See

discussions, stats, and author profiles for this publication at: http://www.researchgate.net/publication/21294705

Osseointegrated implants in the oral

habilitation of a boy with ectodermal
dysplasia: A case report
ARTICLE in INTERNATIONAL DENTAL JOURNAL JULY 1991
Impact Factor: 1.26 Source: PubMed

CITATIONS

READS

40

17

6 AUTHORS, INCLUDING:
Gran Koch
Odontologiska Institutionen i Jnkping
156 PUBLICATIONS 3,761 CITATIONS
SEE PROFILE

Available from: Gran Koch

Retrieved on: 25 November 2015

Dental Implant Therapy for a Child with X-linked

Hypohidrotic Ectodermal Dysplasia
Three Decades of Managed Care
Birgitta Bergendal, DDS, PhDa/Krister Bjerklin, DDS, PhDb/
Tom Bergendal, DDS, PhDc/Gran Koch, DDS, PhDd
Purpose: The aim was to report on oral rehabilitation of a boy with X-linked hypohidrotic
ectodermal dysplasia (XLHED) and anodontia of the mandible between ages 3 and
33 years where treatment involved dental implants and oral care management by
a multidisciplinary team of specialists. Materials and Methods: This case history
report describes the clinical management of a boy born in 1979 with XLHED and
anodontia of the mandible. Two implants were successfully placed in the anterior
region of the mandible in 1985. Genetic analysis later verified the diagnosis by
confirming a mutation in the EDA gene. The case description was based on review
of the patient records and assessment of orofacial function. Results: The patient
had satisfactory orofacial appearance and function throughout his youth. He is still
caries-free at age 33 and has experienced only minor oral complications. Dental
management began at age 3, when he received a maxillary removable dental
prosthesis. At age 7, he received a mandibular implant-supported overdenture. After
two more implants in the mandible and orthodontic treatment in the maxilla, his oral
rehabilitation was completed at age 22 with maxillary tooth-supported and mandibular
implant-supported fixed dental prostheses. Regular follow-ups provided supervision
of oral hygiene, caries prevention, and prosthetic maintenance. Conclusion: This
long-term follow-up of a child with XLHED and anodontia in the mandible supports
the use of dental implants, with consideration given to the dense bone quality
associated with the diagnosis, to establish good orofacial function and appearance
from childhood onward. Int J Prosthodont 2015;28:348356. doi: 10.11607/ijp.4242

ental management of young children with mandibular anodontia is a major dental treatment
challenge. This case history report is a long-term follow-up of one of the first publications on a boy born
in 1979 with a phenotype of X-linked hypohidrotic
ectodermal dysplasia (XLHED) and anodontia of the
mandible. The subject successfully received two implants in the anterior region of the mandible in 1985.1
aHead,

National Oral Disability Centre for Rare Disorders, The

Institute for Postgraduate Dental Education, Jnkping, Sweden.
bAssociate Professor, Department for Orthodontics, The Institute
for Postgraduate Dental Education, Jnkping, Sweden.
cAssociate Professor, Department for Prosthetic Dentistry, The
Institute for Postgraduate Dental Education, Jnkping, Sweden.
dProfessor, Department for Paediatric Dentistry, The Institute for
Postgraduate Dental Education, Jnkping, Sweden.
Correspondence to: Dr Birgitta Bergendal, National Oral Disability
Centre for Rare Disorders, The Institute for Postgraduate Dental
Education, PO Box 1030, SE-551 11 Jnkping, Sweden.
Fax: +46 36 324612. Email: birgitta.bergendal@rjl.se
2015 by Quintessence Publishing Co Inc.

348

In the early 1980s, treatment with dental implants was

routine in the clinical care of elderly edentulous individuals, while the use of implants in young children
with ectodermal dysplasia (ED) and mandibular anodontia was emerging as a new application. The following decade included several case history reports
on favorable outcomes for implant treatment in children as young as 18 months with ED and mandibular
anodontia.25 During typical development, the crowns
of the mandibular permanent incisors are fully developed around 3 years of age. Once these teeth have
erupted, the transversal dimensions in the mandibular anterior area are assumed to be stable.6 When the
first case histories were discussed, lack of experience
contributed to several areas of concern, including the
timing of interventions, special risks attributed to the
diagnosis, choice of prosthodontic intervention, and
development and growth of the face and jaws.
In children with anodontia of the mandible, a minimum age of 6 years for implant surgery has been advocated, at which point the median suture is usually
closed.7,8 In 1998, a consensus conference on ED and

The International Journal of Prosthodontics

2015 BY QUINTESSENCE PUBLISHING CO, INC. PRINTING OF THIS DOCUMENT IS RESTRICTED TO PERSONAL USE ONLY.
NO PART MAY BE REPRODUCED OR TRANSMITTED IN ANY FORM WITHOUT WRITTEN PERMISSION FROM THE PUBLISHER.

