Thorax (1957), 12, 258.

INTESTINAL VASCULAR LESIONS SIMULATINGPOLYARTERITIS NODOSA AFTER RESECTION

OF COARCTATION OF THE AORTABY

R. L. HURT AND W. J. HANBURYFrom St. Bartholomew's Hospital, London

(RECEIVED FOR PUBLICATION MAY 9, 1957)

it is only recently that mesenteric arterial nec-rosis, causing severe abdominal symptoms, hasbeen recognized as a post-operative complicationafter resection of coarctation of the aorta. Thefirst recorded case was mentioned briefly in a reportby Sealy (1953) and later presented in detail,together with another similar case, by Benson andSealy (1956). Two further cases, with lesionsindistinguishable from periarteritis nodosa, werereported by Lober and Lillehei (1954), and anotherby Perez-Alvarez and Oudkerk (1955). No othercases have been reported in the literature, althoughRing and Lewis (1956) have reviewed a series of18 patients in whom abdominal symptoms ofvarying intensity occurred as a marked featureduring the post-operative period. Cleland, Couni-han, Goodwin, and Steiner (1956) have also reportedthe occurrence of severe post-operative abdominalpain in five out of a series of 40 cases of coarctationtreated surgically, but came to no conclusion as tothe cause of the pain.

Patients who develop abdominal pain after aresection of a coarctation of the aorta may betreated on a more rational basis if it is recognizedthat this pain, which can be severe and of sufficientseverity to simulate an acute abdominal emergency,may be due to arterial necrosis. The condition alsohas added significance in relation to the role ofhaemodynamics in the aetiology of polyarteritisnodosa and related arterial diseases. For thesereasons a further case with striking pathologicalchanges is presented in this paper.

CASE HISTORYA 10-year-old boy had been known to have coarctation

of the aorta since the age of 5, after the incidental dis-covery of a precordial systolic murmur. The child hadled a normal, healthy, and active life, and there had beenno appreciable symptoms. There was no history ofallergy, although two grandparents and a brother hadsuffered from asthma.

Clinical examination showed that the brachial bloodpressures were 148/102 mm. Hg (right side) and 156/112mm. Hg (left side). No pulsation could be felt in theabdominal aorta or in either lower limb. Abnormalarterial pulsation could be felt in the chest wall in theposterior axillary line on both sides, indicating thepresence of a collateral circulation. A systolic murmurwas audible all over the precordium, maximal in the thirdleft interspace and faintly conducted into the neck. Achest radiograph showed notching of several ribs on bothsides, from the third rib downwards. An electrocardio-gram showed evidence of considerable left ventricularhypertrophy. The haemoglobin was 13.9 g. per 100 ml.and the white blood cell count was 12,200 per c.mm. Noabnormality was found in the urine.The coarctation was resected by Mr. 0. S. Tubbs on

July 19, 1956. Systemic penicillin was given pre- andpost-operatively. During the operation the bloodpressure was maintained around 100 mm. Hg systolicby the use of an intravenous infusion of " arfonad." Thecoarctation was found at the site of attachment of theligamentum arteriosum and the obstruction was almostcomplete, no pulsation being visible below the narrowedsegment. A fair collateral circulation had developed, asshown by enlargement of the intercostal arteries and leftinternal mammary artery, and also by the large vesselsin the chest wall. The coarctation was resected and anend-to-end anastomosis of the adjacent portions of theaorta performed. There was only a minimal degree ofconstriction at the site of anastomosis. The operationwas completed in four hours, and the patient's conditionon return to the ward was satisfactory, both femoralpulses being easily palpable.For 17 days after operation the blood pressure

remained in the region of 180/130 mm. Hg. On the thirdpost-operative day the patient began to complain ofabdominal pain and distension. This pain was general-ized throughout the abdomen and was in the nature of acolic. The pain and distension continued until theseventeenth day when the patient's condition suddenlydeteriorated, the pain became more severe, and signs ofa severe intra-abdominal haemorrhage developed.Although up to this time the bowels had been openedirregularly, a considerable amount of flatus had beenpassed, and it had therefore been considered unlikely

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INTESTINAL VASCULAR LESIONS SIMULATING POLYARTERITIS NODOSA

that an acute intestinal obstruction had caused thepatient's symptoms. The white blood count had risento 19,800 per c.mm. A laparatomy was performed andabout 1l pints of blood were found in the peritonealcavity. There were numerous nodules throughout themesenteric blood vessels and one of these had rupturedinto the mesentery causing an extensive haematomawhich had then burst through into the peritoneal cavity.This bleeding had also extended into the wall of theadjacent segment of small intestine measuring about 8 in.in length. Other parts of the small intestine showedareas of slight greyish discoloration and numerous newblood vessels had developed under the serosa of thesmall intestine adjacent to the mesenteric nodules. Thehaemorrhagic portion of intestine was excised and anend-to-end anastomosis performed.During the following two weeks the blood pressure

