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Case Report

Nonsyndromic Form of Oligodontia in a Chinese Male Patient:

A Rare Case Report
Preethy Mary Donald1, Ankita Arora2, Renjith George3
1
Department of Oral Medicine and Radiology, Faculty of Dentistry, Melaka‑Manipal Medical College, Departments of 2Pedodontics and 3Oral Pathology, Melaka‑Manipal
Medical College, Melaka, Malaysia

Abstract
Oligodontia is one of the developmental disturbances seen in human with more than six congenitally missing teeth. Two forms of oligodontia
which exist are the syndromic form and the nonsyndromic form. Mutation or deletion of MSX and PAX genes are implicated in oligodontia.
Missing teeth often compromises patient’s esthetics, function, and causes mental stress. Here, we highlight a rare case report of a nonsyndromic
form of oligodontia with concomitant dental anomalies like taurodontism in maxillary permanent first molar and congenitally missing canine
in a 17‑year‑old apparently normal Chinese male patient.

Keywords: Congenital, missing, permanent

Introduction
Developmental disturbances of fewer than normal complement
of teeth can be broadly divided into two categories; hypodontia
and oligodontia. Hypodontia is a condition where <six teeth
are congenitally missing usually third molars, maxillary lateral
incisors, and premolars. Oligodontia is when more than six
teeth are congenitally missing excluding third molars. This is
often observed during routine intraoral examination procedure
by dental professionals. Anodontia is the condition where all
the teeth fail to develop which is extremely rare.[1]
The incidence of oligodontia has been found to vary from
0.08% to 0.16%. Oligodontia has been found to occur with or
without association of syndrome. Literature has reported many
syndromes associated with oligodontia such as ectodermal
dysplasia, Seckel syndrome, Down syndrome, Rieger
syndrome, Wolf‑Hirschhorn syndrome, Klippel‑Feil syndrome,
and Van der Woude syndrome.[2‑5]
Among the syndromic form of oligodontia, ectodermal
dysplasia is the most common form. The nonsyndromic form of
oligodontia (isolated form) lacks the characteristic features of
ectodermal dysplasia such as sparse hair, dry skin, dystrophic
nails, and deficient sweat glands. Nonsyndromic form can be
either familial or sporadic type.[6] For easy understanding to
aid in effective case history taking and to differentiate between
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the two forms clinically, two tables [Table 1 and 2] have been
devised: physical and dental traits.[1,2,6] Congenitally, missing
teeth can be confirmed by taking panoramic radiographs.
Here, we present a case report of a nonsyndromic Chinese
male patient with oligodontia which includes missing canine
which is a rarity.
Case Report
A 17‑year‑old Chinese male patient visited our dental clinic,
Melaka‑Manipal Medical College. He complained of spacing
between his upper teeth. The patient also remarked that he is
depressed about his smile and appearance. His medical history
was unremarkable. As a routine procedure, initial dental
screening was done. Extraoral examination revealed normal
facial profile. Intraoral examination in relation to maxillary
teeth showed midline diastema, clinically missing upper
right and left lateral incisors, retained right and left deciduous
canine, first molar, and second molar. In relation to mandibular
Address for correspondence: Dr. Preethy Mary Donald,
Department of Oral Medicine and Radiology, Faculty of Dentistry,
Melaka‑Manipal Medical College, Jln Batu Hampar, Bukit Baru 75150,
Melaka, Malaysia.
E‑mail: pmdrsc30@gmail.com
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For reprints contact: reprints@medknow.com

DOI: How to cite this article: Donald PM, Arora A, George R. Nonsyndromic
10.4103/jioh.jioh_117_17 form of oligodontia in a Chinese male patient: A rare case report. J Int Oral
Health 2017;9:180‑2.

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Donald, et al.: Nonsyndromic form of oligodontia

