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CASE REPORT
Year : 2016  |  Volume : 5  |  Issue : 1  |  Page : 79-82

Multidisciplinary approach in the management of ectodermal dysplasia: A case report


1 Department of Pedodontics and Preventive Dentistry, Kalinga Institute of Dental Sciences, KIIT University, Bhubaneswar, Orissa, India
2 Department of Pedodontics and Preventive Dentistry, MNR Dental College, Sangareddy, Andhra Pradesh, India
3 Department of Oral and Maxillofacial Surgery, Kalinga Institute of Dental Sciences, KIIT University, Bhubaneswar, Orissa, India
4 Department of Prosthodontics, PDM Dental College, Bahadurgarh, Haryana, India
5 Department of Oral Pathology and Microbiology, Kalinga Institute of Dental Sciences, KIIT University, Bhubaneswar, Orissa, India

Date of Web Publication18-Mar-2016

Correspondence Address:
Kanika Singh Dhull
Department of Pedodontics and Preventive Dentistry, Kalinga Institute of Dental Sciences, KIIT University, Bhubaneswar, Orissa
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2277-8632.178988

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  Abstract 

Ectodermal dysplasias are rare hereditary disorders characterized by abnormal development of certain tissues and structures of ectodermal origin. The condition is of importance to dentists as it affects the teeth resulting in hypodontia or anodontia, and a dentist plays an important role in the rehabilitation of the patient. Apart from having difficulties in eating and speaking, young affected individuals can also feel that they look different from their contemporaries resulting in low self-esteem. Well-fitting and functioning prosthesis could be a great help during their schooling years, as it will improve appearance and thus boost their self-confidence. We report a case of hypohidrotic ectodermal dysplasia in a 12-year-old girl who exhibited partial anodontia, and a multidisciplinary approach comprising restorative, orthodontic, surgical, and prosthetic treatment was planned to rehabilitate the girl.The aim of the treatment was to improve psychological development apart from promoting better functioning of the stomatognathic system.

Keywords: Ectodermal dysplasia, orthodontics, partial anodontia, prosthetic rehabilitation, restorative, surgical


How to cite this article:
Dutta B, Dhull KS, Devi TM, Jain N, Yadav S, Sujatha R. Multidisciplinary approach in the management of ectodermal dysplasia: A case report . J NTR Univ Health Sci 2016;5:79-82

How to cite this URL:
Dutta B, Dhull KS, Devi TM, Jain N, Yadav S, Sujatha R. Multidisciplinary approach in the management of ectodermal dysplasia: A case report . J NTR Univ Health Sci [serial online] 2016 [cited 2019 Oct 23];5:79-82. Available from: http://www.jdrntruhs.org/text.asp?2016/5/1/79/178988


  Introduction Top


Ectodermal dysplasia (ED) is a rare hereditary disorder occurring as a consequence of disturbances in the ectoderm of the developing embryo. [1] Individuals affected by ED syndromes have abnormalities of the glands, tooth buds, hair follicles, and nail development. ED syndrome was first described in the medical literature by Thurnam, who reported two typical patients in 1848, and later in 19th century by Darwin. [2] In 1921, Thadani assigned ED to the X chromosome. [3] ED represents a large and complex group of diseases comprising more than 170 different clinical conditions. [4] ED syndrome affects both males and females of all races and ethnic groups. Prevalence is estimated to be 7 cases in 10,000 births. The mortality rate is 30% in infancy or early childhood because of intermittent hyperpyrexia. [5],[6]

This condition is of two major types depending on the number and functionality of the sweat glands:

  1. X-linked anhidrotic or hypohidrotic, where sweat glands are either absent or significantly reduced in number which is known as Christ-Siemens-Touraine syndrome and
  2. Hidrotic, where sweat glands are normal and the condition is inherited as autosomal dominant which is known as Clouston's syndrome. [1],[4]
The most frequently reported ED syndrome is Christ-Siemens-Touraine syndrome. [7],[8] Though the hereditary patterns and nail and sweat gland manifestations tend to differ, the dentition and hair are affected similarly in both types.

The striking orofacial characteristics of this syndrome include anodontia or hypodontia, hypoplastic conical teeth, underdevelopment of the alveolar ridges, frontal bossing, a depressed nasal bridge, protuberant lips, and hypotrichosis. [5] It can affect both the primary and permanent dentition. The objectives of oral rehabilitation in a young ED patient with partial or complete anodontia are preservation of bone, early development of correct patterns of chewing, swallowing, speaking, and restoring normal facial characteristics. Thus, it facilitates development of normal emotional and psychological profile.

The purpose of this paper is to present a patient with ED who required restorative, orthodontic, surgical, and prosthetic treatment to achieve a satisfactory result. The focus is on the interdisciplinary management of a patient with complex restorative needs.


