hair, and dry skin with linear perioral wrinkles. Other features include heat intolerance, absent finger dermatoglyphics, hypoplastic alveolar ridges in class III relation, and thick and protuberant lips. Family history was noncontributory, with nonconsanguineous parents indicating to be index cases. The patient presented various challenges viz—(1) child-parent submissive behavior, (2) low level of education in the family, (3) limited compliance due to remote location, (4) limited hope and financial problems, and (5) request for nonsurgical treatment only.

Figs 1A to C: Preoperative. (A) Facial profile-classical old man appearance; (B) Intraoral view—anodontia with two maxillary atypical shaped molar teeth; (C) OPG view—showing the presence of two impacted irregular conical shaped maxillary incisors

The treatment was customized with appropriate simplification for preserving unfavorably placed molar teeth. Thermoplastic resin complete denture for the maxillary arch with horseshoe design and window for existing teeth was decided. Thermoplastic resin complete denture for the mandibular arch was planned with the possibility of incorporating implants. Informed consent was taken followed by education of parents and child about the outcome as well as the procedure of prosthesis fabrication through photographs and models.

The denture was fabricated in five major steps (Figs 2 and 3). Firstly, mandibular diagnostic impressions were made with the smallest fitting, plastic perforated trays with flavored fast-setting alginate (Tropicalgin, Zhermack, Italy), followed by maxillary impression to increase comfort and acceptability. The plastic trays are less fear-provoking compared to steel stock trays and offer increased comfort as well as ease of placement. Tell show do, contingency management, giving control to the child by raising a hand, and distraction techniques like deep breathing, and counting of breaths were used. The second step was final impression making, which included—(1) cold cure acrylic resin custom trays were preferred over shellac because of good stability; (2) functional molding of custom tray borders was done with green stick compound to achieve better retention; (3) light-body vinyl polysiloxane material (AFFINIS, Coltene/Whaledent AG, Switzerland) provided cleaner, fast and accurate impression; (4) dual impression technique to include the erupted teeth in the maxillary final cast. The third fabrication step was jaw relation recording, which included (1) the alignment of upper wax rim (fabricated over Shellac base plate) to the camper’s plane; (2) the occlusal vertical dimension was restored to a clinically acceptable position using conventional methods of analysis using esthetics, phonetics, freeway space, and swallowing; (3) face bow transfer was not made due to its limited advantages and cumbersome procedure, (4) the centric relation was registered by simple, unstressing clinical methods using shellac base occlusal rims. The fourth step was custom-made age-appropriate teeth fabrication and teeth arrangement in bilateral balanced occlusion based on the arch space availability (minimum thickness of labial flange given because of already protuberant lips). The fifth step included denture design, incorporating maxillary molars into denture design and processing of denture with thermoplastic resin (Valplast Int Corp, United States of America). The choice of flexible denture material allowed the incorporation of the two unfavorable placed molar teeth without altering the shape and alignment.

Figs 2A to D: Fabrication steps for prosthesis. (A) Functional border molding and final impression in cold cure acrylic resin custom trays; (B) Master casts; (C) Shellac base occlusal rims; (D) Teeth arrangement

Figs 3A to C: Custom-made age-appropriate teeth fabrication. (A) Elastomeric impression mold of deciduous dentition; (B) Mold replica with tooth-colored resin material; (C) Trimmed and polished custom-made teeth

The maxillary denture exhibited excellent retention and stability, while the mandibular had moderate retention with good stability (Fig. 4). The parent and child were educated about the maintenance and the need to revise the prosthesis periodically to accommodate changes in growth. At a 1- and 3-week recall, the patient’s father reported his excellent acclimatization to the prosthesis. Recall appointments were scheduled every 6-month interval.

Figs 4A to C: Postinsertion. (A) Profile-improved appearance and self-confidence; (B and C) Intraoral maxillary and mandibular; protrusive intraoral with stable dentures

DISCUSSION

Pediatric prosthodontic rehabilitation requires an interdisciplinary²^,⁴ team knowledgeable in growth and development, behavioral profile, prosthesis fabrication techniques, and minimally invasive treatment plans. There are various treatment recommendations available in the literature for children with HED.²^,⁴^,⁶^,⁸ Oral rehabilitation options for children with HED viz complete dentures,³ removable partial dentures,⁶ over dentures,⁶^,⁹ and osseointegrated dental implant⁸ individually or in combination have been reported. The implant placement should be avoided until maximum jaw growth has occurred, that is, 13–15 years of age, because of the possibility of implant movement.²^,⁵^,⁸ However, there is a growing trend for implant-supported complete dentures in children older than 7 years of age with HED to overcome retention and stability issues of removable dentures.²^,⁴^,⁸ In the present case, it was deemed better to postpone osseointegrated implants due to various concerns viz, the necessity of preimplant bone augmentation and general anesthesia, financial and biologic cost, compliance, and implant hygiene as compared to the benefits gained from a more stable implant-supported prosthesis.⁴ Oesterle¹⁰ also emphasized conventional prostheses before initiating the implant-assisted treatment. Also, many authors⁴^,⁵^,⁷ reported good adaptation to complete dentures in the early age-group of 2–5 years. In this case, optimal retention and stability were achieved through a precise border seal, extending the denture base to include the entire vestibular sulcus reflection and wide distribution of occlusal load with relief in knife-edge alveolar regions.⁵^,⁷

