Salivary gland atresia is congenital blockage or absence of the orifice of a major salivary gland duct or part of the duct itself.

It is a very rare condition. The submandibular salivary gland duct is usually involved, having failed to cannulate during embryological development. The condition first becomes apparent in the first few days after birth where a submandibular swelling caused by a retention cyst is noticed.

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.

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.

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.

Strictures tend to be diagnosed based on difficulty with insertion and manipulation during sialendoscopy, or by sialography or ultrasound.

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.

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.

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.

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.

The size of oral mucoceles vary from 1 mm to several centimeters and they usually are slightly transparent with a blue tinge. On palpation, mucoceles may appear fluctuant but can also be firm. Their duration lasts from days to years, and may have recurrent swelling with occasional rupturing of its contents.

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).

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.

Salivary gland hypoplasia is relative underdevelopment of the Salivary glands. Salivary gland hypoplasia tends to produce xerostomia (dry mouth), with all the associated problems this brings.

It is a rare condition, which may occur as a congenital abnormality or result from lack of neuromuscular stimulation.

It may be associated with Melkersson–Rosenthal syndrome, and hereditary ectodermal dysplasia.

The most common location to find a mucocele is the inner surface of the lower lip. It can also be found on the inner side of the cheek (known as the buccal mucosa), on the anterior ventral tongue, and the floor of the mouth. When found on the floor of the mouth, the mucocele is referred to as a ranula. They are rarely found on the upper lip. As their name suggests they are basically mucus lined cysts and they can also occur in the Paranasal sinuses most commonly the frontal sinuses, the frontoethmoidal region and also in the maxillary sinus. Sphenoid sinus involvement is extremely rare.

When the lumen of the vermiform appendix gets blocked due to any factor, again a mucocele can form.

The fluid within a ranula has the viscous, jellylike consistency of egg white.

There are no symptoms, and no signs can be elicited on examination. Medical imaging such as traditional radiography or computed tomography is required to demonstrate the defect. Usually the defect is unilateral, but occasionally can be bilateral.

It can be unilateral or bilateral.

- Sometimes, a unilateral choanal atresia is not detected until much later in life because the baby manages to get along with only one nostril available for breathing.

- Bilateral choanal atresia is a very serious life-threatening condition because the baby will then be unable to breathe directly after birth as babies are obligate nasal breathers (they mainly use their noses to breathe). In some cases, this may present as cyanosis while the baby is feeding, because the oral air passages are blocked by the tongue, further restricting the airway. The cyanosis may improve when the baby cries, as the oral airway is used at this time. These babies may require airway resuscitation soon after birth.

Salivary gland hyperplasia is hyperplasia of the terminal duct of salivary glands.

There are two types:

- Acinar adenomatoid hyperplasia

- Ductal adenomatoid hyperplasia

It is a classed as a pseudocyst, since there is no epithelial lining or fluid content. This defect is usually considered with other cysts of the jaws, since it can be mistaken for such on a radiograph.

Atresia is a condition in which an orifice or passage in the body is (usually abnormally) closed or absent.

Examples of atresia include:

- Biliary atresia, a condition in newborns in which the common bile duct between the liver and the small intestine is blocked or absent.

- Choanal atresia, blockage of the back of the nasal passage, usually by abnormal bony or soft tissue.

- Esophageal atresia, which affects the alimentary tract and causes the esophagus to end before connecting normally to the stomach.

- Imperforate anus, malformation of the opening between the rectum and anus.

- Intestinal atresia, malformation of the intestine, usually resulting from a vascular accident in utero.

- Microtia, absence of the ear canal or failure of the canal to be tubular or fully formed (can be related to Microtia, a congenital deformity of the pinna, or outer ear).

- Ovarian follicle atresia, the degeneration and subsequent resorption of one or more immature ovarian follicles.

- Potter sequence, congenital decreased size of the kidney leading to absolutely no functionality of the kidney, usually related to a single kidney.

- Pulmonary atresia, malformation of the pulmonary valve in which the valve orifice fails to develop.

- Renal agenesis, only having one kidney.

- Tricuspid atresia, a form of congenital heart disease whereby there is a complete absence of the tricuspid valve, and consequently an absence of the right atrioventricular connection.

- Vaginal atresia, a congenital occlusion of the vagina or subsequent adhesion of the walls of the vagina, resulting in its occlusion.

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'.

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.

Sometimes babies born with choanal atresia also have other abnormalities:

- coloboma

- heart defects

- mental retardation

- growth impairment

- others (see also CHARGE syndrome)

Also any condition that causes significant depression of the nasal bridge or midface retraction can be associated with choanal atresia. Examples include the craniosynostosis syndromes such as Crouzon syndrome, Pfeiffer syndrome, Treacher Collins and Antley-Bixler syndrome.

The condition most commonly is located at the junction of the hard and soft palate. However, the condition may arise anywhere minor salivary glands are located. It has also been occasionally reported to involve the major salivary glands. It may be present only on one side, or both sides. The lesion typically is 1–4 cm in diameter.

Initially, the lesion is a tender, erythematous (red) swelling. Later, in the ulcerated stage, the overlying mucosa breaks down to leave a deep, well-circumscribed ulcer which is yellow-gray in color and has a lobular base.

There is usually only minor pain, and the condition is often entirely painless. There may be prodromal symptoms similar to flu before the appearance of the lesion.