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Journal of Indian Society of Pedodontics and Preventive Dentistry Official publication of Indian Society of Pedodontics and Preventive Dentistry
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Year : 2005  |  Volume : 23  |  Issue : 1  |  Page : 31-34

Hypohidrotic ectodermal dysplasia: A unique approach to esthetic and prosthetic management: A case report

Dept. of Pedodontics and Preventive Dentistry, K.L.E.S's Institute of Dental Sciences, Belgaum - 590 010, India

Correspondence Address:
A Shigli
Dept. of Pedodontics and Preventive Dentistry, K.L.E.S's Institute of Dental Sciences, Belgaum - 590 010
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0970-4388.16024

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Hypohidrotic ectodermal dysplasia is a rare congenital disease that affects several ectodermal structures. The condition is usually transmitted as an x-linked recessive trait, in which gene is carried by the females and manifested in males. Manifestations of the disease differ in severity and involve teeth, skin, hair, nails and sweat and sebaceous gland. Ectodermal dysplasia is usually a difficult condition to manage. Prosthodontically, because of the typical oral deficiencies, and afflicted individuals are quite young to receive extensive prosthodontic treatment, which restores their appearance and helps them, for the development of positive self-image. This case report describes the management of upper jaw with over denture with copings on existing teeth i.e. two permanent peg shaped centrals as well as lateral incisors. However with adequate of retainer lower denture was provided with a new treatment modality.

Keywords: ED-Ectodermal dysplasia, Unique approach

How to cite this article:
Shigli A, Reddy RV, Hugar S M, Deshpande D. Hypohidrotic ectodermal dysplasia: A unique approach to esthetic and prosthetic management: A case report. J Indian Soc Pedod Prev Dent 2005;23:31-4

How to cite this URL:
Shigli A, Reddy RV, Hugar S M, Deshpande D. Hypohidrotic ectodermal dysplasia: A unique approach to esthetic and prosthetic management: A case report. J Indian Soc Pedod Prev Dent [serial online] 2005 [cited 2023 Feb 6];23:31-4. Available from: http://www.jisppd.com/text.asp?2005/23/1/31/16024

Ectodermal dysplasia is the term used to describe a group of rare, inherited disorder characterized by dysplasia of tissues of ectodermal origin-primarily nail, teeth and skin and occasionally, dysplasia of mesodermally derived tissues.[1] The condition is thought to occur in approximately 1 of 1,00,000 live births.[1],[2] Freire Maia and Pinherio[6] described 117 varieties of ED with multiple combination of abnormal ectodermally derived structures. Clinically ED may be divided into two broad categories the x-linked Hypohidrotic form, characterized by the classical triad of hypodontia, hypohidrosis and hypotrichosis and characterized by dysrmorphic facial features is also termed as Christ-Siemens Tourine syndrome.[3],[4]

The hypohidrotic form of ED usually spares the sweat glands, can affect the teeth, hair, nail and is inherited as an autosomal trait. This was described by Cloustan in 1929 and Lowrey et al in 1966, as an autosornal dominant, which is found in Canadian families of French descent.[4] ED is usually a difficult condition to manage prosthodontically, because of the typical oral deficiencies and afflicted individuals are quite young to receive extensive prosthodontic treatment, which restores their appearance, for the development of positive self-image.[5],[6]

This case report essentially emphasizes on a different approach of prosthetic management of appearance, functionality of treatment in the form of denture provided.

   Case Report Top

A nine year old boy reported with the complaint of lack of teeth and inability to eat properly. He exhibited the classical features of ED: hypodontia, hypohidrosis, hypotrichosis, prominent forehead, saddle nose and everted lips. Intra oral examination [Figure - 1] revealed dry and sticky oral mucosa with localized mild gingivitis, conical shaped permanent maxillary centrals, laterals, one mesiodens and mandibular canines were the only teeth present. He exhibited aplasia of alveolar bone in the edentulous area. OPG [Figure - 2] revealed absence of other teeth buds. Maxillary teeth showed open apices where as in mandibular teeth had their root formation completed. A conventional over denture was the treatment of choice for this patient, because the objective were to preserve the remaining dentition to restore function and esthetics and to allow certain modification to be made to meet the needs of the developing stomatognathic system. Mesiodens was extracted under LA and apexification was done for all the permanent maxillary teeth with regular follow up. Intentional root canal treatment was done for the mandibular teeth.

After l ½ year when the open apex had closed completely the conventional R.C.T carried out and canals were obturated with gutta percha, then maxillary teeth were reduced to use as bare root abutment with slightly parallel proximal walls for retention of over denture.

Similarly mandibular teeth were reduced close to the gingiva to use as short bare root abutments. Root preparation was done by removing gutta percha from the cervical 1/3 of the canal. Wax impression for the post were made for the maxillary teeth and rubber base impression was taken. Wax pattern were tried on the cast. All coping patterns were invested, casted and polished as routine laboratories procedure with Japanese gold alloy, tried on the master cast and copings were cemented on the bare root abutment of maxillary teeth [Figure - 3]. In the mandibular arch an over denture with intra coronal attachment like zest attachment [Figure - 4] was planned in which the female component was processed in the denture base providing the retention when the over denture is inserted, the female retentive lamella engages the stud ball to produce the retention.

