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Males are more commonly affected than females, with firstborn males affected about four times as often, and there is a genetic predisposition for the disease. It is commonly associated with people of Scandinavian ancestry, and has multifactorial inheritance patterns. Pyloric stenosis is more common in Caucasians than Hispanics, Blacks, or Asians. The incidence is 2.4 per 1000 live births in Caucasians, 1.8 in Hispanics, 0.7 in Blacks, and 0.6 in Asians. It is also less common amongst children of mixed race parents. Caucasian male babies with blood type B or O are more likely than other types to be affected.
Infants exposed to erythromycin are at increased risk for developing hypertrophic pyloric stenosis, especially when the drug is taken around two weeks of life and possibly in late pregnancy and through breastmilk in the first two weeks of life.
Pyloric stenosis seems to be multifactorial, with some genetic and some environmental components. It is four times more likely to occur in males, and is also more common in the first born. Rarely, infantile pyloric stenosis can occur as an autosomal dominant condition.
It is uncertain whether it is a congenital anatomic narrowing or a functional hypertrophy of the pyloric sphincter muscle.
Approximately 20–40 percent of all infants with duodenal atresia have Down syndrome. . Approximately 8% of infants with Down syndrome have duodenal atresia.
Prognosis is usually very good, although complications are more likely to occur when there are serious congenital anomalies. Late complications may occur in about 12 percent of patients with duodenal atresia, and the mortality rate for these complications is 6 percent.
The causes are divided into benign or malignant.
- Benign
- Peptic ulcer disease
- Infections, such as tuberculosis; and infiltrative diseases, such as amyloidosis.
- A rare cause of gastric outlet obstruction is blockage with a gallstone, also termed "Bouveret's syndrome".
- In children congenital pyloric stenosis / congenital hypertrophic pyloric stenosis may be a cause.
- A pancreatic pseudocyst can cause gastric compression.
- Pyloric mucosal diaphragm could be a rare cause.
- Malignant
- Tumours of the stomach, including adenocarcinoma (and its linitis plastica variant), lymphoma, and gastrointestinal stromal tumours
In a peptic ulcer it is believed to be a result of edema and scarring of the ulcer, followed by healing and fibrosis, which leads to obstruction of the gastroduodenal junction (usually an ulcer in the first part of the duodenum).
Subglottic stenosis is a congenital or acquired narrowing of the subglottic airway. Although it is relatively rare, it is the third most common congenital airway problem (after laryngomalacia and vocal cord paralysis). Subglottic stenosis can present as a life-threatening airway emergency. It is imperative that the otolaryngologist be an expert at dealing with the diagnosis and management of this disorder. Subglottic stenosis can affect both children and adults.
Subglottic stenosis can be of three forms, namely congenital subglottic stenosis, idiopathic subglottic stenosis (ISS) and acquired subglottic stenosis. As the name suggests, congenital subglottic stenosis is a birth defect. Idiopathic subglottic stenosis is a narrowing of the airway due to an unknown cause. Acquired subglottic stenosis generally follows as an after-effect of airway intubation, and in extremely rare cases as a result of gastroesophageal reflux disease (GERD).
Subglottic stenosis is graded according to the Cotton-Meyer classification system from one to four based on the severity of the blockage.
Grade 1 – <50% obstruction
Grade 2 – 51–70% obstruction
Grade 3 – 71–99% obstruction
Grade 4 – no detectable lumen
Treatments to alleviate the symptoms of subglottic stenosis includes a daily dose of steroids such as prednisone, which reduces the inflammation of the area for better breathing. Other medications such as Methotrexate is also being tested by patients but results are pending.
Gastric atresia is the complete occlusion of the pyloric outlet of the stomach and is an extremely rare event.
Frisch & Simonsen (2016) carried out a very large scale study in Denmark, which compared the incidence of meatal stenosis in Muslim males (mostly circumcised) with the incidence of meatal stenosis in ethnic Danish males (mostly non-circumcised). The risk of meatal stenosis in circumcised males was found to be as much 3.7 times higher than in the intact, non-circumcised males.
Medication (to prevent spasms) or Sphincterotomy (surgical procedure to cut the muscle) are the standard treatments for sphincter of Oddi dysfunction. One or the other may be better based on the classification of the condition.
The resulting syndrome depends on the structure affected.
