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Achalasia

Achalasia or acalasia is a failure of a ring of muscle (as a sphincter) to relax (completely). It refers most commonly to esophageal achalasia, which is a neuromuscular disorder of the esophagus characterized by the reduced ability to move food down the esophagus (peristalsis). In addition, the inability of the cardia ( also called lower esophageal sphincter)- to relax in response to swallowing (there is increased LES pressure- spasms). more...

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Synonyms are achalasia cardiae, cardiospasm, dyssynergia esophagus, esophageal aperistalsis.

Signs and symptoms

  • Dysphagia
  • Regurgitation of undigested food
  • Heartburn
  • Chest pains : increases after eating and may radiate to the back, neck, and arms.
  • Weight loss
  • Coughing, especially at night or when lying down

Diagnosis

Due to the similarity of symptoms, achalasia can be misdiagnosed as other disorders, such as gastroesophageal reflux disease (GERD) and Chagas disease

  • X-ray with a barium swallow or esophagography . Shows narrowing at the level of the gastroesophageal junction ("bird beak"), and various degrees of megaesophagus (esophageal dilation).
  • Endoscopy, which provides a view from within the esophagus.
  • Manometry, the key test for establishing the diagnosis. Measures the pressure induced in different parts of the esophagus and stomach during the act of swallowing.
  • CT scan, which provides further visual evidence.

Complications

  1. Gastroesophageal reflux disease-GERD or heartburn.
  2. Barrett's esophagus or Barrett's mucosa: in 10% of patients.
  3. There are two kinds of esophageal cancer: squamous cell carcinoma and adenocarcinoma. There are predisposing conditions that, if present for a long time, may lead to esophageal adenocarcinoma, like achalasia (in up to 5% of cases, Barrett's esophagus leads to esophageal adenocarcinoma).

Treatment and new expectations

  • Balloon (pneumatic) dilation. The muscle fibers will be stretched. Gastroesophageal reflux (GERD) occurs after dilatation in 25% to 35% of patients. Is a risk to later Heller myotomy.
  • Medication:
    • Intra-sphincteric injection of botulinum toxin (or botox), to paralyze cardia and prevent spasms. It is transitory and symptoms will return in the majority of patients within a year.
    • Drugs that reduce LES pressure such as nifedipine and nitroglycerin may be useful.
  • Heller myotomy laparoscopic surgery.
  • Transplant and artificial cardia.

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Achalasia of the oesophagus in elderly patients responds poorly to conservative therapy
From Age and Ageing, 7/1/94 by S. Ghosh

Summary

In a retrospective study the symptoms and results of pneumatic dilatation in achalasia of the oesophagus were analysed in 11 patients (six women) aged 65 years and above (group 1) and compared with 19 younger patients (two women, group 2) over a 48-month period. Weight loss occurred in 91% of group 1 patients and 53% of group 2 patients. Pulmonary complications were commoner in group 1 than in group 2. All patients in group 1 and 84% of patients in group 2 complained of regurgitation. Lower oesophageal sphincter pressures were similar in the groups but the median diameter of the oesophageal body was greater in group 1 patients. The duration of dysphagia before presentation was longer in group 1 (median 5 years; range 1-9 years) compared with group 2 (median 2 years; range 0.5-4 years). None of the six elderly patients receiving pharmacological treatment with nifedipine or nitrates improved while five of the six younger patients treated with medications reported transient improvement. In group 1 only 50% of the pneumatic dilatations resulted in improvement and the median duration of improvement was 12 weeks. These results were significantly worse than those in group 2 where 90% of the dilatations resulted in improvement which lasted for a median duration of 52 weeks. Long-term results were satisfactory in six of the 11 elderly patients and 16 of the 19 younger patients.

