Non-Syndromic Non-Familial Agenesis of Major Salivary Glands: A Report of Two Cases with Review of Literature
A 15-year-old male patient was referred to the dental out-patient department with an extensive carious breakdown of the mandibular permanent incisor teeth. His medical and family history was unremarkable. Extra-oral examination revealed no obvious abnormality or syndromal stigmata, including any features suggestive of ectodermal dysplasia, but the parotid and submandibular glands were impalpable. Intra-oral examination revealed obvious signs of xerostomia, dry and scaly lips, and rampant dental caries. A careful examination revealed the absence of Stenson's duct and Wharton's duct bilaterally. Minor salivary glands biopsies revealed the presence of labial and palatal glands that were found to be functional.\nComplete complement of teeth was present but florid caries were seen. The decayed missing filled teeth index and decayed missing filled surface index recorded for the patient was 22 and 55 respectively suggestive of the severity of the condition. An unusual form of enamel loss due to chipping was observed in areas such as cervical regions and incisal and cuspal surfaces of incisors and molars. These areas do not normally become carious []. The usual carious soft base was absent on the affected teeth.\nThe patient was unaware of any subjective xerostomia, changes in sweating or lacrimation. Sialometry showed a salivary flow rate of 0.02 mL/min. Sialography was not possible due to congenital absence of ducts and orifices. Laboratory blood test results (complete blood count, electrolytes, erythrocyte sedimentation, C-reactive protein, clotting ability) were normal.\nPanoramic radiography revealed extensive loss of teeth structure []. Further investigations included an ultrasound scan [Figure and ] and contrast enhanced computed tomography in which the submandibular and parotid glands were not visualized [Figure and ]. Magnetic resonance imaging (MRI) was performed which revealed complete absence of all major salivary glands [Figure and ].\nA complete work up by a team of ophthalmologist, Ear Nose Throat (ENT) surgeon, and dermatologist ruled out any other abnormality.