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Deep Learning Technology: Sebastian Arnold, Betty van Aken, Paul Grundmann, Felix A. Gers and Alexander Löser. Learning Contextualized Document Representations for Healthcare Answer Retrieval. The Web Conference 2020 (WWW'20)
Funded by The Federal Ministry for Economic Affairs and Energy; Grant: 01MD19013D, Smart-MD Project, Digital Technologies
It may appear on a CT scan or MRI scan as enhancement and dilation of the duct (sialectasis).
On sialography, it may appear as segments of duct dilation and stenosis. This is sometimes termed the 'sausage link appearance'.
Sialodochitis (also termed ductal sialadenitis), is inflammation of the duct system of a salivary gland. This is compared to sialadenitis, which is inflammation of the gland parenchyma.
Sialodochitis may be associated with salivary duct strictures and salivary stones.
It is common in both the parotid glands and submandibular glands.
The treatment is as for sialadenitis.
In chronic recurrent sialadenitis or chronic sclerosing sialadenitis, acute attacks are managed with conservative therapies such as hydration, analgesics (mainly NSAIDs), sialogogues to stimulate salivary secretion, and regular, gentle gland massage. If infection is present, appropriate cultures should be obtained, followed by empirical antibiotic therapy initially, for example amoxicillin/clavulanate or clindamycin which cover oral flora.
If there are attacks more than approximately 3 times per year or severe attacks, surgical excision of the affected gland should be considered.
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.
Sialadenitis (sialoadenitis) is inflammation of a salivary gland. It may be subdivided temporally into acute, chronic and recurrent forms.
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.
Blockage of the main parotid duct, or one of its branches, is often a primary cause of acute parotitis, with further inflammation secondary to bacterial superinfection. The blockage may be from a salivary stone, a mucous plug, or, more rarely, by a tumor, usually benign. Salivary stones (also called sialolithiasis, or salivary duct calculus) are mainly made of calcium, but do not indicate any kind of calcium disorder. Stones may be diagnosed via X-ray (with a success rate of about 80%), a computed tomography (CT) scan or Medical ultrasonography. Stones may be removed by manipulation in the doctor's office, or, in the worst cases, by surgery. Lithotripsy, also known as "shock wave" treatment, is best known for its use breaking up kidney stones. Lithotripsy can now be used on salivary stones as well. Ultrasound waves break up the stones, and the fragments flush out of the salivary duct.
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.
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.
"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.
Strictures tend to be diagnosed based on difficulty with insertion and manipulation during sialendoscopy, or by sialography or ultrasound.
Infections involving the salivary glands can be viral or bacterial (or rarely fungal).
- Mumps is the most common viral sialadenitis. It usually occurs in children, and there is preauricular pain (pain felt in front of the ear), swelling of the parotid, fever, chills, and headaches.
- Bacterial sialadentitis is usually caused by ascending organisms from the oral cavity. Risk factors include reduced salivary flow rate.
- Human immunodeficiency virus-associated salivary gland disease (HIV-SGD).
Chronic sclerosing sialadenitis is a chronic (long-lasting) inflammatory condition affecting the salivary gland. Relatively rare in occurrence, this condition is benign, but presents as hard, indurated and enlarged masses that are clinically indistinguishable from salivary gland neoplasms or tumors. It is now regarded as a manifestation of IgG4-related disease.
Involvement of the submandibular glands is also known as Küttner's tumor, named after Hermann Küttner (1870–1932), a German Oral and Maxillofacial Surgeon, who reported four cases of submandibular gland lesions for the first time in 1896.
The inflammatory lesions in Küttner's tumor may occur on one side (unilateral) or both sides (bilateral), predominantly involving the submandibular gland, but is also known to occur in other major and minor salivary glands, including the parotid gland.
Overall, salivary gland tumors are relatively rare, with approximately 2.5–3 cases per 100,000 people per year seen in the Western world; however, salivary gland malignancies account for 3–5% of all head and neck cancers. However, salivary tumors show a great deal of morphological diversity, as well as variations in the nature of the lesion (malignant vs. benign): approximately 20% to 25% of parotid tumors, 35% to 40% of submandibular tumors, and more than 90% of sublingual gland tumors are malignant. This situation underscores the diagnostic challenges in respect of Küttner's tumor; despite being benign, this condition mimics the clinical appearance of malignancy in the salivary gland.
The swollen masses of Küttner's tumor are generally painful, and patients are advised surgical resection (known as 'sialadenectomy') of a part or whole of the glandular tissue upon suspicion of possible malignancy. It is only upon post-surgical histopathology of the excised mass that the diagnosis of Küttner's tumor is definitively made.
The term nonpuerperal mastitis describes inflammatory lesions of the breast (mastitis) that occur unrelated to pregnancy and breastfeeding.
It is sometimes equated with duct ectasia, but other forms can be described.
