Keywords
Bifid tongue - parotid - salivary agenesis - supernumerary teeth
Case Summary
A 4-year-old girl with complaints of slight discoloration of her tongue was referred
for MR imaging to rule out any vascular malformation. The patient was otherwise asymptomatic.
On physical examination, the presence of bifid tongue, supernumerary teeth on the
alveolar border of the maxilla, a solitary mandibular incisor and a high-arched palate
were noted. No other orofacial or digital defects were noted.
Diagnosis
Aplasia of unilateral parotid with hypoplasia of contralateral parotid with hypertrophy
of unilateral sublingual gland.
Discussion
Unilateral or bilateral agenesis of the parotid gland is an uncommon condition with
unclear aetiology. Bilateral agenesis is relatively more common and is associated
with other developmental craniofacial anomalies.[[1]]
Gruber reported the first case of salivary gland agenesis.[[2]] As the condition is asymptomatic by itself, the exact incidence is difficult to
ascertain. Other major and minor salivary glands being the main source of saliva,
parotid gland agenesis does not result in xerostomia.
Only 22 cases of unilateral salivary agenesis have been reported excluding the present
case. Teymoortash and Hoch reported a case of unilateral agenesis and analysed 21
available cases from the literature. They noted that the condition showed no sex predilection
with age at the time of diagnosis ranging from 50 to 75 years. Their analyses found
the unilateral absence of the gland to be twice as common on the left, in contrast
to our case where it was on the right.[[1]]
In most of these cases, the authors reported a facial asymmetry or a swelling of the
contralateral parotid gland which was believed to be due to compensatory functional
hypertrophy.[[1]] The present case is unique in this regard as it showed the contralateral parotid
to be hypoplastic with the hypertrophied sublingual gland. Four out of the 22 cases
analysed were noted to have ipsilateral accessory parotid tissue. Our case showed
the presence of accessory parotid tissue bilaterally. No associated with heterotopia
in the brain and cavum septum pellucidum has been reported in the literature so far.
The congenital cleft tongue is also a rare disorder which is usually seen with syndromes
such as orofacial digital syndrome, facial clefts, and also associated with abnormal
tissue growths, usually hamartomas.[[3]] This is also seen in association with supernumerary teeth in oro-facial dental
syndromes, as seen in our case, but none of the other features of the syndromes were
noted.
We were unable to classify our findings into any well-defined syndrome. The patient
does not fit into any of the oro-facial-dental syndromes with a combination of anomalies
not reported in the literature so far to the best of our knowledge.
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 his/her/their consent for his/her/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.
Conclusion
Agenesis of the parotid gland is an uncommon congenital anomaly usually associated
with different craniofacial anomalies. It is essential to evaluate orocraniofacial
structures in all individuals with parotid agenesis.
