Bilateral maxillary canines agenesis:

a case report and a literature review

C. LOMBARDO*, E. BARBATO**, R. LEONARDI***

ABSTRACT. Background Agenesis of permanent canines is an uncommon condition that affects human teeth. In
fact, canine is the most variable positioned tooth, it can be found palatally or facially displaced or ectopically
erupted from the dental arch. On the contrary, canine agenesis is a rare finding in Caucasian populations, but
it may be relatively more common in Asian groups. This dental anomaly is firstly influenced by genetic, and less
often by systemic and environmental factors. Generally, it can occur in association with other dental
disturbances or as a solitary anomaly. Prevalence of agenesis permanent teeth varies between 4.5% and 7.4%
in general Caucasian populatio. Aim The purpose of this report is to discuss the aetiology, prevalence, diagnosis
and clinical implications of canine agenesis. Case report This paper reports an unusual case of bilateral,
congenitally missing canines in a healthy 10 year old Italian male.

KEYWORDS: Agenesis, Aplasia, Canines, Caucasian, Maxillary.

Introduction
Congenitally missing teeth (CMT) is an anomaly
characterised by the lack of one or more teeth. It may
result in dental malposition, periodontal damage, lack
of maxillary and mandibular bone height development,
aesthetic and functional consequences. This anomaly is
relatively common in permanent dentition for some
teeth (third molar, premolars and lateral incisors). The
prevalence of hypodontia varies according to evolutive
[Anderson et al., 1975], local [Stafne and Gibilisco,
1975; Kindelan et al., 1998] systemic [Sperber et al.,
1963] and genetic factors [Arte et al., 1996;
Goldenberg, 2000]. Recent reports have shown that in
the Caucasian population the prevalence of hypodontia
in permanent dentition (third molar excluded) is about
4.5-7.4%, and the most commonly missing tooth is the
mandibular second premolar [Backman and Wahlin,
2001; Nordgarden et al., 2002]. Permanent dentition
hypodontia can occur as a solitary anomaly or in
association with head and neck syndromes [Schalk,
*Department of Orthodontics. Catania Dental College. University of Catania.
Italy. PhD student.
**Department of Orthodontics. Rome Dental College. University “La Sapienza”.
Rome. Italy. Full Professor.
***Department of Pedodontics and Department of Orthodontics. Catania Dental
College. University of Catania. Italy. Associate Professor.
e-mail: rleonard@unict.it
van der Weide et al., 1993]. Often in this latter case
several teeth are missing, including the canine,
although with a lower frequency. On the other hand,
congenitally missing permanent teeth involving
maxillary canine, in nonsyndromic patient, are rare. In
fact very few reports have been found in literature
[Robertson, 1962; Lum and Lim, 1976; Leong et
Calache, 1999; Cho et al., 2004] that deal mostly with
maxillary canine agenesis.
This case report describes a bilateral canine agenesis
in an Italian boy. A literature review has been carried
out and clinical implications discussed.
Case report
A 10 year old Italian boy was referred to the
Orthodontic Clinic of the University of Catania (Italy)
for orthodontic treatment. He was in good health with
no history of maxillary trauma nor any kind of
syndrome. The clinical examination included
recording teeth and their morphology. He presented
the following teeth (fig. 1):
- on the upper right side, central and lateral
permanent incisors, deciduous canine, deciduous
molars, and first permanent molar;
- on the left upper side, central permanent incisor,
deciduous lateral incisor, canine and molars, first
permanent molar;
- on the lower left side, central and lateral permanent

