Intussusception
of the Appendix Carcinoma
Due
to
JEROME J. WEINER, M.D. AND ANGELO M. SALA, ALD., New 1órk, New )‘ork
NTUSSUSCEPTION of the appendix is stil1 cona rare condition; about IOO cases have been reported since 1858 when McKidd [I] reported the Iirst case in a seven year oId boy. In 1910 Moskowitz [2] reported twentyfour cases which he accepted as irrefutabIe. In 1943 Fraser [3] reviewed seventy-hve cases and discussed the etiology and morbid anatomy, and cited his own case in a thirty year old woman who had had an intussusception of the appendix produced by a mucoceie. He also reported a case in a seventy-five year oId woman, in whom resection for carcinoma of the cecum was performed and as an incidentaI hnding the intussusception of the appendix was noted. In 1953 ForshaII [4] reported seven cases which occurred in children. It may we11 be that the condition is more prevalent than the Iiterature indicates. Intussusception may be of the simple or compound variety. In the simpIe type the appendix aIone is involved, whereas m the compound type the ileum, cecum or ascending coIon may be involved. The simple type occurs most frequently in patients who have (1) a feta1 type of cecum with the appendix arising from its tip, (2) an appendix with a widemouthed base, (3) a thin mesoappendix which is free from fat with a narrow base, (4) an appendix that has a mobile appencIicea1 waI1, and (5) an appendix lvhich is not fixed by congenital peritonea1 folds or adhesions. These are al1 anatomie conditions which predispose to simpIe intussusception of the appendix. Other factors which cause increased peristaltic action
1 sidered
susceptum and invaginates into thc proximal portion of the appendix which forms the intussuscipiens; (2) the invaginations can start at the junction of the appenclix and the cecum where the appendix is the intussusceptum and the cecum is the intussuscipiens (the most common type of intussusception); (3) the invagination can start at any point along any portion oftheappendix (very raretype) ; (1) :I rettrograde type of intussusception wherc the proximal portion of the appendix invaginates into the distal end; (5) there may be complete invagination of the appendix into the cecum. These constitute the simple type of intussusception, which can progress and produce a compound or secondary intussusception of the cecocolic variety, m which cases the invaginated appendix forms the apex of the intussusception. In the compound type the appendix is the primar,v factor and secondarilq invol\,es thc
structures in the cecocolic region. Cases have been reported in which the appendix has been drawn into a mass involving the structures in the ileocecal rcgion, as in cases of ileocccaf, iIeoceca1 colonic or cecocolic intussusception. These cannot be classified as true types 01 appendicea1 intussusception. The case presentec1 herein is of interest Iwcause the appendiceal intussusception was procIuced by a papillary adenocarcinom:l of thc appendis and because it occurred in a lifteen year old boy. We have not been nble to tind, up to the time of the present writing, a case that lists this condition as the factor in thc protluction of an nppendicenl intussusccption.
in the wal1 of the appendix, such as fecaliths, foreign bodies, warms, hypertrophic Iymphoid foIIicles, papillomas, polyps, endometriomas or carcinoids, are factors in the production of this Iesion. In the simple forms of intussusception (I) the tip of the appendix becomes the intus-
N. S., a lifteen >‘ear old whitc I)oy, statcd that one month prior to cxaminntion in December, 1954, he had been seized m-ith a colicl;! pain in the right Iow-er quadrant of the ahdo-
Intussusception
of Appendix
Due to Carcinoma
FIG. 1. X-ray taken six hours after barium mea1 shows dilated terminal iIeum (a) and cecocolic intussusception (b‘t.
FIG. 2. X-ray taken meal shows appendix (rS) nnd (c,.
men; this pain radiated to his back. The attacks were intermittent, Iasting a few minutes and then ceasing. However, the pain wouId recur every few days and wouId continue for about twenty-four hours. The boy experienced no nausea or vomiting during the attacks of pain. His boweIs were reguIar and he did not notice any bloed or mucus in the stoo1. His appetite was fair and he did not Iose any weight. NO dysuria or hematuria was noted. Three weeks foIlowing the origina1 attack he had a recurrente of the pain in the right Iower quadrant; the pain was more intense and Iasted a few hours. Physical examination showed a paIe, thin, fìfteen year oId boy of stunted growth, whose physical makeup was that of a ten year oId boy. Ears, nose and throat were normaI. The chest was ffat. Heart and Iungs were normaI. The abdomen was of the scaphoid type. NO distention or visible peristalsis was noted. On paIpation the Iiver, kidneys and spleen were not enlarged or tender. NO tumor masses were paIpated. There was tenderness over McBurney’s point and a crepitating cecum was de-
tected. NO muscIe spasm or rebound tenderness was noted. Recta1 examination showed no abnormalities. In view of the negative findings, except for the tenderness over the appendicea1 region, it was deemed advisabIe that the patient have a compIete gastrointestinal series. X-ray examination showed norma fÌIIing of the stomach; there were no defects in the fundic or pyIoric portions, or along the curvatures. The duodena1 buIb outIined normally and the stomach emptied after six hours. The six-hour fiIm reveaIed some diIatation of the termina1 ileum and evidences of cecocoIic (Fig. 1) intussusception. The appendix was not visualized in this fiIm. In the twenty-four-hour film there was a filling defect with irreguIar striped formations which were ring-like ancl spiral-shaped. These were offshoots of the barium which fÌIIed the narrow spaces between the cecum and the ascending coIon. (Fig. 2.) These findings, according to DahI [y], are pathognomonic of cecocoIic intussusception. In forty-eight hours (Fig. 3) the apex of the intussuscipiens was outIined by gas and had assumed a convex contour just beyond the hepatic Rexure. Sorne of
821
twenty-four hours aftcr barium (al and cecocolic intussusception
Weiner
FIG. 3. ?i-ray taken forty-eight hours after bnrium mc~tl shows appendix (e), papillary tumor of appendicenl basc intussuscepted into cccal wull (b) and cecocolic intussusception Cc).
