Manometric Features Research Articles

Pancreatic pseudocyst as a cause for secondary achalasia

Secondary achalasia is commonly due to cancer. Benign causes are rare and an association with pancreatitis has never been made. Our patient presented with clinical, manometric, endoscopic, and radiographic features of achalasia that occurred in conjunction with a pancreatic pseudocyst extending into the mediastinum. Documentation of this unique relationship was made on two occasions after episodes of complicated pancreatitis and was confirmed by restoration of normal esophageal function after drainage of the pseudocyst. Onset of dysphagia occurring in association with pancreatitis suggests the presence of a pseudocyst and secondary achalasia.

Esophageal chest pain. Value of high-dose provocative testing with edrophonium chloride in patients with normal esophageal manometries.

The unequivocal diagnosis of esophageal chest pain requires the demonstration of simultaneous manometric changes and chest pain. Numerous provocative agents have been used to enhance the diagnostic value of esophageal manometry. Our aims were to: evaluate consecutively a large group of patients with proven noncardiac chest pain and normal baseline manometric studies, using edrophonium chloride, 10 mg, and determine the value of provocative testing in clinical practice. One hundred twenty patients with normal standard baseline esophageal manometries were studied using blinded testing with edrophonium chloride and followed clinically by questionnaire. A positive response of both chest pain and manometric changes was observed in 34%, a negative response in 49%, and an indeterminate response in 17% of patients. Baseline manometric features, including high-amplitude contractions, did not predict the response to edrophonium chloride. Following edrophonium chloride administration, the change in amplitude, duration, and number of repetitive contractions from baseline was significantly greater in positive responders. Edrophonium decreased the velocity of propagated contractions in positive responders (P less than 0.05), but not in nonresponders. Response to edrophonium chloride could not be predicted by patient age, sex, or clinical symptomatology. Seventy percent of patients in both groups had symptoms indistinguishable from ischemic heart disease. After making a specific diagnosis of esophageal chest pain, patients showed a marked clinical improvement, with a significant decrease in physical limitation, emergency room visits, hospital and CCU admissions, and in further cardiac testing. We conclude that provocative testing with edrophonium chloride will make it possible to definitively implicate the esophagus in over 30% of patients with normal baseline manometric findings and noncardiac chest pain.

Barrett's metaplasia and adenocarcinoma of the esophagus in scleroderma

Gastroesophageal reflux is well documented in scleroderma, but the complications of Barrett's metaplasia and adenocarcinoma are not well described. The records of 75 patients with scleroderma seen over a four-year period at the Hospital of the University of Pennsylvania were retrospectively reviewed to determine the prevalence of Barrett's metaplasia and adenocarcinoma of the esophagus and to identify clinical, manometric, laboratory, or radiographic criteria that might predict the presence of these lesions. Twenty-four of these patients underwent endoscopy. In this group, the prevalence of Barrett's metaplasia was 37 percent (nine patients) and adenocarcinoma was also present in two of these patients. The patients with and without Barrett's metaplasia were similar in age (range, 22 to 64 compared with 28 to 79, respectively), sex (six of nine compared with 12 of 15 female, respectively), frequency of esophageal motility disorders, presence of proximal skin involvement, digital ulceration, and pulmonary involvement as measured by diffusion capacity. Barrett's metaplasia was diagnosed on the basis of double-contrast esophagographic results in only one of eight patients with Barrett's metaplasia so-studied. Patients with Barrett's metaplasia tended to have longer duration of heartburn (90 ± 40 months compared with 11 ± 35 months) and dysphagia (39 ± 22 months compared with 7 ± 3 months). Patients with Barrett's metaplasia also tended to have greater impairment of lower esophageal sphincter pressure either at end-expiration (4.0 ± 2.1 compared with 6.1 ± 1.8 mm Hg) or mid-respiration (13.0 ± 3.0 compared with 16.9 ± 2.5 mm Hg). Using chi-square analysis, however, none of these differences reached statistical significance. Discrimination did occur on the basis of the presence of the CREST (calcinosis, Raynaud's phenomenon, esophageal manifestations of scleroderma, sclerodactyly, and telangiectasis) variant (55 percent compared with 7 percent, p < 0.01), a duration of dysphagia of more than five months (p < 0.03), and mid-respiratory lower esophageal sphincter pressure of less than 10 mm Hg (p < 0.05). It is suggested that: (1) Barrett's metaplasia of the esophagus occurs in one third of patients with scleroderma; (2) clinical, manometric, laboratory, and radiographic features are poor predictors of the presence of Barrett's metaplasia; (3) patients with CREST syndrome, prolonged dysphagia, or a very low lower esophageal sphincter pressure may have an increased risk for the development of metaplasia; (4) patients with scleroderma and Barrett's metaplasia have an increased risk of complications such as stricture or adenocarcinoma. A prospective study of patients with scleroderma and the development of Barrett's metaplasia is indicated.