Bergendal et al

dental treatment held in Jnkping, Sweden, established a care program for ED.9 The consensus report
presented clinical case histories, three of which were
young boys with hypohidrotic ED (HED) treated with
implants intended to support an overdenture in the
anodontic mandible. Case History Number 1the
subject of the present studyreceived two implants
at age 6 years 5 months that were successfully integrated after a prolonged healing period of 8 months.
However, in the two other case histories, osseointegration failed and discussions at the conference attributed these implant failures to circumstances other
than the syndrome per se.
Two decades later the Swedish patient support
group for ED reported that two other young children
with HED and mandibular anodontia, a 5-year-old boy
and a 12-year-old girl, had also lost implants before
loading. This encouraged a retrospective study on implant treatment in children up to age 16 in Sweden. In
all, five young children with HED and anodontia of the
mandible received treatment between 1985 and 2005.
Nine of 14 implants were lost before loading, an implant failure rate of 64%.10 Retrospective assessments
of the encountered operative difficulties led the oral
surgical operators to consider the small jaw size and
hard quality of the selected host bone sites.
Lesot et al found increased bone density of the jaw
in individuals with XLHED and concluded that this
skeletal phenotype is associated with the EDA mutation and confirms involvement of the EDA-NF-kB
signaling pathway in bone metabolism.11 Silthampitag
et al used micro-computed tomography imaging to
verify increased bone density in young adults with
HED compared to adult edentulous individuals without HED.12
There are few examples of long-term outcomes of
implants placed at a very young age. Therefore, our
aim was to report on the oral rehabilitation of a boy
with XLHED and anodontia of the mandible between
ages 3 and 33 years where treatment involved dental implants and oral care management by a multi
disciplinary specialist team.

Signs and Symptoms

The patient was diagnosed with XLHED at age 6
months, after being hospitalized for pneumonia and
failure to thrive. At 19 months, when he was referred
by doctors to the Department of Paediatric Dentistry
at the Institute for Postgraduate Dental Education in
Jnkping, his only teeth were two conical primary
incisors in the frontal maxilla. He also had a history
of reduced sweating capacity and so received a clinical diagnosis of XLHED. The typical diagnostic criteria became more evident over time as the phenotype

of a boy with XLHED emerged: severe tooth agenesis; light, sparse hair; decreased sweating capacity;
marked oral dryness; and a hoarse voice.13,14 In 2012,
a genetic examination of the patient and his daughter,
born in 2011, confirmed the diagnosis (EDA c.74dupG
in exon 1).

Treatment Planning
At age 3, a panoramic radiograph showed neither
primary nor permanent teeth in the mandible. In the
maxilla, the primary dentition consisted of two conically shaped central incisors and two late-developed
hybrid canines, and the permanent dentition of two
malformed central incisors and two first molars.
In 1982, a multidisciplinary team formed that included specialists in pediatric dentistry, orthodontics, oral
and maxillofacial surgery, and prosthetic dentistry,
supported by specialists in oral and maxillofacial radiology. Their discussions focused on the possibilities
of using dental implants in a young child and creating
a treatment plan that would continue from childhood
to adulthood. A dental team checked on the patient
regularly to minimize the risk of his developing dental
caries or inflammation of the gingiva or oral mucosa
and to establish and maintain good oral comfort. The
treatment goal was to put in place procedures for
optimal oral health care while the child grew up. All
interventions were planned in close cooperation with
the boy and his family.

Oral Rehabilitation
Table 1 and Figs 1 through 9 show the various treatments performed. The treatment team reshaped the
conical maxillary central incisors at age 2 and fit a
maxillary removable dental prosthesis (RDP) at age 3.
The team continually updated the prosthesis as the patient lost primary teeth and permanent teeth erupted
in the maxilla. At 6 years 5 months, the patient received
two implants (Brnemark system Standard implant
3.75 mm, 10 mm and 13 mm long) in the anterior region of the mandible under general anesthesia. There
were buccally exposed threads on the left implant
at the time of operation.10 After 8 months of healing,
the team connected custom-made gold ball abutments with the patient under conscious sedation, and
he received an overdenture made with silicone cuffs
(Molloplast B, Detax) as female parts. The treatment
with removable dentures in both jaws aimed to create
good appearance and oral function. The team closely
monitored the patients oral hygiene, using fluoride
varnish (Duraphat, Colgate) after professional tooth
cleaning, and recommended self-administered rinsing
with fluoride solution as supplementary prevention.

Volume 28, Number 4, 2015

349

2015 BY QUINTESSENCE PUBLISHING CO, INC. PRINTING OF THIS DOCUMENT IS RESTRICTED TO PERSONAL USE ONLY.
NO PART MAY BE REPRODUCED OR TRANSMITTED IN ANY FORM WITHOUT WRITTEN PERMISSION FROM THE PUBLISHER.