the transverse striations were stil1 noted between the invaginated cecum and ascending colon. The appendix was fiffed with barium and the cecaf waf1 encircfecf the invaginated portion of the appendix. Proximaf to the fower cecaf waff, barium coufd be seen incorporated in a papiffary tumor of the appendix. In view of these findings a preoperative diagnosis of cecocofic intussusception was made and the patient was admitted to the hospita1 for surgery. At operation the abdomen was enterecf through a right rectus muscfe-spfitting incision. The omentum was found to be adherent to the cecum and ascending cofon. Foffowing the clivision of the omentaf adhesions, the cecum was freed and coufd be brought down into the right iliac fossa. NO masses were feft in the ascending or transverse cofon. The cecum was delivered through the incision, and Iying on the fateraf aspect was a sausage-shaped appendix the size of a thumb. The proximaf third of the appendix was invaginated into the waf1 of the cecum ancl formed the intussusceptum. On pafpating the intussusceptum through the cecaf vvaff, a hard,
and Snla
mushroomfike tumor coufcf be feit. The mesoappendix was thin and contained a smafl amount of fat. The Iymph nocfes Ijere nol enlarged. Some frce ffuid vvas present in thc peritoneaf cavitp. A 2 inch cuff of the cecaf \vall was rcmoved together 14th the append is. TIK \valI was closet1 with two lagers of No. oo chromic catgut, onc Iayer consisting of thc mucos:~ and niuscularis, nnd thc second layer of thr serosa. ‘T‘hc cecum gas redeposited into the abdominal c>aviti; ad nnchorctl in thc right ifiac fossa to the Iateral parietal peritoneum. The abdomen was closed in Iayers ~1ithout drainage. On gross esamination of the specimen ( E‘ig. 41 tfre visibfc portion of the nppendir mcasurecf 9 cm. in Icngth and IP~ cm. in its widcst diamcter. Thc csternal surface inas co\-crctl 1,~. :I gre‘-ish yello\\-, smooth, glistening serosa. Tfie prosimal 2 cm. of‘ ttle appendis 11as found invaginatetl into thc cccum. The muc’osa was reddish lx-on.n in cofor and Inu1 ;I pnpillar>appearancc. The samc papilfar~ appearancc was present on cross section. The wal1 of t fle appendix \fas thick and had :r 1~idc lumen. Efsewhere thc mucosa appeared 11‘.perplastic. Therc L\::ISa srndl amount of fecal rnatcrial irl the distaf portion of the appenclis. The I~~UCOS~I of tfw ceïrlm at the pface of origin of’ the appcndis 11as (lefinitcl>1~)pcrplastic,. :I nri papillar->- ovcrgrowth Xkas obvious o\ el- ilil area ,s cm. in diameter incfuding thc lumen 01‘ the appendix at tfiis sitc. .\Iicroscopic cxamination disclosïd ír pnpillar> cnrcinoma inr-ol\~in.g both tfrr appendix and ct'cu111 at thc locations noted hcretofore. (Figs. 5 and 6. :
Intussusception
FIG. 5. Photomicrograph
of Appendix
of appendix.
Due
to Carcinoma
FIG. 6. Photomicrograph
The patient made an uneventfu1 recovery and left the hospita1 eight days after operation. One month after surgery he was free from any pain referabIe to the right Iower quadrant of the abdomen; his boweIs were functioning properIy and his appetite had improved. A barium colon enema at this time failed to revea1 any pathologie disease in the cecum, or ascending, transverse or descending coIon. A one and a half year check-up reveaIed that the patient was in norma heaIth, had grown 2 inches and had gained 10 pounds.
of cecum.
