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Congenital disorders of the salivary glands are rare, but may include:
- Aplasia
- Atresia
- Ectopic salivary gland tissue
- Stafne defect - an uncommon condition which some consider to be an anatomic variant rather than a true disease. It is thought to be created by an ectopic portion of salivary gland tissue which causes the bone of the mandible to remodel around the tissue, creating an apparent cyst like radiolucent area on radiographs. Classically, this lesion is discovered as a chance finding, since it causes no symptoms. It appears below the inferior alveolar nerve canal in the posterior region of the mandible.
Salivary gland diseases (SGD) are multiple and varied in cause.
There are 3 paired major salivary glands in humans (the parotid gland, the submandibular gland, and the sublingual gland), as well as about 800-1000 minor salivary glands in the oral mucosa of the mouth. The parotid gland is located in front of the ear, and it secretes its mostly serous saliva via the parotid duct (Stenson duct) into the mouth, usually opening roughly opposite the maxillary second molar. The submandibular gland is located medial to the angle of the mandible, and it drains its mixture of serous and mucous saliva via the submandibular duct (Wharton duct) into the mouth, usually opening in a punctum located in the floor of mouth. The sublingual gland is located below the tongue, in the floor of the mouth. It drains its mostly mucous saliva into the mouth via about 8-20 ducts which open along the plica sublingualis (a fold of tissue under the tongue).
The function of the salivary glands is to secrete saliva, which has a lubricating function, which protects the oral mucosa of the mouth during eating and speaking. Saliva also contains digestive enzymes (e.g. salivary amylase) and has antimicrobial action and acts as a buffer. Persons with reduced salivary flow or hyposalivation often suffer from dry mouth or xerostomia, which can result in severe dental caries (tooth decay) as a result of the loss of the protective effects of saliva.
Various examples of disorders affecting the salivary glands are listed below. Some are more common than others, and they are considered according to a surgical sieve, but this list is not exhaustive. Sialadenitis is inflammation of a salivary gland, usually caused by infections, although there are other less common causes of inflammation such as irradiation, allergic reactions or trauma.
Salivary gland aplasia (also termed salivary gland agenesis) is the congenital absence of salivary glands. Usually the term relates to the absence of some or all of the major salivary glands.
It is a rare condition, and most known cases have been in association with syndromes of the ectodermal tissues, particularly the lacrimal apparatus. Example syndromes which have been reported with salivary gland aplasia include hereditary ectodermal dysplasia, mandibulofacial dysostosis and hemifacial microsomia.
The main significance of the condition is a lack of saliva, causing xerostomia (dry mouth), with accompanying susceptibility to dental caries (tooth decay), infections of the mouth, and upper respiratory tract infections (e.g., candidiasis, ascending sialadenitis, laryngitis and pharyngitis). Patients with salivary gland aplasia typically require regular application of topical fluoride to prevent tooth decay.
Signs and symptoms are variable and depend largely upon whether the obstruction of the duct is complete or partial, and how much resultant pressure is created within the gland. The development of infection in the gland also influences the signs and symptoms.
- Pain, which is intermittent, and may suddenly get worse before mealtimes, and then slowly get better (partial obstruction).
- Swelling of the gland, also usually intermittent, often suddenly appearing or increasing before mealtimes, and then slowly going down (partial obstruction).
- Tenderness of the involved gland.
- Palpable hard lump, if the stone is located near the end of the duct. If the stone is near the submandibular duct orifice, the lump may be felt under the tongue.
- Lack of saliva coming from the duct (total obstruction).
- Erythema (redness) of the floor of the mouth (infection).
- Pus discharging from the duct (infection).
- Cervical lymphadenitis (infection).
- Bad Breath.
Rarely, when stones form in the minor salivary glands, there is usually only slight local swelling in the form of a small nodule and tenderness.
Ectopic salivary gland tissue which is located in sites other than the normal location is variously described as aberrant, accessory, ectopic, heterotopic or salivary gland choristoma.
