|Year : 2016 | Volume
| Issue : 1 | Page : 53-55
Prosthetic rehabilitation of a child with ectodermal dysplasia: A case report with review of literature
Sunil Kumar Mishra1, Nagalakshmi Chowdhary2, Harsh Mahajan1, Shail Kumari Mishra3
1 Department of Maxillofacial Prosthodontics and Implantology, People's Dental Academy, Bhopal, Madhya Pradesh, India
2 Department of Pedodontics, Sri Siddhartha Dental College, Tumkur, Karnataka, India
3 Department of Orthodontics, Rishiraj College of Dental Sciences and Research Centre, Bhopal, Madhya Pradesh, India
|Date of Submission||06-Jan-2016|
|Date of Acceptance||05-Apr-2016|
|Date of Web Publication||16-Jun-2016|
Sunil Kumar Mishra
Department of Maxillofacial Prosthodontics and Implantology, People's Dental Academy, Bhanpur, Bhopal - 462 037, Madhya Pradesh
Source of Support: None, Conflict of Interest: None
The aim of this case report is to describe the oral rehabilitation of a young patient of hypohidrotic ectodermal dysplasia (ED) with maxillary and mandibular complete dentures. Such patients often present with poor aesthetics, mastication, and social behavior. Treatment resulted in an improvement of esthetics, function and psychology of the patient. Children with EDs do not have normal patterns of growth and a risk and benefit analysis must be made to access the value of implant placement. Replacement of teeth by implants is usually restricted to patients with completed craniofacial growth.
Keywords: Anodontia, children, ectodermal dysplasia, rehabilitation
|How to cite this article:|
Mishra SK, Chowdhary N, Mahajan H, Mishra SK. Prosthetic rehabilitation of a child with ectodermal dysplasia: A case report with review of literature. J Curr Res Sci Med 2016;2:53-5
|How to cite this URL:|
Mishra SK, Chowdhary N, Mahajan H, Mishra SK. Prosthetic rehabilitation of a child with ectodermal dysplasia: A case report with review of literature. J Curr Res Sci Med [serial online] 2016 [cited 2022 Dec 7];2:53-5. Available from: https://www.jcrsmed.org/text.asp?2016/2/1/53/184138
| Introduction|| |
Severe hypodontia or even anodontia in children are very rare conditions, most often associated with congenital syndromes such as Down syndrome (trisomy 21)  or ectodermal dysplasia (ED).  There exist more than 170 clinically distinct hereditary syndromes in which ED is present.  They are caused by an impaired development of epidermal appendages and are characterized by a primary defect at least in one of the following tissues: nails, hair, teeth, or sweat glands.  EDs are rare diseases with an estimated incidence of 7 in 100,000 births.  ED is usually described as being hypohidrotic or hidrotic depending on the degree of sweat gland function.
Hypohidrotic ED (HED) frequently exhibits the most severe dental anomalies. HED is characterized by hypohidrosis, hypotrichosis, and hypodontia. 
Facial symptoms include maxillary hypoplasia, saddle nose, prominent lips, and linear wrinkles around the eyes. The hair is fine, dry, brittle and sparse, and the skin is thin and dry with hypohidrosis. Absent or decreased sweating in patients with anhidrotic, or hypohidrotic ED, respectively, is caused by the absence of sweat glands. 
Oral manifestations include multiple tooth abnormality such as anodontia, hypodontia and tapered, malformed, and widely spaced teeth. Abnormal alveolar ridge development also may be present. , These features give child a distinctly aged appearance. The psychological and functional effect of ED can have a tremendous negative effect on a young individual. The prosthodontist, orthodontist, pedodontist, or the maxillofacial surgeons are probably the first medical professionals to be confronted with complaints of EDs. Most important for the multi-disciplinary team is to take responsibility for providing the growing individual with age appropriate appliances, which make the child satisfied from both esthetic and functional viewpoint. It was planned to give conventional prosthesis to allow as much growth as possible before initiating the implant assisted phase of treatment.
