Prosthodontic Rehabilitation of Ectodermal Dysplasia – A Case Report

INTRODUCTION

Ectodermal dysplasia syndrome, an inherited x-linked recessive triat usually is transmitted with the gene being carried by the female partner and manifested in the male partner¹. However, an anhidrotic ectodermal syndrome may occur in a family with no previous history of this disease because of gene mutation. It occurs in all races, and affects approximately 1to7 per 1,00,000 live births² .

Anhidrotic ectodermal dysplasia is considered to be a triad of hypodontia, hypotrichosis and hypohidrosis³ and associated with other components that result from defective development of structures of ectodermal origin.

Hypotrichosis occurs with the scalp hair being thin in childhood. Body hair and eyebrows are sparse. Variable degrees of hypohidrosis are present, frequently with significant episodes of hyperthermia in infancy and early child hood⁴.

Oral manifestations of Hereditary Ectodermal dysplasia are of particular interest to dentist, because patients with this disorder invariably have missing and misshapen teeth. It can affect both primary and permanent dentition ⁵.

Because of partial development of teeth or the absence of teeth in patients with hereditary ectodermal dysplasia, the restoration of the dentition to proper form and function can be a significant challenge to the dentist, particularly if the patient is young.

CASE REPORT

A male patient aged 18 years reported to department of prosthodontics, Al-Badar Dental College and Hospital, Gulbarga. His medical and dental revealed that he was diagnosed with Anhydrotic ectodermal dysplasia.

The typical characteristic features were protuberant lips, saddle nose, fine hairs and eye brows. He also gave the history of hyperpyrexia, he had no family history of AED. Oral examination revealed thin dry oral mucosa, indistinct vermillion border, under developed maxillary and mandibular alveolar ridges which were small and thin, the palatal vault was flat (loss of vertical dimension). In maxillary arch 2 conical shaped teeth were present in the anterior region⁶ .

Radiographic features: intraoral periapical radiograph (IOPA) relation to upper anterior region and orthopantomograph (OPG) was advised. It revealed the roots of both the anterior teeth were not fully developed with pulp canals. OPG revealed that maxillary and mandibular alveolar processes were under developed. Mental foramen is placed close to the crest of alveolar ridge.

Prosthodontic Treatment

After through discussion and consultation, it was decided to fabricate maxillary over denture and conventional mandibular denture with soft tissue liners.

The patient was sent for root canal treatment of both the maxillary anterior teeth and after obturation, crown were reduced up to the level of gingiva. Upper and lower primary impression with impression compound were made, cast was prepared and special tray was fabricated.

Border molding with green stick compound was done followed by secondary wash impression with light body addition silicone material was recorded (addition silicon material was considered because it is highly bio-compatible, elastic, clean and pleasant for the patient, since he had dry oral mucosa)⁷ .

Permanent denture bases with occlusal wax rims were fabricated and used to record centric relation position. Anatomic posterior teeth were used to develop bilateral balanced occlusion. Maxillary and mandibular trial dentures were tried in centric relation position. After the approval of trial denture bases by patient and his accompanying relative, the waxed up dentures were carved and processed. After final finishing and polishing was accomplished the dentures were inserted.

The patient was recalled for post insertion check-up and necessary correction were made. The patient was instructed for proper maintenance of the oral tissues and prosthesis.

Problem encountered dur ing pros theticrehabilitation⁸ .

1) Patient was uncooperative.

2) The mouth conditions were apparently inadequate Retention and stability of removable denture.

3) Difficulty in management of such patient for executing the necessary procedures in the mouth like impression making, root canal treatment.

Successful management can be achieved by following means⁹

1) Establishing a friendly relationship with a patient and enduring confidence into him.

2) Dentist should have full knowledge to handle the problems associated with treatment.

3) By using materials and techniques which require minimum intra oral working time and which do not produce any unpleasant reaction in the mouth.

4) Tell-Show-Do approach.

5) By providing interdisciplinary approach.

6) By educating patient about continuous follow up appointment for needed adjustment or for the replacement.

DISCUSSION

These patients will have partial or complete absence of sweat glands and cannot perspire and they consequently suffer from hyper pyrexia and inability to endure warm temperature¹⁰.

There is strong psychological impact on the patient and parents due to the absence of the teeth where little can be done for the facial appearance of the patient. Their appearance can gently be enhanced by making removable prosthodontic restoration. So early prosthodontic consultation is therefore an essential part of the management of ectodermal dysplasia¹¹.

Treatment for the patients with ectodermal dysplasia requires the efforts of team approach. For a patient in this clinical report, the two maxillary anterior conical teeth remained as potential over denture abutments and hopefully it stimulate the growth and maintenance of acceptable results for esthetics, psychological support and function.

However underdevelopment of the alveolar ridge makes denture difficult to achieve retention and stability. Since the oral mucosa was dry and thin, denture soft liner was the best option to avoid trauma and discomfort to the patient; and use of implants with attachments can give a good retention to the dentures so this will also help for the psychological support of patient.

The patient shows good adaptation to the upper over denture and lower complete denture lined with soft tissue liners. With proper care and prosthodontic treatment, the patient can enjoy a relatively normal life, therefore it is important that the patient and his parents and close relatives fully understand the dental problems related to his physiologic and psycho logic conditions. Periodic recall is very important for frequent adjustments.