Open communication between appendiceal mucocele & cecum

OPEN COMMUNICATION MUCOCELE RICHARD

BETWEEN APPENDICEAL t3 CECUM”

A. LIPVENDAHL,

M.D.,

AND EMIL RIES, M.D.

CHICAGO

T

conception of the pathogenesis of IocaIized or diffuse distention of the appendix by thick geIatinous mucoid materia1 has practicaIIy aIways presupposed compIete or almost compIete occIusion of the Iumen proxima1 to the diIated portion, absence of pathogenic organisms, and an actively secreting mucosa. In most cases reported no mention is made as regards the patency of the proxima1 portion of the organ and attention has been concentrated upon the method of escape of the jeIIy-like Iiquid into the abdomen, and its reIation to pseudomyxoma peritonei. That a mucoceIe can form without compIete occIusion at the proximal end is proved by Vorhaus who described the process in a forty-six-year-oId woman giving a history of severa attacks of pain and a smaII paIpabIe mass in the right Iower abdomina1 quadrant which upon severa occasions showed an irreguIarIy bariumfiIIed appendix that proved to be, at operation, a mucocele 12 cm. in Iength and 5.5 cm. in circumference, invoIving the appendix and Iower cecum. AIthough there is no detaiIed pathoIogica1 report, it must be assumed that the Iumen may have been cIosed at times but that the obstruction subsided in order to admit the barium. Dodge, in recording a study of 142 cases HE

found that in 37 the lumen was studied, and in 5 of these the lumen was cIosed or partiaIIy cIosed. The case to be reported exempIifies the patency of the proxima1 portion of the appendix as visuaIized on Au9roscopic and pathoIogic examination of the removed * From the GynecoIogicaI Department

organ. The history eIicits repeated exacerbations of an inffammatory process. A white femaIe, aged twenty-five years, married three years, was admitted to the hospita1 March 27, 1930, with the following: History. Seven months before entering the hospita1 she suffered her first attack of cramping pain in the periumbiIica1 region. In two hours this became more and more severe and graduaIIy IocaIized to the right Iower quadrant of the abdomen. This was accompanied by nausea and vomiting on three occasions, and some fever. In the course of three days with appIication of an ice bag and rest in bed these symptoms subsided. FoIIowing this the patient had constant abdomina1 “soreness” which was most marked on the right side and occasionaIIy became sharp but did not radiate to any other part of the abdomen. In December of 1929, three months after the onset of cIinica1 manifestations, she experienced a simiIar seizure of abdominal discomfort; however, on this occasion it was more severe in the epigastrium and radiated to the right shouIder and scapuIa but was not accompanied by vomiting. These symptoms Ied another physician to regard the entire picture as the resuIt of a diseased process of the gaI1 bIadder. FoIIowing this, unti1 her entrance, there was more or Iess continuous epigastric discomfort, repeated beIching unreIated to the ingestion of food, rather marked constipation, and a Ioss of about IO Ib. in weight. The history referabIe to the peIvic organs did not point to any Iesion to account for the compIaints in the right Iower abdomina1 quadrant. However, she had had a forceps deIivery, of a normal infant, fourteen months previous accompanied by a Iaceration of the perineum which was sutured at the time. The post-partum course was afebriIe. Her menstrua1 periods were reguIar and of five days’ duration

of Post-Graduate Hospital and Medicat School, Chicago. CentraI States GynecoIogical Society, October, 1~30. 270

