PEDIATRIC DENTISTRY V 42 / NO 1 JAN / FEB 20

CASE REPORT

Management of Two Cases of Supernumerary Teeth

Allison Scully, DDS, MS1 • Hong Zhang, PhD2 • Hera Kim-Berman, DDS, MS3 • Erika Benavides, DDS, PhD4 • Nina C. Hardy5 • Jan C-C. Hu, BDS, PhD6

Abstract: Supernumerary teeth are commonly observed as an isolated developmental anomaly. While the familial tendency of supernumerary
teeth has been documented, its genetic causality has not yet been determined. The purpose of this paper was to presents two cases with super-
numerary teeth and the process leading to the diagnosis and determination of their underlying conditions. Cases were evaluated and family
histories reviewed. Genetic counseling was recommended for the probands and followed by genetic testing of selected family members. The
proband of family one, who has multiple supernumerary teeth, was determined to have a RUNX2 missense mutation (c.379C>T, p.Pro127Ser) and
diagnosed with cleidocranial dysplasia. The proband of family two, who has a premolar region supernumerary tooth, was reported to have no
bone defects also presented with a RUNX2 missense mutation (c.1381G>C, p.Gly461Arg). When patients present with multiple supernumerary teeth,
a recommendation and guidance to genetic counseling and testing may facilitate accurate diagnosis and management. (Pediatr Dent 2020;
42(1):58-61) Received September 3, 2019 | Last Revision October 16, 2019 | Accepted October 18, 2019
KEYWORDS: CLEIDOCRANIAL DYSPLASIA, GENE, HEREDITY, HYPERDONTIA, PERMANENT TEETH

Anomalies of the dentition often alter the number, size, or
shape of teeth. Hyperdontia, or supernumerary teeth, occurs
when there is at least one extra tooth present in the jaw. 1,2
Hyperdontia occurs less frequently than hypodontia (missing
teeth) in the permanent dentition; the anterior maxilla is most
often involved.3,4 Supernumerary teeth can pose adverse effects
on adjacent teeth, including delayed or failure of eruption,
malpositioning of permanent teeth, over-retention of primary
teeth, resorption of roots, ectopic eruption, and dentigerous
cysts. 5 Accurate diagnosis and carefully planned removal of
supernumerary teeth are crucial to the management of devel-
oping patients.
The reported incidence of supernumerary teeth varies from
0.4 to 3.0 percent, with a male-to-female ratio of 1.5 to one.6-8
In 33 percent of cases, a supernumerary tooth in the primary
dentition is followed by a supernumerary tooth complement in
the permanent dentition.9 In a retrospective case series of 767
four- to 51-year-olds, 74.7 percent were of Hispanic descent
and 20.5 percent had first-degree relatives with supernumerary
teeth.10 Most cases of supernumerary teeth are thought to be
idiopathic in origin, with 80 percent having a single tooth
and 15 to 20 percent having two teeth. 11 Only 0.06 percent
of cases of idiopathic supernumerary teeth have five or more
extra teeth present.
1Dr. Scully an assistant professor, Department of Pediatric Dentistry, Indiana University
School of Dentistry, Indianapolis, Ind., USA. 2Dr. Zhang a postdoctoral fellow, Dental
Research Lab; 3Dr. Kim-Berman an associate professor, Department of Orthodontic and
Pediatric Dentistry and 4Dr. Benavides a professor, Department of Periodontics and Oral
Medicine; and 6Dr. Hu a professor, Department of Biologic and Materials Sciences &
Prosthodontics, all at the University of Michigan School of Dentistry, Ann Arbor, Mich.,
USA. 5Ms. Hardy is a sophomore at the College of Arts and Sciences, Boston University,
Boston, Mass., USA.
Correspond with Dr. Scully at scullya@iu.edu
HOW TO CITE:
Scully A, Zhang H, Kim-Berman H, Benavides E, Hardy NC, Hu JCC.
Management of Two Cases of Supernumerary Teeth. Pediatr Dent
2020;42(1):58-61.
Supernumerary teeth have been theorized to have a genetic
component. 12,13 Many cases with supernumerary teeth are
reported to have additional anomalies, so when multiple super-
numerary teeth are present, they are more likely to be related to
an underlying syndrome. The condition of supernumerary teeth
(Online Mendelian Inheritance of Man [OMIM] 187100) is
present in more than 50 syndromes, suggesting the complexity
of the genetic etiology. The most common syndromes asso-
ciated with supernumerary teeth include cleidocranial dysplasia
(involving RUNX2 gene) and familial adenomatous polyposis
(APC ).11 RUNX2 encodes a transcription factor involved in
the formation of teeth, bones, and cartilage, and mutations
in this gene are associated with multiple supernumerary teeth
and cleidocranial dysplasia.11 Additional syndromes and their
causative genes associated with supernumerary teeth include but
are not limited to Rubinstein-Taybi syndrome 1 and 2 (CREBBP,
EP300), craniosynostosis and dental anomalies (IL11RA),
microphthalmia and esophageal atresia syndrome (SOX2), and
Nance-Horan syndrome (NHS).
Theories for causes of a single supernumerary tooth in-
clude local hyperactivity of the dental lamina, dichotomy of the
tooth bud, and other molecular mechanisms during early tooth
development.14
Case description
Two cases presented for routine dental care were reported as
simplex cases of supernumerary teeth upon initial examination.
The proband from family one had not received comprehen-
sive medical or dental work prior to her dental visit, while the
proband of family two, who had no remarkable medical or
dental history, was seeking orthodontic care to improve occlu-
sion. Upon clinical and radiographic exam, the parents were
informed of the finding of supernumerary teeth. The clinicians
reviewed the patients’ signs and symptoms by referencing the
OMIM, requested a consult from the dental research lab,
and worked with the patients’ physicians to determine a
definitive diagnosis. The study protocol and subject consent
used by the dental research lab were reviewed and approved
by the institutional review board of the University of Michigan
School of Medicine, Ann Arbor, Mich., USA.

