International Journal of Medical and Dental Case Reports (2016), Article ID 010316, 4 Pages

CASE REPORT

Dental prosthetic rehabilitation of a child with ectodermal

dysplasia: A case report
Snehalika More1, Mihir Nayak2, Sandyadevi S. Patil1, Madhu Kakanur1, Rachna Thakur1
1
Department of Pedodontics & Preventive Dentistry, K.L.E Society’s Institute of Dental Sciences & Hospital, Bengaluru, Karnataka, India, 2Department of
Pedodontics & Preventive Dentistry, Sri Rajiv Gandhi College of Dental Sciences & Hospital, Bengaluru, Karnataka, India

Correspondence
Snehalika More, Department of Pedodontics
& Preventive Dentistry, K.L.E Society’s Institute
of Dental Sciences & Hospital, Bengaluru,
Karnataka, India. Phone: +91-8867383901.
Email: snehalika88@hotmail.com
Received 08 March 2016;
Accepted 10 April 2016
doi: 10.15713/ins.ijmdcr.42
How to cite the article:
More S, Nayak M, Patil SS, Kakanur M,
Thakur R. Dental prosthetic rehabilitation
of a child with ectodermal dysplasia: A case
report. Int J Med Dent Case Rep 2016;2: 1-4.
Abstract
Ectodermal dysplasias (EDs) form a part of the complex group of rare congenital diseases,
primarily observed as a result of the abnormal development of embryonic ectodermal
derivatives. A 5-year-old male child reported to the Department of Pedodontics and
Preventive Dentistry, with the typical features of hypohidrotic ectodermal dysplasia
(HED), i.e., diffusely sparse hair, eyelashes and eyebrows, subsequent problems with
thermoregulation and dry skin. The facial profile showed a prominent forehead, sunken
nasal bridge (“saddle nose”), and everted lips. Oral prosthetic rehabilitation was planned
for the child to improve both the vertical and sagittal skeletal relationship, additionally
providing better esthetics, improved speech, and masticatory function. The case report
thus presents the early prosthetic oral rehabilitation of a young boy with HED associated
with severe anodontia in the primary dentition.
Keywords: Anodontia, ectodermal dysplasia, male child, primary dentition, prosthetic rehabilitation

Introduction
Ectodermal dysplasia (ED) is one among those large group of
conditions resulting from the failure of development of two or
more ectodermally-derived anatomical structures.[1] ED may
be inherited in any of the genetic patterns, i.e., autosomal-
dominant (AD), autosomal-recessive (AR), or even X-linked
variant.[2] Although numerous subtypes of ED exists, a low
estimated incidence of 1 in 1 lakh population has been
reported.[3,4] Two major groups of ED have been distinguished
based on the involvement of sweat glands. One among them is
the hypohidrotic or anhidrotic variant (also termed as Christ-
Siemens-Touraine syndrome), where the number of sweat
glands is either reduced significantly or absent. The other variant
is hidrotic form of ED (also called as Clouston syndrome),
where the sweat glands are relatively normal. Hypohidrotic ED
(HED) is the most common type, and it seems to be exhibiting
the X-linked inheritance pattern; thus, the males showing more
susceptibility compared to females. Hidrotic type of ED is
inherited as an AD pattern.[5]
Those affected with ED typically show hypoplasia or aplasia
of skin, hair, nails, nerve cells, sweat glands, parts of the eye, ear
and most relevant to dentists, i.e., the teeth.[1]
Oligodontia or hypodontia is a common feature, and the
teeth that are present often manifest abnormal morphology.
Incisors often appear tapered and conical while the molars might
be dimensionally smaller.[6] Lack of tooth bud formation leads to
the hypoplastic alveolar bone, which in turn causes diminished
vertical dimension of occlusion giving them a senile or aged
appearance.[5]
Hence, an early dental prosthetic rehabilitation (generally
recommended from the age of 5 years) in affected children
would avert the psychosocial stigma and give them more self-
confidence, also helping in facial esthetics, improving their
masticatory function and speech process. If child co-operation
is not a hurdle, dentures are even recommended as early as
3-4 years of age.[7] A few clinicians may opt for implant therapy,
but it is not feasible for young growing children. Non-compliant
bone, the implant itself behaving like an ankylosed tooth,
problem with occlusion, tilting or drifting of teeth and implants,
and cosmetic reasons are some of the unfavorable factors
attributed for it.[8,9]
The present study deals with the early dental prosthetic
rehabilitation in a male child aged 5 years affected with HED.
Case Report
A boy aged 5 years was referred to the Department of
Pedodontics and Preventive Dentistry, KLE Society’s Institute