| Case report|| |
A 10-year-old girl child with hypohidrotic ED and anodontia was referred to the Department of Prosthodontics. On examination, the patient presented with the classical triad of hypohidrosis, hypotrichosis and anodontia along with mandibular overclosure [Figure 1]. Decreased height of the lower third of the face was observed. The orthopantomogram X-ray revealed the absence of tooth in maxillary and mandibular arch and also there is no underlying tooth buds. Treatment considered for this patient is fabrication of complete dentures. Maxillary and mandibular dentures were fabricated in conventional manner. Preliminary impressions were made with impression compound (Pyrex, Roorkee, India), special tray fabricated over primary cast, border molding (DPI, Mumbai, Maharashtra, India) and final impression made with light body elastomeric impression material (Reprosil, Dentsply, Milford, USA). Impressions were poured with dental stone (Kalabhai, Mumbai, Maharashtra, India) and denture base and occlusal rims were fabricated. Tentative jaw relation was recorded and face bow transfer was done on a semi-adjustable articulator (hanau-H2) and teeth were arranged in balanced occlusion. Maxillary and mandibular trial dentures were verified in vertical dimension of occlusion. After approval of the teeth arrangement by the patient and her parents, the waxed dentures were processed in a heat-polymerized denture base resin (Lucitone, Dentsply, York, PA, USA). The complete denture was inserted [Figure 2] and the patient and her parents were instructed for the proper maintenance of oral hygiene. The patient was put on a 72 h follow-ups scheduled for any adjustments. The patient was then schedule for 1 week, 1 month and 3 months follow-up.
| Discussion|| |
Complete denture prosthesis can provide acceptable results for aesthetics, psychological support, and function. However, underdevelopment of the alveolar ridges and xerostomia in the patient makes denture retention and stability difficult to achieve. Endosseous implants can also be considered as an alternative treatment. The benefits of implant use in growing patients are as important as the concerns for their premature use. Shaw  reported that the dramatic growth changes occurring in infancy and early childhood were not conducive to the maintenance of implants. Prosthesis remodeling, as stated by Smith et al.,  Brugnolo et al.,  Guckes et al.  and Kearns et al.  is an undesirable condition, because of the repetitive need to lengthen the transmucosal implant-to-prosthesis ratios and the potential load magnification. The timing of implant placement in growing patients was discussed at a Scandinavian Consensus Conference in Sonkoping, Sweden  where there was a general agreement that implant placement should be postponed until skeletal growth is completed or nearly completed in normal adolescents. In the individual with oligodontia or anodontia, however earlier intervention could be indicated, especially in the mandible. Anodontia and severe oligodontia were mentioned as exceptions to the rule. The consideration of osseointegrated implants were postponed because of age and potential growth of this young patient.
| Conclusion|| |
This clinical report describes prosthodontic rehabilitation of a female patient with hypohydrotic ED. Treatment resulted in an improvement of aesthetics and function that caused a favorable change in the confidence and psychology of the patient. Still, some children are treated today with implants, and there are few in whom the therapy may result in a better quality of life. However, the treatment can only be justified when the anticipated positive effects are greater than the drawbacks of the procedure.
Declaration of patient consent
The authors certify that they have obtained consent from the patient(s) on appropriate 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 patient(s) 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.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Mestrovic SR, Rajic Z, Papic JS. Hypodontia in patients with Down′s syndrome. Coll Antropol 1998;22 Suppl:69-72.
Silverman NE, Ackerman JL. Oligodontia: A study on its prevalence and variations in 4032 children. J Dent Child 1979;46:470-7.
Lamartine J. Towards a new classification of ectodermal dysplasias. Clin Exp Dermatol 2003;28:351-5.
Priolo M, Silengo M, Lerone M, Ravazzolo R. Ectodermal dysplasias: Not only ′skin′ deep. Clin Genet 2000;58:415-30.
Itin PH, Fistarol SK. Ectodermal dysplasia. Am J Med Genet 2004;131:145-51.
Clarke A. Hypohidrotic ectodermal dysplasia. J Med Genet 1987;24:659-63.
Pinheiro M, Freire-Maia N. Ectodermal dysplasias: A clinical classification and a causal review. Am J Med Genet 1994;53:153-62.
Freire-Maia N. Ectodermal dysplasias. Hum Hered 1971;21:309-12.
Lowry RB, Robinson GC, Miller JR. Hereditary ectodermal dysplasia. Symptoms, inheritance patterns, differential diagnosis, management. Clin Pediatr (Phila) 1966;5:395-402.
Shaw WC. Problems of accuracy and reliability in cephalometric studies with implants in infants with cleft lip and palate. Br J Orthod 1977;4:93-100.
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.
Brugnolo E, Mazzocco C, Cordioll G, Majzoub Z. Clinical and radiographic findings following placement of single-tooth implants in young patients - Case reports. Int J Periodontics Restorative Dent 1996;16:421-33.
Guckes AD, McCarthy GR, Brahim J. Use of endosseous implants in a 3-year-old child with ectodermal dysplasia: Case report and 5-year follow-up. Pediatr Dent 1997;19:282-5.
Kearns G, Sharma A, Perrott D, Schmidt B, Kaban L, Vargervik K. Placement of endosseous implants in children and adolescents with hereditary ectodermal dysplasia. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1999;88:5-10.
Koch G, Bergendal T, Kvint S, Johansson UB. Consensus conference on oral implants in young patients. Stockholm: Forlagshuset Gothia; 1996.
[Figure 1], [Figure 2]