Examples of vascular stenotic lesions include:
- Intermittent claudication (peripheral artery stenosis)
- Angina (coronary artery stenosis)
- Carotid artery stenosis which predispose to (strokes and transient ischaemic episodes)
- Renal artery stenosis
The types of stenoses in heart valves are:
- Pulmonary valve stenosis, which is the thickening of the pulmonary valve, therefore causing narrowing
- Mitral valve stenosis, which is the thickening of the mitral valve (of the left heart), therefore causing narrowing
- Tricuspid valve stenosis, which is the thickening of the tricuspid valve (of the right heart), therefore causing narrowing
- Aortic valve stenosis, which is the thickening of the aortic valve, therefore causing narrowing
Stenoses/strictures of other bodily structures/organs include:
- Pyloric stenosis (gastric outflow obstruction)
- Lumbar, cervical or thoracic spinal stenosis
- Subglottic stenosis (SGS)
- Tracheal stenosis
- Obstructive jaundice (biliary tract stenosis)
- Bowel obstruction
- Phimosis
- Non-communicating hydrocephalus
- Stenosing tenosynovitis
- Atherosclerosis
- Esophageal stricture
- Achalasia
- Prinzmetal angina
- Vaginal stenosis
Most damage to the pyloric valve occurs as a complication of gastric surgery. Other causes of biliary reflux may be:
- Peptic ulcer
- Gallbladder surgery (cholecystectomy)
A significant fraction of cases are idiopathic, with no identified specific etiology.
Numerous studies over a long period of time clearly indicate that male circumcision contributes to the development of urethral stricture. Among circumcised males, reported incidence of meatal stricture varies. Griffiths "et al". (1985) reported an incidence of 2.8 percent. Sörensen & Sörensen (1988) reported 0 percent. Cathcart "et al". (2006) reported an incidence of 0.55 percent. Yegane "et al". (2006) reported an incidence of 0.9 percent. Van Howe (2006) reported an incidence of 7.29 percent. In Van Howe's study, all cases of meatal stenosis were among circumcised boys. Simforoosh "et al". (2010) reported an incidence of 0.55 percent. According to Emedicine (2016), the incidence of meatal stenosis runs from 9 to 20 percent. Frisch & Simonsen (2016) placed the incidence at 5 to 20 percent of circumcised boys.
Sphincter of Oddi dysfunction may be suggested by pain which seems to come from a biliary origin, which may or may not be associated with transient increases of liver or pancreatic enzymes. Common bile duct dilation and episodes of pancreatitis are also signs.
Laryngotracheal stenosis refers to abnormal narrowing of the central air passageways. This can occur at the level of the larynx, trachea, carina or main bronchi.
In a small number of patients narrowing may be present in more than one anatomical location.
The treatment for bile reflux is the same as the treatment for acidic reflux. In general, everything that can
reduce acidic reflux can reduce bile reflux. Examples include lifestyle modification, weight reduction, and the avoidance of eating immediately before sleep or being in the supine position immediately after meals. In addition, smoking has been found to be a factor in the development of acidic reflux. Thus, all of these factors should be applied to bile reflux as well.
Likewise, drugs that reduce the secretion of gastric acid (e.g., proton pump inhibitors)
or that reduce gastric contents or volume can be used to treat acidic bile reflux. Because prokinetic drugs increase the motility of the stomach and accelerate gastric emptying, they can also reduce bile reflux. Other drugs that reduce the relaxations of the lower esophageal sphincter, such as baclofen, have also proven to reduce bile reflux, particularly in patients who are refractory to (medically unresponsive to) proton pump inhibitor therapy.
Medications used in managing biliary reflux include bile acid sequestrants, particularly cholestyramine, which disrupt the circulation of bile in the digestive tract and sequester bile that would otherwise cause symptoms when refluxed; and prokinetic agents, to move material from the stomach to the small bowel more rapidly and prevent reflux.
Biliary reflux may also be treated surgically, if medications are ineffective or if precancerous tissue is present in the esophagus.
A stenosis is an abnormal narrowing in a blood vessel or other tubular organ or structure. It is also sometimes called a stricture (as in urethral stricture).
Stricture as a term is usually used when narrowing is caused by contraction of smooth muscle (e.g., achalasia, prinzmetal angina); stenosis is usually used when narrowing is caused by lesion that reduces the space of lumen (e.g., atherosclerosis). The term coarctation is another synonym, but is commonly used only in the context of aortic coarctation.
Restenosis is the recurrence of stenosis after a procedure. The term is from Ancient Greek στενός, "narrow".
