CHRONIC
INFLAMMATORY TUMORS OF THE ILEOCECAL SEGMENT* WITH RALPH
CIinicaI Associate in Surgery,
H.
CASE REPORT FOUSER,
F.A.C.S.
Rush MedicaI CoIIege of The University CHICAGO,
I
M.D.,
of Chicago
ILLINOIS
or “pseudotumors” of the bowe1 were described by Virchow in 1853. Thereafter reports of cIinica1 cases became numerous in the Iiterature. The case reports of the British surgeons Moynihan, Mayo- Robson and DaIzieI caIIed attention to these tumors. Braun added further to this study by his report .. “Uber entzundIiche GeschwiiIste am Darm.” Lawen made an extensive study and advanced the view that “the majority of chronic inffammatory tumors of the cecum arise from a chronic fibropIastic inffammation of the appendix.” Moschowitz and WiIensky in 1923 estabIished chronic nonspecific inflammation of the intestines as a cIinica1 and pathoIogic entity. Bouman in 1924 reviewed the Iiterature up to that date. James states that DaIzieI in 1913 was aware of an entity which he described as “chronic interstitia1 enteritis.” LittIe attention was focused on the condition, however, unti1 1932, when Crohn, Ginzburg and Oppenheimer described a subacute or chronic i nffammatory condition affecting the termina1 portion of the smaI1 intestine which they termed “regiona ileitis.” He further states that several contributions have appeared since, but very IittIe has been added to our knowIedge of the disease. In most of the cases described, the Iesion has shown a prediIection for the termina1 portion of the ileum, but any part of the intestina1 tract may be invoIved; there have been very few exampIes of isoIated invoIvement of the coIon. RaIphs states that foIIowing the report of Crohn, Ginzburg and Oppenheimer in 1932, * From the Surgical Service, West Suburban HospitaI,
a sequence of case reports by many writers has appeared with a muItipIicity of titles, e.g., “IocaIized hypertrophic enteritis ” (Jackman), “chronic hyperpIastic enteritis,” “ chronic cicatrizing enteritis ” (Barbour and Stokes), and so forth. More recentIy Lawen has added to his earIier studies and reports by stating that microscopic features of the entity which he described in 1914 under the name fibropIastic appendicitis are identica1 with those of termina1 iIeitis described in 1932 by Crohn, Ginzburg and Oppenheimer. Lawen had stated in his description that fibropIastic appendicitis may invoIve the ceca1 waI1 and the termina1 portion of the iIeum and thus Iead to a secondary termina1 iIeitis. He concIudes that the tumorforming, chronic stenosing, uIcerative or nonuIcerative iIeitis and fibropIastic appendicitis and the anaIogous disorders of the ascending coIon present an identica1 microscopic picture. They represent the same type of inflammation with a different primary IocaIization. It is a we11 recognized fact that chronic nonspecific inflammation may occur in any portion of the gastrointestina1 tract. However, it occurs most frequentIy in the iIeohas stated two ceca1 region. Larimore reasons for such frequent prediIection for this region. First, the cecum and cecocoIic segments of the Iarge intestine are subject to inffammatory reactions of degrees varying from simpIe catarrha1 irritations to extensive uIcerations by bacteria1 invasion of the waIIs. SecondIy, most bacteria1 growths are due to sIow segmenta transport and greater ffuidity in the iIeoceca1 segment.
