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Congenital aganglionic megacolon
Congenital
Aganglionic
HAROLD AXELROD,
M.D.,
Richmond,
s recently as 1947 it couId justihabIy be said “the surgica1 treatment of megacoIon, aIthough, perhaps, statisticaIIy more satisfactory than the use of medicaments, is far from idea1 and such methods of operation as entero-anastomosis, drainage procedures, resection of segments of the bowe1, and so forth, although they yieId satisfactory resuhs in many instances, have utterIy faiIed to give relief in many other cases.“* At that time it couId aIso be said that “congenital megacoIon coIitis is a poorIy understood disease for which no reahy satisfactory method of treatment has yet been devised.“15 As is customary in those instances in which the etioIogy of a disease is one of specuIation rather than knowIedge, numerous theories were proposed in the search to determine what was the actual pathoIogy in Hirschsprung’s disease. Investigators were we11 aware that the etioIogy remained hidden. It is a matter of not infrequent note that once a discovery is made, it appears to have been so simpIe and apparent that it is a wonder it was not made Iong before. That is the situation in regard to the condition known as Hirschsprung’s disease or, now more accurateIy congenita1 aganghonic megacoIon. termed, With its specific etioIogy now cIear, the wonder is, indeed, that it was not discovered Iong ago. Many anatomists and physioIogists had indicated the Iocation of the abnormality. As earIy as 1901 in a report of one case of megacoIon, TitteIz2 observed that the ganglion ceIIs of the myenteric pIexus of the Iarge bowe1 showed signs of degeneration and were scanty as we11 at different IeveIs. In a paper on megacoIon in 1907, Hawkins9 postmated the idea of a physioIogic obstruction due to a neuromuscular defect as the cause of the disease. In 1920 and in 1924 DaIIa VaIIe6z7 reported two cases of congenita1 megacoIon occurring in two brothers. In each instance he demonstrated the absence of nerve ceIIs in Auerbach’s pIexus in the distal, undiIated portion of the bowe1. Journul oj Sur,qery, Volume PO, July,
,953
Calijornia
Cameron4z5 reported inflammatory ceI1 repIacement of the gangIia of the myenteric pIexus in the dista1, undiIated portion of the bowe1 in 1927 and 1928. Etze18 described absence of the nerve ceIIs of the normal-appearing dista1, Iarge intestine in four cases of megacoIon. In 1934 Perrot and Danon18 reported a case of an autopsied fifteen-day old infant whose death from intestinal obstruction was attributed to a physioIogic obstruction resuIting from poor deveIopment of the myenteric pIexus. In 1938 Robertson and Kernohanlg reported the resuIts of study of a boy whose death resuIted from a typica case of megacolon. They reported that the ganglion ceIIs and fibers of the plexus of Auerbach were definitely smaJIer than normaI, vacuoIated and the ganglion ceIIs were absent or very imperfectly formed. They noted these same hndings in severa other cases of megacoIon. In 1948 Whitehouse and Kernohan24 reported a very exceIIent study of eIeven cases of congenital megacoIon. Th ey concIusiveIy demonstrated the absence of the gangIion ceIIs of the pJexus of Auerbach in the distal, undiIated portion of the bowe1 and showed that this absence was not due to inflammatory change, vacuoIation or any other repIacement of gangJion ceIJs, but rather was due to a compJete absence of the ceIIs which had never been present. This was emphatic support for the congenita1 basis of this disabiIity. They further added support to this concept in their study of Iive cases of secondary megacolon. In no instance in these latter hve cases was there absence of the ganglion ceIJs of Auerbach’s pIexus. In 1923 Ishikawa r2,13stated that interruption of the sacra1 parasympathetic fibers to the coIon was the cause of megacolon. Adamson and Aird’ sectioned the same fibers in their work on cats and produced megacoIon. KIeinschmidtI was abIe to verify this. From these factors it is apparent that much evidence existed to indicate that there was some neurogenie factor which was the cause of this disease. On this basis there evoIved the idea of Iumbar
A
American
Megacolon
18
Congenital
Aganglionic
Megacolon
able to report consistently successful resuits as well. In March, 1951, Hiatt,‘” who had been working aIong Iines simiIar to Swenson, published a paper on the pathologic physiology of congenital megacolon, and clearly expounded the disturbance which was present. He folIowed this at the same time with a paper” describing his modification of the procedure described b3 Swenson and Bill, and reported its use in twenty-three patients.
