Esophageal leiomyomatosis

PURPOSE: To reassess the clinical and radiologic findings in patients with esophageal leiomyomatosis. MATERIALS AND METHODS: A search of the authors' radiologic archives revealed six cases of esophageal leiomyomatosis in a 22-year period. The clinical findings and radiologic images were reviewed retrospectively. RESULTS: The average age of the patients was 10.8 years (range, 6-18 years). Five patients presented with slowly progressive dysphagia. Barium studies revealed smooth, tapered narrowing of the distal esophagus in five patients and characteristic defects on the superomedial aspect of the gastric fundus abutting the cardia, presumably due to bulging of this thickened mass of muscle into the stomach, in four patients. In two patients, computed tomography (CT) revealed marked thickening of the distal esophageal wall. CONCLUSION: Esophageal leiomyomatosis can be suggested in a pediatric patient with long-standing dysphagia in whom smooth, tapered distal esophageal narrowing is seen at barium study and circumferential esophageal wall thickening is seen at CT.     

Acute ethanol inhibits calcium influxes into esophageal smooth but not striated muscle: a possible mechanism for ethanol-induced inhibition of esophageal contractility

In both humans and cats, EtOH administered in vivo and acutely decreases contractility of smooth muscle of lower esophageal sphincter (LES) and lower esophagus (LE), but not striated muscle of upper esophagus. To see if these effects are associated with perturbation of Ca++ homeostasis, esophageal muscle slices were incubated in vitro with EtOH and then 45Ca++. At steady-state Ca++ uptake, some slices were exposed to 1 microM carbachol (CCH). Although 100 mM EtOH had no effect on Ca++ uptake into resting or stimulated striated muscle of upper esophagus, it significantly inhibited Ca++ uptake into smooth muscle of LES and LE. For unstimulated LE and resting LES, 100 mM EtOH significantly inhibited both initial uptake and steady-state levels, whereas lower doses had no significant effect. EtOH at 100 mM also affected changes in Ca++ content induced by CCH stimulation. CCH increased total exchangeable tissue Ca++ content in LE, whereas it decreased Ca++ content in LES. EtOH at 100 mM blunted these CCH-induced effects in both LES and LE. In contrast to resting muscle, inhibition of CCH-stimulated LE muscle was not limited to 100 mM EtOH, because substantial and significant inhibition was also seen at EtOH doses of 25 and 50 mM, doses which are relevant even in social drinking. Thus, EtOH inhibition of Ca++ influx into esophageal muscle is selective for smooth muscle, can occur at pharmacologically relevant EtOH doses and could be the underlying mechanism for EtOH's inhibition of contractility of esophageal smooth muscle.     

Fusiform dilatations of the esophagus secondary to protracted distention and emetogenic injury

We report the case of a 74-year-old woman who presented with a 2-year history of dysphagia, weight loss, nausea, and vomiting. She was diagnosed as having secondary achalasia due to external compression probably by a tumor of the lower part of the esophagus. At autopsy, however, no tumor was found at that site, whereas a pancreatic microcystic serous adenoma and multiple gastric leiomyomata--one of which occupied the pyloric sphincter area leading to gastric outlet obstruction--were noted. The esophagus displayed two fusiform dilatations located at the lower and midportions, the latter being associated with rupture and necrosis of the muscularis and adventitial wall layers. The lower dilatation showed only attenuation of the muscularis, without necrosis. The epithelium was intact in both dilatations. This was an unusual series of pathogenetic events, leading from gastric outlet obstruction to secondary achalasia and protracted vomiting, followed by spontaneous partial esophageal wall rupture (a variant of intramural hematoma) or atrophy of the muscularis, morphologically evident as fusiform dilatations.     

Esophageal wall thickening: a CT finding in diffuse esophageal spasm

We report three patients with esophageal wall thickening, incidentally found at CT, in whom further evaluation led to the diagnosis of diffuse esophageal spasm (DES). All cases showed smooth, symmetric, circumferential wall thickening of the distal two-thirds of the esophagus with normal periesophageal fat. No lung parenchymal abnormalities suggestive of aspiration were seen. DES, although uncommon, is another benign condition that should be included in the differential diagnosis of esophageal wall thickening detected by CT.     

