POLYPOSIS OF THE COLON JOSEPHE. STRODE, M.D., P.A.C.S. The Clinic HONOLULU,
G
ENERALIZED
poIyposisof the coIon is not a new cIinica1 entity, but the reaIization of the frequent association of this condition with carcinoma of the Iarge intestine has progressiveIy broadened the fieId of its interest. ArticIes in recent Iiterature by Erdmann and Morris,l McKenney,2 Rankin and Grimes,3 MiIIer and Sweet,4 Coffey5 and others, not onIy emphasize the frequency of maIignant degeneration in this condition but caI1 attention to the frequent famiIia1 tendency of the disease. It is generaIIy acknowIedged that the Iesions are of the adenomatous type and the term “muItipIe adenomatosis” proposed by Lockhart-Mummery,6 is a more descriptive terminoIogy but “ poIyposis ” seems to be the more popuIar expression. Erdmann and Morris are responsible for the cIinica1 cIassifIcation of poIyposis of the colon into two groups, namely: (I) the aduIt (acquired type), and (2) the adoIescent, or congenita1 disseminated type. This cIassification, whiIe not serving to differentiate morphoIogicaIIy the individual variations frequentIy encountered, does on the whoIe serve as a usefu1 working basis. The acquired type presents itseIf in aduIt life, usuaIIy as a few scattered polypi secondary to frank evidences of chronic inflammation. They are, not infrequentIy, the precursors of maIignant degeneration. The adoIescent (or congenita1 disseminated type) manifests itseIf earIy in Iife by intermittent diarrhea, recta1 bIeeding, abdomina1 cramps, Ioss of weight and secondary anemia. This is the type in which the famihal nature of the disease is evident. Rankin and Grimes point out that these tumors are wideIy distributed in the coIon, frequentIy extending from anus to
T.
H.
cecum, but that their appearance in the sigmoid and the rectosigmoid regions is approximateIy eight times more frequent than in any other portion of the coIon. This, they point out, is particuIarIy significant in the Iight of the simiIar occurrence and ratio of the distribution of carcinoma of the coIon, and immediateIy suggests a reIationship. WhiIe the average age of the individuaIs in which the condition is recognized is around the second and third decade of Iife, patients as young as 2 years and as oId as 70 have been reported. The diagnosis of the condition is easiIy made, the simpIest means being by the paIpating index finger substantiated by inspection through the rectosigmoidoscope. A coIon x-ray, in addition, heIps determine the extent of the invoIvement. In other words, if an examination is made that is caIIed for by the patient’s presenting symptoms, there shouId be no excuse for faiIing to arrive at a proper diagnosis. The treatment of poIyposis of the coIon is surgica1 and when a generalized invoIvement is encountered, compIete remova of the coIon is the onIy assurance against subsequent serious compIications. IsoIated poIyps of the coIon may be deaIt with individuaIIy, either by excision or fuIguration by way of the proctoscope or transabdominaIIy, but x-ray studies of the coIon shouId be made at the time to see if poIyps higher up can be demonstrated. However, when negative evidence is obtained, one has the feeIing that possibIy their presence is being overIooked. Just recentIy, an abdominoperinea1 resection of the coIon was done because of maIignancy deveIoping at the site of remova of a poIyp by Dr. Straub of our CIinic, nine years previousIy. In the removed specimen of rectosigmoid there 353
354
American
Journal
Strode--PoIyposis
ol’ Surgery
were other poIyps and there may be more DroximaIIv situated to the Doint of excision that will give further trouble. I
“,
1
of the CoIon
L’OI’FMBFH. II,,<,
an initial procedure of anastomosing the ileum to this portion of the gut may be considered, but most observers apparentI> prefer doing this as the terminal step. In removing the colon, this mav be done in one stage or several, depending upon how the patient reacts to the operation Inay be discontinued that it seems desirable. CASE
FIG. T. X-ray after a barium encma. The fiIling defects cirused by the polyps zlrc characteristic, showing thr extent of the disc:~sc prcoperativel,v.
