855

for this being previous thrombosis of the femoral oriliac vein on the non-cedematous side with establish-ment of a collateral circulation, and congenital venousabnormalities.The collateral circulation to the superior vena cava

has been well described by Welch (1909) and Keen (1941),the superficial veins concerned being the inferior and

superior epigastric, the long thoracic, the superficialcircumflex iliac and the external pudendal, and the deep,the azygos, hemiazygos, and lumbar veins. There maybe also some return via the liver, through the anastomosesbetween the tributaries of the portal vein and inferiorvena cava. Engorged superficial ’veins are not alwaysfound at arr early stage and may even be absent through-out, a very efficient deep collateral circulation sufficing.In the present case they were almost within the normalrange up to the time of death. In difficult cases abnormalvenous channels may be demonstrated by infra-redphotography and venography (Stowers and Grossman1946). The enlarged left ascending lumbar vein seen inthe venogram in our case may perhaps be the " leftinferior vena cava," the embryological counterpart ofthe normal right inferior vena cava (Keen 1941).The results of therapeutic ligation of the inferior vena

cava are of interest. It was probably first performedby Trendelenburg in 1911 in a patient with puerperalsepsis and has been used more often in recent years toprevent repeated pulmonary embolism. It is stated bysome that the procedure does not - cause more than

slight disability and this only in a few cases, oedemabeing absent or transitory and enlarged collateral veinsonly rarely developing (Wakefield and Mayo 1934). Others,however, have found troublesome cedema more often.

The difficulty of diagnosis in many cases of inferiorvena caval obstruction is evident when it is consideredthat it was made during life in only 4 of 18 of Pleasants’scases from the Johns Hopkins Hospital, in 1 of 15

reported from the Mayo Clinic (Judd and Scholl 1924),and in 2 of 10 in another series (Ney 1946).Certain complications of obstruction of the inferior

vena cava may cause death. It would be expected thatif the renal veins were occluded heavy albuminuria,perhaps hsematuria, and renal failure would developand this may happen if the obstruction occurs suddenly ;but otherwise albuminuria appears to be uncommon(Welch 1909), and the anastomosis of veins in the renalcapsule with tributaries of the azygos usually enables thekidneys to function normally. This was shown in rats

by Addis and Lew (1939) who tied off the inferior venacava above the level of the renal veins and found thatmany of the animals recovered from the uraemia thatusually resulted. Although in our patient the tumourextended both above and below the entrances of the renalveins, albuminuria was never found. Extension of the

, tumour to involve the hepatic veins may lead to a formof Chiari’s syndrome, with tender enlargement of theliver, jaundice, ascites, and splenomegaly. Hepaticenlargement was noted 2 days before our patient died,and at post mortem there was considerable venous

congestion of this organ with centrilobular necrosis,although the tumour did not actually block the hepaticveins. Nevertheless, uniform red clots were found inthem and it is conceivable that death was in fact dueto hepatic failure.Leiomyoma is a fairly common tumour, being found

in the gastro-intestinal and genito-urinary tracts, andthe uterus, breast, and skin. Leiomyosarcoma, on theother hand, is more rare. It has been reported as aprimary tumour in the stomach, duodenum (Golden

, and Stout 1941), uterus, kidney, and mediastinum(Willis 1948). We have studied the published reportsand been able to find only one definite case of leiomyo-sarcoma arising from a vein. This was a case reported

by Puig-Sureda et al. (1947) in which the tumourarose from the superior left colic vein. The only reportsof primary tumours of the inferior vena cava seem tobe two in the German literature of the last century.One of these (Perl 1871) caused complete obstruction ofthe vein below the hepatic veins, and distended thevessel like the tumour in’our patient ; it was histologi-cally a spindle-celled sarcoma but contained smoothmuscle fibres, and Virchow seems to have consideredthat it could be called a myosarcoma. The other wasthat of Unruh (1896), an endothelioma.

SUMMARY

A case of leiomyosarcoma of the inferior vena cavais described. The clinical aspects of obstruction of thisvein are discussed. Smooth muscle tumours are brieflyreviewed, with special reference to those of venous

origin.Our thanks are due to Dr. A. H. Holmes for his interest

and advice, and for permission to publish this case, and alsoto Prof. A. M. Boyd. We also wish to thank Prof. S. L.Baker for his advice and criticism, and Dr. L. Meana for helpwith Spanish publications. We are indebted to Miss D.Davison for fig. 2, and Mr. F. Ward for fig. 3.

