Esophageal dysphagia
Encyclopedia
Esophageal dysphagia arises from the body of the esophagus, lower esophageal sphincter, or cardia of the stomach, usually due to mechanical causes or motility problems.
(DES) or nonspecific esophageal motility disorder (NEMD). Progressive motility dysphagia disorders include scleroderma
or achalasia
with chronic heartburn, regurgitation, respiratory problems, or weight loss. Intermittent mechanical dysphagia is likely to be an esophageal ring. Progressive mechanical dysphagia is most likely due to peptic stricture or esophageal cancer
.
Etiology
Esophageal stricture
, or narrowing of the esophagus, is usually a complication of acid reflux, most commonly due to gastroesophageal reflux (GERD
). These patients are usually older and have had GERD for a long time. Esophageal stricture can also be due to other causes, such as acid reflux from Zollinger-Ellison syndrome
, trauma from NG tube placement, and chronic acid exposure in patients with poor esophageal motility from scleroderma. Other non-acid related causes of peptic strictures include infectious esophagitis, ingestion of chemical irritant, pill irritation, and radiation. Peptic stricture is a progressive mechanical dysphagia, meaning patients will complain of initial intolerance to solids followed by inability to tolerate liquids. When the diameter of the stricture is less than 12 mm the patient will always have dysphagia, while dysphagia is not seen when the diameter of the stricture is above 30 mm. Symptoms relating to the underlying cause of the stricture usually will also be present.
Esophageal cancer
also presents with progressive mechanical dysphagia. Patients usually come with
rapidly progressive dysphagia first with solids then with liquids, weight loss (> 10 kg), and anorexia (loss of appetite). Esophageal cancer usually affects the elderly. Esophageal cancers can be either squamous cell carcinoma or adenocarcinoma
. Adenocarcinoma is the most prevalent in the US and is associated with patients with chronic GERD who has developed Barrett's esophagus
(intestinal metaplasia
of esophageal mucosa). Squamous cell carcinoma is more prevalent in Asia and is associated with tobacco smoking and alcohol use.
Esophageal rings and web
s, are actual rings and webs of tissue that may occlude the esophageal lumen.
Achalasia
is an idiopathic motility disorder characterized by failure of lower esophageal sphincter (LES) relaxation as well as loss of peristalsis
in the distal esophagus, which is mostly smooth muscle. Both of these features impair the ability of the esophagus to empty contents into the stomach. Patients usually complain of dysphagia to both solids and liquids. Dysphagia to liquids, in particular, is a characteristic of achalasia. Other symptoms of achalasia include regurgitation, night coughing, chest pain, weight loss, and heartburn. The combination of achalasia, adrenal insufficiency, and alacrima (lack of tear production) in children is known as the triple A (Allgrove) syndrome
. In most cases the cause is unknown (idiopathic), but in some regions of the world, achalasia can also be caused by Chagas disease
due to infection by Trypanosoma cruzi
.
Scleroderma
is a disease characterized by atrophy
and sclerosis
of the gut wall, most commonly of the distal esophagus (~90%). Consequently, the lower esophageal sphincter cannot close and this can lead to severe gastroesophageal reflux disease (GERD). Patients typically present with progressive dysphagia to both solids and liquids secondary to motility problems or peptic stricture from acid reflux.
Spastic motility disorders include diffuse esophageal spasm
(DES), nutcracker esophagus
, hypertensive lower esophageal sphincter, and nonspecific spastic esophageal motility disorders (NEMD).
Rare causes of esophageal dysphagia not mentioned above
. Commonly patients are found to have esophagitis and may have an esophageal stricture. Biopsies are usually done to look for evidence of esophagitis even if the EGD is normal. Usually no further testing is required if the diagnosis is established on EGD. Repeat endoscopy may be needed for follow up.
If there is a suspicion of a proximal lesion such as:
a barium swallow
may be performed before endoscopy to help identify abnormalities that might increase the risk of prevent at the time of endoscopy.
If achalasia suspected an upper endoscopy is required to exclude a malignancy as a cause of the findings on barium swallow. Manometry
is performed next to confirm. A normal endoscopy should be followed by manometry, and if manometry is also normal, the diagnosis is functional dysphagia.
.
Symptoms, signs, and evaluation
Patients usually complain of dysphagia (the feeling of food getting stuck several seconds after swallowing), and will point to the suprasternal notch or behind the sternum as the site of obstruction. If there is dysphagia to both solids and liquids, then it is most likely a motility problem. If there is dysphagia initially to solids but progresses to also involve liquids, then it is most likely a mechanical obstruction. Once a distinction has been made between a motility problem and a mechanical obstruction, it is important to note whether the dysphagia is intermittent or progressive. An intermittent motility dysphagia likely can be diffuse esophageal spasmDiffuse esophageal spasm
Diffuse esophageal spasm is a condition in which uncoordinated contractions of the esophagus occur. It is thought to result from motility disorders of the esophagus. These spasms do not propel food effectively to the stomach...
