Clinical and manometric course of nonspecific esophageal motility disorders.

BACKGROUND AND AIMS: The evolution of nonspecific esophageal motility disorders remains unclear. The aim of this study was to investigate whether nonspecific esophageal motility disorders progress into specific motility disorders and whether such progression is predictable. METHODS: Seventy-six symptomatic patients (49 males, 27 females, mean age 57 ± 16 years) with newly diagnosed nonspecific esophageal motility disorders were prospectively registered and followed-up. Follow-up visits, with structured interviews and manometric re-evaluation, were recommended biannually and whenever symptoms exacerbated. RESULTS: Forty-three patients were followed for up to 4 years, symptoms worsened in 30% of patients, resolved in 26%, improved in 14% and were unchanged in 30%. Twenty-eight patients agreed to undergo manometric re-evaluation. Fifteen (53.6%) of these patients showed a progression to achalasia. The remaining patients continued to display features of nonspecific esophageal motility disorders (32%) or had normal motility (11%). The only significant association could be determined between age and progression to achalasia reaching nearly 100% in patients' ≤46 years of age. In contrast, none of the patients' ≥68 years progressed. CONCLUSION: More than half of the patients in our cohort with nonspecific esophageal motility disorders showed a transition into achalasia. Neither manometric nor clinical findings predicted the progression of nonspecific esophageal motility disorders. However, young patients were more likely to progress to achalasia.

Is the Schatzki ring a unique esophageal entity?

AIM: To study, whether the association of Schatzki rings with other esophageal disorders support one of the theories about its etiology. METHODS: From 1987 until 2007, all patients with newly diagnosed symptomatic Schatzki rings (SRs) were prospectively registered and followed. All of them underwent structured interviews with regards to clinical symptoms, as well as endoscopic and/or radiographic examinations. Endoscopic and radiographic studies determined the presence of an SR and additional morphological abnormalities. RESULTS: One hundred and sixty-seven patients (125 male, 42 female) with a mean age of 57.1 ± 14.6 years were studied. All patients complained of intermittent dysphagia for solid food and 113 (79.6%) patients had a history of food impaction. Patients experienced symptoms for a mean of 4.7 ± 5.2 years before diagnosis. Only in 23.4% of the 64 patients who had endoscopic and/or radiological examinations before their first presentation to our clinic, was the SR previously diagnosed. At presentation, the mean ring diameter was 13.9 ± 4.97 mm. One hundred and sixty-two (97%) patients showed a sliding hiatal hernia. Erosive reflux esophagitis was found in 47 (28.1%) patients. Twenty-six (15.6%) of 167 patients showed single or multiple esophageal webs; five (3.0%) patients exhibited eosinophilic esophagitis; and four (2.4%) had esophageal diverticula. Four (7%) of 57 patients undergoing esophageal manometry had non-specific esophageal motility disorders. CONCLUSION: Schatzki rings are frequently associated with additional esophageal disorders, which support the assumption of a multifactorial etiology. Despite typical symptoms, SRs might be overlooked.

Treatment and surveillance strategies in achalasia: an update.

Controversy exists with regard to the optimal treatment for achalasia and whether surveillance for early recognition of late complications is indicated. Currently, surgical myotomy and pneumatic dilation are the most effective treatments for patients with idiopathic achalasia, and a multicenter, randomized, international trial has confirmed similar efficacy of these treatments, at least in the short term. Clinical predictors of outcome, patient preferences and local expertise should be considered when making a decision on the most appropriate treatment option. Owing to a lack of long-term benefit, endoscopic botulinum toxin injection and medical therapies are reserved for patients of advanced age and those with clinically significant comorbidites. The value of new endoscopic, radiologic or surgical treatments, such as peroral endoscopic myotomy, esophageal stenting and robotic-assisted myotomy has not been fully established. Finally, long-term follow-up data in patients with achalasia support the notion that surveillance strategies might be beneficial after a disease duration of more than 10-15 years.

Long-term recurrence rates following dilation of symptomatic Schatzki rings.

