Published on 22/03/2015 by admin
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Last modified 22/03/2015
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Chapter 313 Dysmotility
Seema Khan, Susan R. Orenstein
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. These entities are usually detected on videofluoroscopic evaluation of swallowing (sometimes accompanied by visible cricopharyngeal prominence, termed a bar), but often the most precise definition of the dysfunction is obtained with manometry. A self-limited form of cricopharyngeal incoordination occurs in infancy and remits spontaneously in the 1st year of life if nutrition is maintained despite the dysphagia. In older children, idiopathic cricopharyngeal spasm is usually treated by myotomy of the UES. It is important, however, to evaluate such children thoroughly, including cranial MRI to detect Arnold Chiari malformations, which can manifest in this way but are best treated by cranial decompression, rather than esophageal surgery. 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. All of these can produce dysphagia. Medications (nitrazepam, benzodiazepines) and tracheostomy can adversely affect the function of the UES and thereby produce dysphagia.
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