Altman, Ackerman and Hoffman [9] reported a case of invagination of the appendix into itself. In 1952 Morton and Oakman [IO] presented a case of primary intussusception caused by endometria1 rests within the appendicea1 waI1. In 1953 Forshall [s] reported a series of seven cases of intussusception of the vermiform appendix which occurred in children. In live cases the invagination of the appendix was incompIete. In six of these seven cases sec0ndar.v cecocolic intussusception had occurred. In reconstructing the sequence of events in our case, it appears from the roentgen fìndings that the patient had a compound type of intuscecocolic \rariet>-. susception, i.e., appendiceal At surgery the cecocolic component was reduced and the appendicea1 intussusception persisted as a result of the papiIIary adenocarcinomatous tumor which involved the base of the appendix. NO other case in the Iiterature Iists papiIIary adenocarcinoma as the precipitating factor in the production of appendicea1 intussusception occurring in a young 1~0~ fifteen years of age.
COMMENTS
From July, 195 1 to January, 1953 tweIve cases of intussusception of the appendix were reported. Zeifer [6] in 1951 gave the cause of intussusception in his case as a compound type with an incidentaI finding of a mucoceIe of the appendix. In 1951 Hickenbotham [7] reported the case of an intussuscepted appendix produced b‘; an argentaflìn tumor. Dunavant and Wilson [8] reported a case of intussusception of the appendix which was compIeteIy inverted and protruded from the anus. In 1952 823
Weiner
and
From a clinical point of view the cliagnosis of appendiceal intussusception is rather diffrcult to make. We should, however, entertain the possibility of this lesion whenever a patient gives a history of intermittent cramp-like pains IocaIized in the right Iower quadrant of the abdomen lasting for a few minutes, recurring and then followed with a period of well-heing. Nausea, vomiting and bloody stooIs are late findings when the intussusception has become compounded. If the physica1 findings reveal nothing of any significante, either n gastrointestinal series or a barium coIon enema sl~ould be done. More frequent diagnoses of this lesion couId be made if one remembers the roentgenographic lindings as emphasized by Dahl, r.e., (a) a filling defect in the ascending or transverse colon associated with (b) irregular markings which are stripe-formed, ring-Iike or spiral-shaped. These are offshoots of barium which IiII the spaces between the intussuscipiens and the intussusceptum. If the barium is ingested, the apex of the intussuscipiens wil1 assume a convex appearance; and if a barium colon enema is given, the apex wil1 assume a concave outline. In this case the gastrointcstinal series aidecl in estabIishing a working cIiagnosis of cecocolic intussusception. After operation the x-ray lilms were reviewed. The fort\--eight-hour film definitely revealed the proximal third of appendix with the tumefaction invaginated into the cecal waI1. The photograph of the pathologie specimen showed the papiIIary tumor wit11 the cuff of cecum enveIoping the base of the appendix. Transposition of the photograph onto the x-rav lilm showed the precise nature of the lesion as It lvas found at operation and on the x-ray film.
REFERENCES
I. MCKIDD, J. Edinburgh M. J., p. 793, 18& 2. MOSKOWITZ, A. V. M. Rec., 78: 1087, 1910.
824
Sala 3. l:lt..v5hK, K. lntussuscrption ./. Surp., 31 : 23, 1943.
of the
:ippcndi\.
Bl-fJ.
4.
5.
6. 7. 8.
L>. 13.. ACKIXMAY, kl. and t IOIWIA\, 9. Ar~\<,a\, 1-1. S. lntussusception of thc vermiform appendix. J. .4. .M. A., 149: ,133, IC)~Z. intus10. MOR.rC)N, P. C. and O.*IaIA\, C. S. Primary with endometrial susccption of thc appendix implants. A7n. J. Surp., 84: 734, I9j2. E.. M’. Intussusception of the uppcndix. 11. ~C~ITCF~E.LL., CUP&. M. A. J., 25: 194, 1931. C. H. ~l;~lignant carcinoids and c;5rcinom;i 12. RFtz, Ifabnemunninn Of thc vcrmiform appendix. 13. LERKCX, El. 1. Adenocarcinoma of thc appendix. J. Internu~. CX. Surgeons, zo: 481, 19j3. 14. CIU~.~., G., JIJ. and GLEXN, C. G. Primury adenoc;trcinoma of the appendix with deveIopment of :I mucus list&. U. S. Nur. Med. Bu2/., 47: 328, 194’.
18. Rorsron», T. W’., JAQLES, \t’. 1,. :lnd CI.AC-s, R. Primary tumors of the vermiform appendix. Clinicopathologic qproach. /lm. Surgeon, ZO: isi, $054. 19. VAXCF, C. A. Primqv carcinomn of the vrrmiform appendix. Am. .Z. Surg., 24: 8j4, 1934. 20. ~IILSARECIC, J. R. Carcinoma of the appendix. Analysis of 3 srries of cases. I’r«c. Sta//