POSTGRAD. MED. J. (I962), 38, 297

HAEMATEMESIS AND MELAENA DUE TO RUPTUREOF A SACCULAR ANEURYSM OF THE AORTA

INTO THE OESOPHAGUS\V. L. HOOPER, M.B., B.CH., B.Sc.

Late Senior Houise Officer in Pathology, United CardiJff HospitalsFrom the Department of Clinical Pathology, Llandough Hospital

RUPTURE of an aortic aneurysm is a rare cause ofhmmatemesis and melxna. Rupture into thecesophagus is particularly rare, and when it doesoccur, the atiology is usuallv syphilitic and theoutcome immediately fatal. The following case isof interest because the patient survived for Io daysafter the initial htematemesis and the underlyingcause was shown to be medio-necrosis of the aorta.

Case HistoryA female patient, aged 63 years, was admitted as an

emergency to Llandough Hospital on February i 6,1959. She had had sudden severe pain below the lowerend of the sternum and epigastrium while eating herevening meal eight days previously. The pain radiatedthrough to the back and persisted throughout thenight. The following day she vomited a small clot ofblood and during the week before admission shevomited ' coffee-ground ' material on a number ofoccasions. Her motions were black for three days andon the morning of admission she passed a bulky blackstool; after this there was dyspncea with extremeweakness.

There was no previous history of indigestion, epi-gastric pain, heartburn, hsmatemesis or melxena. Herbowel actions had previously been normal and therewas no history of any cardiac or vascular disorder.During a previous admission to hospital her bloodpressure had been recorded as i 6o/ I Io mm. Hg.On examination she was pale and her voice was feeble.

She was not shocked and the extremities were warm.There was no clubbing, lymphadenopathy, jaundice,petechie, cyanosis or cedema. Temperature 980 F.,respirations 20/min. Pulse 104 'min., of fair volume andnot collapsing. Arterial walls were moderately thickenedand blood pressure I20/8o mm. Hg. No prmcordialpulsation, apex beat not palpable; heart sounds clear,no murmurs. She had a frequent cough productive ofmucopurulent sputum, occasionally streaked with brightred blood. Trachea central, chest expansion fair, withgood air entry in all areas and a few scattered rhonchi atboth bases. Tongue: furred and brown, with mildhalitosis. Abdominal examination: slight tendernessand guarding in the epigastrium only; no viscera ormasses palpable. Rectal examination confirmed atypical black tarry melena stool. The pupils wereequal, central, regular and reacted normally to lightand accommodation. The plantar responses flexor, alltendon reflexes normal, no sensorv loss.

Present address: The Institute of PathoIogy andTropical Medicine, R.A.F., Halton, Aylesbury, Bucks.

Laboratory Investigations. On admission the hmmo-globin was 7I% (io.6 g.), W.B.C. io,8oo/cu. mm. Theblood film appeared normal. P.C.V. 32%; E.S.R.62 mm./hr. (Wintrobe) (36 mm. after correction foranmmia). Blood group: A Rh (D) +, serum Wasser-man and Kahn reactions negative. Bacteriologicalexamination of sputum revealed no pathogenic organ-isms. The o-tolidine test for blood in the fmeces wasstrongly positive.

Treatment. The patient had been treated at homeby her general practitioner by bed rest and a light diet.In hospital she was confined to bed, placed on a fluiddiet and was given morphine. During the first 24 hoursshe was transfused slowly with 2 pints of whole bloodafter which she appeared to be very much improved.On the second evening in hospital she suddenly vomitedI6 fluid ounces of fresh blood and collapsed. In spiteof all resuscitative measures, including the rapidinfusion of plasma, she died-ten days after the onsetof her first symptoms.

