Leiomyosarcoma of the esophagus in a patient with chagasic megaesophagus: case report and literature review.
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Leiomyosarcoma constitutes approximately 0.5% of the malignant neoplasias of the esophagus and its association with megaesophagus has not been described. We report on a case of a woman with dysphagia that was slowly progressive from the age of 19 due to chagasic megaesophagus. The woman was subjected to cardiomyotomy at the age of 49. She presented a rapid worsening of the dysphagia due to leiomyosarcoma at the age of 61, and was subjected to subtotal esophagectomy with cervical esophagogastroplasty. She developed pulmonary and hepatic metastases 14 months after surgery and died six months later. (+info)
Early experience with intrasphincteric botulinum toxin in the treatment of achalasia.
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BACKGROUND: Recent reports have suggested that intrasphincteric injection of botulinum toxin is effective and long-lasting in the treatment of achalasia. AIM: To report our experience of botulinum toxin injection in a prospective series of consecutive patients with achalasia. METHODS: Eleven consecutive patients with achalasia (eight male, mean age 55 years, range 20-87) were treated with 60 units of botulinum toxin (Dysport; Speywood Pharmaceuticals Ltd, UK) into each of four quadrants at the lower oesophageal sphincter. Patients were assessed pre-treatment and 1 month after treatment using a symptom score and oesophageal manometry. Median follow-up was 12 months (range 6-28). RESULTS: The injection procedure was simple to perform and free of adverse effects. Although treatment had a beneficial effect on dysphagia (median pre-treatment score 3 [interquartile range 3-3]; post-treatment score 2 [0-3]: P=0.03) 1 month following therapy, there was no significant improvement in chest pain or regurgitation scores. Similarly, no significant reduction in median lower oesophageal sphincter pressure was observed (29.5 mmHg [21-42] pre-treatment, 28.5 [17.5-55.5] post-treatment P=0.67). Four patients (36%) required further therapy within 3 months and the overall relapse rate was 73% (eight of 11) within 2 years. CONCLUSION: Although botulinum toxin injection was well tolerated, these results using Dysport at a dose of 240 mouse units question its efficacy as a treatment for achalasia. (+info)
Minimally invasive surgery for achalasia: an 8-year experience with 168 patients.
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BACKGROUND: Seven years ago, the authors reported on the feasibility and short-term results of minimally invasive surgical methods to treat esophageal achalasia. In this report, they describe the evolution of the surgical technique and the clinical results in a large group of patients with long follow-up. PATIENTS AND METHODS: Between January 1991 and October 1998, 168 patients (96 men, 72 women; mean age 45 years, median duration of symptoms 48 months), who fulfilled the clinical, radiographic, endoscopic, and manometric criteria for a diagnosis of achalasia, underwent esophagomyotomy by minimally invasive techniques. Forty-eight patients had marked esophageal dilatation (diameter >6.0 cm). Thirty-five patients had a left thoracoscopic myotomy, and 133 patients had a laparoscopic myotomy plus a partial fundoplication. Follow-up to October 1998 was complete in 145 patients (86%). RESULTS: Median hospital stay was 72 hours for the thoracoscopic group and 48 hours for the laparoscopic group. Eight patients required a second operation for recurrent or persistent dysphagia, and two patients required an esophagectomy. There were no deaths. Good or excellent relief of dysphagia was obtained in 90% of patients (85% after thoracoscopic and 93% after laparoscopic myotomy). Gastroesophageal reflux developed in 60% of tested patients after thoracoscopic myotomy and in 17% after laparoscopic myotomy plus fundoplication. Laparoscopic myotomy plus fundoplication corrected reflux present before surgery in five of seven patients. Patients with a dilated esophagus had excellent relief of dysphagia after laparoscopic myotomy; none required an esophagectomy. CONCLUSIONS: Minimally invasive techniques provided effective and long-lasting relief of dysphagia in patients with achalasia. The authors prefer the laparoscopic approach for three reasons: it more effectively relieved dysphagia, it was associated with a shorter hospital stay, and it was associated with less postoperative reflux. Laparoscopic Heller myotomy and partial fundoplication should be considered the primary treatment for esophageal achalasia. (+info)
Comparison of two different formulations of botulinum toxin A for the treatment of oesophageal achalasia. The Gismad Achalasia Study Group.
