Postgraduate Medical Journal (January 1974) 50, 41-44.

CASE REPORTS

Oesophago-cardiac fistula complicating achalasia

R. W. STRONGF.R. C.S.

Princess Alexandra Hospital, Brisbane, Australia, 4102

SummaryA case is reported of a patient with carcinoma of theoesophagus, secondary to a longstanding achalasia andsubsequent development of a fistulous communicationbetween the oesophagus and the heart. The literature isreviewed.

IntroductionFistulous communications between the heart and

the oesophagus are extremely rare. They may beoesophago-pericardial, of which nineteen cases havebeen recorded, or oesophago-cardiac of which onlytwo cases have been recorded. The communicationin the oesophago-cardiac cases has been between theoesophagus and left atrium. In the case reportedhere, both communications occurred.

Case reportMiss A.C., aged 56 years, was referred to hospital

by her doctor with general ill health and weightloss.At the age of 26 years, the patient developed

dysphagia. A barium meal was carried out, followingwhich oesophagoscopy and dilatation were per-formed. She was then given a bougie which she usedover the subsequent 6-12 months. From then untilher present illness she was well. However, on closerquestioning, she did admit to bouts of difficulty inswallowing, which seemed to clear spontaneously ifshe placed herself temporarily on a fluid diet.Her present history began 6 weeks before admis-

sion, when she developed general malaise. Twoweeks later, she developed a chest infection, forwhich she received an antibiotic and a linctus fromher practitioner, and her cough improved. Soon after,she noticed a throbbing pain in the left loin, andthis persisted until her admission. She began toregurgitate undigested food and mucus, 2 weeksbefore referral. There was no blood or bile in thevomit. There was associated constipation. A loss of1-2 stone in weight had occurred over the 6 weeks.On examination, she was an ill looking woman,

who showed evidence of weight loss. She did not

appear anaemic or dehydrated. Her pulse was100/min, with sinus rhythm; temperature 37 5'C.The blood pressure was normal. The heart soundswere faint, but normal in character. The jugulo-venous pressure was not raised. There was dullness topercussion and reduced breath sounds at the left base,together with generalized rhonchi. On examination,the abdomen and central nervous system were normal.

Chest X-ray (Fig. 1) showed a fluid level in the rightparatracheal region. This was thought to be a dilatedoesophagus, possibly as a result of achalasia. Herhaemoglobin was 11-3 g%0. White blood count7500/cc. ESR 10 mm/1 hr. The electrolytes and ureawere normal. A gastrografin swallow (Fig. 2) de-monstrated a grossly dilated and tortuous oesophagus,with the lower end having a smooth tapering entryinto the stomach.

There seemed no doubt that she had suffered fromachalasia of the oesophagus for many years andwould need surgery, but, because of the severedeterioration in her general condition, complicatingcarcinoma was suspected and a preliminary oeso-phagoscopy planned. As preparation for this, heroesophagus was aspirated and washed out withsaline twice daily. She was restricted to clear fluidsonly by mouth, and her intravenous feeding includedAminosol and intralipid. She was given physio-therapy for her chest. The oesophageal residue de-creased, and the return became clearer.Four days after treatment was instituted, she was

observed to have atrial fibrillation. There was noobvious predisposing cause. She was digitalized, and2 days later returned to sinus rhythm. Forty-eighthours following her reverting to sinus rhythm, therewas a dramatic change in the course of events. Thepatient became confused, and had a rigor. Hertemperature rose to 38°C. She was again in atrialfibrillation, and her systolic blood pressure fell 40points to 100 mm. Examination did not reveal anynew abnormal signs. Chest X-ray, urine analysis andultrasound testing of the legs were all normal. Bloodcultures were negative.Over the next 4 days, she remained unwell. Her

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42 Case reports

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FIG. 1. Chest X-ray, (a) A.P. and (b) lateral, showing fluid level in right paratracheal region.

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FIG. 2. Gastrografin swallow, showing (a) gross dilatation and tortuosity of oesophagus. The lower end (b) narrowsat the cardia.

