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CASE REPORT
Year : 2019  |  Volume : 10  |  Issue : 3  |  Page : 76-79

Prosthodontic Rehabilitation of Hypohidrotic Ectodermal Dysplasia in Two Young Sisters With Fixed Prostheses: Clinical Case Report


1 Dental Research Center, Department of Prosthodontics, Tehran University of Medical Sciences, Tehran, Iran
2 Department of Prosthodontics, Faculty of Dentistry, University of Al-Ameed, Karbala, Iraq

Date of Web Publication28-Nov-2019

Correspondence Address:
Mohammed Hussein M Hussein Alsharbaty
Department of Prosthodontics, Faculty of Dentistry, University of Al-Ameed, Karbala
Iraq
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/denthyp.denthyp_9_19

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  Abstract 


Introduction: Full mouth rehabilitation in patients with ectodermal dysplasia (ED) is challenging to accomplish, especially because the affected individuals are quite young when they are assessed; therefore, esthetics is an imperative concern. This clinical report explains the oral rehabilitation of two sisters diagnosed with ED, which shows the optimistic effects on the physical, emotional, and social life of the patients. Case Report: In this clinical report, two treated sisters had anodontia that made difficulties in their eating and speaking. The two sisters had been treated with fixed partial dentures and implants supported prostheses after extracting nonrestorable deciduous teeth. The present permanent teeth were maintained to preserve the Periodontal ligament (PDL) and increase the patient occlusal awareness. Discussion: Elements that are necessary to be focused in designing dental treatments for ED patients include age, existent teeth, oral hygiene, psychosocial environment, Occlusal vertical dimension (OVD), bone volume, skeletal growth and development, orthodontics and/or orthognathic surgery, implants, time required for the procedure, maintenance, and cost of treatment.

Keywords: Dental implant, ectodermal dysplasia, fixed partial denture, hypodontia, prosthetic rehabilitation


How to cite this article:
Bahrami M, Hussein Alsharbaty MM. Prosthodontic Rehabilitation of Hypohidrotic Ectodermal Dysplasia in Two Young Sisters With Fixed Prostheses: Clinical Case Report. Dent Hypotheses 2019;10:76-9

How to cite this URL:
Bahrami M, Hussein Alsharbaty MM. Prosthodontic Rehabilitation of Hypohidrotic Ectodermal Dysplasia in Two Young Sisters With Fixed Prostheses: Clinical Case Report. Dent Hypotheses [serial online] 2019 [cited 2019 Dec 7];10:76-9. Available from: http://www.dentalhypotheses.com/text.asp?2019/10/3/76/271958




  Introduction Top


Ectodermal dysplasia (ED) is an enormous heterogeneous inherited disorder associated with atypical growth of two or more tissues derived from embryonic ectoderm, which might affect skin, nails, hair, sweat glands, nerve cells, ear and part of eye, as well as teeth during embryonic growth.[1] The current estimate of ED occurrence is about one in 10,000 in 100,000 newborns.[2] Several classifications have been reported among the authors. Some are based on clinical features and others on genetic characteristics of the disorder.[3] Recently, Priolo[4] has composed a new classification of ED, based on the molecular inherited data. The author has divided the ED into two main groups. The first group incorporates disorder in which the defect is in the epithelial–mesenchymal communication, whereas the second group associates with defect in cell–cell adhesion and interaction. Hypohidrotic ectodermal dysplasia (HED) is the most prevalent form of ED, which associated with delay teeth eruption or even only a few teeth may erupt in those affected patients.[5] Due to missing, atypically formed, and malpositioned teeth, a comprehensive intervention of dental procedures, including all aspects of restorative dentistry, is essential for dental rehabilitation of patients affected by ED. A team of specialists in pediatric, orthodontics, prosthodontics, and oral and maxillofacial surgery are frequently involved in determining and carrying out the whole treatment plan.[6],[7],[8] Because the dental manifestations of ED disorder persist during the individuals’ life, clinician must predict working carefully with children, adolescent, and adults.[9],[10],[11] The aim of this present study is to describe full mouth rehabilitation of two young sisters affected with HED, which is associated with maxillary/mandibular oligodontia, and residual alveolar ridge resorption.