gradually fell to about 140/100 mm. Hg. Colickyabdominal pain of gradually decreasing severity con-tinued, together with considerable abdominal distension.Profuse diarrhoea occurred one week after the intestinalresection, and a week later a faecal fistula developedthrough the abdominal wound. This fistula closedspontaneously 10 days later. Occasional bouts ofintestinal colic continued until the time of the patient'sdischarge from hospital.Within a few days the patient was admitted to another

hospital with a diagno3is of acute intestinal obstruction,and a further laparotomy was performed on October 1,1956. Numerous adhesions were present and inad-vertently the bowel was damaged, necessitating theresection of a short length of ileum. The subsequentconvalescenc. was uneventful.

PATHOLOGYThe specimen from the first laparotomy consisted of a

portion of small intestine measuring 25 cm. in length(after fixation), together with some attached mesentery.A large amount of blood clot was found in the subserouslayer over most of the length of the specimen, and therewas also considerable haemorrhage into the mesentery.When the bowel was divided along its long axis thelumen was found to be narrowed by the subseroushaemorrhage (Fig. 1), but no part appeared necrotic organgrenous. At the mesenteric border of the intestinemany of the terminal branches of the mesenteric arteryshowed numerous small aneurysms which measured upto 0.6 cm. in diameter and contained recent thrombi.

HISTOLOGYSections of the terminal mesenteric arteries showed

aneurysmal dilatation, occlusion by recent thrombi andan intense necrotizing arteritis (Fig. 2). The normalarchitecture of the vessel walls had been entirely lostand replaced by fibrinoid material and cellular inflam-matory exudate consisting mostly of neutrophil poly-morphs. Sections of the bowel wall also showed a similarnecrotizing arteritis affecting many of the submucousvessels (Figs. 3 to 6).The normal architecture of these vessels was largely

replaced by fibrinoid material intermingled with redx

blood cells and inflammatory cells. The inflammatoryexudate, which consisted of large numbers of neutrophilpolymorphs with lesser numbers of eosinophils andhistiocytes, extended widely around the affected arteries.Perivascular proliferation of fibroblasts was also evident.Thrombi and aneurysmal dilatations were seen occa-sionally and fragmentation of the internal elastic lamina(Fig. 5) was another feature. The necrotizing processwas sometimes seen to be focal, affecting only part of avessel wall (Fig. 6), while some of the submucousarteries and arterioles appeared to be completely normal.The veins also showed comparatively little involvement(Figs. 4 and 6). The gut wall itself showed no evidenceof infarction, the mucosa being well preserved andfree from ulceration.A histological section of the portion of ileum removed

at the second laparotomy (eight weeks after the firstintestinal resection) was examined and showed medialmuscular hypertrophy of the submucous arteries, butno changes resembling healed or recent polyarteritisnodosa. There was also evidence of a resolving peri-tonitis, but the bowel wall itself appeared healthy withno suggestion of old or recent infarction.

DISCUSSION

The aetiology of the arterial necrosis in the fivecases previously reported and referred to earlierhas already been extensively discussed by theauthors concerned. The sudden increase in theblood pressure, together with the occurrence of ahypersensitivity reaction, have been thought to bedefinite factors in the production of the lesion.Lober and Lillehei, in commenting on their twocases, conclude that

" the sudden increase in intravascular strain appears tohave been the precipitating influence in producing thearteritis in these cases. It is evident that the changesin arterial blood pressure after coarctation repair mustbe accompanied by an abnormal response of thepatient to produce a necrotizing arteritis. Hyper-sensitivity may be one factor in this altered response."

Perez-Alvarez and Oudkerk, in discussing theircase, state that

" the sudden release of a high pulsating pressure, towhich the vessels below the coarcted area had not beensubjected, represents trauma. In the case reported,the abdominal vessels responded to this trauma withnecrosis which was localized to the media of the smallarteries and arterioles."

Benson and Sealy interpret the arterial necroses intheir cases" to be the result of changes in circulatory dynamicsfollowing the resection of the coarctation. Perhaps themost important of these are the sudden increase insystolic pressure and the change in character of thepulse wave."

In support of the view that changes in circulatorydynamics are responsible for the necrotic arteriallesions is the fact that in the three cases that came

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FIG. 1.-The excised portionof small intestine, sec-tioned to show anextensive subserous andmesenteric haemorrhagewith narrowing of thebowel lumen. Aneurys-mal dilatation andthrombosis ofthe termi-nal mesenteric arterialbranches can also beseen.

FIG. 2.-Section of smallintestine showinganeurysmal dilatation,thrombosis, and necro-tizing arteritis ofmesen-teric vessels. The in-flammatory process canalso be seen involvingthe small submucousarteries. (Haematoxylinand eosin x 8.)

