|Year : 2010 | Volume
| Issue : 4 | Page : 319-321
Congenital absence of multiple primary teeth
Shilpa, A Mohapatra, CP Reddy, N Sivakumar
Department of Pedodontics and Preventive Dentistry, Narayana Dental College, Nellore, Andhra Pradesh - 524 002, India
|Date of Web Publication||25-Jan-2011|
Department of Pedodontics and Preventive Dentistry, Narayana Dental College, Nellore, Andhra Pradesh - 524 002
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Idiopathic agenesis of primary dentition is very rare. This is a case report of a 2-year and 6-month-old girl child who presented with 14 missing primary teeth. A review of the previous case reports is done and the management of these cases is discussed.
Keywords: Missing teeth, multiple, primary teeth
|How to cite this article:|
Shilpa, Mohapatra A, Reddy C P, Sivakumar N. Congenital absence of multiple primary teeth. J Indian Soc Pedod Prev Dent 2010;28:319-21
|How to cite this URL:|
Shilpa, Mohapatra A, Reddy C P, Sivakumar N. Congenital absence of multiple primary teeth. J Indian Soc Pedod Prev Dent [serial online] 2010 [cited 2020 Jul 15];28:319-21. Available from: http://www.jisppd.com/text.asp?2010/28/4/319/76167
| Introduction|| |
Congenitally missing teeth has been defined as either absence of one (or) more teeth or total absence of teeth. Absence may take the form of total absence (anodontia), partial absence (hypodontia) (<6 missing teeth) or oligodontia (>6 missing teeth).
Hypodontia in the permanent dentition is considered a variant of normal. Its frequency ranges between 2% and 10%. Hypodontia in the primary dentition is uncommon, with a prevalence of 0.1-0.9%.  Oligodontia in the primary dentition is rare. It can occur alone or it may be associated with specific syndromes or severe systemic abnormalities.
Characteristic features of oligodontia include lack of development of maxillary and mandibular bone height and reduced lower facial height leading to functional and esthetic compromise. A number of dental anomalies have been associated with oligodontia. These include absence of other teeth, variation in tooth morphology and overall rate of dental development, eruption and exfoliation. This article reports a case of a 2½-year-old child with multiple missing primary teeth.
| Case Report|| |
A 2½-year-old girl child was referred to the Department of Pediatric Dentistry, Narayana Dental College and Hospital, Nellore, Andhra Pradesh, India, with a chief complaint of several missing teeth. On examination, the child appeared to be physically normal, with no underlying systemic condition [Figure 1]. The child was born to nonconsanguineous parents. The mother stated that pregnancy was normal, during which she did not take any medication. The family history did not reveal any missing teeth. Furthermore, the child had no siblings.
Intraoral examination showed the presence of only lower left first primary molar. The tooth was normal in shape, size and color [Figure 2]. Panoramic radiograph revealed congenital absence of 14 primary teeth. (Maxillary right and left central incisors. Lateral incisors, canines and 1 st primary molars. It also showed absence of right and left lower central incisors, lateral incisors, right canine and right 1 st primary molar. It also revealed unerupted mandibular left canine and maxillary and mandibular second primary molars [Figure 3].)
|Figure 2 :An intraoral examination revealed the presence of lower left 1st primary molar|
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|Figure 3 :The panoramic radiograph revealed congenital absence of 14 primary teeth, except the maxillary right and left second primary molars, mandibular left canine, left 1st primary molar and right and left second primary molars|
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| Discussion|| |
This presented case reports 14 missing primary teeth with no associated systemic abnormalities. In this case, a very rare agenesis pattern was observed, i.e. missing primary upper central incisor. Agenesis of primary central incisors is a rare condition seen in holoprosencephaly as a part of more-extended midline malformation, which also includes the brain.  Agenesis of several maxillary incisors is seen in Rieger syndrome.  In the present case, the child had no such associated abnormality.
Further intraoral examination revealed the presence of mandibular left 1 st primary molar only. Radiographic examination showed bilateral agenesis of the maxillary 1 st primary molars. In the mandibular arch, the right 1 st primary molar was missing. The radiographic examination further revealed embedded right mandibular canine and maxillary and mandibular 2 nd primary molars. Idiopathic agenesis of the 1 st primary molars is extremely rare and, in this patient, there was observed agenesis of all the 1 st primary molars except the mandibular left 1 st primary molar, which has not been reported earlier. 
Several factors have been proposed for the etiology of oligodontia. Viral disease during pregnancy has been implicated as a causative factor. Gravely and Johnson concluded that most investigations demonstrated a genetic predisposition to oligodontia and the mode of transmission was uncertain.  Thomsen considered a Mendelian recessive gene to be responsible,  whereas Grahnen suggested an autosomal-dominant gene with varying expression acting possibly in a system involving more than one gene. 
Metabolic imbalances and developmental abnormalities may also be associated with oligodontia. Environmental factors, especially maternal environment, have been implicated in determining oligodontia. In the present case, there was absence of a confirmatory family history and no associated developmental abnormalities.
