|Year : 2013 | Volume
| Issue : 2 | Page : 73-77
Early prosthodontic intervention in a child patient of hypohidrotic ectodermal dysplasia
Culatur Thulasingam, Meenakshi Akshayalingam, Pallavi Vashisht
Department of Prosthodontics, Tamil Nadu Government Dental College and Hospital, Chennai, Tamil Nadu, India
|Date of Web Publication||22-Oct-2013|
Department of Prosthodontics, Tamil Nadu Government Dental College and Hospital, Chennai, Tamil Nadu
The ectodermal dysplasias (EDs) comprise a large, heterogeneous group of inherited disorders that are defined by primary defects in the development of two or more tissues derived from embryonic ectoderm. Patients with this disease often need complex prosthetic treatment. The physiologic and psychosocial value of prosthetic dental treatment increases all the more in very young patients, in which absence of teeth can negatively affect psychological and social growth of child. This clinical report describes the prosthetic management of a 4-year-old child patient with ED.
Keywords: Anodontia, heritable, hyperpigmented, hypodontia, hypohidrotic, onychodysplasia, soft relining silicone
|How to cite this article:|
Thulasingam C, Akshayalingam M, Vashisht P. Early prosthodontic intervention in a child patient of hypohidrotic ectodermal dysplasia. SRM J Res Dent Sci 2013;4:73-7
|How to cite this URL:|
Thulasingam C, Akshayalingam M, Vashisht P. Early prosthodontic intervention in a child patient of hypohidrotic ectodermal dysplasia. SRM J Res Dent Sci [serial online] 2013 [cited 2020 Jan 19];4:73-7. Available from: http://www.srmjrds.in/text.asp?2013/4/2/73/120182
| Introduction|| |
Ectodermal dysplasias (EDs) are described as heritable conditions in which there are abnormalities of two or more ectodermal structures such as the hair, teeth, nails, sweat glands, craniofacial structure, digits, and other parts of the body. It is not a single disorder, but group of syndromes. There is typical triad of nail dysplasia (onychodysplasia), hypotrichosis (scanty, fine light hair on the scalp and eyebrows), and palmoplantar hyperkeratosis; and is accompanied by lack of sweat glands and partial or complete absence of primary and\or permanent dentition. 
There are two categories of ED, the hidrotic and hypohidrotic forms. The teeth and hair are affected in both, but nail and sweat gland manifestations tend to differ. Hypohidrotic ED with an X-linked recessive trait is most frequently reported, with men most frequently and severely affected.  Hypodontia of primary and permanent dentition is common symptom with hypoplasia of alveolar bone and poorly formed alveolar ridges. The number of missing teeth varies, with a higher incidence in the mandible. In the primary dentition; maxillary second molars, canines, central incisors, and mandibular canines are the teeth most commonly present.
| Case Report|| |
A 4-year-old male child patient came to department with chief complaint of missing teeth [Figure 1]. There was delayed eruption of teeth present in mouth. Examination revealed partial anodontia in maxillary and mandibular arches. Teeth present were cone-shaped deciduous maxillary incisors, deciduous maxillary second molars, and young permanent developing mandibular first molars. Orthopantomogram (OPG) was taken and it revealed developing tooth buds of right and left maxillary permanent first molar and right permanent second molar [Figure 2]. There was little growth of alveolar ridge due to absence of teeth. Clinical examination of patient showed fine sparse hair on eyebrows, frontal bossing, wrinkled hyperpigmented skin around eyes, midfacial depression, and low set ears.
Child was withdrawn and introvert due to very few teeth present and was developing poor self-esteem. Psychological impact of this condition on both parents and child was quite evidently seen due to reluctance of child to go to school, therefore psychological counseling was conducted, treatment plan, and future rehabilitation options were also explained to the parents.
Dental conventional removable partial denture with modified approach was planned for prosthetic rehabilitation of this child patient.
