|Year : 2015 | Volume
| Issue : 2 | Page : 124-127
Prosthetic rehabilitation of a patient with Type-I ectodermal dysplasia: A case report
Laxman Rao, Revathy Gounder
Department of Prosthodontics, Army College of Dental Sciences, Secunderabad, India
|Date of Web Publication||12-Jun-2015|
Army College of Dental Sciences, Jai-Jawahar Nagar Post, CRPF-Chennapur Road, Secunderabad - 500 087
Source of Support: None, Conflict of Interest: None
Type-I ectodermal dysplasia (Christ-Siemens-Touraine syndrome) is characterized by clinical triad of hypohidrosis, hypotrichosis, and hypodontia or anodontia. Depending on the severity of the condition, various prosthodontic treatments are recommended to re-establish the masticatory function, appearance, speech, and also to improve the emotional and social aspects of the child. The various treatments may include removable partial/complete prosthesis, fixed and/or implant-supported prosthesis, or a combination of these. In this clinical report, an 11 year old girl is presented with hypohidrotic ectodermal dysplasia. Implant-supported restorations can improve the physiologic and psychosocial function, when compared with removable dentures; but their placement in growing jaws can cause complications. So, a maxillary flexible removable partial denture and a mandibular conventional complete denture with neutral zone technique were fabricated after considering her growth and the number and condition of her present teeth. Composite resin material was used to restore the conical maxillary canine for a favorable aesthetic appearance.
Keywords: Christ-Siemens-Touraine syndrome, Type-I ectodermal dysplasia, X-linked hereditary disorder
|How to cite this article:|
Rao L, Gounder R. Prosthetic rehabilitation of a patient with Type-I ectodermal dysplasia: A case report. J NTR Univ Health Sci 2015;4:124-7
| Introduction|| |
Two major groups of Ectodermal Dysplasias (EDs) are: 1. hypohidrotic or anhidrotic ( Christ-Siemens-Touraine syndrome More Details) in which sweat glands are either absent or significantly reduced in number and 2. hidrotic (Clouston syndrome) in which sweat glands are normal. Hypohidrotic ED is the most common type and seems to show an X-linked inheritance pattern with gene mapping to Xq12-q13; therefore, males are more susceptible than females. Hidrotic type is inherited in an autosomal dominant pattern.  The incidence of hypohidrotic ED is estimated to be from 1 in 10,000 to 1 in 100,000 live births. 
Mutations in the EDA, EDAR, or EDARADD gene results in defective ectodysplasin A formation, thereby preventing normal interactions between the ectoderm and the mesoderm and, hence, impairing the normal development of hair, sweat glands, and teeth. 
Dental management of ED patients traditionally focuses on provision of a series of complete or removable dentures during the growing years and definitive rehabilitation following completion of jaw growth. 
| Case report|| |
An 11-year-old girl reported to the department with the chief complaint of missing teeth, unaesthetic appearance, and difficulty in chewing food. Patient gave a history of dry skin, reduced sweating, and intolerance to heat. There was no contributory family history. General examination revealed a malnourished girl with typical features of Type-I ED, such as frontal bossing, depressed nasal bridge, protuberant and dry lips, periorbital pigmentation, sparse hair, decreased lower facial height, and scanty eyebrows [Figure 1]. 
|Figure 1: Extraoral facial photographs: (a) frontal view and (b) lateral view|
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Intraoral examination revealed dry oral mucous membrane with fewer teeth in the maxilla and completely edentulous mandible. Conical teeth were present in 13, 23 region. Molars with conical cusp tips were seen in the molar teeth region [Figure 2].
Radiographic view revealed completely edentulous mandibular arch with poorly formed alveolar ridge. Maxillary arch showed fully erupted permanent canines and permanent first and second molars with no evidence of any impacted tooth or any permanent tooth buds [Figure 3]. The child was diagnosed as a case of Christ-Siemens-Touraine syndrome (hypohidrotic ED) with typical characteristics of disease in her face and oral cavity.
|Figure 3: Digital ortho-pantograph showing few teeth in the maxilla and edentulous mandibular ridge|
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As the young patient was at a growing stage, treatment options considered for this patient included constructing maxillary flexible removable partial denture after reshaping the conical maxillary canines with composite and to provide a better stability and retention of the denture on a poorly formed mandibular alveolar ridge, a neutral zone technique  was used to make conventional mandibular complete denture. Maxillary canines were restored with direct composite resin restoration (Primedent, Chicago, IL, USA) prior to impression making.