sympathectomy as a specific procedure for this disease. WhiIe beneficial resuIts were claimed to derive from this procedure, many faiIures were reported as well. The disease was stil1 uncomprehended. With the mistaken idea of the dilated portion of the boweI as the seat of pathology, resections of this segment of the bowel were undertaken. Again the resuIts of this were equivocal. Spasm of the sphincter ani was also presumed as a possibIe cause of the disabiIity and attempts were made to rectif.y the pathoIogy by eliminating this factor. AI1 these measures simpIy skirted the actual facts. There was a factor of neurogenic imbalance but the absence of the gangIion ceils of the myenteric pIexus was the precise cause of it. There was a factor of obstruction, not due to an organic cause, but the result of a physiologic obstruction. There was the factor of inspissated feces resulting from marked dehydration due to the increased length of intestine with greater water-absorbing surface lvhich, in turn, was secondary to the physioIogic obstruction. AI1 these factors fringed on the precise cause of this condition but none was quite accurate. In 1948 Swenson and BilIzO reported on a study, made in the previous two years, of twenty patients in whom a cIinica1 diagnosis of congenital megacolon had been made. By means of a specia1 technic they were abIe to demonstrate an area of spasm in the rectosigmoid or rectum. In the miIder cases among these twenty the treatment suggested by Law’6 had been successful. Colostomies performed proxima1 to the area of spasm in six patients had relieved them compIeteIy. In three of these recurrence of the disability ensued when closure of the colostomy was performed. They, therefore, believed that removal of the area of spasm in its entirety would reIieve the functional obstruction. To do this they devised an entirely new modification of the procedures described by MaunselI17 and Weir.23 The procedure was performed on dogs and gave such encouraging resuIts that the operation was performed on three patients with exceIIent results. By August, 1950, Swenson”’ was abIe to report on fifty-two cases with exceIIent results in fifty-one and an anticipated similar result in the fifty-second. (Too dista1 a resection had been performed, requiring a second operation with a more proxima1 resection.) Stephens et a1.,3 who had observed Swenson’s work, were
PATHOLOGIC
PHYSIOLOGY
Only a proper understanding of the anatomy and physiology will make cIear the pathologic disorders present in this disease. The nerve suppIy of the Iarge intestine consists of two plexuses: (I) Auerbach’s or the myenteric pIexus which Iies between the two muscular coats and, (2) Meissner’s pIexus which lies in the submucous Iayer. A peristaltic wave in the coIon is a muscuIar contraction passing from the proxima1 to the dista1 part in a continuous manner. This is mediated through the Auerbach pIexus with the ganglia serving as control stations for the nervous impuIse so that the latter is graded and orderly, reaching the musculature at onIy one point at a time and causing that point to contract; and then passing in a sequentia1, regulated manner to the next distal point, causing the latter to contract while the previously contracted point relaxes. In this manner a we11 regulated peristaItic wave passes aIong the entire colon, from the cecum to the rectum. The time occupied in the passage of this wave from the beginning to the terminus of the Iarge intestine is easily measurable by the eye. However, for all practical purposes the passage of an impulse along a nerve fiber may be considered instantaneous. If the nerve impulse were carried aIong a segment of the colon at this speed, it is readily apparent that such a segment wouId contract throughout its length simultaneously; there wouId be no graded impuIse and there wouId simply exist a spastic contracture aIong such a segment of bowel which, with the passage of the impulse, would reIax aIong its entire length simultaneously. It is apparent that no impetus exists to propel anything along the Iumen of such segment forward or, for that matter, backward, Anything within such segment of bowel will simply be squeezed out at either end, orac1 and caudad. The designation of this disease as congenital ‘9
AxeIrod diIatation of the Iatter occurs. If the bIockage is compIete, acute intestinal obstruction resuIts, in no manner different in resuIt to the proxima1 part of the bowe1 than if an acute voIvuIus were to occur in the narrowed segment. It is characteristic of these patients that they do have episodes of acute intestinal obstruction. This resuIts in the death of many of these infants at an early age. If the obstruction is not reIieved a11 the sequelae of acute intestina1 obstruction ensue up to death. Swenson and BiII noted that when proximal coIostomy was performed as a lifesaving measure in six of their cases, the symptoms of the disease compIeteIy disappeared. When the coIostomies were cIosed in three of these, prompt recurrence of the disease took place and fuI1 reIief was again provided onIy by re-estabIishment of the coIostomies. However, where compIete obstruction does not occur, a chronic obstruction resuIts and it is in this aspect of the disease that the characteristic picture of congenita1 aganglionic megacoIon appears. With the piIing up of the feces in the proxima1, norma portion, the Iatter becomes secondariIy diIated. As this feca1 accumuIation increases, the diIatation increases. As the dilatation continues the peristaltic waves become more forceful. As is true of any muscIe in the body, increased use resuhs in hypertrophy. The hypertrophy taking pIace here refers to both the circuIar and IongitudinaI muscIe. With the hypertrophy there is, of course, thickening of the waI1 of the bowe1 as we11 as Iengthening. In no other physioIogic manner can the diIated, Iengthened and hypertrophic bowel waI1 be expIained. This diIatation and Iengthening of the bowe1 provide a tremendousIy increased mucosa1 surface. As a resuIt of this, continued over a period of time, the proxima1, normal bowe1 becomes tremendousIy diIated with dehydrated feces, distending the abdomina1 waI1 unti1 the characteristic picture of the chiId with the tremendousIy diIated abdomen, the ffaring ribs, the spindly Iegs and the retarded deveIopment appears.
agangIionic megacoIon describes the disease preciseIy. It is congenita1 and agangIionic because of the congenita1 absence of the gangIia of the myenteric plexus in the part of the bowe1 dista1 to the diIated portion. The disease is, in fact, in the dista1 undiluted portion of the Iarge intestine rather than in the dilated portion which contributes the word megacoIon to the designation of the disease. The diIated portion of the bowel is, indeed, entireIy normal; its greatIy increased lumen, thickened waI1 and eIongation are entireIy physioIogic reactions of normal bowe1 to a chronic obstruction distal to it. In the narrowed segment distal to the diIated portion of the coIon, the gangIia of Auerbach’s pIexus are absent but nerve fibers are present. (The nerve fibers are non-myeIinated and Iie where Auerbach’s pIexus wouId normaIIy be situated. These non-myelinated nerve fibers are not present to any marked degree where the myenteric pIexus is present. The significance of these different fibers is unknown.23) The length of this segment is dependent directIy upon the Iength over which the gangha are missing. When the nerve impuIse from the proxima1, normal portion reaches this part of the bowe1, an intense spastic contraction takes place, the lumen of the bowe1 is greatly contracted, if not entirely eIiminated, the norma peristaItic wave reaching it is ended and with it the forward prop& sion of the boIus from the proximal, norma portion. In effect, an aImost complete obstruction is present at this junction of the normal with the agangIionic segment of the bowel. A vicious cycIe is now set up. As the feces from the normal bowel reaches the agangIionic portion, the momentum imparted to them by the peristaIsis propeIs some of them into the Iatter segment. As the forward movement behind continues, feces are packed into the first portion of the narrowed part of the bowe1. This part of the feca1 stream can move forward onIy by sufficient push from the propuIsive efforts of the bowe1 orad to it. MeanwhiIe, though the aganglionic part of the bowe1 is incapabIe of peristaItic function, its other functions are not impaired. Water absorption from the feces in it continues and as it does the feces become drier and harder. The pounding from behind continues. An impaction now results, providing a stronger bIock to the feces constantIy being propeIIed into the aganglionic portion. With the feces now backing up into the norma bowe1,
CLINICAL
COURSE
With the proper use of enemas and other non-operative means of care, these chiIdren are not as severeIy disabIed, but their disabiIity is nevertheIess marked. Hiatt has pointed out the danger of the enema itseIf when it is of the tap-water variety. He has shown that the danger of the tap-water Iavage 20
Congenital
AgangIionic
MegacoIon
borns in an acuteIy obstructed condition. There the need for differentiation wiI1 be onIS between congenita1 aganglionic megacoIon and organic obstruction, compIete or incomplete. With agangIionic megacolon a history will usuaIIy be obtained of some anal expulsron of feces folfowing birth. In those newborns with compIete organic obstruction there will never have been a bowef movement ancf the scout film of the abdomen wiI1 show the point of obstruction inasmuch as there will be no bowe1 gas noted distaf to that site. In those infants and chifdren older than the acute intestina1 obstruction maJ newborn, aIso occur. These patients will have a history pathognomonic of this disease. Certainly no IegaI statute exists stating that these chiIdren cannot develop an obstruction due to other causes. The differential diagnosis here shouIcf not cause serious diffIcuIty.