An "accessory salivary gland" is ectopic salivary gland tissue with a salivary gland duct system. The most common location of accessory salivary gland tissue is an extra major salivary gland in front of the parotid gland. It is typically about 3 cm or less in size, and drains into the parotid duct via a single tributary. Accessory parotid tissue is found in 21-56% of adults. Any disease process which affects the salivary glands, including cancer, may also occur within an accessory salivary gland tissue.
A salivary gland fistula (plural "fistulae") is a fistula (i.e. an abnormal, epithelial-lined tract) involving a salivary gland or duct.
Salivary gland fistulae are almost always related to the parotid gland or duct, although the submandibular gland is rarely the origin.
The fistula can communicate with the mouth (usually causing no symptoms), the paranasal sinuses (giving rhinorrhea) or the facial skin (causing saliva to drain onto the skin).
The usual cause is trauma, however salivary fistula can occur as a complication of surgery, or if the duct becomes obstructed with a calculus.
Most parotid fistulae heal by themselves within a few weeks.
The term is derived from the Greek words "sialon" (saliva) and "lithos" (stone), and the Latin "-iasis" meaning "process" or "morbid condition". A "calculus" (plural "calculi") is a hard, stone-like concretion that forms within an organ or duct inside the body. They are usually made from mineral salts, and other types of calculi include tonsiloliths (tonsil stones) and renal calculi (kidney stones). "Sialolithiasis" refers to the formation of calculi within a salivary gland. If a calculus forms in the duct that drains the saliva from a salivary gland into the mouth, then saliva will be trapped in the gland. This may cause painful swelling and inflammation of the gland. Inflammation of a salivary gland is termed "sialadenitis". Inflammation associated with blockage of the duct is sometimes termed "obstructive sialadenitis". Because saliva is stimulated to flow more with the thought, sight or smell of food, or with chewing, pain and swelling will often get suddenly worse just before and during a meal ("peri-prandial"), and then slowly decrease after eating, this is termed "meal time syndrome". However, calculi are not the only reasons that a salivary gland may become blocked and give rise to the meal time syndrome. Obstructive salivary gland disease, or obstructive sialadenitis, may also occur due to fibromucinous plugs, duct stenosis, foreign bodies, anatomic variations, or malformations of the duct system leading to a mechanical obstruction associated with stasis of saliva in the duct.
Salivary stones may be divided according to which gland they form in. About 85% of stones occur in the submandibular gland, and between 5-10% occur in the parotid gland. In about 0-5% of cases, the sublingual gland or a minor salivary gland is affected. When minor glands are rarely involved, caliculi are more likely in the minor glands of the buccal mucosa and the maxillary labial mucosa. Submandibular stones are further classified as anterior or posterior in relation to an imaginary transverse line drawn between the mandibular first molar teeth. Stones may be radiopaque, i.e. they will show up on conventional radiographs, or radiolucent, where they not be visible on radiographs (although some of their effects on the gland may still be visible). They may also symptomatic or asymptomatic, according to whether they cause any problems or not.
A ranula usually presents as a translucent blue, dome-shaped, swelling in the tissues of the floor of the mouth. If the lesion is deeper, then there is a greater thickness of tissue separating from the oral cavity and the blue translucent appearance may not be a feature. A ranula can develop into a large lesion many centimeters in diameter, with resultant elevation of the tongue and possibly interfering with swallowing (dysphagia). The swelling is not fixed, may not show blanching and is non-painful unless it becomes secondarily infected. The usual location is usually lateral to the midline, which may be used to help distinguish it from a midline dermoid cyst. A cervical ranula presents as a swelling in the neck, with or without a swelling in the mouth. In common with other mucoceles, ranulas may rupture and then cause recurrent swelling. Ranulas may be asymptomatic, although they can fluctuate rapidly in size, shrinking and swelling, making them hard to detect.
"Dehydration:"
This is a common, non-infectious cause of parotitis. It may occur in elderly or after surgery.
"Chronic nonspecific parotitis:"
This term is generally used for patients in whom no definite cause is found. Episodes may last for several days, paralleling the time course of a bacterial or viral illness. Others may experience episodes that last only a few hours from onset to resolution. Some episodes may last for several weeks. Quiescent periods between episodes last for hours, days, or even years.
