Ectodermal Dysplasia In Children:
Clinical Study And Oral Rehabilitation
Omar A.S. EL-Meligy*
The aim of the present study was (1) To describe the dental findings in
children with ectodermal dysplasia (ED), (2) To evaluate the restorative and
prosthodontic treatment that was provided to these children, (3) To evaluate
the related oral health quality of life (QOL) measures.
Patients and Methods:
Eight children suffering from ED were presented to the outpatient
clinic of the Pediatric Dentistry Department for oral rehabilitation. They were
subjected to dental clinical and radiographic evaluation. Restorative and
prosthodontic treatment was provided to improve speech, masticatory
function and facial esthetics of these children. The children were recalled for
clinical and radiographic evaluations after 1 week (baseline), 6, 12 and 18
months. The oral health-related QOL measures were evaluated before and 4
weeks after oral rehabilitation.
Dental clinical examination and panoramic radiographs revealed
a mean of 12.6 missing permanent teeth in the ED group. Maxillary central
incisors, maxillary first molars, maxillary canines and mandibular first
molars showed lowest percentage of absence (were most stable). Asymmetry
in absent teeth was present in almost all tooth types. Clinical follow-up after
6, 12 and 18 months revealed 100% success rate for amalgam fillings,
formocresol pulpotomies and stainless steel crowns. Radiographic follow-up
of formocresol pulpotomies after 6, 12 and 18 months showed 100% success
rate. For composite strip crowns and removable partial overdentures,
a clinical success rate of 100% was found after 6 and 12 months. This
dropped to 87.5% after 18 months.
Lecturer in Pediatric Dentistry, Faculty of Dentistry, Alexandria University
From the present study, it can be concluded that:
(1) Clinical dental and radiographic examinations are important in
diagnosing cases of ED. The number of missing teeth, abnormal
crown forms, asymmetry in missing units are characteristic in cases
of uncertain diagnosis.
(2) A clinical success rate of 87.5% for composite strip crowns and
removable partial overdentures in comparison to 100% for amalgam
fillings, formocresol pulpotomies and stainless steel crowns was
recorded at the end of the study.
(3) Early dental intervention improved the patients’ appearance, speech
and masticatory function.
(4) Oral rehabilitation was successful, establishing the function and
improving the social living and QOL of these children.
Ectodermal dysplasia (ED) is a congenital syndrome characterized
chiefly by abnormality of tissues of ectodermal origin, namely skin, nails,
hair and teeth.(1,2) ED is a relatively rare disorder having a related cases
variation in frequency of 1 : 10,000 up to 1 : 100,000 of those born alive. (3)
There are more than 150 different variants of ED.(4) ED may be
inherited by all Mendelian means of inheritance.(5) Oral findings can be
significant and include multiple tooth abnormalities including anadontia and
hypodontia with associated lack of normal alveolar ridge development. (6)
Other external signs are decreased or absent sweat glands, sparse fine or
coarse curly hair, and abnormally developed nails.(7) These findings
differentiate simple hypodontia from those patients with ED. (8) ED has
a distinctive facies with prominence of the forehead, depressed nasal bridge,
prominent lips, periorbital wrinkling, pigmentation and diminished or absent
subcutaneous fat.(9) Additionally, dysphonia and hoarseness of voice may be
determined due to atrophic pharyngeal and laryngeal mucosa.(10)
The condition includes two major types, hypohidrotic, in which the
sweat glands are absent or decreased significantly, and hidrotic, in which the
sweat glands are normal.(11) The hypohidrotic is the more severe type and is
associated with sensitivity to heat and frequent high fevers.(12) This type is
also thought to have more associated dental defects.(13) Hypohidrotic ED is an
X-linked recessive trait and it is the most frequently reported ED syndrome. (6)
It affects one to seven individuals per 10,000 live births.(14)
Hypodontia is the second most frequently reported sign in ED (80% of
cases).(5) The pattern of missing teeth in severe hypodontia has been reported
in previous studies.(15-17) Although Schalk-van der Weide and colleagues(15)
found no association between congenitally missing teeth and ectodermal
symptoms in a study comparing dental findings in 167 patients with
oligondontia with 135 normal controls, they suggested that an individual with
the most stable teeth missing, or with several teeth missing, should be
examined carefully for signs of ED. In another study, individuals with
hypodontia associated with a syndrome were found to be more likely to be
missing more teeth than individuals with hypodontia not associated with
a syndrome.(16) In a sample of patients with X-linked hypohidrotic ED,
Crawford(17) found more teeth missing in males and a difference in the pattern
of teeth missing between males and females.