58 CASES WITH SUPERNUMERARY TEETH

 PEDIATRIC DENTISTRY V 42 / NO 1 JAN / FEB 20

Figure 1. Family one proband radiograph, cone beam computed tomography (CBCT) images, pedigree, and chromatograms. (A) Panoramic
radiograph of proband at age 11. There is a general delay of primary tooth exfoliation and permanent tooth eruption. The tooth roots, pulp
chamber, and root canals are within normal limits; * denotes mesiodens. CBCT revealed supernumerary teeth in the (B) maxillary incisor region;
(C) mandibular premolar region; (D) right premolar regions; (E) left premolar regions; (F) maxillary right canine region; (G) maxillary left canine
region; and (H) anterior mandible region showing a fused supernumerary tooth on the left (white arrow). (I) The family pedigree was constructed
based on an interview of subject II:4. Both the mother and the affected child participated in the study. Maternal grandmother was reported to
have tooth problems, but no specifics were confirmed. Subjects II:1 and II:2 were reported to have normal dentition. Subject II:4 was reported
to have extra premolars extracted at age 12 and extra molars extracted at age 20. Subject II:7 was reported to have one extra maxillary canine.
The proband has 16 supernumerary teeth at various developmental stages. Chromatogram of (J) affected mother and (K) affected proband
showing the sequence variant of RUNX2, NM_001024630.3: c.379C>T; NP_001019801.3:p.(Pro127Ser).

Figure 2. Family two pedigree and proband radiographs. (A) Panoramic radiograph revealed a single supernumerary tooth, distal to the mandibular
second premolar #29, in crown formation stage (Nolla’s stage five crown almost completed). All the third molars are present but not yet erupted.
(B) Lateral cephalometric film demonstrated normal jaw size and anterior-posterior position. No sign of widening cranial sutures or Wormian
bones. (C) Pedigree shows a simplex case with no other affected individual in the immediate family. Both parents and the proband participated
in the study. Chromatogram of (D) father, (E) mother, and (F) proband showing the sequence variant of RUNX2, NM_001024630.3:c.1381G>C;
NP_001019801.3:p.(Gly461Arg).

