The management of achalasia and other motility disorders of the oesophagus

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The management of achalasia and other motility disorders of the oesophagus

Introduction

Most patients who turn out to have oesophageal motor disorders undergo endoscopy and/or contrast radiology to make sure that their dysphagia is not due to cancer or their chest pain to gastro-oesophageal reflux disease. While these tests can provide the diagnosis, they are often normal, leading to specific investigation of oesophageal motor function. Modified barium swallows to look at solid bolus transit using bread or marshmallow can provide added qualitative information. Radionuclide transit does much the same in a semiquantitative way, but the mainstay of specialised investigation is oesophageal manometry.

For many years oesophageal manometry was done using water-perfused systems that were difficult to set up and with many technical constraints. These were gradually replaced by solid-state pressure transducers.With further miniaturisation and developments in computer software, thin catheters containing multiple pressure recording transducers (high-resolution manometry) have become widely available, leading to novel ways of displaying pressure information as isobaric contour plots using colour gradations to indicate different pressures (high-resolution oesophageal pressure topography). The latter systems have disclosed considerable new information about oesophageal motor disturbances, resulting in new criteria (Chicago classification, Table 16.1) to define these disorders.1 Further details of manometry techniques are described in Chapter 12 and manometry of a normal swallow is shown in Fig. 12.1.

Table 16.1

The Chicago classification of oesophageal motility

Diagnosis Criteria
Achalasia type I Classic achalasia: mean IRP > upper limit of normal, 100% failed peristalsis
Achalasia type II Achalasia with oesophageal compression: mean IRP > upper limit of normal, no normal peristalsis, pan-oesophageal pressurisation with ≥ 20% of swallows
Achalasia type III Mean IRP > upper limit of normal, no normal peristalsis, preserved fragments of distal peristalsis or premature (spastic) contractions with ≥ 20% of swallows
Oesophagogastric junction outflow obstruction Mean IRP > upper limit of normal, some instances of intact peristalsis or weak peristalsis with small breaks such that the criteria for achalasia are not met
Distal oesophageal spasm Normal mean IRP, ≥ 20% premature contractions
Hypercontractile oesophagus At least one swallow DCI > 8000 mmHg • s • cm with single peaked or multipeaked contraction
Absent peristalsis Normal mean IRP, 100% of swallows with failed peristalsis
Weak peristalsis with large peristaltic defects Mean IRP < 15 mmHg and > 20% swallows with large breaks in the 20 mmHg isobaric contour (> 5 cm in length)
Weak peristalsis with small peristaltic defects Mean IRP < 15 mmHg and > 30% swallows with small breaks in the 20 mmHg isobaric contour (2–5 cm in length)
Frequent failed peristalsis > 30%, but < 100% of swallows with failed peristalsis
Rapid contractions with normal Rapid contraction with ≥ 20% of swallows, DL > 4.5 s
Hypertensive peristalsis (nutcracker oesophagus) Mean DCI > 5000 mmHg • s • cm, but not meeting criteria for hypercontractile oesophagus

DCI, distal contractile integral; DL, distal latency; IRP, integrated relaxation pressure.

Achalasia

Background

The term ‘achalasia’ comes from Greek, meaning ‘failure to relax’. It was first used by Sir Arthur Hurst early in the 20th century, although the clinical features were first described in 1697 by Thomas Willis. It is conventionally defined by the absence of peristalsis (this does not mean the absence of contractions) in association with a lower oesophageal sphincter (LOS) that fails to relax completely. High-resolution pressure topography recognises this classic type (type I) but also considers two other types of achalasia, where there can be either pan-oesophageal pressurisation to more than 30 mmHg with at least 20% of swallows (type II) or preserved fragments of distal peristaltic activity or premature (spastic) contractions with at least 20% of swallows (type III).2 These last two types probably represent the entity usually referrred to as ‘vigorous achalasia’ when seen on conventional manometry. Primary or idiopathic achalasia needs to be considered separately from secondary achalasia. While symptoms may be similar, the presence of a specific aetiology can influence management. These secondary causes are discussed later in this chapter.