Coordination of peristalsis in pharynx and esophagus

When a swallowed liquid bolus is followed from mouth to stomach in man by contrast studies or manometry, it traverses its course without hesitation even though the bolus is propelled by striated muscle contraction in the first part of its journey and smooth muscle in the latter part. The striated muscle is innervated by excitatory cholinergic nicotinic cranial nerves whereas the smooth muscle of the esophagus is innervated by the enteric nervous system (ENS) through excitatory and inhibitory nerves. These differences can be demonstrated by observing the inhibitory effects of curare and atropine, the first blocking nicotinic receptors and the second muscarinic receptors. Early students of esophageal motility recognized that peristalsis could be initiated in two ways. The first is initiated by a swallow and is called primary peristalsis and the second called secondary peristalsis is initiated by distension of the esophagus. It was proposed that primary peristalsis was initiated by a single sensory input activated by the bolus entering the pharynx which in turn activated a motor program in the brain stem. Secondary peristalsis was believed to be stimulated by multiple afferent impulses arriving from the esophagus as the bolus passed down the esophagus. More recent studies using manometric techniques have suggested that the only difference between primary and secondary peristalsis is the afferent stimuli and the effector mechanism is the same. Subsequent studies of carefully timed, paired swallows, transection of vagus nerves and esophagus, and single nerve recordings suggest that the answer lies between the two extremes noted above.(ABSTRACT TRUNCATED AT 250 WORDS)     

Use of electromyography in a cow with esophageal paralysis

Electromyography of the esophageal musculature of a cow that had been regurgitating and losing weight after correction of an esophageal obstruction was suggestive of unilateral denervation. Radiography before and after oral administration of barium sulfate revealed a dilated terminal portion of the thoracic esophagus, suggestive of a focal motility problem. Electromyography was performed on the esophageal musculature by positioning the electrode through the rumen fistula and into the thoracic esophagus and by positioning the electrode percutaneously with the aid of a tube placed in the cervical esophagus. Numerous fibrillation potentials and sharp epsilon-positive waves were detected in the muscles of the left side of the distal portions of the cervical and thoracic esophagus. During hospitalization, the cow was given alfalfa pellets, ground corn, and sodium bicarbonate through the rumen fistula 3 times each day for several weeks to maintain body weight.     

Characterization and mediation of inhibitory junction potentials from opossum lower esophageal sphincter

BACKGROUND: Activating nonadrenergic, noncholinergic (NANC) nerves of the lower esophageal sphincter (LES) hyperpolarizes and relaxes its circular smooth muscle. This relaxation is mediated by nitric oxide (NO) or an NO-containing compound. These studies were undertaken to compare the electrophysiological responses of circular smooth muscle from the LES and esophagus in response to NANC nerve stimulation and to test the hypothesis that NO mediates LES hyperpolarization. METHODS: The transmembrane potential difference was recorded with glass microelectrodes. Nerve-mediated membrane responses were evoked by electrical pulses of 0.5 msec duration and 50 V amplitude. RESULTS: Responses of LES muscle differed from those of the esophageal muscle. The duration of hyperpolarization was much longer in sphincteric muscle. The depolarization that followed hyperpolarization of esophageal muscle was not observed in sphincteric muscle. NG-nitro-L-arginine, an inhibitor of NO synthase, attenuated the nerve-induced hyperpolarization. L-arginine, the substrate for NO synthase, antagonized the effect of NG-nitro-L-arginine. Exogenous NO hyperpolarized of the smooth muscle membrane. CONCLUSIONS: These data support the hypothesis that NO or an NO-like compound may mediate nerve-induced hyperpolarization of the opossum LES.     

Pathology of the intrinsic muscles of the larynx

The intrinsic muscles of the larynx were exposed in each of 54 normal larynges derived from the autopsy series of a general hospital and submitted to microscopic examination. Muscle fibre alterations were found in all the posterior cricocarytenoid muscles in patients from 13 years of age on wards. In about half of the patients these changes included necrosis of segments of muscle fibres and secondary cellular reactions to this. In two cases of chronic autonomic failure with multiple system atrophy the intrinsic laryngeal muscles were similarly examined. Both cases showed a marked gross and microscopic atrophy of the posterior cricoarytenoid muscles; there were also microscopic changes similar to those found in the normal population. The significance of the changes in the routine post-mortem larynges, the changes in the cases of chronic autonomic failure with multiple system atrophy and the relationship between the two are discussed.     