The physiologic and psychological value of restorative and prosthetic
dental treatment in patients with ED has been emphasized.(18) Unfortunately,
because of the reduced number of teeth, patients often present with problems
related to occlusal vertical dimension and esthetics.(10) Furthermore, as
a result of the limited tooth structure remaining, these patients may require
extensive restorative and prosthetic treatment to regain appropriate function,
esthetics and comfort.(19) Such needs can create a challenging treatment
Removable prosthodontics is the most frequently reported treatment
modality for the dental management of ED.(20-23) When there are teeth present
for support, overdentures are a desirable treatment option for these
patients.(24-26) Crum(27) provided an excellent overview of the advantages of
conventional overdentures as opposed to complete dentures. One important
advantage is that overdentures preserve alveolar bone, which is imperative in
these patients because the alveolar ridges are vital for prosthesis support. (19)
The use of removable partial overdentures is a reversible treatment that can
significantly improve function and esthetics without jeopardizing
compromised dentitions.(28) The overdenture is a prosthesis that covers and is
partially supported by natural teeth, tooth roots, or dental implants. (18) It can
be used to effectively treat patients with a reduced number of teeth.(29) In
childhood, a removable partial denture or overdenture is often the treatment
of choice because of the need to easily modify the intraoral prosthesis during
rapid growth periods.(30)
Oral rehabilitation of children with ED is recommended early to
improve both the sagittal and vertical skeletal relationship during craniofacial
growth and development(14), as well as to provide improvements in esthetics,
speech, and masticatory efficiency.(31) It is one of the possible means to
improve the social living and quality of life of the these children.(32)
Aim of the Work
The aim of the present study was (1) To describe the dental findings in
children with ectodermal dysplasia, (2) To evaluate the restorative and
prosthodontic treatment that was provided to these children, (3) To evaluate
the related oral health quality of life measures.
Patients and Methods
This study comprised 8 subjects from 4 families who suffered from
ectodermal dysplasia. Four children were presented to the outpatient clinic of
the Pediatric Dentistry Department, Faculty of Dentistry, Alexandria
University, complaining mainly of hypodontia and the other four children
were referred from the Department of Pediatrics, Faculty of Medicine,
Alexandria University because of hypodontia. Their ages ranged from 4-12
A- Dental evaluation:
For each child, clinical dental examination was done to assess the
degree of hypodontia, diagnose abnormal crown form, assess the asymmetry
in missing tooth number.
Panoramic radiographs were obtained for all the subjects to determine
the exact number of erupted and unerupted developing teeth together with
abnormal crown form and confirm clinical examination.
Teeth were considered present if at least one of any tooth type was
present or was reported to have been extracted. Developing tooth buds were
also considered when present. The radiographs were examined using
a standard radiographic viewing box.
B- Oral rehabilitation procedures:
Restorative treatment provided to those children included amalgam
fillings (Tytin, Kerr Manufacturing Co., MI) for restoration of occlusal and
proximal caries, formocresol pulpotomies (Buckley’s Sultan Chemists Inc.
Englewood, NJ, U.S.A) for treatment of primary teeth with vital carious pulp
exposures, stainless steel crowns (3M-ESPE Dental products) for restoration
of pulpotomized primary molars and composite strip crowns (3M-ESPE
Dental products) for cosmetic modification of conically shaped anterior teeth.
Following local or general anaesthesia, the restorative materials were
placed according to manufacturer’s instructions in traditional cavities or
crown preparations using standard armamentarium.(33)
Regarding composite strip crowns, the clinical technique and
procedure involves the placement of a feather edged finish line in enamel at
the gingival margin. The preparations were examined to be sure that no
undercuts were present. The dentition was placed into occlusion to ensure
there was a minimum of 2mm of clearance for the crown fabrication. After
teeth preparation, strip crowns were fitted. A gel etching agent was placed for
15 seconds and rinsed. A bonding agent and resin composite restorative
(Z100 Restorative, 3M-ESPE Dental Products) were used according to the
The children were recalled for clinical and radiographic evaluations
after 1 week (baseline), 6, 12 and 18 months.
The quality of the restorations was assessed using a modified Cvar and
Clinical success of pulpotomized primary teeth was judged using the
following criteria:(36) (1) No pain on percussion, (2) No abscess or fistulation,
(3) No pathologic tooth mobility. Radiographic success was judged using the
following criteria:(36) (1) Presence of normal periodontal ligament space, (2)
Absence of pathologic root resorption, (3) No canal calcification or
Failure of stainless steel crowns was judged using the following
criteria:(37) (1) Occlusal crown perforation (2) Crown loss as a result of
cement wash out.
Prosthodontic treatment provided to those children included acrylic
removable partial overdentures to replace congenitally missing teeth and to
restore function and esthetics. Preliminary impressions were made with
alginate (Bayer Dental, Germany). Custom acrylic trays were fabricated and
were used to make final alginate impressions. Maxillomandibular records
were made, and the casts were mounted in a semi adjustable articulator. The
artificial teeth were arranged in wax for trial evaluation. The occlusion and
position of the prosthetic teeth were evaluated intraorally and the necessary
corrections were made before processing the dentures. Lingualized occlusion
was used to reduce lateral forces and to create an intercuspal contact area
with freedom of movement.(38,39) The lingual cusps of the maxillary posterior
teeth contacted the fossae of the mandibular teeth to prevent lateral
interference. After processing, the maxillary and mandibular overdentures
were inserted with minor occlusal adjustments. The patients were given
instructions on adequate oral hygiene and denture care.(18)
At every recall, abutment teeth and removable partial over dentures
were assessed according to the following standard method: (40) (1) Abutment
teeth (Displacement of the prosthesis, fracture of the prosthesis, tooth fracture
and/or missing tooth: yes or no), (2) Retainers (Fracture and/or deformation:
yes or no), (3) Connectors (Fracture and/or deformation: yes or no), (4)
Denture bases (Fracture and/or deformation: yes or no), (5) Artificial teeth
(Fracture and/or displacement: yes or no).