Dental Implant Therapy for a Child with X-linked Hypohidrotic Ectodermal Dysplasia

Table 1 Treatment Performed

Age of patient

Type of intervention*

Comment

3y

Maxillary RDP with buccal clasps

6y

Tomography of possible implant sites in anterior mandible

6 y, 4 mo

Implant operation under general anesthesia and placement of 2 implants

Abutment operation under conscious sedation after 8 months of healing

7y

Insertion of mandibular overdenture with custom-made male attachments and

permanent soft relining material (Molloplast B) acting as retentive part

12 y

Crown fractures of 11 and 21 caused by trauma, composite buildup 11,

semipermanent metal-acrylic crown 21

19 y, 10 mo

Placement of two additional implants in anterior mandible, 4 months of healing before

abutment connection

Brnemark 13 and 10 mm
3.75 mm

20 y

Provisional mandibular 7-unit acrylic implant-supported FDP

2122 y

Orthodontic treatment to mesialize 11, 21 and distalize 53, 63 with

fixed appliance in the maxilla

22 y, 6 mo

Maxillary tooth-supported metal-ceramic FDPs and preservation of a small midline diastema

Mandibular 9-unit acrylic-titanium implant-supported FDP

Brnemark 15 mm
3.75 mm

RDP = removable dental prosthesis, FDP = fixed dental prosthesis.

*FDI tooth-numbering system.

Fig 1a Extraoral view of patient (2 y, 2

mo).

Fig 1b Tapered primary maxillary central incisors (2 y, 2 mo).

Fig 1cPanoramic radiograph (5 y,

6 mo) showing mandibular anodontia
and maxillary primary central incisors,
hybrid canines, first permanent molars,
and tooth germs for malformed permanent central incisors.

Fig 2a Implants in the canine region of

the mandible after abutment operation
(7 y) and application of custom-made
male attachments.

Fig 2bMandibular implant-supported

overdenture with permanent soft relining
material (Molloplast B) acting as retentive part.

Fig 2cPanoramic radiograph after

placement of two Brnemark system
standard implants (10 and 13 mm) (7 y,
4 mo).

The patients permanent maxillary central incisors

were malformed, with thin pointed tips and incisal
notching as they erupted. These were reshaped with
composite resin. To repair a traumatic injury to both
teeth, the team treated the right central incisor with
composite resin and fit the left central incisor with

350

a metal-acrylic crown. The maxillary RDP and the

mandibular overdenture were adjusted and relined
according to need several times. At age 9 years 5
months, the overdenture was rebased and the acrylic
teeth were replaced with a larger, more permanentlooking set.

The International Journal of Prosthodontics

2015 BY QUINTESSENCE PUBLISHING CO, INC. PRINTING OF THIS DOCUMENT IS RESTRICTED TO PERSONAL USE ONLY.
NO PART MAY BE REPRODUCED OR TRANSMITTED IN ANY FORM WITHOUT WRITTEN PERMISSION FROM THE PUBLISHER.

Bergendal et al

Fig 3aMaxillary removable dental

prosthesis (RDP) with buccal clasps on
the primary central incisors and mandibular implant-supported overdenture
(7 y).

Fig 3b Occlusal view of the maxilla after exfoliation of primary central incisors
(8 y).

Fig 3c Frontal view after replacement

of two central incisors on the maxillary
RDP.

Fig 4a Radiographic facial profile before treatment with implants (4 y).

Fig 4b Radiographic facial profile after

treatment with implants (9 y).

Fig 4cExtraoral facial profile after

treatment with mandibular implantsupported overdenture (7 y, 8 mo).

Fig 5a (left)Erupting malformed permanent maxillary central incisors (9 y).

Fig 5b (right)Maxillary central incisors
reshaped with composite material, new
mandibular overdenture with more permanent-looking incisors (9 y, 5 mo).

When the patient was 16 years old, the treatment

team asked him if he would like more implants in the
mandible to allow a fixed dental prosthesis (FDP).
However, he felt perfectly content with his removable
dentures. Three years later he took up the question
himself and, at age 19 years 10 months, received two
more implants (Nobel Biocare 3.75 mm, 15 mm long),
one lateral and one median to the two existing implants (Fig 7b). After 4 months of healing, the team
connected new abutments, one standard and three
angulated, and placed a provisional mandibular sevenunit implant-supported FDP.
Before prosthetic habilitation in the maxilla, the patient received orthodontic treatment to mesialize the
two central incisors. At age 21, the four anterior teeth
were bonded. After leveling these teeth, orthodontists

placed a wire with coil springs between the central

incisors and the hybrid canines. After 4 months, the
gaps between the canines and the central incisors
were 6.5 mm on each side and the diastema between
the central incisors was 1 mm. One month later, the
fixed appliances were removed and replaced with a
retention plate and retention wire palatal to the central incisors to be used for 6 months. After optimizing
the positions of the six abutment teeth in the maxilla,
two provisional FDPs were placed to allow for a small
midline diastema.
Six months later, at age 22 years 6 months, two
six-unit metal-ceramic tooth-supported FDPs were
inserted in the maxilla and a retrievable nine-unit
implant-supported FDP in the mandible. To compensate for a tendency toward mandibular prognathia

