Polyposis of the colon in children

Polyposis of the colon in children

POLYPOSIS OF THE COLON IN CHILDREN* JACK G. KERR, M.D. Dallas, Texas I occurs in adults a review of our records of chiIdren examined during recen...

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POLYPOSIS OF THE COLON IN CHILDREN* JACK

G. KERR,

M.D.

Dallas, Texas

I

occurs in adults a review of our records of chiIdren examined during recent years was made. The group of children comprising this study consisted of one hundred consecutive patients in whom one or more adenomatous poIyps or lesions arising from them were found in either the rectum, colon or in both. While no data are avaiIabIe from our records to indicate the incidence of coIonic or rectal polyps in genera1 chiIdhood popuIation, knowIedge of the frequency with which they occur in children who present symptoms referable to the coIon or rectum may be gained from the foIIowing statistics: A tota of 349 children including those with polyps was examined. In 106 examination reveaIed no abnormal findings although in nineteen a polyp was strongly suspected because of the history. Anal fissure, ana uIcer or a para-ana abrasion was found in fifty-nine instances; congenital deformities in twenty-seven; prolapse of the rectum in twenty-four; abscess or fistula in twenty and an ulcerative process which involved the mucosa of the rectum or rectum and sigmoid was observed in thirteen. Of the 349 patients there were one hundred in whom polyps were discovered and the singIe symptom common to al1 was the painless passage of blood-streaked mucus from the bowel. In many patients this was the onIy symptom. If a fissure was present, some sign of discomfort was usually observed by the mother of the patient. This statement is mentioned because of the frequency with which parents are told by physicians that rectal bleeding although painIess may he owing to the existence of a fissure. As a rule bIeeding from a poIyp occurs intermittently and weeks may intervene between episodes. In this group the history of bleeding covered periods from a few days to several years. The average duration of symptoms appears to be of no great significance in this series because a surprisingly large number of chiIdren had bIed for we11 over a year whiIe mothers were assured frequently that this sign probabIy was of no significance. It was fre-

N chiIdren when bIeeding from the bowel is the presenting symptom, the source of the blood is more likely to be an adenomatous poIyp of the rectum or colon than any other singIe Iesion. If bleeding is the only symptom, a polyp wiI1 be found more often than the combined lesions of these organs that would be expected to bIeed. PoIyps are usuaIIy mentioned as a possible source of blood m artictes which deal with the subject of blood in the stooIs of infants and chiIdren. Discussions which enumerate the clinical types of poIypoid disease of the colon and rectum usually omit the childhood variety. PoIyps of the colon which occur in children should be incIuded in any discussion of the discrete type of poIypoid disease. The condition occurs with moderate frequency and complicating conditions are often superimposed. Reports in the literature have dealt frequently with poIypoid disease of the Iarge bowel in adults. ProbabIy two clinica types will include a11 cases of polypoid disease mentioned in these reports. These types are (I) multiple polyposis of the familial type and (2) poIypoid disease in which one or very few polyps are found. Cripps described “disseminated polyposis of the large bowel” in which a hereditary basis was evident.’ Erdmann and Morris” classihed this group as congenita1 or adoIescent in type and those cases in which no heredofamilial predisposition existed they designated as acquired or aduIt types. This latter group, frequentIy referred to as discrete poIyposis, has been observed most frequentIy. The incidence, symptoms, distribution and malignant potentialities of single polyps in adults are well known and opinion regarding them is generaIIy unified. Considerable variation of opinion exists regarding their etiology, however. SeveraI authors have expressed the opinion that they are true tumors.‘~3~4 In order that some of the cIinica1 and pathologic features of the disease as it appears in chiIdren might be correlated and compared with the discrete type of polypoid disease as it * From The Department

December,

1948

of Proctology,

Southwestern 667

Medical

College, Dallas, Tel;.