Root preparation seating of male attachment was done, then the female part was snapped in position over the male attachment [Figure - 5], and with the special tray final impression was made with female attachment in it. Male analogs were stabilized before poring the master cast. Wax rims were prepared for the rnaxillary and mandibular denture base, proper vertical height was established. A trial denture base was made for auto polymerizing acrylic resin, and the selected denture teeth were adjusted in size and set in wax. After trying the denture was processed in heat polymerizing acrylic resin in the usual manner then the over dentures were delivered [Figure - 6]. Recall appointment were scheduled for 24 hours, 72 hours, 2 weeks, 4 weeks, every 3 months for the first year and every 4 months for the second year. Written oral hygiene instructions were given and explained to the patient.

   Discussion Top

Nowak stated that "treating the pediatric patient with ED requires the clinician to be knowledgeable in growth and development, behavioral management, techniques in the fabrication of a prosthesis, the modification of existing teeth utilizing composite resins, the ability to motivate the patient and parent in the use of the prosthesis, and the long-term follow-up for the modification and/or replacement of the prosthesis". According to Nowak, a series of introductory visits may be needed before treatment commences, to attain the required patient trust.[7]

Fixed prosthodontic treatment is seldom used exclusively in the treatment of ED, primarily because many afflicted individuals have a minimal number of teeth. In addition, ED patients are often quite young when they are first treated, and fixed partial dentures (FPD) with rigid connectors should be avoided in young, actively growing patients. This is because rigid fixed partial dentures could interfere with jaw growth, especially if the prosthesis crosses the midline. Hogeboom presented a case that dramatically demonstrated the occurrence of jaw growth in an individual treated for ED in which the two segments of a detachable fixed prosthesis separated at the midline because of transverse jaw growth.[6]

Individual crown restorations have no age restriction related to jaw growth, but larger pulp sizes and shorter crown heights may cause concern. In spite of these concerns, crowns are often used in the treatment of young ED patients.[8] Recently, direct composite restorations have become the more desirable method of restoring normal morphology to hypoplastic teeth commonly found in ED patients. Crowns and direct composite restorations are often used in combination with removable partial dentures (RPDs) in the prosthodontic management of these patients. They are usually necessary to provide proper contours on the hypoplastic teeth that will be used as abutments for removable partial dentures. Also, orthodontic treatment may be needed to align the teeth into acceptable positions before removable partial denture fabrication.[9],[10]

Removable prosthodontics is the most frequently reported treatment modality for the dental management of ED. Because anodontia or hypodontia is typical in individuals with this condition, complete dentures, partial dentures, or overdentures are often part of the treatment provided. Although complete dentures can provide an acceptable esthetic and functional result, underdevelopment of the edentulous alveolar ridges in individuals with ED can compromise denture retention and stability.

When there are teeth present for support overdentures are a desirable treatment option for these patients. Cram provided an excellent overview of the advantages of conventional overdentures as opposed to complete dentures. One important advantage is that overdentures preserve alveolar bone. Van Waas et al. verified this claim with a well-designed, randomized controlled clinical trial. The trial compared average rnandibular bone reduction in 74 patients treated randomly with either an immediate overdenture on two mandibular canines or an immediate complete denture. There was a significant reduction in alveolar bone loss in the overdenture patients after 2 years. Preservation of alveolar bone is imperative in individuals with ED because they must depend on the alveolar ridges for prosthesis support from an early age. If an overdenture is fabricated, retention can be augmented by various attachments anchored to the available teeth.[6]

Periodic recalls of young ED patients are also important because prosthesis modification or replacement will be needed as a result of continuing growth and development. In addition to adjustments related to fit, occlusion of prosthesis must be monitored for ages because of jaw growth. Other problems related to removable prostheses are speech difficulties, dietary limitations, and loss of the prosthesis.[7]

   References Top

1.Dhanrajani PJ, Jiffiy AO. Management of Ectodennal Dysplasia. A Literature Review. Dental Update 1998;25:73-5.  Back to cited text no. 1    
2.Hodges J, Sarnantha, Harley KE. Witkop tooth and nail syndrome: Report of two cases in a family. Int J Paediatric Dentistry 1999;9:207-11.  Back to cited text no. 2    
3.Kupietzky K, Milton H. Hypohidrotic ectodermal dysplasia: Characteristics and treatment. Quintessence Int 1995;26:285-91.  Back to cited text no. 3    
4.Richard AS, Karin V, Gerard K, Caries B, Kournjiarn J. Placement of an endosseous implant in a growing child with ecto dermal dysplsia. Oral Surg, Oral Med, Oral Pathol 1993;75:669-73.  Back to cited text no. 4    
5.Shaw RM. Prosthetic management of hypohidrotic ectodermal dysplasia with anodoutia. Case report. Aust Dent J 1990;35;113-6.  Back to cited text no. 5    
6.Piegno MA, Blackrnan RB, Cronin RJ, Cavazos E. Prosthodontic management of ectodermal dysplasia: A review if the literature. J Prosthet Dent 1996;76:541-5.  Back to cited text no. 6    
7.Nowak AJ, Dental Treatment for patients with Ectodermal dysplasia. Birth Defects 1988;24:243-52.  Back to cited text no. 7    
8.Ellis RK, Donly KJ, Wild TW. Indirect composite resins crowns on an esthetic approach to treating Ectodermal dysplasia: A case report. Quintessence Int 1992;22:727-9.  Back to cited text no. 8    
9.Till MJ, Marques AP, Ectodermal Dysplasia: Treatment considerations and case report. Northwest Dent 1992;71:25-8.  Back to cited text no. 9    
10.Goepferd SJ, Carroll CE, Hypohidrotic ectodermal dysplasia: A unique approach to esthetic and prosthetic management. J Am Dent Assoc 1981;102:867-9.  Back to cited text no. 10    


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

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