Bilateral parotid glands aplasia: a case report and literature review

Aplasia of the Major and Minor Salivary Glands: Report of a Rare Case

“…Unfortunately, there is insufficient awareness of general dentists about this rare entity and frequently patients remain undiagnosed for a long time, as in our case in which the patient was diagnosed at the age of 23. Dryness of the oral mucosa and rampant caries are common, but the complete absence of the orifices of the excretory ducts of the parotids (Stenson's duct) and the submandibular glands (Wharton's ducts) has been rarely described, but, when present, it should raise the suspicion of an underlying aplasia, similar to our case [4]. Differential diagnosis encompasses the frequent causes of xerostomia, including diabetes mellitus, Sjögren's syndrome and xerostomia associated with medication.…”

“…Salivary gland aplasia was first described in 1885 by Gruber [8]. Since then, only a few cases of aplasia, incomplete aplasia or hypoplasia have been reported in the literature [3,4,[9][10][11][12][13][14][15][16][17][18][19]. Pham Dang et al [16] in 2010 reported 35 cases of agenesis of the major salivary glands while in a more recent literature review by Kuralt et al [4], 148 radiologically confirmed cases of salivary gland aplasia were found; the median age of patients at the time of diagnosis was 21 years old and the majority were females (52.1%), as in our case who concerned a 23-year-old woman.…”

“…LADD includes nasal lacrimal duct obstruction, aplasia or hypoplasia of major salivary glands, cup shaped ears associated with a sensory or mixed deafness, small and sharp lateral incisors and agenesis of the upper lateral incisors, clinodactylia of the fifth finger and/or phalanx abnormalities [ 11 ]. In fact, Kuralt et al [ 4 ] reported in their review that half of the cases of salivary gland aplasia were combined with craniofacial malformations and hereditary syndromes. In our case, none of the above findings were observed and the family history was not significant, so that aplasia of the salivary glands was considered an isolated feature.…”

“…Aplasia of the major salivary glands mainly affects the parotid salivary gland, unilaterally or bilaterally, and less frequently the submandibular salivary glands. In addition, it may often be associated with other ectodermal defects including several syndromes [ 4 ]. Hyposalivation caused by aplasia of the salivary glands may lead to several problems, such as dysphagia, dysgeusia, rampant dental caries, susceptibility to oral infections and even malnutrition and psychological problems [ 2 ].…”