Cone-shaped deciduous maxillary central incisors were built to normal anatomic contours with composite restorative material. Shade selection was done and teeth were polished with prophylactic paste. Mesial, distal surfaces, and incisal edges of cone-shaped maxillary incisors were etched for 60 s, rinsed, and bonding agent was applied. Bonding agent was light, cured, and composite build up was done in increments to obtain proper shape of deciduous central incisors. Diastema was closed and restoration was finished and polished [Figure 3].
A preliminary impression was made of both arches using irreversible hydrocolloid impression material. The impressions were poured with dental plaster to obtain primary casts. Teeth were blocked out with wax and a custom tray was fabricated with autopolymerizing acrylic resin. Extensions of the tray were checked in the patient's mouth and adjusted. Border molding was carried out using low fusing compound. Retention and stability of the tray was checked and final impressions were made using light body polyvinyl siloxane impression material [Figure 4]. Master cast was obtained and temporary denture bases and occlusion rimes were prepared [Figure 5]. Maxillomandibular jaw relations were recorded in conventional manner and transferred to the articulator.
Selection of teeth was made such that shade and size matches with the natural teeth present, lighter shade was preferred to give appearance of milky white deciduous teeth and smallest size available was selected. Further modification of teeth was carried to make their size suitable for child patient. Generalized spacing was incorporated in between teeth to simulate spacing present in the deciduous dentition, thereby giving natural look to the prosthesis [Figure 6]. Wax try in was carried out to evaluate esthetics and processing was done in a conventional manner.
The acrylic denture base surrounding the natural teeth was trimmed to give adequate clearance for proper insertion and removal in the patient's mouth [Figure 7]. Conventionally, circumferential clasps are used to encircle the natural teeth to provide retention to removable partial denture, but it would have reduced patient compliance in this case as child is too young to manage insertion and removal and wire can poke and traumatize the adjacent soft tissues. O-rings could have been possibly used if the teeth were fully erupted, due to natural cervical constriction in the normal morphology of tooth to hold the ring at place, but unavailability of cervical constriction due to incomplete eruption of permanent teeth in this case eliminated this option.
Therefore, it was decided to use autopolymerizing soft relining silicone material (DMG Silagum Comfort Soft Relining) at the area around the teeth to provide seal, thus aiding in retention of prosthesis [Figure 8]. Primer was applied on the acrylic denture area around the teeth and was allowed to dry. Base and catalyst paste were mixed together and applied on the denture around both upper and lower molars area and upper central incisor area. Denture was inserted in the patient mouth, held for 5 min and was allowed to set. Denture was removed and inspected for any excess relining material [Figure 9] and [Figure 10]. Overextension of denture borders was checked before delivering the prosthesis, as even one episode of pain can defer child patient from wearing prosthesis.
| Discussion|| |
Prosthetic rehabilitation of young patient of ED at the deciduous dentition stage is challenging due to incomplete development of permanent teeth and very few deciduous teeth. Any permanent fixed prosthesis cannot be supported by these primary teeth.
Because of early-age intervention and the need to easily modify intraoral prosthesis during rapid-growth periods (generally every 2-4 years), removable partial denture is indicated initially. , As the child matures, orthodontic treatment can be initiated and osseointegrated implants can be placed to provide support to the prosthesis.
Reversibility of procedure was the main decisive factor in deciding treatment plan in this case due to unerupted permanent teeth, and patient being too young. Erupted permanent mandibular first molars showed incomplete formation of roots (less than one-third) precluding proper root canal treatment of these teeth, making overdentures less suitable treatment option. Fixed restoration could hinder the growth of the jaws and high pulp horns contraindicate tooth preparation, making fixed prosthesis as a treatment option less suitable.
Conventional removable partial denture with modified approach, being both reversible and favorable to young age of the patient was selected and followed. Due to very young age of the patient, it was best to proceed with the treatment plan as conservative as possible. Child patient was apprehensive about undergoing any treatment procedure, so behavior management was done and tell, show, and do (TSD) technique was followed.
The main aim in rehabilitating this patient was to provide him with prosthesis which should be comfortable, maintain esthetics, easy to insert, remove, and clean. Orthodontics wires were not used as retainers as it can traumatize and poke soft tissues. Instead, soft silicone liner was preferred to provide seal as it would be easier for the child patient to manage, improving patient compliance.
