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
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.
"Esophageal motility disorders appear in various forms, such as achalasia, diffuse esophageal spasm, and nutcracker esophagus. Unfortunately, only achalasia has a known cause and pathophysiology.2 Sifrim et al.10 reported that the spectrum of primary esophageal motility disorders is an expression of a progressively failing deglutitive inhibition based on an inverse relationship between the degree of inhibition and the propagation velocity of the deglutitive contraction among achalasia, diffuse esophageal spasm, and intermediate forms. "
[Show abstract][Hide abstract] ABSTRACT: The aim of this study was to assess changes between primary classification of esophageal motility disease and follow-up classification by high resolution manometry (HRM) and to determine whether previously classified diseases could be recategorized according to the updated Chicago Classification published in 2011. We reviewed individual medical records and HRM findings twice for each of 13 subjects. We analyzed primary and follow-up HRM findings based on the original Chicago Classification. We then reclassified the same HRM findings according to the updated Chicago Classification. This case series revealed the variable course of esophageal motility disorders; some patients experienced improvement, whereas others experienced worsening symptoms. Four cases were reclassified from variant achalasia to peristaltic abnormality, one case from diffuse esophageal spasm to type II achalasia and one case from peristaltic abnormality to variant achalasia. Four unclassified findings were recategorized as variant achalasia. In conclusion, esophageal motility disorders are variable and may not be best conceptualized as an independent group. Original classifications can be recategorized according to the updated Chicago Classification system. More research is needed on this topic.
Gut and liver 05/2013; 7(3):377-381. DOI:10.5009/gnl.2013.7.3.377 · 1.81 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: . Diffuse esophageal spasm (DES) is a mo-tility disorder of undetermined etiology, typically pre-senting with chest pain, dysphagia or both. The aim of this paper is to provide a critical review of the prevalen-ce, pathogenesis, diagnosis and therapy of DES. Data from referral centers indicates that this is a rare disor-der with a prevalence of 4-7%. The diagnosis is based on the combination of typical symptoms, radiological findings and manometry (simultaneous contractions (SC) in the distal esophagus in ≥ 20% of wet swallows mixed with normal peristalsis). The pathogenesis remains po-orly understood. Recent evidence suggests that nitric oxide deficiency may explain the SC that characterizes this condition at manometry. Gastroesophageal reflux (GER) can coexist in DES and GER has also been im-plied in the pathogenesis of DES. Whether patients with DES and GER represent a subtype of DES with a diffe-rent prognosis or outcome is unknown. We present a cri-tical appraisal regarding different therapeutic appro-aches available for DES and conclude suggesting a management algorithm based on current available lite-rature. Key words: Esophageal motility disorders, esophageal spasm, non cardiac chest pain, dysphagia. DEFINICIÓN El espasmo esofágico difuso (EED) es un trastorno de la motilidad esofágica de causa no establecida, el cual clí-nicamente se manifiesta con episodios de dolor torácico recurrente que semejan a la angina coronaria y/o disfa-gia a sólidos y líquidos. RESUMEN. El espasmo esofágico difuso (EED) es un trastorno de la motilidad esofágica de causa desconoci-da. Típicamente se manifiesta con episodios de dolor torácico recurrente y/o disfagia a sólidos y líquidos. Este trabajo sintetiza la evidencia científica disponible en relación a la prevalencia, patogenia, diagnóstico y tra-tamiento del EED. Su prevalencia varía de un 4-7% en centros de referencia. El diagnóstico se basa en la com-binación de la información clínica, el esofagograma y la manometría esofágica (contracciones simultáneas en ≥ 20% de degluciones líquidas en el esófago distal alternando con peristalsis normal). La fisiopatología permanece incierta, pero un defecto del óxido nítrico podría explicar las contracciones simultáneas anor-males. El reflujo gastroesofágico (RGE) también ha sido relacionado con el EED, sugiriéndose que los pacientes que presentan RGE podrían constituir un grupo con un comportamiento diferente. Presentamos una revisión crítica de la literatura existente en rela-ción a las modalidades terapéuticas existentes y su-gerimos un algoritmo de manejo clínico basado en la evidencia publicada a la actualidad. Palabras clave: Trastornos de la motilidad, espasmo eso-fágico, dolor torácico no cardiaco, disfagia.
[Show abstract][Hide abstract] ABSTRACT: Primary esophageal motility disorders comprise various abnormal manometric patterns which usually present with dysphagia or chest pain. Some, such as achalasia, are diseases with a well defined pathology, characteristic manometric features, and good response to treatments directed towards the palliation of symptoms. Other disorders, such as diffuse esophageal spasm and nutcracker esophagus, have no well defined pathology and could represent a range of motility abnormalities associated with subtle neuropathic changes, gastresophageal reflux and anxiety states. On the other hand, hypocontracting esophagus is generally caused by weak musculature commonly associated with gastresophageal reflux disease. Although manometric patterns have been defined for these disorders, the relation with symptoms is poorly defined and in some cases the response to medical or surgical therapy unpredictable. The aim of this paper is to present a wide spectrum of the primary esophageal motility disorders, as well as to give a concise review for the clinicians encountering these specific diseases.
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