Autopsy FindingsThe body was that of an elderly woman, of good

general nourishment. Rigor mortis was present in alllimbs, but apart from marked pallor there was noexternal evidence of disease or injury.The heart weighed 320 g. and the endocardium and

myocardium appeared normal. The valves were like-wise normal and the left and right coronary arteriesand their main branches were patent and free fromatheroma. There were several atheromatous plaques inthe ascending aorta, especially so immediately distal tothe cusps of the aortic valve and at the origin of thegreat vessels. In addition there were several circum-scribed translucent areas where the thickness of theaorta was greatly reduced. The smallest of these weremerely indentations in the intimal surface while thelargest measured I x I cm. and was situated in theascending aorta.There was a small saccular aneurysm measuring

I.3 cm. in diameter which communicated with theaorta through a well-defined circular orifice measuring7 mm. in diameter. The latter was situated at thejunction of the aortic arch and the descending part ofthe thoracic aorta immediatelv below and posterior tothe origin of the left subclavian artery (Fig. i). Thesac was lined W'ith intima and the aorta was entirelyfree of atheroma.The aneurysm had ruptured into the cesophagus

through a longitudinal tear 2 cm. in length in thecesophageal wall (Fig. 2). The stomach was distendedbl a recent blood clot which formed a cast of the viscus.The stomach mucosa was perfectly healthy and there

D1

copyright. on 13 July 2019 by guest. P

rotected byhttp://pm

j.bmj.com

/P

ostgrad Med J: first published as 10.1136/pgm

j.38.439.297 on 1 May 1962. D

ownloaded from

298 POSTGRADUATE MEDICAL JOURNAI, May I962

INNOMINATE ARTERYL:CO:MMON CAROTID: ARTERY

.L SUBCLAVIA N

ARTERYg ii ll /. iA:NEURY.SM

S|INi-V...~~ ~ .fr.

FIG. i.-Anterior Viezw: The heart has been removedand the aortic arch opened up showing the originsof the arterial branches above and the opening ofthe aneurysm below and directed posteriorly.

A.ORA TEA

FIG. 2.-Posterior Viezw: Showing the longitudinal tearin the oesophagus and the position of the aorta inrelation to the cesophagus.

..

...

............. ..:

-.. ......... .... :.:..

...............................

..:.........

FIG. 3.-Neck of Aneurysm ( x 150): Elastic stain of entrance to the aneurysmalsac showing the abrupt ending of the elastic fibres of the media.

was no ulceration or scarring of the duodenum. Thesmall intestine was filled with fresh blood and thelarge bowel contained a considerable quantity of alteredblood. The gall bladder showed no abnormality and

contained a small amount of bile only. The liver waspale and the spleen soft and shrunken. Pituitary,thyroid and adrenal glands were normal. Both kidneyswere pale but otherwise normal. The uterus contained

copyright. on 13 July 2019 by guest. P

rotected byhttp://pm

j.bmj.com

/P

ostgrad Med J: first published as 10.1136/pgm

j.38.439.297 on 1 May 1962. D

ownloaded from

May I962 HOOPER: Hcematemesis and Melena 299

several small fibroids showing hyaline degeneration andcalcification. The lungs were congested and (edematousand there was blood in the branches of the right mainbronchus due to aspiration at the time of the terminalbleed. There was no atelectasis or aspiration pneu-monia.

Histological ExaminationNo significant abnormality was found in the heart,

lungs, liver, spleen or kidneys.Examination of the Aorta. Blocks were taken from

ascending aorta, aortic arch, descending thoracic andabdominal aorta (I4 in all). Sections were stained withHiematoxylin-eosin, Weigert's elastic stain, Mucicarmineand Periodic-acid-Schiff reaction (PAS).The intima showed a moderate degree of atheroma

only, but marked degenerative changes were found inthe media throughout the entire length of the aorta.The lesions were focal in distribution and consisted ofhyaline and cystic degeneration of the media, resultingin partial and occasionally complete destruction of theelastic, collagen and muscle fibres. In addition, mucoidmaterial stained by Mucicarmine and giving a positivePAS reaction could be detected in the cystic areas andto a lesser degree in the interlamellar spaces of themedia. In areas with destruction of the entire mediathere was definite thinning of the vessel wall. A sectiontaken from the aortic wall near the opening of theaneurysm exhibited a similar extensive area of medio-necrosis with more or less complete destruction of theelastic, collagen and muscle fibres (Fig. 3). None ofthe numerous sections examined showed any evidenceof syphilis.

