Non-syndromic occurrence of true generalized microdontia with mandibular mesiodens - a rare case.

CASE REPORT Open Access
Non-syndromic occurrence of true generalized
microdontia with mandibular mesiodens - a
rare case
Seema D Bargale* and Shital DP Kiran
Abstract
Abnormalities in size of teeth and number of teeth are occasionally recorded in clinical cases. True generalized
microdontia is rare case in which all the teeth are smaller than normal. Mesiodens is commonly located in maxilary
central incisor region and uncommon in the mandible. In the present case a 12 year-old boy was healthy; normal
in appearance and the medical history was noncontributory. The patient was examined and found to have
permanent teeth that were smaller than those of the average adult teeth. The true generalized microdontia was
accompanied by mandibular mesiodens. This is a unique case report of non-syndromic association of mandibular
hyperdontia with true generalized microdontia.
Keywords: Generalised microdontia, Hyperdontia, Permanent dentition, Mandibular supernumerary tooth
Introduction
Microdontia is a rare phenomenon. The term microdontia
(microdentism, microdontism) is defined as the condition
of having abnormally small teeth [1]. According to Boyle,
“in general microdontia, the teeth are small, the crowns
short, and normal contact areas between the teeth are fre-
quently missing” [2] Shafer, Hine, and Levy [3] divided
microdontia into three types: (1) Microdontia involving
only a single tooth; (2) relative generalized microdontia
due to relatively small teeth in large jaws and (3) true gen-
eralized microdontia, in which all the teeth are smaller
than normal. According to these authors, aside from its
occurrence in some cases of pituitary dwarfism, true gen-
eralized microdontia is exceedingly rare. Microdontia of a
single tooth can be further classified into (1) microdontia
of the whole tooth, (2) microdontia of the crown of the
tooth, and (3) microdontia of the root alone [4].
Involvement of the entire dentition is rare and been
reported in radiation or chemotherapeutic treatment dur-
ing the developmental stage of the teeth [5], pituitary
dwarfism [3] and Fanconi’s anemia [6]. The syndromes
associated with microdontia are Gorlin-Chaudhry-Moss
syndrome, Williams’s syndrome, Chromosome d/u, 45X
[Ullrich-Turner syndrome], Chromosome 13[trisomy 13],
Rothmund-Thomson syndrome, Hallermann-Streiff, Oro-
faciodigital syndrome (type 3), Oculo-mandibulo-facial
syndrome, Tricho-Rhino-Phalangeal, type1 Branchio-
oculo-facial syndrome.
Supernumerary teeth are defined as any supplementary
tooth or tooth substance in addition to usual configuration
of twenty deciduous and thirty two permanent teeth [7].
Classification of supernumerary teeth may be based on
position or morphology. Positional variations include ante-
rior mesiodens, para-premolars, para-molars and disto-
molars. Variations in morphology consist of supplemental
and rudimentary types [8].
Supernumerary teeth are common in the maxillary ante-
rior region although supernumerary teeth have been
reported in the incisor region of the mandible are very
rare. Although supernumerary teeth have been reported in
the incisor region of the mandible, they are very rare
[9-14].
Conditions, in which supernumery teeth found, are
cleidocranial dysplesia, cleft lip and cleft palate [15]. Syn-
dromes associated with supernumery teeth are Familial
adenomatous polyposis [Gardner’s], Apert, Klippel-Tren-
aunay-Weber, Craniometaphyseal dysplasia, Trisomy 21
[Down’s], Nance-Horan, Orofaciodigital syndrome (type
3), Sturge-weber and Tricho-Rhino-Phalangeal, type1.
* Correspondence: drseemabargale@gmail.com
Department of Pedodontics and Preventive Dentistry, AECS Maruthi Dental
College and Research Center, India
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reproduction in any medium, provided the original work is properly cited.

In the case described here is a bizarre generalized
microdontia involving the entire dentition along with
mandibular mesiodens without any other apparent
systemic conditions.
Case Report
The patient was a 12 year old boy, only child of consan-
guineous parents, reported to the department of pedo-
dontics and preventive dentistry with the complaint of
small teeth. Parents noted small teeth ever since the
eruption of permanent teeth. No abnormalities were
reported, however, in their extended family.
Physical examination
Physical growth was within normal limits. The patient was
of normal in stature, appearance, height, and weight for
his age. Upon examination of the limbs, hands, skin, hair,
nails and eyes were all appeared normal. No abnormality
was noted in neck, back, muscles, cranium and joints as
well. Intellectual and scholastic performance was also nor-
mal. His medical history was unremarkable; no other
abnormalities were noted in the history apart from the dif-
ficult delivery. The child was examined and found to be
free of any gross abnormalities.
