Transition from diffuse esophageal spasm to achalasia.

A 19-year-old man first presented with clinical, radiological, and manometric features of diffuse spasm. Within a year his motility disorder progressed to "vigorous" and, finally, classic achalasia. After pneumatic dilatation and subsequent long myotomy, the features of classic achalasia disappeared and he again exhibited radiological and manometric evidence of "vigorous" achalasia. The evolution of this patient's disease provides evidence that diffuse spasm and achalasia are different stages of the same disease and lie at opposite ends of a spectrum of related esophageal motility disorders.     

Lower esophageal sphincter dysfunction in diffuse esophageal spasm

Although lower esophageal sphincter (LES) dysfunction has been reported in patients with diffuse esophageal spasm, recent changes in manometric criteria for spasm and for LES relaxation suggested a need for reassessment. Moreover, LES relaxation in reflux-associated spasm has not been reported previously. On clinical criteria and independent of manometric findings, 22 patients with spasm were assigned to either idiopathic (I-DES, N = 9) or reflux-associated spasm (R-DES, N = 13) groups. Patients who underwent manometry for chest pain (C-NL, N = 10) or reflux (R-NL, N = 10) and had normal peristalsis served as control groups. Percent LES relaxation was significantly reduced in both spasm groups, and R-DES had significantly lower percent relaxation than I-DES. Post-deglutitive nadir sphincter pressure was significantly greater in R-DES than in I-DES. Duration of relaxation was normal in I-DES, but was significantly decreased in R-DES. This study indicates that 1) LES relaxation may be impaired in I-DES patients meeting current criteria for spasm, 2) the impairment in I-DES is primarily in "amplitude" of relaxation, i.e., percent relaxation and nadir pressure, but not duration, 3) LES relaxation may also be impaired in R-DES, and 4) the impairment in R-DES is to a greater degree than in I-DES patients and may be seen in both "amplitude" and duration of relaxation. This study shows that there is a spectrum of sphincter dysfunction in patients with esophageal spasm. It also suggests that there may be separate mechanisms for LES relaxation in R-DES patients, one with impaired relaxation and the other with near complete relaxation, "transient" or otherwise, to allow for reflux.     

Manometric diagnosis of diffuse esophageal spasm

There are no requirements concerning the amplitude of simultaneous contractions among the present criteria for the manometric diagnosis of diffuse esophageal spasm. The purpose of this investigation was to determine whether the current criteria effectively identify an appropriately homogeneous patient population. Sixty consecutive motility tracings that met the criteria for diffuse esophageal spasm were evaluated. A bimodal distribution of the highest simultaneous esophageal contraction for each patient was observed. One group's (N=29) highest simultaneous esophageal contractile amplitude was 74 mm Hg, the other's (N=31) highest simultaneous esophageal contractile amplitude was 100 mm Hg. Group 1 had significantly decreased lower esophageal sphincter pressure, lower peristaltic amplitude, more aperistalsis, fewer simultaneous contractions, and fewer complaints of chest pain. These comparisons suggest that consideration be given to the amplitude of simultaneous esophageal contractions in the manometric diagnosis of diffuse esophageal spasm.     

Surgical myotomy in patients with high-amplitude peristaltic esophageal contractions

High-amplitude peristaltic esophageal contractions, or the nutcracker esophagus, may be associated with chest pain or dysphagia. Medical treatment for this disorder is sometimes not satisfactory. We report the manometric and clinical effects of myotomy in four patients with high-amplitude peristaltic contractions who underwent surgery because of the severity of their symptoms and recalcitrance to various medical treatments. Manometry 1-5 years after surgery showed a reduction in amplitude, duration, and percent bipeaked waves at 5 and 10 cm above the lower esophageal sphincter. Peristalsis was abolished or decreased in the distal 10 cm of the esophageal body but was not affected more proximally. Lower esophageal sphincter pressure was decreased in all patients. The manometric changes were least marked in one patient, who was the only one who had some chest pain when last seen five years after myotomy. We conclude that in severely symptomatic patients with high-amplitude peristaltic contractions, myotomy results in marked manometric changes and marked clinical improvement. Patients with this disorder and whose chest pain is recalcitrant to extensive medical therapy may be successfully treated by surgical myotomy.     

Sororal occurrence of diffuse esophageal spasm

Summary In this report, we have described two sisters with classical symptoms, radiological appearances, and manometric features of diffuse esophageal spasm. To the best of our knowledge, familial occurrence of this disorder has not been documented previously.     

Biliary dyskinesia with associated high amplitude esophageal peristaltic contractions

A 60-yr-old woman with recurrent biliary-type pain and pancreatitis was found to have abnormal biliary manometrics. Sphincter of Oddi basal and phasic contraction pressures were significantly elevated. Esophageal manometrics revealed high amplitude peristaltic contractions that were unassociated with chest discomfort. High amplitude dysmotility syndromes may perhaps represent a spectrum of disease involving the entire gastrointestinal tract.     