Differential DiagnosisThe differential diagnosis included gastric or

duodenal ulceration, portal hypertension withcesophageal varices, acute cesophagitis and neoplasmof the upper gastro-intestinal tract. The unusualfeature in this case was the sudden onset of severepain during a meal without previous history ofheartburn, indigestion or other gastro-intestinalupset. Ramseyer (1956) reported a similar case inwhich the onset of pain and dysphagia had beenattributed by the patient to swallowing a chickenbone. In the present case the sudden pain whileeating could also have been due to swallowing a

fragment of bone. Frequent cough was a prominentsymptom and the patient's general practitioner hadconsidered influenza as a possible diagnosis whenhe first saw her. In large aneurysms of the arch ofthe aorta cough is often a presenting symptom andin this instance it is also probable the cough was dueto compression of the left main bronchus by thepulsating aneurysm even though the aneurysmwas small. Unfortunately straight X-ray of thechest was not performed, but it would probablyhave proved of little help in diagnosis in view ofthe small size of the aneurysm and the absence ofgeneral dilatation of the aorta. In the absence ofcardiovascular signs, of palpable liver, spleen orother masses in the abdomen, bleeding from apeptic ulcer was the tentative diagnosis until thefatal haemorrhage. Indeed the signs, symptoms andpresentation of this case fully supports the statementmade by Osler over half a century ago that ' thereis no disease more conducive to clinical humilitythan aneurysm of the aorta' (Osler, I909).

DiscussionThe incidence of aortic aneurysm is less than it

was a few decades ago since the introduction ofantibiotic therapy for syphilis. Saccular aneurysmsof the aorta are most frequently syphilitic inorigin or due to rare causes such as idiopathicmedio-necrosis cystica, septic embolus, tuberculosisor trauma.

Rupture of an aortic aneurysm anywhere intothe gastro-intestinal tract, without being im-mediately fatal, is a rare cause of hematemesis andmelena. Calenda and Uricchio reviewed theliterature in I953 and found that a total of 55 caseshad been reported, the majority of which weresecondary to rupture of an abdominal aneurysminto the stomach or small bowel. Since then a fewmore cases have been reported but these also wereeither immediately fatal or were ruptures at sitesother than the cesophagus. Rupture of an aneurysmof the thoracic aorta into the cesophagus presentingas h,ematemesis and melena, with survival for any

TABLE I

CASES REPORTED SINCE 1929 OF RUPTURE OF AN AORTIC ANEURYSM INTO THE (ESOPHAGUS, AND WHICHWERE NOT IMMEDIATELY FATAL

Author and Date Sex Age' ?Etiology Symptomatology

Koppisch (1933) .. .. Male 47 Syphilis Dyspncea, pain and fever-2 months; hwmatemesis-several hours.

Watanabe (I936)*Spearman (I936) .. .. Male I9 Trauma Fatal bleeding of short duration 3 years after a shot

in the chest.Kaplan (1942) .. .. Male 58 Syphilis Pain-2-3 weeks; hematemesis-7 days.Bernstein (I944) .. .. Male 43 Syphilis Pain-6 days; haematemesis-4 days.Lutembacher (I949) .. Female 48 Syphilis Severe dyspnea-3 days; hxmatemesis-3 days.Calenda and Uricchio (1953) Male 65 Syphilis Pain of unrecorded duration; hematemesis- I I days.Krudy (1955) .. .. Male 48 Syphilis Pain-I day; h2ematemesis-5 days.Brunner (I956) .. .. Female 37 Septicamia Fatal bleeding of short duration following septic

abortion.Ramseyer (I956) .. .. Male 59 Arteriosclerosis Dyspnnea-8 months; pain-7 days; hematemesis-

3 days.

* Described in Japanese literature-details not available. Cited by Calenda and Uricchio.D2

copyright. on 13 July 2019 by guest. P

rotected byhttp://pm

j.bmj.com

/P

ostgrad Med J: first published as 10.1136/pgm

j.38.439.297 on 1 May 1962. D

ownloaded from

POSTGRADUATE MEDICAL JOURNAL May I962

TABLE 2

PUBLISHED SERIES OF THE OVERALL INCIDENCE OF RUPTURE OF ANEURYSMS OF THETHORACIC AORTA, TOGETHER WITH PERCENTAGES OF ALL SUCH RUPTURES INVOLVING

THE CESoPHAGUS

Number of Percentage ofNo. of Cases Aneurysms Number which All Ruptures

Author of Thoracic which Ruptured RupturingAneurysm Ruptured into the into theStudied (Any Site) CEsophagus CEsophagus

Boyd .. .. 4,000 I,197 I12 9.4%Lucke and Rea .. 263 51 5 9.8%Kampmeier .. 633 98 20 20.4%Lemann .. Ruptures only 592 50 I i.8%

length of time, appears to be exceedingly rare.Only io cases have been reported in the last30 years (Table i).

