Diffuse oesophageal spasm - Thorax · Diffuse oesophageal spasm OESOPHAGEAL DILATATION In all...

Thorax (1966), 21, 511.

Diffuse oesophageal spasm

D. R. CRADDOCK, A. LOGAN, AND P. R. WALBAUM

From the Thoracic Unit, Royal Infirmary, Edinburgh

Incoordination of muscular contraction is some-times seen in the apparently healthy oesophagus,but it is a prominent feature of two conditions-achalasia and diffuse spasm.

This review is concerned with the second ofthese. In the 12-year period 1954-66, 12 cases ofdiffuse spasm were recorded in our unit. Le Rouxand Wright (1961) reported on 55 cases ofaChalasia in the same unit during the 10-yearperiod 1948-58. In our experience, achalasia,which is a rare disease, is approximately fivetimes more common than diffuse oesophagealspasm. This is the incidence of the two conditionsas seen in a thoracic surgical unit, but the radio-logical pattern of 'corkscrew oesophagus' is seenfrequently in the course of barium examinationin patients without dysphagia (Sheinmel, Priviteri,and Poppel, 1949). The patients referred to in thisreport all presented with dysphagia and demon-strated the radiological pattern and intraluminaloesophageal pressure tracings of diffuse spasm.

CLINICAL MATERIAL

Twelve patients have been reviewed. They wereinvestigated and managed with a uniform policy.All had a barium swallow examination andoesophagoscopy. All except one had oesophagealpressure tracings recorded. Unfortunately, two ofthe earlier patients subjected to operation did nothave oesophageal pressure measurements pre-operatively.

CLINICAL FEATURES

AGE AND SEX Ages ranged from 44 to 69 yearsat the time of initial presentation. There were sixwomen and six men.

FAMILY HISTORY Only one patient, a 62-year-oldwoman, gave a family history of oesophagealdisease. Her mother had died of carcinoma of theoesophagus.

SYMPTOMS Dysphagia was the symptom commonto all these patients. It was intermittent and variedin severity. It was rarely severe enough to havean appreciable effect on nutrition, but two patientshad lost over 2 stones (12-7 kg.) in weight beforepresentation. Most claimed to have come to termswith their dysphagia and, although it preventedtheir enjoyment of normal meals, they adjustedtheir diet accordingly. Two had the unusualcomplaint of difficulty in swallowing liquids,whereas solids were managed relatively well. Thecommonest site of food 'sticking' was behind thexiphisternum. The interval between episodes ofdysphagia varied, some claiming difficulty at everymeal and some swallowing normally for three tofour days, with all ranges in between. Duringexacerbations, or else when the patients becameweary of their symptoms, they presented forhospital treatment.The duration of dysphagia varied from eight

weeks to 15 years, with an average of four years,before the first examination. All patients hadsome regurgitation, and in three there was also aconsiderable amount of acid reflux and heartburn.Pain on swallowing was not always a feature, andin this respect our series differs from that ofCreamer, Donoghue, and Code (1958), who re-ported that 13 of their 16 patients complained ofpain. It is difficult to separate pain from discom-fort during swallowing, but in our series onlyfour patients admitted painful swallowing.

INVESTIGATIONS

BARIUM SWALLOW The characteristic appearanceproduced by diffuse oesophageal spasm during abarium swallow is one of incoordinated, irregularcontractions of the oesophagus most marked fromthe aortic arch to the diaphragm. The consequentsegmentation of barium gives rise to the name'corkscrew oesophagus'. A second but less com-mon appearance is of tight contraction of theoesophagus over a length of several centimetres.

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D. R. Craddock, A. Logan, and P. R. Walbaum

This incoordinate action may cause the swallowedbarium to be squeezed back into the upperoesophagus and is sometimes eliminated by anormal peristaltic wave (Figs 1 to 3).

Simultaneous and repetitive contractions maycontinue for a considerable period after a singleswallow.Although Ellis, Olsen, Holman, and Code

(1958) stated that dilatation is rare in this con-dition, four out of the 12 patients in this seriesdeveloped it in varying degrees (Figs 4 and 5).As the disease progresses diverticula may form,

and in three of the 12 patients this occurred, intwo in a gross, degree (Figs 1 and 6).Johnstone (1950) and Creamer (1962) pointed

out the association of diffuse spasmh with hiatalhernia, and in two of the patients there was anassociated sliding hiatal hernia. In a third, thepicture of diffuse spasm developed only afterrepair of a para-oesophageal hiatal hernia.