(DES) or nonspecific esophageal motility disorder (NEMD). Progressive motility dysphagia disorders include scleroderma
Scleroderma
Systemic sclerosis or systemic scleroderma is a systemic autoimmune disease or systemic connective tissue disease that is a subtype of scleroderma.-Skin symptoms:...
or achalasia
Achalasia
Achalasia , also known as esophageal achalasia, achalasia cardiae, cardiospasm, and esophageal aperistalsis, is an esophageal motility disorder involving the smooth muscle layer of the esophagus and the lower esophageal sphincter...
with chronic heartburn, regurgitation, respiratory problems, or weight loss. Intermittent mechanical dysphagia is likely to be an esophageal ring. Progressive mechanical dysphagia is most likely due to peptic stricture or esophageal cancer
Esophageal cancer
Esophageal cancer is malignancy of the esophagus. There are various subtypes, primarily squamous cell cancer and adenocarcinoma . Squamous cell cancer arises from the cells that line the upper part of the esophagus...
.
Tree diagram of esophageal dysphagia
Schematically the above can be presented as a tree diagram:EtiologyEtiologyEtiology is the study of causation, or origination. The word is derived from the Greek , aitiologia, "giving a reason for" ....
and differential diagnosisDifferential diagnosisA differential diagnosis is a systematic diagnostic method used to identify the presence of an entity where multiple alternatives are possible , and may also refer to any of the included candidate alternatives A differential diagnosis (sometimes abbreviated DDx, ddx, DD, D/Dx, or ΔΔ) is a...
(causes)
Esophageal stricture
Benign esophageal stricture is a narrowing or tightening of the esophagus that causes swallowing difficulties.-Causes:It can be caused by or associated with gastroesophageal reflux disease, esophagitis , dysfunctional lower esophageal sphincter, disordered motility, lye ingestion, or a hiatal hernia...
, or narrowing of the esophagus, is usually a complication of acid reflux, most commonly due to gastroesophageal reflux (GERD
Gerd
In Norse mythology, Gerðr is a jötunn, goddess, and the wife of the god Freyr. Gerðr is attested in the Poetic Edda, compiled in the 13th century from earlier traditional sources; the Prose Edda and Heimskringla, written in the 13th century by Snorri Sturluson; and in the poetry of skalds...
). These patients are usually older and have had GERD for a long time. Esophageal stricture can also be due to other causes, such as acid reflux from Zollinger-Ellison syndrome
Zollinger-Ellison syndrome
Zollinger–Ellison syndrome is a triad of gastric acid hypersecretion, severe peptic ulceration, and non-beta cell islet tumor of pancreas . In this syndrome increased levels of the hormone gastrin are produced, causing the stomach to produce excess hydrochloric acid. Often the cause is a tumor of...
, trauma from NG tube placement, and chronic acid exposure in patients with poor esophageal motility from scleroderma. Other non-acid related causes of peptic strictures include infectious esophagitis, ingestion of chemical irritant, pill irritation, and radiation. Peptic stricture is a progressive mechanical dysphagia, meaning patients will complain of initial intolerance to solids followed by inability to tolerate liquids. When the diameter of the stricture is less than 12 mm the patient will always have dysphagia, while dysphagia is not seen when the diameter of the stricture is above 30 mm. Symptoms relating to the underlying cause of the stricture usually will also be present.
Esophageal cancer
Esophageal cancer
Esophageal cancer is malignancy of the esophagus. There are various subtypes, primarily squamous cell cancer and adenocarcinoma . Squamous cell cancer arises from the cells that line the upper part of the esophagus...
also presents with progressive mechanical dysphagia. Patients usually come with
rapidly progressive dysphagia first with solids then with liquids, weight loss (> 10 kg), and anorexia (loss of appetite). Esophageal cancer usually affects the elderly. Esophageal cancers can be either squamous cell carcinoma or adenocarcinoma
Adenocarcinoma
Adenocarcinoma is a cancer of an epithelium that originates in glandular tissue. Epithelial tissue includes, but is not limited to, the surface layer of skin, glands and a variety of other tissue that lines the cavities and organs of the body. Epithelium can be derived embryologically from...
. Adenocarcinoma is the most prevalent in the US and is associated with patients with chronic GERD who has developed Barrett's esophagus
Barrett's esophagus
Barrett's esophagus refers to an abnormal change in the cells of the inferior portion of the esophagus. A positive diagnosis generally requires observing specific macroscopic and microscopic changes...