Studies have shown that when the primary teeth are absent, there is a high prevalence of absence of succedaneous teeth. Ravn reported that patients with aplasia in the primary dentition showed a similar pattern in the permanent dentition in 80% of the cases.  By age 3 years, maxillary and mandibular central and lateral incisors, canine and first molar tooth buds should be present. In the present case, radiographic examination revealed absence of the succedaneous tooth buds, except the 1 st permanent molar tooth buds. This case needs to followed-up to determine the correlation between agenesis of the primary and permanent teeth.
According to Daugaard Jensen, agenesis in the primary dentition may be an isolated rare phenomenon, without consequence in the permanent dentition.  Oligodontia in the primary dentition with permanent successors was reported by Oshima et al. There has been a reported case of idiopathic oligodontia in primary dentition by Shashikiran et al., with nine missing teeth. 
A similar case of idiopathic oligodontia (14 missing primary teeth) was reported by us, in which only the primary maxillary central incisors, maxillary and mandibular 2 nd primary molars were present. An OPG revealed corresponding succedaneous permanent maxillary anterior tooth buds and absence of corresponding succedaneous lower tooth buds. 
Venkataraghavan et al. have reported one case of multiple missing primary teeth (n = 18). But, there is no mention as to whether this particular case is idiopathic or not. According to the author, thorough medical evaluation could not be carried out as the patient resided in a different state. OPG showed congenital absence of all primary teeth except the maxillary right and left primary second molars with the presence of all the developing permanent tooth buds showing defective formation of dentin, roots of the permanent molars and lower central incisors. 
Treatment of oligodontia requires multidisciplinary management to improve function and esthetics. Numerous clinical reports have demonstrated the importance of prosthetic dental treatment in patients with oligodontia for physiological and psychological reasons. The prosthetic treatment of oligodontia varies, and includes removable partial dentures, fixed partial dentures and over-dentures. In the present case, rigid fixed prosthesis is contraindicated. Removable partial denture is recommended, keeping in view the growing age of the patient.
The rationale for use of removable partial denture is its easy care, acceptable cost, easy adjustment during growth, restoration of vertical dimension and easy replacement of missing teeth.
Dental implants can provide more support, retention and stability and improve the function and esthetics of the prosthesis. In this case, because of the severe atrophy of the alveolar ridge and the age of the patient, dental implants were not suitable at this time. The use of dental implants in the future may be a possibility.
| Conclusion|| |
This case highlights the importance of proper diagnosis and knowledge about the growth and development of the child patient, which can aid in proper early correct diagnosis and treatment planning, which can potentially reduce orthodontic complications in patients with oligodontia.
| References|| |
|1.||Grahnen H, Granath L. Numerical variations in primary dentition and their correlation with the permanent dentition. Odont Rev 1961;12:348-57. |
|2.||Simon AR, Roberts MW. Solitary incisor syndrome and holoprosencephaly. J Clin Pediatr Dent 1993;17:175-7. |
|3.||Jorgenson RJ. Rieger Syndrome. In: Buyse ML, editor. Birth Defects Encyclopedia. Vol. 2. Dover, MA 02030 USA: Center for Birth defects Information Service Inc;1990. p. 1497. |
|4.||Bennet CG, Ronk SL. Congenitally missing primary teeth: Report of case. J Dent Child 1980;47:346-8 |
|5.||Gravely JF, Johnson DB. Variation in the expression of hypodontia in monozygotic twins. Dent Pract Dent Rec 1971;21:212-20. |
|6.||THOMSEN SΨ. Missing teeth with special reference to the population of Tristan da Cunha. Am J Phys Anthropol 1952;10:155-67. |
|7.||Odont GH. Revy 1956;7:73. |
|8.||Ravn JJ. Aplasia, supernumerary teeth and fused teeth in the primary dentition. Scand J Dent Res 1971;79:1-6. |
|9.||Daugaard-Jensen J, Nodal M, Skovgaard LT, Kjaer I. Comparison of pattern of agenesis in the primary and permanent dentition in population characterized by agenesis in the primary dentition. Int J Paediatr Dent 1997;7:143-8. |
|10.||Ooshima T, Sugiyama K, Sobue S. Oligodontia in the primary dentition with permanent successors: Report of case. J Dent Child 1988;55:75-7. |
|11.||Shashikiran ND, Karthik V, Subbareddy VV. Multiple congenitally missing primaryteeth: Report of a case. Pediatr Dent 2002;24:149-52. |
|12.||Shilpa, Thomas AM, Joshi JL. Idiopathic oligodontia in primary dentition: Case report and review of literature. J Clin Pediatr Dent 2007;32:65-7. |
|13.||Venkagaraghavan K, Anantharaj A, Prasanna P, Sudhir R. Oligodontia in the primarydentition: Report of a case. J Dent Child 2007;74:154-6. |
[Figure 1], [Figure 2], [Figure 3]
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