Table 1: Characteristic features in ectodermal dysplasia

Physical Physical features in hypohidrotic
traits ectodermal dysplasia
Skin Dry hypoplastic skin
Scalp Sparse scalp, fine hair
Forehead Prominent forehead
Hair Alopecia or hypotrichosis
Nails Dystrophic fingernails
Sweat glands Deficient sweat glands or hypohidrosis
Eyes Sunken eyes
Figure 1: Panoramic radiograph of a Chinese male patient
Nose Sunken nasal bridge (saddle nose)
Lips Everted lips
Discussion
Oligodontia is a rare developmental disturbance observed
Table 2: Characteristic features of teeth and dental in primary or permanent dentition. The underlying cause
anomalies associated with syndromic and nonsyndromic is due to failure in proliferation of tooth buds from dental
form of oligodontia lamina.[7] The factors attributed to this condition could be either
Dental traits Characteristic features hereditary or environmental. The condition can be transmitted
Shape of teeth Conical shaped, bud‑shaped crown, malformed as autosomal dominant, autosomal recessive, or X‑linked. The
Size of teeth Smaller, microdontia, decreased mesiodistal main genes implicated in early tooth formation are MSX‑1
diameter of molars and PAX gene; hence, mutation in any one of the gene can
Concomitant Taurodontism, idiopathic generalized short‑root result in agenesis.[8] The environmental factors that can cause
dental anomalies anomaly, dens invaginatus, talon cusp, enamel
hypoplasia oligodontia are mainly due to trauma, infection such as rubella,
Other dental Transmigration of teeth, midline diastema, exposure to drugs such as thalidomide, hormonal influences,
features rotation, retained deciduous teeth, malocclusion previous irradiation, or presence of tumor.[9,10]
Alveolar Hypoplastic, sharp, and narrow
edentulous ridges In this case report, there were 14 congenitally missing
teeth. However, the patient did not have any other physical
characteristics of a syndrome. The teeth missing were
teeth, both right and left deciduous second molar was retained.
maxillary laterals, canine, premolars, mandibular second
There were no restorations. Gingiva in relation to lower premolar, and all third molars. Researchers have reported that
anteriors had mild inflammation and calculus was evident. the most common congenital missing teeth are mandibular
A panoramic radiograph was taken and assessment confirmed second premolars followed by maxillary second premolars
missing #12, #13, #14, #15, #18, #22, #23, #24, #25 total of 14 and maxillary laterals. However, studies have reported that
congenitally missing teeth including third molars [Figure 1]. missing canine is very rare.[8] Interestingly, in our case, both
Taurodontism was evident with #17 and #27. For further the maxillary canines were congenitally missing which brings
assessment, few more questions were asked. As this was his the need for reporting this case and to bring awareness of the
first dental visit, the patient claimed that neither extractions same among dentists.
were done in the past nor remembers an incident of trauma.
He also stated that none of his family members had congenital Considering the age of patient, it was surprising again that
missing teeth. His general examination revealed moderately there was no evidence of third molar crown or follicle in all
built and well‑nourished condition. His skin and nails were the four quadrants as literature states that crown completion of
normal. He did not have any intolerance to heat. Based on the third molar would be completed by the age of 13 and 14 years
given history, clinical features, and radiographic features, it for boys and girls, respectively.[11] According to Gupta, it can
was provisionally diagnosed as nonsyndromic oligodontia. be termed as severe oligodontia when complete set of molars
is missing.[12] Rathore et al. reported nonsyndromic hereditary
A comprehensive treatment was planned for this patient. oligodontia in a 20‑year‑old female with the absence of ten
Scaling, extraction of all retained deciduous teeth, orthodontic teeth involving first and second molars and mandibular
consultation for closure of midline diastema, and prosthetic second premolar. [13] Dental problems concomitant with
rehabilitation with removable partial denture were planned. oligodontia seen in this case were his midline diastema and
Osseointegrated implants can be planned at a later date for this taurodontism. A significant association between taurodontism
patient once his skeletal growth of the jaw has been completed. and oligodontia had been reported by Seow and Lai.[14]
All the treatment procedures were explained to the patient. Oligodontia in many patients has been reported to have a
Scaling was done; however, he did not follow‑up for further strong impact on declining the level of confidence, especially
appointments and hence management of this case came to a in high schoolchildren and at workplace, due to unsatisfied
halt unfortunately. appearance of smile owing to weak performance. In addition

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Donald, et al.: Nonsyndromic form of oligodontia
to this, mastication, speech, and malocclusion problems do
exist in these kinds of patients, thus deteriorating one’s quality
of life. Overall, this affects an individual’s emotional and
psychological balance.[15]
In the present case, a multidisciplinary team of dental
professionals consisting of oral surgeon, orthodontists, and
prosthodontists is imperative to achieve the goal of restoring
esthetics, functional and psychological stress. However, the
limitations of this study are that, first, there are no systematic
reviews done in relation to this topic to compare, and second,
we lost the patient to further complete the treatment procedures
as planned.
Conclusion
Reporting of this, rare case will improve further understanding
and raise awareness of dental professionals. Dentists while
encountering with such patients with oligodontia, a detailed
case history, physical examination to rule out syndromic form
of oligodontia, and radiographs should be done to diagnose
the case. Early systematic intervention will help in improving
esthetics, speech, mastication, and social life.
Acknowledgment
The authors would like to express gratitude and appreciation
to the radiographer of MMMC, Mrs. Anisah Binti Ismail, for
acquisition, exporting, and retrieving image for our publication
purpose.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
182 Journal of International Oral Health  ¦  Volume 9  ¦  Issue 4  ¦  July‑August 2017
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