BACKGROUND AND AIMS: This study investigated the long-term clinical course of patients with Schatzki rings, who were treated by single bougie dilation. Furthermore, it analyzed possible predictors for the time of recurrence. PATIENTS AND METHODS: A total of 133 patients (100 males, 33 females) with a mean age of 57 ± 14.6 years who were treated by single dilation with the use of Maloney bougies without the aid of fluoroscopy were prospectively registered and followed-up for a mean duration of 58.3 months (range 12-240 months). Duration of remission was evaluated by Kaplan-Meier estimates with regard to recurrence. Log-rank test was performed to analyze possible predictors for the time to second dilation (recurrence). RESULTS: No complications occurred and all patients were symptom-free at the first follow-up examination 4 weeks after dilation. However, later on, 73 patients required a second dilation. The estimate remission rates were 63.8% (95% CI: 55.6-72.0%) after 2 years, 44.3% (95% CI: 35.4-53.4%) after 5 years, and 39.9% (95% CI: 30.5-49.3%) after 10 years. Neither the initial morphological findings, nor age or gender determined the need for repeated dilation. Only patients treated with a large bougie diameter (≥52 F) seemed to have a tendency for a longer time until symptomatic recurrence. CONCLUSIONS: Single dilation of symptomatic Schatzki rings is a safe and effective therapy. However, more than half of the patients will need a second treatment. Recurrences are unrelated to initial morphological findings, age, or gender. Only the treatment with a large bougie diameter (≥52 F) showed a tendency for a longer time of remission.

Editorial: Cancer surveillance in achalasia: better late than never?

Patients with achalasia have an increased risk for the development of esophageal squamous cell cancer. Endoscopic surveillance in long-standing achalasia has been advocated by some, but the most recent American Society of Gastrointesinal Endoscopy guidelines regard current data as insufficient to support such an approach. In this issue of the American Journal of Gastroenterology, Leeuwenburgh and colleagues report on the results of a long-term prospective study with fixed surveillance intervals. The authors confirm earlier observations of an increased cancer risk after 10 years of symptomatic achalasia. Despite some limitations, this study and earlier data suggest that it might be worthwhile to consider endoscopic surveillance in patients with long-standing achalasia.

Electron microscopic studies of esophageal wall structures in patients with achalasia: casting more light on unresolved aspects of pathogenesis.

BACKGROUND/AIMS: Previous investigations of esophageal tissue and serum probes failed to identify a common etiologic agent predisposing to, triggering or causing achalasia. In order to further examine the detailed pathologic processes resulting in achalasia we performed electron-microscopic studies of muscle biopsies taken from the LES high pressure zone in patients undergoing surgery--either Heller myotomy or esophageal resection. METHODOLOGY: Smooth muscle biopsies with a 20 x 15-mm longitudinal segment of the myenteric plexus from the distal esophagus (lower border of the esophageal incision) in patients undergoing Heller myotomy for achalasia were taken. In patients with end-stage achalasia and mega-esophagus with esophageal resection, the complete esophageal body was available. For electron microscopy, ultrathin sections were contrasted with uranyl-acetate and plumbic citrate. The photographs were taken by a digitalized electron-microscope (ZEISS, Leo 905). RESULTS: A striking finding was the large number of mast cells in the region of the smooth muscle layers as well as in the surrounding connective tissue and also in close vicinity to the nerve cells and to the nerve fibres. The smooth muscle cells in these regions were very often stained less intensively, and they showed signs of an acute degenerative process. CONCLUSION: Our electron microscopic studies suggest that mast cells may play an important role in the secondary pathogenesis of achalasia. Esophageal retention and bacterial overgrowth with stasis esophagitis causing mucosal injury may be a mechanism of increased antigen exposure.

Achalasia: Should pneumatic dilation be the primary treatment strategy?

The best long-term treatment results for patients with idiopathic achalasia have been achieved with pneumatic dilation or surgical myotomy. A new study adds evidence that good or excellent symptomatic results can be achieved in the long term when pneumatic dilation is used as the primary treatment strategy. In cases of early treatment failure (<2 years), surgery was found to be the best rescue therapy.

Current clinical approach to achalasia.

