Treatment Considerations for a Patient with Ectodermal Dysplasia

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					J. Int Oral Health 2010                                                       Case Report
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                                                                           P- ISSN
Treatment Considerations                                                 0976 – 7428
for a Patient with
                                                                           E- ISSN
Ectodermal Dysplasia: A                                                  0976 – 1799
Case Report
                                                                       Journal of
Patel Jayantilal R* Sethuraman Rajesh** Naveen Y G
Patel Nandini ##                                                      International
*M.D.S, Professor and Head, **M.D.S, Reader, #Senior                   Oral Health
Lecturer, ## Post Graduate Student, Department of
Prosthodontics, K.M Shah Dental College, Vadodara, Gujarat,
India. Contact:                                    Prosthodontics

Abstract:                                                                  Case Report
Ectodermal dysplasia is a hereditary disease characterized by
congenital dysplasia of one or more ectodermal structure and
other accessory appendages. The oral manifestations are
anodontia and poor bony foundation which impairs both                 Received: Aug, 2010
aesthetic as well as the masticatory function. The
                                                                      Accepted: Nov, 2010
Prosthodontic management of patients with such dysplastic
condition necessitates a multidisciplinary approach. However
the definitive treatment can only be rendered after the
completion of growth period, and till then a provisional
treatment can be given to enhance the aesthetic and functional
requirement of the patient. This case report describes the
Prosthodontic provisional oral rehabilitation of a 12 year old
male pediatric patient with ectodermal dysplasia.
                                                                      Bibliographic listing:
Key words: Ectodermal dysplasia, Anodontia, Provisional                EBSCO Publishing
Prosthodontic Rehabilitation.
                                                                         Database, Index
Introduction:                                                         Copernicus, Genamics
                                                                      Journalseek Database
       Ectodermal dysplasia (ED) is a hereditary disorder that
can affect several ectodermal structures. These structures may
include: skin, hair, nails, teeth, nerve cells, sweat glands, parts
of the eye and ear, and parts of other organs.

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ED syndromes have been described as a group of         available to improve appearance, mastication, and
disorders of morphogenesis displaying two or more      speech (12).
of the following signs and symptoms.(1,2,3)
                                                                 Children with ED usually have a normal
1. Trichondysplasia (abnormal hair)                    mentality and life expectancy, and their facial
                                                       appearance warrants professional concern for their
2. Abnormal dentition                                  emotional well being and social progress (7, 13).
                                                       Tanner (14) states ectodermal dysplasia with an
3. Onchondysplasia (abnormal nails)
                                                       abnormal appearance may affect normal social and
4. Dyshidrosis (abnormal or missing sweat glands)      psychological development in young patients.
                                                       Functional needs also must be considered since the
       The most reported ED syndrome is x-linked       difficulty these children experience in masticating
hypohidrotic (anhidrotic) ED (Christ-Siemens-          may cause nutritional problems (7, 13). Therefore,
Touraine syndrome) which affects one to seven          dental care for ED patient is important.
Individuals per 10,000 with males afflicted more
frequently than, females(1,2,4,5-8). Patients with           This clinical case report describes the oral
hypohidrotic ED generally have prominent               rehabilitation of a 12 year-old male ED patient
supraorbital ridges, frontal bossing, and a saddle     diagnosed with hypodontia
nose. The maxilla may be under developed and the
lips are thick and prominent. The nose may appear      Case report:
pinched and the aleque nasi appears hypoplastic.       A 12-year-old male patient with ectodermal
The patient may resemble like an old edentulous        dysplasia was referred by his physician to the K.
person (7, 8). The skin is usually dry, scaly, and     M. Shah Dental College & Hospital, Piparia for
easily irritated as a result of poorly developed or    an examination due to the non-eruption of his
absent sebaceous glands. Sweat glands may also be      permanent teeth [Figure-1].
absent or few in number or nonfunctioning which
may result in a increased body temperature. Scalp
hair may be absent, sparse, very fine pigmented, or
abnormal in texture. Eyebrows, eye lashes, and
other body hair may also be sparse or absent. When
hairs are present, they may be fragile, dry, and
generally disorderly because of the lack of
sebaceous glands. Finger and toe nails are usually
normal (8). Orofacial characteristics of this
syndrome include anodontia or hypodontia,
hypoplastic conical teeth, underdevelopment of the
alveolar ridges, frontal bossing, a depressed nasal
bridge, protuberant lips, and hypotrichosis (9, 10).
Teeth in the permanent dentition are frequently
small, conical, tapered (peg shaped), and widely
spaced [Figure – 2]. Lack of alveolar growth may
be associated with this condition and frequently
results in increased interocclusal distance which      Fig-1: Preoperative patient photo
allows optimum artificial tooth placement (11).
Patients may present with a marked mandibular
protrusion. Depending on the severity of the                His parents consulted a general dentist
condition, various prosthodontic treatments are        when the boy was 4 years old, because his milky

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teeth were not erupted and he had difficulty in
speaking and chewing. Treatment was not
possible at that time due to behavioral problems
at that age. At age six, the boy became
uncomfortable with the appearance of his teeth
and very reluctant to smile. He was now
motivated to seek help from a dentist.