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BACKGROUND: Intrasphincteric injection of botulinum toxin has been reported as a safe and effective alternative treatment in oesophageal achalasia, especially in high-risk and elderly patients. AIM: : To compare two formulations of botulinum toxin in the management of achalasia. PATIENTS AND METHODS: We randomly compared the efficacy and safety of 100 U of Botox (Allergan, Irvine, USA) and 250 U of Dysport (Ipsen, Milan, Italy), injected through a sclerotherapy needle at the level of the lower oesophageal sphincter, in 78 consecutive patients with achalasia. Symptom score, oesophageal manometry and 24 h pH-metry were recorded (before and 1 month after therapy). Symptom score was also obtained 6 months after treatment. RESULTS: One month after treatment, the effects of the toxin on symptoms and oesophageal tests were similar for both formulations. Lower oesophageal sphincter pressure decreased from 31 +/- 12 to 18 +/- 5 mmHg after Botox, and from 35 +/- 9 to 18 +/- 10 after Dysport. At the end of the follow-up period (6 months), symptom score decreased from 5 +/- 1.2 to 1.2 +/- 0.8 after Botox and from 5.2 +/- 1.5 to 1.5 +/- 1 after Dysport. Moreover, the percentages of patients who failed to respond to treatment (10% and 17.5%) and who relapsed during follow-up (12% and 24%) did not differ significantly. No patient complained of reflux symptoms after treatment, although abnormal acid exposure was documented in two subjects. CONCLUSIONS: Both formulations of botulinum toxin have comparable efficacy in the treatment of oesophageal achalasia, for up to 6 months of follow-up. (+info)
Review article: pharmacological options in achalasia.
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Achalasia is a common primary oesophageal motor disorder. Treatment has been based traditionally on a surgical approach; however, there is new evidence that some medical strategies may be of benefit. The purpose of the present article was to review the current medical management of achalasia. A Medline search identified original articles and reviews published in the English-language literature between 1966 and 1998. This search has revealed that the pharmacological treatment of achalasia is limited to some subgroups of patients (for example, early stages of the disease and elderly patients), and that nitrates, nifedipine, and botulinum toxin are the best studied and most effective compounds. (+info)
Complete lower esophageal sphincter relaxation observed in some achalasia patients is functionally inadequate.
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Generally accepted manometric criteria for the diagnosis of achalasia are absent peristalsis and incomplete lower esophageal sphincter (LES) relaxation. However, in some patients with otherwise typical features of achalasia, esophageal manometry shows complete LES relaxation during swallowing. To establish whether such apparently complete LES relaxations are functionally adequate, we quantified changes in resistance to flow at the esophagogastric junction (EGJ) during wet swallowing. We studied seven achalasia patients with manometrically complete (>80%) LES relaxation, eight achalasia patients with incomplete (<40%) LES relaxation, and eight healthy volunteers. Complete LES relaxation on standard manometry (open-tip catheters) was confirmed in five of the seven achalasia patients by a Dentsleeve. Changes in EGJ resistance to flow were quantified using a pneumatic resistometer. Manometrically, the relaxation time span was significantly longer in patients with complete LES relaxation than in those with incomplete relaxation (7. 3 +/- 0.5 vs. 4.4 +/- 0.7 s; P < 0.05). The fall in EGJ resistance from basal values during swallowing was markedly reduced in both achalasia groups (21 +/- 8% in those with manometrically complete relaxation and 4 +/- 2% in those with incomplete relaxation) by comparison with healthy individuals, in whom resistance fell by 90 +/- 3% (P < 0.05 vs. both achalasia groups). The duration of EGJ resistance drop was also much shorter in achalasia with (0.7 +/- 0.2 s) and without (0.2 +/- 0.1 s) complete LES relaxation compared with healthy control values (6.6 +/- 1.2 s). Our results reveal that the apparently complete LES relaxation observed manometrically in some patients with achalasia is functionally inadequate since it is not associated with the normal profound fall in EGJ resistance to flow. (+info)
A multicentre randomised study of intrasphincteric botulinum toxin in patients with oesophageal achalasia. GISMAD Achalasia Study Group.
