International Journal of Oral Care and Research

: 2020  |  Volume : 8  |  Issue : 3  |  Page : 66--69

Numerous anomalies of teeth with concomitant nonsyndromic oligodontia in primary and permanent dentition: A rare case report

Senthil Kumar, Madhumitha Mohanraj 
 Department of Pedodontics, RVS Dental College & Hospital, Sulur, Coimbatore, Tamil Nadu, India

Correspondence Address:
Dr. Senthil Kumar
Department of Pedodontics, RVS Dental College & Hospital, Sulur, Coimbatore, Tamil Nadu.


Aim: The case report aimed at reporting a rare case of numerous morphological anomalies of teeth with concomitant nonsyndromic oligodontia in primary and permanent dentition in the oral cavity. Background: Oligodontia is agenesis of numerous teeth preferably missing more than six teeth in the oral cavity, affects both primary and permanent dentition, and is commonly associated with ectodermal dysplasia or specific abnormalities. Agenesis of the primary teeth is rare, especially of the primary canines. The exact etiology is unknown. Case description: This paper reports a very rare case of an 8-year-old boy with multiple morphological anomalies of teeth, with missing primary canines, and 19 permanent teeth at 8 years of age, later followed up from 11 to 15 years. This paper discusses the prevalence, etiology, and management of oligodontia which was not associated with any syndrome like Van Der Woude, Reiger syndrome as it usually is, because the patient did not exhibit any of the associated findings with any syndrome, thereby making it rare of the rare cases. Conclusion: A rare case report of nonsyndromic oligodontia with multiple congenitally missing primary and permanent teeth associated with morphological anomalies of the teeth and without the absence of any systemic disorders or syndromes is presented. Clinical Significance: Since oligodontia is always associated with ectodermal dysplasia or other syndromes, recognizing cases without associated syndromes which are necessary to evaluate any other underlying factors. Thus, the incidence of oligodontia which usually varies from 0.08% to 0.16% citing this case report falls into that 1% making it rare.

How to cite this article:
Kumar S, Mohanraj M. Numerous anomalies of teeth with concomitant nonsyndromic oligodontia in primary and permanent dentition: A rare case report.Int J Oral Care Res 2020;8:66-69

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Kumar S, Mohanraj M. Numerous anomalies of teeth with concomitant nonsyndromic oligodontia in primary and permanent dentition: A rare case report. Int J Oral Care Res [serial online] 2020 [cited 2020 Dec 2 ];8:66-69
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Oligodontia is defined as the congenital absence of six or more teeth whereas hypodontia means the absence of one or few teeth and anodontia means a complete absence of teeth. Reports have shown that the prevalence of hypodontia in the permanent dentition (excluding third molars) is about 4.5–7.4%.[1] But this condition is rarer in the primary dentition with a prevalence of 0.5% or less and has no significant sex distribution.[2] It occurs either isolated nonsyndromic or as a part of the syndrome. Oligodontia has associated anomalies with a tendency toward delayed tooth formation, reduced tooth size, ectopic maxillary canines, ectopic eruption of other teeth, short roots of teeth, enamel hypoplasia, and hypocalcification. The causes of oligodontia are either hereditary, trauma, infection, radiation, metabolic disorders, and idiopathic.

Oligodontia of primary dentition can cause impaired growth of alveolar process, reduced lower facial height, pseudo-prognathism, speech impairment, and deep bite causing esthetic, functional, physiological, and psychological problems.[3] Lateral incisors are the most commonly missing teeth in the primary dentition. In a study on the pattern of agenesis in 193 children with hypodontia in the primary dentition, Daugaard-Jensen et al. confirmed that congenitally missing primary canines, molars, and maxillary central incisors were rare.[4] Hence, this is a rare case report of numerous morphological anomalies with the rarity of missing primary canines, permanent teeth, and their management, followed up at 8 years then from 11 to 15 years of age.

