Cricopharyngeal achalasia


Cricopharyngeal achalasia

Description, Causes and Risk Factors:

Functional obstruction at the level of the upper esophageal sphincter due to failure of relaxation of the cricopharyngeal muscles; often associated with a pharyngoesophageal diverticulum.

Cricopharyngeal achalasia is a disorder characterized by an abnormal pattern of deglutitive inhibition of tone in the cricopharyngeus muscle. The condition is distinct from abnormalities of the normal relaxation response to the esophageal air distention and elevated resting tone. Diverse etiologies may cause the disorder, and a sequence of tests is usually necessary to confirm the diagnosis and exclude other conditions that might cause similar symptoms. Guidance on treatment options is limited due to the lack of controlled trials for the disorder as well as concern about accuracy of the diagnosis.

Asherson was the first to consider the possibility that defective opening of the upper esophageal sphincter (UES) might cause swallowing problems. He describes in his study several patients with a characteristic profile radiograph of the pharynx: a hold-up of the barium bolus above the cricopharyngeus, swallowed boli which piled up in the hypopharynx forming a reservoir which acts as a «drip feed» to the esophagus. Asherson introduced the term "Cricopharyngeal achalasia" to describe this functional disturbance. He suggested that there was partial or complete failure, as well as a delay in the relaxation of the cricopharyngeal sphincter in these patients. However, Asherson gave no clear radiological description of the cricopharyngeus muscle itself.

Cricopharyngeal achalasia

Unfortunately, the literature on cricopharyngeal achalasia has been made confusing by the lack of uniform definition of the condition. In addition, the presence of cricopharyngeal achalasia has been inferred from diagnostic testing modalities without an appreciation of alternative disorders that could produce findings that have been attributed to achalasia. Ideally, detection of abnormalities in the deglutitive inhibition would be accomplished by direct recordings from the motor neurons supplying the cricopharyngeus muscle or from the muscle itself. Such techniques either are not available clinically or the availability is confined to a few tertiary centers.

In an attempt to develop some consistency within the field, the following definitions are proposed:

A condition in which during deglutition, the cricopharyngeus muscle or the motor neurons supplying the exhibit one or more of the following abnormal patterns of activity:

    Incomplete inhibition of activation.

  • Abnormally short duration of complete activation inhibition.

  • Abnormal timing of inhibited activation, relative to the activation of the other motor neurons controlled by the swallowing central pattern generator.

  • Increased activation during the normal interval of inhibition.

Symptoms:

It is very difficult or impossible for a patient with cricopharyngeal achalasia to swallow food. Food may fall from the mouth. Upon drinking water, the patient may cough as liquid enters the trachea, then the lungs.

Diagnosis:

The diagnosis of cricopharyngeal achalasia canoften be made by a carefully taken history andby physical examination. Endoscopy and radiographic examination of the pharynx, larynx, tracheaand esophagus are essential, however, since bythese examinations the presence or absence of apharyngeal diverticulum can be determined, andother causes of dysphagia, such as carcinoma of theproximal esophagus, can be excluded. In patientswith cricopharyngeal achalasia, the characteristicradiographic features in the pharynx are:

    The presence of a posterior shelf-like, semicircular filling defect caused by hypertrophy of thecricopharyngeus muscle.

  • Pooling of barium in the vallecular folds whichgives rise to a "cross-bow" appearance in anteroposterior views of the pharynx.

  • Distension of the hypopharynx during swallowing and delay in the passage of barium into theesophagus.

  • Folding and redundancy of the pharyngealmucous membrane, which in some cases simulatesa web in appearance.

  • Endoscopic examination often reveals redundantfolds of atrophic mucous membrane, or even leukoplakia, and in addition the cricopharyngeus musclemay present as a posterior transverse rather unyielding bar to the passage of the esophagoscopeinto the proximal esophagus.

Treatment:

Treatment consists of cricopharyngeal myotomy or cricopharyngeal and thyropharyngeal myectomy, which usually results in normal swallowing immediately after surgery. The success rate of surgery approaches 65%. People with acquired neuromuscular disorders are less likely to respond to surgery, but may respond to treatment of the underlying disease. Aspiration pneumonia should be treated aggressively if present.

The postoperative management should present no difficulties, but in cases in which a diverticulum has been excised, or the pharynx has been opened, a small drain should be left down to the line of closure of the diverticulum for two or three days.

NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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