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BACKGROUND: Intrasphincteric injection of botulinum toxin (Botx) has been proposed as treatment for oesophageal achalasia. However, the predictors of response and optimal dose remain unclear. AIMS: To compare the effect of different doses of Botx and to identify predictors of response. PATIENTS/METHODS: A total of 118 achalasic patients were randomised to receive one of three doses of Botx in a single injection: 50 U (n=40), 100 U (n=38), and 200 U (n=40). Of those who received 100 U, responsive patients were reinjected with an identical dose after 30 days. Clinical and manometric assessments were performed at baseline, 30 days after the initial injection of botulinum toxin, and at the end of follow up (mean 12 months; range 7-24 months). RESULTS: Thirty days after the initial injection, 82% of patients were considered responders without a clear dose related effect. At the end of follow up however, relapse of symptoms was evident in 19% of patients who received two injections of 100 U compared with 47% and 43% in the 50 U and 200 U groups, respectively. Using Kaplan-Meier analysis, patients in the 100x2 U group were more likely to remain in remission at any time (p<0.04), with 68% (95% CI 59-83) still in remission at 24 months. In a multiple adjusted model, response to Botx was independently predicted by the occurrence of vigorous achalasia (odds ratio 3.3) and the 100x2 U regimen (odds ratio 3.2). CONCLUSIONS: Two injections of 100 U of Botx 30 days apart appeared to be the most effective therapeutic schedule. The presence of vigorous achalasia was the principal determinant of the response to Botx. (+info)
Upper esophageal sphincter pressure in patients with Chagas' disease and primary achalasia.
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The most important component of the upper esophageal sphincter (UES) is the cricopharyngeal muscle. During the measurement of sphincter pressure the catheter passed through the sphincter affects the pressure value. In Chagas' disease and primary achalasia there is an esophageal myenteric plexus denervation which may affect UES pressure. We measured the UES pressure of 115 patients with Chagas' disease, 28 patients with primary achalasia and 40 healthy volunteers. We used a round manometric catheter with continuous perfusion and the rapid pull-through method, performed in triplicate during apnea. Pressures were measured in four directions, and the direction with the highest pressure (anterior/posterior) and the average of the four directions were measured. The highest UES pressure in Chagas' disease patients without abnormalities upon radiologic esophageal examination (N = 63) was higher than in normal volunteers (142.8 +/- 47.4 mmHg vs 113.0 +/- 46.0 mmHg, mean +/- SD, P<0.05). There was no difference in UES pressure between patients with primary achalasia and patients with Chagas' disease and similar esophageal involvement and normal volunteers (P>0.05). There was no difference between patients with or without esophageal dilation. In the group of subjects less than 50 years of age the UES pressure of primary achalasia (N = 21) was lower than that of Chagas' disease patients with normal radiologic esophageal examination (N = 41), measured at the site with the highest pressure (109.3 +/- 31.5 mmHg vs 149.6 +/- 45.3 mmHg, P<0.01) and as the average of the four directions (64.2 +/- 17.1 mmHg vs 83.5 +/- 28.6 mmHg, P<0.05). We conclude that there is no difference in UES pressure between patients with Chagas' disease, primary achalasia and normal volunteers, except for patients with minor involvement by Chagas' disease, for whom the UES pressure at the site with the highest pressure was higher than the pressure of normal volunteers and patients with primary achalasia. (+info)