"Duct ectasia" in the literal sense (literally: duct widening) is a very common and thus rather unspecific finding, increasing with age. However, in the way in which the term is mostly used, duct ectasia is an inflammatory condition of the larger-order lactiferous ducts. It considered likely that the condition is associated with aseptic (chemical) inflammation related to the rupture of ducts or cysts. It is controversial whether duct dilation occurs first and leads to secretory stasis and subsequent periductal inflammation or whether inflammation occurs first and leads to an inflammatory weakening of the duct walls and then stasis. When the inflammation is complicated by necrosis and secondary bacterial infection, breast abscesses may form. Subareolar abscess, also called Zuska's disease (only nonpuerperal case), is a frequently aseptic inflammation and has been associated with squamous metaplasia of the lactiferous ducts.
The duct ectasia—periductal mastitis complex affects two groups of women: young women (in their late teens and early 20s) and perimenopausal women. Women in the younger group mostly have inverted nipples due to squamous metaplasia that lines the ducts more extensively compared to other women and produces keratin plugs which in turn lead to duct obstruction and then duct dilation, secretory stasis, inflammation, infection and abscess. This is not typically the case for women in the older group; in this group, there is likely a multifactorial etiology involving the balance in estrogen, progesterone and prolactin.
Treatment of mastitis and/or abscess in nonlactating women largely the same as that of lactational mastitis, generally involving antibiotics treatment, possibly surgical intervention by means of fine-needle aspiration and/or incision and drainage and/or interventions on the lactiferous ducts (for details, "see also" the articles on treatment of mastitis, of breast abscess and of subareolar abscess). Additionally, an investigation for possible malignancy is needed, normally by means of mammography, and a pathological investigation such as a biopsy may be necessary to exclude malignant mastitis. Although no "causal" relation with breast cancer has been established, there appears to be an increased statistical risk of breast cancer, warranting a long-term surveillance of patients diagnosed with non-puerperal mastitis.
Nonpuerperal breast abscesses have a higher rate of recurrence compared to puerperal breast abscesses. There is a high statistical correlation of nonpuerperal breast abscess with diabetes mellitus (DM). On this basis, it has recently been suggested that diabetes screening should be performed on patients with such abscess.
Patients mostly present with a hard lump in one breast without any sign of a systemic disease. Other possible symptoms include nipple retraction, pain, inflammation of the overlying skin, nipple discharge, fistula, enlarged lymph nodes, in rare case peau d'orange-like changes.
Presentation is mostly unilateral although a significant share of cases is bilateral, also in many cases contralateral or bilateral recurrences were documented.
Several cases occurring together with fever, polyarthralgia and erythema nodosum were documented.
Characteristic for idiopathic granulomatous mastitis are multinucleated giant cells and epithelioid histiocytes forming non-caseating granulomas around lobules. Often minor ductal and periductal inflammation is present. The lesion is in some cases very difficult to distinguish from breast cancer and other causes such as infections (tuberculosis, syphilis, corynebacterial infection, mycotic infection), autoimmune diseases (sarcoidosis, granulomatosis with polyangiitis), foreign body reaction and granulomatous reaction in a carcinoma must be excluded.
The condition is diagnosed very rarely. As the diagnosis is a lengthy differential diagnosis of exclusion there is considerable uncertainty about incidence. It has been suspected that some cases diagnosed as IGM in developing countries may have other explanations. On the other hand, IGM is usually diagnosed only after complications and referral to a secondary breast care center so light cases may resolve spontaneously or after symptomatic treatment and thus never be diagnosed as IGM. As a completely pathogen free breast will be exceedingly rare even in completely healthy population there is also uncertainty when to consider pathogens as causative or as mere coincidental finding.
Idiopathic granulomatous mastitis is defined as granulomatous mastits without any other attributable cause such as those above mentioned. It occurs on average two years and almost exclusively up to six years after pregnancy, usual age range is 17 to 42 years. Some cases have been reported that were related to drug induced hyperprolactinemia.
Exceptionally rarely it has been diagnosed during pregnancy and in men.
The presentation is dependent upon the underlying cause. The course can be rapid or chronic.
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.
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:
Vanishing bile duct syndrome (or "ductopenia") is a loose collection of diseases which leads to the injury to hepatic bile ducts and eventual ductopenia.
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.
Autoimmune pancreatitis (AIP) is an increasingly recognized type of chronic pancreatitis that can be difficult to distinguish from pancreatic carcinoma but which responds to treatment with corticosteroids, particularly prednisone. There are two categories of AIP: Type 1 and Type 2, each with distinct clinical profiles.
Type 1 AIP is now regarded as a manifestation of IgG4-related disease, and those affected have tended to be older and to have a high relapse rate. Type 1 is associated with pancreatitis, Sjogren syndrome, Primary sclerosing cholangitis and Inflammatory bowel disease. Patients with Type 2 AIP do not experience relapse, tend to be younger and not associated with systemic disease. AIP occurring in association with an autoimmune disorder has been referred to as "secondary" or "syndromic" AIP. AIP does not affect long-term survival.
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.