Volume 28, Number 4, 2015

351

2015 BY QUINTESSENCE PUBLISHING CO, INC. PRINTING OF THIS DOCUMENT IS RESTRICTED TO PERSONAL USE ONLY.
NO PART MAY BE REPRODUCED OR TRANSMITTED IN ANY FORM WITHOUT WRITTEN PERMISSION FROM THE PUBLISHER.

Dental Implant Therapy for a Child with X-linked Hypohidrotic Ectodermal Dysplasia

Fig 6a (left)Cephalometric drawings

from profile radiographs at 7 y, 4 mo
(black) and 10 y, 4 mo (red) illustrating
the development of the jaws.
Fig 6b (below)Fixed orthodontic
appliance in the maxilla for mesializing
of central incisors and positioning of
abutment teeth before preparation for
fixed prosthesis (21 y).

Fig 6cLateral head radiograph at

33 years illustrating a good soft tissue
profile.

angulated abutments were used and the FDPs were

made with a normal frontal jaw relation. The most distal contacts on the left side were the first premolars
and, on the right side, the second premolars.
The hybrid canines in the patients maxilla are something in between primary and permanent canines,
with a different course of development and eruption,
and possibly also a different morphology. In this case
they erupted before age 3 and remain intact at age 33.
One of these hybrid teeth had an apical periodontitis
2 years after preparation for crown therapy and received root filling at age 25.

Growth and Development of the

Face and Jaws
In normal growth, the midface is well developed but
smaller than the neurocranium at the time of birth.
The sutures are all present and active as sites of bone
growth. Postnatal growth of the mandible occurs
mainly in these functional units: the coronoid processes, condyles, gonion region, corpus, and alveolar
processes. The mandibular condyles are related to
the articular function of the temporomandibular joints
(TMJs). The function and movement of the TMJs are
very important for growth. Transversal growth of the
mandible occurs through apposition of the bone along
the buccal surfaces. At the same time there is resorption on the palatal surfaces.15
Vertical growth of the maxilla and the mandible
depends heavily on tooth eruption. Both the maxilla
and the mandible grow dramatically during the transition period from early mixed to permanent dentition.16
Results from a longitudinal study in individuals aged 5

352

to 31 years showed that eruption of the permanent incisors in the maxilla seems to cause increased height of
the maxillary alveolar process between 7 and 13 years
of age.17 However, the study found that palatal height
continued to increase throughout the observation time
and increased most between ages 5 and 16 years.
In the present case, at age 7 years 3 months, the
cephalometric radiographic analysis showed a normal
maxilla and a normal sagittal relation of the mandible,
and thus a normal maxillomandibular relationship.
Vertically, the maxilla and the mandible showed a normal relationship as well (Table 2). Three years later, at
age 10 years 4 months, the sagittal relation between
the jaws showed a slight Class III relationship with an
ANB angle of 1 degree and an ANPg of 2 degrees.
There was anterior growth in the entire face with more
vertical growth than sagittal growth in both the maxilla
and the mandible. However, there was no evident increased height of the corpus of the mandible (Fig 6a).
Despite the lack of erupting teeth in the mandible,
the present case history showed average proportions
for individuals with teeth on an extraoral view at age
20 years 4 months. The cephalometric analysis revealed a midface height that was somewhat lower and
a lower face height that was somewhat higher than
the average for a 20-year-old man. There was now
a tendency to a slightly greater Class III relationship
than there had been 10 years earlier (Table 2).
At the last checkup, at age 32 years 5 months, both
the maxilla and the mandible had grown vertically
(Table 2, Fig 6c). The face grew in a Class III direction and
the treatment compensated for this with the use of angulated abutments. The profile looks similar to a Class I
case with normal dental occlusion at age 32 years.

The International Journal of Prosthodontics

2015 BY QUINTESSENCE PUBLISHING CO, INC. PRINTING OF THIS DOCUMENT IS RESTRICTED TO PERSONAL USE ONLY.
NO PART MAY BE REPRODUCED OR TRANSMITTED IN ANY FORM WITHOUT WRITTEN PERMISSION FROM THE PUBLISHER.

Bergendal et al

R1

R2 R1 L1 L2

L2

10
mm

15 10 15 13
mm mm mm mm

13
mm

1985

1998

Fig 7aSchematic illustration of natural teeth and dental

implants after placement of first two implants in the mandible
(6 y, 5 mo).