consists in the very rapid absorption of the hypotonic soIution through the greatIy increased absorbing surface combined with the increased hydrostatic pressure. The resuIt is water intoxication and these patients, not uncommonly, are found to be in extreme weakness and pallor with marked sweating folIowing this type of enema. He ascribed the death of one of these patients, occurring immediateIy after such enema, to this fact. This is another factor accounting for the small number of these patients reaching adulthood. To minimize the dangers of the enema in these patients he has recommended a sofution of 7 per cent geIatin which has an osmotic force equa1 to that of the circulation. As is not unusuaf when one congenitaf defect exists, others often accompany it. Swenson investigated twenty patients with agangfionic megacofon by means of cystograms. He found that nineteen of them showed hypotonicity of the bladder, the bladder capacity of these patients being far above average before any detrusor contractions occurred. This is an indication of a deficient pelvic parasympathetic nerve supply. This is a matter of importance, for in the course of freeing the rectum prior to its removal from the pelvis, as is done in the surgical treatment of this condition, injury to the parasympathetic nerve suppIy to the bladder may easily occur since their precise course is unknown and the nerve fibers are too small to he dissected out.
TECHNIC
Where the obstruction is complete, expforatory Iaparotomy will be required with the performance of the shortest possible procedure consistent with saving the patient’s fife. This wiff usuafly consist of a transverse loop colostomy definitely proximal to the contracted segment. It would usually be unwise to attempt to give an enema with the idea of loosening the impacted feces and thereby allow the gas behind to be expelled. The increased intracoIonic pressure may possibIy be enough to resuIt in a bfowout of the tightly distended cofon. It shouId be a matter of note that in any acute obstruction there is insuffcient time for the bowel muscuIature to become hypertrophic. Consequently the bowel w-all proximal to the obstruction is thinned. Bowel wall hypertrophy occurs only where there is adequate time for development as in chronic obstruction. When no emergency exists, the patient may be fufIy assayed and prepared for definitive surgery. While no dogmatic rufes about performance of this operation reIative to the size of the patient can he made, the child with this disease who is two years of age or older is a suitabIe candidate. In those younger, a transverse cofostomy weIf proximal to the narrowed, distal segment should be performed until the child’s size is sufficient to permit performance of the specific procedure. Those who are experienced in performing this procedure can
DIAGNOSIS
The diagnosis of congenita1 agangIionic megacofon is not too diffIcuIt. At or shortIy after birth obstinate constipation becomes apparent. By means of enemas aIone these chifdren are able to evacuate their boweIs. So long as enemas are diIigentfy given, these children will have bowel movements. Despite the administration of enemas the overwhefming majority of children will appear with a greatIy distended abdomen, although not necessariIy sick. Their parents will report that they eat well if they are given enemas with any degree of regularity. Some wifI definiteIy show physical and mental retardation; others wiIf not. There wiIf usually be a history of treatment by medical means over a Iong period of time, but this treatment wiI1 not have affected them any more than when they received enemas afone. Some of these patients may be seen as new21
AxeIrod certainIy execute it in smaIIer chiIdren. On the average, however, it is desirabIe to wait unti1 the patient’s size is such that the structures may be seen and dissected with greater accuracy. The operation consists of remova of the entire narrowed, distal segment, the transitional area and a variabIe part of the distal, diIated part of the colon. Beside the customary means used to prepare a patient in this age group for major abdominal surgery of an eIective nature, it is desirable to decompress the diIated bowel before going to surgery. By means of diet, enemas and intestina1 antiseptics this can be obtained, as we11 as providing a steriIe, colonic Iumen. It is aIso highIy desirabIe to intubate a vein, usuaIIy the greater saphenous at the ankIe, with a poIyethyIene catheter prior to the start of surgery. Once the patient is properIy prepared the procedure used is the operation devised by Swenson with Hiatt’s modification. Entrance to the peritonea1 cavity is provided by either a Iower Ieft, vertical rectus incision or an obIique one starting in the region of the right pubic spine and extending in an obIique Iine across the midline, cutting the Ieft rectus muscIe and extending to the Ieft flank. The finding of the junction of the diIated, hypertrophied segment with the dista1, narrowed segment (the transitional area) is the point at which bearings are taken. The superior hemorrhoida vessels are found and cIamped along the transitiona segment. The transitiona portion and a11 the Iarge bowe1 dista1 to it are now dissected free from a11 the structures attached to them. It must be constantIy kept in mind that in this dissection it is the absence of the gangIion ce1Is lying between the Iongitudina1 and circuIar muscuIatures that is the cause of this disease. Therefore, the removal of the tube of bowe1 where this deficiency exists without the incIusion of any attached structures is the desired dissection. Furthermore, since so many of these patients have poor innervation of the pelvic viscera, disturbance of any of the structures other than the bowe1 itseIf is highIy undesirabIe. The narrowed segment is, therefore, stripped down very cIoseIy aIong its outer coat to the insertion of the Ievator ani into the anus. Because the normal but diIated and hypertrophied portion has been propeIIing feces into the proxima1 part of the abnorma1 segment, some diIatation of a variabIe Iength of the proximal part of the
Iatter is present. For this reason it is necessary to resect a variabIe portion of the diIated segment orad to the transitiona area. More preciseIy, frozen sections of the muscuIaris taken at intervals aIong this part of the bowel wiI1 revea1 where the ganglia are present in norma number. This may, however, resuIt in undue proIongation of an aIready lengthy procedure. By pathoIogic examination 12 cm.21 and 20 crn.lO have been suggested as a sufIicientIy safe orad point of resection. In Iine with those suggestions the Iength of bowel resected will vary with the distance between the transitiona area and the anus. It may require anastomosing the spIenic flexure or even the transverse colon to the anus. The bIood suppIy to the unresected portion of the bowe1 must, of course, remain unimpaired. It may be found that the only thing preventing approximation of the point of resection of the bowe1 wall to the anus without tension is the inabihty of the blood vesseIs to stretch that distance. Where this is so, the mesentery must be cut cIose to its origin and the bIood vessels hindering this movement must be Iigated close to their origin, usuaIIy the inferior mesenteric vesseIs. With that done the arcades and the margina artery are preserved, permitting the vascuIar suppIy to remain intact and at the same time aIlowing the necessary mobiIity. This having been accomplished, attention IS now directed to the perineum. The anus is diIated, an AlIis clamp inserted from outside the body into the rectum and directed by a hand in the abdomen to as high a position as possible, the bowel is cIamped and is drawn down to the anus and out, intussuscepting the bowe1 through the anus. It is drawn out far enough so that the point which has been eIected for anastomosis to the anus is brought into proper position for the performance of the anastomosis on the perineum. A two-layer anastomosis may be done here, cutting away at the same time the diseased howeI. At the completion of this step the newly formed anastomosis is pushed back into the body through the anus. A drain may or may not be inserted into the presacraIretro-anastomotic area through a stab incision between the tip of the coccyx and the posterior margin of the ana ring. CASE REPORTS CASE I. D. L., a six year oId boy, was seen with the compIaints of abdomina1 distention, 22