                                                      Fig-3: A panoramic       radiograph    revealed   six
                                                      permanent teeth
Fig-2: Patient photo showing peg shaped central and
lateral incisor                                         Since patient was only 12 year of age with still
       A clinical examination revealed only           growth period left and also pulp horns were
permanent teeth present in the maxilla. The four      significantly high, the treatment plan was
existing teeth were extremely tapered (peg-           formulated and executed to give maxillary
shaped) and widely spaced [Figure-2]. All             removable partial denture and mandibular
Mandibular teeth were missing. The color of           complete denture with composite build up for
alveolar mucosa and gingiva was normal, but           maxillary central and lateral incisors for the
the alveolar ridges were rather atrophic except       patient’s aesthetic concerns related with the peg
in the areas where teeth were present.                shaped teeth.
       The      patient    exhibited     typical
characteristics of hypohidrotic ED, including a
saddle nose, fine sparse hair, everted lips in
profile, and hypodontia. The skin of the body
was dry and atrophic. However, the shape of the
fingernails and toenails appeared normal.
During the physical examination he showed no
signs of mental problems and his vital signs
were normal. A panoramic radiograph revealed
six permanent teeth (central incisors, lateral
incisors and molar) in the maxilla & completely
                                                      Fig-4: Composite resin build up for maxillary central
edentulous mandibular arches [Figure-3].              and lateral incisors

Treatment plan and procedure                           Diagnostic impressions were made with
                                                      alginate impression material and diagnostic
                                                      mounting was done. Maxillary Central &
                                                      Lateral Incisors were restored to give
                                                      appropriate shape with Composite Resin

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[Figure-4]. Final impressions were made with
elastomeric impression material in upper arch
and zinc-oxide eugenol in lower arch. Master
casts were made with dental stone. Face bow
transfer of maxillary arch was done. Vertical
jaw relations were established. Centric relation
was recorded. Then casts were mounted on semi
adjustable (Hanau) articulator and teeth were set
in balanced occlusion [Figure-5].

                                                     Fig-6: Denture inserted in to patient mouth

Fig-5: Maxillo mandibular relation mounted on semi
adjustable articulator

   Final trial was taken in the patient’s mouth to
verify vertical relations, centric relations etc.
Then acrylisation was carried out to fabricate       Fig-7: Postoperative patient photo
upper acrylic removable partial and lower
complete dentures were fabricated. Laboratory        month intervals to make necessary adjustments
remounting was done to remove interference in        and monitor the oral hygiene.
centric and protrusive movements. Dentures                  The patient adapted well to the dentures,
were then inserted in to patient’s mouth and         and the treatment improved his masticatory and
checked for high points [Figure-6].                  speech functions, aesthetics, and established a
       The dentures were planned to remake at        more favorable plane of occlusion. The patient’s
intervals to allow for the eruption of the           social confidence also improved significantly as
permanent teeth. The patient and his mother          a result of treatment. The patient will be
were instructed to handle the dentures carefully     evaluated regularly and a more definitive
and to have the patient wear them at all times       treatment will be performed once the growth of
except while tooth brushing and sleeping. The        child is complete.
dentures were adjusted carefully and regular
recall appointments were scheduled at two-           Discussion

                                                           ED is usually a difficult condition to treat
                                                     with prosthodontic restorations because of the
                                                     typical oral deficiencies and the young age
                                                     when they are evaluated for treatment.
                                                     Therefore, when treating a child with ED, it is
                                                     important to motivate both the child as well as

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his parents prior to the treatment and to work       bone and also the growing age might cause
with them to ensure their compliance (15).           problems in placing endosseous implants in
       Literature shows children rejected by their   growing children is not recommended as a
peer groups are more likely to become                routine practice (18, 19). The short-term
aggressive, delinquent, and may experience           survival data reported by Guckes et al (20)
mental health problems in adulthood (16).            suggests that it is possible to successfully place
Therefore, successful treatment of the present       dental implants in male and female patients of
case can be expected to assist the patient both      different ages with ED and congenitally missing
physically and psychologically. The boy’s            teeth. However, they stated that a careful
attitude, self confidence, and peer group            evaluation of each patient is necessary to
interaction showed signs of significant              determine the bone volume available for implant
improvement during treatment. Clinical reports       placement (20).
have stated the importance of prosthetic dental             Due to the boy’s young age, on-going
treatment in patients with anodontia or              development of the jaws and insufficient
hypodontia for physiological and psychosocial        quantity of alveolar bone, endosseous implant
reasons (10, 13).                                    placements were not possible. The bone height
       The successful use of any prosthesis is       and width was not sufficient for implant
dependent       on    the      cooperation    and    insertion. Application of removable dentures
communication between the dental team and the        may be the only restorative option for this
patient and his parents. For example, great care     patient. Implant retained prosthesis can be
will need to be taken to maintain the boy’s oral     planned after the completion of growth.
hygiene in order to benefit from the long-term
treatment plan. In addition, the boy and his         Summary:
parents must be educated and motivated about                Young patients with ED need to be
the dental problems related to his genetic and       evaluated early by a dental professional to
psychological conditions (15, 16).                   determine the oral ramifications of the
       Treatment of the ED patient generally         condition. When indicated, appropriate care
includes a removable and/or fixed partial            needs to be rendered throughout the child’s
denture, a complete denture prosthesis               growth cycle to maintain oral functions as well
(overlaying affected teeth if the vertical           as to address the aesthetic needs of the patient.
dimension of occlusion permits), and an implant      This clinical report demonstrates that removable
retained prosthesis when indicated (17). These       partial dentures associated with direct composite
treatment modalities can be used individually or     restorations can be a reversible and economical
in combination to provide an optimal result. The     method of treatment for young ED patients.
proper sequencing of treatment is important to
achieve the desired function and aesthetic           References:
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                                                       Source of Support: Nil
                                                       Conflict of Interest: Not Declared

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