C- Oral health-related quality of life (QOL) measures:
Oral health-related QOL measures were evaluated before the dental
treatment was started (baseline assessment) and 4 weeks after the completion
of dental treatment (follow-up assessment).
All children with ED baseline and follow-up surveys were conducted
in face to face interviews. This face to face format was chosen because of the
young age of the children.
The children’s oral health-related QOL was measured with the
Michigan Oral Health-related QOL Scale-Child Version.(41) This scale was
originally developed as a multidimensional measure of oral health-related
QOL in children 4 years of age and older. The original scale consisted of
7 items and covered 3 dimensions, namely pain/discomfort (Do your teeth
hurt you now? Did your teeth hurt you in the last days? Do your teeth hurt
when you eat something hot or cold?), functioning (Is it difficult for you to
chew? Is it difficult for you to bite), and psychological aspects (Are you
happy with your teeth? Do you have a nice smile?)
This original scale was modified in order to assess the social aspect of
oral health-related QOL by adding 2 questions (Does a hurting tooth stop you
from playing? Do kids make fun of your teeth?)(42)
Data was collected, tabulated and the results were statistically
I- Dental Findings:
The results of the present study were focused only on permanent
erupted and unerupted teeth. The third molars were not included in any of the
calculations. Deciduous teeth were difficult to assess since the history was
not always reliable. The patients were sometimes unable to verify the
presence of absence of some primary units.
From the clinical examinations, models and panoramic radiographs, it
was found that the mean number of missing teeth in the study group was
Percentage tooth absence is shown in Figure (1). In the lower arch, the
highest percentage absence was found to be 94% for the central incisors. The
lowest percentage was 25% scored for the canines and first molars. The
lateral incisors, second bicuspids, second molars and first bicuspids scored
a percentage absence of 75, 56, 44 and 38% respectively.In the upper arch,
the highest percentage absence was 88% scored for the lateral incisors, while
the lowest was 13% scored for both central incisors and first molars.
Percentage absence of 50, 44, 38 and 31% were recorded for second
bicuspids, second molars, first bicuspids and canines respectively.
When comparing upper and lower arches, a percentage teeth absence
of 39.2 for the upper arch was recorded in comparison to 50.8% for the lower
arch. There was no statistically significant difference found between the
2 arches, using the chi-square test (X2 = 3.047, P > 0.05).
Abnormal crown form is shown in Figure (2). A clinically abnormal
crown form was observed more frequently in the maxillary central incisor
area, scoring 85%. The maxillary first molars showed 10% abnormality in
crown form. In the lower arch, the lateral incisors and first molars showed
20% abnormality in crown form. The morphology of the remaining present
tooth types was favourable. Radiographically, taurodontism (elongated pulp
chambers and short root canals) of the maxillary and mandibular second
primary molars was detected in all cases.
Percentage asymmetry of permanent missing tooth number is shown in
Figure (3). The highest percentage of asymmetry was 20% recorded for the
lower laterals and second bicuspids. The remaining teeth ranged between
5-10% in both upper and lower arches. Zero percentage was recorded for the
lower central incisors and upper first molars, indicating total symmetry.
100 94 8
40 38 38
1 2 3 4 5 6 7
Upper arch Lower arch
Figure (1): % Permanent tooth absence
0 0 0 0 0 0 0 0 0 0
1 2 3 4 5 6 7
Upper arch Lower arch
Figure (2): Abnormal crown form
10 10 10 10
5 5 5 5 5 5
1 2 3 4 5 6 7
Upper arch Lower arch
Figure (3): Asymmetry of permanent missing tooth number
II- Oral rehabilitation evaluation:
The clinical study consisted of 8 children with ectodermal dysplasia
that needed oral rehabilitation. The mean age was 7.5 years ± 2.4. They were
4 females and 4 males. Restorative treatment that included 24 amalgam
fillings, 16 formocresol pulpotomies, 16 stainless steel crowns and 32
composite strip crowns was provided to those children. Prosthodontic
treatment included 16 acrylic removable partial overdentures (8 maxillary
and 8 mandibular overdentures) was also provided to those children. The
children were recalled for clinical and radiographic evaluations after 1 week
(baseline), 6, 12 and 18 months.
Table (I) shows the number and percentage of successful restorative
and prosthodontic procedures at each follow-up period.