EUROPEAN JOURNAL OF PAEDIATRIC DENTISTRY • 1/2007


incisors, deciduous canine, deciduous molars, and
first permanent molar;
- on the lower right side, central and lateral
permanent incisors, deciduous canine, first
permanent premolar, deciduous second molar and
first permanent molar.
His chief complaint was the agenesis of bilateral
maxillary canines associated with monolateral absence
of the left lateral incisor. The clinical history ruled out
any possibility of the missing canines having been
extracted or the patient having undergone dental
trauma that could have caused alteration or deviation
of the correspondent dental germs. The persistence of
the deciduous maxillary and mandibular canines could
be detected both clinically and radiographically (fig. 1,
2).
The upper deciduous canines and the left lateral
incisor displayed no initial process of radicular
resorption (fig. 2) in contrast to the lower elements that
were reabsorbed due to permanent teeth eruption. The
medical and dental family history was not contributory
for this kind of teeth agenesis.
One and a half year later this case was visited again:
FIG. 1 - Intraoral view of patient’s teeth. a) front view; b) lateral right view; c) lateral left view. Arrows show the deciduous
canines.
FIG. 2 - Orthopantomography shows aplasia of maxillary
canines bilaterally (arrows) and left lateral incisor. No
initial resorption can be detected.
EUROPEAN JOURNAL OF PAEDIATRIC DENTISTRY • 1/2007
BILATERAL MAXILLARY CANINES AGENESIS
the primary maxillary canines and lateral incisor
showed a slight degree of resorption, less than half of
the initial radicular length (fig. 3).
Discussion
The permanent maxillary canine, on its long eruptive
pathway, may often be affected by anomalies. It is
perhaps the most variably positioned tooth in human
dentition. It may be frequently displaced in the dental
arch either facially or palatally or, less often, deflected
mechanically and may undergo subsequent ectopic
drift resulting in transposition. On the contrary, canine
agenesis is a very rare condition. It has been reported
mainly in the Chinese population: Cho et al. (2004)
described the radiographic findings in 32 Chinese
children with congenitally missing permanent teeth
regarding only maxillary canines. Of the 32 cases,
only 9 were “bilateral”. However in the Caucasian
population it is a very rare kind of agenesis and, except
for a few published studies, this condition is not
clinically known. One of these studies reported only
five cases of congenitally missing permanent teeth
FIG. 3 - Twenty months later orthopantomographic x-ray
shows that root resorption of maxillary bilateral canines
had started.
39
C. LOMBARDO, E. BARBATO, R. LEONARDI
involving maxillary canines among 13.459 white
American children (0.037%), whereas two of such
cases were found out of 1481 black children (0.14%)
in the same study [Muller et al., 1970]. The
aetiopathogenesis of nonsyndromic canine agenesis in
permanent dentition is not well clarified. Surely some
variation in population, continents and among races
may play a fundamental role in the development of
canine aplasia.
The main cause of tooth agenesis has a genetic
origin. It seems to be an autosomal dominant model
with incomplete penetrance and variable expressivity
[Pirinen and Arte, 2004]. Moreover, an autosomal
recessive model inheritance is also possible [Pirinen et
al., 2001]. Sex-linked inheritance patterns and a
polygenic multifactorial model of inheritance have
also been suggested [Suarez and Spence, 1974;
Chosak et al., 1975; Brook, 1984; Peck et al., 1993].
Other causes that may arrest canine tooth development
are environmental factors that operate in the dental
region, such as fracture, surgical jaw procedures and
extraction of the preceding primary tooth [Grahnen,
1956]. Permanent dentition hypodontia is often
characterised by retention of deciduous teeth beyond
the age at which they are normally shed. Generally the
canine teeth (apart from age) are more likely to show
little or no resorption [Haselden et al., 2001].
This case report supports the genetic theories about
canine agenesis, which is a congenital disorder able to
cause functional and aesthetic problems, altering the
development of an ideal occlusion, in fact this tooth
guides the jaw during lateral movements creating a
distance between dental arches. Tooth development is
under strict genetic control; it has become evident that
gene networks regulate the development of teeth as in
other vertebrate organs. Mutations in these genes,
during morphogenesis processes, have been identified
in humans as causes of dental defects, mainly
hypodontia. Clinical and radiographic diagnosis of
tooth agenesis in permanent dentition should be made
after the age of 6 excluding the third molar [Pirinen
and Thesleff, 1995].
Conclusion
Congenitally missing permanent canines require a
specific treatment planning. The ability to predict the
morbidity of retained deciduous teeth, without
permanent successors would be of considerable value
in treatment planning. In this type of therapy many
factors must be considered, the preferences and
compliance of the patient, the condition of deciduous
elements, the number of missing teeth, the patient’s
40
occlusion, alignment and facial biotype [Barberìa et
al., 1988]. The first therapeutic choice can be the
extraction of primary teeth accompanied by
orthodontic space closure and premolar coronoplasty.
The second one is retaining the deciduous teeth until
the end of the growth. The advantage of this treatment
is that deciduous teeth preserve the alveolar bone
tissue until the full growth has been reached. This
provides maximum potential for implant-prosthetic
treatment without bone grafting in favour of an
excellent aesthetic and functional behaviour.
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