Primary impressions were made with irreversible hydrocolloid material (alginate, imprint). Diagnostic casts were prepared. Custom trays were fabricated using autopolymerizing acrylic resin and border molding was done with green stick compound (DPI, Bombay Co Ltd). Final impressions were made using light viscosity elastomeric impression material (Aquasil, Germany) for maxillary arch and zinc oxide eugenol impression paste (DPI, Bombay) for mandibular arch. Maxillary and Mandibular occlusal wax rims were fabricated and vertical dimension was determined with the usual 2-4 mm freeway space. Face bow transfer was done and both models were mounted on semi-adjustable articulator (Hanau-Wide Vue Arcon Articulator, Fort Collins, CO, USA) in centric relation. Mandibular occlusal wax was removed. Low fusing modeling compound [mixture of impression compound cake (Y-Dent; Delhi) and green stick compound] was softened in a preheated water bath and placed over mandibular denture base. Mandibular record base with softened compound was carefully placed in the patient's mouth, along with maxillary occlusal rim. Patient was asked to carry out various functional movements such as swallowing, sucking, licking, and smiling. After 3-5 min, the set mandibular record base was removed and examined. Plaster matrices were made around the neutral zone impression [Figure 4]. The neutral zone impression was removed and wax was poured into the space of neutral zone. Mandibular teeth were arranged exactly within the neutral zone space. Maxillary teeth were arranged according to the position of lower teeth with esthetic rules. Try in was done to check the occlusion and esthetic appearance. With the help of injection moulding technique, maxillary denture was fabricated in valplast (Flexible Valplast; USA) resin and the mandibular denture was fabricated in conventional heat cure fiber reinforced acrylic resin. Valplast denture was kept in warm water for 2-3 min prior to denture insertion. Post-insertion instructions were given and recall appointments were scheduled after 24 h, 1 week, and 1 month during which the patient reported only minor problems and also significant improvements in appearance, speech, masticatory function, and social interaction [Figure 5].
| Discussion|| |
The restoration of a natural and pleasing appearance [Figure 6] is important in hypohidrotic ED children for their normal psychological development and social involvement. Early prosthetic treatment leads to a significant improvement in speech, masticatory function, and facial aesthetics.  Initially, prosthodontic intervention can be accomplished with a removable prosthesis, which gives a rapid and painless result and, at the same time, minimizes the onset of emotional and psychological problems for the patient and her/his family. Later on, implant-retained prosthesis can be planned when the jaw bone growth stops.  According to Cronin et al.,  early implant placement can cause complications during the growth period.
Due to oligodontia or anodontia, children with Type-I ED have little or no bone ridge upon which to construct dentures. In such cases, a denture fabricated by neutral zone technique will ensure that the muscular forces aid in the retention and stability of the denture rather than dislodging the denture during function. 
Flexible denture results in better esthetic outcome as metal clasps can be eliminated and there is no excessive stress on the abutment teeth. It also offers toxological safety to patients allergic to conventional metals. Furthermore, it has higher elasticity and significant strength for use as a denture base material. 
Continuing skeletal growth and development of the young ED patient might require prosthesis modification and replacement. Thus, periodic recalls and maintenance of young ED patients are also important. 
The first goal of a prosthodontic aspect is to meet the immediate needs of the affected young patient, which include mastication, esthetics, speech development, and improvement of psychological factors. This clinical case demonstrates that maxillary flexible denture with direct composite restoration of conical canines and conventional complete mandibular dentures are a more economical and non-invasive method for treating a patient with Type-I ED during their growth period.
| Conclusion|| |
Conventional prosthodontic treatment should be started as soon as an ED patient reports to the dentist for the physiological and psychosocial development (begins at the age of 3 years). At the same time, the provided dentures can maintain the facial contour, vertical dimension, masticatory function, and esthetics of the patient. This should be followed by definitive (implant-retained prosthesis) prosthodontic treatment after completion of the jaw growth.
| Acknowledgment|| |
The author likes to thank Dr. M. N. Khasim for his technical support.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]