CASES WITH SUPERNUMERARY TEETH 59

PEDIATRIC DENTISTRY V 42 / NO 1 JAN / FEB 20
The proband of family one, a Hispanic girl, was eight
years, nine months old when she first presented to the pediatric
dental clinic. The mother reported that the patient had devel-
opmental, speech, and cognitive delays. Physically, the proband
and her mother were below average in height. The proband
presented with a brachyfacial appearance with a concave profile
and hypoplastic midface. She also had slight frontal bossing
and hypertelorism. The proband’s hands were noted to be small
and have short fingers in relation to her palm. She was referred
from a general dentist for the extraction of primary molar tooth
#K. The maxillary occlusal radiograph showed the presence
of two mesiodens. It was recommended that teeth #D, #E, #F,
and #G be extracted to allow for potential eruption of the
mesiodens. The recommended extractions were completed;
however, there was no evidence of eruption of the mesiodens
or the permanent incisors after 13 months. At the recall appoint-
ment, a panoramic radiograph was taken that revealed multiple
supernumerary teeth in both jaws (Figure 1A, Table). The
patient’s mother was further consulted. She denied any family
history of syndromes but stated that she had extra premolar
and molar teeth removed at 12 and 20 years old. She also indi-
cated that her sister had an extra canine in her mandible. At
this time, referrals were made for the patient to have a cone
beam computed tomography (CBCT) scan taken by the radio-
logy department and a comprehensive evaluation by the oral
and maxillofacial surgery department. The CBCT covering the
maxilla, mandible, and maxillary sinuses was obtained using
the 3D Accuitomo 170 (J. Morita USA, Irvine, Calif., USA).
The image was taken in one-mm cross-sections and recon-
structed. Sixteen supernumerary teeth were identified. One
of the supernumerary teeth was fused with tooth #27 (Figures
1B-H). The patient was noted to have Class III malocclusion
with maxillary deficiency. Other notable findings included
visible spheno-occipital synchondroses and aplasia of the
sphenoid sinus. The radiologist recommended a medical evalu-
ation to rule out cleidocranial dysplasia.
A phone interview with the family was conducted. The
mother’s questions about the potential disorder, genetic study,
and consent forms were answered. A family pedigree was
constructed according to the mother’s report (Figure 1I). At
this time, the proband was 11 years, two months old. Her
weight and height were 42.5 kg (70th percentile) and 135 cm
(10th percentile). The CBCT scan was effective in identifying
the number and three-dimensional locations of supernumerary
teeth. Removal of supernumerary teeth was to be carried out
in phases. Orthodontic consultation was initiated for the
Table. LOCATION OF PROBANDS’ TEETH. NUMBERS IN THE MAXILLA AND MANDIBLE
ROWS REPRESENT UNERUPTED TEETH. NUMBERS IN THE CASE 1 AND CASE 2
ROWS REPRESENT ERUPTED TEETH
Maxilla 1 2 4 *5* 6* 7 9
Case 1 3 A B C 8* * *H* I
30 T* S* R* 26 25 24 23 M* 21
Mandible 32 31 29 28 27*
Maxilla 1 16 RUNX2 Gly461 is located in a highly conserved
Case 2 2 3 4 5 6 7 8 9
31 30 29* 28 27 26 25 24 23 22 21 20 19 18
Mandible 32
“*” denotes the location of each supernumerary tooth associated with the indicated permanent or primary tooth. KAT6B interactions cannot be predicted
60 CASES WITH SUPERNUMERARY TEETH
evaluation of mid-face deficiency and management of delayed
eruption and malocclusion. Following the recommendation,
the mother brought the child to a pediatrician who ordered
genetic counseling and confirmed the diagnosis of cleidocranial
dysplasia. Unfortunately, the patient did not follow-up after
completion of initial treatment at the pediatric dental clinic.
The proband of family two was a 16-year-old Asian boy
of average height and weight (50 th percentile). During the
initial workup, he was determined to have normal cephalic
with Class I malocclusion and minor crowding. A supernu-
merary tooth (29s) was detected on his panoramic radiograph
(Figure 2A). The patient was referred to an oral surgeon for the
extraction of 29s and to the dental research lab for consulta-
tion. The family was interviewed by phone and consented to
participate in the genetic study. The father reported that no
other family members have supernumerary teeth. Careful exam-
ination of the panoramic and lateral cephalometric radiographs
revealed a straight lateral profile, no frontal bossing, no cranial
suture widening, and no Wormian bones (Figure 2B). The
father was unaware of any growth or developmental anomalies
of the boy. Orthodontic treatment was subsequently initiated.
Research-based genetic analysis was conducted at the dental
research lab using subject saliva samples from which genomic
DNA was extracted and subjected to whole-exome sequencing
and validation. The proband of family one was determined to
have a single allele RUNX2 c.379C>T change that resulted in
a substitution of Pro 127 with Ser (p.Pro127Ser), a damaging
mutation. This single nucleotide change has a SIFT score of
0.002 and Polyphen 2 score of 0.966, indicative of a damaging
mutation. Pro 127 is highly conserved and is never a Serine
among the known sequences from other species (Figure 1J-K).
This change is also present in the proband’s mother, although
the mother was never diagnosed with a medical condition.
The proband of family two has a single allele RUNX2
c.1381G>C change that resulted in a substitution of Gly 461
with Arg (p.Gly461Arg), a potentially damaging variant (Figure
2C-F). This single nucleotide change has a SIFT score of zero
and Polyphen 2 score of one, indicative of a damaging variant.
Discussion
The affected girl from family one at age 11 years, two months
had a short stature, body weight of 42.5 kg (70th percentile),
height of 135 cm (10th percentile), and multiple supernumerary
teeth with severe maxillary hypoplasia. RUNX2 Pro127 is located
within the Runt domain (residues 101 to 229 of the protein),
which confers DNA binding ability and mediates the interaction
with the core-binding factor beta to increase
the DNA binding affinity. Altered DNA
binding ability will likely result in changes in
gene transcription and cell function.
The affected boy from family 2 was of
10 11* 12 13 15 16 normal stature and has one supernumerary
J 14
tooth in his right mandible. His lateral
cephalometric film revealed normal jaw size
19 and anteriorposterior position without appar-
22 ** 20* 18 17 ent deficiencies of surrounding skull bones.
region of the protein that interacts with
10 11 12 13 14 15
histone acetyltransferase KAT6B. 15 While
RUNX2 Gly 461 is highly conserved, which
17 typically infers functional significance, the
downstream effect of RUNX2 Gly 461 and