Idiopathic achalasia is a rare primary motility disorder of the esophagus. The classical features are incomplete relaxation of a frequently hypertensive lower esophageal sphincter (LES) and a lack of peristalsis in the tubular esophagus. These motor abnormalities lead to dysphagia, stasis, regurgitation, weight loss, or secondary respiratory complications. Although major strides have been made in understanding the pathogenesis of this rare disorder, including a probable autoimmune mediated destruction of inhibitory neurons in response to an unknown insult in genetically susceptible individuals, a definite trigger has not been identified. The diagnosis of achalasia is suggested by clinical features and confirmed by further diagnostic tests, such as esophagogastroduodenoscopy (EGD), manometry or barium swallow. These studies are not only used to exclude pseudoachalasia, but also might help to categorize the disease by severity or clinical subtype. Recent advances in diagnostic methods, including high resolution manometry (HRM), might allow prediction of treatment responses. The primary treatments for achieving long-term symptom relief are surgery and endoscopic methods. Although limited high-quality data exist, it appears that laparoscopic Heller myotomy with partial fundoplication is superior to endoscopic methods in achieving long-term relief of symptoms in the majority of patients. However, the current clinical approach to achalasia will depend not only on patients' characteristics and clinical subtypes of the disease, but also on local expertise and patient preferences.

Life expectancy, complications, and causes of death in patients with achalasia: results of a 33-year follow-up investigation.

BACKGROUND: Patients with achalasia require repeated invasive therapies and may experience multiple complications. The objectives of this study were to determine the incidence of such complications, causes of death, and life expectancy in 253 patients. METHODS: Patients consisted of two groups: group A comprised 177 patients with newly diagnosed achalasia; group B consisted of 76 patients in whom the diagnosis had been established in external institutions. All patients regularly underwent structured interviews and were reinvestigated if changes in health status occurred. Survival rates were determined by Kaplan-Meier estimates and were compared with those of an average German population. Causes of death were determined from hospital records, information supplied by private physicians, and from death certificates. RESULTS: Complete follow-up was obtained in 98.9% (group A) and in 100% (group B) of all patients. The observation period for group A ranged from 2 to 33 years and for group B from 2 to 26 years (disease duration: 4-68 years). The most frequent complications were reflux esophagitis (group A: 6.2%, group B: 19.7%) and megaesophagus (group A: 6.2%, group B: 21.0%). Thirty-six patients had died during follow-up. Five of these deaths were related to achalasia. In group A, the estimated 20-year survival rates in patients with achalasia [76% (95% confidence interval (CI): 66-85%)] did not significantly differ from those in controls 80% (95% CI: 71-89%). In group B, 25-year survival rates were also similar in patients [87% (95% CI: 78-97%)] and controls [86% (95% CI: 76-97%)]. CONCLUSION: Patients with achalasia experience a significant number of complications. Causes of death and life expectancy, however, do not differ from those of the average population.

Reduction of interstitial cells of Cajal (ICC) associated with neuronal nitric oxide synthase (n-NOS) in patients with achalasia.

BACKGROUND: The etiology of achalasia is still unknown. The current theories of chronic inflammation leading to autoimmune response with destruction and loss of the inhibitory myenteric ganglion cells enlighten its pathogenesis in a limited way only. Interstitial cells of Cajal (ICC) have been shown to be involved in nitrergic neurotransmission of the lower esophageal sphincter (LES). AIM: To investigate the significance of ICC and neuronal nitric oxide synthase (n-NOS) in esophageal wall tissue of patients undergoing surgery for achalasia. METHODS: In 53 patients with a median age of 45 (6-78) yr undergoing surgery for achalasia, the immunoreactivity of ICC (CD117/c-kit) and n-NOS was assessed. In 42 patients, biopsies were taken from the LES high-pressure zone during Heller myotomy, whereas in 11 patients with end-stage achalasia and a decompensated megaesophagus, the complete esophagus was resected. A semiquantitative analysis was carried out and ICC and n-NOS impairments were classified into four grades. Staining intensity was correlated with preoperative clinical, radiologic, and manometric findings and with long-term postoperative Eckardt score. RESULTS: Grade III/IV ICC reduction (severe reduction to complete loss) was seen in 59.5% of all biopsy specimens of the LES high-pressure zone. Patients with grade III/IV ICC reduction had a significantly longer duration of achalasia symptoms (3 [0-43] yr) than patients with minor to marked (grade I/II) impairment (1 [0-16] yr, P= 0.028). A majority (72.5%) of tissue samples revealed severe reduction to complete loss of n-NOS immunoreactivity. The preoperative Eckardt score was statistically significantly different between patients with grade I/II and those with grade III/IV n-NOS reductions (P= 0.031). CD117 (c-kit) positivity was statistically significantly correlated with n-NOS staining intensity (correlation coefficient r= 0.781, P < 0.0001). CONCLUSION: The present results suggest that in the pathogenesis of achalasia, especially in the development of the LES high-pressure zone, depletion of ICC networks and potential changes in the electrical activity of smooth muscle cells may play a crucial role. The reduction in CD117-positive ICC in a few patients also seemed to be of relevance, even if the cells of Auerbach's plexus were unscathed. The associated reduced NOS release might underlie the profound ICC impairment and could possibly be responsible for the lack of LES relaxation, because of missing inhibitory neurotransmission. It is unclear, however, whether the ICC loss is primarily caused by the accelerated attrition of mature cells or their impaired regeneration.

