Symptomatic Unilateral Submandibular Gland Aplasia Associated with Ipsilateral Sublingual Gland Hypertrophy

Excerpt

Aplasia or agenesis of the salivary gland is an uncommon disorder. Since Gruber first described a case of bilateral submandibular gland aplasia in 1885, about 30 cases have been reported in the world medical literature [1]. One or all of the glands may be absent. The absence of all major salivary glands was the most frequently encountered pattern in the reported cases [1, 2]. Other developmental anomalies could be found in association with aplasia of the salivary glands, such as atresia of the lacrimal puncta, mandibulofacial dysostosis (Treacher-Collins syndrome), or congenital malformations of the temporomandibular component [3, 4]. Unilateral aplasia of the submandibular gland is an extremely rare disorder and only 11 cases have been reported since Bruno described the first case encountered during a cadaver dissection in 1894. Most reported cases of unilateral submandibular aplasia were asymptomatic, probably because secretions from other salivary glands compensated for the aplasia [5]. Congenital unilateral absence of the submandibular gland is an isolated phenomenon and may result from other unique defects of early fetal development, unlike the cause of multiglandular agenesis associated with other facial anomalies [6]. In this study we report on a extremely rare case of isolated unilateral submandibular aplasia presenting with xerostomia and dysphagia which is associated with ipsilateral sublingual gland hypertrophy. …