1985

Fig 7bSchematic illustration of natural teeth and dental

implants showing implant positions, time of insertion, and
implant length of four implants in the mandible after second
implant operation (20 y).

Fig 8aMulti-curve reconstruction from cone

beam computed tomography examination (33 y).
Fig 8bAxial scout image, *denotes implant
R2.
Fig 8cCross-sectional
image perpendicular to
the curved line at R2.

*
a

Fig 9a Extraoral frontal view (33 y).

Fig 9b Intraoral view (33 y).

Evaluation of Oral Health and

Orofacial Function
At age 26, 3 years 6 months after the prosthetic
treatment was completed, the patient underwent a
comprehensive evaluation of orofacial function using
the Nordic Orofacial Test-Screening (NOT-S).18 NOT-S
consists of twelve domains of orofacial function and
results in a total between 0 and 12, where 0 denotes
no orofacial dysfunction. The patient had a total NOT-S
score of 2, with points in the Chewing and swallowing domain (items IV B: Difficulties eating food with
certain consistencies, and IV C: Main meal takes 30
minutes or more) and the Dryness of the mouth domain (item VI A: Must drink to be able to eat a cracker).

Fig 9c Panoramic radiograph (33 y).

Table 2 C
 ephalometric Values at Four Different Ages:
7 Years 5 Months, 10 Years 4 Months,
20 Years 4 Months, and 32 Years 5 Months
7 y, 5 mo

10 y, 4 mo

20 y, 4 mo

32 y, 5 mo

SNA

81.5

80.0

83.5

85.5

SNB

80.0

81.0

85.5

87.5

SNPg

80.0

82.0

87.0

89.5

ANB

1.5

-1.0

-2.0

-2.0

ANPg

1.5

-2.0

-3.5

-4.0

NSL/NL

4.0

3.5

3.5

5.0

NSL/ML

29.5

29.0

25.0

24.0

NL/ML

25.5

26.5

22.5

19.0

SNA: maxillary position; SNB and SNPg: mandibular position;

ANB: sagittal jaw relation; NSL/NL: maxillary inclination; NSL/ML:
mandibular inclination; NL/ML: vertical jaw relation.

Volume 28, Number 4, 2015

353

2015 BY QUINTESSENCE PUBLISHING CO, INC. PRINTING OF THIS DOCUMENT IS RESTRICTED TO PERSONAL USE ONLY.
NO PART MAY BE REPRODUCED OR TRANSMITTED IN ANY FORM WITHOUT WRITTEN PERMISSION FROM THE PUBLISHER.

Dental Implant Therapy for a Child with X-linked Hypohidrotic Ectodermal Dysplasia

When the patient was 29 years old, a clinical functional analysis of occlusal support, occlusal force, and
chewing efficiency was performed at the Department
of Clinical Oral Physiology, Faculty of Health Sciences,
University of Copenhagen. His occlusal force was
212 N, which is lower than average for those in that
age group with at least 24 teeth (mean: 530.5, SD
97.5).19 There were 14 teeth/units with firm contact,
which does not differ from the normal values for this
age group (mean: 19.9, SD 4.0). In the side segments,
however, there were only four firm contacts, which is
lower than normal for that age group with 24 teeth
(mean: 15.8, SD: 2.0).20 Chewing efficiency was within
the normal range for individuals with natural teeth,
despite a low number of functional units, a reduced
occlusal force, and a prolonged chewing cycle.
Evident oral dryness was present from a young age.
Testing of salivary secretion at age 33 showed no unstimulated salivary secretion and a chewing-stimulated
salivary secretion rate of 0.36 mL/min.

Discussion
The patients dental treatment with dental implants at
a young age to treat anodontia of the mandible as a
sign of XLHED demonstrates a successful outcome
over a 25-year period, and the prognosis for his lifelong oral health is favorable. His treatment, performed
by a multidisciplinary team of specialists and carried
out over 3 decades, has been successful from both
patient and professional perspectives. The treatment
was provided within the framework of the Swedish
dental care system. In this system, dental care is free
of charge up to age 19, and treatment of adults with
syndromes that affect teeth and oral conditions can
be given at the same low fees as general health care
after application to a board within the county council. Treatment was time consuming, involving many
different professionals. This support has prevented
the patient from developing caries, despite severely
compromised salivation, and has resulted in good oral
function and comfort.
Treatment planning began when the patient was
3 years old with the formation of a multidisciplinary
team of specialists. The team approach has several
advantages, providing better information and support
to the child and family, as well as increased experience and shared responsibility in making treatment
decisions.21 In cases where treatment is complicated
and must be carried out over several years, the team
approach has additional benefits, taking advantage of
the best skills of each team member and increasing
continuity and planning coordination.
At the start of cooperation, the team focused their
discussions and literature review on choosing the