An overall clinical success rate of 100% was reported after 6, 12 and
18 months for amalgam fillings, formocresol pulpotomies and stainless steel
crowns. Radiographic success rate of 100% was reported after 6, 12 and 18
months for formocresol pulpotomies. Composite strip crowns and removable
partial overdentures showed a clinical success rate of 100% after 6 and 12
months. After 18 months, 4 maxillary composite strip crowns showed a large
loss of crown material clinically, lowering the success rate to 87.5%.
Concerning removable partial overdentures, failure was observed in
2 dentures. A maxillary denture had fracture of its denture base, while
a mandibular denture had retainers failure in the form of clasps fracture,
reducing the clinical success rate to 87.5%.
Figures 4 and 5 show preoperative and 1 week postoperative
panoramic radiographs of a successfully treated 5 year old female patient
with ectodermal dysplasia.
Figures 6 through 9 show pretreatment, 6, 12 and 18 months post-
treatment views of the same female patient.
Table (I): Number and percentage of successful dental procedures at each
Follow up Baseline 6 months 12 months 18 months
Procedures No % No % No % No %
Amalgam 24 100% 24 100% 24 100% 24 100%
Pulpotomy 16 100% 16 100% 16 100% 16 100%
St. st. crown 16 100% 16 100% 16 100% 16 100%
32 100% 32 100% 32 100% 28 87.5%
16 100% 16 100% 16 100% 14 87.5%
Figure (4): Preoperative panoramic radiograph showing the pattern of
Figure (5): 1 week postoperative panoramic radiograph showing successful
Figure (6): Preoperative view showing hypodontia and conically shaped
Figure (7): Six months postoperative anterior view showing composite strip
crowns, stainless steel crowns and removable partial overdentures.
Figure (8): Twelve months postoperative anterior view of the same patient
showing successful restorative and prosthodontic treatment.
Figure (9): Eighteen months postoperative anterior view of the same patient
showing successful dental treatment.
III- Oral health-related QOL outcomes:
Table (II) shows the percentages of agreement with items that indicate
negative oral health-related QOL at baseline and 4 weeks after oral
The responses to the questions “Are you happy with your teeth? “ and
“Do you have a nice smile?” were reversed to achieve unidirectional scores.
The no response to these 2 questions indicates negative QOL, while the yes
response indicates negative QOL for the other 7 questions.
The results show that children with ED who received dental treatment
has a significantly improved oral health-related QOL at the follow-up
assessment when compared with their baseline measurement as measured
with the children’s self-ratings of oral health-related QOL (X2 = 17.32,
P < 0.05).
Table (II): Children’s percentages of agreement with items that indicated
negative oral health-related QOL at baseline and 4 weeks after oral
Do your teeth hurt you now? 25% 0%
Do your teeth hurt you in the last days? 25% 0%
Do your teeth hurt you when you eat something hot or
Is it difficult for you to chew? 75% 25%
Is it difficult for you to bite? 75% 25%
Are you happy with your teeth? (reversed-no)* 100% 0%
Do you have a nice smile? (reversed-no)* 100% 0%
Does a hurting tooth stop you from playing? 12.5% 0%
Do kids make fun of your teeth? 75% 25%
* The responses to the questions “Are you happy with your teeth?” and “Do
you have a nice smile?” were reversed to achieve unidirectional scores.
Ectodermal dysplasia is the heterogenous group of disorders involving
one or more ectodermal structures.(10)
Silverman and Ackerman(43), showed that when there was hypodontia
in normal subjects, 80% of those affected had one or more missing teeth and
only 5% had 5 or more missing teeth. A mean of 12.6 missing teeth in
children affected by ectodermal dysplasia in the present study, which is a
markedly different from the values reported in the dental literature for normal
individuals can provide additional information in cases of uncertain
The results of this study support those of other studies in that the
maxillary central incisors, maxillary first molars, maxillary canines and
mandibular first molars are the most conserved teeth in severe hypodontia,
that is, they were the most often present.(16,45,46,47) (Figure 1)
The maxillary lateral incisor in the ED group followed the normal
distribution pattern of congenitally missing teeth in normal subjects. (43) On
the other hand, the mandibular central and lateral incisors scored a percentage
absence of 94 and 75% respectively (Figure 1). This finding is in accordance
with the results reported by Redapth and Winter(8), but in contrast with the
findings of Abdel Rahman and Wahba(48), who reported that the lower central
and lateral incisors were missing in 100% of the cases. The second bicuspid
recorded a higher percentage absence in comparison to the first bicuspid in
both upper and lower arches. This is a typical pattern since the prevalence is
higher for the lower second bicuspid in the normal population(44).
The abnormality in crown forms is one of the features of ectodermal
dysplasia.(44,49) The fact that the lower central and upper lateral incisors
showed the maximum frequency of missing teeth did not allow for evaluation
of their abnormal crown forms. The canine is already slightly conical, so
a variation in crown form was not substantially detectable. The highest
tendency of abnormal crown form was focused in the upper incisor region
(85%) (Figure 2). This finding coincides with Crawford et al (17) and Kargul et
al.(49) This was followed by the lower lateral incisors and lower first molars
(20%) then the upper first molars (10%). The remaining tooth types were
more or less favourable in shape. Radiographically, taurodontism of the
maxillary and mandibular second primary molars was observed in all cases.
