Open Journal of Stomatology, 2013, 3, 37-41 OJST Published Online December 2013 (
Non-syndromic hypo-hyperdontia—A rare case report and
review of literature
Kanika Gupta Verma1, Pradhuman Verma2, Navneet Singh3, Richa Bansal4, Rameen Khosa2,
Gagandeep Kaur Sidhu5, Vikas Setia6
1Department of Pediatric & Preventive Dentistry, Surendera Dental College & RI, Sriganganagar, India
2Department of Oral Medicine & Radiology, Surendera Dental College & RI, Sriganganagar, India
3Department of Oral Path ology & Microbiology, Maharaja Ganga Singh Dental College & RI, Sriganganagar, India
4Department of Oral Pathology & Microbiology, Seema Dental College & RI, Rishikesh, India
5Department of Oral Pathology & Microbiology, Darshan Dental College & Hospital, Udaipur, India
6Department of Pediatric & Preventive Dentistry, Adesh Dental College & RI, Bathinda, India
Received 20 September 2013; revised 26 October 2013; accepted 18 November 2013
Copyright © 2013 Kanika Gupta Verma et al. This is an open access article distributed under the Creative Commons Attribution Li-
cense, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abnormalities of tooth number in development of the
dentition are quite common; however, concomitant
hypo-hyperdontia is a rare mixed numeric anomalous
condition, especially when it occurs in the same dental
arch and in a non-syndromic situation. The presence
of this condition specifically in the mandibular ante-
rior region is reported very infrequently. This case
report presents the case of a 9 years old non-syn-
dromic male with missing mandibular central inci-
sors and an erupted mandibular mesiodens along
with the review of literature. To ensure optimum
function and aesthetics, cosmetic recontouring was
performed after oral prophylaxis. This is the ninth
case reported in the dental literature till date, with
the two anomalies manifesting in the anterior region
of the mandible. This article also discusses the review
of literature of concomitant hypo-hyperdontia.
Keywords: M esiodens; Hypodontia; Hyperdontia;
Mandibular Arch
Concomitant hypo-hyperdontia (also known as oligo-
pleiodontia) is a rare mixed numeric anomalous condi-
tion, where developmental absence of teeth (Hypodontia)
and supernumerary teeth (Hyperdontia) co-exist in same
individual. The probability of the condition is between
0.0008 and 0.0015 , with most of the cases docu mented in
maxillary arch [1]. The aetiopathogenesis of this simul-
taneous hyper-hypodontia is obscure [2]. Disturbance in
migration, proliferation and differentiation of the neural
crest cells and interaction between the epithelial and
mesenchymal cells during the initiation stage of tooth
development have been suspected as possible causes [3].
Very few cases have been reported with this condition in
the English literature till date. Here is the ninth case to be
reported with two anomalies manifesting in mandibular
anterior region. The rarity of this condition of mixed
numerical superiority and inferiority of the human denti-
tion in the mandibular incisor region prompted this re-
9-year-old male child and his parents were made aware
in advance that the facial pho tograph will be used fo r the
academic and publication purpose. After the explanation
to the patient, written informed consent was taken in
local as well as in English language for the same.
Proper institutional protocols regarding ethical issues
have been followed. Conflict of interest is disclosed as
Over the years Mesiodens has been studied extensively.
But these have been predominantly in the maxillay arch.
Very few cases have been reported in the mandibular
arch & Hypohyperdontia is extremely rare. Here we re-
view this condition (Table 1).
The first case of concomitant hypohyperdontia with
missing central incisors and erupted mandibular mesio-
dens was observed by Low in 1977 [4], followed by
Gibson [5], Das et al. [6], Karthik V et al. [7] and Ve rma
KG et al. [8]. Nayak et al. [9] the coexistent hypo-hy-
K. G. Verma et al. / Open Journal of Stomatology 3 (2013) 37-41
Table 1. List of the documented cases in the literature similar
to this case.
Authors Age/
in addition to
Agenesis Any other
Low (1977) 7/M - 31, 41 -
Gibson (1979) 6/F - 31, 41 -
Das et al.