REFERENCES

Addis, T., Lew, W. (1939) Proc. Soc. exp. Biol. N.Y. 42, 602.Battle, W. H. (1887) St Thom. Hosp. Rep. 17, 49.Golden. T., Stout, A. P. (1941) Surg. Gynec. Obstet. 73, 784.Judd, E. S., Scholl, A. J. (1924) J. Amer. med. Ass. 82, 75.Keen, J. A. (1941) Brit. J. Surg. 29, 105.Ney, C. (1946) J. Urol. 55, 583.Perl, L. (1871) Virchows Arch. 53, 378.Pleasants, J. H. (1911) Johns Hopk. Hosp. Rep. 16, 36.Puig-Sureda, J., Gallart-Esquerdo, A., Roca de Vinals, R., Salleras

Llinares, V. (1947) Med. Clin. Barcelona, 8, 104.Stowers, J. M., Grossman, M. E. (1946) Lancet, ii, 868.Unruh (1896) Dtsch. med. Wschr. 22, 746.Wakefield, E. G., Mayo, C. W. (1934) J. Amer. med. Ass. 31, 92.Welch, W. H. (1909) Thrombosis, in Allbutt and Rolleston’s

System of Medicine. London; vol. VI, p. 751.Willis, R. A. (1948) Pathology of Tumours. London.

MELÆNA FROM HETEROTOPIC GASTRIC

MUCOSA IN THE ILEUM

EDRIC WILSONF.R.C.S.

SURGEON, PLYMOUTH, SOUTH DEVON, AND EAST CORNWALLGENERAL HOSPITALS GROUP

MELaeN A as a symptom of ulceration of gastric mucosain Meckel’s diverticulum is well known, but heterotopicgastric mucosa in the small intestine seems to be rare.Brown and Pemberton (1936) state that solitary ulcer

of the ileum has been noted not - infrequently-4examples from necropsy in fifteen years, and 10 clinicalcases, the patients being aged 20-60. The common

symptoms were abdominal pain in the umbilical areaand pallor. The ulcer was found in the terminal5-90 cm. of the ileum in 9 cases, and midway between theduodenum and the caecum in 1 case. Microscopicallythey were simple ulcers with or without a tendency toannular constriction. Brown and Pemberton note a

similarity in symptoms to cases of bleeding Meckel’sdiverticulum but mention no case of gastric mucosa in adiverticulum associated with a simple ulcer in theileum.

Because of the similarity in symptoms the followingreferences to heterotopic gastric. mucosa in Meckel’sdiverticulum are of interest. Hallendorf and Lovelace

(1947) describe ulceration in -Aleckel’s diverticulum causedby heterotopic gastric mucosa. Sibley (1944) states thatMeckel’s diverticulum is the most common site of hetero-

topic gastric mucosa, and thinks that the mucosa of theintestine becomes progressively more sensitive to hydro-chloric acid and pepsin the farther it is from the stomach.He uses the term " dyspepsia 11eckeli

" to describe theclinical picture caused by ulceration in the diverticulumor adjacent ileum. Maddock and Coventry (1941)

856

describe perforation of the ileum opposite a Meckel’sdiverticulum. Matt and Timpone (1940) found thatheterotopic mucosa was often present in the neck of thesac and extended into the adjacent ileum, and regard itas a disease of childhood.

CASE-RECORD

A married woman, aged 46, was admitted under my careon March 3, 1949, having passed a considerable amount ofblood per rectum four hours before admission. She was

previously admitted to a medical ward in 1947 with the

history that in 1944 she had undergone hysterectomy forfibroids ; for a few months she had had a nagging painin the lower abdomen, and on two occasions had passed bloodper rectum ; and on the day of admission she had faintedfive times following a sudden urge to defsecate, and passageof a great deal of blood. On admission there were no abnormal

physical signs in the abdomen, but dark-red blood was present

Fig. I-External view of portion of ileum removed.Fig. 2-internal view of portion of ileum removed.-

in the rectum. The haemoglobin was 55%. Palliativetreatment was given. A fortnight later a sigmoidoscopyshowed no abnormality. After a month in hospital, in whichthere was no obvious bleeding but occult blood was foundin the stools, she was discharged.On readmission in 1949 she had been having " windy "

pains in the lower abdomen for several weeks and passingmuch wind. There was some tenderness over the descendingcolon with general distension of the abdomen, and shortlyafter admission she passed another 6 oz. of blood. She wasgiven enough morphine to keep her sleepy and the bleedingsoon ceased.