This observation is consistent with previous studies by Vierucci et al(50) and
Kargul et al(49) who reported taurodontism in primary molars as a result of
disruption during morphodifferentiation stage of tooth development.
Livshits and Kobliansky(51), mentioned that findings of increased
fluctuating asymmetry in cases of congenital malformations and genetic
disorders suggest a defective stabilization that resulted in the defect together
with increased fluctuating asymmetry. Asymmetry in missing teeth was
noticed in almost all cases of ED (Figure 3). This ranged from 0% for the
upper first molars to 20% for lower laterals and second bicuspids. The 0%
recorded for the upper first permanent molar substantiates the fact that it is
the most symmetrical tooth among all units.
Dental defects associated with ED can cause severe esthetic and
functional problems. Various studies have shown that when dental changes
exist, they cause a greater disability than the other ectodermal
manifestations.(45) Psychological effects of the disorder on the child are
adverse to the extent of regarding these patients disabled.(48,52) Appearance
can be greatly enhanced by providing an esthetically acceptable dentition and
restoring the vertical dimension. This is especially important when the child
is subjected to peer evaluation, the child who appears different to his peers
may suffer ridicule and rejection and may have difficulty socializing. Early
identification and management of the dental aspects of the disorder are
important to help these patients lead a normal life and overcome their
Oral rehabilitation is often difficult for children with ectodermal
dysplasia.(53) In the present study the affected children required extensive
dental treatment in order to restore their appearance and function. Although
the study was carried out on a relatively small sample of patients (8) for
a relatively short follow-up period (18 months), the results are remarkably
consistent at all 3 observation periods.
Restorative treatment provided to those children included 24 amalgam
restorations, 16 formocresol pulpotomies, 16 stainless steel crowns and 32
composite strip crowns. In the present study, an overall clinical success rate
of 100% was reported after 6, 12 and 18 months for amalgam restorations
and stainless steel crowns (Table I). This is in accordance with the results
reported by Van den Steen and Bottenberg(54) who concluded that amalgam
restorations and stainless steel crowns showed a high success rate in a 4 year
old child with nursing bottle caries and ED.
Formocresol pulpotomies showed a clinical and radiographic success
rate of 100% after 6, 12 and 18 months (Table I). In the present study, most
of the pulpotomies were performed in patients who were treated under
general anesthesia. General anesthesia allows treatment to be rendered under
optimal conditions, ensuring ideal outcomes.(37) In addition, all pulpotomized
teeth were restored with a stainless steel crown, thereby increasing their
success rate. In the present study, all pulpotomized teeth showed absence of
calcific degeneration. This was probably due to the relatively short period of
this study (18 months). Many studies showed that pulp calcification in teeth
treated with formocresol was observed after 24 months.(55,56)
Composite strip crowns were used in this study for cosmetic
modification of conically shaped anterior teeth because of their superior
esthetics and ease of repair if the crown should chip or fracture. Several
studies have demonstrated that composite strip crowns are most esthetic of all
the available restorations for the treatment of decayed or malformed primary
incisors.(52,57,58,59) In the present study, composite strip crowns showed
a clinical success rate of 100% after 6 and 12 months. After 18 months,
4 maxillary composite strip crowns showed a large loss of crown material
clinically, lowering the success rate to 87.5% (Table I). The reason of failure
was probably due to the limitations and technical difficulties of fabricating
composite strip crowns on malformed, small sized teeth with thin enamel and
large pulps. These results are similar to those of Kupietzky and Waggoner (60)
who reported that only 12% of the restorations failed after 18 months due to
loss of some resin material, but none of the crowns were completely lost. In
contrast, Tate et al(61) reported only 49% success rate.
Prosthodontic treatment provided to the children of the present study
included 16 acrylic removable partial overdentures (8 maxillary and 8
mandibular overdentures). Multiple implant placement was not possible
because of the ongoing development of the jaws and insufficient bone.(62) In
addition, the bone height and width will not be sufficient for implant
insertion without advanced surgical approaches.(63) Also early implant
placement in a growing child may cause cosmetic problems, as the implants
act like ankylosed teeth.(63,64) Therefore, the use of removable partial
overdentures was the only restorative option in these patients. In addition,
overdentures are a simple, conservative and reversible non-surgical
alternative for those children.(28)
In the present study, removable partial overdentures showed a clinical
success rate of 100% after 6 and 12 months. After 18 months, 2 overdentures
(one maxillary and one mandibular) failed, reducing the clinical success rate
to 87.5% (Table I). The maxillary overdenture had fracture of its denture base
due to careless handling of the denture, while the mandibular denture had
retainers fracture resulting from inappropriate insertion and removal of the
denture by holding it from the clasps. Saito et al(65) reported complications
and failures in removable partial dentures similar to those of the present
In ED patients, dryness of the oral mucosa and the underdeveloped
maxillary tuberosities and alveolar ridges are problematic factors for
resistance and stability of dentures.(66) When planning dentures in those
patients, care should been taken to obtain a wide distribution of occlusal load
fully extending the denture base.(31) For removable partial dentures, the
occlusion should be in harmony with the patient’s occlusion, generally an
occlusal scheme utilizing linear occlusal contact is recommended to preserve
the existing teeth and to create freedom of movement.(38) For optimal esthetic
results, denture teeth should be age appropriate.(67)
In the present study, children with ED who received dental treatment
reported a significantly improved oral health-related QOL 4 weeks after the
treatment as compared to their responses before the treatment. The results of
this study additionally showed that children as young as 4 years of age are
able to answer questions about their own oral health in a valid fashion.
