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Non Syndromic Oligodontia … Tangade P and Batra M 219

CASE REPORT

NON SYNDROMIC OLIGODONTIA: CASE REPORT

Pradeep Tangade1*, Manu Batra2


ABSTRACT

Oligodontia is a rare genetic disorder which represents the congenital absence of more than six teeth in
primary, permanent or both dentitions. It is usually a part of a syndrome and seldom occurs as an
isolated entity. Genes responsible for non syndromic oligodontia are found to be MSX1 and PAX9 genes.
In this case report a 13 year old boy is presented who had absence of all four second permanent molars
and permanent mandibular incisors. The maxillary central incisors presented with conical shape. During
physical examination, there was no abnormality in either hairs or nails, perspiration was normal and no
congenital clefts of lip or palate was seen. Hence in this case, Oligodontia is not associated with any
syndrome which is a rare finding.

INTRODUCTION eruption of permanent teeth, an increased freeway


space and retention of deciduous teeth. Syndromic
Missing of one or more teeth congenitally is most and non syndromic form of Oligodontia can be
often seen during dental examination. Absence of differentiated by conducting thorough physical
all teeth is quite rare but commonest forms are examination of hairs, nails, sweat glands, eyes and
hypodontia and Oligodontia. The condition is to check for any congenital disorders.
genetic in origin. Hypodontia involves the absence
of 1 to 6 teeth whereas Oligodontia describes CASE REPORT
condition in which more than six teeth are missing
(1). A 13 year old boy reported to the Department for a
In previous studies, it was seen that more routine dental check-up. His past medical history
than 80% of hypodontia present with one or two was non-contributory and family history revealed
congenitally missing teeth and only less than 1% that he was born to non-consanguineous marriage
will present with six or more. The incidence of with normal delivery and no one in his family
oligodontia usually varies from 0.08% to 0.16%. have congenitally missing teeth. The patient had
It is a rare condition and the occurrence is no history of trauma or extractions. Extra oral
common in girls in the ratio of 3:2 (2). examination revealed a face with normal facial
Oligodontia can occur in association with profile and normal skeletal dental base relations
various genetic syndromes, like ectodermal (fig 1). During clinical examination, maxillary
dysplasia, Van Der Woude syndrome, Down central incisors were conical in shape along with
syndrome and Reiger syndrome or as a bilateral peg shaped maxillary lateral incisors. The
nonsyndromic isolated familial trait, or as an deciduous right mandibular central incisor and
infrequent finding. Oligodontia is often related maxillary canines, first and second molars were
with conical shaped teeth, microdontia, delayed retained and permanent mandibular incisors were

1,2
Department of Public Health Dentistry, Kothiwal Dental College & Research Centre, Kanth Road, Moradabad-
244001, Uttar Pradesh, India
*Corresponding author: Dr. Pradeep Tangade, Professor & Head of Department, Department Of Public Health
Dentistry. Kothiwal Dental College & Research Centre, Kanth Road, Moradabad-244001, Uttar Pradesh, India. Email:
[email protected]
220 Ethiop J Health Sci. Vol. 22, No. 2 July 2012

missing. Radiographically, in Orthopentomogram permanent mandibular central and lateral incisors


(OPG) all four permanent second molars and were missing (fig 2).

Figure 1: Clinical photograph of the subject

A provisional diagnosis of non syndromic The treatment plan considered for the patient
oligodontia was given with differential diagnosis included reshaping conical maxillary teeth,
of Ectodermal Dysplasia; Rieger syndrome and followed by the extraction of all the retained
Van der Woude syndrome. deciduous teeth present in both maxillary and
Complete set of investigations were done mandibular arches. After healing of extraction
which included routine examination of blood sites, removable partial denture will be considered
including serum calcium, alkaline phosphate, for aesthetic and functional rehabilitation of the
TSH, T3, T4. The findings of these investigations patient.
were normal. During physical examination, hairs
were not thin and sparse, nails were not brittle and DISCUSSION
no difficulty in perspiration was seen which ruled
out ectodemal dysplsia; on occular examination, Oligodontia is the term used most commonly in
no signs of glaucoma was seen ruling out Rieger describing the phenomenon of congenitally
syndrome and lastly Van Der Woude syndrome missing teeth. Oligodontia has been classified as
was left out as there was cleft palate or any isolated or non-syndromic and syndromic
mucosal cysts in lower lip. Hand wrist hypodontia (3). Although oligodontia can occur
radiographic examination was normal. Finally over with 60 different syndromes, these anomalies
based on above findings non syndromic can occur without any syndrome or systemic
Oligodontia as final diagnosis was justified.
Non Syndromic Oligodontia … Tangade P and Batra M 221

Figure 2: Close up view of permanent dentition of patient showing conical permanent maxillary incisors;
and Panoramic radiograph showing pattern of hypodontia: missing all four permanent second molars and
mandibular incisors; retained primary mandibular central incisor is seen

disease. However, oligodontia is seen more consideration. Taking this fact to account, the
common in non-syndromic or familial form than dental fraternity should aim at treating the
syndromic form (4). condition as early as possible and achieve both
The biologic basis for the congenital absence prosthetic and aesthetic functionality of teeth.
of permanent teeth is partially explained by the
failure of the lingual or distal proliferation of the REFERENCES
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