Introduction

It is increasingly being recognized that achalasia of the oesophagus can present at any age including later life (1)(2). Previous studies have shown that younger patients respond less well to pneumatic dilatation than older patients and need more frequent dilatations (3)(4) and a recent study has shown that the response is particularly poor in patients under 18 years of age (5). No study has specifically addressed the question of response to pneumatic dilatation in elderly patients. It is important to have information concerning the association of age with response rates to pneumatic dilatation so that management policies in achalasia may be formulated.

We present our experience of achalasia and its response to balloon dilatation in elderly patients.

Methods

This is a retrospective analysis of 30 consecutive achalasia patients treated by balloon dilatation over the period March 1988 to February 1992. Eleven patients (six women) were aged 65 years or over, range 65-97 years (group 1), while 19 patients (two women) were aged less than 65 years, range 25-64 years (group 2). The median duration of follow-up from the first pneumatic dilatation was 104 weeks (range 20-184 weeks).

Swallowing was graded as: (a) no complaints; (b) minor complaints such as intermittent sticking of food or slowness of swallowing; (c) significant difficulties such as weight loss of over 2 kg during the preceding 6 months, change to a semisolid or liquidized diet or pulmonary complications including nocturnal cough or documented aspiration pneumonia; (d) complete dysphagia with inability to swallow either solids or liquids. Grades 1 and 2 were classified as satisfactory swallowing and grades 3 and 4 as unsatisfactory swallowing.

The diagnosis of achalasia was made on the basis of a compatible history obtained by an experienced gastroenterologist, a typical barium swallow and exclusion of mechanical obstruction or mucosal disease by endoscopy. Oesophageal manometry was performed using Arndorfer triple lumen catheter, a continuous perfused system and a station pullthrough technique in 27 patients. Nonrelaxation of the lower oesophageal sphincter and aperistalsis of the oesophageal body were found in all patients. The three remaining patients were all from group 1 and manometry was not performed in these patients because of extreme frailty or intolerance of the manometric procedure.

Twelve patients had received pharmacological therapy with nifedipine (10-20 mg), crushed and swallowed before each meal; five of them had also received a trial with isosorbide dinitrate (5 mg) sublingually before meals. None responded well and all 30 patients eventually had to be treated with pneumatic balloon dilatation. The dilatations were carried out using either a Rigiflex (Microvasive) achalasia balloon dilator (diameter 35 mm) or a Witzel endoscopic pneumatic dilator. The Rigiflex achalasia dilator was used under fluoroscopic control and inflated to 7-10 psi for 2 min (6). The Witzel balloon dilator was carried 'piggyback' on a gastroscope (Olympus GIF XQ10 or 20) and positioned under endoscopic guidance. It was inflated to a pressure of 200 mmHg and maintained for 2 min followed by a further inflation to 250 mmHg for another 2 min(7). Fluoroscopy was available for all Witzel dilatations though it is not essential for the performance of this procedure. All procedures were carried out by experienced operators (K.R.P. 32 procedures, S.G. 8 procedures, others 13 procedures).

Non-parametric tests of significance were carried out using Wilcoxon's rank tests, [[chi].sup.2] test with Yates' correction and Fisher's exact test.

Results

Symptoms (Table I): All patients presented with grade 3 or 4 dysphagia. The mean dysphagia scores were similar in the two groups. Four group 1 patients had grade 4 dysphagia while only one patient from group 2 presented with complete dysphagia. In group 1 patients the duration of dysphagia before presentation was significantly longer than that in group 2 patients.

Table I. Presenting symptoms

(*)p<0.05.

Weight loss of 2 kg or more over the preceding 6 months was common and occurred in 91% of group 1 patients and 53% of group 2 patients. Regurgitation and chest pain occurred in 11 and eight patients respectively in group 1 and in 16 and nine patients respectively in group 2. The chest pain was generally mild and the frequency of occurrence of these symptoms did not differ between the two groups of patients. Pulmonary complications (nocturnal cough, documented aspiration pneumonia) occurred more commonly in group 1 than group 2.