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Physical Examination. The patient was apparentIy emaciated, weighing onIy 97 Ib., and presented a saIIow paIe brown appearance of the skin. The essentia1 tindings were contined to the abdomen. The abdomina1 waI1 was 2 to 3 fingers beIow the IeveI of the chest and the anterior waI1 was soft in a11 areas. The cecum and sigmoid were paIpabIe and tender and IateraI traction in the former region resuIted in pain at the site which aIso radiated towards the umbiIicus. The edge of the right hepatic Iobe was feIt 2 fingers beIow the right Costa1 edge and was slightly tender. She offered no compIaints on pressure in the region of the gaII bIadder. The Iower one-third of the right kidney couId be feIt on deep inspiration. BimanuaI peIvic examination demonstrated a slight biIatera1 heaIed Iaceration of the cervix. The corpus uteri was smaI1, in axia1 position, and the appendages were free from any paIpabIe pathoIogica1 changes. The urine was devoid of any pathoIogicaI findings, there were 8750 white bIood ceIIs per cubic miIIimeter and the differentia1 count showed 75 per cent poIymorphonucIear Ieucocytes. FIuoroscopic examination of the gastrointestinal tract reveaIed the fundus of the stomach in the peIvis and contained 15 per cent residue at the five hour examination. The duodena1 cap was spastic but showed no constant deformity. The coIon was aIso in a cIonic state. On jhoroscopic examination, the appendix was visible, tender, and fixed behind the cecum. PIates confIrmed these findings except that the appendix was not visuaIized because of its retroceca1 position. Operation March 3 I, 1930. Through a right rectus incision expIoration of the upper abdomen reveaIed no abnormaIity of the gaI1 bIadder, Iiver, or stomach. A freeIy movabIe cecum covered by a Jacksonian membrane was deIivered. The appendix was curIed around the lower posterior portion of the cecum and its midportion sIightIy kinked by moderateIy firm fibrous adhesions extending from the cecum to the mesoappendix. SimiIar adhesions extended from the dista1 2 cm. of the appen-

Communication

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dix as a pyramid-shaped structure with its base formed by a 2 by 3 cm. area OS a high Ioop of the iIeum. Between these thin bands were 2 cyst-Iike structures arising from the end of appendix, the Iargest of which was 1.5 cm. in diameter and the other about 8 mm. across. In dividing the connective tissue strands the cysts were broken discharging a thick, jeIIy-Iike, coIIoid Iiquid. After the adhesions were freed the sIightIy bleeding denuded area of the iIeum was sutured over. The mesoappendix, after the adhesions myere cut, was cIamped and ligated. The appendicea1 base was crushed, incIuding part of the cecum, ligated and dropped. The peIvic organs were free from any abnorma1 changes. No jeIIy-like material was found free in the abdomen. Pathologically, the appendix measured 6.5 cm. from its base to the tip and the diameter varied from I cm. across the base and 1.2 cm. at the tip which was sIightIy bulbous. On the antimesenteric border there was a buibous sweIIing 2 cm. in the Iong axis of the organ, protruding as high as g mm. above the serosa, and having a transverse diameter of 7 mm. The termina1 portion of this tumefaction had its distal end 2 cm. from the tip of the appendix. The surface was sIightIy IobuIatecI and of a gray gIistening coIor which was interrupted by very thin hyperemic fibrous adhesions. The serous surface of the appendix and mesoappendix were the site of simiIar adhesions. Transverse sections through the appendix at various IeveIs between the base and the proxima1 portion of sweIIing on the antimesenteric border reveaIed a patent Iumen, 2 to 4 mm. in diameter, which was free from any materia1 of any nature. Surrounding the cavity the mucosa was of norma appearance, and beIow it were 7 to 8 large discrete and occasionaIIy conff uent Iymph follicles. In the submucosa were foca1 patches of fibrosis and spaces fiIIed by fat. The circuIar muscIe fibers were occasionaIIy encroached upon by these and here and there were scattered Iymphocytes. The Iayer of Iongitudinal muscIe fibers were of norma histoIogica1 appearance. The serosa in places presented a vacuoIated appearance in which were smaI1 accumuIations of basophiIic homogeneous material. About these spaces was a fibriIIar network enmeshed in which were many varying sized capiIIaries about which were occasiona smaI1 accumuIations of Ivmphocytes.