Several studies had suggested that children 4 years of age or older can
participate the questionnaire studies and provide information about their pain
experiences.(68,69,70) Additionally, results of a study with 203 Hispanic
migrant worker children in Michigan (83 girls/120 boys; age range = 4 to 16
years of age; mean age = 8.18 years) showed that 183 children could answer
questions similar to those of the present study.(71)
Results of the present study showed that the largest and most
satisfactory change observed after the accomplishment of oral rehabilitation
was from the psychological and pain/discomfort aspects (Table II). Only 2
children had some difficulties in chewing and biting because they did not
follow the instructions that were given to them on how to use their new
dentures. Another observation point was that 2 children after oral
rehabilitation, still had difficulty socializing because other children in school
were making fun of their teeth. After 2 months, the class mates negative
attitude towards those 2 specific children was better as they got used to their
In summary, this study showed that oral rehabilitation significantly
improved the quality of life of these children and that children’s reports of
their own oral health-related QOL are an important diagnostic tool when
assessing children’s needs for dental care.
Further studies of children with ED with a larger sample size and
longer follow-up periods are recommended to evaluate dental treatment and
oral health-related QOL measures.
From the present study, it can be concluded that:
1- Clinical dental and radiographic examinations are important in diagnosing
cases of ED. The number of missing teeth, abnormal crown forms,
asymmetry in missing units are characteristic in cases of uncertain
2- A clinical success rate of 87.5% for composite strip crowns and
removable partial overdentures in comparison to 100% for amalgam
fillings, formocresol pulpotomies and stainless steel crowns was recorded
at the end of the study.
3- Early dental intervention improved the patients’ appearance, speech and
4- Oral rehabilitation was successful, establishing the function and
improving the social living and quality of life of these children.
1- Gorlin RJ, Cohen MM, Hennekam RCM, editors. Syndromes of the head
and neck. 4th ed. New York. Oxford University Press; 2001. p.540-5
2- Bergsma D, editor. Birth defects compendium. 2nd ed. New York: Alan
Liss; 1979. p.92.
3- Abadi B, Herren C. Clinical treatment of ectodermal dysplasia: a case
report . Quintessence Int 2001; 32: 743-5.
4- Pinherio M, Freire-Maia N. Ectodermal dysplasias: a clinical
classification and a causal review. Am J Med Genet 1994; 53: 153-62.
5- Friere-Maia N, Pinheiro M: Ectodermal Dysplasia: A clinical and genetic
study. New York: Alan R. Liss Inc 1984. 187-95.
6- Levin LS: Dental and oral abnormalities in selected ectodermal dysplasia
syndromes. Birth defects Orig Artic Ser 1988; 24: 205-227.
7- Anton-Lamprecht I, Schleiermacher E, Wolf M: Autosomal recessive
anhidrotic ectodermal dysplasia: report of a case and discrimination of
diagnostic features. Birth Defects Orig Artic Ser 1988; 24: 183-95.
8- Redpath TH, Winter GB: Autosomal dominant ectodermal dysplasia with
significant dental defects. Br Dent J 1969; 4: 123-128.
9- Clark A: Hypohydrotic ectodermal dysplasia. J Med Genet 1987; 24:
10- Itthagarun A, King NM. Ectodermal dysplasia: A review and case report.
Quintessence Int 1997; 28: 595-602.
11- Buyse ML. Birth defects encyclopedia. St. Louis: Blackwell Publishing;
1990. p. 597-8.
12- Ramchander V. Anhidrotic ectodermal dysplasia in an infant presenting
with pyrexia of unknown origin. Clin Pediatr 1978; 17: 51-54.
13- Guckes A, Roberts MW, McCarthy GR. Pattern of permanent teeth
present in individuals with ectodermal dysplasia and severe hypodontia
suggests treatment with dental implants. Pediatr Dent 1998; 20: 278-280.
14- Buyse ML. Birth defects encyclopedia. Cambridge: Blackwell Scientific
Publications, 1990; 596-605.
15- Schalk-van der Weide Y, Beemer FA, Faber JA, Bosoman F:
Symptomatology of patients with oligodontia. J Oral Rehabil 1994; 21:
16- Schalk-van der Weide Y, Steen WH, Bosoman F: Distribution of missing
teeth and tooth morphology in patients with oligodontia. ASDC J Dent
Child 1992; 59: 133-140.
