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 Table of Contents  
CASE REPORT
Year : 2012  |  Volume : 3  |  Issue : 4  |  Page : 271-274

Recreating the missing smile: A case report on ectodermal dysplasia


Department of Prosthodontics and Implantology, Mahatma Gandhi Postgraduate Institute of Dental Sciences, Gorimedu, Puducherry, India

Date of Web Publication12-Jul-2013

Correspondence Address:
R Shakila
Department of Prosthodontics and Implantology, Mahatma Gandhi Postgraduate Institute of Dental Sciences, Gorimedu, Puducherry - 605006
India
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DOI: 10.4103/0976-433X.114977

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  Abstract 

Ectodermal dysplasia syndrome is a group of hereditary disorders affecting the structures developing from the ectoderm. More than 150 different types have been described with the most common being the hypohidrotic and the hidrotic types. Complete or partial anodontia, trichodysplasia, onychodysplasia, and dyshidrosis are the major symptoms of this condition. Prosthetic treatment often involves replacement of the congenitally missing teeth. This article describes a case report on prosthetic management of an ectodermal dysplasia patient.

Keywords: Anhidrosis, anodontia, ectodermal dysplasia, hypohidrosis, prosthetic management


How to cite this article:
Shakila R, Jeevanandam L, Jagdish S K, Arun Kumar V R. Recreating the missing smile: A case report on ectodermal dysplasia. SRM J Res Dent Sci 2012;3:271-4

How to cite this URL:
Shakila R, Jeevanandam L, Jagdish S K, Arun Kumar V R. Recreating the missing smile: A case report on ectodermal dysplasia. SRM J Res Dent Sci [serial online] 2012 [cited 2020 May 28];3:271-4. Available from: http://www.srmjrds.in/text.asp?2012/3/4/271/114977


  Introduction Top


Embryonic development takes place from three primary layers in a zygote, the endoderm, the mesoderm, and the ectoderm. The term "Ecto" means "Outer" thus referring to the layer that gives rise to most of the surface structures of the body. These include the skin, nails, hairs, sweat glands, the salivary glands, and the teeth. Any disruption of the development of the ectoderm in turn results in impairment of the structures arising from it. Ectodermal dysplasia syndrome is a group of hereditary disorders affecting the structures developing from the ectoderm. More than 150 different types of this syndrome have been described in literature. [1] The first description of this disease was by John Thurnam of New York in the year 1848. Later Wedderburn reported this condition in an Indian Hindu family from Sindh. These findings were recorded by Charles Darwin in his book ''The variations of animals and plants under domestication'' in the year 1875. Wedderburn described the members of the affected family as "…ten men, in the course of four generation, were furnished, in both jaws taken together, with only four small and weak incisor teeth and with eight posterior molars. The men thus affected have very little hair on body and become bald early in life" . [2] This statement gives a clear picture of an ectodermal dysplasia patient.

The affected individuals present with sparse hair in scalp and body with deficient eyebrows. The skin of these patients is usually rough and thin. Hyperkeratosis in elbows and knees is also seen. Skin is hypopigmented with hyperpigmentation present around the eyes alone. Sweat glands are affected and thus the patient rarely sweats. In some conditions, the salivary glands are malformed resulting in decreased salivation or xerostomia. A common oral finding is anodontia with most of the teeth congenitally missing. [1],[3]

Freire-Maia and Pinheiro [4] classified 11 possible groups of ectodermal dysplasia; the condition occurred as X-linked, autosomal dominant, or recessive forms. It may also arise due to mutations and affect children with normal parents, who are not even carriers of the gene. The most common types are the hypohidrotic (anhidrotic) type and the hidrotic type. The hypohidrotic type is also termed as Christ-Siemens-Touraine syndrome. The X-linked variety mostly affects only men with women being carries of the gene. The prevalence of the anhidroitic type is 1 to 7 in 1,00,000 live births. [5] The X-linked recessive is the most common type of hypohidroitic ectodermal dysplasia. Bashyam et al., [6] have recently reported high frequency in mutation of the ectodysplasin (EDA) gene among Indians affected with anhidrotic ectodermal dysplasia. These workers reported that the disease is caused by inactivation of one of the three genes EDA-A1, which encodes a ligand belonging to tumor necrosis factor family; EDA receptor (EDAR) and EDAR-associated death domain (EDARADD), an encoding adaptor protein. X-linked recessive is caused by mutation of EDA-A1, autosomal recessive by EDAR, and autosomal dominant by EDARADD mutations. [6]


  Case Report Top


An 18-year-old male patient, Mr. Paneer, came to the Department of Prosthodontics and Implantology with the chief complaint of multiple missing teeth since birth. The patient gave a history that only one tooth had erupted since his birth in his lower jaw. The patient has sparse eyebrows and brownish soft hair. His skin was rough and dry with very less of hair on the body and the face. The premaxilla was hypoplastic giving the appearance of an edentulous old man [Figure 1]. Intraoral examination revealed presence of only one second molar in the left lower quadrant [Figure 2]. The radiographs revealed congenitally missing permanent tooth buds. The alveolar process of the maxilla and mandible was hypoplastic. The salivary flow was decreased. A diagnosis of hypohidrotic ectodermal dysplasia was made with these findings. Prosthodontic significance of hypohidrotic ectodermal dysplasia is the difficulty in achieving acceptable function and esthetics with limited tooth and tissue support available for the prosthesis and with decreased salivary secretion.
Figure 1: Preprosthetic extraoral view

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Figure 2: Preprosthetic intraoral view

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In managing this patient, both esthetic and functional requirements had to be met. The patient could not afford for implant-supported prosthesis. The next alternative in managing this patient was to fabricate a removable complete denture for this patient. DeVan's dictum states ''preservation of what remains is more important than meticulous replacement of what is missing''. With this philosophy in mind, it was decided not to extract the single second molar in the mandible. Presence of this tooth was the only key to preserve the alveolar bone in that area.