Evaluation and treatment of speech and swallowing disorders associated with myopathies

Dysphagia, or disordered swallowing, can be demonstrated at any time over the course of many myopathies. Ability to swallow may be impaired because of weakness, inflammation, or dysfunction of the oropharyngeal, laryngeal, and esophageal musculature. Dysphagia may occur during the progression of disease regardless of whether the patient is properly treated. The presentation of signs of dysphagia can vary among patients because of differing patterns of weakness or incoordination of the facial muscles, lips, tongue, palate, pharyngeal constrictors, or smooth and striated muscles of the esophagus. Although the literature has focused on problems in the esophagus, scant attention has been paid to the oropharynx, which is often equally affected. Studies suggest that surgical myotomy and botulinum toxin injection may provide benefits for some patients with esophageal dysfunction. Although the condition is pervasive, there is little information on the incidence of dysphagia in muscular disorders. Because a major complication of dysphagia is aspiration, any sign of swallowing impairment demands medical attention and treatment.     

Motor performance in Alzheimer''s disease

A 47-year-old man with Hirayama's disease who developed cervical spondylotic amyotrophy (CSA) is presented. The patient had noted weakness and atrophy of hand and forearm muscles bilaterally at the age of 16. At the age of 40, he developed proximal muscle atrophy and weakness bilaterally after 20 years of a non-progressive state. Myelography and computed tomography (CT)-myelography revealed that ventral cord compression at multiple levels of C4-7 vertebral bodies was increased when the neck was extended. The clinical diagnosis was CSA associated with Hirayama's disease. To our knowledge, this is the first such case to be reported.     

The relationship between stapling doughnuts: characteristics and functional results after total gastrectomy

BACKGROUND/AIMS: The objective of this study was to determine whether the thickness of the esophageal doughnut is related to postoperative results in total gastrectomy. METHODOLOGY: Thirty-eight total gastrectomy patients were studied, including 18 who underwent jejunal pouch reconstruction and 20 who did not have pouch reconstruction. We used the Proximate-ILS circular stapler, with purse-string suturing at the cut end of the esophagus performing only the mucosal layer manually in all cases. We divided the esophageal doughnuts after stapling into two groups: Group A: doughnut involving muscle tissue of 0-25% of the esophageal circumference; Group B: doughnut involved muscle tissue of 25-100% of the esophageal circumference. Reflux scores and the scintigraphic reflux index were determined. RESULTS: No significant difference was found between the two groups in reflux score or scintigraphic reflux index. CONCLUSIONS: These findings indicate that the degree of postoperative reflux esophagitis was not affected by the amount of the muscular layer included in the esophageal doughnut. Continuity, and not variability of the degree of involved muscle in the esophageal doughnut, is an important factor at the time of stapling.     

Thoracoscopic surgery for benign esophageal diseases

The thoracoscopic surgery for two benign esophageal diseases, esophageal leiomyoma and esophageal diverticulum, were successfully performed. Case 1 was a 37-year-old female with esophageal leiomyoma that was located at 30 cm from the incisor of the left anterior esophagus. The tumor was enucleated under the thoracoscopy, combined mini-thoracotomy for 3 cm in length. It was useful enough to rotate the left side to the right with two slings traction for better visualization of lesion site. After resection, the proper muscle layer of the esophagus was closed. Case 2 was a 70-year-old male, who complained of dysphagia because of esophageal diverticulum. It was 8 cm in size and located at 28 cm from the incisor of the right wall of the esophagus. It was also resected under the thoracoscopy, combined mini-thoracotomy for 3 cm in length. Intraluminal esophagoscope was useful to dissect safely and confirm the intralumen of the diverticulum. Its neck was divided parallel to the longitudinal axis of the esophagus by two endo-staplers. And then, the muscle layer was closed. It was suggested that esophageal leiomyoma and esophageal diverticulum were suitable for thoracoscopic surgery.     