One patient in this series was initially regarded,on radiological evidence, as suffering fromachalasia, but subsequently developed the charac-teristic radiological and manometric features ofdiffuse spasm.

OESOPHAGOSCOPY The findings at oesophago-scopy were often surprisingly normal. In the twopatients with a sliding hiatal hernia the diagnosiswas confirmed by finding a high mucosal transi-tion. In the three patients with oesophageal dila-tation there was food residue and excess salivain the upper oesophagus. In three, narrowedsegments were encountered in the lower half ofthe oesophagus, but in only one was it impossibleto pass the endoscope into the stomach; this wasin the patient whose radiographs are shown inFig. 6 and who had the most severe form of thedisease. In the others the endoscopic findings werenormal.

FIG. 2

FIGS 1 AND 2. Barium swallow radiographs of twopatients with diffuse oesophageal spasm illustrating themultiple, irregular, segmental oesophageal contractionsthat are afeature ofthe disease. It isfrom this appearancethat the name 'corkscrew oesophagus' originates.FIG. 1

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FIG. 3. Barium swallow radiograph of a patient withdiffuse oesophageal spasm showing the appearances lesscommonly found, namely a long, spastic oesophagealsegment which is in this case associated with a smalldiverticulum.

FIG. 4. Early oesophageal dilatation occurring in a

patient with diffuse oesophageal incoordination.

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FIG. 5. Advanced oesophageal dilatation occurring in apatient with diffuse oesophageal incoordination. Theseradiographs are of the same patient as the radiographin Fig. 4 but at a later stage.

FIG. 6. Barium swallow radiograph shows the grossoesophageal deformity which may ultimately occur in asevere case of diffuse oesophageal spasm. The largeoesophageal diverticulum was excised at the time oflower oesophagomyotomy.

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OESOPHAGEAL PRESSURE MEASUREMENTS Thesewere made in 11 patients; in the twelfth it wasnot possible to pass the tube. The pressures wererecorded by means of three balloons spaced at5-cm. intervals on a triple-lumen tube connectedto pressure transducers. The tracings obtained allshowed the same features. The normal pattern ofperistaltic contractions produced by swallowing(Fig. 7) was replaced by simultaneous contrac-

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Swallow SwallowFIG. 7. Oesophageal pressure tracings showing normalprogressive peristaltic contractions in a normal person.The aortic arch is approximately 30 cm. from the noseand the cardia approximately 45 cm. from the nose. Theimportant features are the normal pressures, the sequentialappearance of the peristaltic peaks as the wave advancesdown the oesophagus, and the fact that one swallowevokes only one set of contractions. Upper tracing, 30cm. from nose; middle tracing, 35 cm. from nose;lower tracing, 40 cm. from nose.

tions, often of increased amplitude. In severalcases peak pressures of 150 to 200 cm. H20 wereobtained, although in the majority pressures wereat the upper limit of normal. In addition, a singleswallow often produced a series of repetitive con-tractions which persisted for varying periods (Figs8 and 9). These changes were always most markedin the lower half of the oesophagus, and in somecases normal peristaltic contractions occurred inthe upper half. The pattern in patients with ademonstrable hiatal hernia differed in no wayfrom that in patients without a hernia.

TREATMENT

Two methods of treatment were used oesopha-geal dilatation and lower oesophagomyotomy.

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Swallow SwallowFIG. 8. Abnormal pressure tracings from a patient withdiffuse oesophageal spasm. The lower tracing shows thecharacteristic feature of the disease; non-progressivecontractions, high pressures, and the multiple contractionsthat follow a single swallow. Upper tracing, 30 cm.from nose; middle tracing, 35 cm. from nose; lowertracing, 40 cm. from nose.

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SwallowFIG. 9. Grossly abnormal oesophageal pressure tracingsfrom a patient with diffuse oesophageal spasm. Theperistaltic peaks in all three tracings occur simultaneouslyand have peak pressures of over 150 cm. of water, whichis approximately double the normal value. Upper tracing,28 cm. from nose; middle tracing, 33 cm. from nose;

lower tracing, 38 cm. from nose.

