Subtotal esophageal resection in motility disorders of the esophagus

BACKGROUND: Esophagectomy for motility disorders is performed infrequently. It is indicated after failed medical therapy, pneumatic dilation, non-resecting surgical and redo procedures. Patient selection in this group is challenging and the operative risk has to be weighted carefully against the poor quality of life with persistent or recurrent dysphagia. PATIENTS AND METHODS: Between September 1985 and April 2004, subtotal esophageal resections for advanced esophageal motility disorders of the esophagus not responding to previous therapy were carried out in 8 patients (6 females, 2 males). The median age of these patients was 59.5 (43-78) years. Six patients had a megaesophagus secondary to achalasia; 1 patient had a non-specific esophageal motility disorder with a stenosis of the distal esophagus, and a further patient displayed a recurrent huge epiphrenic diverticulum, which occurred in the context of a collagen disease. A transhiatal esophageal resection was performed in 6, a transthoracic procedure in 2 patients. RESULTS: Outcome assessment was done after a follow-up of 43.5 (3-92) months in median. The resection and reconstruction of the esophagus in advanced and decompensated esophageal motility disorders led to a marked functional improvement with disappearance of dysphagia. Despite previous therapeutic failures, alimentation could be restored in all patients. CONCLUSION: Favourable long-term results with significant improvement of symptoms can be achieved by esophageal resection even if endoscopic therapy or non-resecting surgical measures are unsuccessful. Transhiatal esophagectomy with gastric pull-up should be the preferred procedure and can be performed with low morbidity.     

Hypertrophy of the muscularis propria of the lower esophageal sphincter and the body of the esophagus in patients with primary motility disorders of the esophagus

OBJECTIVES: Patients with diffuse esophageal spasm (DES) and nutcracker esophagus/high amplitude esophageal contraction (HAEC) have a thicker esophageal muscularis propria than do healthy subjects. The goals of this study were to determine the esophageal muscle cross-sectional area (MCSA), a measure of muscle mass, in patients with achalasia of the esophagus; and to compare it with that in patients with DES, patients with HAEC, and normal subjects. METHODS: Using a high-frequency ultrasound probe catheter, concurrent manometry and ultrasound images of the esophagus were recorded in four subject groups: normal volunteers, patients with HAEC, patients with DES, and patients with achalasia of the esophagus. Recordings were obtained from the lower esophageal sphincter (LES) and multiple sites in the esophagus 2, 4, 6, 8, and 10 cm above the LES. RESULTS: The LES and esophageal muscle thickness as well as esophageal MCSA were greater in all three patient groups than in the normal subject group. Muscle thickness and MCSA were observed to be greatest in patients with achalasia, which were greater than in patients with DES, which were greater than in those with HAEC, which in turn were greater than in normal subjects. CONCLUSIONS: We propose that an increase in the MCSA is an important feature of patients with primary motility disorders of the esophagus. The degree of increase in muscle mass may be an important determinant of the type and the severity of esophageal motor dysfunction.     

Current advances in the physiopathology of primary motility disorders of the esophagus

The authors review the recent literature about the classification of primary motor disorders of the oesophagus: achalasia, diffuse oesophageal spasm, nutcracker oesophagus, hypertensive lower oesophageal sphincter and non-specific intermediary disorders. In fact these motility disorders belong to a spectrum of diseases closely related, with chronological transformation of a specific disorder to another one or to intermediary disorders. Most of the recent pathophysiological research concerned primary achalasia, secondary achalasia syndromes being a kind of experimental model. These studies point to a morphological or functional deficiency of postganglionic nerves inhibiting the lower oesophageal sphincter (LOS) through noncholinergic nonadrenergic neurotransmitters. Recent advances in the treatment of achalasia and other motility disorders are not yet based on these findings. Although calcium channel blockers, like nifedipine, lower LOS basal pressure, they are not very useful on long term relief of symptoms of achalasia. Pneumatic dilatations or cardiomyotomy still remain the best methods of treatment of this disease.     