"Recurrent parotitis of childhood:"
An uncommon syndrome in which recurring episodes clinically resembling mumps. Generally, episodes begin by age 5 years, and virtually all patients become asymptomatic by age 10–15 years. The duration of attacks averages 3–7 days but may last 2–3 weeks in some individuals. The spectrum varies from mild and infrequent attacks to episodes so frequent that they prevent regular school attendance.
Local heat applied to the gland, massaging the gland from back to front, and taking penicillin usually cure individual episodes. Treatment of individual infections may prevent injury to the gland parenchyma. Severe disease may be treated by parotidectomy.
"Sialadenosis (sialosis):"
In this disorder, both parotid glands may be diffusely enlarged with only modest symptoms. Patients are aged 20–60 years at onset, and the sexes are equally involved. The glands are soft and non-tender. Approximately half of the patients have endocrine disorders such as diabetes, nutritional disorders such as pellagra or kwashiorkor, or have taken drugs such as guanethidine, thioridazine, or isoprenaline.
"Sarcoidosis:"
The lungs, skin, and lymph nodes are most often affected, but the salivary glands are involved in approximately 10% of cases. Bilateral firm, smooth, and non-tender parotid enlargement is classic. Xerostomia occasionally occurs. The Heerfordt-Waldenstrom syndrome consists of sarcoidosis with parotid enlargement, fever, anterior uveitis, and facial nerve palsy.
"IgG4-related sialadenitis":
This term refers to IgG4-related disease (IgG4-RD) involving any of the major salivary glands, i.e. parotid or submandibular glands. This is often symmetrical and is usually associated with manifestations of IgG4-RD elsewhere in the body. IgG4-related sialadenitis is particularly associated with involvement of one or both of the lacrimal glands (referred to as IgG4-related dacryo-sialadenitis). "Mikulicz's disease", now considered to be a subtype of IgG4-related disease, was a term used when (i) any two of the parotid, submandibular and lacrimal glands were persistently and symmetrically enlarged and (ii) other diseases that may mimic this presentation were excluded.
"Pneumoparotitis:"
Air within the ducts of the parotid gland with or without inflammation. The duct orifice normally functions as a valve to prevent air from entering the gland from a pressurized oral cavity. Rarely, an incompetent valve allows insufflation of air into the duct system. Pneumoparotitis most commonly occurs in wind instrument players, glass blowers, and scuba divers.
Several lymph nodes reside within the parotid gland as a superficial and deep group of nodes. These nodes may be involved with any process that affects lymph nodes, including bacterial, fungal, viral, and neoplastic processes. Rarely, drugs such as iodides, phenylbutazone, thiouracil, isoproterenol, heavy metals, sulfisoxazole, and phenothiazines cause parotid swelling.
The fluid within a ranula has the viscous, jellylike consistency of egg white.
Strictures tend to be diagnosed based on difficulty with insertion and manipulation during sialendoscopy, or by sialography or ultrasound.
The size of the swelling is variable, but it is soft and can occur on one side or both sides. It is typically non tender, although sometimes there may be pain. It usually resolves over minutes to hours, however occasionally this may take days. The condition can be transient or recurrent.
Strictures are the second most common cause of chronic obstructive sialadenitis, after salivary stones. In line with this, strictures may give rise to the "meal time syndrome", where there is pain and swelling of the involved salivary gland upon salivary stimulation with the sight, smell and taste of food. In other cases, there is irregular and intermittent pain and swelling of the gland which is not related to meal times. Typically the swelling is present upon waking or occurs before the first meal of the day. After several hours, the swelling goes down suddenly with a rush of foul tasting saliva. Strictures are more common in the parotid duct system compared to the submandibular duct system.
Pneumoparotitis is often misdiagnosed and incorrectly managed. The diagnosis is based mainly on the history. Crepitus may be elicited on palpation of the parotid swelling, and massaging the gland may give rise to frothy saliva or air bubbles from the parotid papilla. Further investigations are not typically required, however sialography, ultrasound and computed tomography may all show air in the parotid gland and duct.
Management is simply by avoidance of the activity causing raised intraoral pressure which is triggering this rare condition.