CongenitaI
AgangIionic
Megacolon
extends completely across the abdomen to the Ieft side. No attempt was made to fill the proximal colon. Wide mucosal foIds are visible in this ditated intestine.” The patient was prepared for surgery b?means of daily enemas of physiologic saline until cIear return, administration of oral sulfasuxidine and miIk of magnesia and cascara cathartics. Orally the patient received daily multivitamin capsuIes and a Iow residue, high protein, high calorie diet. When the distention of the abdomen had been reduced to an almost norma appearance and the enema return showed soft stool without odor, the patient was subjected to the operation. His postoperative course was uneventful. He had a spontaneous bowe1 movement on the third postoperative day. OraI feedings were started that same day. Sutures were not removed until the tenth postoperative day and the patient was discharged on the thirteenth postoperative day. There has been no diarrhea and the patient has one to two formed, soft boweI movements daily. In the eighteen months since the operation the patient has shown a much more rapid rate of growth than previousIy. and he has put on weight in accordance with it. The treatment of the Perthes disease of the left hip was started four months after the operation under orthopedic care and is continuing. CASE II. C. hI., a two and a half year old girl, had had a few bowel movements immediately after birth but had aIso become distended abdominally within one month after birth. Treatment bv the various medical procedures was carried out in conjunction with enemas; and when the medication was haIted and the enemas aIone used, the child did as weII. AIthough enemas had been given daiIy there had always been present a marked degree of abdomina1 distention. No history of urinary dif&uIty was obtained. Physical examination revealed a two and one-half year old girl who did not appear to be behind the developmental pattern for her age. She appeared to be fairIy active. Her large protuberant abdomen, which in its state was symmetric, bulged up to the costal margins, causing the ribs to Aare. Her hemic component appeared to be normal. The abdomen was firm but not hard, not tender and no masses couId be felt. Neither liver nor spIeen was paIpabIe. No other abnormalities were noted. A barium enema reveaIed the findings
frequent episodes of iIIness and obstipation. Since the second month of his birth the parents could bareIy recaI1 when he had had a bowe1 movement spontaneously. Enemas and cathartics were used regularly to obtain bowe1 movements. There had been episodes when he was particuIarIy ill and when he wouId fInaIIy have a bowel movement after several attempts. At such times the excreta consisted of very firm, scybaIous balIs. Between such episodes the feces were very firm as weJl. The child went to school and was an average student. His mixture with his classmates was greatly limited because of his inability to keep up with their activity. He had been under the care of doctors who had treated him medicaIIy with very IittIe improvement above that obtained by enemas and cathartics aIone. Physica examination reveaIed a paIe, white boy who appeared his age of six but who definitely lacked the vigor seen in a we11 child of that age. His abdomen was the most prominent part of his anatomy because of its marked distention. It was symmetricaIIy distended and bulged the ribs outward, causing them to flare. Several Iarge veins coursed over the abdomen. On palpation the abd omen was firm but not hard. No tenderness was present unIess heavy and deep paIpation were done. No masses could be feIt. There was no rebound tenderness. The extremities were somewhat thinner in appearance than a norma chiId of the same age. He was paIe and the skin, as a resuIt of this and the absence of bIemishes, had an “ethereal” appearance to it. The only other abnormality of note was a Iimp in the Ieft lower extremity due to Perthes disease of the ieft hip. Barium enema reveaIed the typica findings in this disease. The radioIogist’s report foIIows: “Examination of the colon, by barium enema, demonstrates that the rectum and dista1 sigmoid did not distend to the degree that is ordinariIy seen in the course of a barium enema, but these structures were normaI in contour. The proxima1 rectum extends directIy supcriorIy in the midIine to a point anterior to the interspace between L 4 and 3 and then turns anteriorly and to the right. At the point of turn the lumen fairly abruptIy widens to about 3 times the caIiber of the sigmoid distal to this point. The sigmoid is very Iong and this dilated portion extends to the right to overIie the IateraI portion of the right ileum and then 23
AxeIrod typica of the disease. The radio1ogistY.s report foIIows: “A preIiminary fiIm of the abdomen shows severa markedIy diIated gas-fiIIed Ioops of intestine, undoubtedIy coIon, in the right abdomen and mid-line and severa Ioops of smaI1 intestine, not diIated, and containing onIy a IittIe gas, in the Ieft abdomen. Examination of the coIon by barium enema showed that the rectum and dista1 sigmoid were essentiaIIy normal in caIiber, but beginning at about the mid-sigmoid, there was very marked diIatation of the proxima1 coIon. The sigmoid extends upward and to the right so that the point of junction of non-diIated and diIated sigmoid is in the right upper quadrant. The post-evacuation fiIm shows that the dista1 sigmoid and rectum has expeIIed most of the barium from that area, but the proxima1 sigmoid and the coIon retained the barium. This examination shows evidence of megacoIon. The narrow segment of intestine begins at about the midsigmoid and involves the dista1 sigmoid and rectum.” After four days of preparation (morning and evening saIine enemas, miIk of magnesia with cascara daiIy, and suIfasuxidine combined with a low residue, high protein, high caIorie diet) the patient was taken to the operating room. The Iarge bowe1, extending orad from a point z cm. above the pectinate line (incIuding the entire narrowed segment) and 20 cm. cephaIad to the transitiona area, was dissected free, intussuscepted through the anus and resected as the anocoIonic anastomosis was performed on the perineum. The postoperative course was uneventfu1. On the second postoperative day the patient started to have bowe1 movements of gelatinous consistency and was aIIowed up and about freeIy. By the fifth postoperative day she was eating a reguIar diet, her bowel movements were onIy two per day and she was discharged. Since that time (sixteen months ago) she has had no diffIcuIty, her abdomen has receded to norma size and she has been norma in every respect for a chiId her age.