(2006) 8/F - 31, 41 -
Nayak A.G.
et al. (2010) 28/M - 32, 42 -
Nuvvula S.
et al. (2010) 15/M - 31, 41
Agenesis of
18, 28, 48
Karthik V.
et al. (2011) 9/F - 31, 41 -
Verma K.G.
et al. (2012) 15.6/M - 31, 41 -
Marya C.M.
et al. (2012) 20/M - 31, 41
Agenesis of
18, 28, 48
Verma K.G.
et al. (2013)
Present case 9/M - 31, 41
Agenesis of
18, 28, 38, 48
perdontia with missing lateral incisors and erupted man-
dibular mesiodens. Nuvvula S et al. [10] and Marya CM
et al. [1] reported similar cases of hypohyperdontia along
with agenesis of 18, 28 and 48. Other than the eight cases
described above, our is reported to be the ninth case with
coexistent hypohyperdontia in mandibular anterior re-
gion along with congenitally missing 18, 28, 38 and 48
to the best of our knowledge.
4.1. Case History
A 9-year-old male reported to the department of pedo-
dontics and preventive dentistry with a chief complaint
of blackish stains in his maxillary and mandibular teeth
for the previous 3 to 4 months. A history of presenting
illness revealed that there were moderate amounts of
blackish stains in maxillary and mandibular teeth, as the
patient was not maint ai ni ng hi s oral hygi ene properly. He
brushes his teeth once a week using a horizontal brushing
technique. There was no history of trauma, infection or
metabolic disorders in his childhood. This was the pa-
tient’s first dental visit with no prior experience of hav-
ing undergone any dental proced ure. Medical history and
family history was non-contributory. The patient was the
only child, born to parents of a non-consangious mar-
riage. On complete physical examination, no syndromic
features were noticed. The boy was of normal build and
height and well oriented to time and place with a normal
I.Q. All the vital signs were within normal limits.
Haematological and serological values were within
normal limits. Ultrasound examination of the abdomen
did not reveal any abnormality of the kidney, liver, intes-
tine, genitourinary tract etc.
4.2. Intra-Oral Examination
Soft tissue examination showed pinkish red, firm, resil-
ient gingivae with presence of stippling. Hard tissue ex-
amination revealed the absence of the mandibular central
incisors bilaterally and presence of a supplemental su-
pernumerary tooth in the midline (Figure 1).
The morphology of the clinical crowns of the two in-
cisors present and the absence of spacing between the
incisors and canines indicated the congenitally missing
central incisors.
4.3. Radiographs
An intra-oral periapical radiograph of the lower anterior
region showed the absence of 31 and 41. There was a
supplemental mesiodens with complete root formation,
with no evidence of any pathologic periapical changes
(Figure 2).
The close approximation of the roots of the mesiodens
and incisors, and no spacing between the incisors and
canines, highlighted the absence of the central incisors.
An orthopantomogram confirmed the absence of both the
mandibular central incisors along with the presence of a
mesiodens in the midline. Other findings in the ortho-
pantomogram were within normal limits considering the
age of child (Figure 3).
4.4. Diagnosis
Based upon the history and clinical examination, a pro-
visional diagnosis of mandibular hypo- and hyperdontia
was made and the differential diagnosis of orodigitofa-
cial dysostosis, Hallerman Streiff and Cleidocranial dys-
Figure 1. Intra-oral photograph showing the absence of man-
dibular central incisors bilaterally and presence of a conical
shaped mesiodens.
Copyright © 2013 SciRes. OPEN ACCESS
K. G. Verma et al. / Open Journal of Stomatology 3 (2013) 37-41 39
Figure 2. Intraoral periapical radiograph showing
the conical shaped mesiodens with complete root
formation without any evidence of pathologic
periapical chang es.
Figure 3. Orthopantomogram showing the mesiodens in ap-
proximation with mandibular lateral incisors and no other sig-
nificant findings.
plasia syndrome were considered. Based upon the history,
clinical features and radiological investigations the final
diagnosis of mandibular anterior hypohyperdontia was
4.5. Treatment
A full mouth prophylaxis with scaling and removal of
calculus and stains was performed. The treatment of
choice here would have been to restore the mesiodens to
the appearance of a normal incisor. Although this would
have been a centrally placed “incisor” the alignment in
the arch would not have appeared abnormal. This ap-
proach to treatment was fully explained to the patient’s
parents and the cosmetic recontouring of mesiodens was
done (Figure 4). Oral hygi ene instruction s w ere gi ven.
