Aplasia of right submandibular gland with compensatory parotid and sublingual gland hypertrophy

Findings

There is aplasia of the right submandibular gland, and severe atrophy of the left submandibular gland. There is hypertrophy of the sublingual glands bilaterally, with herniation of the right sublingual gland through the mylohyoid boutonniere. There is also hypertrophy of the parotid glands bilaterally, without a discrete mass and without ductal dilatation or sialolithiasis.

Differential diagnosis:
- Parotiditis
- Parotid gland tumor
- Parotid hyperplasia
- Lymphadenopathy
- Amyloidoma


Diagnosis: Aplasia of right submandibular gland with compensatory parotid and sublingual gland hypertrophy


Key points

Congenital absence of a major salivary gland is extremely rare. It is reported to be associated with other congenital craniofacial anomalies and absence of all four major salivary glands is the most common pattern.
Most patients are asymptomatic. Hypertrophy of other salivary glands may result in an asymptomatic neck mass.
Symptoms such as dysphagia, xerostomia, dental caries, and local pain have been reported. Symptomatic patients generally have aplasia of multiple glands.
Agenesis of the major salivary glands may be associated with other first and second branchial arch defects.
The cause is not known , but is thought to be a result of a fetal development disturbance in the 4th-8th weeks of gestation
Enlargement of the other salivary glands is felt to be compensatory in etiology.