354

region for placement of the first implants. Enlow presented convincing evidence that the canine region of
the mandible was more stable than the frontal and
lateral segments due to appositional buccal growth
and palatal resorption of the bone.7 This was later
confirmed in studies on growing pigs where some
implants became situated palatally to their original
insertion sites due to palatal resorption distal to the
canines.22 The authors concluded that the osseo
integration technique is not to be recommended in
the lateral regions in young children.
So far, the patient has not experienced dental caries in the maxillary teeth; the periodontal tissues, as
well as the peri-implant mucosa, have been healthy
during the follow-up period. We attribute this to continual maintenance care and regular application of
fluoride varnish supplementary to self-administered
rinsing with fluoride solution and use of fluoridated
toothpaste.
The patient has been satisfied with his oral function
throughout his childhood and adolescence, and also
as an adult. There have been no technical complications related to the implants or the FDPs other than
moderate wear of the mandibular acrylic occlusal
surfaces. One objective of the care program, worked
out at a consensus meeting on ED in 1998, was for
the patient to have dentures to replace his missing
teeth by the time he started school to improve his oro
facial function, with special reference to esthetics and
speech.9,23 In the present case, because the patient
was only 6 years old at the time of operation, the width
of the mandible at the implant sites was small and
there were exposed threads on the buccal surface
of one of the two implants. For future treatment of
children with mandibular anodontia, we recommend
postponing insertion of the first implants some years
to allow for growth of the mandible. Now, however,
implants are available with a smaller diameter that can
be used when the width at the implant sites is limited.
The patients overall orofacial function as an adult
was good, as assessed by both screening with the
NOT-S (where the total score was 2) and clinical examinations by an independent observer. In a study using the NOT-S in 46 individuals with various forms of
ED, aged 3 to 55 years (mean: 14.5) in Sweden and the
United States, the mean NOT-S total score was 3.5,
compared to 0.4 in a healthy reference sample.24 The
ED group consisted of 32 individuals with HED and 14
with other ED syndromes and had total NOT-S scores
of 3.0 and 4.6, respectively. For each of the twelve domains of NOT-S there were some individuals in the ED
sample who had scores. The most common domains
for dysfunction scores were, in order of frequency:
Chewing and swallowing (82.6%), Dryness of the
mouth (45.7%), and Speech (43.5%). These results

The International Journal of Prosthodontics

2015 BY QUINTESSENCE PUBLISHING CO, INC. PRINTING OF THIS DOCUMENT IS RESTRICTED TO PERSONAL USE ONLY.
NO PART MAY BE REPRODUCED OR TRANSMITTED IN ANY FORM WITHOUT WRITTEN PERMISSION FROM THE PUBLISHER.

Bergendal et al

in individuals with varying levels of oral habilitation

indicate the need for optimal prosthetic care for this
group of patients to restore function and appearance
and improve quality of life.
The team abandoned its original plan to insert more
than the two implants in the mandible they added at
age 19 due to a lack of bone and the extension of the
canal of the mandibular nerve. Bone augmentation
from the iliac crest was not an option because of the
risk of donor site morbidity in a young patient. Due to
wear and fatigue of the prosthetic materials, the FDPs
might need to be replaced at some point.
Before publication of the high implant failure rate
in the treatment of small Swedish children with HED,
there were many case reports on successful treatment outcomes. The first indication of a different
response to implant treatment and its higher risk of
failure appeared in the only prospective study with a
large sample of patients with ED by Guckes et al.25
Among 51 patients with ED, the survival rate in the
anterior mandible was 91% (out of 243 implants) and
76% in the anterior maxilla (out of 21 implants). All
but two failures occurred before or during secondstage surgery. These early failures are in accordance
with results from Swedish children with HED, where
four out of five subjects lost implants before loading.10
Stanford et al confirmed a high risk of failure and
complications in patients with different EDs treated
with dental implants.26 That study used a self-reported survey for data collection to evaluate patient satisfaction, outcomes, and complications. The response
rate was 43%. Three out of four respondents reported
some form of implant failure and half reported either
an implant or prosthetic failure. However, all but 9% of
the respondents were satisfied or very satisfied with
the dental implants.
Later studies have increased our understanding of
syndrome-specific factors in XLHED related to bone
density through histologic studies in bone,11 and by
using ultramicroscopy of trephine bone plugs at implant sites in adolescents with ED.12 After publishing
on implant failures in small children with HED, we have
had comments from clinicians and researchers from
around the world indicating that they have also experienced early implant failures in these patients. Since
most reports on the use of implants in small children
with ED are favorable, there seems to be a bias toward
reporting and publishing positive treatment outcomes.
To avoid report bias and delay in understanding the
risks of negative treatment outcomes, the publishing
of negative results should be promoted.27
The advantages of a multiprofessional approach
to management of patients with ED were recently
confirmed in a study using the Delphi method. The
study engaged 11 internationally recruited specialist

