This report aims to describe the clinical case of a 10-year-old male patient (R.C.B.C.), presenting nonsyndromic oligodontia with maternal family trait and absence of 22 permanent teeth. Clinically, the patient had prolonged retention of some primary teeth and presence of permanent maxillary left central incisor, permanent mandibular right central incisor and four first permanent molars. Radiographically, only permanent maxillary second molars and mandibular third molars were developing, whereas all other germs of permanent teeth were missing. Treatment consisted of maxillary expansion followed by moving permanent maxillary left central incisor bucco-mesially, and rehabilitation of spaces with removable denture, where the main goal was to restore function and esthetic harmony. The case has been monitored for the past six years. Nonsyndromic oligodontia is a rare condition that poses significant functional and psychosocial difficulties. Treatment usually requires a multidisciplinary approach among which pediatric dentistry and orthodontics play a major role. In view of its complexity, treatment should be initiated as early as possible to minimize future functional and aesthetic issues. Dental implants are considered as definitive treatment of cases of oligodontia, however, until the patient reached the ideal age, other treatments to ensure aesthetics and function must be performed.33123223

Carolina Ribeiro

Caroline Mathias

Marcos Alan

Rogerio Frederico

Starling

Bioscience Journal

English

Repositorio da Producao Cientifica e Intelectual da Unicamp

232 
Case report 
Biosci. J., Uberlândia, v. 33, n. 1, p. 232-237, Jan./Feb. 2017 
EARLY TREATMENT OF NONSYNDROMIC OLIGODONTIA: A CLINICAL 
CASE REPORT 
 
TRATAMENTO PRECOCE DA OLIGODONTIA NÃO-SINDRÔMICA: RELATO 
DE CASO CLÍNICO 
 
Carolina Ribeiro STARLING1; Caroline Mathias Carvalho de SOUZA2;  
Rogério Frederico Alves FERREIRA3; Marcos Alan Vieira BITTENCOURT3 
1. PhD student, Department of Pediatric Dentistry and Orthodontics, Federal University of Rio de Janeiro, Rio de Janeiro, RJ, Brazil; 2. 
PhD student, Department of Restorative Dentistry, Piracicaba Dental School, State University of Campinas, Piracicaba, SP, Brazil; 3. 
Associate Professor of Orthodontics, Federal University of Bahia, Salvador, BA, Brazil. alan_orto@yahoo.com.br 
 
ABSTRACT: This report aims to describe the clinical case of a 10-year-old male patient (R.C.B.C.), presenting 
nonsyndromic oligodontia with maternal family trait and absence of 22 permanent teeth. Clinically, the patient had 
prolonged retention of some primary teeth and presence of permanent maxillary left central incisor, permanent mandibular 
right central incisor and four first permanent molars. Radiographically, only permanent maxillary second molars and 
mandibular third molars were developing, whereas all other germs of permanent teeth were missing. Treatment consisted 
of maxillary expansion followed by moving permanent maxillary left central incisor bucco-mesially, and rehabilitation of 
spaces with removable denture, where the main goal was to restore function and esthetic harmony. The case has been 
monitored for the past six years. Nonsyndromic oligodontia is a rare condition that poses significant functional and 
psychosocial difficulties. Treatment usually requires a multidisciplinary approach among which pediatric dentistry and 
orthodontics play a major role. In view of its complexity, treatment should be initiated as early as possible to minimize 
future functional and aesthetic issues. Dental implants are considered as definitive treatment of cases of oligodontia, 
however, until the patient reached the ideal age, other treatments to ensure aesthetics and function must be performed. 
 
KEYWORDS: Diagnosis. Tooth Agenesis. Pediatric Dentistry. Orthodontics. 
 