Manometric characteristics of the distal oesophageal sphincter and patterns of gastro-oesophageal reflux in healthy volunteers and patients.

Three manometric characteristics of the distal oesophageal sphincter (DOS; pressure, length below the respiratory inversion point, i.e., abdominal length, and overall length) were investigated in 10 healthy volunteers and in 66 patients with typical symptoms of gastro-oesophageal reflux (GOR) disease. The aim of the work was to correlate the DOS deficiency with symptoms, endoscopic oesophagitis and with specific patterns of GOR as determined by means of 24-hour oesophageal pH monitoring. The results showed that patients with and without GOR disease cannot be separated solely on the basis of the standard manometric test, even adopting more parameters besides the traditional DOS pressure measurement. A functional defect of the DOS may be hypothesized for those patients with an apparently normal DOS on standard oesophageal manometry.

Correlation of esophageal motility abnormalities with neuropsychiatric status in diabetics

Esophageal motility abnormalities, neuropathy, and psychiatric illness were independently determined in 30 patients with type 1 or type 2 diabetes mellitus to clarify the interrelationship of these findings in diabetics. Fifteen patients (50%) were found to have esophageal contraction abnormalities, a specific cluster of manometric derangements. Diagnoses of depression, dysthymia, or generalized anxiety disorder were made in 87% of those with contraction abnormalities but in only 21% of the patients with normal manometric patterns (p = 0.002). Log-linear analysis confirmed that this association was independent of neuropathy effects (p < 0.001). Several changes in individual manometric parameters related to neuropathy alone were appreciated only when the patients with psychiatric illness were excluded from the analysis. These data indicate that some of the esophageal neuromuscular dysfunction observed in diabetics is independent of neuropathy yet is strongly associated with psychiatric disorder. Such findings help to clarify the discrepant relationship of motility disturbances to neuropathy noted in prior reports. We conclude that consideration should be given to psychiatric illness as well as to neuropathy when interpreting manometric features suggestive of autonomic dysfunction in diabetic patients.

Gastroesophageal sphincter pressure and histological changes in distal esophagus in patients with achalasia of the esophagus.

A prospective study was performed in 17 patients with achalasia of the esophagus determining the manometric characteristics of the gastroesophageal sphincter, correlating it with histological analysis by biopsies taken during surgery at the distal narrowed segment of the esophagus, at the location of the sphincter. The histological findings were compared to 10 control cases. Presence or absence of ganglion cells at the Auerbach's plexuses and appearance of smooth muscle fibers were evaluated. Only one case (6%) had Chagas' disease. The mean sphincter pressure was 41 mm Hg, with incomplete relaxation in all patients. Histological analysis showed a complete disappearance of ganglion cells in 94% of the cases and a decrease in the number of neurons with marked chronic inflammatory cells in one case (6%). In all control cases, the ganglion cells were normal. Smooth muscle fibers were normal on light microscopy. No relationship was found between resting gastroesophageal sphincter pressure, length and relaxation, and histological findings at the distal esophagus. These findings suggest that the denervation in the majority of cases is located in the Auerbach plexus, with complete absence of ganglion cells and, therefore, absence of postganglionic nerve fibers.