teams to establish a state-of-the-art protocol in oral

treatment and management of children with ED and
severe tooth agenesis. After three rounds of questionnaires, the degree of consensus reached 90%.28
A subsequent consensus meeting strongly advocated
international multicenter studies in this very rare patient cohort to get more reliable evidence on the prognosis.29 Furthermore, establishing quality registries of
treatment outcomes would enhance the possibilities
for early risk assessment in implant treatment for rare
disorders and chronic diseases.14,30
In view of the denser bone quality associated with
XLHED and reports of difficulties related to the small
size of the mandible at around 6 years of age, we suggest postponing treatment with implants for a few
years, using implants with a small diameter, and adhering to a protocol for scrupulous implant surgery
that takes into account the risk of overheating dense
bone. Despite the associated risks, this long-term
follow-up of a child with XLHED and anodontia in the
mandible supports the use of dental implants to establish good orofacial function and appearance from
childhood onward.

Conclusions
This long-term follow-up report of a child with XLHED
and anodontia in the mandible endorses the use of
dental implant treatment, with consideration given to
the dense bone quality associated with the diagnosis.
A multidisciplinary team approach with a continuum
of maintenance care was identified as an important
prerequisite for a good prognosis.

Acknowledgments
We are indebted to many colleagues at the Institute for
Postgraduate Dental Education in Jnkping who participated in
the planning and treatment of the patient over three decades. Sven
Kvint, specialist in oral surgery, performed the implant operations.
He was, together with Anna-Lena Hallonsten, specialist in paediatric dentistry, and Olof Eckerdal, specialist in oral and maxillofacial
radiology, involved in the original multidisciplinary team around
the patient. We also thank Merete Bakke, Department of Clinical
Oral Physiology, School of Dentistry, Faculty of Health and Medical
Sciences, University of Copenhagen, Copenhagen, Denmark, for
independent evaluation of orofacial function. The authors reported
no conflicts of interest related to this study.

References
1. Bergendal T, Eckerdal O, Hallonsten AL, Koch G, Kurol J,
Kvint S. Osseointegrated implants in the oral habilitation of
a boy with ectodermal dysplasia: A case report. Int Dent J
1991;41:149156.

Volume 28, Number 4, 2015

355

2015 BY QUINTESSENCE PUBLISHING CO, INC. PRINTING OF THIS DOCUMENT IS RESTRICTED TO PERSONAL USE ONLY.
NO PART MAY BE REPRODUCED OR TRANSMITTED IN ANY FORM WITHOUT WRITTEN PERMISSION FROM THE PUBLISHER.

Dental Implant Therapy for a Child with X-linked Hypohidrotic Ectodermal Dysplasia

2. Smith RA, Vargervik K, Kearns G, Bosch C, Koumjian J.

Placement of an endosseous implant in a growing child with
ectodermal dysplasia. Oral Surg Oral Med Oral Pathol 1993;75:
669673.
3. Guckes AD, McCarthy GR, Brahim J. Use of endosseous implants in a 3-year-old child with ectodermal dysplasia: Case
report and 5-year follow-up. Pediatr Dent 1997;19:282285.
4. Kearns G, Sharma A, Perrott D, Schmidt B, Kaban L, Vargervik
K. Placement of endosseous implants in children and adolescents with hereditary ectodermal dysplasia. Oral Surg Oral
Med Oral Pathol 1999;88:510.
5. Bonin B, Saffarzadeh A, Picard A, Levy P, Romieux G, Goga D.
Early implant treatment of a child with anhidrotic ectodermal
dysplasia. Apropos of a case [in French]. Rev Stomatol Chir
Maxillofac 2001;102:313318.
6. Moorrees CFA. The Dentition of the Growing Child: A
Longitudinal Study of Dental Development Between 3 and 18
Years of Age. Cambridge: Harvard University Press, 1959.
7. Enlow DH. Handbook of Facial Growth. Philadelphia: Saunders,
1982.
8. Sharma AB, Vargervik K. Using implants for the growing child.
J Calif Dent Assoc 2006;34:719724.
9. Bergendal B, Koch G, Kurol J, Wnndahl G (eds). Consensus
Conference on Ectodermal Dysplasia with Special Reference
to Dental Treatment. Stockholm, Sweden: Frlagshuset Gothia,
1998.
10. Bergendal B, Ekman A, Nilsson P. Implant failure in young children with ectodermal dysplasia: A retrospective evaluation of
use and outcome of dental implant treatment in children in
Sweden. Int J Oral Maxillofac Implants 2008;23:520524.
11. Lesot H, Clauss F, Maniere MC, Schmittbuhl M. Consequences
of X-linked hypohidrotic ectodermal dysplasia for the human
jaw bone. Front Oral Biol 2009;13:9399.
12. Silthampitag P, Klineberg I, Jones AS, et al. Ultramicroscopy
of bone at oral implant sites: A comparison of ED and control
patients. Part 1: Defining the protocol. Int J Prosthodont 2011;
24:147154.
13. Mikkola ML. Molecular aspects of hypohidrotic ectodermal
dysplasia. Am J Med Genet A 2009;149A:20312036.
14. Bergendal B. Orodental manifestations in ectodermal dysplasia: A review. Am J Med Genet A 2014;164A:24652471.
15. Carlsson DS, Buschang PH. Craniofacial growth and development: Evidence-based perspectives. In: Graber L, Vanarsdall R,
Vig K (eds). Orthodontics Current Principles and Techniques.
Philadelphia: Elsevier Mosby, 2012:215246.
16. Thilander B. Basic mechanisms in craniofacial growth. Acta
Odontol Scand 1995;53:144151.