ease, have definitely estabIished the cause of this disease entity and resulted in a curative procedure. An attempt has been made to cIarify the steps in the deveIopment of the cIinica1 entity Iong noted in this disease. Two cases have been presented to iIIustrate the exceIIent resuIts that can be achieved by operative correction of this congenita1 anomaIy. REFERENCES I. ADAMSON, W. A. D. and AIRD, I. MegacoIon:
2.
3.
A. 7
5.
6.
7. 8.
9. IO.
evidence in favor of a neurogenic origin. Brit. J. Surg., 20: 220-233, 1932. BANCROFT, F. W. and WADE, P. A. SurgicaI Treatment of the Abdomen. PhiIadeIphia, 1947, J. B. Lippincott Co. BODIAN, M., STEPHENS, F. D. and WARD, B. C. H. Hirschsprung’s disease and idiopathic megaCOIOn.LQnCet, I : 6-I I, 1949. CAMERON. J. A. M. OesoDhaeectasia in chiId. Arch. Dis. Childhood, 2: 358-360, 1927. CAMERON, J. A. M. On aetiotogy of Hirschsprung’s disease. Arch. Dis. Childhood, 3: 210-211, 1928. DALLA VALLE, A. Richerche IstoIogiche su di un Cas di MegacoIon Congenito. Pediatria, 28: 74*752, 1920. DALLA VALLE, A. MegacoIon FamiIia. Pediatria, 32: 569-599. 1924. ETZEL, E. La Dilatation de1 Esofago Frente a Ias Lesiones de1 PIexo de Auerbach en eI Megaesofago. Bol. y trab., Sot. de cir. de Buenos Aires, 21: 131-148, 1937. HAWKINS, H. P. Remarks on idiopathic diIatation of the colon. Brit. hf. J., I: 477-483, 1907. HIA~, R. B. The pathologic physioIogy of congenitaI megacoIon. Ann. Surg., 133: 313-320, 1
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195’. I I. HIATT, R. B. The surgica1 treatment of congenitaI megacoIon. Ann. Surg., 133: 321-329, 1951. 12. ISHIKAWA. N. ExperimenteIIe Untersuchungen iiber die Djckdarminnervation, Insbesondere des CoIon Descendens et Sigmoideum. Mitt. a. d. med. Fak. d. k. Univ. Kyusbu Fukuoka, 7: 295338, 1923. 13. ISHIKAWA, N. ExperimenteIIe und KIinische Untersuchungen uber die Pathogenese und das Wesen des MegakoIons. Mitt a. d. med. Fak. d. k. Univ. Kyusbu Fukuoka, 7: 339-400, 1923. 14. KLEINSCHMIDT, cited by BOCKUS, H. L. GastroEnteroIogy, vol. 2, p. 404, PhiIadeIphia, 1943. W. B. Saunders Co. 15. LADD, W. E. and GROSS, R. E. AbdominaI Surgery of Infancy and ChiIdhood. PhiIadeIphia, 1947. W. B. Saunders Co. 16. LAW, J. L. Treatment of megacoIon with acetyI beta methy choIine bromide. Am. J. Dis. Child., 60: 262, 1940. 17. MAUNSEL, H. W. A new method of excising the two upper portions of the rectum and the lower segment of the sigmoid ffexure of the coIon. Lancet, 2: 473, 1892.
CONCLUSIONS
Experimenta and pathoIogic observations over a Iong period of time, cuIminating in an operative procedure devised by Swenson to verify concIusions reached concerning the pathologic physioIogy of Hirschsprung’s dis24
CongenitaI
Aganglionic
I8. PEKROT,A. md DAIXOX,L. Obstruction IntestinaIe de Cause Rare, Chez un Nourison. Ann. d’anat. &J., 12: 157-165, 1935. 19. ROBERTSON, H. E. and KEKNOHAN,J. W. Myenteric megacolon. Proc. Staff pJtXUS in congenital *Meet., izIayo Clin., 13: 123-125, 1938. 20. SL\ENSOY,0. and BILL, A. H., JR. Resection of rectum and rectosigmoid with preservation of the sphincter for benign spastic lesions producing megacolon: experimenta study. Surgery, 24: 2 I 2-220, 1948.