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Case reports 43

temperature returned to normal, but her blood pres-sure continued to be low. At this stage her white countrose to 21,500/cc and the ESR to 56 mm. She againdeveloped a pyrexia, became confused, and the bloodpressure fell even lower. She gradually became coma-tose, and at this stage auscultation revealed a peri-cardial rub. Her condition continued to deteriorate,and she died 12 hr after lapsing into coma.

Post mortemThe oesophagus was grossly dilated, with a con-

striction about the level of the tracheal bifurcation.The lower end was narrow at the oesophago-gastricjunction. There was a 6 x 4 cm ulcer on the anteriorwall of the oesophagus, the edges of the ulcer beingadherent to the mediastinal tissues (Fig. 3). Thefloor of the ulcer opened into a mediastinal cavitywhich contained pus. There was a track leadingfrom this cavity into the pericardial sac. The peri-cardial space contained 150 ml of dark colouredfluid, with food particles evident. There was also atrack leading on to the left atrium of the heart(Fig. 3b). The pericardium was thickened. There wasa large left sided subphrenic abscess, in the retro-peritoneal region, in relation to the left kidney andadrenal gland. The abscess was bounded above bythe diaphragm, in which there was a 1-5 cm diameteropening into the left side of the chest, adjacent tothe mediastinal structures. No direct communicationcould be found between the subphrenic and media-stinal abscesses. Posteriorly, the subphrenic abscessformed loculations in the quadratus lumborum andpsoas major muscles. The brain showed abscesses inboth frontal lobes.

Histological examination of the ulcer showed amoderate to poorly differentiated epidermoid car-cinoma, infiltrating to a depth of 1 cm. There weretwo perforations through the centre of the carcinoma-one into the pericardial cavity, and one into the

left atrium. The adrenals showed involvement withcarcinoma consistent with metastases from theoesophagus.

DiscussionThe post mortem findings would seem to explain

theclinical course of this patient. The gross dilatationand tortuosity of the oesophagus as seen on X-rayand at post mortem, were consistent with her 30-year-history of difficulty in swallowing due to acha-lasia. Her final illness began with general malaise,which was soon followed by a cough and pyrexia,vomiting and left loin pain. Perforation of thecarcinoma of the oesophagus, with formation of amediastinal abscess, probably occurred at this stage,and its subsequent tracking down into the sub-phrenic retroperitoneal position on the left side,accounted for the left loin pain. However, directcommunication between the two abscesses couldnot be demonstrated. The development of atrialfibrillation was evidence either of the developmentof pericarditis, or of the oesophago-atrial fistula.The cerebral abscesses undoubtedly arose as aresult of this, and were heralded by the suddencollapse, rigor, hypotensive state and confusion.The association of carcinoma of the oesophagus

and achalasia is well established and widely reported(Just-Viera and Haight, 1969). There are, however,disparate figures regarding the incidence in theliterature, varying from 1% to 20%. Even acceptingachalasia as a possible precursor to malignant changein the oesophagus, early diagnosis of a carcinoma isdifficult. They are, therefore, frequently too advancedfor surgical treatment. There have been manyreports of tracheo-oesophageal fistulae terminally.Perforation of the oesophagus, resulting in apneumothorax, empyema or pulmonary abscess mayoccur, and perforation of the aorta with massivebleeding can constitute the terminal stage.

(a)A-a: ;0 0

FIG. 3. (a) Post mortem specimen with large carcinoma of the oesophagus. Probe in position of oesophago-cardiac fistula.(b) Probe in orifice of fistula in left atrium. The orifice of the oesophago-pericardial fistula not demonstrated in thisphotograph.