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OESOPHAGEAL DILATATION In all patients this wasthe initial method of treatment. In six, oesophago-scopy alone was performed before the diagnosiswas established, and in all of these the dilatationproduced by simply passing the Negus oesophago-scope resulted in considerable relief of symptoms.In four of these six, the benefit was for only ashort time. Where a narrowed segment was en-countered it was dilated by bouginage and theNegus hydrostatic dilator. In seven of the 12patients, oesophagoscopy with dilatation was theonly treatment.

LOWER OESOPHAGOMYOTOMY If symptoms wereunrelieved by dilatation, or if dilatation was re-quired too frequently, the patient was advised toundergo lower oesophagomyotomy. Five of the12 patients were treated in this way. The oeso-phagus was mobilized through a low left lateralthoracotomy, and the muscularis externa wasdivided with a single longitudinal incision extend-ing from the aortic arch to the proximal stomach.The findings at operation varied from normal

to grossly abnormal. In two of the five patients,the oesophageal muscle was greatly hypertrophiedand measured 15 mm. and 11 mm. in thicknessrespectively. The former was the patient with thelarge diverticulum whose radiographs are shownin Figure 6. When the diverticulum had beenexcised a finger inserted into the oesophagus feltas if it was enclosed in a stiff rubber hose. Thisthickening was greatest in an area beginning 3 cm.above the hiatus oesophagus and extendingproximally for 5 cm. The diverticulum was 5 cm.in diameter. In one patient the oesophagealmuscle was moderately thickened but in the othertwo it was macroscopically normal. In each casehistological examination showed normal muscle.Autonomic ganglia were seen in two of thespecimens.

ASSOCIATED HIATAL HERNIA Of the 12 patients,three had a hiatal hernia, two of the sliding typeand one para-oesophageal. The latter had the herniarepaired before it was recognized that she hadoesophageal incoordination, which has been sub-sequently managed by oesophagoscopy anddilatation. Neither of the patients with a slidinghiatal hernia had oesophagitis. Both were sub-jected to repair of the hernia, and in one a loweroesophagomyotomy was also performed. In theother, no myotomy was carried out in an attemptto see whether dealing with the hernia wouldimprove the dysphagia.

RESULTS

The assessment of results has been difficult insome cases because these patients learn to livewith their disease and often minimize theirsymptoms. This happens similarly in patients withachalasia, and Ellis (1960) suggested that radio-logical progress is often a better guide thansymptoms when assessing the results of treatment.

WITH DILATATION ALONE Seven of the 12 patientswere managed by dilatation alone and none re-quired hospital admission more than twice a year.The relief of symptoms produced by dilatationusually lasted several months at least and in oneit lasted five years. This patient has had fivedilatations since 1945, and when last reviewed inDecember 1965 claimed to be having onlyoccasional dysphagia. She was advised to undergooesophagomyotomy on two occasions, but de-clined each time. Four patients had intervalsgreater than two years between dilatations.

Dilatation made little or no difference to theradiological appearance or to the oesophagealpressure measurements but significantly reducedthe severity of symptoms. The patient who hadonly a hiatal hernia repair had her first oesopha-geal dilatation in 1951 and, in spite of intermittentminor dysphagia, did not require admission againuntil 1965, when the hiatal hernia was demon-strated for the first time and subsequently re-paired. Her oesophageal incoordination had notshown significant progression in that period.

LOWER OESOPHAGOMYOTOMY Five patients weretreated in this way. Myotomy greatly improvedthe radiological appearances, though it did notcompletely abolish them. In the oesophagealtracings, the hypertonic, repetitive, non-peristalticwaves were eliminated by myotomy (Fig. 10).The results in all five patients have been good,

with an almost total relief of symptoms and, intwo, a weight gain in excess of 2 st. (12-7 kg.).The follow-up periods in these cases have rangedfrom 12 years to three months. The earliest opera-tion was in 1954 and the latest in 1965. Allpatients experienced relief of symptoms and onlyone patient required a further dilatation. Thispatient, who underwent operation in 1954,recently returned with minor intermittentdysphagia. Radiological examination, oesophagealpressure measurements, and oesophagoscopicfindings all suggested that there was significantincoordination above the aortic arch. Theoesophagus was dilated and his progress is beingwatched.