NFLAMMATORY
Oak Park, HospitaI. 124
IIIinois. Read before the Surgical Staff of the
CASE
REPORT
Id. A. V., aged 26, a housewife, compIained of pain in the Iower abdomen. Two months before she had had a first attack of severe pain in the region beIow the umbilicus and in the Iower right abdomen. The attack lasted about three days and was not accompanied by nausea or vomiting. The pain had no relation to food intake or her menstruaI period. Since this attack there had been several others at four to five day intervals which lasted for a few days. Soreness in lower abdomen persisted between attacks. The pain was aggravated by waIking. There had been considerabIe constipation and bowel distress. The patient further stated that for about three years she had had miId attacks of pain in the Iower right quadrant with gastric upsets. The past history was essentiaIIy negative. PhysicaI examination revealed an obese, welI-developed white femaIe, with temperature of 100.4 degrees and purse of 92. Abdominal palpation reveaIed a heavy panniculus and symmetrical abdomina1 wall. A tender, fixed mass about the size of a small grapefruit could be paIpated in the region just beIow and to the right of the umbilicus. BimanuaI examination reveaIed an infantiIe uterus, a mass in the Iower right abdomen and peIvis which aIso involved the right uterine tube and the right ovary. The bIood count was within normal limits. The diagnosis made was chronic inflammatory tumor of the cecum, appendix, right uterine tube and right ovary. Operation. A right paramedian incision was made from a point lateral to the umbiIicus dou-n to the pubic region. The deep fascia was covered with a layer of subcutaneous fat of approximately IO cm. thickness. The fascia was incised and the edges dissected from the underrying muscIe tissue. The recti muscles were separated and the peritoneum incised to the right of the midline. The Iower portion of the greater omentum was found to be cIoseIy adherent over the anterior and media1 surfaces of the tumor mass. The omentum was cramped, cut and ligated and the adherent mass of distaI omentum carefuIIy dissected free from the underlying tissue mass. The abdomina1 and pelvic cavities were expIored and the mass outlined in the ceca1 region which involved the cecum, terminal Ioops of ileum, appendix,
mesentery, IateraI pelvic waI1, right tube and the right ovary. Upon examination it was found that the left tube was essentially negative, the Ieft ovary was smaI1 and fibrotic, and the uterus was infantiIe in size and outline. AII structures invoIved in the mass were closely adherent to each other by the infiltration and abundant overgrowth of dense white fibrous connective tissue. The right tube and Iigament of the right ovary were dissected free from the mass by finger dissection, cIamped with considerable dificuIty, cut and ligated with fixation sutures of No. I pIain catgut. The herniated loop of the termina1 ileum was then carefully dissected free from the position of its attachment under the mass and above the region of the right tube. This loop of iIeum was partiaIIy obstructed due to its herniated position. The most dista1 loop of termina1 ileum was separated from the media1 surface of the mass by finger dissection. Dense white fibrous connective tissue abundant in quantity characterized a11 the adherent attaching tissue. The appendix was Iocated with difhculty in a retroceca1 position, intimateIy reIated to the waI1 of the mass. Owing to its Iarge size and hard, fibrous consistency, it was separated with great diff&Ity from the cecum; an appendicea1 artery was cut and ligated with fixation suture. It was not possible to Iigate the appendix because of its size and the density of the tissue. The appendix was removed at its base from the waI1 of the cecum, which was very friabIe, markedly thickened and firm in character. A cecostomy was done by the insertion of a rubber tube in the appendiceal opening of the cecum, fixed by a pursestring and interrupted sutures of chromic No. I catgut. AI1 raw surfaces were peritoneaIizec1 as much as possibIe. The omenta1 tissues were drawn down and placed in the region of the cecostomy. A rubber Penrose drain was inserted to the right pelvic region. The peritoneum was cIosed with pIain No. I catgut. Four silkworm gut retention sutures with buttons were pIaced in the skin, subcutaneous and fascia1 tissue. The fascia was cIosed with chromic No. I catgut. Two thin rubber tissue drains were placed in the subcutaneous tissues, one at each extremity of the incision for fat drains; four interrupted subcutaneous sutures of pIain No. I catgut were inserted. The skin was cIosed with interrupted silkworm gut sutures. The cecos-
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tomy extended through the midincisiona1 region as the immobihty of the cecum prevented a right stab incision in the IateraI abdomina1
Tumor
JULY, rgqo
a herniated position under the mass and above the uterine tube. This Ioop was partiaIIy obstructed; no gangrenous areas were present.
FIG. z. CecaI tumor and markedIy enlarged and adherent appendix. The adhesions have been dissected, releasing the adherent terminal iIeum from the tumor mass. The Ioop of iIeum is elevated to expose the appendix which is in a retrocecal position. The right uterine tube and right ovary have been removed.