Severe Idiopathic Constipation Is Associated With a Distinctive Abnormality of the Colonic Myenteric Plexus

We analyzed the clinical, radiographic, esophageal manometric, and pathologic features of 26 women with severe, idiopathic constipation. Twenty-four patients were between 19 and 39 yr of age. Stool frequency was once every 5-28 days. On barium enema examination, 9 of 24 patients had colons of increased length and 4 of these 9 patients had colons of increased width (greater than 10 cm). Radionuclide solid-meal gastric-emptying studies were normal in 23 patients tested. Esophageal manometry demonstrated high-amplitude waves in 10 of 22 patients and long-duration waves in 3 of these 10 patients. Rectal biopsy specimens showed normal submucosal neurons in all patients and melanosis coli in 6. Twelve patients underwent subtotal colectomies for constipation. Conventional light microscopy using hematoxylin and eosin serial sections showed (a) melanosis coli in 4 patients; (b) normal smooth muscle in 11; (c) thinning of the circular muscle in 1; and (d) no apparent abnormalities of the myenteric plexus in any. In contrast, silver stains of the myenteric plexus showed (a) quantitatively reduced numbers of argyrophilic neurons in 10 patients; (b) morphologically abnormal argyrophilic neurons in 11; (c) decreased numbers of axons in 11; and (d) increased numbers of variably sized nuclei within ganglia in all 12. A coded analysis of the silver stains of colons from 8 patients with constipation and 19 control cases demonstrated that the pathologic abnormalities of severe idiopathic constipation could be differentiated from controls. Thus, severe idiopathic constipation is associated with a pathologically identifiable abnormality of the myenteric plexus. This abnormality appears different from anything previously described in intestinal pseudoobstruction.

Clinical and manometric features of the solitary rectal ulcer syndrome.

We report 33 patients with the solitary rectal ulcer syndrome seen between 1975 and 1982. Twenty-four were women and the median age was 32 years. The principal symptoms were bleeding (89 per cent), chronic constipation (64 per cent), rectal pain (42 per cent), tenesmus (42 per cent), and mucous discharge (45 per cent). Twenty-eight patients gave a history of straining (85 per cent). A full-thickness rectal prolapse was present in six patients, an anterior rectal prolapse was observed in 11, and 12 patients had clinical evidence of perineal descent. Defecation was only possible by digital evaluation in six women, and three male patients were passive homosexuals. Manometric studies were performed on 16 patients; eight patients were unable to tolerate rectal distention with 200 cc of air and impaired rectal sensation was present in two. Rectal distention was associated with bursts of involuntary external sphincter activity in three patients, and the distention reflex was absent in six, despite normal ganglia on rectal biopsy. In only four patients was there electromyographic evidence of contraction of the puborectalis during attempted defecation. Fourteen patients were treated by rectopexy; healing of the ulcer occurred in five of six with a full-thickness prolapse compared with only two of eight without a complete prolapse.

Elevated ANA titers in patients with severely abnormal gastrointestinal motility.

We studied a group of six patients with clinical, radiological, and/or manometric features of severely abnormal gastrointestinal motility. Symptoms suggestive of esophageal, small bowel, or colonic involvement were present from 1 1/2 to 40 years. All patients had elevated antinuclear antibody (ANA) titers. None had clinical or radiographic features suggestive of progressive systemic sclerosis or other connective tissue diseases. Two patients had pathologic examinations of intestinal specimens, and these did not show changes suggestive of progressive systemic sclerosis. We conclude that patients with severe gastrointestinal motility disorders can have elevated ANA titers without features of progressive systemic sclerosis or other connective tissue diseases.