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Transverse sections through the site of the tumefaction reveaIed the picture seen in Figure I, demonstrating a narrow, then wider tract

FIG. I. Antimesenteric

appendiceal

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At the site of the defect, or hiatus, the epitheIium extended we11 out to Iine the proxima1 portion of the mucin-HIed cavity, and beIow

mural defect Ieading into mucocele, contents icaI reactions for mucin.

penetrating the waI1 on the antimesenteric side of the appendix which Ieads into a space fiIIed by materia1 that gave positive microchemica1 reactions for mucin. The intact portion of the appendix was Iined by coIumnar epitheIium which, onIy here and there, dipped down a sIight distance. The submucosa was markedIy thickened by dense Iayers of eosinophiIic Ieucocytes, poIymorphonucIear Ieucocytes, Iymphocytes, and a thin fibriIIar connective tissue network, and a few isolated gIands. IrreguIar and poorIy defined masses of Iymphocytes and extensive hyaIinized areas resuIted in marked thickening of the submucosa. The circuIar muscIe fibers were separated in pIaces by simiIar inflammatory ceIIs as seen immediateIy beIow the epitheIium lining the cavity. The IongitudinaI muscIe layer was moderateIy thickened and beIow the serosa was an isoIated group of Iymphocytes. The Iumen at this IeveI contained no mucus.

AUGUST, 1932

of which give positive microchem-

this was the same inflammatory exudate as seen in the intact portion. The muscuIar waIIs extended part way out to make up part of the waI1 of the cyst; however, in these portions considerabIe hyaIinization and fragmentation had taken pIace. The cavity of the cyst proper was fiIIed by homogeneous paIe bIue staining materia1 (hemotoxyIin and eosin) which was interrupted by varying sized and shaped masses of Iymphocytes and occasiona pIasma ceIIs and erythrocytes which were most abundant on the appendicea1 side of the cavity, next to the jutting out muscuIar Iayers of the appendix (see Fig. 2). The waI1 of the dista1 portion of the cavity was composed of markedIy thinned out fibers containing eIongated sIightIy pycnotic nucIei. Discussion. By correIation of the history and the pathologica findings we are led to assume that the patient suffered graduaI

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peri foration of the appendicea1 waII over a peri iod of seven months, with accompanying subacute exacerbations, with referred

z. Wall

of mucoccle

close to appendix. Litter forming

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in a diffuse or IocaIized dilatation of the organ. In our patient, it is possible that the appendix may have been occlude d at

Cavity. contains mu& and groups n Iayer mtcrnnl to connective-tissue

pain into the upper abdomen and gastric symptoms. The site of perforation is typica of mucoceIes following an inff ammatory process rather than a congenita1 defect. In the latter state the defect is attributed to apertures in the waI1 about the vessels entering on the mesenteric side. The other theories favoring a congenital pathogenesis apply as we11 to an inff ammatory process, in that separation of the muscIe fibers and weakness of the muscuIature do occur with an inflammatory process. As to whether an ulceration or an intramura1 abscess was present with the onset or in the course of the disease, it is impossibIe to say at this stage of the condition. If the lumen of the proxima1 portion of the appendix is occluded it is reasonabIe to assume that the secretory pressure of the residual intact mucosa is sufficient to result

A me&no

of Iymphocqtes w:lII.

and

plnsm:~

some time earIier in the process. However, this is hard to believe because intermittent occluding spasm of the appendix wouId be almost impossibIe in view of the amount of fibrosis of the waI1 and the changes in the muscuIar Iayers. Also, the specimen shows no evidences of mucus in the lumen proximal to the mucocele indicating that the fluid had not regurgitated into the cecum from the possible previousIv distended portion. In addition the degree of submucosa1 fibrosis distal to the base wouId tend to prevent dilatation of this region. It has been assumed that the contents of a mucoceIe is sterile as otherwise gangrene or empyema of the appendix wouId resuIt. In our patient the contents of the appendix and the cavity of the mucoceIe had access to the ceca1 portion of the large