The unfavorably placed maxillary molar teeth presented another challenge of invasive and complex treatment. It was preferred to preserve these firm, noncarious molars following Schnabl et al.⁴ and in contrast to reporting by Jain et al.⁹ Over-dentures are a desirable option when natural teeth are present,² however, the procedure is often invasive and extensive, that is, require endodontic treatment and crown preparation for extracoronal coping.²^,⁶^,⁹ The thermoplastic removable denture using undercut of molar teeth for retention due to the path of insertion and flexibility of denture was a good alternative in this case. The horseshoe design in this report aids in greater flexibility besides better adaptability in terms of speech and thermal perception.

Another prerequisite is age-appropriate denture teeth to look like peers,²^,³^,⁵ and these were custom fabricated to match deciduous dentition in this report. Bidra et al.³ recommended custom fabricating age-appropriate denture teeth, while Tarjan et al.⁵ modified adult denture teeth to provide optimal esthetics and acceptability. The acrylic teeth were chosen because of comparatively less transmission of forces to the mucosa, occlusal adjustment, and freedom of jaw movement.

Also, the parents and child required constant motivation through pictures and models for the clinical procedure and reassurance of no harm. The behavior modification was done through the tell-show-do technique,² simplifications of each clinical step,³^,⁹ and making the child comfortable with various materials and tools. Children and parents might have abandoned complex treatments like implants. The successful early oral rehabilitation in the present case was possible due to a minimally invasive, customized, and simplified treatment plan. These interim prostheses increased the hope and cooperation for future complex treatment options for restoring a child’s masticatory function as well as esthetics. The limitation of the treatment in this case is the frequent requirement of prosthesis modification and refabrication to accommodate continuing jaw growth. Another limitation includes the unavailability of pediatric teeth matching sex and ethnicity. Nevertheless, this rehabilitation brought a smile to the child’s face and will encourage the clinicians to seek pediatric prosthodontics to improve the child’s psychosocial development. Thus, it can be concluded that simplification of prosthesis leads to the successful restoration of a young HED child’s oral function as well as increased hope and cooperation for future definitive treatment.

ORCID

Hitesh Chander Mittal https://orcid.org/0000-0002-5610-8171

REFERENCES

1. Buyse ML. Birth Defects Encyclopedia. Chicago, St Louis: Mosby; 1990. pp 597–598.

2. Pigno MA, Blackman RB, Cronin RJ Jr, et al. Prosthodontic management of ectodermal dysplasia: a review of the literature. J Prosthet Dent 1996;76(5):541–545. DOI: 10.1016/s0022-3913(96)90015-3

3. Bidra AS, Martin JW, Feldman E. Complete denture prosthodontics in children with ectodermal dysplasia: review of principles and techniques. Compend Contin Educ Dent 2010;31(6):426–433.

4. Schnabl D, Grunert I, Schmuth M, et al. Prosthetic rehabilitation of patients with hypohidrotic ectodermal dysplasia: a systematic review. J Oral Rehabil 2018;45(7):555–570. DOI: 10.1111/joor.12638

5. Tarjan I, Gabris K, Rozsa N. Early prosthetic treatment of patients with ectodermal dysplasia: a clinical report. J Prosthet Dent 2005;93(5):419–424. DOI: 10.1016/j.prosdent.2005.01.012

6. Maroulakos G, Artopoulou II, Angelopoulou MV, et al. Removable partial dentures vs overdentures in children with ectodermal dysplasia: two case reports. Eur Arch Paediatr Dent 2016;17(3):205–210. DOI: 10.1007/s40368-015-0212-x

7. Kościelska N, Bogucki Z. Clinical factors in prosthodontic treatment of children with genetic defects. Adv Clin Exp Med 2017;26(6):1005–1012. DOI: 10.17219/acem/63094

8. Guckes AD, Scurria MS, King TS, et al. Prospective clinical trial of dental implants in persons with ectodermal dysplasia. J Prosthet Dent 2002;88(1):21–25. DOI: 10.1067/mpr.2002.127099

9. Jain D, Jain S, Kumar A, et al. Hypohidrotic ectodermal dysplasia: prosthetic and endodontic management. Int J Clin Pediatr Dent 2010;3(1):63–67. DOI: 10.5005/jp-journals-10005-1056

10. Oesterle LJ. Orthodontic Applications of Osseointegrated Implants. Chicago, United States: Quintessence Publishing Co; 2000. pp. 133–159.

________________________
© The Author(s). 2024 Open Access. This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (https://creativecommons.org/licenses/by-nc/4.0/), which permits unrestricted use, distribution, and non-commercial reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.