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FIG. 10. Oesophageal pressure tracings of a

patient who had undergone lower oesophago-myotomy for diffuse spasm. The abnormal con-tractions have been eliminated below the aorticarch. Upper tracing, 30 cm. from nose; middletracing, 35 cm. from nose; lower tracing, 40cm. from nose.

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WITH ASSOCIATED HIATAL HERNIA These threepatients are at present free from dysphagia. Thepatient with the para-oesophageal hernia has beendiscussed previously. The patient who underwentmyotomy and repair of the hernia has now beensymptom-free for six months, and the patient whohad a hernia repair only has been symptom-freefor five months.

DISCUSSION

Little is known about the aetiology of this condi-tion and all suggested theories lack proof. Manypatients without dysphagia show some degree ofoesophageal incoordination. However, if thedisease progresses to dilatation and diverticulumformation, symptoms probably always occur.When the patient is asymptomatic no treatmentis indicated. Once symptoms begin, treatment isnecessary. Oesophagoscopic dilatation should betried initially. If this proves unsatisfactory loweroesophagomyotomy should be undertaken.Creamer (1962) stated that a sliding hiatal

hernia is almost invariably present in cases ofdiffuse spasm because of the effect of contractinglongitudinal muscle which pulls the proximalstomach into the chest. In this series we have notbeen able to demonstrate such a high incidenceof hernia in spite of repeated radiological andendoscopic examinations. A sliding hiatal hernia

can cause dysphagia in the absence of ulcerationand stricture formation, and where hernia anddiffuse spasm coexist it may be important toestimate the contribution of each to dysphagia.It was with this idea in mind that one of the twopatients with sliding hiatal hernia was subjectedto myotomy plus repair of the hernia and theother to a hernia repair alone. As in other formsof oesophageal obstruction there is a tendency forfood to become impacted, and three patientssuffered from this during the course of thedisease.The relationship of diffuse spasm to achalasia is

a close one. Achalasia after dilatation may exhibita somewhat similar radiological appearance, andit is only by following the course of the diseasethat the two can ultimately be distinguished.The normal relaxation of the oesophago-cardiac

sphincter, which is the usual finding in diffusespasm, does not occur in achalasia. In one patientin the series, the first diagnosis was achalasia, butthis was later changed when the characteristico,esophageal pressure tracings and radiologicalappearances of diffuse spasm developed. Non-peristaltic oesophageal contractions are sometimesfound at manometry in people without dysphagia.This is consistent with the finding that somepatients demonstrating the corkscrew appearanceradiologically are asymptomatic. However, wehave not seen a severe form of this condition

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without symptoms. Early in the series an attemptwas made to treat these patients with antispasmo-dic drugs; but, as most other workers have found,the relief produced was transient. This form oftreatment was then abandoned in favour of thetwo methods discussed above. In this series no

patient has died from the disease, illustrating thefact that although this is an unpleasant conditionit is not a fatal one.

SUMMARY

Twelve cases of diffuse oesophageal spasm are

presented. Their symptomatology and clinicalfindings are considered. An attempt has beenmade to trace the progress of the disease over a

period which in some cases exceeds 12 years.

Two types of management are outlined and theresults are discussed.

REFERENCESCreamer, B. (1962). In Surgical Physiology of the Gastro-Intestinal

Tract: Proc. Symp. roy. Coll. Surg. Elin., p. 47, ed. A. N. Smith.Donoghue, F. E., and Code, C. F. (1958). Pattern of oeso-

phageal motility in diffuse spasm. Gastroenterology, 34, 78 2.Ellis, F. G. (1960). The natural history of achalasia of the cardia.

Proc. roy. Soc. Med., 53, 663.Ellis, F. H., Olsen, A. M., Holman, C. B., and Code, C. F. (1958).

Surgical treatment of cardiospasm (achalasia of the esophagus):considerations of aspects of esophagomyotomy. J. Amer. med.Ass., 166, 29.

Johnstone, A. S. (1950). In A Text-book of X-ray Diagnosis, 2nded., ed. S. C. Shanks and P. Kerley, Vol. 3, p. 36. Lewis, London.

Le Roux, B. T., and Wright, J. T. (1961). Cardiospasm. Brit. J. Surg.,48, 619.

Sheinmel, A., Priviteri, C. A., and Poppel, M. H. (1949). A study of theeffect of certain drugs on curling of the esophagus: A prelimi-nary report. Amer. J. Roentgenol., 62, 807.

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