Prior to that, Blumenstatt in 1928 had reviewedeight cases in the literature and reported two casesof his own, making a total of 20 cases in theliterature of survival after gastro-intestinal bleedingfrom a thoracic aneurysm. Blumenstatt describedtwo types of aneurysmal rupture:

(i) The immediately fatal type in which alllayers of the wall of the aneurysm and the cesophagusrupture at the same time causing a massivehaemorrhage. The majority of ruptures are of thistype.

(2) The type in which, on the first occasion, thewall of the cesophagus is dissected longitudinallyby the force of the blood, and massive haemorrhageoccurs subsequently when the cesophageal mucosaruptures. Patients who survive for days, weeks oreven months after the first symptom of rupture,and who may present with hamatemesis andmelaena, are in this category.Our patient experienced severe pain behind the

sternum with dysphagia when the aneurysmal sacruptured into the outer wall of the cesophagus. Thiswas followed during the next week by severalsmall haemorrhages through the mucosa, culmina-ting in a massive haemorrhage on the tenth dayafter the first episode. The smaller episodes ofbleeding probably occurred from minute erosionsthrough the cesophageal mucosa, and the finalmassive haematemesis resulted from the longitudinaltear in the mucosa which was found at autopsy.

Several authors have analysed series of cases ofthoracic aortic aneurysm and their subsequentoutcome (Table 2).Kampmeier (1938) studied the notes of 633

patients with aneurysm of the thoracic aorta andfound that pain was the presenting symptom in40% of cases, dyspncea in a further 40% and theremaining 20% presented with cough, palpitations,hoarseness or dysphagia. Of the cases in Table i.pain and/or dyspncea preceded heematemesis byweeks and even months in some instances. Inthe majority, rupture occurred in large aneurysmsin grossly diseased portions of the thoracic aortawhich could be detected by X-ray examination ofthe chest. Our patient had an unusually smallaneurysm, the cause of which was revealed onlyon microscopy, namely cystic medio-necrosis ofthe aorta. Although the exact nature of this condi-tion is not fully understood, it is not related tosyphilis or to atherosclerosis. It is generallyaccepted that cystic medio-necrosis can be apredisposing factor in dissecting aneurysms but thecase here described could in no way fall into thatcategory. With the present day advances inthoracic surgery it may well have been that thisaneurysm could have been treated surgically if thecondition could have been diagnosed earlier.

SummaryA patient with a ruptured saccular aneurysm of

the aorta, involving the cesophagus, is presented.Death occurred i0 days after the initial gastro-intestinal bleeding. The post-mortem findings andthe histological appearances are described. Theliterature is reviewed.

I am indebted to Dr. Byron Evans for permission topublish the case, to Dr. T. E. Parry for his valuablehelp and encouragement in the preparation of thescript, and to Dr. F. K. Storring for the histologicalexamination. Thanks are also due to Mr. LeightonWilliams for the photographs.

REFERENCESBERNSTEIN, B. M. (I944): Perforation of the Aorta into the Alimentary Tract, Clinics, 3, 447.BLUMENSTATT (1 928): Zur Kenntnis der Bildung Intramuraler Blutungen der Speiserohre nach Durchbruch

eines Aortenaneurysmas, Virchows Arch. path. Anat., 268, 475.BOYD, L. J. (I924): A Study of 4,000 reported Cases of Aneurysm of the Thoracic Aorta, Amer. J7. med. Sci., I68, 625.BRUNNER, H. E. (1956): Akute Aortitis mit Ruptur in den C3Esophagus beu Septischen Abort, Schweiz. med. Wschr.,

86, 7I6.CALENDA, D. G., and URICCHIO, J. F. (1953): Gastrointestinal Hemorrhage due to Rupture of Aortic Aneurysm into

the CEsophagus, J. Amer. med. Ass., 153, 548.KAMPMEIER, R. H. (1938): Saccular Aneurysm of the Thoracic Aorta: A Clinical Study of 633 Cases, Ann. intern.