Barrett's Esophagus: Clinical, Endoscopic, Histologic, Manometric, and Electrical Potential Difference Characteristics

The clinical, endoscopic, histologic, manometric, and esophageal potential difference characteristics of 20 patients with columnar epithelia lining the lower esophagus (Barrett's esophagus) are presented. Endoscopically, two distinct types were identified: a circumferential-type and an island-type Barrett's esophagus. Patients with these types exhibited similarities in mean age, duration of symptoms, mean lower esophageal sphincter pressure, and frequency of gross esophagitis. Only patients with the circumferential lesion, however, had esophageal strictures or esophageal ulcers. Manometric testing revealed a range of lower esophageal sphincter pressures from 3 to 33 mmHg and qualitative motor abnormalities (i.e., aperistalsis, repetitive waves, tertiary waves) in 3 patients. Histologically, the frequency of epithelial types was junctional greater than specialized columnar greater than atrophic fundic epithelium. More importantly, dysplasia was identified in 2 patients with the circumferential lesion and in 1 patient with the island lesion. Potential difference measurements demonstrated that a high potential difference (greater than -25 mV) was highly specific (92%), but only moderately sensitive (70%) for detecting Barrett's esophagus. Based on these findings, we conclude (a) that there are at least two endoscopically distinct types of Barrett's esophagus involving the lower esophagus--a circumferential type and an island type, (b) that both types are associated with chronic gastroesophageal reflux, with the island type being accompanied by less severe epithelial injury than the circumferential type, and (c) that the identification of dysplasia in the two types suggests that both are unstable lesions requiring continued surveillance with endoscopy and biopsy.

Jejunal diverticulosis: A heterogenous disorder caused by a variety of abnormalities of smooth muscle or myenteric plexus

We analyzed the clinical, radiographic, esophageal manometric, and pathological features of 10 patients referred with jejunal diverticulosis. Nine patients were over age 59 yr and had symptoms of intestinal pseudoobstruction of 5–43 yr duration. Seven had surgery for mechanical obstruction, although none was found. Eight had diarrhea, steatorrhea, and weight loss. Five had Raynaud's phenomenon and heartburn, and 2 had dysphagia. At radiography, 9 had jejunal diverticula with or without duodenal or ileal diverticula, or both. Two each had abnormal structure or motility of the esophagus or stomach. At manometry, 3 of 7 had a nonspecific motor abnormality, and 1 other had low amplitude peristaltic waves. Light microscopy of small intestinal tissue in 7 patients showed that 4 had fibrosis and decreased numbers of normal-appearing muscle cells, findings consistent with progressive systemic sclerosis. Two others had fibrosis associated with degenerated smooth muscle cells, findings consistent with a visceral myopathy. The seventh patient had neuronal and axonal degeneration and neuronal intranuclear inclusions, findings consistent with a visceral neuropathy. We conclude that (a) intestinal pseudoobstruction is a major clinical manifestation of jejunal diverticulosis, (b) jejunal diverticulosis is a heterogenous disorder associated with at least three abnormalities of the smooth muscle or myenteric plexus, (c) in contrast to intestinal pseudoobstruction without diverticulosis, the esophagus, stomach, and colon are less frequently involved in jejunal diverticulosis, and (d) some patients with jejunal diverticulosis probably have clinically inapparent progressive systemic sclerosis.

Contraction abnormalities of the esophageal body in patients referred for manometry

A method is proposed for classifying conventional esophageal manometry findings according to the features observed in the esophageal body rather than the disease name. Patients who demonstrate increases in mean distal wave amplitude, mean distal wave duration, or number of abnormal motor responses (repetitive or simultaneous contractions) or the presence of triple-peaked waves are classified as having distal contraction abnormalities. Severity is scored by the cumulative number of these abnormalities. Of the 210 patients referred for esophageal manometry over a 23-month period, 119 (57%) were found to have one or more of the contraction abnormalities, making this the most common esophageal body manometric classification. The presence of one or two abnormalities was most common. Patients with all four abnormalities represented less than 5% of the referred population and, as a group, would satisfy usual manometric criteria for diffuse esophageal spasm. The proposed system allows for recognition of patients with only one or several of the manometric features typically seen in diffuse esophageal spasm and provides a method for intra- and interstudy comparison of patients with these common findings.

Manometric characteristics of the extrahepatic biliary tract in dogs.