Treatment phase

During the first appointment, maxillary and mandibular primary impressions were made, using irreversible hydrocolloid for mandible and impression compound for the maxilla [Figure 3]. The primary casts were obtained and custom trays were fabricated on them [Figure 4]. The lower custom tray was kept short of the second molar and covered only the edentulous ridge. In the second visit, border moulding was completed in maxilla and mandible. Final impressions were made with zinc oxide eugenol impression paste. For the mandible, the final impression was placed back in the mouth and a pick-up impression was made using irreversible hydrocolloid. The master casts were obtained. Temporary shellac record bases and occlusal rims were made on the master cast. Jaw relation was completed in the third visit and the maxillary and mandibular casts were articulated. Teeth arrangement was completed. Nonanatomic teeth were used to eliminate unnecessary contacts during excursive movements. Denture try-in was done, and the processed and finished dentures were inserted. The final esthetic and functional outcomes were satisfactory [Figure 5] and [Figure 6]. The main advantage for the functional satisfaction in the final prosthesis was mainly due to the prosthodontic approach of following the DeVan's dictum and preserving the single solitary molar in the left mandibular posterior region, which improved the proprioceptive mechanism and compensated for the patient's condition of decreased salivary flow. This case actually elevates the mind in treating any patients with developmental disturbances to be approached with the open goal of preservation of all that what is existing.
Figure 3: Primary impressions

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Figure 4: Primary casts

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Figure 5: Postprosthetic intraoral view

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Figure 6: Postprosthetic extraoral view

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  Discussion Top


Prosthetic treatment of patients with ectodermal dysplasia is crucial in preventing both functional and psychological collapse. [7] Patients with congenital absence of primary teeth can be easily identified by the parents at the age of 2 to 3 years. Immediate referral to a dentist will help in determining the presence or absence of the tooth buds and to rehabilitate the patient. Rehabilitation at an early stage is very useful as the patient can easily accommodate to the prosthesis. [8] This prevents the patient from becoming a functional cripple. Patient is easily able to learn to adapt to the prosthesis along with nearly normal development of function. Initial treatment is always to provide a removable prosthesis-either a complete or partial denture. This requires cooperation from the patient his/her parents. A removable prosthesis can be relined or adjusted as the patient grows older. Definitive treatment plan is to provide an implant-supported prosthesis whenever possible. This is done when the growth is complete. Usually, ectodermal dysplasia patients exhibit deficient bone for placement of implants. In these bases, ridge augmentation procedures are necessary. Rehabilitation with implant-supported prosthesis for ectodermal dysplasia has been discussed in literature. [9],[10] In treating these patients, it should be remembered that psychological effect of treatment is as important as the functional and esthetic outcomes.


  Conclusion Top


In this paper, a case report of an ectodermal dysplasia patient has been described. The patient was treated with a removable complete denture as implant-supported prosthesis was not feasible due to economic factors. The esthetic and functional outcomes of the treatment were satisfactory.


  Acknowledgment Top


The author would like to thank the patient for providing consent to use his photograph in this article.

 
  References Top

1.Rajendran R. Diseases of the skin. In: Rajendran R, Sivapathasundaram B, editors. Shafer's textbook of oral pathology. 6 th ed. New Delhi: Elsevier Publications; 2009. p. 797-8.  Back to cited text no. 1
    
2.Singh A, Jolly SS, Kaur S. Hereditary ectodermal dysplasia. Br J Dermatol 1962;74:34-7.  Back to cited text no. 2
    
3.Clarke A. Hypohidrotic ectodermal dysplasia. J Med Genet 1987;24:659-63.  Back to cited text no. 3
    
4.Freire-Maia N, Pinheiro M. Ectodermal dysplasias-Some recollections and a classification. Birth Defects Orig Artic Ser 1988;24:3-14.  Back to cited text no. 4
    
5.NaBadalung DP. Prosthodontic rehabilitation of an anhidrotic ectodermal dysplasia patient: A clinical report. J Prosthet Dent 1999;81:499-502.  Back to cited text no. 5
    
6.Bashyam MD, Chaudhary AK, Reddy EC, Reddy V, Acharya V, Nagarajaram HA, et al. A founder ectodysplasin A receptor (EDAR) mutation results in a high frequency of the autosomal recessive form of hypohidrotic ectodermal dysplasia in India. Br J Dermatol 2012;166:819-29.  Back to cited text no. 6
    
7.Hickey A, Vergo TJ Jr. Prosthodontic consideration in the treatment of patients with maxillary and mandibular deficiencies. J Prosthet Dent 1991;66:645-9.  Back to cited text no. 7
    
8.Hickey AJ, Vergo TJ Jr. Prosthetic treatments for patients with ectodermal dysplasia. J Prosthet Dent 2001;86:364-8.  Back to cited text no. 8
    
9.Smith RA, Vargervik K, Kearns G, Bosch C, Koumjian J. Placement of an endosseous implant in a growing child with ectodermal dysplasia. Oral Surg Oral Med Oral Pathol 1993;75:669-73.  Back to cited text no. 9
    
10.Pigno MA, Blackman RB, Cronin RJ Jr, 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
    


    Figures

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



 

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Abstract
Introduction
Case Report
Discussion
Conclusion
Acknowledgment
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