Hyposalivation in a 16‐year‐old girl: a case of salivary gland aplasia

Abstract

Salivary gland aplasia is a rare condition with only a small number of cases reported worldwide. It is more commonly seen in males and can occur either in isolation or association with other defects or syndromes. It may or may not occur with a hereditary background. Scant literature exists detailing the status of sublingual salivary glands in patients with any form of major salivary gland aplasia. This case report describes the clinical and magnetic resonance imaging presentation of a 16-year-old girl with major salivary gland aplasia detailing the status of all six major salivary glands.Keywords: Hyposalivation, salivary gland aplasia.Abbreviations and acronyms: CPP-ACP = casein phosphopeptide-amorphous calcium phosphate; LADD = lacrimo-auriculo-dentodigital; MRI = magnetic resonance imaging.

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

“…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.…”

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

“…A few cases of aplasia of all major salivary glands has been reported in the literature, nevertheless incomplete aplasia or hypoplasia is described (1,2,4-6,12,13). Parotid is more usual affected but submandibular gland agenesis seems to be not common (14).…”

“…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).…”

“…The first line of treatment in ALSG syndrome it is early diagnosis to prevent future consequences, specially related with orodental alteration. Multiple saliva substitutes have been proposed such as glycerine and lemon, carboxymethhylcellulose, oil and water emulsions, lactoperoxidase, glucose oxidase, xylitol, and many others (2,4,5). Also pharmacological sialagogue such as pilocarpine has been proposed to improve the salivary function when a small part of it is still working (5,19).…”

“…Abnormal salivary glands can be associated with other ectodermal defects, such as the lack of development of the lacrimal apparatus, dental hipodontya, affectation of skin, hair and nails, constituting a syndrome known as lacrimo-auriculo-dento-digital (LADD) (4). Down syndrome, Treacher Collins syndrome, hemi-facial microstomia, cleft lip and palate, Klinefelter syndrome have also been associated with lack of formation of salivary glands (5-7).…”