17- Crawford PJ, Aldred MJ, Clorke A: Clinical and radiographic dental
findings in X linked hypohidrotic ectodermal dysplasia. J Med Genet
1991; 28: 181-185.
18- Pavarina AC, Machado AL, Vergani CE, Giampolo ET. Overlay
removable partial dentures for a patient with ectodermal dysplasia:
a clinical report. J Prosthet Dent 2001; 86: 574-577.
19- Dhanrajani PJ, Jiffry AO. Management of Ectodermal Dysplasia:
A literature Review. Dent Update 1998; 25: 73-75.
20- Wraith Ed. True ectodermal dysplasia. Dent Update 1983; 662-668.
21- Show RM. Prosthetic management of hypohydontic ectodermal dysplasia
with anadontia: case report. Aust Dent J 1990; 35: 113-116.
22- Boj JR, Von Arx JD, Cortada M, Jimenez A, Golobart J. Dentures for
a 3-year old child with ectodermal dysplasia. Am J Dent 1993; 6:
23- Tape MW, Tye E. Ectodermal dysplasia: literature review and a case
report. Compendium 1995; 16: 524-528.
24- Nowak AJ. Dental treatment for patients with ectodermal dysplasias.
Birth Defects 1988; 24: 243-259.
25- Renner RP, Kleinerman V. Overdenture techniques in the management of
oligodontia: a case report. Quintessence Int 1980; 4: 57-65.
26- Album MM. Ectodermal dysplasia: a crown and bridge approach in
treatment technique. J Int Assoc Dent Child 1980; 11: 53-61.
27- Crum RJ. Rationale for the retention of teeth for overdentures. In: Brewar
AA, Morrow RM, editors. Overdentures. 2nd ed. St. Louis: CV Mosby,
28- Graser GN, Rogoff GS. Removable partial overdentures for special
patients. Dent Clin North Am 1990; 34: 741-758.
29- Windchy AM, Morris JC. An alternative treatment with the overlay
removable partial denture: a clinical report. J Prosthet Dent 1998; 79:
30- Hickey AJ, Vergo TJ Jr. Prosthetic treatments for patients with
ectodermal dysplasia. J Prosthet Dent 2001; 86: 364-368.
31- Tarjan I, Gabris K, Rozsa N. Early prosthetic treatment of patients with
ectodermal dysplasia. A clinical report. J Prosthet Dent 2005; 93:
32- Valle DD, Chevitarese ABA, Maia LC, Farinhors JA. Alternative
rehabilitation treatment for a patient with ectodermal dysplasia. J Clin
Pediatr Dent 2004; 28(2): 103-106.
33- Andlaw RJ, Rock WP. A Manual of Paedodontics. 2 nd ed, Edinburgh:
Churchill Livingstone, 1987, pp 61-112.
34- Kupietzky A. Bonded resin composite strip crowns for primary incisors.
Pediatr Dent 2002; 24: 145-148.
35- Cvar JF, Ryge G: Criteria for the clinical evaluation of dental restorative
materials. USPHS pub. No. 790, P244. SanFrancisco: US government
printing office, 1971.
36- McDonald RE, Arevy DR, Dean JA. Dentistry for the child and
adolescent, ed8, Mosby Co, 2004. Treatment of deep caries, vital pulp
exposure and pulpless teeth. 389-412.
37- Ng MW, Tate AR, Needleman HL, Acs G. The influence of medical
history on restorative procedure failure rates following dental
rehabilitation. Pediatr Dent 2001; 23: 487-490.
38- Bonilla Ed, Guerra L, Luna O. Overdenture prosthesis for oral
rehabilitation of hypohidrotic ectodermal dysplasia: a case report.
Quintessence Int 1997; 28: 657-65.
39- Pigno MA, Blackman RB, Cronin RJ Jr, Cavazos E. Prosthodontic
management of ectodermal dysplasia: a review of the literature. J Prosthet
Dent 1996; 76: 541-545.
40- Saito M, Notani K, Miura Y, Kanasaki T. Complications and failures in
removable partial dentures: a clinical evaluation. J Oral Rehabilitation
2002; 29: 627-633.
41- Inglehart MR, Bagramian RA. Introduction and overview. In: Inglehart
MR, Bagramian RA, eds. Oral Health-related Quality of Life. Chicago,
III: Quintessence Publishing Company; 2002.
42- Filstrup SL, Briskie D, Da Fonseca M, Lawrence L, Wandera A, Inglehart
MR. Early Childhood Caries and Quality of Life: Child and Parent
Perspectives. Pediatr Dent 2003; 25: 431-440.
43- Silverman NE, Ackerman JL. Oligodontia: a study of its prevalence and
variation in 4032 children. J Dent Child 1979; 46: 470-477.
44- Broot AH. Dental anomalies of number, form and size: their prevalence in
British school children. J Int Assoc Dent Child 1974; 5: 35-53.