Megacolon
SWENSON,0. A new surgica1 treatment for 1 lirschsprung’s disease. Surgery, 28: 371-383, 1950. 22. TITTLE, K. Ueber eine Augeborene Missbildung des Dickdarmes. Wien. klin. Wchnschr., r4: 903-907, ,901. 23. WEIK, R. F. An improved method of treating high-seated cancer of the rectum. J. A. ,2/1. A., 37: 801, 19or. 24. WHITEHOUSE,F. R. and KERNOHAY,.J. W. hlycnteric plexus in congenital megacolon; study of I I cases.Arch. IN. Med., 82: 75--1 I I, 1948. 2 I.
KINSELL has had a very favorabIe experience using hormone therapy in addition to antibiotics in acute inflammatory surgica1 cases. Of course the hormones he used (ACTH or cortisone) are known to increase the breakdown of protein tissue and the loss of chlorides and potassium. Therefore, these patients need increased quantities of proteins intravenously and potassium chloride. These hormones also cause an increase in body retention of sodium, so the use of intravenous sodium shouId be greatly limited. KinseII reaIizes there are certain contraindications to the use of these hormones, especiaIIy in patients with gastrointestiona1 uIceration, insuffrcient renaI function, congestive heart disease or tuberculosis. ManJ patients in a debiIitated condition who need non-emergency surgery wiI1 make a better convaIescence if treated by these hormones for two days prior to surgery and three days afterward. (Richard A. Leonardo, M.D.)
Aganglionic
HAROLD AXELROD,
M.D.,
Richmond,
s recently as 1947 it couId justihabIy be said “the surgica1 treatment of megacoIon, aIthough, perhaps, statisticaIIy more satisfactory than the use of medicaments, is far from idea1 and such methods of operation as entero-anastomosis, drainage procedures, resection of segments of the bowe1, and so forth, although they yieId satisfactory resuhs in many instances, have utterIy faiIed to give relief in many other cases.“* At that time it couId aIso be said that “congenital megacoIon coIitis is a poorIy understood disease for which no reahy satisfactory method of treatment has yet been devised.“15 As is customary in those instances in which the etioIogy of a disease is one of specuIation rather than knowIedge, numerous theories were proposed in the search to determine what was the actual pathoIogy in Hirschsprung’s disease. Investigators were we11 aware that the etioIogy remained hidden. It is a matter of not infrequent note that once a discovery is made, it appears to have been so simpIe and apparent that it is a wonder it was not made Iong before. That is the situation in regard to the condition known as Hirschsprung’s disease or, now more accurateIy congenita1 aganghonic megacoIon. termed, With its specific etioIogy now cIear, the wonder is, indeed, that it was not discovered Iong ago. Many anatomists and physioIogists had indicated the Iocation of the abnormality. As earIy as 1901 in a report of one case of megacoIon, TitteIz2 observed that the ganglion ceIIs of the myenteric pIexus of the Iarge bowe1 showed signs of degeneration and were scanty as we11 at different IeveIs. In a paper on megacoIon in 1907, Hawkins9 postmated the idea of a physioIogic obstruction due to a neuromuscular defect as the cause of the disease. In 1920 and in 1924 DaIIa VaIIe6z7 reported two cases of congenita1 megacoIon occurring in two brothers. In each instance he demonstrated the absence of nerve ceIIs in Auerbach’s pIexus in the distal, undiIated portion of the bowe1. Journul oj Sur,qery, Volume PO, July,
,953
Calijornia
Cameron4z5 reported inflammatory ceI1 repIacement of the gangIia of the myenteric pIexus in the dista1, undiIated portion of the bowe1 in 1927 and 1928. Etze18 described absence of the nerve ceIIs of the normal-appearing dista1, Iarge intestine in four cases of megacoIon. In 1934 Perrot and Danon18 reported a case of an autopsied fifteen-day old infant whose death from intestinal obstruction was attributed to a physioIogic obstruction resuIting from poor deveIopment of the myenteric pIexus. In 1938 Robertson and Kernohanlg reported the resuIts of study of a boy whose death resuIted from a typica case of megacolon. They reported that the ganglion ceIIs and fibers of the plexus of Auerbach were definitely smaJIer than normaI, vacuoIated and the ganglion ceIIs were absent or very imperfectly formed. They noted these same hndings in severa other cases of megacoIon. In 1948 Whitehouse and Kernohan24 reported a very exceIIent study of eIeven cases of congenital megacoIon. Th ey concIusiveIy demonstrated the absence of the gangIion ceIIs of the pJexus of Auerbach in the distal, undiIated portion of the bowe1 and showed that this absence was not due to inflammatory change, vacuoIation or any other repIacement of gangJion ceIJs, but rather was due to a compJete absence of the ceIIs which had never been present. This was emphatic support for the congenita1 basis of this disabiIity. They further added support to this concept in their study of Iive cases of secondary megacolon. In no instance in these latter hve cases was there absence of the ganglion ceIJs of Auerbach’s pIexus. In 1923 Ishikawa r2,13stated that interruption of the sacra1 parasympathetic fibers to the coIon was the cause of megacolon. Adamson and Aird’ sectioned the same fibers in their work on cats and produced megacoIon. KIeinschmidtI was abIe to verify this. From these factors it is apparent that much evidence existed to indicate that there was some neurogenie factor which was the cause of this disease. On this basis there evoIved the idea of Iumbar
A
American
Megacolon
18
Congenital
Aganglionic
Megacolon
able to report consistently successful resuits as well. In March, 1951, Hiatt,‘” who had been working aIong Iines simiIar to Swenson, published a paper on the pathologic physiology of congenital megacolon, and clearly expounded the disturbance which was present. He folIowed this at the same time with a paper” describing his modification of the procedure described b3 Swenson and Bill, and reported its use in twenty-three patients.
