Non-neoplastic Disorders of Salivary Glands

Non-neoplastic Disorders of Salivary Glands

The salivary glands secrete saliva, which is crucial for lubricating food and oral hygiene. A lack of saliva increases the risk of dental caries, gum disease, and infections like sialadenitis. Disorders affecting the salivary glands can be congenital, inflammatory, systemic, or tumours. While these conditions are less common in children than adults, some are unique to pediatric cases, requiring specialized surgical management that considers the anatomy and physiology of children. Most tumours in major salivary glands are benign, while those in minor glands are often malignant.

CONGENITAL ABNORMALITIES OF SALIVARY GLANDS

1. Salivary gland aplasia or agenesis. It is a condition characterized by the absence or underdevelopment of one or more salivary glands. It is a very rare disease. As a consequence of this aplasia, affected children typically experience xerostomia, which is a dry mouth resulting from decreased saliva production. The effects of xerostomia can be severe, leading to dental caries, fissuring of the lips, and oral ulceration. The diagnosis can usually be made with imaging in the form of ultrasound or MRI. Management of xerostomia in these cases focuses on supportive and symptomatic measures, which may include the use of artificial saliva and rigorous dental care to avoid complications arising from dry mouth.
2. Congenital salivary cysts. Congenital salivary cysts are more frequently seen in the parotid gland. Cysts mainly occur due to developmental irregularities during gland formation, resulting in abnormal fluid-filled sacs. It often presents as a mass that is confirmed to be cystic through ultrasound imaging. Differentiation between a congenital salivary cyst and a branchial anomaly is difficult; however, the treatment approaches for both conditions are similar. The available treatment options for congenital salivary cysts include aspiration to remove the cystic fluid, sclerotherapy to collapse the cyst, and surgical excision to completely remove the cyst and prevent recurrence. Recurrence can lead to persistent swelling and discomfort due to the mass effect they exert on adjacent structures.
3. Congenital ductal abnormalities. There can be duplicate or imperforate ducts in the salivary gland. It is a rare condition. These are typically diagnosed through sialography or MR sialography, revealing ductal obstructions that can cause saliva to build up within the glands. If symptomatic, these abnormalities may lead to infections or gland damage due to saliva stasis. Duct marsupialization is done to alleviate the blockage, restore normal salivary flow, and prevent recurrent infections.
4. Ectopic salivary tissue. Ectopic salivary tissue can occur in unusual locations, including the neck, mandible, and cervical lymph nodes. It mainly occurs due to aberrations in the migration of salivary gland tissue during embryogenesis. These ectopic salivary tissues may give rise to discharging sinuses in the affected areas. The difference between ectopic lesions from branchial cleft sinuses is difficult, and in several cases, the primary pathology may relate to a first branchial cleft anomaly. Treatment for ectopic salivary tissue typically involves surgical excision, ensuring the complete preservation of the facial nerve.
5. Cystic fibrosis. It is an autosomal recessive genetic disorder. Like other exocrine mucus-secreting glands, salivary glands are also affected. There is excessive production of saliva leading to obstruction in the ducts and damage to the glandular tissues (parenchyma) of salivary glands. The parotid gland being a serous gland is less affected as compared to the submandibular gland. There is dry mouth and difficulty in swallowing, affecting the overall oral hygiene.

