Esophageal motility disorders (distal esophageal spasm, nutcracker esophagus, and hypertensive lower esophageal sphincter): modern management.

The group of hypercontractile esophageal motility disorders includes distal esophageal spasm (DES), nutcracker esophagus (NE), and hypertensive lower esophageal sphincter (LES). The clinical relevance of these abnormalities identified during esophageal manometry is debated, and their management can be challenging. Hypercontractile esophageal motility abnormalities are defined through specific manometric criteria. Current pathophysiologic concepts for these abnormalities include defects in the nitronergic neural pathways and imbalances between the cholinergic and nitronergic pathway. Proposed treatments for NE, DES, and hypertensive LES include proton-pump inhibitors, nitrates, calcium channel blockers, phosphodiasterase inhibitors, and tricyclic antidepressants or serotonin reuptake inhibitors. Small case series reported benefits after botulinum toxin injections, dilatations, and myotomies. The optional management of esophageal spasm, NE, and hypertensive LES is still debated. Treatment recommendations are based on controlled studies with small numbers of patients or on case series. Medical treatment, including acid suppression, smooth muscle relaxants, and visceral analgesics, should be tried first. In nonresponding patients, botulinum toxin injections or balloon dilatations can be tried. Pneumatic dilatations or myotomies should be regarded as last-option treatments for nonresponding patients.