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Salivary Gland Diseases in Children and Adolescents 255

Investigative modalities in the diagnosis of salivary gland disorders children, probably due to the difficulty in localising the lesion with a
in children are listed below. In an African setting, however, the cost of periapical radiograph in children and because it is usually asymptom-
computed tomography (CT) and magnetic resonance imaging (MRI) atic. Locations include the tonsils, rectum, and the mandible. Inclusion
systems usually limits investigations to ultrasonography and fine needle of the gland in the angle of the mandible is seen frequently and referred
aspiration (FNA) cytology. to as Stafne’s idiopathic bone cyst. Radiographically, this anomaly
1. Ultrasonography (US) is useful in assessing the size of the gland presents as a round or oval radiolucency between the mandibular
and the vascularity of the lesion. It differentiates between a focal and canal and the inferior border of the mandible. The usual treatment is
diffuse disease, cystic and solid lesions, and is a useful adjunct for the exploration of the cavity, whereas others believe it should just be kept
assessment of adjacent vascular structures. US also guides FNA. The under observation.
normal gland is hyperechogenic, whereas the diseased gland varies in Haemangiomas
hypogenicity. Haemangiomas are congenital malformations of the vascular system.
2. A CT scan defines the nature and exact extent of the disease. It is They are the most common benign salivary gland lesion in children
useful in the diagnosis of acute inflammatory glands, abscess, and and the commonest benign tumour of children and adolescents. They
solid tumours. This is the imaging modality of choice in salivary gland have a female predilection. The majority are capillary in nature, occur-
diseases in children. ring during the first year of life. Haemangiomas are usually soft masses
noted shortly after birth, mainly in the parotid region, and rarely in the
3. MRI defines the extent of the lesion similar to the CT scan. It is
sublingual gland (Figure 39.1). They grow rapidly during the first year,
superior to CT, though, in that MRI also demonstrates the facial nerve
with slow spontaneous complete regression at adolescence. They could
within the parotid gland.
be unilateral or bilateral.
4. FMA cytology is a useful diagnostic tool. It can be used to establish At ultrasound, haemangiomas are hypoechoic relative to the gland,
whether a lesion is inflammatory benign or malignant. When used in with a variable abnormal flow at Doppler US; contrast-enhanced CT
children, however, there may be a need for sedation due to lack of reveals hypervascular mass with variable intensity of enhancement
cooperation. FNA cytology accurately diagnoses whether a lesion is with an occasional demonstration of phleboliths. FNA cytology
benign or malignant in about 84–97% of the cases.
demonstrates elongated spindle cells arranged in coils and arcades.
5. Sialography is very useful in the evaluation of autoimmune and Histopathologic analysis shows areas composed of an unencapsulated
chronic inflammatory diseases. The appearance is described as mass of closely packed, thin-walled capillaries with plump endothelial
“cherry-blossom” or “branchless fruit laden tree” or ‘”snow storm”. cells. Immunochemistry reveals vascular spaces lined by CD34 and
6. Sialochemistry is useful in inflammatory and nonneoplastic diseases factor VIII-positive flattened endotheliail cells.
of the salivary glands. In inflammatory diseases, the IgA, IgG, IgM, Most capillary haemangiomas regress spontaneously, so surgical
albumin, transferring, lysozyme, Na, and protein are raised, and the treatment should be delayed. Treatment options include injection of
phosphate level is decreased. sclerosants, such as injection of boiling water normal saline, steroid,
2
alfa 2a or 2b interferone (3 million units/m per day, occlusion of
7. Sialometry is the determination of salivary flow rate. It is useful in
the detection of salivary gland hypofunction. feeder vessels, ligation of feeder vessels, surgical and laser ablation,
or a combination of these modalities. Surgery is indicated in rapidly
8. Chest x-rays are used to detect any lung metastasis. growing haemorrhagic tumours or following failure of the tumour to
9. Radiographs of the jaws are used for the localisation of ectopic regress after achieving fibrosis postsclerotherapy.
salivary gland tissues. Complications of haemangiomas include infection, ulceration, and
Congenital and Developmental Diseases occlusion of the larynx.
Aplasia (Agenesis) and Hypoplasia
Aplasia is the absence of any or a group of salivary glands; they could
be unilaterally or bilaterally absent. Hypoplasia of the gland is reduced
glandular tissue associated with hypofunction. Aplasia and hypoplasia
are very rare, and only case reports have been documented.
Aplasia is of unknown aetiology; it may be isolated or occur in
association with other developmental defects, such as hemifacial
micostomia and the mandibulo-facial dysostosis (Treacher Collins
syndrome), Down syndrome, and ectodermal dysplasia.
Aplasia presents with the development of xerostomia and its
effects; however, other causes of xerostomia should be excluded. The
effects of aplasia (agenesis) include dryness of the mouth, difficulty in
mastication and swallowing of solid foods, an unusual pattern of dental
caries, erosion of teeth, the presence of plaque, periodontal disease,
soft tissue infection, chelitis, atrophic mucositis, and the absence of
the salivary ducts. CT and MRI indicate the absence of the glands and
replacement with fatty tissues; a salivary flow rate lower than 50% of
its normal value is diagnostic.
Treatment is usually conservative; artificial saliva is used for
frequent lubrication of the oral cavity. Comprehensive dental preventive
and restorative therapy is strongly advocated.
Figure 39.1: Haemangioma involving the parotid gland in a 13-year-old girl.
Aberrant/Ectopic Salivary Gland
In aberrant/ectopic glandular anomaly, there is the presence of the
salivary gland tissue in an abnormal location. This condition is rare in