(intestinal metaplasia
Metaplasia
Metaplasia is the reversible replacement of one differentiated cell type with another mature differentiated cell type. The change from one type of cell to another may generally be a part of normal maturation process or caused by some sort of abnormal stimulus...
of esophageal mucosa). Squamous cell carcinoma is more prevalent in Asia and is associated with tobacco smoking and alcohol use.
Esophageal rings and web
Esophageal web
-Presentation:Its main symptoms are pain and difficulty in swallowing .Esophageal webs are thin membranes of normal esophageal tissue consisting of mucosa and submucosa. They can be congenital or acquired. Congenital webs commonly appear in the middle and inferior third of the esophagus, and they...
s, are actual rings and webs of tissue that may occlude the esophageal lumen.
- Rings --- Also known as Schatzki ringSchatzki ringA Schatzki ring or Schatzki-Gary ring is a narrowing of the lower part of the esophagus that can cause difficulty swallowing. The narrowing is caused by a ring of mucosal tissue or muscular tissue. Patients with Schatzki rings can develop intermittent dysphagia , or, more seriously, a completely...
s from the discoverer, these rings are usually mucosal rings rather than muscular rings, and are located near the gastroesophageal junction at the squamo-columnar junction. Presence of multiple rings may suggest eosinophilic esophagitisEosinophilic esophagitisEosinophilic esophagitis is an allergic inflammatory condition of the esophagus. Symptoms are swallowing difficulty, food impaction, and heartburn. The disease was first described in children but occurs in adults as well...
. Rings cause intermittent mechanical dysphagia, meaning patients will usually present with transient discomfort and regurgitation while swallowing solids and then liquids, depending on the constriction of the ring.
- Webs --- Usually squamous mucosal protrusion into the esophageal lumen, especially anterior cervical esophagus behind the cricoidCricoidThe cricoid cartilage, or simply cricoid , is the only complete ring of cartilage around the trachea.-Location:...
area. Patients are usually asymptomatic or have intermittent dysphagia. An important association of esophageal webs is to the Plummer-Vinson syndromePlummer-Vinson syndromePlummer–Vinson syndrome , also called Paterson–Brown–Kelly syndrome or sideropenic dysphagia presents as a triad of dysphagia , glossitis, and iron deficiency anemia...
in iron deficiencyIron deficiency (medicine)Iron deficiency is one of the most common of the nutritional deficiencies. Iron is present in all cells in the human body, and has several vital functions...
, in which case patients will also have anemia, koilonychia, fatigue, and other symptoms of anemiaAnemiaAnemia is a decrease in number of red blood cells or less than the normal quantity of hemoglobin in the blood. However, it can include decreased oxygen-binding ability of each hemoglobin molecule due to deformity or lack in numerical development as in some other types of hemoglobin...
.
Achalasia
Achalasia
Achalasia , also known as esophageal achalasia, achalasia cardiae, cardiospasm, and esophageal aperistalsis, is an esophageal motility disorder involving the smooth muscle layer of the esophagus and the lower esophageal sphincter...
is an idiopathic motility disorder characterized by failure of lower esophageal sphincter (LES) relaxation as well as loss of peristalsis
Peristalsis
Peristalsis is a radially symmetrical contraction and relaxation of muscles which propagates in a wave down the muscular tube, in an anterograde fashion. In humans, peristalsis is found in the contraction of smooth muscles to propel contents through the digestive tract. Earthworms use a similar...
in the distal esophagus, which is mostly smooth muscle. Both of these features impair the ability of the esophagus to empty contents into the stomach. Patients usually complain of dysphagia to both solids and liquids. Dysphagia to liquids, in particular, is a characteristic of achalasia. Other symptoms of achalasia include regurgitation, night coughing, chest pain, weight loss, and heartburn. The combination of achalasia, adrenal insufficiency, and alacrima (lack of tear production) in children is known as the triple A (Allgrove) syndrome
Triple A syndrome
Triple-A syndrome , also known as Achalasia-Addisonianism-Alacrimia syndrome or Allgrove Syndrome, is a rare autosomal recessive congenital disorder. In most cases, there is no family history of it. The syndrome was discovered by Jeremy Allgrove and colleagues in 1978. Triple A stands for...
. In most cases the cause is unknown (idiopathic), but in some regions of the world, achalasia can also be caused by Chagas disease
Chagas disease
Chagas disease is a tropical parasitic disease caused by the flagellate protozoan Trypanosoma cruzi. T. cruzi is commonly transmitted to humans and other mammals by an insect vector, the blood-sucking insects of the subfamily Triatominae most commonly species belonging to the Triatoma, Rhodnius,...
due to infection by Trypanosoma cruzi
Trypanosoma cruzi
Trypanosoma cruzi is a species of parasitic euglenoid trypanosomes. This species causes the trypanosomiasis diseases in humans and animals in America...
