Congenital agenesis of all major salivary glands and absence of unilateral lacrimal puncta: a case report and review of the literature.

Abstract Congenital agenesis of the salivary glands is an extremely rare congenital condition, which may cause severe xerostomia, progressive dental caries, and oropharyngeal candidiasis in children. To date, there have been few documented cases of aplasia of the major salivary glands. Congenital agenesis of the salivary glands accompanied by absence of the lacrimal puncta is even more rare. We report a case of a 5-year-old boy with xerostomia and extensive dental caries. Salivary gland imaging with sodium pertechnetate 99mTcO(4) showed bilateral aplasia of the parotid, submandibular, and sublingual glands. The patient was found to lack the left lacrimal puncta through physical examination. Here we describe the clinical presentation, diagnostic essentials, and medical and dental management of the patient.