  Case report Top


A 12-year-old female patient came to the Department of Pedodontics and Preventive Dentistry with the complaint of missing teeth causing difficulty in mastication and speech. She was accompanied by her mother who mentioned that child was intolerant to heat and preferred to be in cold environment. Patient also reported about absence of sweating.

Family history revealed that she was the eldest of the two siblings of parents with consanguineous marriage and none of the other members in the family had a similar condition.

The child was moderately built with no signs of mental retardation. Her skin was dry with scanty body hair. The nails were not affected. Extraoral examination revealed the typical features of ED such as sparse scalp hair, frontal bossing, thin eyebrows, saddle nose, and protuberant lips. A diminished lower facial height contributed to a senile facial expression [Figure 1]a and b.
Figure 1: Pre-treatment extra-oral profile: (a) frontal view; (b) lateral view

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Intraoral examination revealed thin, atrophic, partially edentulous maxillary and mandibular alveolar ridges with multiple missing permanent teeth and over-retained deciduous second molars in all four quadrants [Figure 2]a-c. Anodontia as well as underdevelopment of alveolar ridges was confirmed by panoramic radiography [Figure 3].
Figure 2: Pre-treatment intra-oral view: (a) maxillary arch; (b) mandibular arch; (c) maxillary and mandibular casts

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Figure 3: Orthopantomograph

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Correlating the clinical and radiographic findings, the child was diagnosed with hypohidrotic ED with partial anodontia.

A multidisciplinary treatment plan was formulated to rehabilitate the child and the same was discussed with her parents. Informed consent was obtained for the agreed treatment. The treatment we planned was completed in three phases:

  • Phase I: Orthodontic phase. Orthodontic correction included approximation followed by retraction and intrusion of maxillary central incisors [Figure 4]a and b.
  • Phase II: Surgical phase. Maxillary labial frenectomy and mandibular vestibuloplasty was performed using Diode laser (Zolar) [Figure 5]a and b.
  • Phase III: Restorative and prosthetic rehabilitation. Anterior facial height was increased by adding a layer of 1 mm of composite on the occlusal surface of permanent mandibular first molars. [9] This will, in turn, facilitate the supraeruption of the disoccluded permanent second molars in due course of time. The polycarbonate crowns were cemented in maxillary central incisors to enhance the esthetics, which was followed by insertion of acrylic partial dentures in maxillary and mandibular arches [Figure 6]a and b.
The facial profile and expression improved significantly following complete oral rehabilitation [Figure 7]a and b. Recall appointments were scheduled after 1 and 4 weeks. After 4 weeks, the patient was able to function well with both maxillary and mandibular partial dentures. In order to accommodate growth and development, the patient was scheduled for ongoing follow-up visits every 6 months. At recall appointments, good retention was observed and the parents reported a significant improvement in terms of speech and mastication.
Figure 4: Orthodontic intervention: (a) labial view; (b) palatal view

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Figure 5: Surgical intervention: (a) maxillary labial frenectomy; (b) mandibular vestibuloplasty

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Figure 6: Prosthetic rehabilitation: (a) maxillary partial denture; (b) labial view

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Figure 7: Post-treatment extra-oral profile: (a) frontal view; (b) lateral view

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  Discussion Top


Oral rehabilitation of the ED patient results in improvement in esthetics, speech, masticatory efficiency, and both sagittal and vertical skeletal relationship. [10] Therefore, it is important to diagnose the associated problems in dento-alveolar complex, so that an accurate treatment plan can be established to rehabilitate the patient at optimum level. Treatment should be administered by a multidisciplinary team involving pediatric dentist, orthodontist, oral and maxillofacial surgeon, and prosthodontist. Factors that govern the treatment plan are age of the patient, inter-arch spaces and relationships, number of teeth present and their position and shape, volume of alveolar bone, and mucosal attachment. [6],[7],[11]

The most conventional and widely accepted dental treatment in cases of ED with complete or partial anodontia is removable dentures. Dental implants with fixed prosthesis are also being considered as an alternative treatment options in patients with completed jaw growth. However, the main difficulty with implant therapy is the presence of insufficient alveolar bone; implant placement may not be possible without bone grafting. [12] Furthermore, implantation and reconstruction surgery is subject to a greater risk of failure. [13],[14] The other important drawback of implant is the cost factor which limits its affordability by the low socio-economic group. In the present case, implant-supported denture was ruled out due to insufficient alveolar bone support and low socio-economic status of the patient.

Although there is a controversy regarding definitive time to begin treatment, Till and Marques [15] recommend that an initial prosthesis could be delivered when the child starts school, so that the child could have a better appearance and have time to adapt to the prosthesis. Usually, by the time the child reaches school age, he or she is old enough to recognize the esthetic handicap and is willing to cooperate.