.
Scleroderma
Scleroderma
Systemic sclerosis or systemic scleroderma is a systemic autoimmune disease or systemic connective tissue disease that is a subtype of scleroderma.-Skin symptoms:...
is a disease characterized by atrophy
Atrophy
Atrophy is the partial or complete wasting away of a part of the body. Causes of atrophy include mutations , poor nourishment, poor circulation, loss of hormonal support, loss of nerve supply to the target organ, disuse or lack of exercise or disease intrinsic to the tissue itself...
and sclerosis
Sclerosis (medicine)
In medicine, sclerosis refers to the stiffening of a structure, usually caused by a replacement of the normal organ-specific tissue with connective tissue.Types include:...
of the gut wall, most commonly of the distal esophagus (~90%). Consequently, the lower esophageal sphincter cannot close and this can lead to severe gastroesophageal reflux disease (GERD). Patients typically present with progressive dysphagia to both solids and liquids secondary to motility problems or peptic stricture from acid reflux.
Spastic motility disorders include diffuse esophageal spasm
Diffuse esophageal spasm
Diffuse esophageal spasm is a condition in which uncoordinated contractions of the esophagus occur. It is thought to result from motility disorders of the esophagus. These spasms do not propel food effectively to the stomach...
(DES), nutcracker esophagus
Nutcracker esophagus
Nutcracker esophagus is a disorder of the movement of the esophagus, and is one of many motility disorders of the esophagus, including achalasia and diffuse esophageal spasm. It causes difficulty swallowing, or dysphagia, to both solid and liquid foods, and can cause chest pain; it may also be...
, hypertensive lower esophageal sphincter, and nonspecific spastic esophageal motility disorders (NEMD).
- DES can be caused by many factors that affect muscular or neural functions, including acid reflux, stress, hot or cold food, or carbonated drinks. Patients present with intermittent dysphagia, chest pain, or heartburn.
Rare causes of esophageal dysphagia not mentioned above
- DiverticulumDiverticulumA diverticulum is medical or biological term for an outpouching of a hollow structure in the body. Depending upon which layers of the structure are involved, they are described as being either true or false....
- Aberrant subclavian arteryAberrant subclavian arteryAberrant subclavian artery, or aberrant subclavian artery syndrome refers to a rare anatomical variant of the origin of the right or left subclavian artery...
, or (dysphagia lusoriaOrtner's syndromeOrtner's syndrome is a rare cardiovocal syndrome and refers to recurrent laryngeal nerve palsy from cardiovascular disease. It was first described by N...
) - Cervical osteophytes
- Enlarged aorta
- Enlarged left atrium
- Mediastinal tumor
Diagnostic tools
Once a patient complains of dysphagia they should have an upper endoscopyEsophagogastroduodenoscopy
For other expansions of the initialism "OGD", see the disambiguation page.In medicine , esophagogastroduodenoscopy is a diagnostic endoscopic procedure that visualizes the upper part of the gastrointestinal tract up to the duodenum...
. Commonly patients are found to have esophagitis and may have an esophageal stricture. Biopsies are usually done to look for evidence of esophagitis even if the EGD is normal. Usually no further testing is required if the diagnosis is established on EGD. Repeat endoscopy may be needed for follow up.
If there is a suspicion of a proximal lesion such as:
- history of surgery for laryngeal or esophageal cancer
- history of radiation or irritating injury
- achalasia
- Zenker's diverticulum
a barium swallow
Barium swallow
A barium swallow is a medical imaging procedure used to examine the upper GI tract, which includes the esophagus and, to a lesser extent, the stomach.-Principle:...
may be performed before endoscopy to help identify abnormalities that might increase the risk of prevent at the time of endoscopy.
If achalasia suspected an upper endoscopy is required to exclude a malignancy as a cause of the findings on barium swallow. Manometry
Esophageal motility study
An esophageal motility study or esophageal manometry is a test to assess motor function of the Upper Esophageal Sphincter , Esophageal body and Lower Esophageal Sphincter .-Indications:...
is performed next to confirm. A normal endoscopy should be followed by manometry, and if manometry is also normal, the diagnosis is functional dysphagia.
Treatment
The patient is generally sent for a GI, pulmonary, or ENT, depending on the suspected underlying cause. A consultation with a speech therapist may also be needed, as many patients may need dietary modifications such as thickened fluidsThickened fluids
Thickened fluids are often used for people with dysphagia, a disorder of swallowing function. The thicker consistency makes it less likely that an individual with dysphagia will aspirate while they are drinking...
.