 Case Report

An 8-year-old boy reported with his parents to a private dental clinic with the chief complaint of missing and nonexfoliated primary teeth in the month of May 2011. A detailed case history was taken, the boy was in good health, and he did not suffer from any medical conditions. He was born to nonconsanguineous parents and was forceps delivered with no complications during delivery. The mother did not suffer from any systemic diseases during pregnancy. On taking a detailed family history, it was noted that all his family members including his younger sibling had a normal set of dentition, however, his maternal grandfather had a similar condition of missing teeth.

On general physical examination, his height and weight were found to be normal, well oriented, and reactive. No abnormalities of skin, nails, and hair were seen. Extraorally, the patient had a convex facial profile, with no facial asymmetry. On the basis of the above-mentioned findings, a provisional diagnosis of oligodontia with nonsyndromic isolated familial trait was made.

Intraoral examination revealed the presence of 51,52,54, 55,61,62,64,65,71,72,74,75, 81,82,84,85 [Figure 1]A and [B]. The occlusal surfaces of upper and lower first and second primary molars neither exhibited the morphology of a normal primary molar nor were any cusps seen, thus molar relationship could not be established. There was a total absence of both maxillary and mandibular primary canines due to which abnormally excessive spacing was seen between lateral incisors and primary first molars in all the quadrants [Figure 2] and [Figure 3]. The lower central incisors were found to be rudimentary. Both maxillary and mandibular arches were knife-edged.{Figure 1} {Figure 2} {Figure 3}

An orthopantomogram (OPG) was taken to further investigate the condition. The OPG revealed the presence of only permanent bilateral central incisors, left lateral incisor, bilateral canines, and right premolar tooth bud in the maxilla. The mandible showed a complete absence of permanent tooth buds except for permanent bilateral canine tooth buds [Figure 3].

A small tuberculate-like structure was observed in the place of the upper left lateral incisor. The morphology of all the primary molars was conical. The pulp chambers of maxillary primary central incisors were unusually wide. The patient/patient’s parents were informed about the findings in OPG and explained that further investigations were needed to confirm the presence of any associated syndromes. The patient was not willing for any further investigations. Though the patient did not report to the dental clinic from 8 to 11 years of age but was followed up by the dental clinic every 6 months through telephonic conversation enquiring about the dental health status of the child.

The same patient reported back at the age of 11 in July 2014 with the chief complaint of proclined upper anteriors. Intraoral examination revealed the presence of 11,52,13,54,55, 21,22,23,64,65, 71,72,33,74,75, 81,82,43,84,85. The maxillary central incisors were abnormally shaped. The upper left lateral incisor was conical. In the maxillary arch, 11,13,21,22,23 were permanent teeth and 52,54,55,64,65 were retained deciduous teeth, whereas in the mandible 71,72,74,75, 81,82,84,85 were retained deciduous teeth and 33,43 were permanent teeth. There was an increased overjet with an excessive deep bite. Gingival recession was present in the lower anterior region with severe attrition of the lower central and lateral incisors.

Another OPG was taken, which revealed abnormally shaped maxillary central incisors with widened pulp chambers and open apex [Figure 4]. The left lateral incisor was conical in shape. The pulp chambers of permanent canines were widened. Crypts of permanent right first and second premolars were seen.{Figure 4}

An attempt to re-confirm the earlier differential diagnosis at the age of 11 for oligodontia with nonsyndromic isolated familial trait was done by physical examination of hair for sparseness, nails if they were brittle, sweat glands for difficulty in perspiration, thereby ruling out ectodermal dysplasia, Van Der Woude syndrome was ruled out since the patient did not exhibit any cleft palate or presence of mucosal cyst of the lower lip, and eye examination was done for the presence of glaucoma which was absent, thus ruling out Reiger syndrome. The patient did not exhibit any other extra findings other than the eruption of permanent canine in the mandible 33,43 which was unerupted at the age of 8 years when the patient first reported to the clinic. No other investigation was performed as the patient was not willing for the same. Henceforth, the earlier diagnosis of Nonsyndromic Oligodontia of Primary and Permanent teeth was retained instead.