Med., 12, 624.

copyright. on 13 July 2019 by guest. P

rotected byhttp://pm

j.bmj.com

/P

ostgrad Med J: first published as 10.1136/pgm

j.38.439.297 on 1 May 1962. D

ownloaded from

May I962 HOOPER: Haematemesis and Meltena 301

KAPLAN, B. (0 942): Hematemesis due to Rupture of an Aortic Aneurysm: Report of a Case, New Engl. 7. Med., 226,984.KoPPISCH, E. (1933): Clinico-Pathological Analysis of Interesting Cases, Bol. Asoc. mo'd. P. Rico., 25, 140.KRUDY, A. G., and SMITH, J. A. (1955): Multiple Saccular Aneurysms of the Thoracic Aorta with Spontaneous Rupture

into the Esophagus: Report of a Case, Dis. Chest., 27, 690.LEMANN, I. I. (I9I6): A Study of Thoracic Aneurysm, Amer. 7. med. Sci., 152, 210.LucKE, B., and REA, M. H. (I923): Studies on Aneurysm, II-Aneurysm os the Aorta, Y. Amer. med. Ass., 8i, I I67.LUTEMBACHER, R. (1949): Aneurysmes de L'Aorte: Infiltration et Rupture dans les Parois de L'CZEsophage, Presse

Medicale, 57, 857.OSLER, W. (I909): Syphilis and Aneurysm, Brit. med. 37., ii, 1509.RAMSEYER, M. (1956): Perforation dans L'CEsophage d'un Aneurysme Dissequant de l'Aorte, Radiol. Clin., 25, 297.SPEARMAN, M. (1936): Traumatic Aneurysm of Arch of Aorta with Rupture into Esophagus, Southwest Med., 20, 174.WATANABE, T., and USUKU, S. (1936): Ein Fall von Aortenaneurysma mit Einer Broncho-CEsophagealfistel, Nagasaki

Igakkai Zasshi, 14, 520.

THROMBOSIS-IN-SITU OF THEMAIN PULMONARY ARTERIES

T. A. DON MICHAEL, M.B., M.R.C.P. (Lond.), M.R.C.P. (Edin,).Registrar, St. Stephen's Hospital, London, S.W.Io

MASSIVE thrombotic occlusions of the main pul-monary arteries is a rare condition occurring per-haps once in five thousand post-mortem examina-tions. Its clinical recognition is even rarer(Magidson and Jacobson, 1955). Posselt (I909)could find only three examples of completeocclusions of the pulmonary artery in its mainbranches, and Brenner (I93 I), six cases oforganizingthrombus in the main pulmonary arteries.

Case ReportF.M.L., 5I-year-old female patient, was admitted to

Guy's Hospital on 4.5.6I with a history of progressivebreathlessness and palpitations of four years' duration.A heart murmur had been heard at the age of 7 butalthough she attended a special school she had led anormal life up to the age of 4I. She then sustained aninjury to her left leg and had suffered from recurrentswelling in it since. She also suffered accidental injuryto the front of her chest and thereafter complained ofpain in the chest. For the past ten years she hadnoticed gradually increasing breathlessness and oc-casional palpitations and suffered from frequent chestcolds. Prior to admission her breathlessness was of sucha degree that she was confined to bed.

Examination revealed a thin patient with cyanosedmucous membranes and early clubbing of the fingers.The jugular venous pressure was raised 4 cm. above thesternal angle and well marked ' a' and ' v' waves werepresent. The apex beat was in the 6th space i in.outside and the mid-clavicular line and was diffuse incharacter. There was a moderate left parasternalpulsation. A pansytolic murmur was audible at theapex and left sternal edge with a soft ejection murmurin the pulmonary area. The second sound in thepulmonary area was accentuated and rather widely split.There were no abnormal physical signs present in therespiratory system. The liver was palpable, I finger'sbreadth below the costal margin.

lll........ ..:::.. .:

..:... .:..:::...|I. ..............

..... .. ... ..;a

.. .. . .

FIG. I

'l'herc was no peripheral edema present and notenderness over the deep veins of the legs.

copyright. on 13 July 2019 by guest. P

rotected byhttp://pm

j.bmj.com

/P

ostgrad Med J: first published as 10.1136/pgm

j.38.439.297 on 1 May 1962. D

ownloaded from