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bowe1 and therefore the ordinary intestina1 bacteria1 flora couId have reached the peritonea1 cavity, by way of the gap in the Iumen, and resuIted in generalized peritonitis. The absence of peritonitis is hard to expIain unIess the mucosa or mucus had bactericida1 properties or the quantity of infected material was of such a smaI1 amount that the peritoneum could overcome this infection. The end-resuIt at times has been the typica picture of pseudomyxoma peritonei in which the entire abdomina1 cavity becomes filIed by this thick materia1. This condition has been attributed to the secretory property of the peritoneum or to activeIy secreting epitheIia1 ceIIs freed from their primary seat and continuing their function of producing mucus. In no other form of peritoneal irritation does the abdomina1 cavity lining respond in this manner. HistoIogicaI examination of the jelIy-like noduIes attached to the peritoneum shows that they are superimposed on this structure rather than part of it so that this expIanation is hardly tenable. AIthough examination of this Auid has, in some instances, reveaIed the presence of isoIated and occasionaIIy short rows of epithelial ceIIs after very carefu1 and excertainIy this smaI1 tensive searching, number of infrequentIy found celIs couId give rise to no such enormous accumuIation of intra-abdomina1 mucoid material. The theory has also been advanced that the ffuid is not a product of epitheIia1 secretion but rather a mucoid degeneration of newly formed connective tissue at the site of the mucoceIe. This may expIain the presence of a smal1 amount but it is hardIy possible that this can account for the tremendous amounts seen in some instances. It seems more IogicaI to concIude that the primary focus is in the secreting epitheIium of the ovary, enterocystoma, or appendix. For if these offending foci are removed, providing that they are not associated with a carcinomatous process, or if not too extensive, and keeping in mind the

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possibiIity of their arising from two sources, then the process usuaIIy stops. This is we11 exemplified in the 2 cases reported by Ries. Both patients have recentIy been examined and show no evidences of reaccumuIation of the Auid. The fn-st, in IgoT, had a headsized pseudomucinous cystoma of the ovary and an appendix, the site of a mucoceIe, removed. Large quantities of coIIoid materia1 escaped when the peritoneum was opened and as much of it as possible were scooped out of the abdominaI cavity. The second patient operated on in 1923 had the omentum covered by coIIoid which was found to be escaping from a Iarge cystic tumor of the right ovary and a similar but smaIIer and intact mass was found in the Ieft ovary and the cu1 de sac was filled by jelly-like materia1. CONCLUSIONS

From roentgenoIogica1 and pathoIogica1 evidence permanent stenosis of the appendix is not necessary for the formation of a mucoceIe. Pathogenic organisms may have access to a mucoceIe but do not necessariIy resuit in peritonitis, empyema, or gangrene of the organ. The bactericidal properties of the mucus or pseudomucinous materia1 and the epithelium of the appendix deserve further study. REFERENCES I. DAVIDSON,M. Internat. Clin., 32: 303, Ser. 2, 1922. 2. DEAVER, J. B. Appendicitis. Ed. 4. PhiIa., BIakiston, 1913. 3. Donc~, G. E. Ann. Surg., 63: 334, 1916. 4. ELBE. Beitr. z. klin. Cbir., 64: 635, IQOQ. 5. MICHAELSSON,E. Acca Cbir. &and., 68: 37, 1931. 6. MILLIKEN, G., and POINDEXTER.Am. J. Path., I: 397, 1925. 7. MOSCHC~WITZ,E. Ann. Surg., 63: 6~7, 1916. 8. NASH, W. G. Brit. M. J., 2: 595, IgIg. 9. RIES, E. Surg. Gynec. Obst., 39: 569, 1924. IO. ROYSTER, H. A. Appendicitis. SurgicaI Monographs. N. Y., AppIeton, 1927. II. SIMON, S. Deutscbe Ztscbr. f. Cbir., 187: I, 1924. 12. STOUT,A. P. Arch. Surg., 6: 793, 1923. 13. VORHAUS,M. G. J. A. M. A., 94: 165, 1930. 14. WEAVER, D. D. Calij. @ West. Med., 28: 500, 1928.