No tooth preparation was done in this case to modify existing teeth. Cone-shaped deciduous maxillary central incisors were contoured to normal morphology. Minimal tooth preparation preserves existing teeth for fixed treatment options in near future as the child grows.
Parents were instructed regarding cleaning and maintenance of dentures. It was instructed to use soft brush to clean the dentures taking care not to peel away the soft liner around the teeth. Regular follow-up was done at 7 days, 21 days, 1 month, 3 month, and 6 months. , At 6 months, there was slight loss of soft liner around maxillary first molar tooth. Soft relining material was applied again ensuring adequate seal.
As the patient grows, the maxillofacial prosthodontist should follow the patient closely to intercept tissue irritations and occlusal discrepancies that result from growth. Due to growth of alveolar ridges and eruption of teeth, prosthesis may need modification after 2-3 years, relining or new prosthesis can be fabricated depending upon the situation. Repeated procedures of relining and rebasing in such patients should not defer a clinician from early intervention, as it provides the child an opportunity to develop normal forms of speech, chewing, and swallowing, normal facial support; and improved temporomandibular joint function. ,,,
When the ED child reaches his/her early teenage years, orthodontic treatment may be indicated, as consolidation of spaces may better prepare the mouth for a fixed partial denture or implants in the future.
When growth has stabilized in early ED patient, osseointegrated implants can be used to support, stabilize, and retain the prosthesis. Depending on the pattern of missing teeth and remaining available alveolar bone, the ideal long-term prosthetic prognosis often requires implants. Implants have been shown to help preserve alveolar bone. ,, If bone atrophy progresses to the extreme in these already alveolar-deficient patients, implant placement may not be possible without bone grafting. 
| Conclusion|| |
This clinical report describes the characteristics and prosthetic habilitation of young male child with anhidrotic ED. Early dental intervention can improve the patient's appearance and minimize the onset of emotional and psychosocial problems often experienced by ED patients.
| References|| |
|1.||NaBadalung DP. Prosthodontic rehabilitation of anhidrotic ectodermal dysplasia patient: A clinical report. J Prosthet Dent 1999;81:499-502. |
|2.||Hickey A, Vergo TJ Jr. Prosthodontic consideration in the treatment of patients with maxillary and mandibular deficiencies. J Prosthet Dent 1991;66:645-9. |
|3.||Pavarina AC, Machado AL, Vergani CE, Giampaolo ET. Overlay removable partial dentures for a patients with ectodermal dysplasia: A clinical report. J Prosthet Dent 2001;86:574-7. |
|4.||Patel MI. Prosthodontic rehabilitation of a patient with partial anodontia: A clinical report. J Prosthet Dent 2002;88:132-4. |
|5.||Hickey A, Vergo TJ Jr. Prosthetic treatments for patients with ectodermal dysplasia. J Prosthet Dent 2001;86:364-8. |
|6.||Abadi B, Herren C. Clinical treatment of ectodermal dysplasia: A case report. Quintessence Int 2001;32:743-5. |
|7.||Bergendal B. The role of prosthodontists in habilitation and rehabilitation in rare disorders: The ectodermal dysplasia experience. Int J Prosthodont 2001;14:466-70. |
|8.||Yap AK, Klineberg I. Dental implants in patients with ectodermal dysplasia and tooth agenesis: A critical review of the literature. Int J Prosthodont 2009;22:268-76. |
|9.||Stanford CM, Guckes A, Fete M, Srun S, Richter MK. Perceptions of outcomes of implant therapy in patients with ectodermal dysplasia syndromes. Int J Prosthodont 2008;21:195-200. |
|10.||Guckes AD, Scurria MS, King TS, McCarthy GR, Brahim JS. Prospective clinical trial of dental implants in persons with ectodermal dysplasia. J Prosthet Dent 2002;88:21-5. |
|11.||Guckes AD, Brahim JS, McCarthy GR, Ruby SF, Cooper LF. Using endosseous dental implants for patients with ectodermal dysplasia. J Am Dent Assoc 1991;122:59-62. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10]