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INTESTINAL VASCULAR LESIONS SIMULATING POLYARTERITIS NODOSA 261

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FIG. 4.-Necrotizing arteritis affecting a submucous artery with little involvement of the accompanying vein. (Haemnatoxylinand eosin x 160.)

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R. L. HURT and W. J. HANBURY

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FiG. 6.-A subserous artery showing focal inflammatory necrosis. The accompanying veinis unaffected. (Haematoxylin and eosin x 60.)

262

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INTESTINAL VASCULAR LESIONS SIMULATING POLYARTERITIS NODOSA

to necropsy the lesions were limited to the vesselsoriginating distal to the coarctation repair (Loberand Lillehei; Perez-Alvarez and Oudkerk; Bensonand Sealy). As regards the actual changes inbrachial arterial blood pressures before and afteroperation, Ring and Lewis found that " generallythe patients developing abdominal complicationsseemed to have an earlier and more marked dropin pressure." This is not apparent, however, in thefive cases of arterial necrosis revealed at laparotomy,and in the present case the brachial blood pressuresactually rose after resection of the coarctation andonly fell slowly after the subsequent laparotomy.The present case does not differ in any great

respect from the five previously reported; althoughareas of greyish discoloration were seen in thesmall intestine at operation, no infarction wasdem.onstrated pathologically in spite of the extensivevascular lesions. All the other five reported casesshowed intestinal infarction of varying degree.Another interesting feature of the present case isthe focal nature of the arterial necrosis, somevessels showing involvement of only parts of theirwalls. This was also noted by Lober and Lilleheiin their cases.

Perhaps the most striking feature in the presentcase is the close resemblance of the arterial lesionsto those found in polyarteritis nodosa. Althoughno definite conclusions can be drawn, the evidenceprovided by this post-operative complication mustlend weight to the idea that excessive vascularstrain plays some part in the aetiology of poly-arteritis nodosa.The occurrence of arterial necrosis as a com-

plication of an operation for coarctation of theaorta is of considerable clinical importance. Aspreviously stated, the abdominal pain that mayoccur may be of such severity as to simulate anacute abdominal emergency, with the result thatseveral of the reported cases have undergone alaparotomy with negative findings. Operation isnecessary only if an intraperitoneal haemorrhageoccurs, as in the case reported, or if the arterialnecrosis is of such severity as to lead to a localizedarea of gangrene and consequent perforation.

In 17 consecutive cases of resection of coarctationof the aorta, there have been two patients in whomsevere abdominal pain has developed post-opera-tively. One of these patients has been described indetail above, and the second patient, aged 12 years

whose pre operative blood pressure was 190/100 mm.Hg and in whom the blood pressure remainedelevated for several days after operation, developedmarked abdominal pain three days post-operatively.This pain was aching in character and was suffi-ciently severe to cause considerable concern. Thepain subsided spontaneously during the followingweek and has not recurred during the two yearssince operation. It is highly probable that the painwas due to the development of areas of arterialnecrosis consequent on the continued elevation ofblood pressure in the mesenteric vessels.

In those cases in whi h the blood pressure remainselevated following a successful operation for coarc-tation of the aorta, the use of a hypotensive drugsuch as " arfonad" may be advisable in order toreduce the blood pressure and prevent the occur-rence of arterial necrosis in the distal vessels dueto their sudden exposure to a high blood pressure.It has been noted by Brock (1957) that reactivehypertension may occur post-operatively after theuse of " arfonad " during operation, but thisobservation would not seem to preclude the use ofthis or any other hypotensive drug during thepost-operative period if the blood pressure remainselevated.

SUMMARYA case of intestinal arterial necrosis and haemor-

rhage after operative repair of coarctation of theaorta is described. Only five similar cases werefound in the literature, and these are brieflyreviewed. The condition is considered to be ofimportance both on account of its clinical implica-tions and because of the close resemblance of thelesions to those of polyarteritis nodosa.

We should like to thank Mr. 0. S. Tubbs for hispermission to publish this case and for his most valuablecriticism. We are also grateful to Professor J. W. S.Blacklock for his helpful advice, to Mr. N. K. Harrisonfor the photograph of the small intestine, to Mr. J. W.Miller for histological sections, and to Dr. G. S. Sansomfor the photomicrographs.

REFERENCESBenson, W. R., and Sealy, W. C. (1956). Lab. Invest., 5, 360.Brock, R. C. (1957). Guy's Hosp. Rep., 106. 131.Cleland, W. P., Counihan, T. B., Goodwin, J. F., and Steiner, R. E.

(1956). Brit. med. J., 2, 379.Lober, P. H., and Lillehei, C. W. (1954). Surgeryv, 35, 950.Perez-Alvarez, J. J., and Oudkeik, S. (1955). Ibid., 37, 833.Ring, D. M., and Lewis, F. J. (1956). J. thorac. Surg., 31, 718.Sealy, W. C. (1953). Surg. Gynec. Obstet., 97, 301.

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