In the present case, the patient was close to her growth completion and was skeletally mature and, therefore, ready to accept a definitive and long-term treatment plan. In order to achieve optimal level of esthetics, support, and function, it was important to complete orthodontic, restorative, and periodontal part of the treatment prior to prosthetic rehabilitation.

Orthodontic treatment was initiated using twin bracket appliance with elastic chain in order to close the midline diastema. A removable plate with a helical spring and a hook was inserted to intrude and retrocline the maxillary central incisors.

Maxillary labial frenectomy was performed to prevent orthodontic relapse. Mandibular vestibular deepening was undertaken to get a better retention and stability of the denture. Congenital absence of multiple teeth resulted in reduced facial height, with insufficient inter-arch space for the artificial teeth to set on dentures. Therefore, an attempt was made to increase the inter-arch space by increasing the crown height of mandibular molars, which eventually improved the facial esthetics. Removable dentures were fabricated, maintaining the new facial height.


  Conclusion Top


In the present case, the initial molding was a key stage in determining the patient's cooperation, as she wanted to have a better appearance. A multidisciplinary approach comprising restorative, orthodontic, surgical, and prosthetic treatment was done to achieve a satisfactory result. The treatment led to significant improvements in appearance, speech, and masticatory function. Young ED patients should be recalled periodically for the prosthetic modification that is required due to continuing growth and development. The transitional prosthesis should be replaced by more definitive prosthesis once the skeletal growth is completed.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

 
  References Top

1.
Nunn JH, Carter NE, Gillgrass TJ, Hobson RS, Jepson NJ, Meechan JG, et al. The interdisciplinary management of hypodontia: background and role of paediatric dentistry. Br Dent J 2003;194:245-51.  Back to cited text no. 1
    
2.
Nyhan WL. Diagnostic recognition of genetic disease. 1 st ed. Philadelphia: Lea and Febiger; 1987. p. 680-5.  Back to cited text no. 2
    
3.
Gupta S, Tyagi P. Prosthodontic management of anhidrotic ectodermal dysplasia. Indian J Dent Res 2011;22:348-51.  Back to cited text no. 3
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4.
Vieira KA, Teixeira MS, Guirado CG, Gaviao MB. Prosthodontic treatment of hypohidrotic ectodermal dysplasia with complete anodontia: Case report. Quintessence Int 2007;38:75-80.  Back to cited text no. 4
    
5.
McLaughlin WS. Congenital absence of all primary and permanent lateral incisors in a carrier of X linked hypohidrotic ectodermaldysplasia. Int J Paed Dent 1991;2:99-103.  Back to cited text no. 5
    
6.
Paschos E, Huth KC, Hickel R. Clinical management of hypohidrotic ectodermal dysplasia with anodontia: Case report. J Clin Pediatr Dent 2002;27:5-8.  Back to cited text no. 6
    
7.
Jenson OT, Leopardi A, Gallegos L. The case for bone graft reconstruction including sinus grafting and distraction osteogenesis for the atrophic edentulous maxilla. J Oral Maxillofac Surg 2004;62:1422-8.  Back to cited text no. 7
    
8.
Ziada H, Holland T. Ectodermal dysplasia: A case report. J Irish Dent Assoc 1997;43:127-30.  Back to cited text no. 8
    
9.
Poyser NJ, Porter RW, Briggs PF, Chana HS, Kelleher MD. The Dahl Concept: Past, present and future. Br Dent J 2005;198:669-76.  Back to cited text no. 9
    
10.
Pigno MA, Blackman RB, Cronin RJ, Cavazos E. Prosthodontic management of ectodermal dysplasia: A review of the literature. J Prosthet Dent 1996;76:541-5.  Back to cited text no. 10
    
11.
Tarjan I, Gabris K, Rozsa N. Early prosthetic treatment of patients with ectodermal dysplasia: A clinical report. J Prosthet Dent 2005;93:419-24.  Back to cited text no. 11
    
12.
Imirzalioglu P, Uckan S, Haydar SG. Surgical and prosthodontic treatment alternatives for children and adolescents with ectodermal dysplasia: A clinical report. J Prosthet Dent 2002;88:569-72.  Back to cited text no. 12
    
13.
Lo Muzio L, Bucci P, Carile F, Riccitiello F, Scotti C, Coccia E, et al. Prosthetic rehabilitation of a child affected from anhydrotic ectodermal dysplasia: A case report. J Contemp Dent Pract 2005;6:120-6.  Back to cited text no. 13
    
14.
Rad AS, Siadat H, Monzavi A, Mangoli AA. Full mouth rehabilitation of a hypohidrotic ectodermal dysplasia patient with dental implants: A clinical report. J Prosthodont 2007;16:209-13.  Back to cited text no. 14
    
15.
Till MJ, Marques AP. Ectodermal dysplasia: Treatment considerations and case reports. Northwest Dent 1992;71:25-8.  Back to cited text no. 15
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]



 

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