An orthodontic consultation was done and explained that a fixed appliance cannot be given for this patient. Hence, modified Hawley’s with Robert’s retractor and modified Adams clasp was given to correct the overjet [Figure 5] for a period of 12–14 months. The patient was asked to report every 4 weeks for the activation of Robert’s retractor. The treatment concluded by taking OPG which revealed the final set of teeth present in the patient at 15 years of age. [Figure 6]. The patient had developed carious lesions in relation to 74,75 when reported last and was restored with composites, and the patient was kept under observation for any future treatment modifications.{Figure 5} {Figure 6}


Congenital absence of teeth may be from physical obstruction or disruption of the dental lamina, space limitations, functional abnormalities of the dental epithelium, or failure of initiation of the underlying mesenchyme. Characteristic dental symptoms were a reduced number of teeth, reduction in tooth size, morphological anomalies of tooth form, and delayed eruption.[5] Several studies have shown that MSX1 and PAX9 genes play a role in early tooth development. PAX9 is a paired domain transcription factor that plays a critical role in odontogenesis. All mutations of PAX9 identified to date have been associated with a nonsyndromic form of tooth agenesis.[6]

Several studies involving 927 Icelandic children done by Magnusson et al.,[7] 750 Belgian children done by Carvalho et al.,[2] 1141 Finnish children done by Jarvinen et al.,[8] and 1300 Saudi children have reported that, in the primary dentition, lateral incisors were the most commonly missing teeth done by Salama et al.[9] Cho et al. reported congenitally missing maxillary primary canines in three cases.[10] But this case was very rare since both the maxillary and the mandibular primary canines were congenitally missing.

In the permanent dentition, the mandibular second premolar is the most frequently missing tooth after the third molar, followed by the maxillary lateral incisor and the maxillary second premolar. Literature reviews show only a countable number of cases reporting a maximum number of congenitally missing permanent teeth. Tsai et al. have reported a case of oligodontia in a 6-year-old patient with congenital absence of 16 permanent teeth.[11] Akkya et al. reported oligodontia of six permanent teeth.[12] Nagaveni et al. reported a case with 14 missing permanent teeth.[13]

In this case, the congenital absence of 19 permanent teeth is evident, which makes it rarest of the rare to be ever reported. However, agenesis of the maxillary centrals, laterals, canines, and first permanent molars seems to be very rare. In the maxilla, only the centrals, left laterals, and canines were permanent with the crypts of permanent right first and second premolars were seen. In the mandible, only the permanent canines were seen. Adding to multiple numerous morphological anomalies such as rudimentary lower primary centrals and laterals which have conical crowns, flattened cusps of primary molars, abnormally shaped permanent centrals with widened pulp chambers, making this care report very rare and exceptional.

Treatment of patients with oligodontia generally requires a multidisciplinary approach. The age of the patient is the most important factor for treatment planning. Other conditions include the present condition of retained teeth, number of teeth present, missing, carious teeth, condition of supporting structure and tissues, occlusion, and interocclusal rest space.[14] Hence, the treatment options include composite restorations, orthodontic corrections, partial dentures, and implant-retained prosthesis.[15] In this patient, the retained primary teeth had no mobility, hence removable partial dentures were unnecessary. The carious tooth was restored with composites. A modified Hawley’s and Adam’s clasp with Robert’s retractor was given to correct the underlying overjet since the fixed appliance was contraindicated for the current condition. The patient is kept under periodic observation to initiate future treatment modification if and when the need arises.


Oligodontia is a frequent finding in many syndromes, but this case was not associated with any syndrome, which is a rare finding.

Hypodontia in primary dentition is very rare and, in addition, congenitally missing primary canines is exceptionally unique.

This case also reports the absence of 19 permanent teeth, which is again a rare condition. Thus, early detection of developmental anomalies in the primary and permanent dentition may allow pediatric dentists and other disciplinary for early intervention.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given their consent for the use of their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.


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