His blood profile was normal. Serum calcium, phosphor-
ous and alkaline phosphatase levels were also normal.
Endocrinological investigation was carried out to rule in
or out the possibility of hormonal disorder, and the results
were within normal limits.
Intraoral examination
The intraoral soft tissues were healthy, but the teeth were
abnormal in size and shape (Figure 1 and 2). Diagnostic
casts were obtained to aid in diagnosis (Figure 3). Patient
was in permanent dentition, teeth present were small in
size. The patient had normal occlusion with excessive
spacing between the teeth. Fully erupted mandibular
mesiodens was present between the central incisors.
The anterior teeth lacked normal size in all dimensions.
Most of the anterior teeth were “peg-shaped” without the
typical variation in mesiodistal and labiolingual dimen-
sions. Almost all the maxillary anterior teeth did not have
lingual pits whereas mandibular central and lateral incisors
had prominent pits on the lingual surfaces. The posterior
teeth were also small and exhibited a short occlusogingival
dimension. Overall, the dentition was smaller than that of
the average adult (Table 1 and 2). Orthopantomogram or
the Intra oral periapical radigraph could not be taken
because the patient was not able to afford.
The simultaneous presence of microdontia and
supernumery teeth is been reported in the Cleidocra-
nial dysplasia, Craniometadiaphyseal dysplasia, Der-
moodontodysplasia, Hypodontia and nail dysgenesis,
Orofaciodigital syndrome type 3 and Tricho-rhino-pha-
langeal syndrome type 1. However in this case, except
for the dental abnormality in the form of generalized
microdontia and the presence of fully erupted mandib-
ular mesiodens between the central incisors were
found and no other clinical features observed, there-
fore all the syndrome associated with the simultaneous
presence of microdontia and supernumery teeth were
ruled out along with Taurodontism, microdontia, and
dens invaginatus as well as Distal symphalangism,
Figure 1 Intra oral view of the upper arch.
Figure 2 Intra oral view of the lower arch.
Figure 3 Diagnostic casts showing the morphology of the
teeth.
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hypoplastic carpal bones, microdontia, dental pulp
stones, narrowed zygomatic arch (Table 3).
A diagnosis of non-syndromic occurance of true gen-
eralized microdontia with mandibular mesiodens was
made as no systemic condition was observed. The fully
erupted mandibular mesiodens was extracted under
local anesthesia in order to correct midline and to facili-
tate the orthodontic treatment.
Discussion
The initiating factor or factors responsible for microdon-
tia remain obscure. Mutation in developmental regularity
genes are known to cause variety of dental defects [16].
Both genetic and environmental factors are involved in
the complex etiology of microdontia. Genetic factors
probably play a role in the formation of microdontia.
Although the proband was the only child, the presence of
consanguinity in the form of both parents being maternal
first cousins could suggest recessive or polygenic
inheritance.
The development of a tooth has been shown to have
ectodermal, mesodermal, and neural crest contributions.
The variation in size of a particular tooth arises during the
period when the form of the tooth is being determined by
the enamel organ and the sheath of hertwig at the bell
stage of enamel organ. The determination of the form of
the crown is thought to be related to different regions of
the oral epithelium or to the ectomesenchyme. Studies
have shown that different regions of the oral epithelium
rather than the underlying ectomesenchyme are initially
responsible for the shape of the crown [17]. Bones dating
from the Middle Ages which were excavated at Alborg,
Denmark proved evidence for generalized microdontia
resulting from intrauterine growth retardation [18].
