Case Report

Oral Care Program for Successful Long-Term Full Mouth Habilitation of Patients with Hypohidrotic Ectodermal Dysplasia

Table 1

The challenges and advised oral care program for long-term success treatment of HED patient.

PhaseAge range and dental stageChallenges and considerations in oral habilitationAdvised oral care programMultidisciplinary dental specialists

(1) Preschool phase(i) Younger than 5
(ii) Primary dentition
(i) Early diagnosis
(ii) Child’s cooperation in dental therapy
(iii) Limited dental treatment options
(iv) Child’s hand dexterity to handle removable prosthesis
(v) Maxillary prosthesis is in high priority in terms of appearance and verbal development
(vi) Due to extensive defects and continuing growth, HED patient should have access to a team of multidisciplinary specialists for planning and treatment
Goal: early intervention and accessibility to an interdisciplinary team of dental specialists
(i) Clinical, imaging, and genetic examination for early diagnosis
(ii) Enhance child’s familiarity with the environment of the dental clinic and inform the parents about short/long-term treatment options
(iii) Fabrication of first interim denture prosthesis (“learning prosthesis”)
(iv) Early oral treatment benefits HED child in better chewing function, adequate nutrient, normal appearance, and speech development
(i) Pedodontist
(ii) Prosthodontist

(2) Childhood phase(i) Age 6–12
(ii) Mixed dentition
(i) Affected patients begin to deal with distinct appearance among peers
(ii) “Nine-year crisis” may cause social withdrawal
(iii) Jaw bone growth and tooth shedding may cause the prosthesis to be gradually unfitted
Goal: to maintain the function and comfort of first prosthesis
(i) Periodically recall to adjust existing prosthesis
(ii) Replacement of existing prosthesis when necessary
(iii) Implants on mandibular anterior area may be considered in fully edentulous patients
(i) Pedodontist
(ii) Prosthodontist
(iii) Periodontist
(iv) Oral surgeon

(3) Adolescence phase(i) Age 13–18
(ii) Early permanent dentition
(i) Esthetic considerations to strengthen self-confidence
(ii) Interproximal space and malocclusion cannot be corrected solely by dental prosthesis
(iii) Oral hygiene maintenance is difficult if orthodontic therapy is carried out
Goal: preparation for permanent oral habilitation
(i) Periodically recall to adjust existing prosthesis
(ii) Reshaping of cone-shaped teeth with direct composite restoration to enhance esthetics
(ii) Orthodontic therapy to align residual teeth and correct occlusion
(iv) Preservation of existing teeth and alveolar bone
(v) Enhancement of oral hygiene and maintenance
(i) Pedodontist
(ii) Orthodontist
(iii) Prosthodontist

(4) Adult phase(i) Age 19 and beyond
(ii) Permanent dentition
(i) Extensive missing teeth and knife-edged alveolar ridge pose challenge in support, retention, and stability of permanent prosthesis, such as RPD or implants
(ii) Skeletal growth is matured and favorable for permanent oral habilitation
Goal: implementation of oral habilitation with permanent prosthesis
(i) Fabrication of permanent prosthesis with different modalities (complete denture, RPD, implant-supported removable/fixed prosthesis)
(ii) Ridge augmentation may be necessary prior implant therapy
(i) Prosthodontist
(ii) Periodontist
(iii) Oral surgeon