Primary achalasia

This is an uncommon condition with an incidence in the Western world that is probably less than 1 case per 100  000 people per year.3 It is due to progressive loss of ganglion cells in the myenteric plexus of unknown cause. The neural loss is somewhat selective as there is particularly severe loss of inhibitory nitrergic neurotransmission.4,5 This process is often accompanied by an inflammatory infiltrate that has led to many theories regarding aetiology. While there is circumstantial evidence of viral exposure and autoimmune phenomena, neither provides a satisfactory explanation for all patients.5

Clinical features

The disease is most common in middle life, but can occur at any age. It typically presents with dysphagia and characteristically this affects fluids as well as solids. Symptom severity varies from day to day and patients often develop tricks to assist oesophageal emptying, such as Valsalva manoeuvres or air swallowing. Many admit to having been ‘slow eaters’ for many years. In patients who have remained untreated for many years, regurgitation is frequent and there may be overspill into the trachea, especially at night. In the early stages, achalasia may present with retrosternal discomfort and this may lead to a mistaken diagnosis of gastro-oesophageal reflux disease (GORD). Chest pain is common in achalasia and particularly so in those individuals said to have ‘vigorous achalasia’.

There are few physical signs that point specifically to any underlying motility disorder, including achalasia. There are two important areas of physical examination that should be carefully evaluated as positive findings will play an important part in management. A careful examination of the respiratory system is essential. Recurrent chest infections due to episodes of aspiration from an oesophagus that is unable to clear itself may lead to acute and chronic signs as pulmonary performance deteriorates. It is not rare for these patients to be labelled as asthmatic. The other important area is to make a careful assessment of the patient’s nutritional state. Insidious nutritional failure is easily missed in patients with a long history, although it is rare to see this in achalasia, where regular filling of the oesophagus with food and an upright posture eventually create a hydrostatic pressure that will overcome the LOS, allowing the oesophagus to partially empty.

Investigations

Most patients with dysphagia are offered endoscopy as their first investigation. While achalasia may be suspected at endoscopy by finding a tight cardia and food residue in the oesophagus, early or vigorous achalasia is easily missed as the oesophagus is not dilated and still contracts.

Barium radiology may show hold-up in the distal oesophagus, dilatation of the oesophageal body, peristaltic dysfunction and a tapering stricture in the distal oesophagus, often described as a ‘bird’s beak’. The gastric gas bubble is usually absent, because most patients cannot swallow air through their non-relaxing LOS. It should, however, be emphasised that, like endoscopy, these typical features of well-developed achalasia are often absent and radiology is frequently passed as normal.

A firm diagnosis can only be made by oesophageal manometry. High-resolution techniques suggest that conventional manometry, however, only diagnoses about a quarter of patients with achalasia on the basis of the classic features of a hypertensive lower oesophageal sphincter that does not relax completely on swallowing, aperistalsis of the oesophageal body and a raised resting pressure in the oesophagus (Fig. 16.1). The other two variants can be separated by the Chicago classification and this may be clinically relevant. Limited evidence suggests that patients with type II achalasia with pan-oesophageal pressurisation do respond well to treatment, but this seems not to be the case for type III.2

Treatment

Most patients with achalasia respond well to treatment. There is no reliable drug therapy. Patients can be treated by endoscopic botulinum toxin injection into the LOS, but the two main methods are forceful (pneumatic) dilatation of the cardia and operative cardiomyotomy. Very rarely, patients require oesophagectomy.

Pneumatic dilatation

This involves stretching the cardia with a balloon to disrupt the muscle and render it less competent. The treatment was first described by Plummer in 1908. Many varieties of balloon have been described, but nowadays plastic balloons with a precisely controlled external diameter are used. If the pressure in the balloon is too high the balloon is designed to split along its length rather than expand further. Balloons of 30–40 mm diameter are available and are inserted over a guidewire.

Perforation is the major complication. With a 30-mm balloon, the incidence of perforation should be < 0.5%. The risk of perforation increases with the bigger balloons and they should be used cautiously for progressive dilatation over a period of weeks.

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Forceful dilatation produces good to excellent relief of symptoms for more than a year in 70–90% of patients.9,11,12

There is wide variation in the incidence of GORD (between 4% and 40%) after successful dilatation, which reflects method of assessment (symptomatic versus endoscopic), the number of repeat dilatations and the length of follow-up.13–15 In most patients, however, this can usually be controlled satisfactorily with a proton-pump inhibitor.

Cardiomyotomy

This operation is generally associated with Heller, who first carried it out in 1913. Heller’s original description involved a double myotomy on the anterior and posterior walls at the cardia, but over the years a single anterior cardiomyotomy has become widely used, often in conjunction with an anterior partial fundoplication (Heller–Dor operation). Cardiomyotomy involves cutting the muscle of the lower oesophagus and cardia. The major complication is gastro-oesophageal reflux and this is less problematic by limiting the incision so that it does not extend for more than 1 cm on to the stomach and including a prophylactic antireflux operation.

