Faculty Bibliography

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

Controversy exists as to whether the hypertensive lower esophageal sphincter (HLES) represents a clinical motility disorder of the esophagus or is merely the right-sided expression of a normal distribution curve. In the present study we describe 16 patients with HLES, defined as a lower esophageal sphincter (LES) pressure of greater than or equal to 40 mm Hg (mean + 3 SD of controls) with normal peristalsis. All of the patients suffered from chest pain and nine from dysphagia. Delayed bolus transit at the gastroesophageal junction was demonstrated in four patients by radiography. Manometric studies showed that during swallowing the LES residual pressures were significantly greater (9.2 +/- 5.0 mm Hg) than observed in normal controls (1.8 +/- 2.2 mmHg) (mean +/- 1 SD). However, the percent LES relaxation in patients did not differ significantly from controls. Clinical improvement was associated with pharmacological or mechanical reduction of resting LES pressure with an accompanying fall in the nadir pressure. These observations suggest that HLES may have clinical and pathophysiological significance and that evidence for the entity should be sought during manometric studies in the clinical laboratory

High amplitude peristaltic contractions in the distal esophagus ('nutcracker esophagus') is the most common manometric disorder seen in patients with noncardiac chest pain. Although this abnormality is found in the distal esophagus, the definition regarding its precise level in the esophagus is unclear. A careful analysis of 99 consecutive manometric tracings performed during a 1-yr period revealed that in patients with noncardiac chest pain and/or dysphagia, the location of the abnormal esophageal contractions varied: 1) in 11 patients the esophageal contractions were abnormal at 2 cm, as well as 7 cm, above the lower esophageal sphincter (LES); 2) the abnormality was limited to the 2-cm location above the LES in six patients; and 3) was confined to the 7-cm location above the LES in five patients. If the conventional criteria of averaging the distal esophageal contraction amplitudes at 2 and 7 cm above the LES were adopted, six of the 11 patients with segmental esophageal contraction abnormality would not have been identified. We suggest that, by inspection of each location of the distal esophagus separately, localized high amplitude contractions can be identified, and the distal 2 cm segment of the esophagus should be routinely included in the manometric evaluation

An impression that achalasia remains an elusive diagnosis led us to review our recent experience. From August 1, 1985 to March 31, 1987, we saw 25 patients with 'previously untreated' achalasia for consultation and/or treatment. Data was extracted from review of their records. Achalasia was the initial diagnosis in only 12 patients. The others were given diagnoses of gastroesophageal reflux (4), presbyesophagus (2), esophageal spasm (2), psychiatric disorders (2), and combination of various disorders (3). In the latter patients, various diagnostic studies were either inappropriately delayed or misinterpreted, so that incorrect diagnoses were given. Errors in diagnosis led to further inappropriate testing and therapies. We conclude that: (a) achalasia remains an elusive diagnosis in current practice, (b) errors in diagnosis are related to delay in obtaining appropriate studies or misinterpretation of such studies, and (c) this delay leads to persistent symptoms and ineffective and/or inappropriate therapies

Perforation of the esophagus is a well-described complication of pneumatic dilatation in patients with achalasia. Although successful management of these patients without surgical intervention has been reported, little follow-up data exist. We report the successful nonsurgical management of esophageal perforation after pneumatic dilatation in three patients. Manometric and radionuclide esophageal emptying studies in these patients showed satisfactory results after the dilatations despite the occurrence of perforation, and the excellent symptomatic response has been maintained during a follow-up period ranging from one to four years

Squamous cell carcinoma and adenocarcinoma constitute the majority of malignancies of the esophagus. Although lymphoma may involve any part of the gastrointestinal tract either primarily or secondarily, esophageal involvement is rare. We describe two cases of primary esophageal non-Hodgkin's lymphoma and review the literature, with particular attention to roentgenographic studies, esophagoscopic findings, and endoscopic biopsy results

Although some patients with chest pain and dysphagia have manometric evidence of classic esophageal motor disorders, other patients with these symptoms may have only nonspecific findings of unknown importance. We describe five patients with chest pain and dysphagia in whom esophageal manometry showed a segment of esophagus with an increased frequency of simultaneous contractions associated with normal motility in the more proximal and distal esophagus. All patients had corresponding segmental abnormalities on video-esophagograms augmented with a solid bolus; in four patients, the solid bolus caused reproduction of symptoms during the esophagography. We conclude that 'segmental aperistalsis' may cause chest pain and dysphagia, and that the diagnosis may be made by careful manometric analysis of the entire esophagus, complemented by esophagography with a solid bolus

The aim of this study was to examine systematically the manometric characteristics of symptomatic patients with high-amplitude peristaltic esophageal contractions, or the nutcracker esophagus (n = 20), in comparison to normal subjects (n = 30). In both normals and patients, amplitude and duration of contractions were more at 5 cm than at 10 cm above the lower esophageal sphincter. The patients differed significantly from normals not only in amplitude at 5 cm, but also at 10 cm and in duration at both sites. Bipeaked waves were seen more frequently in patients than in normals at either 5 or 10 cm above the sphincter. Two patients, but none of the normal subjects, had triple-peaked waves. Lower esophageal sphincter pressure was significantly elevated in patients as compared to normals. Although percent relaxation of the sphincter was the same in patients and normals, the postrelaxation residual, or nadir, sphincter pressure was higher in patients. We conclude that patients with high-amplitude peristaltic contractions may also have abnormalities in duration of contractions, percent bipeaked waves, triple-peaked waves, or in parameters of the lower esophageal sphincter

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

A patient with dysphagia and chest pain was shown by manometry to have high-amplitude peristaltic esophageal contractions (nutcracker esophagus). Worsening symptoms over the next two years led to the performance of repeated manometric studies, which showed diffuse esophageal spasm. This demonstration of a transition from nutcracker esophagus to diffuse esophageal spasm lends further support for consideration of the nutcracker esophagus as a manometric disorder associated with chest pain or dysphagia. Furthermore, it suggests a pathophysiologic relationship between the nutcracker esophagus, a disorder with preserved peristalsis, and diffuse esophageal spasm, the classic dysmotility considered to be of neurogenic origin