Progress.-Three days after admission there was very slighttenderness just to the right of the umbilicus. There was no

history suggesting peptic ulceration, and the appendix hadbeen removed at the previous operation. Haemorrhage fromulceration in a Meckel’s diverticulum was considered probableand, since the bleeding had been recurrent, unexplained, andlikely to endanger life, exploration was thought advisable.

Oe/’oo.—Laparotomy through a right paramedianincision showed that the appendix had been removed, therewas no sign of gastric or duodenal ulcer, and no Meekel’sdiverticulum was found. In the ileum, 18 in. from the ileo-caecal junction, three constricting rings reduced the diameterto about half the normal (fig. 1) ; the pathology was not clear,but it did not look like regional ileitis. Eight inches ofileum was resected and end-to-end anastomosis performed.No other abnormality was found in the abdomen. The

patient made an uninterrupted recovery, and she remainswell.Pathology.-The specimen removed at operation was a

piece of small intestine 4 in. long with mesentery attached.It had an hour-glass constriction 1 in. long, marked externallyby three narrow circular whitish bands just visible in theserosa, 1/2 in. apart. Running transversely across the gut inthe middle of the constricted area was an annular groove-like defect in the mucosa about 2 mm. wide. The edges of thedefect were regular, and its floor was formed by smoothshiny cedematous submucosa. The wall of the gut deep to thedefective part was just perceptibly indurated. On eitherside of the groove the mucosa was hypertrophic and presentedan irregularly folded pattern quite distinct from the regularring-like folds of normal mucosa. At one point 1/4 in. away

Fig. 3-Mucosa near annular defect. (x 30.)

from the groove there was a localised cyst-like patch ofoedema 1 cm. in diameter in the submucosa. This could befelt more easily than seen (fig. 2). The mucosa over ithad split to expose the submucosa, but this was probablyan artefact. Both the mucosa and the external surface ofthe gut were of normal colour, and there was no evidence ofinflammation or inflammatory ulceration, but the vesselsof the serosa in the region of the annular defect were justperceptibly congested. The mesentery appeared normal.

Histology.-The ulcerated area resembled gastric mucosa(fig. 3). Mr. L. W. Proger, pathological curator of the RoyalCollege of Surgaons, commented as follows : "The sectionshows undoubted gastric mucous membrane with simpleulceration. These patches of heterotopic gastric mucosaare usually associated with some congenital malformation,such as atresia, reduplication, Meckel’s diverticulum, or

enteric cyst. I have not yet seen one associated withcongenital stricture, as in your case."

I am indebted to Dr. E. Wordley and Dr. M. R. Thomas fortheir assistance in preparing this article.

REFERENCES

Brown, P. W., Pemberton, J. de J. (1936) Proc. Mayo Clin. 11, 259.Hallendorf, L. C., Lovelace, W. R. (1947) Ibid, 22, 53.Maddock, W. G., Coventry, M. B. (1941) Surg. Gynec. Obstet. 73, 105.Matt, J. G., Timpone, P. J. (1940) Amer. J. Surg. 47, 612.Sibley, W. L. (1944) Arch. Surg. 49, 156.

PEMPHIGUS ACUTUS TREATED WITHANTIBIOTICS

THERESA LAZARM.B. Birm., M.R.C.P.

MEDICAL REGISTRAR, QUEEN ELIZABETH HOSPITAL, BIRMINGHAM

THE distinction between different forms of pemphigusis not always clear-cut. Pemphigus acutus, however, isgenerally regarded as a definite entity (Sutton andSutton 1939, MacKenna 1945). It occurs mostly in

people whose work involves handling animal products,although cases have followed vaccination (Howe 1903).It is probably an infection caught through some breachin continuity of the skin, since many patients give ahistory of a cut or infective lesion on the fingers. Adiplococcus was isolated from the lesions by Demme(1886), Claessen (1893), and Bulloch (1895), but this hasnot satisfied Koch’s postulates. More recently a virusaetiology has been suggested (Markolf and Knauer 1939).Pemphigus acutus resembles pemphigus vulgaris in

many respects, but runs a more rapid course. It has a

mortality of 50-70% (MacLeod 1933, MacKenna 1945).Hitherto no treatment has been satisfactory.

Since the advent of antibiotics penicillin has beentried in various forms of pemphigus without success

(Kolmer 1947, Florey et al. 1949, Spink and Hall 1945),but I have been unable to find any specific reference toits use in pemphigus acutus.

Recent encouraging reports by Philip (1949), Bettley(1950), and AV, hittle (1950) on the use of ’ Aureomycin’