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In a small randomised trial, the addition of a Dor fundoplication reduced acid reflux documented by pH study from 48% to 9% at 6 months.16

It is customary to perform a partial rather than a total fundoplication in this situation because of the risk of causing dysphagia in the presence of an aperistaltic oesophagus. The proximal extent of the myotomy does not seem to matter provided that the obstructing segment is divided and this is easily determined by intraoperative endoscopy. Heller’s myotomy is ideally suited to a minimal access approach and although it can be undertaken by thoracoscopy or laparoscopy, the latter approach seems far more popular.

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There is some variation in success rates that mainly reflects the proportion of patients with marked oesophageal dilatation and the length of follow-up in different case series. In the normal calibre oesophagus or where dilatation is minimal, cardiomyotomy is successful in more than 80% of patients.13,17,18

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The impact of previous treatments on the safety and efficacy of subsequent surgery is unclear. Two large European case series involving nearly 350 patients (including more than 80 patients who had undergone previous botulinum toxin injection or pneumatic dilatation) came to different conclusions regarding the likelihood of perforation at laparoscopic surgery.19,20

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Three randomised trials have compared balloon dilatation with surgical cardiomyotomy. Two found the surgical technique to be more effective in relieving dysphagia and one found no difference.21,22

The two trials that claimed a difference are open to major criticism, not least because both were statistically underpowered. In the trial published by Csendes et al. in 1981,21 a superior result in favour of surgery in terms of relieving dysphagia needs to be balanced against a higher rate of reflux. In addition, the pneumatic dilatation was of very short duration and there is no doubt that the results in that arm of the study were inferior to those achieved subsequently with more modern balloons. A trial in Sweden with 51 patients included a routine partial fundoplication as part of the surgery and found more treatment failures in the dilatation arm. A pan-European study involving over 200 patients found no difference in success rates at 2 years (dilatation 86% and cardiomyotomy 90% success).23

A number of studies have used decision analysis (Markov modelling) techniques to identify optimal treatment strategies in the absence of a large number of randomised trials.

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When the outcome difference was expressed as quality-adjusted life years (QALYs), dilatation and surgery were equally effective.24 Similar analyses examining cost-effectiveness tend to favour pneumatic dilatation.25–27

For these reasons, patient preference and levels of local expertise should be the main determinants in selecting treatment.

Revisional procedures and oesophagectomy

Failure to relieve dysphagia is usually because the myotomy is too short. The diagnosis is generally made on a contrast swallow but repeat manometry may be necessary. Balloon dilatation can be undertaken and there is no convincing evidence that this is more hazardous after a previous failed cardiomyotomy. The alternative is a redo operation conducted by thoracoscopy if the first attempt was laparoscopic or vice versa. Recurrent dysphagia is occasionally due to a slipped wrap and if symptoms are sufficiently troublesome, this should be surgically corrected.

Chest pain after surgery is more difficult to diagnose and manage. Some of these patients will have symptomatic gastro-oesophageal reflux, but chest pain related to swallowing and obstruction may not seem very different to heartburn for some patients. In type III achalasia, chest pain related to powerful simultaneous contractions can still persist after successful cardiomyotomy and is probably the main symptom that is associated with a poor result in these patients. Careful re-evaluation is necessary, potentially involving endoscopy, contrast radiology, manometry and 24-hour pH studies. A therapeutic trial of a proton-pump inhibitor may be worthwhile as well as being diagnostic. The addition of a fundoplication, where this was not done at the original operation, merits consideration in patients who are intolerant of proton-pump inhibitors. In other circumstances, unless a clear mechanical problem can be demonstrated (e.g. wrap disruption), revisional surgery is best avoided.

In a small proportion of patients, presentation is with a hugely dilated, flaccid oesophagus, and symptoms and signs of aspiration. Some patients also develop this as a late complication of previous treatment. While standard first-line treatments can be attempted, they often provide only very short-term relief of symptoms. Stapled cardioplasty to create a wide anastomosis between the oesphagus and the stomach may be an alternative, and this can be supplemented by antrectomy and Roux-en-Y reconstruction to minimise reflux, although the small case series describing these operations deal only with short-term outcomes.