More recently, cases have been reported in which the colon has been removed to the rectosigmoid region, the poIyps in this area being removed by fuIguration and this part of the gut later utiIized for anastomosis to the iIeum. A permanent iIeostomy is certainly to be avoided if this can be accompIished with safety to the patient, but this hardIy seemed feasibIe in the case which we are reporting. When complete excision of the colon is to be done, a preIiminary iIeostomy is imperative, since this pIaces the coIon at rest and permits a considerable degree of recuperation of the structures before extirpation. The contents of the coIon can be thoroughIy cleaned out; many virulent bacteria are meanwhiIe destroyed, making peritonitis at subsequent operation Iess IikeIy; the 10~s of bIood diminishes and meanwhiIe the patient’s depleted reserve is buiIt up by transfusions ; etc. If the rectum is to be preserved,
ordeal. The at an!. time
REPORT
The patient, a Ha\vaiian male, aged 20, entered Queen’s Hospital on March, 23, 1939, under the care of Dr. Rlin Hin Li, who made the diagnosis. He had apparently been perfecti! well until two months before admission to the hospital, when he developed diarrhea, abdominal cramps, dizziness and chills. The stools wert water! and frequently contained gross blood. His appetite was poor and during this time he had lost 20 pounds in weight. General physical examination on admission was negative. Blood examinations at this time revealed $3 per cent hemoglobin, 4,390,ooo red cells and a Tvhitc blood count of I 5,900, w+h polymorphonuclears 78 per cent, eosinophiles I per cent, monocytes 3 per cent, lymphocytes 9 per cent. The red cells showed many macrocytes, much poikilocytosis and considerable achromic staining. Repeated stoo1 examinations, including cultures, showed nothing abnormal except for a strongly positive test for blood. The urine was negative, as \verc the Wassermann and Kahn of 500 tests. On March 25 and 30, transfusions cc. of titrated blood were given; the blood 70 per count on April I re\.eaIed hemoglobin cent, rec1 blood count 4,78o,ooo, white count I 5,200, polymorphonuclears 61 per cent, eosino1 per cent, ant1 philes 3 per cent, monocytes lymphocytes 32 per cent. Digital and proctoscopic examination of the rectum revealed many polypi, but microscopic sections from biopsies of the most suspicious appearing ones showed nothing suggestive of malignancy. There m-as much oozing of bIoocf from the irritated polyps during this examination. Colon x-rays indicated a generalizect invoIvement of this structure by the polyps. The temperature record previous to surgery showed only an occasionaI rise to 99’F. had apparently sufFerec1 from a No relative similar condition. However, it is easily possible that this part of the history might be in error.
NEW
SERIES VOL. L, No. z
Strode-Polyposis
The patient Ieft the hospita1 on April 6, feeling better, but was readmitted eight days Iater because of a recurrence of the symptoms for
of the Colon
Aunericnn Journal
of Surgery
355
Under spina anesthesia (pontocaine) a McBurney type of incision was made. The ileum was divided just proxima1 to its termination in
--_.- ._.. _l___I FIG. 2. Specimen removed at second operation (first operation ileostomy). Appendix at left, with its mucosa also polypoid; cecum and first part of co1on. Note the Iong pedicle of the polyp above, and the size of the one beIow, backed by white paper.
which he had originally come. Blood examina74 per tion at this time showed hemogIobin red celIs and 25,440 white cent, with 4,530,ooo blood ceIIs, with 75 per cent poIymorphonucIears. Stool examinations continued uniformIy positive for bIood.
FIG. 3. Specimen removed at third operation, including the second portion of the colon, the sp1enic flexure, which was not so heaviIy studded with polypi, and the first portion of the sigmoid.
the cecum, with the cautery between Stone aseptic intestina1 clamps. The distaI end was turned in and dropped back. The proxima1 end of the iIeum was mobiIized so that it couId be drawn out beyond the abdomina1 wal1 for approximateIy 4 inches. This was accompIished by severing the mesentery near the root, first
FIG. 5 Very Iow power (3>5 times enlargement) of an entire poIyp, showing, on the right its stretched base of relatively normal colon mucosa, on the left the structure of the poIyp itself.
the vessels before severing, to see if the circulation in the iIeum to be mobiIized remained adequate. It is a point worth remembering that onIy a small amount of mesentery can be divided near the bowe1 without interfering with the circulation, whiIe a considerabIy greater amount can be divided near the mesenteric root without troubIe, due to the anastomosing Ioops of vesseIs dista1 to this region. The proxima1 iIeum was drawn we11 out of the wound, attached to the peritoneum and fascia, and the incision cIosed around it. A large sized Pezzer catheter was then pIaced into the procompressing
4. Specimen removed at fourth operation, consisting of rectum and perirecta1 tissues, sliced longitudinally to show muscutar and mucosa1 layers.
FIG.
It was felt at this time that there was no treatment for this condition except surgery, and that a complete removal of the coIon was necessary due to the Iarge number of poIypi visibIe and paIpable in the rectum. The patient was transfused on ApriI rg and 24 and the first stage operation was done on ApriI 25.
