The named primary esophageal motility disorders (PEMDs) are achalasia, diffuse esophageal spasm (DES), nutcracker esophagus (NE), and hypertensive lower esophageal sphincter (HTN-LES). Although the diagnosis and treatment of achalasia are well defined, such is not the case with the other disorders.
(1) Symptoms do not reliably distinguish PEMDs from gastroesophageal reflux disease; (2) esophageal function tests are essential to this distinction and to identifying the type of PEMD; (3) minimally invasive surgery is effective for each condition; and (4) the laparoscopic approach is better than the thoracoscopic approach.
University hospital tertiary care center.
Retrospective review of a prospectively collected database.
Patients and Methods
A diagnosis of PEMD was established in 397 patients by esophagogram, endoscopy, manometry, and pH monitoring. There were 305 patients (77%) with achalasia, 49 patients (12%) with DES, 41 patients (10%) with NE, and 2 patients (1%) with HTN-LES. Two hundred eight patients (52%) underwent a myotomy by either a thoracoscopic or a laparoscopic approach.
Ninety-nine patients (25%) had a diagnosis of gastroesophageal reflux disease at the time of referral and had been treated with acid-suppressing medications. In achalasia and DES, a thoracoscopic or laparoscopic myotomy relieved dysphagia and chest pain in more than 80% of the patients. In contrast, in NE the results were less predictable, and the operation most often failed to relieve symptoms.
These results show that (1) symptoms were unreliable in distinguishing gastroesophageal reflux disease from PEMDs; (2) esophageal function tests were essential to diagnose PEMD and to define its type; (3) the laparoscopic approach was better than the thoracoscopic approach; (4) a laparoscopic Heller myotomy is the treatment of choice for achalasia, DES, and HTN-LES; and (5) a predictably good treatment for NE is still elusive, and the results of surgery were disappointing.