Muscle pathology in idiopathic cricopharyngeal dysphagia. Enzyme histochemical and electron microscopic findings.

The structural changes in the cricopharyngeal muscle (CM) were examined ultrastructurally and by enzyme histochemistry in five patients suffering from idiopathic cricopharyngeal dysphagia (ICD). Diagnosis was established by fiberoptic esophagoscopy, esophageal manometry and cineradiography. Cricopharyngeal myotomy was performed with marked improvement in all patients. Intraoperatively, a biopsy was taken from the CM. Additionally, all patients underwent neurological examination for possible generalized muscle disease, and a biopsy was taken from a limb muscle. CM from nine cadavers without known history of dysphagia served as control. The control samples disclosed structural changes which were considered to be pathological in other skeletal muscles, and required that the criteria for CM pathology we modified accordingly. In three patients changes in CM histology suggested specific pathogenesis: one patient had evidence for a generalized myositis but was only symptomatic for dysphagia. Another patient had muscle fiber atrophy and slight inflammation in her CM, possibly due to alcohol abuse. The third patient had loss of CM fibers with replacement by connective tissue enough to cause functional disturbances. In two patients no cause for dysphagia was found in either immunohistochemistry or electron microscopic studies. These results demonstrate the special structural features of the CM and indicate that ICD can have multiple etiologies.