INTRODUCTION 
 
Tooth agenesis is a very common 
developmental anomaly (LIANG et al., 2016; 
HIREMATH, 2012; DE COSTER et al., 2009; 
RÓZSA et al., 2009; PUNITHAVATHY, PRIYA, 
INDHUMATHI, 2012) whereby primary and/or 
permanent tooth formation is impaired due to 
compromised odontogenesis (HIREMATH, 2012). 
As a result, there is a reduction in the number of 
teeth in the oral cavity. Clinically, this anomaly can 
affect various combinations of teeth, and may be 
associated with other dental development anomalies. 
It can occur as a sporadic case or as a family trait, 
whether in isolation (nonsyndromic), or associated 
with cleft lip and palate, or with syndromes 
(HIREMATH, 2012; DE COSTER et al., 2009; 
PUNITHAVATHY, PRIYA, INDHUMATHI, 
2012; ZHANG, PENG, FENG, 2015). Tooth 
agenesis can be classified, according to the number 
of missing teeth, as hypodontia, the congenital 
absence of one to six teeth, excluding third molars; 
oligodontia, more than six teeth, and anodontia, all 
teeth (HIREMATH, 2012; PUNITHAVATHY, 
PRIYA, INDHUMATHI, 2012; LIU et al., 2015; 
GURUPRASAD et al., 2011). 
Although diagnosis is performed clinically, 
it should be confirmed by radiographic examination 
(PUNITHAVATHY, PRIYA, INDHUMATHI, 
2012). Clinical diagnosis can be performed by 
inspecting primary dentition from the age of three, 
and permanent teeth from ages twelve to fourteen. 
Radiographically, the diagnosis can be made earlier, 
from the age of six, being panoramic radiography 
the best investigation tool (HIREMATH, 2012; 
GURUPRASAD et al., 2011). Tooth agenesis poses 
daunting functional and psychosocial challenges 
(ZHANG, PENG, FENG, 2015). Early diagnosis 
and treatment options are often limited 
(HIREMATH, 2012; DE COSTER et al., 2009). 
However, dental treatment is important since oral 
health is essential for proper nutrition and speech as 
well as for facial aesthetics and emotional 
development of the individual (HIREMATH, 2012; 
ZHANG, PENG, FENG, 2015; GURUPRASAD et 
al., 2011). Treatment can vary from conservative 
management using removable dentures to oral 
rehabilitation with implant-supported dentures after 
preliminary orthodontic treatment and, when 
necessary, surgical procedures (ZHANG, PENG, 
FENG, 2015). Therefore, cases should be planned 
based on an interdisciplinary approach, as this 
anomaly directly affects occlusion due to improper 
position of adjacent teeth, supraocclusion of 
antagonist teeth, alveolar process growth deficiency 
associated with missing teeth, periodontal problems, 
Received: 24/05/16 
Accepted: 05/12/16 
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Early treatment of nonsyndromic…  STARLING, C. R. et al. 
Biosci. J., Uberlândia, v. 33, n. 1, p. 232-237, Jan./Feb. 2017 
undesirable aesthetics and phonetic problems 
(HIREMATH, 2012; PANNU et al., 2014). 
The purpose of this article is to report an 
unusual case, i.e., that of R.C.B.C., a 10-year-old 
male patient suffering from nonsyndromic 
oligodontia (NO) with 22 permanent teeth missing. 
Hopefully it will assist pediatric dentists and 
orthodontists in planning the treatment of this 
anomaly. 
 
CASE REPORT 
 
R.C.B.C., a male, 10-year-old patient, 
presented at the outpatient clinic of the Department 
of Orthodontics, School of Dentistry, Federal 
University of Bahia, with the following complaint: 
“My teeth are crooked, I want them to be normal; 
and one tooth is missing, too.”  
His medical history revealed no relevant 
events, whereas his family history showed that his 
mother had agenesis of the maxillary premolars and 
second mandibular premolars. A functional analysis 
disclosed atypical speech and swallowing, and 
thumb sucking habit. Extraoral clinical examination 
revealed no irregularities in his skin, hair or nails, 
which might be compatible with other syndromes. 
He had a mesocephalic, symmetrical facial pattern 
with a slightly convex profile (Figure 1). 
Intraoral clinical examination revealed that 
patient had satisfactory oral hygiene with no caries 
activity. The patient had mild atresia of the 
maxillary dental arch, anterior dental crossbite and 
anterior open bite. Prolonged retention was detected 
in some incisors, all primary canine and molar teeth. 
All permanent first molars, the maxillary left central 
incisor (MLCI) and mandibular right central incisor 
had erupted. The maxillary right central incisor and 
maxillary left lateral incisor (MLLI) were missing, 
and the MLCI was tipped distally. His first 
permanent molars displayed normal occlusion on 
the left side, and a class II relationship was detected 
on the right side. All teeth exhibited normal size, 
shape and color (Figure 2). 
Panoramic radiography revealed agenesis of 
22 permanent teeth, with the development of only 
permanent maxillary second molars and mandibular 
third molars (Figure 3). Based on these findings, the 
patient was diagnosed as having NO with maternal 
family trait. 
 
 
Figure 1. Initial extraoral photographs: A – Profile, B – Frontal, C – Smiling. 
 
Figure 2. Initial intraoral photographs: D – Right side, E – Frontal, F – Left side, G – Upper occlusal, H – 
Lower occlusal. 
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Early treatment of nonsyndromic…  STARLING, C. R. et al. 
Biosci. J., Uberlândia, v. 33, n. 1, p. 232-237, Jan./Feb. 2017 
 
Figure 3. Initial panoramic radiograph. 
 