FIG. I. Drawing of tumor mass in ceca1 region. The termina1 iIeum is adherent to the ceca1 tumor. A portion of the termina1 ileum is herniated and partially obstructed. The right uterine tube and right ovary are adherent to the tumor mass. Dense fibrous connective tissue adhesions are shown which attach the tumor mass to the parieta1 peritoneum and peIvic waI1. The adherent Iower portion of the omentum has been clamped and cut.
wall. A silkworm gut retention suture was used at the skin margin of the incision to hold the rubber tube in position. removed Pathologic Report. The specimen consisted of a hard mass the size of a small grapefruit in the ceca1 region, which extended medialIy to the midabdomina1 region. It consisted of a markedIy enlarged cecum with the Iower portion of the greater omentum adherent to the underIying mass. The ceca1 waIIs were much thickened and indurated; the terminal Ioop of the iIeum was adherent to the mass; the appendix with no apparent Iumen except at its base was markedly enlarged and densely fibrotic. The appendix was in a retroceca1 position and cIoseIy adherent to the ceca1 waI1 by an overgrowth of dense fibrous connective tissue. The mesoappendix had been repIaced and obIiterated by dense white fibrous connective tissue. A Ioop of the termina1 &urn was in
The mass was adherent to the IateraI peIvic waII by the infiltration of dense fibrous connective tissue which extended downward to the region of the ihac vessels. The mesenteric tissues were thickened and very firm and noduIar due to the fibrous infiItration. Sections of the appendix and tube reveaIed the characteristic appearance of a chronic inffammatory Iesion. There was an abundance of fibrous connective tissue which had repIaced the tissues which make up the waIIs of the appendix and intestine. This repIacement was most evident in the submucosa and muscularis Iayers. The tissues were invaded by neutroeosinophiIes and Iymphocytes. The philes, sections of the mesentery and ovary presented the same pathoIogic changes-those of chronic proliferative inff ammation. The patient made an excehent recovery. The ceca1 tube was removed the tenth day. There was a smaI1 amount of drainage from the site of the tube for a number of days, after which time the opening in the tissues closed. The patient has had no distress; her appetite is good and her boweIs reguIar. The patient’s heaIth has been exceIIent since operation. Etiology and Pathology. James states that nothing definite is known regarding
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the etiology of the disease. AIthough it is quite generahy agreed that the origin is infectious, no specific organism has been recovered from the feces, the affected intestina waI1, or the Iymph glands. Braun has cahed attention to the fact that chronic nonspecific inflammatory tumors of the intestinal tract may be caused by streptomycosis as we11 as staphyIomycosis and infections with the Bacillus cob. MaiIer reported Streptococcus viridans as a possible infective agent. It is we11 recognized that Streptococcus viridans is capable of producing a reIativeIy Iow-grade infIammation simiIar to that present in these tumor masses. RaIphs emphasizes the frequency of a lesion of the mucosa as the porta of entry for the infection. He further states that through some break in the mucosa the submucosa is invoIved and becomes the seat of a marked inflammatory reaction with round-ceIIed infiItration. Although the path of the infection may be via the mucous membrane, it is possibIe to be from without, as well. The muscularis may be penetrated. MuraI inhItration may be compIete, and an infiItrated and thickened serosa may cause contact adhesions to the parietes, adjacent organs, neighboring Ioops of bowe1, or the retroperitoneum. Kirschner and Nordmann present further etiologic evidence, quoting the view of Lawen that “the majority of chronic inflammatory tumors of the cecum arise from a chronic IibropIastic infIammation of Their own observations the appendix.” confirm these facts in that chronic appendicitis of sIow deveIopment may transform the cecum into a tumor of wooden consistency and fuse the cecum to the retroperitonea1 tissues by a cartiIaginous induration. It may be stated that the Iiterature is in quite genera1 agreement in the following fact, nameIy, the proIiferating fibropIastic tumors in the iIeoceca1 region are the result of a low-grade inflammatory process insidious in nature and extending over a period of months or years.
