Bilateral Submandibular Gland Aplasia With Hypertrophy of the Sublingual Glands of a Patient With a Cleft Lip and Palate

Abstract

Aplasia of major salivary glands is a rare disorder with only a handful of cases reported in the literature. The cause of congenital absence of the salivary glands has not been determined, but it may be associated with ectodermal defects of the first and second branchial arches. Patients may be asymptomatic or may experience dysphagia, xerostomia, several periodontal disease, or multiple caries. There are few reports of patients with congenital gland aplasia with cleft lip and palate. We document the case of a 41-year-old patient with repaired unilateral cleft lip palate, bilateral submandibular gland aplasia, and compensatory hypertrophy of the sublingual glands. To the best of our knowledge, there are no previous reports that can be found in the literature with a combination of such findings.

Aplasia of the lacrimal and major salivary glands (ALSG). First case report in spanish population and review of the literature

“…Parotid is more usual affected but submandibular gland agenesis seems to be not common (14). According to our knowledge, in the Spanish population only two cases of salivary glands aplasia have been described, a bilateral aplasia of submandibular glands associated with a cleft lip palate (15) and one case of unilateral aplasia of parotid gland (16). Our case it’s the first of ALSG syndrome in the Spanish literature.…”

“…The correct function of oral tissue it’s possible by means of saliva, and the lack of function may cause dental caries, infections, xerostomia and difficulty of mastication and speech. Sialometry can be developed to demonstrate the diminished salivary flow (15). Alteration of lacrimal glands it’s related with irritable eyes symptoms, infections and even epiphora if the duct or puncta is involved (1).…”

“…Imaging tests are necessary to confirm the lack of formation of salivary glands and alteration of lacrimal system. Many techniques have been described such as US, scintigraphy, TC and RMI (1,2,4,7,15-23).…”