ACQUIRED ABNORMALITIES OF SALIVARY GLANDS

1. Mucous extravasation cysts (Mucocele). It mainly occurs due to trauma of the ducts of a minor salivary gland which causes mucus to escape into the surrounding tissues. They appear as a soft and cystic mass of bluish colour. The commonest site is the lower lip but it can also occur in the buccal mucosa or in the floor of the mouth. Treatment is surgical excision as they do not resolve spontaneously.
2. Ranula. A ranula is a fluid-filled translucent cystic swelling arising from the floor of the mouth on one side of the frenulum. It is almost always unilateral. It is formed due to obstruction in the ducts of the sublingual salivary gland. The obstruction can be spontaneous or due to trauma to the sublingual gland duct. It is divided into two types: (i) simple or intraoral ranula, and (ii) plunging ranula. A simple ranula presents as smooth swelling in the floor of the mouth with a bluish appearance, pushing the tongue up. It can cause difficulty in swallowing, speech, mastication or respiration. A plunging ranula extends beyond the floor of the mouth and below the mylohyoid muscle and presents as swelling in the neck in the submandibular triangle. Treatment is complete surgical excision if the swelling is small, or marsupialization, if the swelling is large. Aspiration or drainage of a ranula results in a high recurrence rate.
3. Viral parotitis (mumps). Mumps is the most common viral infection of the salivary glands, primarily affecting the parotid glands. It most commonly affects children. It is caused by the paramyxovirus, which spreads through respiratory droplets or contact with contaminated surfaces. The incubation period for mumps is typically 2 to 3 weeks. Patients often experience a prodromal phase characterized by malaise and fever, followed by the hallmark clinical feature of painful bilateral swelling of the parotid glands. Although other salivary glands may be affected, asymptomatic infections occur in around 20% of individuals.

Diagnosis of mumps is primarily clinical but difficulties arising when parotid enlargement is absent. Serum and urinary amylase are raised during the first week of parotitis.  Laboratory tests may reveal elevated serum and urinary amylase levels during the first week of illness. Serological tests measuring serum IgG and IgM can help confirm recent infection.

Complications can arise, especially in post-pubertal males, including painful orchitis, which can lead to subfertility, although this is rare. Other potential complications include unilateral sensorineural hearing loss, aseptic meningitis, pancreatitis, and other inflammatory conditions like myocarditis and nephritis.

Treatment. The condition is usually self-limiting, requiring symptomatic management, such as hydration, rest, and pain relief. Preventive measures include MMR (Mumps, Measles, Rubella) vaccine at the age of 15 months. An infant has maternal immunity for 1 year.

4. Acute suppurative parotitis. It is a painful condition commonly seen in vulnerable populations like the elderly, debilitated and dehydrated patients. Dry mouth due to any cause is a predisposing factor. Causative organisms are Staphylococcus aureus and other Gram-positive and anaerobic organisms. The patients have sudden severe pain over the parotid region with painful jaw movements, fever and there is enlargement of the gland. The route of infection is through the opening of Stensen’s duct which may be found swollen and red and may be discharging pus. The pus can also be expressed by gentle pressure over the gland. White cell count shows leukocytosis with an increase in polymorphs. Blood culture and pus culture sensitivity can be done. It consists of I.V. antibiotics, maintaining hydration, oral hygiene and sialagogues to stimulate salivary flow. Incision and drainage can be done if fever does not subside or induration increases.
5. Human immunodeficiency virus (HIV). Generally, the parotid gland is affected. There is swelling of the parotid gland, which may be painful or painless. The clinical picture is similar to Sjogren’s syndrome but HIV salivary gland disease does not have anti-Ro or anti-La antibodies. Imaging may show cystic disease or lymphadenopathy.
   Treatment. It is largely symptomatic and supportive. Cyst aspiration, sclerotherapy, surgical excision and supportive treatment for xerostomia are done.
6. Acute pyogenic parotitis of infancy. There is a painful swelling of the gland with erythema of the overlying skin. The patient has a fever and refuses to feed. The causative organisms are Staphylococcus aureus, Streptococcus pyogenes and anaerobes. Ultrasound is done to see the collection of pus.
   Treatment. It consists of appropriate I.V. antibiotics, maintain hydration and drainage of pus. Incision and drainage should be done carefully as the facial nerve is quite superficial in infants. Hence, Needle aspiration is preferred.
7. Acute suppurative sialadenitis. It is a bacterial infection of the salivary glands, commonly occurring in older kids. It mainly occurs due to stasis of the salivary flow. Obstructive causes, such as salivary stones, can contribute to this condition, but it is more frequently seen in children with comorbidities like cystic fibrosis, malignancy, HIV, and cerebral palsy. The most common bacteria responsible for this infection include Staphylococcus aureus, Streptococcus pyogenes, Streptococcus pneumoniae, and anaerobic organisms. Patients present with fever, pain, swelling, and tenderness in the affected gland. If an abscess forms, it may be visible at the duct orifice of the affected gland. Though it is usually diagnosed through imaging, often with ultrasound.
   Treatment. It consists of broad-spectrum intravenous antibiotics, maintain hydration, and sialagogues to stimulate salivary flow. Drainage of the abscess may be required but needs to take into account the superficial nature of the facial nerve in children. Incision and drainage is necessary but it should be done carefully as the facial nerve is quite superficial in children.
8. Juvenile recurrent parotitis. Juvenile recurrent parotitis (JRP) is another condition It is characterized by repeated episodes of unilateral or bilateral painful swelling of parotid glands, commonly occurring in children between the ages of 3 and 6. Each episode typically lasts 2–3 days but can persist for up to two weeks.
   Treatment. It consists is mainly symptomatic with analgesics and anti-inflammatory drugs.
9. Sjogren’s syndrome. It is an autoimmune disorder that affects multiple systems in the body, primarily targeting the exocrine glands. It can manifest as either primary or secondary Sjogren’s syndrome.