668

Kerr-Polyposis

quently the anxiety of the mothers that led to adequate, early examination of their children. The passage of small clots of blood occurs frequently. Blood in an amount suflicient to cause alarm occurred in a few instances. The source of these hemorrhages usuaIIy was the pedicle stump of a polyp from which all or a Iarge portion of the body of the polyp had occurs fresloughed away. Self amputation quently in children but rarely in adults. It occurred in eighteen patients of this group. It is not unusua1 for a mother to find polyps which have been extruded through the anus of a child. In sixty-eight patients a single polyp was found and thirty-two patients had more than one. UsuaIIy there were only two or three when multiple polyps existed and in onIy one patient resemblance to heredofamilial polyposis was observed. This patient was a three year old boy who had ten or tweIve polyps in the terminal 25 cm. of his coIon which varied in size up to 55 cm. in diameter. Throughout this area there were also numerous areas of thickened mucosa which presented the gross appearance of hyperplasia. X-ray examination of his colon reveaIed a polyp I cm. in diameter in the transverse coIon and severa small polyps in the cecum. This child’s father had undergone right colectomy for carcinoma when he was thirty-four years of age. Ninety-eight of this group of one hundred patients with poIyps were eleven years of age or younger. One patient was tweIve years of age and one was twenty-four and were included because of significant histories. The average age including the two oldest patients was 3.4 years. Ninety-four patients were five years of age or younger. The youngest chiId in whom a polyp was found was nine months oId. There were fifty-six boys and forty-four girls in the group. Symptoms other than thti passage of bloody mucus were, in the order of their frequency: protrusion of the poIyp in 28 per cent; diarrhea in 19 per cent and abdomina1 cramps in 18 per cent. Protrusion of polyps situated near the anal outlet is not an unusua1 compIication but in severa patients of this series protrusion occurred when the poIyp was situated IO or 12 cm. above the ana outIet. In one four year with intussusception old child protrusion occurred and it was found that the poIyp was attached 23 cm. above the anaI outIet. In

of Colon children, prolapse of the rectum can usually be differentiated from protrusion of a polyp by the history and the dark, port wine color of the protruded polyp is easily distinguished from the familiar color and configuration of rectal proIapse. True diarrhea is not an uncommon symptom when a poIyp is present. In a few instances in this series oral medication n-as administered over long periods of time in an effort to allay the stoo1 frequency. The proximity of ‘the polyp to the anal outlet as well as the frequent? with which Iarge doses of mineral oi1 preparations were given with the intent of checking the bIeeding by softening the stool, make the evaluation of this symptom possess doubtful significance. Abdominal cramps varied in intensity with the size of the polyp and its location. Cramps were severe in three cases and affected the lower portion of the abdomen. Every child who had a polyp situated 25 cm. or higher above the anal outlet complained of this symptom and usuaIIy discomfort was severe. In thirteen patients one or more poIyps were present which couId not be visualized through a proctoscope 25 cm. in Iength and were diagnosed by roentgenographic examination of the coIon. Eight of these aIso had one or more polyps which couId be visualized on proctosigmoidoscopy. The diagnosis of poIyps of the colon and rectum depends upon an adequate examination which includes proctosigmoidoscopy and roentgenographic examination of the coIon. Frequently each of these procedures must be repeated. This is particuIarIy true of examination by roentgenography. However, if symptoms are significant, one should never fail to repeat these examinations. If the painIess passage of bloody mucus persists, the physician should assume that a poIyp is present unti1 he can prove otherwise. One negative examination is not necessariIy concIusive. One child in this group continued to pass blood-tinged mucus after two poIyps had been removed from the rectum and Iower portion of the sigmoid. Another polyp was found in the descending coIon on the fourth x-ray examination of the coIon. CarefuI preparation of the bowel is essential prior to each examination. Poor preparation can defeat the purpose of the examination. In this group of one hundred cases each of the folIowing serious complications was en-

American

Journal

of Surgery

Kerr-PoIyposis countered one or more times: severe bIeeding, anemia, obstruction, intussusception and carcinoma. Definite indication exists, therefore, for the removal of these lesions when found and this dictum applies to those poIyps beyond the reach of the proctoscope as well as those which can be visualized through that instrument. Removal may be accompIished by electrofulguration when polyps lie within the terminal 25 cm. of the coIon and by colotomy when above that segment of the Iarge bowel. In children these Iesions are adenomatous and on microscopic examination present a gIand pattern which always seems to be we11 preserved and appears Iike normal colon mucosa. GobIet celIs are very numerous and cell nuclei are smaI1. In aduIts about one-half of the polyps have the same appearance on section as those in children. The others have fewer gobIet cells; nucIei tend to be larger and gIand reproduction is not accompIished as well. In a certain few definite anaplastic changes are present in the epitheIium but no invasion is An inflammatory reaction may be present. present in either the chiIdhood or adult type of polyp but there is no evidence that either Iesion if it is a true adenoma is dependent upon this reaction for its origm. That they are true neoplasms and not the result of a Iocalized or diffuse inflammatory reaction seems to be a IogicaI concIusion. AIthough we do not have reason to believe that the tendency for malignant change to occur in polyps of childhood is as great as it is in those of adults, one cannot dismiss this possibiIity. Two patients in this group, with untreated poIyps, deveIoped maIignant Iesions at an earIy age and from the history of each patient IittIe doubt can exist that the cancer was superimposed upon a poIyp of Iong-standing. One of these patients, a twenty-four year oId maIe, gave the foIlowing history: Since he couId remember, a mass which frequentIy bled protruded from his rectum foIIowing each defecation. Six months prior to his first consuItation regarding this condition the protrusion stopped aIthough bleeding had continued. Examination reveaIed a Iesion of the anterior rectal waI1 which was 3 cm. in diameter and its lower border was 2 cm. above the mucocutaneous junction. Biopsy reveaIed a grade II mucoid adenocarcinoma. A one-stage abdominoperinea1 resection was performed and regiona Iymph nodes were found to be inDecember,

