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256 Salivary Gland Diseases in Children and Adolescents
Lymphangiomas for sporadic outbreaks that occur in adolescents and adults. However,
Lymphangiomas are congenital malformations of the lymphatic system in the developing world, it is the most common cause of parotitis in
usually involving the parotid gland. They are the second most common children, primarily affecting children younger than 15 years of age.
nonneoplastic salivary gland tumours in children, occurring from birth Mumps frequently occurs as an epidemic between ages 5 to 15 years.
to about 12 years of age, with a majority at 4 years of age and younger. It is usually contagious, with an attack conferring a lifelong immunity.
About 65% are present at birth and 90% are detected during the sec- The aetiology of mumps is due to the paramyxovirus group with
ond year, with a peak during the first decade. Lymphangiomas occur an incubation period of about 21 days. A similar clinical picture may
more in girls than boys. They present as a soft, asymptomatic swelling present in Coxasackie A or B or parainfluenza virus. It is characterised
with facial asymmetry (Figure 39.2). Unlike haemangiomas, lymph- by mild fever, malaise, and pain and sudden distention of the involved
angiomas rarely undergo spontaneous regression due to the extent of gland, usually the parotid gland. Initially, it involves one side, but
involvement and multispatial character of the lesion. Lymphangiomas within 3 to 5 days both glands become involved. The involved gland
are classified on the basis of the size of cystic spaces as simplex, cav- feels tensed and tender with congested punctum.
ernous, and venolymphatic. Mumps is a self-limiting disease. The treatment is primarily
symptomatic: analgesic for pain, antibiotic to prevent secondary
infection, and rehydration with adequate bed rest. Its postpuberty
complications are orchitis and oophoritis in the male and female,
respectively. Prevention is by the administration of the MMR vaccine.
HIV-associated salivary gland lesions
HIV-associated salivary gland lesions have become common in the
African setting following the HIV/AIDS pandemic. The pandemic is
a leading cause of immunodeficiency in infants and children. Lesions
commonly involve the parotid glands; however, parotid involvement
in the paediatric group is associated with a better prognosis. Typical
lesions are of the benign lympyhoepithelial types and cystic. Other
presentations are with xerostomia and sialorrhrea. On ultrasonography,
70% show multiple hypoechoic or anechoic areas, with the remain-
ing 30% being anechoic. CT and MRI demonstrate bilateral parotid
enlargement with intraglandular cystic and solid masses. No surgical
intervention is needed, as resolution of swellings occurs following the
administration of antiretroviral drugs.
Immunological: Sjogren’s Syndrome
Sjogren’s syndrome is an autoimmune disease characterised by
mononuclear infiltration and destruction of the salivary and lacrimal
glands. Two types are recognised: primary Sjogren’s is a sialolacri-
Figure 39.2: Lymphangioma of the parotid gland. mal disease without associated autoimmune disease, and secondary
Sjogren’s is a sialolacrimal disease with an autoimmune disease, usu-
ally rheumatoid arthritis.
Ultrasonography typically reveals thin-walled septations with Clinical features include those due to xerostomia with dryness
occasional solid areas, CT shows a multispatial mass with heterogeneous of the eye and keratatis. Diagnosis is based on determination of
septation and cystic areas often containing fluid levels; however, solid the parotid rate lower than 1–2 ml/min; ultrasonography studies
portions of the lesion may show enhancement. MRI demonstrates showing a snowstorm, cobblestone appearance; labial gland biopsy;
heterogeneous multiple cystic areas. Contrast-enhanced imaging may detectable rheumatoid factor; antinuclear and antisalivary duct; and
show enhancement of the solid portions of the lesion. antithyroid antibodies. Histologically, it is characterised by infiltration,
Treatment options for lymphangioma include intralesional injection replacement, and destruction of the salivary and lacrimal glands by
of sclerosing agents; OK432 (picibalin), bleomycin, or surgical lymphocytes and plasma cells.
excision. Complications include infection and haemorrhage. Treatment for Sjogren’s syndrome is symptomatic, as for xerostomia:
Parotid Swelling treat connective tissue diseases and keratoconjuctivitis with artificial
Several nonspecific conditions are characterised by unilateral or bilat- saliva and ophthalmic lubricants.
eral enlargement of the parotid gland. Differential diagnosis of bilateral Unknown Aetiology
parotid swellings in a child may have the following aetiologies: Recurrent parotitis in children
• viral (mumps, HIV-associated salivary disease); RPC is a nonobstructive, nonsuppurative inflammatory disease of the
• immunological (Sjogren’s syndrome); and parotid salivary gland of unknown aetiology in children, although con-
genital autoimmune duct defects are implicated. It is the second most
• nutritional (obesity, hypervitaminosis A, beri-beri, hypoproteinaemia). common salivary gland disease in children after mumps. Clinically, it
In addition, of unknown aetiology are hypertrophy of the masseter is characterised by a sudden onset of intermittent unilateral or bilateral
muscle and juvenile recurrent parotitis (JRP, also known as recurrent parotid swellings over a period of years. The child is usually not ill,
parotitis in children, or RPC). although there may be a mild rise in temperature; leucocytic count
Viral differentiates it from mumps. This lesion should also be differentiated
from Sjogren’s syndrome and HIV-associated salivary gland diseases.
Mumps It predominates in male children between 3 months and 16 years of age
Mumps (epidemic parotitis) is now rare in the developed world due to with remission at puberty. There is usually a widening of the Stenson’s
the availability of the measles-mumps-rubella (MMR) vaccines, except duct with mucopurulent discharge.