Effect of isosorbide and hydralazine in painful primary esophageal motility disorders

Five patients with painful primary esophageal motility disorders underwent pharmacologic testing with isosorbide and hydralazine. While neither agent affected baseline amplitude or duration of distal esophageal contractions, pretreatment with hydralazine significantly blunted the response to bethanechol (mean esophageal contraction duration, 31.4 +/- 4.8 s after bethanechol alone vs. 12.7 +/- 1.8 s after bethanechol and hydralazine p less than 0.005). Premedication with isosorbide was significantly less effective. In addition, while all 5 patients experienced chest pain in response to bethanechol alone, only 1 of 5 experienced chest pain in response to bethanechol after previous hydralazine administration; 3 patients had chest pain after previous administration of isosorbide. Patients who were placed on long-term oral hydralazine therapy experienced improvement in chest pain and dysphagia with concomitant decrease in amplitude and duration of esophageal contractions on repeat motility study (176.5 +/- 23.8 mmHg to 97.3 +/- 27.0 mmHg, p less than 0.05, 7.5 +/- 0.8 s to 5.2 +/- 0.5 s, p less than 0.005). Hydralazine appears to be of value in the treatment of diffuse esophageal spasm and other painful primary esophageal motility disorders.     

Esophageal motility in cirrhotics with and without esophageal varices

Esophageal manometry was performed in 45 cirrhotics with varices, in 15 cirrhotics without varices, and in 20 normal subjects, to define the effect of varices on esophageal motility. Cirrhotics with varices showed a decreased amplitude of motor waves in the lower half of the esophagus (p less than 0.01), an increased duration of primary peristaltic waves along the entire length of the esophagus (upper esophagus, p less than 0.05; lower esophagus, p less than 0.01), and an increased peak-to-peak speed of primary peristaltic waves (p less than 0.01). Resting lower esophageal sphincter pressure and duration of sphincter relaxation were similar in patients and controls. The above-mentioned abnormalities might be due to the mechanical effect of the presence of varices.     

Early complications of pneumatic dilatation in the treatment of primary motility disorders of the esophagus

We analyze the incidence and evolution of the early complications of 96 consecutive patients with primary esophagus motor disorders, treated with pneumatic dilatation under endoscopic control (1.4 sessions per patient). In 4 (0.042/patient, 0.029/dilatation) patients the esophagus was perforated; the diagnosis was made in the first 24 hours; pneumomediastinum was a constant finding in the radiological exploration. In three cases the complication was suspected because of the apparition of sustained thoracic pain after the dilatation maneuver and in one case the presentation symptom was bleeding of cardial mucosa, larger than usual, at the end of the dilatation. The four patients evolved favorably with conservative treatment (avoidance of oral food intake, gastroesophageal aspiration, antibiotic therapy and parenteral nutrition).     

Neuropathological study of the esophagus in cirrhotic patients with esophageal varices

A neuropathological study was performed in autopsy specimens of the esophagus of 8 cirrhotic patients with large esophageal varices, in which cases marked dilatation as well as motility disorders of the esophagus were demonstrated by esophagography, esophagoscopy and esophageal manometry. Histological findings of the esophagus of cirrhotics were also compared to those of 7 control patients without esophageal or liver disease. Total and normal ganglion cell counts/cm2 at the Auerbach's plexus were found to have been significantly decreased, while the rate of the ganglion cells with the deformity of nucleus or with degenerated Nissl granules significantly increased at the upper, middle and, in particular, lower esophagus. Thus, the reduction of the number of and the degeneration of ganglion cells at the Auerbach's plexus in the esophagus of cirrhotic patients with large varices are clearly demonstrated, and it is further suggested that such neuropathological changes as described above may have contributed to, at least in part, the development of impaired motility of the esophagus with large varices.     

Upper-extremity deep vein thrombosis and downhill esophageal varices caused by long-term pacemaker implantation

Upper-extremity deep vein thrombosis is common after pacemaker or cardioverter-defibrillator implantation. Only 1% to 3% of patients with upper-extremity deep vein thrombosis become symptomatic. Downhill esophageal varices develop in the upper third of the esophagus as a result of the obstruction of the superior vena cava. Herein, we report the case of a 54-year-old man--a recipient of multiple implanted cardiac pacemakers--who presented with bilateral upper-extremity deep vein thrombosis. This severely symptomatic condition was complicated by very rare and life-threatening downhill varices of the upper esophagus, but without bleeding. To the best of our knowledge, this is the 1st report of this array of conditions.     