sympathectomy as a specific procedure for this disease. WhiIe beneficial resuIts were claimed to derive from this procedure, many faiIures were reported as well. The disease was stil1 uncomprehended. With the mistaken idea of the dilated portion of the boweI as the seat of pathology, resections of this segment of the bowel were undertaken. Again the resuIts of this were equivocal. Spasm of the sphincter ani was also presumed as a possibIe cause of the disabiIity and attempts were made to rectif.y the pathoIogy by eliminating this factor. AI1 these measures simpIy skirted the actual facts. There was a factor of neurogenic imbalance but the absence of the gangIion ceils of the myenteric pIexus was the precise cause of it. There was a factor of obstruction, not due to an organic cause, but the result of a physiologic obstruction. There was the factor of inspissated feces resulting from marked dehydration due to the increased length of intestine with greater water-absorbing surface lvhich, in turn, was secondary to the physioIogic obstruction. AI1 these factors fringed on the precise cause of this condition but none was quite accurate. In 1948 Swenson and BilIzO reported on a study, made in the previous two years, of twenty patients in whom a cIinica1 diagnosis of congenital megacolon had been made. By means of a specia1 technic they were abIe to demonstrate an area of spasm in the rectosigmoid or rectum. In the miIder cases among these twenty the treatment suggested by Law’6 had been successful. Colostomies performed proxima1 to the area of spasm in six patients had relieved them compIeteIy. In three of these recurrence of the disability ensued when closure of the colostomy was performed. They, therefore, believed that removal of the area of spasm in its entirety would reIieve the functional obstruction. To do this they devised an entirely new modification of the procedures described by MaunselI17 and Weir.23 The procedure was performed on dogs and gave such encouraging resuIts that the operation was performed on three patients with exceIIent results. By August, 1950, Swenson”’ was abIe to report on fifty-two cases with exceIIent results in fifty-one and an anticipated similar result in the fifty-second. (Too dista1 a resection had been performed, requiring a second operation with a more proxima1 resection.) Stephens et a1.,3 who had observed Swenson’s work, were
PATHOLOGIC
PHYSIOLOGY
Only a proper understanding of the anatomy and physiology will make cIear the pathologic disorders present in this disease. The nerve suppIy of the Iarge intestine consists of two plexuses: (I) Auerbach’s or the myenteric pIexus which Iies between the two muscular coats and, (2) Meissner’s pIexus which lies in the submucous Iayer. A peristaltic wave in the coIon is a muscuIar contraction passing from the proxima1 to the dista1 part in a continuous manner. This is mediated through the Auerbach pIexus with the ganglia serving as control stations for the nervous impuIse so that the latter is graded and orderly, reaching the musculature at onIy one point at a time and causing that point to contract; and then passing in a sequentia1, regulated manner to the next distal point, causing the latter to contract while the previously contracted point relaxes. In this manner a we11 regulated peristaItic wave passes aIong the entire colon, from the cecum to the rectum. The time occupied in the passage of this wave from the beginning to the terminus of the Iarge intestine is easily measurable by the eye. However, for all practical purposes the passage of an impulse along a nerve fiber may be considered instantaneous. If the nerve impulse were carried aIong a segment of the colon at this speed, it is readily apparent that such a segment wouId contract throughout its length simultaneously; there wouId be no graded impuIse and there wouId simply exist a spastic contracture aIong such a segment of bowel which, with the passage of the impulse, would reIax aIong its entire length simultaneously. It is apparent that no impetus exists to propel anything along the Iumen of such segment forward or, for that matter, backward, Anything within such segment of bowel will simply be squeezed out at either end, orac1 and caudad. The designation of this disease as congenital ‘9
AxeIrod diIatation of the Iatter occurs. If the bIockage is compIete, acute intestinal obstruction resuIts, in no manner different in resuIt to the proxima1 part of the bowe1 than if an acute voIvuIus were to occur in the narrowed segment. It is characteristic of these patients that they do have episodes of acute intestinal obstruction. This resuIts in the death of many of these infants at an early age. If the obstruction is not reIieved a11 the sequelae of acute intestina1 obstruction ensue up to death. Swenson and BiII noted that when proximal coIostomy was performed as a lifesaving measure in six of their cases, the symptoms of the disease compIeteIy disappeared. When the coIostomies were cIosed in three of these, prompt recurrence of the disease took place and fuI1 reIief was again provided onIy by re-estabIishment of the coIostomies. However, where compIete obstruction does not occur, a chronic obstruction resuIts and it is in this aspect of the disease that the characteristic picture of congenita1 aganglionic megacoIon appears. With the piIing up of the feces in the proxima1, norma portion, the Iatter becomes secondariIy diIated. As this feca1 accumuIation increases, the diIatation increases. As the dilatation continues the peristaltic waves become more forceful. As is true of any muscIe in the body, increased use resuhs in hypertrophy. The hypertrophy taking pIace here refers to both the circuIar and IongitudinaI muscIe. With the hypertrophy there is, of course, thickening of the waI1 of the bowe1 as we11 as Iengthening. In no other physioIogic manner can the diIated, Iengthened and hypertrophic bowel waI1 be expIained. This diIatation and Iengthening of the bowe1 provide a tremendousIy increased mucosa1 surface. As a resuIt of this, continued over a period of time, the proxima1, normal bowe1 becomes tremendousIy diIated with dehydrated feces, distending the abdomina1 waI1 unti1 the characteristic picture of the chiId with the tremendousIy diIated abdomen, the ffaring ribs, the spindly Iegs and the retarded deveIopment appears.