1
More, et al.
of Dental Sciences, Bengaluru, Karnataka, India, presenting
with the absence of teeth as the chief complaint. Parents came
to know about the medical problem of the child when he was
2 weeks old. Diagnosis of ED was confirmed by a pediatrician.
Family history revealed that none in the family were afflicted
with the same condition. Parents had consulted the dentist when
the child was 2 years old, but no treatment could be done as he
was too young to co-operate.
The child suffered from social stigma from his peers at school.
Frequent episodes of fever which would regress only with cold
sponge bath were reported. Less sweating was noted as the cause
for this by the physician as a part of symptoms of ED. The child
also had a difficulty in chewing food, probably because of dry
mouth associated with the low salivary flow.
On extraoral examination, typical features of ED such as dry
and rough skin, sparse fine hair, lack of eyelashes and eyebrows,
hypohydrosis, hypoplastic mid-face, prominent lips, visible loss
of facial vertical dimension giving the child a senile appearance
were observed [Figure 1].
Figure 1: Extraoral view – front profile (before treatment)
Figure 2: Intra-oral view – maxillary and mandibular arch
2
Dental rehabilitation of a child with ectodermal dysplasia
Intraoral examination showed completely edentulous
mandibular arch, oligodontia in the maxillary arch with only
two deciduous maxillary second molars (55 and 65) clinically
erupted. Decreased vertical bone height, loss of vestibular depth,
and thin alveolar ridge, especially in the mandibular arch, were
noticed [Figure 2].
Panoramic radiograph taken when the child was only 2 years
old [Figure 3] revealed four tooth germs in maxillary arch,
resembling deciduous maxillary molars and deciduous maxillary
central incisors/canines.
Panoramic radiograph taken when the child was 4½ years
[Figure 4] revealed two deciduous maxillary second molars
(55 and 65) at Nolla stage 9 (root completed with open apex).
Two tooth germs resembling deciduous maxillary central
incisors/canines were also observed. No other tooth germs
were noted. No other pathological changes associated with
the alveolar bone/arches were noticed, except that the vertical
height of lower alveolar bone was decreased.
To fabricate removable partial dentures, two impressions
were made, one with hydrocolloid material (alginate) with stock
tray and later using an elastomeric material with custom tray
[Figure 5]. As two maxillary molars were clinically present, it was
decided to fabricate over-dentures in relation to maxillary arch,
and a complete denture was fabricated for the mandibular arch
[Figure 5]. Removable partial dentures were thus delivered to
the child [Figure 6], which significantly improved esthetics and
helped in mastication. Recall visits were scheduled to evaluate
the compliance of the dentures for relining if required and to
assess the growth changes in the developing jaws.
Figure  3: Panoramic radiographic image – when child was
2 years old
Figure  4: Panoramic radiographic image – when child was
4½ years old
Dental rehabilitation of a child with ectodermal dysplasia
Figure 5: Secondary elastomeric impressions; removable maxillary
over-denture and mandibular complete denture
Figure  6: Extraoral view – front profile (during follow-up after
3 months post-treatment)
Discussion
The male child mentioned in the case had deciduous maxillary
central incisors/canines, which were yet to erupt and clinical
presence of only deciduous maxillary second molars. Similar
findings were observed in a previous report by Sweeney et al.
(2005),[10] who mentioned that the permanent teeth most
often present among patients with ED in the upper arch were
the central incisors followed by the first molars and the canines,
whereas in the mandible, the canines, first premolars, and first
molars were most likely to be found.[10,11]
Rehabilitation of missing teeth by implants is usually limited
to patients in whom the craniofacial growth is assumed to be
complete. Thus, it would be safe to wait until the adolescence.
Implants exhibit higher chances of failure when placed in
younger children, posing a risk to the developing tooth germs
and indirectly hampering the overall skeletal craniofacial
growth.[12]
3
More, et al.
In the particular case, implant therapy was excluded as a
treatment option citing the above-mentioned reasons and also
because of the insufficient alveolar bone available.
Although removable dentures are commonly advised, growth
changes in children may underline the need for frequent repairs/
adjustments such as relining of removable partial or complete
dentures. Replacement by a new denture is considered when
there is a gross discrepancy in patients with reduction in vertical
dimension of occlusion and associated abnormal mandibular
posture.[13]
Retention and stability of these dentures are another concern,
as it is common to witness dry mucosa and under-developed
alveolar ridges.[14] Methods to prevent mucosal dryness and
wider coverage of the denture base would help to overcome the
problems associated with denture retention and stability.
In children suffering from ED, lack or absence of teeth with
abnormal craniofacial vertical and sagittal skeletal relationships
hampers the esthetics, masticatory ability, speech and temporo-
mandibular joint function.[15] Thus, it is valid to tell that the
most widely followed treatment plan of fabricating removable
prosthesis can also be regarded as the best available option for
our young growing patients, with the added advantage of early
age intervention providing long-term psychosocial benefits.
Conclusion
Children affected with ED often present with a compromised
dental status. The overall management includes restorative and
prosthetic therapy to implant placements, frequently needing
multidisciplinary assistance. Pediatric dentists are imposed with a
challenge to treat them with a prime concern to delicately handle
the psychosocial implications of the child patient as well as to
treat them at the earliest so as to minimize further complications.
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