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Gran Koch
Odontologiska Institutionen i Jnkping
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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,
348
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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.
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.
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Dental Implant Therapy for a Child with X-linked Hypohidrotic Ectodermal Dysplasia
Type of intervention*
Comment
3y
6y
6 y, 4 mo
7y
12 y
19 y, 10 mo
Brnemark 13 and 10 mm
3.75 mm
20 y
2122 y
22 y, 6 mo
Brnemark 15 mm
3.75 mm
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Bergendal et al
Fig 3b Occlusal view of the maxilla after exfoliation of primary central incisors
(8 y).
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Dental Implant Therapy for a Child with X-linked Hypohidrotic Ectodermal Dysplasia
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.
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Bergendal et al
R1
R2 R1 L1 L2
L2
10
mm
15 10 15 13
mm mm mm mm
13
mm
1985
1998
1985
*
a
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
353
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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
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Bergendal et al
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
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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
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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
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