Manometric findings: The median lower oesophageal sphincter pressure was 44 (range 30-62) mmHg in group 1 and 40 (range 34-68) mmHg in group 2.

Radiological findings: The maximum diameter of the oesophageal body in six barium swallows obtained from group 1 patients ranged from 72 to 95 mm (median 84 mm). In eight group 2 patients the maximum diameter of the oesophageal body ranged from 48 to 78 mm (median 60 mm) and the difference was significant (p<0.05).

Response to therapy: Six group 1 patients had received pharmacological treatment with nifedipine (three of them also received nitrates) but none responded. In contrast, six younger patients had received pharmacological treatment with nifedipine (two of them had received nitrates) and five responded by at least one grade on the dysphagia score. The imporvement was shortlived and pneumatic dilatation was needed in all patients.

Twenty-two pneumatic dilatations (11 Rigiflex, 11 Witzel) were carried out in 11 group 1 patients, while 31 pneumatic dilatations (16 Rigiflex, 15 Witzel) were carried out in 19 group 2 patients (Table II). The number of dilatations per patient was similar in the two groups. Significantly fewer dilatations in group 1 resulted in satisfactory swallowing 1 month after dilatation compared with group 2. The duration of improvement was also significantly shorter in group 1 patients than in group 2.

Table II. Response to pneumatic dilatation

(**)p<0.01.

Long-term swallowing was satisfactory (grades 1 and 2) in six group 1 patients. In contrast, long-term swallowing was satisfactory in 16 group 2 patients. Significantly more patients in the younger group had no complaints about swallowing at the end of their follow-up period (p<0.01) and the minimum period of follow-up after the last dilatation was 20 weeks. All three group 2 patients who had unsatisfactory swallowing underwent Heller's cardiomyotomy while only one of the five elderly patients with unsatisfactory swallowing had a Heller's cardiomyotomy.

Complications: One oesophageal perforation occurred in this series in an 81-year-old lady and this settled with conservative therapy. Her swallowing remained unsatisfactory and she had a percutaneous endoscopic gastrostomy to maintain nutrition. Gastro-oesophageal reflux (defined as heartburn necessitating [H.sub.2]-receptor antagonist or omeprazole therapy) occurred in six group 1 and seven group 2 patients.

Discussion

Thirty-seven per cent of this unselected group of achalasia patients were aged 65 years or over at presentation. This is typical of recent studies which show that achalasia presents throughout adulthood without increase in incidence in middle life(1)(2)(8). Elderly patients often have a longer duration of symptoms than younger patients. A long duration of symptoms before commencement of treatment is evident in our study and this was also described by Fellows et al. from Nottingham(3) who reported a mean duration of symptoms prior to initial treatment of 54.3 months. Fourteen patients in the Nottingham series were over 70 years old but the mean duration of symptoms in them was not specifically mentioned. In this series, as in the Nottingham study, weight loss was commoner in older patients. Pulmonary symptoms were common in our elderly patients although this has not been highlighted in previous studies.

None of our elderly patients responded to pharmacological therapy. Some of the younger group did respond to drug therapy albeit temporarily and incompletely. The poor response in the elderly patients is disappointing because this is the group in which conservative approaches may be particularly desirable.

Our results show that fewer patients aged 65 years and above respond well to pneumatic dilatation than do their younger counterparts. Fewer of the elderly patients were swallowing well 1 month after dilatation and the duration of improvement was shorter. In the long term five out of 11 patients (45%) were left with unsatisfactory swallowing. Repeated pneumatic dilatations have been shown to be safe and effective(5) and whether more frequent dilatations in the elderly patients will produce better results is an interesting question which remains to be answered. Others have raised concerns about the effectiveness and safety of repeated pneumatic dilatations(9).

