Multidisciplinary Rehabilitation of Ectodermal Dysplasia : A Case Report

This case report describes a joint multidisciplinary approach of restoring function and esthetics in an 18-year-young female with ectodermal dysplasia. Ectodermal dysplasia is both physically and emotionally devastating to patients with dental manifestations. It is important that they are treated at an early age to help their social interaction. With proper dental intervention, the quality of life can be improved for patients with ectodermal dysplasia.


INTRODUCTION
Ectodermal dysplasias (ED) are a heterogeneous group of disorders characterized by developmental dystrophies of ectodermal structures. 1 Hypohydrotic ectodermal dysplasia is the most common type and is usually inherited as an X-linked recessive trait.It is characterized by the triad of signs which comprises of sparse hair (atrichosis or hypotrichosis), abnormal or missing teeth (anodontia or hypodontia), and inability to sweat due to lack of sweat glands (anhidrosis or hypohidrosis).
The incidence of ED is estimated at 1 in 100,000 births. 2 The earliest recorded case of ectodermal dysplasia was described in 1792. 3There are more than 100 different ED syndromes, 4 clinical manifestations depend on the specific syndrome afflicting an individual.The most frequently reported ED syndrome is X-linked hypohidrotic dysplasia, 5 also known as Christ-Siemens-Touraine syndrome, 6 which affects one to seven individuals per 10,000 live births. 6todermal dysplasia syndromes have been described as a group of disorders of morphogenesis displaying two or more of the following signs and symptoms: (1)   trichodysplasia, (2) dental anomalies, (3) onychodysplasia, and (4) dyshidrosis.These malformations result from developmental defects in tissues in which progenitor cells were originally derived from the ectoderm of the embryo.
Congenital malformation of teeth, hair, nails, or sweat glands may occur either as single isolated malformations or as a part of ectodermal dysplasia syndrome. 7Freire-Maia and Pinheiro classified ectodermal dysplasias into 11 possible groups, based on all possible combinations of Dr Alka Gupta, 1 Dr Sunita Karki, 2 Dr Deepak Thapa, 3  Orthodontic treatment was performed on the maxillary arch with fixed orthodontic appliance of 0.018" slot.
Leveling and alignment was performed with gradual 0.012", 0.014", 0.016" Niti wires followed by 0.016", 0.018" round stainless steel wires; then 0.016x0.022"rectangular stainless wire to stabilize the arch.Meanwhile, closing and maintenance of space was done using elastomeric chains; proper spaces were maintained to allow fixed partial dentures.
After completing the orthodontic phase, patient was referred to the Dept. of Prosthodontics for prosthetic rehabilitation.On upper arch, teeth were prepared for porcelain fused to metal (PFM) bridge then cemented with fixed partial denture with A2 Vita shade (Figure 3, 4).PFM bridge was given in three segments i.e. from canine to molar on either sides and lateral to lateral bridge in the anterior segment.Lower arch was initially planned for implant placement; however it could not be considered due to the thin cortical plate.Lower arch was rehabilitated with cast partial denture utilizing semiprecision attachments (vertex) in the missing area along with metal crowns in relation to 36, 37 and 46, 47 (Figure 5).
OPG revealed numerous conical teeth in upper anterior and impacted teeth in lower anterior regions (Figure 6).
Based on the clinical and radiographic findings, a case of Hypohydrotic Ectodermal Dysplasia was diagnosed.The most frequent treatment for such group of patient is orthodontic alignment and rehabilitation followed by fixed or removable prosthodontics.In such patients; diagnosis should be made at earliest followed by thorough history and clinical examination of the patient and also of the family members.Multidisciplinary joint approach involving oral medicine, oral surgery, periodontics, orthodontics and prosthodontics is a need for the patient being treated for ectodermal dysplasia.
photographs and study model.On extra-oral examination, patient gave a characteristic appearance of sparse hair on scalp and eyebrow (fine and dry), frontal bossing, saddle nose, sunken cheeks, macroglossia, pigmentation around eyes and thick

Figure 1 :
Figure 1: Pre and post-treatment extra-oral photographs

Figure 6 :
Figure 6: Pre and end-treatment radiographs