Salivary gland tumours usually present as a lump or swelling in the affected gland which may or may not have been present for a long time. The lump may be accompanied by symptoms of duct blockage (e.g. xerostomia). Usually, in their early stages it is not possible to distinguish a benign tumour from a malignant one. One of the key differentiating symptoms of a malignant growth is nerve involvement. For example signs of facial nerve damage (e.g facial palsy) are associated with malignant parotid tumours. Facial pain, and paraesthesia are also very often associated with a malignant tumours. Other red flag symptoms which may suggest malignancy and warrant further investigation are fixation of the lump to the overlying skin, ulceration and induration of the mucosa.
True hyposalivation may give the following signs and symptoms:
- Dental caries (xerostomia related caries) – Without the anticariogenic actions of saliva, tooth decay is a common feature and may progress much more aggressively than it would otherwise ("rampant caries"). It may affect tooth surfaces that are normally spared, e.g., cervical caries and root surface caries. This is often seen in patients who have had radiotherapy involving the major salivary glands, termed radiation-induced caries.
- Acid erosion. Saliva acts as a buffer and helps to prevent demineralization of teeth.
- Oral candidiasis – A loss of the antimicrobial actions of saliva may also lead to opportunistic infection with "Candida" species.
- Ascending (suppurative) sialadenitis – an infection of the major salivary glands (usually the parotid gland) that may be recurrent. It is associated with hyposalivation, as bacteria are able to enter the ductal system against the diminished flow of saliva. There may be swollen salivary glands even without acute infection, possibly caused by autoimmune involvement.
- Dysgeusia – altered taste sensation (e.g., a metallic taste) and dysosmia, altered sense of smell.
- Intraoral halitosis – possibly due to increased activity of halitogenic biofilm on the posterior dorsal tongue (although dysgeusia may cause a complaint of nongenuine halitosis in the absence of hyposalivation).
- Oral dysesthesia – a burning or tingling sensation in the mouth.
- Saliva that appears thick or ropey.
- Mucosa that appears dry.
- A lack of saliva pooling in the floor of the mouth during examination.
- Dysphagia – difficulty swallowing and chewing, especially when eating dry foods. Food may stick to the tissues during eating.
- The tongue may stick to the palate, causing a clicking noise during speech, or the lips may stick together.
- Gloves or a dental mirror may stick to the tissues.
- Fissured tongue with atrophy of the filiform papillae and a lobulated, erythematous appearance of the tongue.
- Saliva cannot be "milked" (expressed) from the parotid duct.
- Difficulty wearing dentures, e.g., when swallowing or speaking. There may be generalized mucosal soreness and ulceration of the areas covered by the denture.
- Mouth soreness and oral mucositis.
- Lipstick or food may stick to the teeth.
- A need to sip drinks frequently while talking or eating.
- Dry, sore, and cracked lips and angles of mouth.
- Thirst.
However, sometimes the clinical findings do not correlate with the symptoms experienced. E.g., a person with signs of hyposalivation may not complain of xerostomia. Conversely a person who reports experiencing xerostomia may not show signs of reduced salivary secretions (subjective xerostomia). In the latter scenario, there are often other oral symptoms suggestive of oral dysesthesia ("burning mouth syndrome"). Some symptoms outside the mouth may occur together with xerostomia.
These include:
- Xerophthalmia (dry eyes).
- Inability to cry.
- Blurred vision.
- Photophobia (light intolerance).
- Dryness of other mucosae, e.g., nasal, laryngeal, and/or genital.
- Burning sensation.
- Itching or grittiness.
- Dysphonia (voice changes).
There may also be other systemic signs and symptoms if there is an underlying cause such as Sjögren's syndrome, for example, joint pain due to associated rheumatoid arthritis.
Little is known about the total incidence of salivary gland tumours as most benign tumours go unrecorded in national cancer registries. The majority of salivary tumours are benign (65-70%). Within the parotid gland 75 - 80% of tumours are benign. Around 50% of the tumours found in the submandibular glands are benign. Sublingual gland tumours are very rare but if present, they are most likely to be malignant.
In the United States, salivary gland cancers are uncommon with an incidence rate of 1.7 in 100000 between 2009 and 2013.