Our results differ from published data. Fellows et al. (3) reported that dilatations were required more often in patients aged under 45 years than in those aged 45 years or more. Duration of improvement after pneumatic dilatation and the long-term results were not mentioned in this study. Vantrappen et al.(4) also reported that patients over the age of 45 years did better than younger ones after pneumatic dilatation. Their series had ten septuagenarians and three octogenarians but these were not analysed separately. A recent study from Germany (5) reported that the least satisfactory treatment results occured in adolescents (<18 years) after pneumatic dilatation. Our series did not contain any patient in this age group. In the German study, patients in the younger age group (18-40 years) fared better than the adolescents but worse than patients older than 40 years. The proportion of patients above 65 years in their older group is not known.

There may be two reasons for the different conclusions of our study. First, Vantrappen et al.(4) have shown that if the oesophageal diameter was more than 80 mm the results of pneumatic dilatation are poor. The median oesophageal diameter in our elderly patients was 84 mm. Second and not independently, the duration of symptoms before treatment was significantly longer in our elderly patients. The longer duration of symptoms before treatment may be the underlying factor behind the greater diameter of the oesphagus before treatment. Thus, the poor response in our elderly group may be due to their presenting at a particularly late stage rather than to any intrinsic problem relating to the aged oesophagus.

There is an understandable reluctance to subject frail elderly patients to cardiomyotomy. However, cardiomyotomy is a remarkably safe operation(10) and a laparoscopic approach to it is an additional option; the outcome in elderly patients is not yet well defined.

In conclusion, in elderly subjects achalasia of the oesophagus is not uncommon, tends to present late, and often responds poorly to medical therapy and pneumatic dilatation.

Acknowledgement

This study was presented at the British Society of Gastroenterology Meeting in Warwick, in September 1992 and published in abstract form (Gut 1992;33(suppl 2):S30).

References

(1.)Howard PJ, Maher L, Pryde A, Cameron EWJ, Heading RC. Five year prospective study of the incidence, clinical features, and diagnosis of achalasia in Edinburgh. Gut 1992;33:1011-5.

(2.)Mayberry JF, Atkinson M. Studies of the incidence and prevalence of achalasia in the Nottingham area. Q J Med 1985;56:451-6.

(3.)Fellows IW, Ogilvie AL, Atkinson M. Pneumatic dilatation in achalasia. Gut 1983;24:1020-3.

(4.)Vantrappen G, Hellemans J, Deloof W, Valembois P, Vandenbroucke J. Treatment of achalasia with pneumatic dilatations. Gut 1971;12:268-75.

(5.)Eckardt V, Aignherr C, Bernhard G. Predictors of outcome in patients with achalasia treated by pneumatic dilatation. Gastroenterology 1992;103:1732-8.

(6.)Cox J, Buckton GK, Bennett JR. Balloon dilatation in achalasia: a new dilator. Gut 1986;27:986.

(7.)Elta GH, Nostrant TT, Wilson JAP. Treatment of achalasia with the Witzel pneumatic dilator. Gastrointest Endosc 1987;33:101-3.

(8.)Reynolds JC, Parkman HP. Achalasia. Gastro Clin North Am 1989;2:223-55.

(9.)Okike N, Payne S, Neufeld D, Bernartz PE, Pairolero PC, Sanderson DR. Esophagomyotomy versus forceful dilatation for achalasia of the esophagus: results in 899 patients. Ann Thorac Surg 1979;28:119-23.

(10.)Richter JE. Surgery or pneumatic dilatation for achalasia: a head-to-head comparison. Now are all the questions answered? Gastroenterology 1989;97:1340-1.

Authors' addresses

S. Ghosh, K. R. Palmer(*)

Gastrointestinal Unit, Western General Hospital, Crewe Road, Edinburgh EH4 2XU

R. C. Heading

Department of Medicine, Royal Infirmary of Edinburgh, Edinburgh

(*)Address correspondence to Dr K. R. Palmer

Received in revised form 13 November 1993

COPYRIGHT 1994 Oxford University Press
COPYRIGHT 2004 Gale Group

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