On the basis of visual documentation, the patient in the
current case seems to have been more severely affected in
all his teeth which exhibited aberrant morphology and all
were smaller than normal. MEDLINE search in the Eng-
lish dental literature for true generalized microdontia
revealed zero search results. Although child’s mother had
Table 1 Comparison of buccolingual/labiolingual and mesiodistal crown dimensions with an anatomic average* of the
right side maxillary and mandibular teeth
Right side Central incisor Lateral incisor Canine First premolar Second premolar First molar Second molar Total
Maxillary MD
LL
MD
LL
MD
LL
MD
BL
MD
BL
MD
BL
MD
BL
MD
LL/BL
Average 8.5
7.0
6.5
6.0
7.5
8.0
7.0
9.0
7.0
9.0
10.0
11.0
9.0
11.0
55.5
61.0
Patient 7.5
6.1
4.7
5.6
6.9
7.4
6.4
7.8
6.5
7.7
9.5
10.4
8.7
10.9
50.2
55.9
Mandible MD
LL
MD
LL
MD
LL
MD
BL
MD
BL
MD
BL
MD
BL
MD
LL/BL
Average 5.0
6.0
5.5
6.5
7.0
7.5
7.0
7.5
7.0
8.0
11.0
10.5
10.5
10.0
53.0
56.0
Patient 4.4
5.6
5.2
5.8
6.6
7.1
6.7
7.3
6.8
7.7
10.7
10.3
9.9
9.4
50.3
53.2
Measurements in millimeters were taken at widest portion of clinical crown on diagnostic casts. *Anatomic average taken from Wheeler, R. C.: Textbook of Dental
Anatomy and Physiology, ed. 7, Philadelphia, 1993, W. B. Saunders Company, pp. 25.
Table 2 Comparison of buccolingual/labiolingual and mesiodistal crown dimensions with an anatomic average* of the
left side maxillary and mandibular teeth
Left side Central incisor Lateral incisor Canine First premolar Second premolar First molar Second molar Total
Maxillary MD
LL
MD
LL
MD
LL
MD
BL
MD
BL
MD
BL
MD
BL
MD
LL/BL
Average 8.5
7.0
6.5
6.0
7.5
8.0
7.0
9.0
7.0
9.0
10.0
11.0
9.0
11.0
55.5
61.0
Patient 7.2
5.8
4.5
5.4
7.1
7.5
6.2
7.6
6.3
7.7
9.3
10.2
8.7
10.9
49.3
52.1
Mandible MD
LL
MD
LL
MD
LL
MD
BL
MD
BL
MD
BL
MD
BL
MD
LL/BL
Average 5.0
6.0
5.5
6.5
7.0
7.5
7.0
7.5
7.0
8.0
11.0
10.5
10.5
10.0
53.0
56.0
Patient 4.2
5.7
5.3
6.1
6.7
7.3
6.7
7.2
6.8
7.7
10.7
10.2
9.6
8.9
50.0
53.1
Measurements in millimeters were taken at widest portion of clinical crown on diagnostic casts. *Anatomic average taken from Wheeler, R. C.: Textbook of Dental
Anatomy and Physiology, ed. 7, Philadelphia, 1993, W. B. Saunders Company, pp. 25.
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Table 3 Comparison of conditions associated with the simultaneous presence of microdontia and supernumery teeth along with taurodontism, microdontia,
and dens invaginatus as well as distal symphalangism, hypoplastic carpal bones, microdontia, dental pulp stones, narrowed zygomatic arch.
Taurodontism,
microdontia, and
dens invaginatus
Cleidocranial
dysplasia
Craniometadiaphyseal
dysplasia
Dermoodontodysplasia Hypodontia and
nail dysgenesis
Orofaciodigital
syndrome type 3
Tricho-rhino-
phalangeal
syndrome type
1
Distal symphalangism, hypoplastic
carpal bones, microdontia, dental pulp
stones, narrowed zygomatic arch
Generalized
microdontia
Autosomal
dominant
Autosomal recessive Autosomal dominant Autosomal
dominant
Autosomal
recessive
Autosomal
dominant
Autosomal dominant
Taurodontism of
first permanent
molars
Chromosome 6 Macrocephaly Dry skin Chromosome 4 Thin/hyperconvex/
hypoplastic nails
Autosomal
recessive
Absent/small nails
Multiple teeth with
one or more dens
invaginatus
Arm p Frontal bossing Ichthyosis Arm p Prominent occiput Chromosome 8 Microdontia
X-linked recessive
inheritance
Normal height
(with skeletal
dysplasia)
Large fontanelle Thin skin Dry skin Frontal bossing Arm q Other dental abnormality
Short stature -
postnatal
Prominent eyes Pigmented naevi Fine hair Round face Normal height
(with skeletal
dysplasia)
Abnormal clinical features of the limbs
Absent/small nails Mandibular
hyperostosis/sclerosis
Abnormal hair texture Brittle hair/
trichorrhexis
nodosa/pili torti
Hypertelorism Short stature -
postnatal
Brachydactyly
Macrocephaly Optic nerve
abnormality/atrophy
Sparse/absent scalp hair
- localised
Sparse/absent
scalp hair -
generalised
Down-slanting
palpebral fissures