  Case Presentation Top


Clinical case 1

An 18-year-old woman was referred to the Prosthodontics Department of Tehran University of Medical Sciences with chief complaint of general spacing between teeth, difficulty in chewing and speech, and mainly unesthetic appearance. The patient’s medical history revealed that she had hypohidrosis and hypotrichosis. The patient was diagnosed with HED disorder. There were no abnormal findings, which impede the dental management. Intraoral examination revealed that all the present maxillary teeth were deciduous, and only permanent canines and right second premolar were present in the mandibular arch [Figure 1]. Clinically, the existing mandibular permanent teeth had cone-shaped appearance with no caries found on the occlusal surfaces. Panoramic radiograph showed that these teeth had small and deciduous-like crowns with enlarged pulp chambers. The patient manifested with loss of Occlusal vertical dimension (OVD) with underdeveloped alveolar ridges. Because the mandibular permanent teeth were malshaped with wide pulp chambers, the elective RCT was mandatory. The decision was to extract all the deciduous teeth and maintain the present permanent three teeth. Six implants of various height and width (Implantium/Dentium system, Seoul, South Korea) were planned in the maxillary arch, whereas three implants (Implantium/Dentium system) were inserted in the mandibular arch simultaneously after deciduous teeth extraction using immediate implants placement technique. During the healing period, the remaining three teeth were endodontically treated, and metal posts and cores were fabricated [Figure 2]. After a 6-month healing period, due to small size of the present teeth, the teeth were prepared with minimal occlusal and axial reduction (0.7 mm and 0.8, respectively) with a shallow chamfer margin. Final impressions were taken with medium viscosity A-silicone (Panasil monophase, Kettenbach, GmbH, Germany) using open-tray technique. After abutments selection, framework patterns were casted with base metal alloy (Palladium-Silver Alloy; Ivoclar Vivadent, Schaan, Liechtenstein). The fitness of the metal frameworks was clinically and radiographically evaluated in the mouth. At delivery appointment, all abutment screws were torqued to 30 Ncm as recommended by the manufacturer. All implant-supported restorations were delivered and cemented using temporary cement (Tempbond; Kerr Corp, Orange, CA, USA), and the tooth-supported fixed partial denture (FPD) was cemented using resin-modified glass inomer cement (Fuji-CEM; GC America, Alsip, IL, USA) [Figure 3]. Oral hygiene was meticulously instructed. The patient has been followed-up for 6 years without complication and the result was satisfactory.
Figure 1 Panoramic view reveals that all maxillary teeth were deciduous.

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Figure 2 Panoramic view after healing period.

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Figure 3 Intraoral view after final restorations delivery.

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Clinical case 2

After 4 years, the first patient’s sister who was previously diagnosed with HED, at the age of 18, was referred to the Prosthodontics Department of Tehran University of Medical Sciences. Comprehensive clinical and radiographic examination reported missing maxillary anterior teeth, difficulty in speech, and mostly unesthetic appearance [Figure 4]. Intraoral examination showed that multiple permanent teeth were absent in the maxillary arch, whereas in the mandibular arch, only the canines, right second premolar, and left first premolar were present. Consultations were obtained from Orthodontics and Oral and Maxillofacial Surgery Departments. Orthodontic treatment was not indicated. Based on the prosthodontist and surgeons’ consultations, it was agreed to extract all nonrestorable deciduous teeth and fabricate the FPD prostheses in maxillary and mandibular arches. In the posterior region of mandibular arch, the clinical decision was to extract mandibular deciduous second molars and insert two implants in position #35, 36, 46, 47, and fabricate Implant-supported fixed prostheses (ISFPs). Two implants (Implantium/Dentium system) were inserted in each mandibular posterior segment. Four months later, the patient returned to continue the definitive treatment plan. The deciduous teeth were extracted, and the present permanent teeth were prepared with chamfer finishing line as abutments for FPDs [Figure 5]. The definitive restorations were fabricated in the same manner as in the aforementioned first case. All the definitive tooth-supported restorations were cemented with resin-modified glass inomer cement (Fuji-CEM; GC America), and implant-supported restorations were cemented using temporary cement (Tempbond; Kerr Corp). Postoperative motivation and instruction for proper prostheses maintenance was clarified. The patient has been followed-up for 2 years without any complication and to date she has been pleased with the result [Figure 6].
Figure 4 Panoramic view shows that multiple permanent teeth were missed.

