Ultrastructure of the Esophageal Muscle in Achalasia and Diffuse Esophageal Spasm

Abstract

The pathogenesis of dysphagia in achalasia and diffuse esophageal spasm (DES) is not understood completely, and the primary site of damage in these two entities is not known. This project was undertaken to study the ultrastructural changes present in the muscular wall of the esophagus in eight patients with achalasia and nine patients with DES, compared with seven controls. The ultrastructural alterations in the smooth muscle of all patients were neither pronounced nor consistent. However, nonspecific changes, including filament disarray, mottling of the fiber density in myocytes, thick and long cytoplasmic dense bodies, long dense plaques, and few nexus junctions were seen in both achalasia and DES. In addition, the smooth muscle cells in achalasia exhibited nuclear and cytoplasmic inclusions. Statistical analysis of the number of muscle cells per unit area suggested that the gross thickening of the muscular wall of the esophagus in cases of DES is because of hyperplasia and not hypertrophy of muscle cells. A striking change seen in achalasia esophagi and a few cases of DES, as compared to the controls, was the marked loss of small nerve fibers and the paucity of granules in the remaining fibers. This study points to a neurogenic pathogenesis for dysphagia in achalasia with secondary nonspecific changes in the smooth muscle in these two diseases. No nerve abnormalities were noticed in DES.

Facioscapulohumeral Muscular Dystrophy: A Radiologic and Manometric Study of the Pharynx and Esophagus

“…Furthermore, histologic and immunohistochemical studies indicated that a primary degenerative neuropathic cause usually underlies motility disorders of the esophagus [30][31][32][33][34]. Esophageal smooth muscle histology was reported normal or showed changes consistent with denervation atrophy [30,35]. Although we had no access to such tissue in our patients, such findings are incompatible with a primary muscle disease such as FSHD that is not known to involve the central or peripheral nervous systems.…”

Achalasia: What’s New in Diagnosis and Treatment?

“…The histopathologic changes of end-stage achalasia are well described [33,34,37,[46][47][48][49][50], Specimens have been obtained either sur gically. during esophagomyotomy or esopha gectomy.…”

“…Achalasia patients usually have smooth muscle changes of the muscularis propria, particularly of the inner circular layer, when examined by light or electron microscopy [33,37,48,50], The circular muscle of the distal esophagus and LES is hypertrophic and may contain small leiomyomas [37], Smooth mus cle degenerative changes are related to dener vation [48,50], Mucosal changes of the esoph agus are nonspecific and related to chronic stasis and retention [37], Other histopathologic features of achalasia arc extraesophageal. These include changes in the vagus nerve and the dorsal motor nucleus (DMN) of the vagus [45.…”

Achalasia and thyroid disease

“…The observations that achalasia is associated with HLADQw1 and that affected patients often have circulating antibodies to enteric neurons suggest that achalasia may be an autoimmune disorder [5][6][7] . Nonspecific degeneration of smooth muscle cells and a loss of small nerve fibers have been reported [8][9][10] . Another controversial issue is the role of inflammatory infiltrates in the pathogenesis of the disease.…”