Diffuse esophageal spasm: not diffuse but distal esophageal spasm (DES)

Diffuse esophageal spasm is an uncommon motility disorder that is found in less than 5% of patients undergoing esophageal motility testing for dysphagia. It is defined manometrically by the presence of 20% or more simultaneous contractions in the distal esophageal body with normal peristalsis. This motility abnormality has been traditionally identified as occurring primarily in the smooth muscle portion of the distal esophagus yet, the term diffuse persists in the medical literature to identify DES. The aim of our study was to assess the diffuse or limited nature of this entity by evaluating the prevalence of simultaneous contractions in both proximal and distal esophagus in patients with DES. We reviewed esophageal motility tracings of 53 consecutive patients (32 F, 21 M) with DES and compared them with 53 age-matched patients with manometric normal studies. In the distal esophagus we found 195 simultaneous contractions (37% of swallows) with a median of 3 and range of 2–7 per patient. Of the 53 patients with DES a total of 13 simultaneous contractions (2% of swallows) occurred in the proximal esophagus with only 3 (5.6%) of the 53 patients having 2 or more simultaneous contractions in 10 swallows. None of the patients with normal manometry showed more than one simultaneous contraction in either proximal or distal esophagus. In conclusion, these findings suggest that the term diffuse esophageal spasm is a misnomer and the DES is more appropriately described as distal esophageal spasm.     

弥漫性食管痉挛的诊治现状

弥漫性食管痉挛是以食管的一种不协调收缩运动为动力学特征的原发性食管运动障碍性疾病.其发病原因尚不十分清楚.本文较系统的阐述了弥漫性食管痉挛的发病机制、诊断方法及治疗措施等最新进展,并作出较客观的评析,为弥漫性食管痉挛的诊断及治疗提供了新的思路.     

Botulinum toxin in the treatment of diffuse esophageal spasm

Diffuse esophageal spasm is a primary esophageal motility disorder. The prevalence is 3–10% in patients with dysphagia and treatment options are limited. This review summarizes the treatment of diffuse esophageal spasm, including pharmacotherapy, endoscopic treatment, and surgical treatment with a special focus on botulinum toxin injection. A PubMed search was performed to identify the literature using the search items diffuse esophageal spasm and treatment. Pharmacotherapy with smooth muscle relaxants, proton pump inhibitors, and antidepressants was suggested from small case series and uncontrolled clinical trials. Endoscopic injection of botulinum toxin is a well‐studied treatment option and results in good symptomatic benefit in patients with diffuse esophageal spasm. Surgical treatment was reported in patients with very severe symptoms refractory to pharmacologic treatment. This article summarizes the present knowledge on the treatment of diffuse esophageal spasm with a special emphasis on botulinum toxin injection. Endoscopic injection of botulinum toxin is presently the best studied treatment option but many questions remain unanswered.     

Management of diffuse esophageal spasm with balloon dilatation.

Of 1200 patients referred to the esophageal laboratory at Guy's Hospital for investigation of suspected esophageal motility disorders, 61 (5.1%) were diagnosed as diffuse esophageal spasm. Twenty of these patients whose symptoms were severe did not respond to conservative treatment and were treated by balloon dilatation. Results were good in 14 and poor in six patients, which included one esophageal perforation. Diffuse esophageal spasm was diagnosed where more than 30% nonperistaltic activity was demonstrated by manometry. Lower esophageal sphincter pressure and relaxation were normal in all cases except one. Gastroesophageal reflux was present in four of five poor responders who were examined by 24-h ambulatory pH monitoring, and in only one of 10 good responders. Three of the six patients in whom balloon dilatation was successful proceeded to full-length myotomy, with relief of symptoms in two. The indications for, and results of, balloon dilatation in this condition are discussed, and a new radiological sign is described.     

Progression of diffuse esophageal spasm to achalasia: incidence and predictive factors

The progression of certain primary esophageal motor disorders to achalasia has been documented; however, the true incidence of this decay is still elusive. This study aims to evaluate: (i) the incidence of the progression of diffuse esophageal spasm to achalasia, and (ii) predictive factors to this progression. Thirty‐five patients (mean age 53 years, 80% females) with a manometric picture of diffuse esophageal spasm were followed for at least 1 year. Patients with gastroesophageal reflux disease confirmed by pH monitoring or systemic diseases that may affect esophageal motility were excluded. Esophageal manometry was repeated in all patients. Five (14%) of the patients progressed to achalasia at a mean follow‐up of 2.1 (range 1–4) years. Demographic characteristics were not predictive of transition to achalasia, while dysphagia (P= 0.005) as the main symptom and the wave amplitude of simultaneous waves less than 50 mmHg (P= 0.003) were statistically significant. In conclusion, the transition of diffuse esophageal spasm to achalasia is not frequent at a 2‐year follow‐up. Dysphagia and simultaneous waves with low amplitude are predictive factors for this degeneration.