Persistent and recurrent achalasia after Heller myotomy: analysis of different patterns and long-term results of reoperation.

HYPOTHESIS: Two groups of patients with inadequate therapeutic success after surgical treatment for achalasia can be identified, patients with type 1 recurrence (early recurrence after technical failure of myotomy or a scarring process requiring remyotomy) and patients with type 2 recurrence (late recurrence with irreversible progression of the disease and development of megaesophagus requiring esophagectomy). DESIGN: Prospective study. SETTING: University-based tertiary care center. PATIENTS: One hundred sixty-three patients undergoing surgery for achalasia during 20.3 years. INTERVENTIONS: Conventional remyotomy for type 1 recurrence (group 1) and esophagectomy (transhiatal or transthoracic) for type 2 recurrence (group 2). MAIN OUTCOME MEASURES: Long-term results after reoperation, including Eckardt score, body mass index, reflux esophagitis, manometric lower esophageal sphincter resting pressure, and radiologic maximum diameter of the esophageal body and minimum diameter of the cardia. RESULTS: After reoperation, a postoperative Eckardt score of 1 (corresponding to clinical stages 1 to 2) was calculated in 92.3% of group 1 patients and in 80.0% of group 2 patients. In group 1 patients, the maximum diameter of the esophagus decreased to a median value of 25 mm (range, 20-60 mm), while the minimum diameter of the cardiac sphincter increased to a median value of 10.0 mm (range, 5.0-12.0 mm). After surgery, the resting pressure of the lower esophageal sphincter was reduced to a median value of 8.3 mm Hg (range, 4.0-10.0 mm Hg). CONCLUSIONS: Reoperation for achalasia yields good long-term symptomatic outcomes, with relief of dysphagia. Subjective, radiographic, and manometric findings after remyotomy duplicate the good results reported for primary open myotomy.

Long-term results of conventional myotomy in patients with achalasia: a prospective 20-year analysis.

Myotomy has proved to be an efficient primary therapy in patients with achalasia, especially in younger patients (<40 years of age). The results of laparoscopic myotomy cannot be finally assessed, on account of the shorter postoperative follow-up. Thus, there are considerable data regarding intermediate-term outcomes after laparoscopic myotomy. The aim of our study was a 20-year analysis of the conventional cardiomyotomy as the underlying basis assessing the results of minimal-invasive surgery. Within 20 years (September 1985 through September 2005), 161 operations for achalasia were performed in our clinic. Enrolled in this study were 108 patients with a conventional, transabdominal myotomy in combination with an anterior semifundoplication (Dor procedure) and a minimal follow-up of 6 months. All patients were prospectively followed and, in addition to radiologic and manometric examinations of the esophagus, the patients were asked for their clinical symptoms by structured interviews in 2-year intervals. The median age at the time of surgery was 44.5 (range, 14-78) years, and 72.2% of the patients were males. The median length of the preoperative symptoms was 3 years (3 months to 50 years), and the postoperative follow-up was 55 (range, 6-206) months. In 70 (64.8%) patients, a pneumatic dilation had been performed. The preoperative Eckardt score of 6 (range, 2-12) could be reduced to 1 (range, 0-4) after myotomy (P<0.0001). Consequently, with 97.2% of all patients, a good-to-excellent result was achieved in the long-term follow-up, corresponding to a clinical stage I-II. Postoperatively, 69 patients (63.9%) gained weight. The radiologically measured maximum diameter of the esophagus decreased from preoperatively 45 (range, 20-75) mm to postoperatively 30 (range, 20-60) mm, while the minimum diameter of the cardia increased from 3.4 (range, 1-10) mm to 10 (range, 5-15) mm. The resting pressure of the lower esophageal sphincter could be reduced from 28.4 (range, 9.4-56.0) mm Hg to 8.6 (range, 3.0-22.5) mm Hg. Conventional myotomy leads in the long run with high efficiency to an improvement of the symptoms evident in achalasia. These results may be regarded as the basis for assessment of the minimal-invasive procedure.