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Figure 5 Intraoral view shows permanent teeth preparation and implants insertion.

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Figure 6 Smile view of the patient after definitive restorations delivery.

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  Discussion Top


The literature regarding prosthodontic management of ED patients comprises case reports of patients treated at various ages with complete or partial removable prostheses, over dentures with or without tooth preparation, or overdenture with or without attachments, long-span FPD prostheses, and implant-supported prostheses.[12],[13] Elements that are necessary to be focused in designing dental treatment include age, existent teeth, oral hygiene, psychosocial environment, occlusal vertical dimension, bone volume, skeletal growth and development, orthodontics and/or orthognathic surgery, implants, time required for the procedure, maintenance, and cost of treatment. Oral rehabilitation of ED patients necessitates teamwork efforts and treatment of ED is a polemic matter. Osseointegrated dental implants regularly offer further retention and esthetics for young adult patients. Because some patients are still in their ongoing growth, moreover, due to the lack of sufficient bone in the arch, dental implants should be used with more caution.[14] Therefore, we preferred to insert implants at the age of 18 for both the patients. Several clinical reports recommend that implants can be positioned successfully in patients with ED if the bone volume is sufficient for implants placement.[15] The current treatment plan had the following strengths: preserving the teeth which can increase the occlusal awareness for controlling the forces on implants and teeth-supported FPDs; psychological benefit of not extracting some of patient’s teeth; using pink porcelain in interdental papillae for increasing the aesthetic results; and anticipation of elective RCT before preparation of the malshaped teeth; to insert a long-span FPD, the abutment teeth should be as parallel as possible to have adequate retention and resistance simultaneously. Considering the large pulp chamber of these teeth in the young patients, elective RCT before preparation was mandatory. Some of the limitations of the current treatment plan were as follows:
  1. The midline of the teeth and the face were off; the main reason for this problem was preserving the patients’ teeth; if these teeth had been extracted and implants had been inserted in the ideal positions, dental and facial midlines would had been correct. Of course, in many adults, 2 to 3 mm dental and facial off-center midlines cannot be recognized.
  2. Elective RCTs of vital teeth needs patient’s cooperation and understanding.
  3. Long-span teeth-supported FPDs need more hygienic care because of increased risk of caries due to difficulty in using superfloss.
  4. Case reports are not always very reliable.


However, ED patients who can afford implant treatments and can be included in a scientific study are rare. Considering all these pros and cons, this clinical study raises this question whether it is better to extract all the malshaped and malposed teeth and deliver implant-supported FPDs with correct midline and better occlusogingival height, or accept this relatively conservative treatment plan? Answering this question depends on the patient’s expectations. Summary of the key points of these case reports are as following:
  1. In ED patients with hypodontia, it is better to postpone the definitive treatment planning until complete eruption of all permanent teeth. Preserving the deciduous teeth can preserve the bone for implant insertion.
  2. If the patient has high demand for esthetic, it is recommended to show the diagnostic wax-up and to use digital esthetic software to show the final results before preparation of the teeth, especially if the malposed teeth have been preserved.
  3. Occlusal awareness can be maintained with the Periodontal ligament (PDL) of the teeth, so that the long-term longevity of the FPDs will be increased.