Xerostomia is the subjective feeling of oral dryness, which is often (but not always) associated with hypofunction of the salivary glands. The term is derived from the Greek words ξηρός ("xeros") meaning "dry" and στόμα ("stoma") meaning "mouth". A drug or substance that increases the rate of salivary flow is termed a sialogogue.
Hyposalivation is a clinical diagnosis that is made based on the history and examination, but reduced salivary flow rates have been given objective definitions. Salivary gland hypofunction has been defined as any objectively demonstrable reduction in whole and/or individual gland flow rates. An unstimulated whole saliva flow rate in a normal person is 0.3–0.4 ml per minute, and below 0.1 ml per minute is significantly abnormal. A stimulated saliva flow rate less than 0.5 ml per gland in 5 minutes or less than 1 ml per gland in 10 minutes is decreased. The term subjective xerostomia is sometimes used to describe the symptom in the absence of any detectable abnormality or cause. Xerostomia may also result from a change in composition of saliva (from serous to mucous). Salivary gland dysfunction is an umbrella term for the presence of either xerostomia or salivary gland hypofunction.
Presents as painless, slow-growing mass that is firm or hard. Most appear clinically as mixed tumors.
A sialocele is a localized, subcutaneous cavity containing saliva. It is caused by trauma (e.g. violence, accident or surgery) or infection. They most commonly develop about 8–14 days after injury.
They are a relatively common complication following surgery to the salivary glands, commonly parotidectomy (removal of the parotid gland). In this case the sialocele is the result of saliva draining out of remaining parotid tissue, and occurs about 5 to 10% of cases of superficial (partial) parotidectomy.
It is usually not painful, and a mild and self-limiting complication, and is managed by repeated aspiration (draining) of the swelling via a needle after the skin has been disinfected with an antibacterial. The fluid is usually a clear yellow, and contains amylase (in contrast to fluid from a seroma). Pressure dressings do not tend to be used. They are rarely chronic, however if persistent a surgical drain may be required. Botulinum toxin injections have also been used to manage this condition.
IgG4-related disease has been described as an indolent condition. Although possibly based on opinion rather than on objective assessments, symptoms, if any, are commonly described as mild in the medical literature. This can be in spite of considerable underlying organ destruction. People are often described as being generally well at the time of diagnosis, although some may give a history of weight loss.
Pain is generally not a feature of the inflammation. However it may occur as a secondary effect, for example due to either obstruction or compression.
Often diagnosis is made due to the presence of painless swellings or mass lesions, or due to complications of masses, e.g. jaundice due to involvement of the pancreas, biliary tree or liver. Symptoms are commonly attributed to other conditions and other diagnoses may have been made years before diagnosis, e.g. urinary symptoms in men attributed to common prostate conditions. Lesions may also be detected incidentally on radiological images, but can be easily misdiagnosed as malignancies.
Reported cases do include some significant symptoms or findings however:
Hypersalivation (also called ptyalism or sialorrhea) is excessive production of saliva. It has also been defined as increased amount of saliva in the mouth, which may also be caused by decreased clearance of saliva.
Hypersalivation can contribute to drooling if there is an inability to keep the mouth closed or in difficulty in swallowing the excess saliva (dysphagia) and lead to excessive spitting.
Hypersalivation also often precedes emesis (vomiting), where it accompanies nausea (a feeling of needing to vomit).
IgG4-related disease (IgG4-RD), formerly known as IgG4-related systemic disease, is a chronic inflammatory condition characterized by tissue infiltration with lymphocytes and IgG4-secreting plasma cells, various degrees of fibrosis (scarring) and a usually prompt response to oral steroids. In approximately 51–70% of people with this disease, "serum" IgG4 concentrations are elevated during an acute phase.
It is a relapsing–remitting disease associated with a tendency to mass forming, tissue-destructive lesions in multiple sites, with a characteristic histopathological appearance in whichever site is involved. Inflammation and the deposition of connective tissue in affected anatomical sites can lead to organ dysfunction, or even organ failure, if not treated.
Early detection is important to avoid organ damage and potentially serious complications. Treatment is recommended in all symptomatic cases of IgG4-RD and also in asymptomatic IgG4-RD involving certain anatomical sites.