Treatment consisted in placing a Porter arch 
(Figure 4) – activated for six months – to correct the 
atresia in the maxillary arch; placing a removable 
appliance with a coil spring – activated for seven 
months – to move the MLCI mesially, correct the 
midline and create space for replacement of the 
MLLI, which was missing (Figure 5); and other 
removable appliance with a finger spring – activated 
for two months – to correct the anterior crossbite of 
the MLCI (Figure 6). This latter appliance had two 
stock teeth to replace the maxillary right central 
incisor and MLLI, restoring aesthetics and function. 
 
  
Figure 4. Porter arch to expand the upper dental arch 
 
 
Figure 5. Plate with coil spring to move tooth 21 mesially: A and B – Frontal photographs before and after 
spring action, C and D – Upper occlusal photographs before and after spring action. 
235 
Early treatment of nonsyndromic…  STARLING, C. R. et al. 
Biosci. J., Uberlândia, v. 33, n. 1, p. 232-237, Jan./Feb. 2017 
 
Figure 6. Plate with finger spring to move tooth 21 buccally: A and B – Upper occlusal photographs before and 
after spring action, C and D – Left side photographs before and after spring action. 
 
Thereafter, another appliance with only 
stock teeth was placed, functioning as a removable 
partial denture and maintaining the orthodontic 
movements performed so far (Figure 7). Aiming to 
correct functional problems and anterior open bite, 
the patient was referred to a speech therapist. 
 
 
Figure 7. Plate with stock teeth, post-orthodontic treatment, functioning as removable partial dentures: A – 
Frontal intraoral photograph, B – Upper occlusal photograph, C – Frontal extraoral photograph, 
smiling. 
 
The primary teeth were kept and the patient 
has been monitored for the last six years. The 
removable appliance with stock teeth should be 
often replaced in order not to hinder the 
physiological alveolar vertical growth of the maxilla 
and replace primary teeth exfoliated over time. 
During follow-up, the patient was re-evaluated 
every six months. At each visit, we have made the 
necessary adjustments on the device and it was 
replaced twice. Following the end of bone growth, 
the patient will be referred for a removable partial or 
an implant-supported denture in order to achieve a 
functional occlusion with appropriate guides both 
laterality as protrusive.  
In this case, the patient was under early 
treatment with pediatric dentist, orthodontist and 
speech therapist. There was no need to follow up 
with psychologist because the patient had good 
collaboration to dental treatment, neither prosthetist 
yet, since he had only one tooth missing, which was 
placed back in his own orthodontic device. 
 