An operational model of the antigen-antibody interaction

We investigated smooth muscle cell proliferation associated with restenosis after percutaneous transluminal coronary angioplasty (PTCA) in 8 arteries with fragmented internal elastic lamina obtained at autopsy in 7 patients who died between 2 months to 2 years 11 months after coronary angioplasty. The internal elastic lamina fragmentation, measured longitudinally along the blood vessels, measured 6.6 +/- 6.9 mm. Smooth muscle cell proliferation was concentrated around the fragmented internal elastic lamina, extending longitudinally even to unfragmented areas. The proliferation of smooth muscle cells extended for 1.8 +/- 2.2 mm in the proximal portion of the fragmentation, and for 2.0 +/- 2.9 mm in the distal portion. The possibility of new stenoses resulting from smooth muscle cell proliferation at sites adjacent to those subjected to PTCA should be borne in mind when PTCA of the proximal segments of the left anterior descending coronary artery is contemplated.     

Thoracoscopic resection of a giant leiomyoma of the esophagus with a mediastinal outgrowth

We reported a case of a 20-year-old man with a giant leiomyoma of the esophagus resected under video-assisted thoracic surgery (VATS). The patient demonstrated an abnormal shadow on a chest x-ray and a posterior mediastinal tumor 11 cm in diameter on a computed tomogram (CT) and on magnetic resonance imaging (MRI). A leiomyoma or a neurogenic tumor of the esophagus was suspected, and VATS was performed. The resected tumor was pathologically confirmed to be a leiomyoma of the esophagus. A giant esophageal leiomyoma showing extraluminal outgrowth should be treated by VATS.     

Beneficial effect of proteases on allograft arteriosclerosis in a rat aortic model

PURPOSE: Our goal was to describe the CT findings in patients with an apparent mass at the thoracic inlet on barium swallow and to further explore its etiology by making appropriate measurements on CT. METHOD: Barium swallows and CT scans of five patients with esophageal pseudomass were reviewed and compared with CT scans of 65 controls. Anteroposterior (AP) diameters of the thoracic inlet were measured, and the relationships of the esophagus to the trachea were determined on CT. RESULTS: Absence of a demonstrable mass on CT in patients with an extrinsic impression on barium swallow was associated with narrowed AP diameter of the thoracic inlet (< 5 cm) and the esophagus to the left of the trachea. A significant correlation was observed between the AP diameter of the inlet and the position of the esophagus in relation to the trachea in control subjects (r = 0.52, p < 0.001); with diminished diameter, the esophagus is more frequently located to the left side of the trachea. CONCLUSION: An apparent mass is simulated by lateral deviation of the lower cervical esophagus, due to diminished available space between the trachea and the esophagus in subjects with a narrow (< 5 cm) AP diameter of the thoracic inlet.     

Isometric and isotonic contractions in airway smooth muscle

Canine tracheal smooth muscle was used as an in vitro model of smooth muscle in intrapulmonary airways to determine whether active tension curves derived from isometric and isotonic muscles are similar, and thus resemble striated muscle in this respect. Isometric, isotonic after-loaded, and isotonic free-loaded contractions elicited at different lengths and loads, were analysed. The data demonstrate that length-tension (L-T) diagrams were different in these various types of contractions for electrically and carbachol driven tracheal smooth muscles strips. In general, at any given length active tension is less in isotonic and free-loaded modes of contraction as compared with isometric. We conclude that the ability to actively develop tension at a given length in airway smooth muscle depends on the mode of contraction.     

Extranodal non-Hodgkin lymphoma involving the muscles of mastication

Involvement of the skeletal muscle by non-Hodgkin lymphoma (NHL) is unusual. The most commonly affected muscles are those of the extremities, pelvis, and gluteal regions; rarely are the muscles in the head and neck involved. We report the clinical, CT, and MR imaging findings in two patients with extranodal NHL in the head and neck region involving the muscles of mastication. One patient was immunocompetent and had lymphoma that arose within the muscles; the other was a patient with AIDS who had disseminated disease at diagnosis. In both patients, the involved muscles were isodense with normal muscles on CT scans. On MR images, the infiltrated muscles were isointense with normal muscles on the T1-weighted sequence and hyperintense on the fast spin-echo T2-weighted sequence, with variable enhancement after administration of intravenous contrast material.     