Pressure measurements of the whole extrahepatic biliary tract were performed in dogs using constant perfusion techniques. Studies were done during general anesthesia and in conscious trained dogs. Gallbladder intraluminal fasting pressure was significantly lower than common bile duct pressure. No motor activity was demonstrated in the common bile duct. At the cysticocholedochal junction a static high-pressure zone was observed, probably due to mechanical factors. At the level of the choledochoduodenal junction, a high-pressure zone with a sphincter behavior was demonstrated, with a resting pressure of 38 cm H2O above intraduodenal pressure and with rhythmic contractions with a mean amplitude of 88 cm H2O above duodenal pressure. Duodenal motility was completely different from sphincter dynamics. No differences in pressure values were seen during general anesthesia or in conscious state.

Clinical and manometric findings in benign peptic strictures of the esophagus.

To determine the possible factors that may contribute to the development of peptic stricture of the esophagus, clinical and manometric features were compared in patients with symptomatic gastroesophageal reflux and those with peptic strictures of the esophagus. Patients with stricture were older and had a longer duration of heartburn than patients without a stricture. Most importantly, patients with stricture had a more marked decrease in lower esophageal sphincter (LES) pressure, 4.9 +/- 0.5 mm Hg, than patients without a stricture, 7.5 +/- 0.6 mm Hg, P less than 0.01. The LES pressure in all patients with stricture was below 8 mm Hg, and did not overlap with normal values. Patients with stricture had either a nonspecific motor abnormality of aperistalsis (64%), compared to patients with symptomatic reflux (32%), P less than 0.05. Thus, peptic stricture of the esophagus is commonly associated with a long duration of reflux symptoms in patients with a very low LES pressure and esophageal motor disorder.

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.

Achalasia secondary to carcinoma: manometric and clinical features.

The clinical and diagnostic features of a secondary type of achalasia of the esophagus are described in seven patients with various types of malignancies. Patients with secondary achalasia presented with dysphagia of short duration and marked weight loss; mean age was 64 years. Esophageal manometry showed features identical to those of idiopathic primary achalasia: aperistalsis, poor lower esophageal sphincter relaxation, and elevated sphincter pressure. Endoscopy and barium swallow showed evidence of a tumor in only two cases. Various types of malignancies may produce a secondary form of achalasia that has diagnostic features identical to those of primary achalasia and is best identified by its clinical presentation.

Combined Collis-Nissen Reconstruction of the Esophagogastric Junction

Recent reports have indicated that combined Collis-Belsey reconstruction of the esophagogastric junction fails to control reflux in 30 to 46% of patients undergoing the procedure. The major factor thought to be responsible for this result is the limited Belsey fundoplication possible after construction of the gastroplasty tube. This report describes our technique of combining the Collis gastroplasty with a 360-degree Nissen type fundoplication. The radiographic and manometric characteristics of the distal esophageal high-pressure zone produced by the Collis-Nissen operation are discussed.

Sororal occurrence of diffuse esophageal spasm

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.

Oropharyngeal dysphagia Manometric and cine esophagraphic findings.

The manometric and cineradiographic features of 14 patients with oropharyngeal dysphagia are described. Failure of the upper-esophageal sphincter (UES) to relax completely and incoordination of UES relaxation with pharyngeal contraction were noted as was poor initiation of swallows and disorganization of pharyngeal contraction. The relative merits of cine esophagography and esophageal manometry in evaluating oropharyngeal dysphagia are assessed, and a functional classification for this disorder is proposed. Clinical improvement in 4 of 5 patients who underwent UES myotomy for severe intractable dysphagia did not always correlate with improvement in manometric and cine studies.

Dysfunction of the lower esophageal sphincter in disorders other than achalasia.

Nine patients, all with symptoms of dysphagia and/or substernal chest pain, are described. In 5, radiologic studies of the esophagus were normal, and in none was there an increase in esophageal caliber. Each patient had dysfunction of the lower esophageal sphincter, as assessed by manometry, the chief abnormality being impairment of relaxation in 7, and derangement of contraction in 2. All showed peristaltic activity in the esophageal body during manometric studies. One patient had the classic manometric pattern of diffuse esophageal spasm. In the remainder, some but not all of the manometric features of this disorder were present. These observations serve to emphasize that the boundaries between achalasia, diffuse esophageal spasm, and other less well-characterized disorders of esophageal motility are blurred; and they are consistent with the view that such disorders represent parts of a continuous spectrum.