DISCUSSION 
 
Permanent teeth exhibit a higher prevalence 
of tooth agenesis than primary teeth 
(GURUPRASAD et al., 2011). Hypodontia is the 
most common form of agenesis, corresponding to 
58.7% to 88% of cases. Only about 0.1-0.3% of 
population have oligodontia (PUNITHAVATHY, 
PRIYA, INDHUMATHI, 2012; THIMMEGOWDA 
et al., 2015). 
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Early treatment of nonsyndromic…  STARLING, C. R. et al. 
Biosci. J., Uberlândia, v. 33, n. 1, p. 232-237, Jan./Feb. 2017 
Oligodontia is a more serious form of 
expression, considered rare (LIANG et al., 2016; 
GURUPRASAD et al., 2011). It is often associated 
with syndromes and/or severe systemic 
abnormalities, and individuals with syndromic 
oligodontia have, on average, more missing teeth 
than patients with NO (HIREMATH, 2012). In this 
case report, the patient was diagnosed with NO, 
with absence of 22 permanent teeth, being 
considered a rare case. 
Although it may on occasion be caused by 
environmental factors, in most cases tooth agenesis 
has a genetic etiology. Among the genetic causes 
are changes in the expression of specific genes 
involved in odontogenesis. Some environmental 
factors include infections, such as rubella and 
measles during pregnancy; trauma to the alveolar 
processes; use of chemicals or drugs such as 
thalidomide and dioxin; chemotherapy; 
radiotherapy; and disturbances in the innervations of 
the jaw. Other etiologies have been described, such 
as nutritional factors, endocrine disorders and 
association with syndromes (HIREMATH, 2012; 
DE COSTER et al., 2009). 
It is widely accepted that failures in 
odontogenesis that result in agenesis are caused by 
variations in the sequence of genes expressed during 
tooth development (JIMÉNEZ-CASTELLANOS et 
al., 2005). So far, three genes have been primarily 
associated with nonsyndromic agenesis, especially 
in cases of severe oligodontia: MSX1, AXIN2 and 
PAX9 (LIANG et al., 2016; HIREMATH, 2012; DE 
COSTER et al., 2009; LIU et al., 2015; 
GURUPRASAD et al., 2011). In this case, it was 
not possible to identify the etiologic factor of the 
NO. The most likely hypothesis is heredity, since 
his mother had agenesis of the maxillary premolars 
and second mandibular premolars. However, genetic 
evaluations were not performed.  
Severe cases have been attributed do female 
gender in some reports, with a ratio of 3:2 
(HIREMATH, 2012; DE COSTER et al., 2009; 
GURUPRASAD et al., 2011). In this case, the 
patient's gender does not match the most severe 
cases found in the literature. 
According to Punithavathy et al. (2012), 
bilateral agenesis is more common than unilateral 
and the proportion of missing teeth in the maxilla is 
predominantly larger than in the mandible. In this 
case, there was no preference between sides and 
dental arches, however, there was a prevalence of 
bilaterality– with the exception of the incisors.  
Regarding location, all case reports 
reviewed in the literature showed absence of all 
third molars (HIREMATH, 2012; RÓZSA et al., 
2009; PUNITHAVATHY, PRIYA, 
INDHUMATHI, 2012; JIMÉNEZ-
CASTELLANOS et al., 2005). In this case, two 
developing third molars were present among the ten 
permanent teeth, contradicting all authors reviewed. 
Guruprasad et al. (2011) and Rózsa et al. 
(2009) argue that agenesis of the maxillary central 
incisors, canines or permanent first molars appears 
to be quite exceptional. Furthermore, according to 
Jiménez-Castellanos et al. (2005), canines are the 
least affected teeth as their absence is considered 
rare. It is possible to conclude that the case reported 
in this article is unusual as it presents with agenesis 
of a maxillary central incisor and all permanent 
canines. 
The choice of treatment depends on the 
analysis of various criteria such as occlusion, 
patient's age, position, color and morphology of the 
remaining teeth, and patient desire (MALLAYYA, 
2012). The preservation of retained primary teeth is 
essential for treatment planning, particularly the 
canines. Retained primary teeth with minimal root 
resorption preserves the integrity of the dental arch, 
providing good potential for future prosthetic 
rehabilitation (RÓZSA et al., 2009; JIMÉNEZ-
CASTELLANOS et al., 2005), as was done in this 
case, combining orthodontic and prosthodontics 
approach, due to the patient’s age and condition, and 
an early diagnosis. 
Patients presenting with oligodontia must be 
evaluated carefully for associated syndromes since 
nonsyndromic cases are rare, with few reports in the 
literature. Treatment usually requires a 
multidisciplinary approach, including pediatric 
dentistry, orthodontist, prosthodontist, 
implantodontist, speech therapist and psychologist, 
and in view of the complexity involved treatment 
should be initiated as early as possible to minimize 
future functional and aesthetic issues. Dental 
implants are considered as definitive treatment of 
cases of oligodontia, however, until the patient 
reached the ideal age to install them, other 
treatments to ensure aesthetics and function must be 
performed (ZHANG, PENG, FENG, 2015).  
 
RESUMO: O objetivo deste trabalho é descrever o caso clínico do paciente R.C.B.C., dez anos de idade, gênero 
masculino, portador de oligodontia não associada à síndrome, com traço familiar materno e ausência de 22 dentes 
permanentes. Clinicamente, o paciente apresentava retenção prolongada de alguns incisivos, todos os caninos e molares 
decíduos e presença do incisivo central superior esquerdo, incisivo central inferior direito e os quatro primeiros molares, 
237 
Early treatment of nonsyndromic…  STARLING, C. R. et al. 
Biosci. J., Uberlândia, v. 33, n. 1, p. 232-237, Jan./Feb. 2017 
todos da dentição permanentes. Radiograficamente, apenas os segundos molares superiores e os terceiros molares 
inferiores estavam em desenvolvimento, com ausência dos demais germes dos dentes permanentes. Terapeuticamente, foi 
instituída a expansão maxilar seguida de mesialização e vestibularização do incisivo central superior esquerdo e de 
reabilitação com prótese removível, sendo o objetivo principal restaurar a harmonia estética e a função. O caso foi 
acompanhado há seis anos. A oligodontia não-sindrômica é considerada rara e impõe importantes dificuldades funcionais e 
psicossociais aos seus portadores. Geralmente, o tratamento requer uma abordagem interdisciplinar, onde a 
odontopediatria e a ortodontia desempenham papel primordial. Devido à complexidade envolvida, o tratamento deve ser 
iniciado o mais cedo possível, para minimizar futuras questões funcionais e estéticas. Os implantes dentários são 
considerados como tratamento definitivo para os casos de oligodontia, entretanto, até os pacientes alcançarem a idade 
ideal, outros tratamentos para garantir estética e função devem ser realizados. 
 
 PALAVRAS-CHAVE: Diagnóstico. Agenesia Dentária. Odontologia Pediátrica. Ortodontia. 
 
 
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Tratamento Precoce Da Oligodontia NÃ£o-sindrÃ´mica: Relato De Caso ClÃ­nico

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Tratamento Precoce Da Oligodontia NÃ£o-sindrÃ´mica: Relato De Caso ClÃnico