Background: 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. Hypothesis: (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. Design: University hospital tertiary care center. Setting: 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 HTNLES. Two hundred eight patients (52%) underwent a myotomy by either a thoracoscopic or a laparoscopic approach. Results: 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. Conclusions: 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 HTNLES; and (5) a predictably good treatment for NE is still elusive, and the results of surgery were disappointing.
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