Oral rehabilitation of a patient with ectodermal dysplasia with prosthodontics treatment.

Ectodermal dysplasia (ED) is a rare, congenital disease that involves the sweat glands, scalp hair, nails, skin pigmentation, and craniofacial structure. Oral symptoms of ED include multiple tooth abnormalities (such as hypodontia, anadontia, impacted teeth, and peg-shaped or conical anterior teeth) and lack of normal alveolar ridge development. A 12-year-old male patient, in the absence of any other systemic abnormalities, exhibited typical characteristics of ED, visited our department of pedodontics and preventive dentistry at Panineeya Mahavidyalaya Institute of Dental Sciences and Research Centre. In the clinical and radiographic evaluation, it was occurred that he had only maxillary and mandibular first molars in his mouth. A maxillary and mandibular denture with clasps for retention was fabricated for prosthodontic rehabilitation after considering his growth and the number and condition of his present teeth. At the 18-month follow-up no major complications occurred and further future treatment included implants retained fixed partial dentures.

What was known?

Ectodermal dysplasia is a rare, congenital disease that involves the sweat glands, scalp hair, nails, skin pigmentation, and craniofacial structure with oral symptoms like hypodontia, anadontia, etc.

Introduction

Ectodermal dysplasia (ED) have been described as a group of disorders of morphogenesis displaying two or more of the symptoms of trichodysplasia, dental anomalies, onychodysplasia, and dyshidrosis.[1] It is usually described as being hypohidrotic or hidrotic, depending upon the degree of sweat gland function. Congenital malformation of teeth, hair, nails, or sweat glands may occur either as single isolated malformations or as a part of an ectodermal dysplasia syndrome. Anhidrotic ectodermal dysplasia is considered to be a triad of hypodontia or anodontia, hypotrichosis, and hypohidrosis, and associated with other components that result from defective development of structures of ectodermal origin.[2] Anhidrotic ectodermal dysplasia is X-linked condition, and is found in all races, with an incidence of 1-7 per 100.000 live births.[3] Affected males usually have prominent supraorbital ridges, frontal swelling, thin eyebrow, fine-linear wrinkles, sparse hair, detective nails, and saddle nose.[34] Due to the altered anatomy in the lower third of the face, they resemble to edentulous old individuals.

Oral characteristics include complete or partial hypodontia, anodontia, impacted teeth, loss of vertical dimensions of occlusion, protuberant lips, malformed and peg-shaped or conical teeth, and lack of alveolar growth. Oral symptoms of ED include multiple tooth abnormalities and lack of normal alveolar ridge development can complicate the restoration of an ideal occlusion, especially in a growing child. Lack of alveolar growth frequently results in increased interocclusal distance, which allows optimum artificial tooth placement.[5] For rehabilitation, it is crucial to know the age, number and condition of present teeth, and the state of growth of the patient.

Case Report

A 12-year-old boy reported to our out patient department with a chief complaint of missing teeth, inability to eat, difficulty in speech. The patient even had a psychological set back due to lack of esthetics. The general medical and family history was non contributory. The boy presented with classical features of ectodermal dysplasia: Partial anodontia [Figures 1 and 2], hypohidrosis, hypotrichosis, frontal bossing, saddle nose, diminished lower facial height, sparse scalp hair, missing eye lashes and eyebrows, protuberant lips. The intraoral examination revealed presence of maxillary and mandibular first molars [Figure 2]. The alveolar ridge was efficient in both height and width. The oral mucosa was dry in appearance. This was the patient's first dental visit and he never wore any dentures before.

Figure 1

Intra oral view of only maxillary and mandibular molars in occlusion

Figure 2

Frontal view of the face

Current treatment done and future treatment plans

Removable prosthesis through dentures was planned for maxilla and mandible. Steps included are primary impression followed by special tray fabrication [Figure 3], then border molding was done on special trays and dual impression technique opted for better impression recording [Figures 4 and 5]. Then wax rims were made and jaw relationship is recorded, followed by trial dentures and finally finished partial dentures [Figure 6] were given to complete oral and psychological rehabilitation [Figure 7]. Future treatment includes implants and fixed partial dentures for both maxilla and mandible.

Figure 3

Special trays for maxilla and mandible

Figure 4

Secondary dual impression for maxilla

Figure 5

Secondary dual impression for mandible

Figure 6

Final dentures for maxilla and mandible

Figure 7

Oral and psychologically rehabilitation done

Discussion

Hypodontia and anodontia were frequently seen in many cases of ectodermal dysplasia.[67] Prosthetic rehabilitation of patients with ED is an ordinary process. Early and extensive dental treatment is needed throughout childhood because of the absence of most of the deciduous and permanent dentition. A multidisciplinary team approach to management of these patients is recommended. [8] Osseo-integrated implants should be an alternative treatment in older patients with ED.[9-13] Considering the poor economical situation of the present case, it was deemed better to postpone Osseo-integrated implants. This is commonly a difficult condition to manage the patients with prosthodontics because of the typical oral deficiencies and because the afflicted individuals are quite young when they are evaluated for treatment. It is important that these individuals receive dental treatment at an early age for physiologic and psychosocial reasons. Adaptation to the prosthesis depends on various factors as, capability of the specialist, age of the patient, and cooperation in a growing child. A removable partial denture or an overdenture is often a suitable treatment choice, because of the need to easily modify the intraoral prosthesis during rapid growth period[1213] and also these treatment options are easy, affordable, and reversible rehabilitation methods.

The treatment goals for this patient were to establish a functional occlusion with prosthetic rehabilitation and, to obtain an esthetic smile with adult-sized teeth, because any prosthesis was not worn by the patient before. Also nutrition of the patient is improved, and a self-confident appearance is provided.

What is new?

Early and extensive dental treatment is needed throughout childhood. A multidisciplinary approach to management of these patients is recommended.