Esophageal motility disorders in cirrhotics treated by sclerosing the varices

We performed esophageal manometry on 17 cirrhotic patients (group I) treated with repeated varicose sclerosis (VS) after the varices had been completely eradicated. We used 5% ethanolamine oleate with the free hand technique, administering intra-varix injections at the cardia level, never exceeding 25 cc per session. The first two sessions were spaced a week apart, and the consecutive ones were on a monthly basis. The mean number of VS sessions was 5.52. The esophageal motility study was carried out on the average 12.3 months after the last VS session, with a minimum of six and maximum of 17 months. As controls we used 16 cirrhotic patients with unsclerosed varices (group II) and 26 healthy subjects (group III). The mean age and patient distribution were similar, according to Pugh grading. The length of the lower esophageal sphincter (LES) and the amplitude of the propulsive waves in the middle esophagus were similar in all three groups. We found the LES pressure to be significantly reduced in group I (17.52 +/- 2.8 mmHg) in relation to group II (20.26 +/- 2.49 mmHg) (p less than 0.001) and group III (22.86 +/- 3.73 mmHg) (p less than 0.01). The group II patients showed significantly lower pressure levels than the group III ones (p less than 0.05). The amplitude of peristaltic waves in the distal esophagus was significantly less in group I (22.94 +/- 7.31 mmHg) than in group II (37.46 +/- 10.95 mmHg) (p less than 0.01) and group III (44.8 +/- 11 mmHg) (p less than 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)     

Clinical use of ambulatory 24-hour esophageal motility monitoring in patients with primary esophageal motor disorders

The development of miniaturized electronic pressure transducers and portable digital data recorders with large storage capacity has made ambulatory monitoring of esophageal motor function over an entire circadian cycle possible. Broad clinical application of this new technology in a large number of asymptomatic normal volunteers and patients with symptoms suggestive of a primary esophageal motor disorder provides new insights into esophageal motor function under a variety of physiologic conditions in health and disease. These studies suggest that ambulatory esophageal motility monitoring allows for a more precise classification of esophageal motor disorders than standard manometry and can identify abnormal esophageal motor patterns associated with nonobstructive dysphagia or noncardiac chest pain. Ambulatory esophageal motility monitoring performed in combination with pH monitoring is currently the most physiologic way to assess esophageal function and has potential to improve diagnosis and management of patients with esophageal motor disorders. Ambulatory 24-h esophageal motility monitoring should become the gold standard for assessing motor function of the esophageal body.     

A case of superficial carcinoma of the esophagus on esophageal varices

We report a case of superficial esophageal carcinoma in a patient with esophageal varices. Esophagogram revealed a 2 cm large irregular mucosal elevation during work-up for esophageal varices which was acertained on endoscopical examination to be carcinoma. Biopsy material showed moderately differentiated squamous cell carcinoma. Subtotal esophagectomy was performed followed by reconstruction by cervical esophagogastrostomy through the retrosternal route. Few resected cases of esophageal carcinomas accompanied by esophageal varices have been reported because 1) early radiographic abnormalities of small superficial esophageal carcinomas can be overlooked in the case of large varices and 2) the number of operable cases is limited because of associated liver cirrhosis.     

Treatment of spastic esophageal motility disorders

Treatment of spastic motility disorders continues to be challenging.Therapeutic options remain limited due in part to our lack of understanding of the pathophysiology and significance of these disorders. Furthermore, most of therapeutic trials to date are hampered by the poorly designed nature of the study, including the small size of the trials and the lack of placebo arm. Most of the available information suggests that there seems to be an important dissociation between symptoms (chest pain/dysphagia) and esophageal dysmotility. Drug treatment aimed at visceral sensitivity seems more effective in relieving symptoms than spasmolytic medications. Recent trials with Botox, nitric oxide derivatives, and SSRIs offer promising results. Rigorous study design that includes large placebo-controlled trials is needed in this area.     