Figure 4. Intra-oral photograph showing complete oral pro-
phylaxis and cosmetic recontouring of mesiodens.
4.6. Follow-Up
The patient was advised to give follow-up every six
months to assess his erupting dentition and oral health
Agenesis of teeth is the most common developmental
craniofacial anomaly in humans. Mutations in Genes
PAX9, MSX1 and AXIN2 have been implicated as
probable cause for the agenesis in non-syndromic hypo-
dontia [11]. The usual mode of inheritance of hypodontia
is autosomal dominant, but occasionally autosomal re-
cessive and X-linked and polygenic/multifactorial mod-
els of inheritance have also been reported [4]. Many termi-
nologies have been used in the past to describe this con-
dition, such as “concomitant hypodontia and hyperdon-
tia”, and oligopleiodontia [9]; however, now the pre-
ferred term is “hypo-hyperdontia” as suggested by Gib-
son [5] and reported by Anthonappa et al., [12]. Hypo-
hyperdontia is rare in isolation and has been associated
with over 50 syndromes, notably orodigitofacial dy-
sostosis, Hallerman Streiff, Cleidocranial dysplasia syn-
drome, Ellis van Creveld, Downs syndrome, cleft lip and
palate, and many others [12-14]. Agenesis of teeth is
more common in permanent dentition and is also more
prevalent in females (1.37:1; F:M) as reported by Polder
et al., in 2004 [15]. The case reported is recorded in
mixed dentition stage in a male child. Studies have
demonstrated that hypodontia tends to be more common
in mandible than maxilla. In Asian populations, the man-
dibular incisors are the most commonly missing teeth,
followed by the mandibular second premolars [16].
There is a tendency for dental agenesis to occur more
unilaterally than bilaterally. In the case reported herein
there were missing mandibular central incisors along
with agenesis of four third molars; however the super-
numerary tooth was a mandibular mesiodens, a rare oc-
currence by itself. The reported prevalence for hypo-
Copyright © 2013 SciRes. OPEN ACCESS
K. G. Verma et al. / Open Journal of Stomatology 3 (2013) 37-41
hyperdontia [12] from various studies has been calcu-
lated to range from 0.002% to 3.1%. Hypo-hyperdontia
does not usually manifest in the same arch and very
rarely in the same area of an arch [6,12]. Patients with
hypo-hyperdontia usually do not present any symptoms
and are usually detected during examination for other
causes or on radiographic examination of the jaws [10,
17,18]. The current case was only recognised when the
patient came for oral prophylaxis. When any numeric
anomaly of the dentition is noted, a thorough clinical
intra-oral examination is warranted, combined with a
judicious use of radiographs. Panoramic radiographs are
the best screening modality available by virtue of dem-
onstrating the entire teeth-bearing segment of the jaws
and supporting structures in a single image [19]. Addi-
tional information about the root morphology and root
development can be obtained using periapical views. In
the present case, the age of the patient (9 years) pre-
cluded any possibility o f late development of at least the
mandibular central incisors, as some radiographic evi-
dence would have been noted. Having stated this it will
still have to be emphasised that in most cases supernu-
merary as well as unerupted teeth go undetected and cli-
nicians have to make the effort to determine these condi-
tions by a thorough clinical as well as radiographic ex-
amination. Such rare situations can at times significantly
alter the treatment plan and enough latitude should be
considered in managing such clinical circumstances.
Due to the rarity of combined hypodontia of the man-
dibular incisors and the presence of the mandibular me-
siodens, treatment of affected children is generally un-
dertaken. Multidisciplinary treatment planning, which tak e s
account of established and emerging techniques, needs to
be practiced [8]. Different treatment options that take
account of growth and development of the dentition and
of the compliance of child can lead to a treatment plan
that can produce desirable interim results, which do not
compromise any future treatment. Careful treatment plan-
ning is importan t, becaus e th ere is a ne ed to deal with not
only the immediate but also the long-term adverse im-
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