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44 Case reports

In the patient reported here, perforation of thecarcinoma resulted in the formation of a mediastinalabscess. This subsequently eroded into the pericardialcavity and left atrium, thus establishing the rareoesophago-cardiac and oesophago-pericardial fistu-lae. They have not previously been reported as acomplication of achalasia.Only two cases of oesophago-cardiac fistulae have

been reported. In one (Laubscher, 1970), a 20-year-old man, there were no alimentary symptoms. Hepresented with a syncopal attack of short durationand no abnormal physical signs were elicited. He wasfound dead in his bed the next morning. Post mor-tem examination showed a chronic oesophagealulceration, with a fistulous track leading to the leftatrium. There was blood in the oesophagus. Therewas no evidence of hiatus hernia, reflux oesophagitisor peptic ulceration.The other case (Hojgaard and Raaschou-Nielsen,

1970), a 73-year-old woman, had suffered fromdifficulty in swallowing for 12 years, and 18 monthsprevious to her final illness, underwent surgery forhiatus hernia and stenosis. However she continuedto experience difficulties in swallowing. The patientwas admitted after a series of syncopal attacks. Onexamination she appeared to have suffered a myo-cardial infarct. She was anaemic. Her conditiondeteriorated, and the terminal phase was marked bya pyrexia, and non-characteristic cerebral symptoms.She died 6 days after admission. At post mortem shehad a constricted area of oesophagus, adherent tomediastinal structures, with a small fistula leadingfrom this area, into the left atrium. There weremoderate amounts of blood in the oesophagus andstomach. She had fresh cortical infarcts of theoccipital lobe of the brain.The absence of overt bleeding seems surprising;

although at post mortem examination, blood wasfound in the oesophagus in the two previouslyreported cases, there had been no haematemesis. Thefistulae in all cases were of small diameter. In thepresent case the lack of blood in the oesophagus atpost mortem was probably due to the differencebetween the left atrial pressure which is normallylow, and the higher pressure in the oesophagus as aresult of the column of retained fluid.

Oesophago-pericardial fistulae are somewhat morecommon, but still rare. Nineteen previous cases havebeen reported in the literature (McDaniel andKnepper, 1957; Dons et al., 1964). The pyopneumo-

pericardium, like the oesophago-cardiac fistulae,carry a poor prognosis. One case, however, has beensuccessfully repaired (Dons et al., 1964). There isa variable aetiology in the reported series. Five caseswere due to carcinoma of the oesophagus. Five caseswere caused by swallowing a foreign body, includinga case of a swallowed denture and an occupationalaccident by a sword swallower. There have been fiveinstances of benign idiopathic ulcers of the oeso-phagus, eroding into the pericardial sac. The re-maining cases were of ulceration of a congenitaltracheo-oesophageal diverticulum, and an attemptedrepair of a diverticulum of the lower oesophagus.

Provided early diagnosis can be established inoesophago-pericardial fistula, surgical repair ispossible in benign cases. The prognosis in malignantcases is hopeless. Many of the cases have only beenrecognized at post mortem. The more frequentphysical signs recorded are: precordial pain, splash-ing, churning, tinkling, metallic sounds, these mur-murs synchronous with the heartbeat (McDanieland Knepper, 1957). They may develop arrhythmias,as in this case. Pneumopericardium may be demon-strated on X-ray. Gastrografin swallow may demar-cate the fistulous communication, although thetrack is usually very narrow, and therefore difficultto demonstrate.The chances of diagnosing, and repairing an

oesophago-cardiac are even more remote. The rarityof the condition, and the swiftness of its dire con-sequences, probably preclude successful diagnosisand treatment.

AcknowledgmentsThis patient was under the care of Mr A. York Mason and

I am grateful for his permission to report this case, and forhis helpful advice in the preparation of this paper. Also MrT. W. Brandon, Department of Medical Photography, StThomas' Hospital, for the photographic plates.

ReferencesDONS, M., ERICKSEN, K.R., RYssING, E. & THERKELSIN, F.

(1964) Pyopneumopericardium with oesophagopericardialfistula. Acta Chirurgica Scandinovia, 128, 766.

HOJGAARD, K. & RAASCHou-NIELSEN, T. (1970) Oesophago-cardiac fistula. Acta Pathologica Mirobiologica Scandina-vica, Suppl. 212, 114.

JUST-VIERA, J.O. & HAIGHT, C. (1969) Achalasia and carci-noma of the oesophagus. S.C.O. 1081.

LAUBSCHER, F.A. (1970) Oesophago-cardiac fistula. NewEngland Journal of Medicine, 794.

MCDANIEL, J.R. & KNEPPER, P.A. (1957) Oesophago-peri-cardial fistula. Journal of Thoracic Surgery, 173.

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