Spectrum of histopathologic findings in patients with achalasia reflects different etiologies.

BACKGROUND: The etiology of achalasia is still unknown. The aim of the present study was to elucidate its underlying pathologies and their chronology by investigation of esophageal specimens in patients undergoing surgery (esophageal resection or myotomy) for achalasia. METHODS: In 17 patients with achalasia, histopathologic examinations of the esophageal wall focussing on the myenteric plexus were performed. Preoperative diagnosis was based on clinical evaluation, esophagogastroscopy, barium esophagogram in all, and esophageal manometry in eight patients. The median age at the time of surgery was 54 years (range: 14-78 years). In eight cases, the complete esophageal, body and in nine cases a smooth muscle biopsy including parts of the myenteric plexus from the distal part of the esophagus (high pressure zone) was available. The tissue specimens were fixed in formalin and embedded in paraffin. The staining procedures were hematoxylin and eosin (HE), Elastica van Gieson (EvG), and periodic acid-Schiff (PAS) reaction. Immunohistochemical examinations were performed with antibodies against B and T lymphocytes, neurofilament, protein gene-related product (PGP 9.5), S-100 protein, myosin, desmin, smooth muscle actin and substance P. RESULTS: In 13 of 17 patients, a significant reduction of the number of intramural ganglion cells was present. Common findings were a severe fibrosis of the smooth muscle layer (10/17) and obvious myopathic changes of the smooth muscle cells (5/17). Staining for B and T lymphocytes found signs of inflammation in mucosal and muscular areas. Three patients exhibited a marked invasion of eosinophilic granulocytes of the muscularis propria (eosinophilia). Esophageal carcinoma had developed in three patients (squamous cell carcinoma in two and carcinoma in situ in another patient with Barrett's esophagus and high-grade dysplasia). Severe inflammatory reactions (neural, eosinophilic and mucosal) dominated in patients with a longstanding history of achalasia (>10 years) as well as a marked endomysial fibrosis. CONCLUSIONS: The histopathological investigations of the esophageal wall in 17 patients undergoing esophageal resection or myotomy for achalasia suggest that the reduction of intramural ganglion cells might be a secondary change, probably due to inflammation triggered by autoimmune mechanisms or a chronic degenerative process of the central and/or peripheral part of the vagal nerve. The primary lesion could also be a severe myopathy of the smooth muscle cells.

Pseudoachalasia: a case series and analysis of the literature.

OBJECTIVE: Pseudoachalasia frequently cannot be distinguished from idiopathic achalasia by manometry, radiologic examination or endoscopy. Mechanisms proposed to explain the clinical features of pseudoachalasia include a circumferential mechanical obstruction of the distal esophagus or a malignant infiltration of inhibitory neurons within the myenteric plexus. MATERIAL AND METHODS: Between January 1980 and December 2002, the clinical features of 5 patients with pseudoachalasia and 174 patients with primary achalasia, diagnosed in a single center, were compared. A literature analysis of the etiology of pseudoachalasia for the time period 1968 to December 2002 was performed. The search concentrated on the databases and online catalogues PubMed, Web of Science, Cochrane Library and Current Contents Connect. RESULTS: In our case series, patients with pseudoachalasia reported a shorter duration of symptoms and tended to be older than patients with primary achalasia. Conventional manometry, endoscopy and radiologic examination of the esophagus proved to be of little value in distinguishing between the diseases. In the majority of cases only surgical exploration revealed the underlying cause. A coincidence of primary achalasia and disorders of the gastroesophageal junction was excluded by showing return of peristalsis following treatment. The analysis of the literature showed a total of 264 cases of pseudoachalasia in 122 publications. Most cases of were due to malignant disease (53.9% primary and 14.9% secondary malignancy), followed by benign lesions (12.6%) and sequelae of surgical procedures at the distal esophagus or proximal stomach (11.9%). In rare instances, the disease was an expression of a paraneoplastic process due to distant neuronal involvement rather than to local invasion with destruction of the myenteric plexus (2.6%). CONCLUSIONS: The diagnosis of pseudoachalasia is difficult to establish by conventional diagnostic measures. The main distinguishing feature of secondary versus primary achalasia is the complete reversal of pathologic motor phenomena following successful therapy of the underlying disorder.