based on available databases. The father, who also has this
variant, is of average height and reported to have no supernu-
merary teeth. This observation suggested variable expressivity
of RUNX2 (p.Gly461Arg) mutation.
While cases of various ethnic backgrounds with premolar
region supernumerary teeth have been reported, 16-19 the only
report of a potential genetic etiology for premolar region super-
numerary teeth considered single allele change of PDGFRB
(c. 2053C>T, p.R685C) to be responsible in a three-generation
Korean family with dominant inheritance of mandibular pre-
molar supernumerary teeth.20 The proband of family two does
not have any PDGFRB mutation that is predicted to be dama-
ging. The parents were recommended to follow up with the
boy’s physician. A thorough medical examination and clini-
cal genetic testing should be conducted in order to afford the
patient a definitive diagnosis.
In case one, the research-based genetic testing supported
the medical diagnosis and rendered the child eligible to receive
state support for the management of her medical and dental
needs. It is understandable that, following a definitive medical
diagnosis, the family may feel overwhelmed. Strong support
from health care providers and arranging to bring in social
workers to guide the family in leveraging resources, such as
additional medical and dental insurance coverage and travel
support, may make a huge difference to the affected child.
While the definitive diagnosis of case two relies on the physi-
cian’s order of a clinical genetic test, the family was made aware
of the importance to have the proband undergo a thorough
medical evaluation to define additional clinical phenotypes out-
side of the dentition.
Both pediatric dentists and orthodontists are care providers
in positions to discover growth and developmental anomalies
associated with their patients’ dentitions. When encountering
patients with genetic conditions, OMIM is an accurate and
frequently updated online resource. The dental research lab at
the University of Michigan School of Dentistry works with
affected families and health care providers to provide informa-
tion and conduct research-based genetic analyses. While many
dental anomalies are isolated, there are syndromic conditions
in which dental anomalies are the early manifestations that can
potentially lead to the correct diagnosis of the underlying dis-
orders. As the awareness of genetic testing and precision
medicine are heightened, the expectation on health care pro-
viders’ knowledge and ability to guide affected patients to proper
diagnosis and treatment may soon become a standard of care.
Acknowledgments
The authors wish to thank the families involved for their parti-
cipation in the study. This study was supported by NIDCR/
NIH research grant DE015846.
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CASES WITH SUPERNUMERARY TEETH 61