45- Nakata M, Koshiba H, Eto K, Nance W: A genetic study of anodontia in
X-linked hypodontic ectodermal dysplasia. Am J Hum Genet 1980; 32:
46- Tso MS, Crawford PJ, Miller J. Hypodontia, ectodermal dysplasia and
sweat pore counts. Br Dent J 1985; 158: 56-60.
47- Sweeny IP, Ferguson JW, Heggie AA, Lucas JO. Treatment outcomes for
adolescent ectodermal dysplasia patients treated with dental implants. Int
J Pediatr Dent 2005; 15: 241-248.
48- Abdel Rahman AA, Wahba NA. Genetic and dental study in patients with
ectodermal dysplasia. ADJ 1998: 23(1): 55-72.
49- Kargul B, Alcan T, Kabalay U, Atasu M. Hypohidrotic ectodermal
dysplasia: dental, clinical, genetic and dermatoglyphic findings of three
cases. J Clin Pediatr Dent 2001; 26(1): 5-12.
50- Vierucci S, Baccetti T, Tollaro I. Dental and craniofacial findings in
hypohidrotic ectodermal dysplasia during the primary dentition phase.
J Clin Pediatr Dent 1994; 18(4): 291-297.
51- Livishits G, Kobliansky E: Dermatoglyphic traits as possible workers of
developmental processes in humans. Amer J Med Genet 1987; 26:
52- Goepferd SJ, Carroll CE. Hypohidrotic ectodermal dysplasia: a unique
approach to esthetic and prosthetic management. JADA 1981; 102:
53- Vasan N. Management of ectodermal dysplasia in children: an overview.
Ann R Australas Coll Dent Surg 2000; 15: 218-222.
54- Van den steen E, Bottenberg P. Removable prosthesis in a 4-year old
child with nursing bottle caries and possible ectodermal dysplasia. Rev
Belge Med Dent 2004; 59: 89-93.
55- Hicks MJ, Barr ES, Flaitz CM. Formocresol pulpotomies in primary
molars: A radiographic study in pediatric dentistry J Pedod 1986; 10: 331.
56- Fuks AB, Holan G, Davis JM, Fidelman E. Ferric sulfate versus dilute
formocresol in pulpotomized primary molars: long term follow up.
Pediatr Dent 1997; 19: 327-330.
57- Ram D, Peretz B. Composite crown-form crowns for severely decayed
primary molars: a technique for restoring function and esthetics. J Clin
Pediatr Dent 2000; 24: 257-260.
58- Waggoner WF. Restoring primary anterior teeth. Pediatr Dent 2002; 24:
59- Lo Muzio L, Bucci P, Carile F, Riccitiello F, Scotti C, Coccia E, Rappelli
G. Prosthetic rehabilitation of a child affected from anhydrotic ectodermal
dysplasia: A case report. J Contemp Dent Pract 2005; 6(3): 120-126.
60- Kupietzky A, Waggoner WF. Parental satisfaction with bonded resin
composite strip crowns for primary incisors. Pediatr Dent 2004; 26:
61- Tate AR, Ng NW, Needleman HL, Acs G. Failure rates of restorative
procedures following dental rehabilitation under general anesthesia.
Pediatr Dent 2002; 24: 69-71.
62- Bryant SR. The effects of age, jaw site, and bone condition on oral
implant outcomes. Int J Prosthodont 1998; 11: 470-490.
63- Imirzalioglu P, Uchan S, Haydar SG. Surgical and Prosthodontic
treatment alternatives for children and adolescents with ectodermal
dysplasia: A clinical report. J Prosthet Dent 2002; 88: 569-572.
64- Zechver W, Bernhart T, Zauzak, Celar A, Watzek G. Multidimensional
osteodistraction for correction of implant malposition in edentulous
segments. Clin Oral Implants Res 2001; 12: 531-538.
65- Saito M, Notani K, Miura Y, Kawasaki T. Complications and failures in
removable partial dentures: a clinical evaluation. J Oral Rehab 2002; 29:
66- Show RM. Prosthetic management of hypohidrotic ectodermal dysplasia
with anadontia. Case report. Aust Dent J 1990; 35: 113-116.
67- Ramos V, Giebink DL, Fisher JG, Christensen LC. Complete denture for
a child with hypohidrotic ectodermal dysplasia: a clinical report.
J Prosthet Dent 1995; 74: 329-331.
68- Gaffney A, Dunne FA. Developmental aspects of children’s definitions of
pain. Pain 1986; 26: 105-117.
69- Parsons SK, Barlow SE, Levy SL, Supran SE, Kaplan SH. Health-related
quality of life in pediatric bone marrow transplant survivors: according to
whom? Int J Cancer Suppl 1999; 12: 46-51.
70- Robok G, Riley A, Forrest C. Elementary school-aged children’s reports
of their health: a cognitive interviewing study. Qual Life Res 2001; 10:
71- Watson Do, Inglehart MR, Bagramian RA. Oral health promotion for
under-represented minority children and parents. J Dent Res 2002; 79
(abstract # 500).