1948

of Colon valved. The patient Iived approximateIy eighteen months following the operation. The other patient, a tweIve year old boy, was admitted to the hospita1 suffering from partia1 obstruction of the Iarge bowe1. Similar attacks had recurred at intervaIs during several months prior to his admission. An appendecomy had been performed previously during one attack. When seen on this last admission, the obstruction prevented a thin suspension of barium which had been injected into the rectum from passing proxima1 to the sigmoid lesion. His past history revealed that since the age of three or four bloody mucus had been passed repeatedIy and that lower abdominal, cramping pains occurred frequentIy. In ten days under a medica regimen his obstructive symptoms were reIieved suffrcientIy to permit an operation which was directed to the obstructing lesion rather than proxima1 to it. Perforation of the Iesion and firm fixation to the left side of the parieta1 peritoneum had occurred and metastasis had extended to the regiona Iymph nodes and Iiver. As a paIIiative procedure the Iesion was freed from the abdomina1 waI1 and a modified MikuIicz procedure was performed. The patient Iived approximately ten months. SUMMARY

AND

CONCLUSIONS

Bleeding from the bowe1 in children strongly suggests the presence of an adenomatous polyp. During the earlier years these polyps are usuaIIy of the non-famiIia1 discrete type. The examination of 349 chiIdren presenting bowe1 symptoms disclosed the presence of one or more poIyps in one hundred instances. The painIess passage of bIood-tinged mucus was the single symptom common to this group. Protrusion of the tumor from the anus does not determine accurateIy the height of its attachment. The diagnosis depends upon adequate examination which incIudes proctoscopic examination and repeated roentgen ray studies when necessary. The possibility of malignant degeneration and of other serious complications incIuding anemia, obstruction and intussusception constitutes a definite indication for remova of the Iesions. RemovaI may be accomplished by fulguration if the Iesion is situated within 25 cm. of the

670 anaI outlet Ievel.

Kerr-Polyposis and by coIotomy

if it is above that

REFERENCES I. COFFEY, R. J. Multiple adenomatosis of the colon. Proc. Staff Meet., Mayo Cl&., 13: 741-742, 1938. 2. ERDMAUN. J. F. and MORRIS. J. H. Polvnosis of the colon. Surfi., G.nec. P Ok., 40: 460-468, 1925. 3. SWINTON, NEIL W. and WARREPU, SHIELDS. Polyps of the colon and rectum and their relation to malignancy. J. A. !bf. A., 113: 1927-r933, 1939. 4. HELWIG, E. B. Evolution of adenomas of the large intestine and their relation to carcinoma. Surg., Gynec. c++Ohst., 84: 36-47, 1947. DISCUSSION ROBERT TUKELL (New York, N. Y.): It is both a privilege and a pIeasure to discuss Dr. Kerr’s dealing with one hundred instances of paper, colonic PoIyps in juveniIes, of which onIy one may have been of the heredofamiIia1 type of poIyposis. The linding of colonic polyps in IOO out of 349 chiIdren who presented intestina1 symptoms is considerabIy higher than the one I obtained iI a comparabIe series of adults. Dr. Kerr’s high yieId of polyps in juveniIes raises two pertinent questions: (I) The possibihty of spontaneous regression or sIoughing of coIonic poIyps in children and (2) the possibility of the existence of some specific causative factors in the DaIIas series, future search for which may we11 shed some Iight on the formation of poIyps in generaI. BIeeding, as emphasized, is an important symptom, whiIe protrusion is a signilicant sign. Protruding poIyps may originate a Iong distance from the ana outlet. These either protrude by virtue of their Iong pedicIe or because of the spontaneousIy reducibie proIapse or even intussusception of the sigmoid into the rectum. The foregoing aIso hoIds true for digitaIIy paIpabIe but non-protruding PoIYPs+ The fact that thirty-two of Kerr’s patients had more than one poIyp imposes an obIigation upon us to search for additiona poIyps whenever one is discovered. The search incIudes proctosigmoidoscopy and repeated roentgenographic examinations. As emphasized, poIyps may undergo noninvasive and/or invasive malignant degeneration. 1 have encountered sessile polypoid Iesions the centers of which were the seat of an invasive adenocarcinoma that was hanked on each side by histoIogicalIy proved benign adenomatous poIypoid tissue. These are perfect exampIes of adenocarcinema deveIoping in a benign adenoma. As indicated, poIyps that are situated within the termina1 25 cm. of the coIon may be eradicated by surgica1 diathermy. The long-pedicled variety may be removed safeIy by means of an eIectrosurgica1 snare. Mediumor Iarge-sized sessiIe or short-