  Conclusion Top


Dental defects associated with ED can cause severe esthetic, psychological, and functional problems. A multidisciplinary team approach to oral rehabilitation of adult patients with reduced number of teeth, loss of vertical dimension, and underdeveloped alveolar bone has been presented. The treatment not only improved the patients’ functional and esthetic status, but also significantly increased their self-assurances. In the management of ED, it is also essential to involve oral hygiene instructions, fluoride treatment, and periodic recall visits.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Hickey AJ, Vergo TJ Jr. Prosthetic treatments for patients with ectodermal dysplasia. J Prosth Dentistry 2001;86:364-8.  Back to cited text no. 1
    
2.
Yavuz I, Baskan Z, Ulku R, Dulgergil TC, Dari O, Ece A et al. Ectodermal dysplasia: retrospective study of fifteen cases. Arch Med Res 2006;37:403-9.  Back to cited text no. 2
    
3.
Priolo M, Lagana C. Ectodermal dysplasias: a new clinical-genetic classification. J Med Genet 2001;38:579-85.  Back to cited text no. 3
    
4.
Priolo M. Ectodermal dysplasias: an overview and update of clinical and molecular-functional mechanisms. Am J Med Genet Part A 2009;149a:2003-13.  Back to cited text no. 4
    
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Bergendal B. Orodental manifestations in ectodermal dysplasia: a review. Am J Med Genet Part A 2014;164a: 2465-71.  Back to cited text no. 5
    
6.
Ioannidou-Marathiotou I, Kotsiomiti E, Gioka C. The contribution of orthodontics to the prosthodontic treatment of ectodermal dysplasia: a long-term clinical report. J Am Dental Assoc 2010;141:1340-5.  Back to cited text no. 6
    
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Lypka M, Yarmand D, Burstein J, Tso V, Yamashita DD. Dental implant reconstruction in a patient with ectodermal dysplasia using multiple bone grafting techniques. J Oral Maxillofac Surg 2008;66:1241-4.  Back to cited text no. 7
    
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Yenisey M, Guler A, Unal U. Orthodontic and prosthodontic treatment of ectodermal dysplasia: a case report. Br Dental J 2004;196:677-9.  Back to cited text no. 8
    
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Bergendal B, Bjerklin K, Bergendal T, Koch G. Dental implant therapy for a child with X-linked hypohidrotic ectodermal dysplasia: three decades of managed care. Int J Prosthodont 2015;28:348-56.  Back to cited text no. 9
    
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Vergo TJ Jr. Prosthodontics for pediatric patients with congenital/developmental orofacial anomalies: a long-term follow-up. J Prosth Dentistry 2001;86:342-7.  Back to cited text no. 10
    
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Nowak AJ. Dental treatment for patients with ectodermal dysplasias. Birth Def Orig Art Series 1988;24:243-52.  Back to cited text no. 11
    
12.
Lakomski J, Kobielak K, Kobielak A, Trzeciak WH. Correcting facial dysmorphism in a patient with anhidrotic ectodermal dysplasia: a clinical report. J Prosth Dentistry 1998;80:524-6.  Back to cited text no. 12
    
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Penarrocha-Diago M, Uribe-Origone R, Rambla-Ferrer J, Guarinos-Carbo J. Fixed rehabilitation of a patient with hypohidrotic ectodermal dysplasia using zygomatic implants. Oral Surg Oral Med Oral Pathol Oral Radiol Endodont 2004;98:161-5.  Back to cited text no. 13
    
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Ritto FG, Medeiros PJ, de Oliveira Mussel RL, de-Sa-Silva E. Rehabilitation of an adolescent with ectodermal dysplasia. Two-stage orthognathic, graft, and implant surgery: case report. Implant Dentistry 2009;18:311-5.  Back to cited text no. 14
    
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Guckes AD, Scurria MS, King TS, McCarthy GR, Brahim JS. Prospective clinical trial of dental implants in persons with ectodermal dysplasia. J Prosth Dentistry 2002;88:21-5.  Back to cited text no. 15
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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