Decreased body
hair/hypotrichosis
Irregularities of length/shape of fingers
Flat occiput
(brachycephaly)
Microdontia Abnormal nails Absent/small nails Other orbital
abnormality
Decreased hair
pigmentation -
general
Syndactyly of fingers
Frontal bossing Abnormal tooth
position/malocclusion/
open bite
Midface hypoplasia/flat/
short midface
Thin/
hyperconvex/
hypoplastic nails
Paresis of ocular
muscles/squint
Decreased hair
pigmentation -
patchy
Short foot (including brachydactyly)
Wide sutures/
delayed fusion of
sutures
Missing permanent
teeth/retained
deciduous teeth
Micrognathia/agnathia/
retrognathia
Dysplastic/
grooved/thick/
discoloured nails
Other eye
movement
disorder
Fine hair Syndactyly (other than minimal 2nd and
3rd toes)
Large fontanelle Anodontia/oligodontia Microdontia Depressed
premaxillary
region
Broad/bulbous
nasal tip
Brittle hair/
trichorrhexis
nodosa/pili torti
Irregular length or shape of toes
Facies significantly
abnormal
Natal/neonatal teeth Anodontia/oligodontia Midface
hypoplasia/flat/
short midface
Cleft soft palate/
bifid uvula/
submucous cleft
Sparse/absent
scalp hair -
generalised
Other skull abnormality
Small face Supernumerary teeth Supernumerary teeth Micrognathia/
agnathia/
retrognathia
Microdontia High hairline -
front
Absent/small/hypoplastic carpals
Hypertelorism Dental caries Other dental
abnormality
Absent/decreased
eyebrows/lateral
thinning
Abnormal tooth
position/
malocclusion/open
bite
Thin/
hyperconvex/
hypoplastic nails
Symphalangism
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Table 3 Comparison of conditions associated with the simultaneous presence of microdontia and supernumery teeth along with taurodontism, microdontia,
and dens invaginatus as well as distal symphalangism, hypoplastic carpal bones, microdontia, dental pulp stones, narrowed zygomatic arch. (Continued)
Prominent
supraorbital ridges
Low set ears Simian creases Absent/decreased
lashes
Supernumerary
teeth
Dysplastic/
grooved/thick/
discoloured nails
Cone shaped epiphyses
Depressed
premaxillary
region
Scoliosis Dislocated hip Everted/
protruding lips
Cleft/notched
tongue
Broad/bifid nails Symphalangism
Midface
hypoplasia/flat/
short midface
Bowed limbs Tooth crown
shape
abnormality
Hamartoma/other
tumours of the
mouth
Frontal bossing Cone-shaped epiphyses of middle
phalanges
Prognathism Mental retardation of
any degree
Microdontia Other abnormality
of tongue/
gingivae/mucosa
High forehead
Depressed nasal
bridge
Boney sclerosis of any
type
Abnormal tooth
position/
malocclusion/
open bite
Low set ears Facies
significantly
abnormal
Paramedian/lateral
cleft lip (uni/
bilateral)
Multiple fractures/
increased boney fragility
Delayed eruption
of teeth
Tragus abnormal Long face
Cleft soft palate/
bifid uvula/
submucous cleft
Enchondroma/
radiolucencies -
localized
Anodontia/
oligodontia
Pectus excavatum
(funnel chest)
Grooved/dimpled
chin
High vaulted and
narrow palate
Lytic/lucent lesions of
bone
Supernumerary
teeth
Abnormally placed
nipples
Micrognathia/
agnathia/
retrognathia
Microdontia Fibrous dysplasia of
bone
Thoracolumbar
general kyphosis
Medial flare of
eyebrows
Developmental
defect of enamel
Wide diaphyses
(undertubulation)
Irregularities of
length/shape of
fingers
Absent/decreased
eyebrows/lateral
thinning
Tooth
discolouration
Submetaphyseal
undermodelling/
expansion
Syndactyly of
fingers
Absent/decreased
lashes
Delayed eruption
of teeth
Thin cortex of diaphyses Polydactyly -
postaxial (ulnar)/
type unspecified
Long/large nose
Missing
permanent teeth/
retained
deciduous teeth
Bowing of long bones Abnormal palmar
dermatoglyphics/
skin creases
Broad nasal
bridge (see
telecanthus)
Supernumerary
teeth
Cartilage tongues of
metaphyses - localized
Polydactyly of feet
- postaxial/type
unspecified
High nasal bridge
Dental cysts/
tumours
Hyperostosis/thickened/
sclerotic calvarium
Syndactyly (other
than minimal 2nd
and 3rd toes)
Broad/bulbous
nasal tip
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