of CoIon pedicled adenomas that cannot be looped safely by a snare wire may be removed expeditiousIy and effectiveIy by means of a doubIe-loop eIectrosurgica1 resector. Of course, polypoid lesions situated above the reach of the sigmoidoscope are removed transabdominaIIy by appropriate surgica1 procedures. J. &fILTON STOCKhlAK (KnoxviIIe, Tenn.) : There are some very interesting ideas given us by Dr. Kerr’s paper. First, in this series of one hundred cases of polyps in children, he reports two who deveIoped carcinoma foIlowing a delinite poIyp history. The occurrence of these carcinomas took us into the third decade of Iife. In checking over the percentage of carcinomas in this region we lind that roughIy 4 per cent of those discovered occurred within the first thirty years of life. It makes us wonder if, by earIy diagnosis and eradication of these growths, we may not hope to reduce carcinoma markedIy in this age group. It is very true that a11 poIyps do not undergo maIignant changes but we know that carcinoma in the young is very rapid and the chances of proIonged surviva1 are very poor. I wouId Iike to emphasize the ease of diagnosing a majority of these polyps in chiIdren. FuIIy 70 per cent occur near the rectosigmoida1 juncture. The Iower bowe1 of the child is more mobiIe than that of the aduIt and, due to the shaIIow buttocks, one is abIe to paIpate we11 beyond the vaIves. The type that we usuaIIy lind is a true adenoma and quite easiIy feIt, whereas the papiIlomatous type rareIy occurs in chiIdren. These are the spongy, soft tumors that are dillicuIt to fee1 (Binkley). The squatting position faciIitates paIpabiIity over an even higher area, and with straining enabIes those situated higher on long pedicIes to descend. Dr. Kerr has emphasized that each patient gave a history of painIess bIeeding and I do not think that we can say this too often. We Iike to think of blood streaking of the stool as a diagnostic sign whiIe bIood-streaked mucus is a suggestive sign. In our experience we seem to meet proIapsing of the rectum compIicated by a poIyp more frequentIy than in this report. Schwartz, GoIdberger and Crockett state that a polyp is “frequentIy the etioIogy of the proIapsing of the rectum.” Our ratio is approximately one to three, and WC never instigate treatment of this condition until we are relativeIy sure there is not a complicating polyp. In our opinion this paper is a distinct contribution to the series that has been presented to this society over a period of years and should make us

more cognizant of the occurrence of poIyps in a11 age groups and urge us on toward early remova1. AIthough on several occasions the parents have come in with evidence to show that the polyp has sIoughed and a perfectIy good operation has been ruined, I believe that we shouId check these areas

American

Journal of Surgery

Kerr-Polyposis at the time for a possible bleeding or incompIetc slough, and recheck every six months for either additiona polyps, as advocated by W. J. Martin, or the recurrence, as suspected by Swinton and proved by the rare pyogenic poIyp as reported by Tom Smith. In conclusion, may I say that throughout this discussion, I wish in no way to minimize the use of the sigmoidoscope and x-ray studies as diagnostic aids. JACK G. KERR (cIosing): I appreciate the discussion of Dr. TureIl and Dr. Stockman. I agree with them that a considerabIe number of these Iesions {vi11sIough from their attachment and be extruded. I think this happened in approximateIy nineteen of the patients studied in this group. Frequently this is the explanation for the singIc episode of meIena that occurs in children who have had no previous symptoms referable to the coIon. A significant amount of bIood may be passed and concrete evidence of a polyp having sloughed may bc furnished by the mother who has recovered the tumor foIIowing its expuIsion. Dr. Stockman mentioned that, if possible to

December,

I 948

of Colon

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reach it by sigmoidoscopy, the area from which the poIyp sIoughed should be examined. I think this precaution is important: first, because the pedicle may continue to bIeed and, secondIy, the adenomatous tissue may not separate compIeteIy and the smaI1 remaining nidus wiI1 be responsibIe for a recurrence of the poIyp. Light fulguration of the area usuaIIy controIs either situation. One other thought about the diagnostic use in chiIdren of a barium enema: It is of practica1 vaIuc that the enema foIlow rather than precede the remova of a11 poIyps visuaIized through the sigmoidoscope. ShouId a poIyp be found by x-ray methods after those in the distaI25 cm. of the borne1 have been removed, it is immediateIy apparent that Iaparotomy wiI1 be necessary for its remova1. The routine use of a barium enema in each patient immediateIy foIIowing removal of a11 poIyps in the dista1 25 cm. of the coIon is perhaps not mandatory as once considered. If poIyps are present, symptoms wiI1 be observed if the mother is properIy and adequateIy instructed. In onIy those patients whose symptoms continue are barium enema studies advised.