356
American Journal of Surgery
Strode-PoIyposis
truding ileum and the gut wall inverted around it. By this means the intestinal contents during its most irritating period, was conveyed away
Low power photomicrograph of the area outlined in Figure 5, showing normal structures at the right, below, and the adenomatous poIypoid structures above and at Ieft. Note the difference in the char-
FIG. 6.
acter of the glands, and nous type of the stroma portion.
the loose gelatiof the polypoid
from the abdominal wall and at no time during the postoperative period was there excoriation of the skin. Tube drainage of the intestinal contents was successfu1 for two or three weeks. After removal of the tubes the skin was protected by zinc oxide sprinkIed with kaoIin and overIaid with vaseIine gauze. Between the first and second operations, the patient was given another transfusion of 500 cc. of blood. On May 12, or seventeen days after the first operation, again under spinal anesthesia, a long Ieft rectus incision was made with a transverse incision to the right across the right rectus muscIe between the umbiIicus and the costa margin. The cecum and ascending colon were mobilized by an incision aIong the Iateral side, the vessels ligated, and the dissection carried over to the region of the spIenic ffexure. The omenturn was freed from the transverse coIon and preserved. Posteriorly, the ureter and duodenum were identified and protected. The dissection was rather diffrcuIt due to adhesions, edema, and the enormous size of the mesenteric gIands. Examination, microscopicaIIy, of some of these gIands reveaIed no maIignant deposits. By the time the spIenic ffexure had been reached, the patient’s condition did not justify proIongation of the operation. The incisions
of the Colon
NOY~.MLW+, IWO
were closed up to the protruding colon, the Stone cIamp was again applied, the gut cut off with a cautery and an aseptic closure of the gut
FIG. 7. kligh power photomicrograph
of cent ral portion of :I poIyp to show the mucoid type or the essential ct~li of the gland, and the Ioos~, gelatinous tvpc of the stroma.
lumen carried out, following kvhich it was dropped back into the abdominal cavity and the incision closed. The patient was given 500 cc. of blood and intra\.enous saline and gIucose during the operation. On May 30, eighteen days following the second operation, a third operation \vas done under spinal anesthesia. A Ieft rectus incision was made with transverse incision across the Ieft rectus muscle in the spIenic region. The remaining colon was removed into the hollow of the sacrum. The superior hemorrhoida arter?was preserved in order to prevent loss of viability to the upper end of the rectum. The distal end of the colon was inverted, dropped back and covered over I~\: peritoneum from each side of the pelvis, forming a diaphragm of peritoneum between the upper and lower pelvis similar to that formed during an abdominoperinesl resection for carcinoma of the rectum. The raw areas left by remova of the colon w’ere closed over by sewing together the lea\-es of the coIonic mesenteq-. Through an cstraperitonral stab wound in the left McBurne?; region, a rubber tissue drain was carried down to the stump of the dista1 coIon and one carried upward below the Ieft renal area. Again, the patient was supported during operation by intravenous saline and glucose and by a bIood transfusion. Twenty-se\ren days after the third operation a fourth was carried out with the patient under spina anesthesia, in prone position, with head
New
SERIES
Strode-PoIyposis
VOL.L. No. 2
lowered, feet Iowered, and Iegs separated. A Iongitudinal incision over the midportion of the lower sacrum was carried down and around the anus. The coccyx was removed. The rectum and anus were then extirpated without much diffrc&y, after Iigating the middIe hemorrhoida vessels. The cavity remaining was packed with Vaseline gauze and rubber tissue drains and the wound partiaIIy sutured around these drains. The patient was again transfused at the completion of the operation. One month after the last operation, the patient was doing weI1, and the fecal discharge from the iIeostomy was becoming more solid and Iess frequent.
of the
CoIon
AmericanJournalof surgery
REFERENCES
ERDMANN, J. F. and MORRIS,J. H. PoIyposis of the colon. Surg., Gynec. em Obst., 40: 460, 1925. 2. MCKENNY, D. C. MuItipIe polyposis of colon: famiIia1factor and malignant tendency. J. A. M. A., 107: 1871, 1936. 3. RANKIN,F. W. and GRIMES, A. E. Diffuse adenomatosis of colon. J. A. M. A., 108: 711, 1937. 4. MILLER,R. H. and SWEET,R. H. Multiple poIyposis of colon; a famiIia1disease. Ann. Surg., 105: 51 I, I.
1937.
5.
COFFEY,R. J. Multiple adenomatosis of the coIon; a cIinicopathoIogic study. Proc. Sta$ Meet., Mayo
6.
LOCKHART-MUMMERY, J. P. The causation and treatment of multiple adenomatosis of the colon. Ann. Surg., go: 178, 1934.
Clin., 13: 541, 1938.
THERE is ampIe proof that poIyposis intestini is an inheritabIe disease, and Dukes has published charts and thirteen famiIy pedigrees which are most convincing on this point. THE brief Surgery”
excerpts
in this issue have
by Rose & Carless (WiIIiams
357
been taken
from
& WiIkins Company).
“ManuaI
of