Medical treatment of esophageal motility disorders

Swallowing is a complex mechanism that is based on the coordinated interplay of tongue, pharynx, and esophagus. Disturbances of this interplay or disorders of one or several of these components lead to dysphagia, non-cardiac chest pain, or regurgitation. The major esophageal motility disorders include achalasia, diffuse esophageal spasm, hypercontractile esophagus (nutcracker esophagus), and hypocontractile esophagus (scleroderma esophagus). Other esophageal diseases such as hypopharyngeal (Zenker's) diverticula or gastroesophageal reflux disease also may be sequelae of primary esophageal motility disorder. Finally, a substantial group of patients referred for evaluation of possible esophageal motor disorders have milder degrees of dysmotility—referred to as nonspecific esophageal motor disorder—that are of unclear clinical significance. Medical treatment of esophageal motility disorders involves the uses of agents that either reduce (anticholinergic agents, nitrates, calcium antagonists) or enhance (prokinetic agents) esophageal contractility. Despite the beneficial effect of the various drugs on esophageal motility parameters, the clinical benefit of medical treatment is often disappointing. From clinical and epidemiological studies there is some evidence for a psychological component in the pathogenesis or perception of esophageal symptoms. Further understanding of esophageal pathophysiology, as well as development of new receptor selective drugs, might increase our chances of successful treatment of esophageal motility disorders.     

Reoperations after esophagomyotomy in esophageal motility disorders

Out of 96 patients with the diagnosis of primary esophageal motor disorders and treated by esophagomyotomy, a group of 9 patients is reported in whom reoperation was necessary because of persistence or worsening of the previous symptoms (8 patients) or persistent reflux esophagitis (one patient). Clinical and laboratory examinations together with the operative findings allowed classification of these patients: incomplete myotomy proximally (4 patients) or distally (one patient), fibrotic scar at the site of previous myotomy (2 patients), persistence of intact muscle fibers (one patient) and reflux esophagitis for lack of an antireflux intervention during myotomy. Treatment consisted of completing myotomy proximally or distally, resection of the fibrous tissue and an antireflux operation when indicated. Clinical results were excellent in 6 patients (66.6%), fair in 2 patients (22.2%) and bad in one case (11.1%). Fair or bad results were seen in patients with total absence of motor response to deglutition. After operation there was disappearance of vigorous contractions in the esophagus, as shown by manometry and recovery of esophageal peristalsis in another patient. We conclude that in order to improve the results of the surgical treatment of motor esophageal disorders it is essential to correctly classify the type of disorder present by means of manometry and to add a partial funduplication to ensure absence of reflux without dysphagia.     

Therapeutic approach to "downhill" esophageal varices bleeding due to superior vena cava syndrome in Behcet's disease: a case report

Background  

One of the rare presentations of superior vena cava syndrome is bleeding of "downhill" esophageal varices (DEV) and different approaches have been used to control it. This is a case report whose DEV was eradicated by band ligation for the first time.     

Morphology and function of the esophagus after sclerotherapy of esophageal varices in cirrhotic patients

To assess the effects of endoscopic variceal sclerotherapy on esophageal symptoms and function, we prospectively studied 24 consecutive cirrhotic patients (group I), 60 days after variceal eradication had been achieved. Nine cirrhotics with varices (group II) and 16 normal volunteers (group III) were control groups. After sclerotherapy, 9 patients had persistent dysphagia and two others had heartburn. Nine patients developed an esophageal stricture, without dysphagia in 2 cases. Distal esophageal scars were observed in 8 out of 9 patients with stricture and 2 out of 15 patients without stricture. The percentage of patients with abnormal peristaltic waves (abnormal pattern, non propulsive contractions, respectively) was significantly (p less than 0.01) more important in group I (83 p. 100, 96 p. 100) than in group II (22 p. 100, 22 p. 100). A very particular manometric "en plateau" waveform pattern, never seen before, was observed in 75 p. 100 of patients in group I. Relaxation of lower esophageal sphincter (LES) was significantly (p less than 0.01) lower in patients with stricture (38 p. 100 median) than in the others (71 p. 100 median). Motility disturbances were observed in the 6 +/- 3 last centimeters of the esophagus, and were unchanged 9 months later in 5 patients who had further examination. The percentage of time below pH 4 and the Kaye's score did not differ between group I (n = 17) and group III on 3 hours postprandial esophageal pH monitoring. The percentage of time at pH less than 4 was more than 9 p. 100 in 31 p. 100 of group I patients.(ABSTRACT TRUNCATED AT 250 WORDS)     

A case of fibrosing mediastinitis with obstruction of superior vena cava and downhill esophageal varices: a rare cause of upper gastrointestinal hemorrhage

Fibrosing mediastinitis (FM) is an excessive fibrotic reaction that occurs in the mediastinum and may lead to compression of mediastinal structures (especially vascular or bronchial). In the present study we describe the first case report of FM, in a patient who developed downhill esophageal varices and bleeding, which was secondary to superior vena cava obstruction.