A genetic disorder called “Brickers-Adams-Edwards syndrome” or “X-linked hydrocephalus” has been discovered that leads to aqueductal stenosis. This disease is transmitted from mother to son. This disorder is caused by a point mutation in the gene for neural cell adhesion. Most males born with this have severe hydrocephalus, adducted thumbs, spastic motions, and intellectual problems. Females with this defect may have adducted thumbs or subnormal intelligence.
Bacterial meningitis can also result in gliotic blockage of the aqueduct. In utero infection or infection during infancy could both result in glial cell build up to make an obstruction.
The exact prevalence of the syndrome is unknown, because both aortic stenosis and angiodysplasia are common diseases in the elderly. A retrospective chart review of 3.8 million people in Northern Ireland found that the incidence of gastrointestinal bleeding in people with any diagnosis of aortic stenosis (they did not subgroup people by severity) was just 0.9%. They also found that the reverse correlation—the incidence of aortic stenosis in people with gastrointestinal bleeding—was 1.5%. However, in 2003 a study of 50 people with aortic stenosis severe enough to warrant immediate valve replacement found GI bleeding in 21% of people, and another study done in the USA looking at angiodysplasia rather than GI bleeding found that the prevalence of aortic stenosis was 31% compared to 14% in the control group.
An extracranial shunt is essentially a sturdy tube with a catheter on one end to drain the third ventricle. The shunt also has a valve which serves to maintain one-way flow of the CSF and regulates the flow rate. The end with the catheter is placed in the third ventricle to drain the excess CSF and the other end is placed in the peritoneal cavity or atrium of the heart (making it a ventriculoperitoneal or ventriculoatrial shunt, respectively). The excess CSF which is diverted to a cavity is then reabsorbed by the surrounding tissue where it is drained to.
The procedure to insert this device is a technically straightforward endoscopic surgery with a low mortality rate (essentially 0% mortality since the 1970s). If the shunt has an adjustable valve the current method of setting the valve pressure is to choose one setting, observe the patient to see if CSF flow improves and the symptoms lessen over time, and adjust the pressure setting as needed if improvement isn't seen. For example, if there is not enough CSF flow, another surgery is performed to lower the valve pressure so that less force needs to be applied to open the valve and thereby drain more CSF.
This treatment method has several possible problems with it (with a 50% failure rate in 2 years), and unfortunately shunt malfunctions and associated complications cause a death rate of 1.2% per year. Problems which can necessitate a secondary surgery to fix them include: mechanical failure, incorrect catheter size, inappropriate valve drainage pressure, and infection.
- Inappropriate valve pressure can lead to "overdraining" or "underdraining", both of which should be treated by adjusting the valve pressure. Overdraining occurs when the valve pressure is too low and CSF flows out of the third ventricle too quickly. The ventricle then collapses and blood vessles can be torn in the process. This in turn can lead to headache, hemorrhage, or slit ventricle syndrome. Underdraining occurs when the valve pressure is too high and CSF flows out too slowly. This results in symptoms of hydrocephalus as the CSF is still collecting rather than being absorbed or diverted.
- Risk of infection is due to the fact that a foreign object is being introduced into the body. Infection can have symptoms of fever and soreness of the neck and shoulders.
Laryngotracheal stenosis is an umbrella term for a wide and heterogeneous group of very rare conditions. The population incidence of adult post-intubation laryngotracheal stenosis which is the commonest benign sub-type of this condition is approximately 1 in 200,000 adults per year. The main causes of adult laryngotracheal stenosis are:
Heyde's syndrome is a syndrome of gastrointestinal bleeding from angiodysplasia in the presence of aortic stenosis.
It is named after Edward C. Heyde, MD who first noted the association in 1958. It is caused by the induction of Von Willebrand disease type IIA (vWD-2A) by a depletion of Von Willebrand factor (vWF) in blood flowing through the narrowed valvular stenosis.
The epidemiology of pulmonary valve stenosis can be summed up by the congenital aspect which is the majority of cases, in broad terms PVS is rare in the general population.
Anal stricture or anal stenosis is a narrowing of the anal canal. It can be caused by a number of surgical procedures including: hemorrhoid removal and following anorectal wart treatment.
If untreated, severe symptomatic aortic stenosis carries a poor prognosis with a 2-year mortality rate of 50-60% and a 3-year survival rate of less than 30%. Prognosis after aortic valve replacement for people who are younger than 65 is about five years less than that of the general population; for people older than 65 it is about the same.