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Oesophagectomy (ideally with vagal nerve preservation) may be the only solution.28

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Primary achalasia is associated with a small increase in risk of developing squamous cell carcinoma of the oesophagus, presumably as a result of chronic inflammation related to food retention and fermentation. Most large studies estimate the increased risk to be about 30- to 40-fold.29–32 A Swedish study also highlighted a 10-fold increased risk of adenocarcinoma in men with achalasia.33

Secondary achalasia

In South America, chronic infection with the parasite Trypanosoma cruzi causes Chagas’ disease, which has marked similarities to achalasia. The oesophagus becomes dilated (‘megaoesophagus’) and tortuous with a persistent retention oesophagitis due to fermentation of food residues. A severe cardiomyopathy is the main cause of death in these patients but some do require oesophagectomy.34

Pseudo-achalasia is an achalasia-like disorder that is usually produced by adenocarcinomas at the cardia or by any other tumour in the oesophageal wall at that level (e.g. gastrointestinal stromal tumours). While it seems attractive to suppose that the structural abnormalities related to these neoplasms must interfere with local neurotransmitters, pseudo-achalasia is sometimes also seen in patients with cancers outside the oesophagus (e.g. lung, pancreas), suggesting a paraneoplastic process.35

Secondary achalasia occasionally follows antireflux surgery. Provided there was preoperative evidence of peristalsis and this is not simply a case of misdiagnosis, the condition probably represents a wrap that is too tight for that patient. Interestingly, endoscopy is usually normal and manometry is required to establish that this is the problem. Truncal vagotomy is also recognised as a rare cause of secondary achalasia, but this can probably be condemned to history now.

Diffuse oesophageal spasm

This is a rare condition of unknown cause characterised clinically by episodes of severe chest pain and/or dysphagia.36 The upper oesophagus covered by striated muscle is usually unaffected, in contrast to the lower two-thirds, where there is pronounced muscular thickening. Chest pain can be very severe and often occurs in isolation at night. Sometimes chest pain and dysphagia occur at the same time. Many patients undergo detailed assessment for cardiac causes of chest pain or reflux disease.

The diagnosis is rarely made by endoscopy or contrast radiology. Corkscrew oesophagus on a barium swallow is the exception rather than the rule. Diffuse oesophageal spasm is defined by conventional manometry as the presence of two or more non-peristaltic sequences in a series of 10 wet swallows, but high-resolution techniques suggest that this is inadequate (Table 16.1). In many patients, abnormal contractions are characterised by multipeaked waves of increased duration and amplitude37 (Fig. 16.2) exceeding 300 mmHg on conventional studies or 8000 mmHg • s • cm as defined by high-resolution pressure topography (hypercontractile or jackhammer oesophagus). It is evident that not all abnormal contractions produce a symptomatic event, but all symptomatic events are associated with abnormal manometric appearances.38

These patients develop considerable thickening of the muscular wall of the oesophagus and treatment is usually directed towards this. Short-acting nitrates, calcium-channel blockers, phosphodiesterase inhibitors and botulinum injection have all been used to provide some patients with a degree of relief from mild symptoms, but there is no evidence that any specific drug treatment will reliably prevent attacks or provide sustained relief from symptoms. A careful explanation of the cause of their symptoms and reassurance will often suffice for patients with mild symptoms, as there is no real evidence that this is a progressive condition in the majority.

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A small number of patients have very severe symptoms and long oesophageal myotomy provides good symptomatic improvement in about 80% of patients.39

It seems important that the myotomy should encompass the entire length of the manometric abnormality, so most surgeons advocate that this should be from the aortic arch down to within a few centimetres of the oesophagogastric junction. There is no consensus regarding the need to cross the cardia and incorporate an antireflux procedure. The operation is generally completed thoracoscopically.

Oesophagogastric junction outflow obstruction and non-specific oesophageal motor disorders

A number of ‘conditions’ can be identified by oesophageal manometry. Often, the correlation between manometric abnormalities and symptoms is poor. Inevitably, patients undergoing the test have some oesophageal symptoms that have initiated the investigation in the first place and it is tempting to imply a causal relationship.

Nutcracker oesophagus merely refers to high-amplitude contractions (> 180 mmHg) with normal peristalsis during standard manometry and undoubtedly some of these patients can be re-classified by high-resolution techniques. So-called ‘hypertensive LOS’ (on the basis of a resting pressure > 45 mmHg) used to be diagnosed when the sphincter was thought to still exhibit normal relaxation and there was normal peristalsis. The ability of high-resolution pressure topography to separate effects of the lower sphincter from the diaphragmatic crura has shed new light on this phenomenon, implying that in a proportion of such patients the true functional obstruction is at the diaphragm and not the sphincter, potentially related to the presence of a sliding hiatus hernia. Non-specific motor disorders cover a ragbag of manometric abnormalities that lie outside the normal ranges covered by conventional or high-resolution manometry. Many patients with reflux disease will have one or more of these abnormalities and treatment should be directed towards their reflux disease. Inevitably, most patients with a non-specific manometric abnormality have oesophageal symptoms, but correlation with these manometric abnormalities is poor. Great care should be exercised in labelling patients with a manometric diagnosis.40 There is virtually no evidence that any of these abnormalities responds to a specific treatment.