Tumor
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Diagnosis. The differentia1 diagnosis may present a very difficult problem. The specific inflammations (tubercuIosis, syphiIis, actinomycosis) and maIignant disease may cause intestina1 tumor masses which are difficult to differentiate from nonspecific inflammatory masses before operative expIoration. PathoIogic examination and histologic studies are the determining factors in the more complicated cases. Treatment. The operative procedure must be based upon the extent of the pathoIogic invoIvement and the judgment of the operating surgeon. RaIphs has stated that resection of the tumor mass is the idea1 course, but this is often impracticabIe due to the presence of adhesions to adjacent structures and the retroperitoneal tissues. Meyer and Rosi have resected wherever practicabIe. Short circuiting anastomoses have been empIoyed with success by Hodgson. Cases of a less involved nature have been reported where spontaneous recovery has taken pIace foIIowing simpIe expIoration. SUMMARY
A brief review has been made of the Iiterature on chronic infIammatory tumors of the iIeoceca1 region. A case has been recorded of an iIeoceca1 tumor mass which presented an extensive invoIvement of the adjacent structures as the resuIt of a chronic proIiferative inflammation. The mass was not resectabIe. The appendix, ovary, uterine tube and a portion of the mesentery were surgicaIIy removed. A cecostomy was done for drainage of the cecum. REFERENCES
BARBOUR, R. F., and STOKES, A. B. Lancer, I: 299, ‘936. BOUMAN, H. A. H. FibropIastic typhlitis and appendicitis: pseudo or chronic inflammatory tumors of the iIeoceca1 region. Journal Lancet, 45: 264-270, 1924.
BRAUN, H. ijber entziindIiche Geschwiilste am Darm. Deutsche Ztscbr. f. Cbir., IOO: I, 1909. Arch. f. klin. Cbir., 13: 742-759. 1909. CROHN, B., GINZBURG, L., and OPPEKHEIMER, G. D. J. A. M. A., 99: 1323, 1932.
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DALZIEL, T. K. &it. M. J., II: 1068, 1913, HODGSON,J. C. Lancet, I: 926, 1937. JACKMAN, W. A. Localized hypertrophic enteritisas a cause of intestina1 obstruction; with a report of two cases. Brit. J. Surg., 22: 27, 1934-1935. JAMES, T. G. I. Chronic regiona cohtis. Brit. J. Surg., 25: 99. 51 I, ‘938. KIRSCHNER, M., and NORDMANN, 0. Cbirurg., 5: 982, 1927. LARIMORE, J. W. IIeocecaI segment. J. Missouri M. A., 34: 48-53, 1937. LAWEN. A. FibrooIastic aooendicitis. chronic stenosine termina1 ileiiis and nonspecific inff ammatory ileoceca1 tumors. Zentralbl. f. Cbir., 65: 91 I, 1938. LAWEN, A. Ueber Appendicitis FibropIastica. Deutscbe Ztscbr.f. Cbir., 124: 221, 1914. A
1
Tumor MAILER, R. Acute regiona iIeitis. Brit. J. Surg., 25: 99, 5 17, 1938. MAYO-ROBSON, A. W. An address on some abdomina1 tumors simmating maIignant disease and their treatment. Brit. M. J., I: 425, rgo8. MEYER, K., and Rosr, P. RegionaI iIeitis (nonspecific). Surg., Gynec. @ Obst., 62: 977-988, 1936. MOSCHOW~TZ, E., and WILLENSKY, 0. 0. Am. J. SC., 166: 48-66, 1923. MOYNIHAN, B. Mimicry of malignant diseases in the Iarge intestine. Edinburgb M. J., Dec. rgo6. RALPHS, F. G. Chronic inffammatory tumors of the gastrointestinaI tract. Brit. J. Surg., 25: gg, 524, 1938. VIRCHOW. Vircbow’s 1853.
Arch.
f.
patb.
Anat.,
PERIARTERITIS nodosa is a rare form of arterial disease. . . . It is presumabIy an infectious disease of the medium sized and smalIer arteries, characterized by fever, pain, and noduIes along the course of the vesseIs. From-“ Peripheral VascuIar Diseases” by Kramer (BIakiston).
Bd.
5,