Prosthodontic Management of Hypohidrotic Ectodermal Dysplasia with Anodontia: A Case Report in Pediatric Patient and Review of Literature
An eight-year-old male patient reported to the Rural Dental Hospital with the complain of missing teeth causing difficulty in mastication and speech. He was accompanied by his father who gave the history of missing teeth of his son since infancy. He mentioned that child was intolerant to heat and prefers to be in cold environment. Patient also reported about absence of sweating. Family history revealed that he was the second kid of parents with non consanguineous marriage and none of the other members or relatives had similar condition.\nThe boy was moderately built and poorly nourished with BMI value of 12.5 kg/m2. His skin was dry with scanty body hair. The nails were not affected. Extraoral examination revealed the typical features of ectodermal dysplasia like sparse scalp hair, frontal bossing, absent eyebrows, loss of eyelashes, hyper pigmentation around the eyes and mouth, saddle nose and protuberant lips. A diminished lower facial height contributed to a senile facial expression [].\nIntraoral examination revealed thin, atrophic and complete edentulous maxillary and mandibular alveolar ridges []. Anodontia as well as underdevelopment of alveolar ridges was also confirmed by panoramic radiography []. Correlating clinical, radiographic and skin biopsy findings, the boy was diagnosed with hypohidrotic ectodermal dysplasia with complete anodontia.\nTreatment plan was discussed with patient and his father. Informed consent was obtained for the agreed treatment. The child was rehabilitated with removable maxillary and mandibular complete dentures. Routine procedures were followed for the construction of the complete dentures which included preliminary impressions with irreversible hydrocolloid [], fabrication of special trays using autopolymerizing acrylic resin after applying spacers, border-molding with low fusing compound, final impression with zinc oxide non eugenol impression paste [], recording of maxillomandibular relations and mounting the casts on semi adjustable articulator []. Rather than primary tooth forms, permanent tooth forms were selected in order to provide better static and dynamic occlusion. Teeth arrangement was done and bilateral balanced occlusion was given. Trial dentures were checked for retention, phonetics, occlusion and esthetics. The denture base was processed through compression molding using heat cure fiber reinforced acrylic material (DPI, Mumbai, India) for stability and longevity. The processed dentures were finished and polished. Occlusal equilibrium was confirmed. After the insertion of dentures [], postplacement instructions were given to child and his parents. The facial profile and expression improved significantly with complete dentures [].\nPatient was very cooperative throughout the clinical procedures. Recall appointments were scheduled after 24 hours, 1 week, and 4 weeks. After 4 weeks the patient was able to function well with both maxillary and mandibular dentures. In order to accommodate growth and development, the patient was scheduled for ongoing follow up visits every 6 months. At recall appointments, good retention was observed and the parents reported a significant improvement in terms of speech and mastication.