• Primary Sjogren’s Syndrome: This form is characterized by symptoms of dry mouth (xerostomia) and dry eyes (xerophthalmia) due to the involvement of the salivary and lacrimal glands. The parotid gland is the most commonly affected. Historically, it has also been referred to as a benign lymphoepithelial lesion of the parotid or Mikulicz’s disease. Unlike secondary Sjogren’s syndrome, primary Sjogren’s affects both sexes equally.
• Secondary Sjogren’s Syndrome: This type includes three main components:

1. 1. Keratoconjunctivitis sicca: Caused by lacrimal gland involvement, leading to dry eyes.
   2. Xerostomia: Resulting from the involvement of the salivary glands and minor mucous glands in the mouth, leading to dry mouth.
   3. Autoimmune connective tissue disorders: Often associated with rheumatoid arthritis or systemic lupus erythematosus (SLE).

Secondary Sjogren’s syndrome commonly presents with bilateral swelling of the salivary glands and is far more prevalent in females, with 90% of cases occurring in women.

Diagnosis of Sjogren’s syndrome is based on the patient’s history and physical examination findings, particularly the symptoms of keratoconjunctivitis and xerostomia. Schirmer’s test can be used to assess reduced tear production, while a biopsy of the lower lip may reveal the involvement of the minor salivary glands. Additionally, the presence of SS-A and SS-B antibodies is crucial for diagnosis. Other supportive tests include elevated erythrocyte sedimentation rate (ESR), positive rheumatoid factor, and positive antinuclear antibody tests, which help in identifying associated conditions like rheumatoid arthritis or SLE.

         10. Salivary gland stones (Sialolithiasis)

Salivary stones are formed due to the deposition of calcium phosphate on the organic matrix of mucin or cellular debris. Calculi may form in the ducts or parenchyma of submandibular or parotid glands. The submandibular gland is a mixed gland producing both serous and mucus secretions, while the parotid gland produces only serous secretions. The secretions produced by the submandibular gland are thicker as compared to secretions from the parotid gland and the drainage of the secretions from Wharton’s duct is against the gravity, hence 90% of the stones are seen in the submandibular gland and 10% in the parotid gland. Salivary stones are uncommon in children. The patient complains of intermittent swelling of the involved gland, and pain during meals due to obstruction to the outflow of saliva. Sometimes, the stone is visible at the duct opening or can be palpated. About 80% of the stones are radio-opaque and can be seen on appropriate X-rays. Sialography may be required for radiolucent stones. Stones in the peripheral part of submandibular or parotid ducts can be removed intraorally (e.g. sialendoscopy), while those at the hilum or in the parenchyma require excision of the gland.

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Learning resources.

• Scott-Brown, Textbook of Otorhinolaryngology-Head and Neck Surgery.
• Susan Standring, Gray’s Anatomy.
• Stell and Maran’s, Textbook of Head and Neck Surgery and Oncology
• Frank H. Netter, Atlas of Human Anatomy.
• B.D. Chaurasiya, Human Anatomy.
• P L Dhingra, Textbook of Diseases of Ear, Nose and Throat.
   

  Author:

Dr. Rahul Bagla
MBBS (MAMC, Delhi) MS ENT (UCMS, Delhi)
Fellow Rhinoplasty & Facial Plastic Surgery.
Renowned Teaching Faculty
Mail: msrahulbagla@gmail.com
India

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