Leiomyomata of the esophagus. An analysis of 838 cases

Review of the world literature to the end of 1971 has provided data on 838 cases of esophageal leiomyomata, including our own 19 surgically removed lesions. Although esophageal leiomyoma is the most common of the benign tumors of the esophagus, it is still rare compared with carcinoma. It occurs in more men than women, by a ratio of 1.9 to 1. Over 50% of the patients with leiomyoma of the esophagus are asymptomatic. Dysphagia and vague pain are the most frequent symptoms. Pyrosis is mentioned in the literature as present in 40% of the cases, but it is considered mainly as symptom of coexistent hiatal hernia. Diagnostic problems often arise, as the smooth muscle tumors may mimic mediastinal neoplasms, cysts, or even aneurysms, or complicate coexisting hiatal hernia and esophageal diverticulum. Operative management by transthoracic enucleation is the procedure of choice, although resection of the esophagaus may be required in few cases. Postoperative morbidity is minimal and results are excellent.     

Two cases of gastric involvement of esophageal cancer: intramural metastasis and intramural lymph node metastasis

Cases of esophageal cancer with intramural metastasis to the stomach and esophageal cancer with metastasis to an intramural lymph node of the stomach are herein reported. The former patient was a 52 year-old male. Squamous cell carcinoma (SCC) of the lower esophagus with an intramural metastasis located at the gastric cardia and a small leiomyoma at the fornix were resected. The latter patient was a 70 year-old female. SCC of the lower esophagus and an intramural lymph node metastasis located at the anterior wall of the gastric cardia were resected. The patient died nevertheless of multiple liver metastases. These gastric involvements were detectable by endoscopy before surgery. The clinicopathological features of these esophageal cancers were characterized; the sites were the lower part of the esophagus, and extreme lymphatic and vascular invasions were shown histologically. The gastric tumors were located in the upper third of the stomach, and the findings revealed submucosal tumors. It is therefore important to discriminate other gastric tumors, and to resect them simultaneously with esophageal cancer when a gastric tube has been used for reconstruction after esophagectomy.     

Differentiation of achalasia from pseudoachalasia by computed tomography

OBJECTIVES: The purpose of this study was to determine the computed tomography (CT) findings in idiopathic achalasia and in the pseudoachalasia of malignancy. METHODS: We identified 12 patients with the manometric diagnosis of achalasia who also had CT scans available for review: eight had idiopathic achalasia, and four had pseudoachalasia. As controls, we selected nine patients with endoscopically obvious esophageal cancer who also had CT scans. The CT scans were blindly reviewed to determine esophageal wall thickness, symmetry of the esophageal wall, presence of esophageal dilation or mass, and a radiological diagnosis. RESULTS: Six of the eight patients with achalasia had a dilated esophagus. Five had symmetric wall thickening >5 mm (range 7-10 mm) at the gastroesophageal junction. One patient with a 10-mm wall thickening was incorrectly diagnosed with a mass. All others were correctly diagnosed with achalasia. Three of the four patients with pseudoachalasia had esophageal dilation. Two had an obvious esophageal mass. The other two were given an indefinite diagnosis: one had asymmetric wall thickening (11 mm) at the gastroesophageal junction, and the other had symmetric thickening of 18 mm. Eight of the nine patients with obvious esophageal cancer had a mass on CT; the other patient had asymmetric wall thickening of 6 mm at the gastroesophageal junction and was given an indefinite diagnosis. CONCLUSIONS: Most achalasia patients have CT findings of esophageal dilation and mild, symmetric wall thickening. Therefore, symmetric esophageal wall thickening (<10 mm) should not dissuade one from the diagnosis of achalasia. Most pseudoachalasia patients have CT findings of esophageal dilation, more marked and/or asymmetric wall thickening, or mass. In this group, asymmetric or marked thickening (>10 mm) indicated pseudoachalasia. Therefore, CT can be helpful in differentiating between achalasia and the pseudoachalasia of malignancy.