17. Thilander B. Dentoalveolar development in subjects with normal occlusion. A longitudinal study between the ages of 5 and
31 years. Eur J Orthod 2009;31:109120.
18. Bakke M, Bergendal B, McAllister A, Sjogreen L, Asten P.
Development and evaluation of a comprehensive screening for
orofacial dysfunction. Swed Dent J 2007;31:7584.
19. Bakke M. Mandibular elevator muscles: Physiology, action, and
effect of dental occlusion. Scand J Dent Res 1993;101:314331.
20. Bakke M, Holm B, Jensen BL, Michler L, Mller E. Unilateral,
isometric bite force in 8 68-year-old women and men related
to occlusal factors. Scand J Dent Res 1990;98:149158.
21. Bergendal B, Bergendal T, Hallonsten AL, Koch G, Kurol J, Kvint
S. A multidisciplinary approach to oral rehabilitation with osseointegrated implants in children and adolescents with multiple aplasia. Eur J Orthod 1996;18:119129.
22. Thilander B, Odman J, Grondahl K, Lekholm U. Aspects on osseointegrated implants inserted in growing jaws. A biometric
and radiographic study in the young pig. Eur J Orthod 1992;
14:99109.
23. Bergendal B. Children with ectodermal dysplasia need early
treatment. Spec Care Dentist 2002;22:212213.
24. Bergendal B, McAllister A, Stecksen-Blicks C. Orofacial dysfunction in ectodermal dysplasias measured using the Nordic
Orofacial Test-Screening protocol. Acta Odontol Scand
2009;67:377381.
25. 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;88:2125.
26. 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;21:195200.
27. Bergendal B. Interpretive and report bias in publications on implants in patients with ectodermal dysplasia. Int J Prosthodont
2011;24:505506.
28. Klineberg I, Cameron A, Whittle T, et al. Rehabilitation of children with ectodermal dysplasia. Part 1: An international Delphi
study. Int J Oral Maxillofac Implants 2013;28:10901100.
29. Klineberg I, Cameron A, Hobkirk J, et al. Rehabilitation of
children with ectodermal dysplasia. Part 2: An international
consensus meeting. Int J Oral Maxillofac Implants 2013;28:
11011109.
30. Bergendal B. Oligodontia and ectodermal dysplasiaon signs,
symptoms, genetics, and outcomes of dental treatment. Swed
Dent J 2010;35(suppl 205):1378.

Literature Abstract
Mucosal microbiome in patients with recurrent aphthous stomatitis
Composition profiles of bacterial microbiota in the oral mucosa associated with recurrent aphthous stomatitis (RAS) was studied.
A group of unspecified number of subjects with RAS history was involved in this study. The subjects were medically healthy and
maintained proper oral hygiene using manual tooth brushing. Samples were taken from the oral cavity (buccal sulcus and ulcerated
area) before breakfast by direct swapping. The same sampling was performed in a match healthy control group. The character of the
microbiata in RAS-associated mucosa was studied using high-throughput 16S RNA gene sequencing. This study showed there was
a change in the microbiota in idiopathic RAS mucosa. An increase in Bacteroidales species was observed (not affected by active
ulcerations). This data only enhanced understanding of potential microbial roles in RAS.
Hijazi K, Lowe T, Meharg C, Berry SH, Foley J, Hold GL. J Dent Res 2015;94(suppl 3):7S94S. References: 34.
Reprints: Email: k.hijazi@abdn.ac.ukAnsgar C. Cheng, Singapore

356

The International Journal of Prosthodontics

2015 BY QUINTESSENCE PUBLISHING CO, INC. PRINTING OF THIS DOCUMENT IS RESTRICTED TO PERSONAL USE ONLY.
NO PART MAY BE REPRODUCED OR TRANSMITTED IN ANY FORM WITHOUT WRITTEN PERMISSION FROM THE PUBLISHER.