Effects of pneumatic dilation and myotomy on esophageal function and morphology in patients with achalasia.

Only two treatment modalities-pneumatic dilation and Heller myotomy-promise long-term relief from dysphagia and regurgitation in patients with achalasia. The objective of this study was to determine whether both options differ in their effects on esophageal function, morphology, and improvement in symptoms. Eighty-nine patients diagnosed with achalasia between January 1980 and December 2002 at a single center were enrolled in this study. Sixty-four patients underwent pneumatic dilation and 25 Heller myotomy in combination with an anterior semifundoplication (Dor procedure). Clinical evaluation (Eckardt-Score), esophageal manometry, and barium swallow were performed before and within 6 months after treatment. Our data shows that Heller myotomy reduces the LES resting pressure more markedly (7.9 [3.7-16.9] mm Hg) than pneumatic dilation (14.5 [2.7-36.0] mm Hg) (P < 0.0001) with similar pressures at diagnosis in both groups. Morphologic changes, assessed by the diameter of the esophageal corpus, were also more pronounced after surgical therapy (P > 0.05). Both options will lead to an immediate and significant improvement in symptoms, although the two treatment modalities did not differ in their subjective results. As only objective findings, such as those obtained by manometry and the timed barium swallow, have proven relevance for the assessment of long-term results, surgical therapy is the superior and more effective treatment option in patients with achalasia.

Heller myotomy for failed pneumatic dilation in achalasia: how effective is it?

OBJECTIVES: This long-term prospective study describes the effect of myotomy in patients who fail to respond to repeated pneumatic dilations and compares their clinical course with that of patients responding to dilation therapy. METHODS: Nineteen consecutive patients who had never reached a clinical remission after repeated pneumatic dilation underwent myotomy. Their clinical course was compared with that of patients who had reached a clinical remission after a single (n = 34) or multiple (n = 14) pneumatic dilation(s). Symptoms were graded with a previously described symptom score ranging from 0 to 12. Remission was defined as a score of 3 or less persisting for at least 6 months. Duration of remission was summarized using Kaplan Meier survival curves. Association between baseline factors and the need for surgery was evaluated using logistic regression. RESULTS: Complete follow-up was obtained for 98.5% of the patients. The median duration of follow-up was similar in patients treated by myotomy (10.0 years), in patients reaching a clinical remission after a single dilation (10.6 years), but differed in patients undergoing repeated dilations (6.9 years). The 10-year remission rate was 77% (95% CI 53-100%) in patients undergoing myotomy, 72% (95% CI: 56-87%) in patients "successfully" treated with a single pneumatic dilation and 45% (95% CI: 16-73%) in patients undergoing several dilations. Among all baseline factors investigated, young age was associated with an increased need of surgery. CONCLUSIONS: Myotomy is an effective treatment modality in patients with achalasia who have failed to respond to pneumatic dilation. Young patients may benefit from primary surgical therapy.

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.

Complement components and terminal complement complex in oesophageal smooth muscle of patients with achalasia.

This study investigates whether patients with achalasia exhibit autoimmune reactions with subsequent complement activation within oesophageal smooth muscle, vessels and neurones. Oesophageal muscular biopsies from 8 patients undergoing surgery for achalasia and from 6 patients operated for oesophageal cancer were investigated by immunofluorescence for the presence of the complement components C1q, C4, C3c, C3d, C9 and the C9 neoantigen of the terminal C5b-C9 complement complex. Tissues were also investigated for the expression of immunoglobulins (G,A,M) and of the antigens of rubella and varicella zoster viruses. In addition, sera of both patient groups were tested for the presence of autoantibodies against Auerbach's plexus. The terminal complement complex C5b-C9 was found within muscle cells from all patients with achalasia but in only one specimen from a patient with cancer. Two patients with achalasia also exhibited the terminal complement complex as well as IgM within ganglion cells. Muscle cells stained positive for the complement component C9 in all five patients with achalasia in whom this test was performed but in none of the control tissues. In addition, sera from four patients with achalasia contained antibodies against Auerbach's plexus. Studies for the complement components C1q, C4, C3c and for antigens of rubella and varicella zoster viruses revealed negative results in all patients and controls. The results of this study suggest that a complement activation is involved in the autoimmune pathogenesis of achalasia. However, the triggering mechanism of this phenomenon remains to be determined.