Oesophageal motor disturbances and autoimmune disease

Systemic sclerosis, polymyositis, dermatomyositis, systemic lupus erythematosus, polyarteritis nodosa and rheumatoid disease can all be associated with oesophageal dysmotility. The condition of most clinical relevance is systemic sclerosis. It is rare for the oesophageal involvement to occur as an early feature in any of these conditions.

Systemic sclerosis

This condition has characteristic cutaneous appearances, with thickening, oedema and sclerosis of the skin associated with subcutaneous calcinosis. Unlike the other autoimmune collagen vascular disorders, visceral involvement is unusual except for the oesophagus, which is affected in up to 80% of patients. The striated muscle of the oesophagus is unaffected and there is smooth muscle atrophy involving the LOS. Peristalsis is weak and reflux common. The spectrum of oesophageal symptoms is wide, from mild to severe dysphagia with regurgitation and aspiration, as well as reflux symptoms related to the LOS defect and poor clearance. Endoscopy, barium radiology and manometry are used as appropriate to understand the extent of disease in individual patients.

Treatment usually centres around reflux symptoms and the management of complications such as stricture development. Most patients are adequately managed by proton-pump inhibitors and antireflux surgery is only rarely required.

Oesophageal diverticula

These can occur anywhere in the oesophagus. They are either congenital (rare) or acquired. In the case of the latter, they are described as traction (rare) or pulsion (common) diverticula.

Traction diverticula were said to arise from the effects of enlarged mediastinal lymph glands (particularly due to tuberculosis) and this was meant to account for the predominant location in the upper half of the oesophagus. Malignant mediastinal nodes, however, rarely cause these diverticula and with the reduction in tuberculosis, it is clear that most mid-oesophageal diverticula are of the pulsion type.

Pulsion diverticula, therefore, occur anywhere in the oesophagus but are most common in the lower half. Those that occur near the diaphragm are called epiphrenic diverticula. Most of these occur just above the diaphragm and for some reason tend to arise from the posterolateral wall of the oesophagus on the right.

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All pulsion diverticula represent the effects of an underlying motor disturbance where normally coordinated peristaltic activity is inconsistent and where a degree of functional distal obstruction is present.41 Achalasia and diffuse oesophageal spasm can both lead to diverticulum formation and identifying the underlying motor abnormality may be important in management.42

Treatment

Small diverticula require no treatment in their own right and management should be directed towards the underlying motor disturbance. When the diverticulum itself is perceived to contribute to symptoms, surgery is aimed at correction of the motor disorder and excision of the diverticulum. There are three elements to consider: removal of the diverticulum with secure closure of the oesophagus, correction of distal obstruction by a myotomy of appropriate length and the need for an associated antireflux procedure. Good historical results with open surgery have largely been replicated by stapled excision and closure of the oesophagus with myotomy and a partial fundoplication using minimal access approaches.

Acknowledgements

All high-resolution manometry figures were kindly supplied by Dr Mark Fox.

Key points

• High-resolution pressure topography is likely to supplant conventional manometry.

• Achalasia is an uncommon disorder that generally responds well to treatment by either pneumatic dilatation or surgery.

• Surgery and pneumatic dilatation appear equally effective in relieving dysphagia and improving quality of life in achalasia.

• Botulinum toxin injection for achalasia should be reserved for patients with significant comorbidities.

• Diffuse oesophageal spasm usually presents with chest pain. It is difficult to diagnose because symptoms are intermittent.

• A long myotomy may be indicated in patients with very severe diffuse oesophageal spasm but most patients should be treated non-surgically.

• Oesophageal involvement occurs in a variety of autoimmune disorders. It is particularly common in systemic sclerosis (affected in 80% of cases).

• The vast majority of oesophageal diverticula are of the pulsion type and arise within or proximal to an area of oesophageal motor disturbance.

• Treatment of oesophageal diverticula is aimed at identifying and treating the underlying motility disorder. Additional diverticulectomy is only indicated when it contributes to symptoms.

• Non-specific manometric abnormalities are not diagnoses. The correlation with symptoms is poor. Patients should not be labelled with a manometric diagnosis. Many have underlying reflux and treatment of this is often effective.

References

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28. Devaney, E.J., Lannettoni, M.D., Orringer, M.B., et al, Esophagectomy for achalasia: patient selection and clinical experience. Ann Thorac Surg 2001; 72:854–858. 11565670 A highly informative case series that highlights the problems faced in dealing with end-stage disease.

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