Motility And Dysmotility Disorders Of Esophagus Flashcards
The most common external masses that compress the esophagus and produce obstructive symptoms
Enlarged mediastinal or subcarinal lymph nodes, caused by infection (tuberculosis, histoplasmosis) or neoplasm (lymphoma)
Vascular anomalies can also compress the esophagus ?
Dysphagia lusoria is a term denoting the dysphagia produced by a developmental vascular anomaly,
which is often an aberrant right subclavian artery or right-sided or double aortic arch
Schatzki ring
The lower esophagus is the most common location for peptic strictures, which are generally somewhat ragged and several cm long.
Thin membranous rings, including the Schatzki ring at the squamocolumnar junction, can also occlude this area
Eosinophilic esophagitis ?
Eosinophilic esophagitis is one of the most common causes for esophageal obstructive symptoms.
Although the pathogenesis of obstructive eosinophilic esophagitis is not yet completely explained and seems to vary among individual patients, endoscopy or radiology demonstrates stricture formation in some children with eosinophilic esophagitis, and in others a noncompliant esophagus is evident, with thickened wall layers demonstrable by ultrasonography
Cricopharyngeal achalasia v/s cricopharyngeal incoordination
Cricopharyngeal achalasia signifies a failure of complete relaxation of the upper esophageal sphincter (UES)
Whereas cricopharyngeal incoordination implies full relaxation of the UES but incoordination of the relaxation with the pharyngeal contraction
In children, treatment options for non-self-limited cricopharyngeal achalasia ?
Consist of dilation,
Botox injection, and
transcervical myotomy
Zenker diverticulum
Cricopharyngeal spasm may be severe enough to produce posterior pharyngeal (Zenker) diverticulum above the obstructive sphincter;
this entity occurs rarely in children.
Systemic causes of swallowing dysfunction that can affect the oropharynx, UES, and upper esophagus include
cerebral palsy, Arnold-Chiari malformations, syringomyelia, bulbar palsy or cranial nerve defects (Möbius syndrome, transient infantile paralysis of the superior laryngeal nerve), transient pharyngeal muscle dysfunction, spinal muscular atrophy (including Werdnig-Hoffmann disease), muscular dystrophy, multiple sclerosis, infections (botulism, tetanus, poliomyelitis, diphtheria), inflammatory and autoimmune diseases (dermatomyositis, myasthenia gravis, polyneuritis, scleroderma), and familial dysautonomia
Achalasia ?
Achalasia is a primary esophageal motor disorder of unknown etiology characterized by loss of LES relaxation and loss of esophageal peristalsis, both contributing to a functional obstruction of the distal esophagus.
Possible etiology for achalasia…..
Degenerative,
autoimmune (antibodies to Auerbach plexus), and
infectious (Chagas disease caused by Trypanosoma cruzi)
Triple A syndrome or Allgrove syndrome
In rare cases, achalasia is familial or part of the
achalasia,
alacrima, and
adrenal insufficiency
Pseudoachalasia refers
Achalasia caused by various forms of cancer via obstruction of the gastroesophageal junction, infiltration of the submucosa and muscularis of the LES, or as part of the paraneoplastic syndrome with formation of anti-Hu antibodies
Pathology of achalasia
Inflammation surrounds ganglion cells, which are decreased in number.
There is selective loss of postganglionic inhibitory neurons that normally lead to sphincter relaxation, leaving postganglionic cholinergic neurons unopposed.
This imbalance produces high basal LES pressures and insufficient LES relaxation.
The loss of esophageal peristalsis can be a secondary phenomenon.
Manifestations of achalasia……
Regurgitation and dysphagia for solids and liquids and
may be accompanied by undernutrition or
chronic cough;
retained esophageal food can produce esophagitis.
Most common D/D of achalasia
The presentations of chronic regurgitation/vomiting with weight loss, and chronic cough have led to misdiagnoses of anorexia nervosa and asthma, respectively.