agangIionic megacoIon describes the disease preciseIy. It is congenita1 and agangIionic because of the congenita1 absence of the gangIia of the myenteric plexus in the part of the bowe1 dista1 to the diIated portion. The disease is, in fact, in the dista1 undiluted portion of the Iarge intestine rather than in the dilated portion which contributes the word megacoIon to the designation of the disease. The diIated portion of the bowel is, indeed, entireIy normal; its greatIy increased lumen, thickened waI1 and eIongation are entireIy physioIogic reactions of normal bowe1 to a chronic obstruction distal to it. In the narrowed segment distal to the diIated portion of the coIon, the gangIia of Auerbach’s pIexus are absent but nerve fibers are present. (The nerve fibers are non-myeIinated and Iie where Auerbach’s pIexus wouId normaIIy be situated. These non-myelinated nerve fibers are not present to any marked degree where the myenteric pIexus is present. The significance of these different fibers is unknown.23) The length of this segment is dependent directIy upon the Iength over which the gangha are missing. When the nerve impuIse from the proxima1, normal portion reaches this part of the bowe1, an intense spastic contraction takes place, the lumen of the bowe1 is greatly contracted, if not entirely eIiminated, the norma peristaItic wave reaching it is ended and with it the forward prop& sion of the boIus from the proximal, norma portion. In effect, an aImost complete obstruction is present at this junction of the normal with the agangIionic segment of the bowel. A vicious cycIe is now set up. As the feces from the normal bowel reaches the agangIionic portion, the momentum imparted to them by the peristaIsis propeIs some of them into the Iatter segment. As the forward movement behind continues, feces are packed into the first portion of the narrowed part of the bowe1. This part of the feca1 stream can move forward onIy by sufficient push from the propuIsive efforts of the bowe1 orad to it. MeanwhiIe, though the aganglionic part of the bowe1 is incapabIe of peristaItic function, its other functions are not impaired. Water absorption from the feces in it continues and as it does the feces become drier and harder. The pounding from behind continues. An impaction now results, providing a stronger bIock to the feces constantIy being propeIIed into the aganglionic portion. With the feces now backing up into the norma bowe1,
CLINICAL
COURSE
With the proper use of enemas and other non-operative means of care, these chiIdren are not as severeIy disabIed, but their disabiIity is nevertheIess marked. Hiatt has pointed out the danger of the enema itseIf when it is of the tap-water variety. He has shown that the danger of the tap-water Iavage 20
Congenital
AgangIionic
MegacoIon
borns in an acuteIy obstructed condition. There the need for differentiation wiI1 be onIS between congenita1 aganglionic megacoIon and organic obstruction, compIete or incomplete. With agangIionic megacolon a history will usuaIIy be obtained of some anal expulsron of feces folfowing birth. In those newborns with compIete organic obstruction there will never have been a bowef movement ancf the scout film of the abdomen wiI1 show the point of obstruction inasmuch as there will be no bowe1 gas noted distaf to that site. In those infants and chifdren older than the acute intestina1 obstruction maJ newborn, aIso occur. These patients will have a history pathognomonic of this disease. Certainly no IegaI statute exists stating that these chiIdren cannot develop an obstruction due to other causes. The differential diagnosis here shouIcf not cause serious diffIcuIty.
consists in the very rapid absorption of the hypotonic soIution through the greatIy increased absorbing surface combined with the increased hydrostatic pressure. The resuIt is water intoxication and these patients, not uncommonly, are found to be in extreme weakness and pallor with marked sweating folIowing this type of enema. He ascribed the death of one of these patients, occurring immediateIy after such enema, to this fact. This is another factor accounting for the small number of these patients reaching adulthood. To minimize the dangers of the enema in these patients he has recommended a sofution of 7 per cent geIatin which has an osmotic force equa1 to that of the circulation. As is not unusuaf when one congenitaf defect exists, others often accompany it. Swenson investigated twenty patients with agangfionic megacofon by means of cystograms. He found that nineteen of them showed hypotonicity of the bladder, the bladder capacity of these patients being far above average before any detrusor contractions occurred. This is an indication of a deficient pelvic parasympathetic nerve supply. This is a matter of importance, for in the course of freeing the rectum prior to its removal from the pelvis, as is done in the surgical treatment of this condition, injury to the parasympathetic nerve suppIy to the bladder may easily occur since their precise course is unknown and the nerve fibers are too small to he dissected out.
TECHNIC
Where the obstruction is complete, expforatory Iaparotomy will be required with the performance of the shortest possible procedure consistent with saving the patient’s fife. This wiff usuafly consist of a transverse loop colostomy definitely proximal to the contracted segment. It would usually be unwise to attempt to give an enema with the idea of loosening the impacted feces and thereby allow the gas behind to be expelled. The increased intracoIonic pressure may possibIy be enough to resuIt in a bfowout of the tightly distended cofon. It shouId be a matter of note that in any acute obstruction there is insuffcient time for the bowel muscuIature to become hypertrophic. Consequently the bowel w-all proximal to the obstruction is thinned. Bowel wall hypertrophy occurs only where there is adequate time for development as in chronic obstruction. When no emergency exists, the patient may be fufIy assayed and prepared for definitive surgery. While no dogmatic rufes about performance of this operation reIative to the size of the patient can he made, the child with this disease who is two years of age or older is a suitabIe candidate. In those younger, a transverse cofostomy weIf proximal to the narrowed, distal segment should be performed until the child’s size is sufficient to permit performance of the specific procedure. Those who are experienced in performing this procedure can
DIAGNOSIS
The diagnosis of congenita1 agangIionic megacofon is not too diffIcuIt. At or shortIy after birth obstinate constipation becomes apparent. By means of enemas aIone these chifdren are able to evacuate their boweIs. So long as enemas are diIigentfy given, these children will have bowel movements. Despite the administration of enemas the overwhefming majority of children will appear with a greatIy distended abdomen, although not necessariIy sick. Their parents will report that they eat well if they are given enemas with any degree of regularity. Some wifI definiteIy show physical and mental retardation; others wiIf not. There wiIf usually be a history of treatment by medical means over a Iong period of time, but this treatment wiI1 not have affected them any more than when they received enemas afone. Some of these patients may be seen as new21
AxeIrod certainIy execute it in smaIIer chiIdren. On the average, however, it is desirabIe to wait unti1 the patient’s size is such that the structures may be seen and dissected with greater accuracy. The operation consists of remova of the entire narrowed, distal segment, the transitional area and a variabIe part of the distal, diIated part of the colon. Beside the customary means used to prepare a patient in this age group for major abdominal surgery of an eIective nature, it is desirable to decompress the diIated bowel before going to surgery. By means of diet, enemas and intestina1 antiseptics this can be obtained, as we11 as providing a steriIe, colonic Iumen. It is aIso highIy desirabIe to intubate a vein, usuaIIy the greater saphenous at the ankIe, with a poIyethyIene catheter prior to the start of surgery. Once the patient is properIy prepared the procedure used is the operation devised by Swenson with Hiatt’s modification. Entrance to the peritonea1 cavity is provided by either a Iower Ieft, vertical rectus incision or an obIique one starting in the region of the right pubic spine and extending in an obIique Iine across the midline, cutting the Ieft rectus muscIe and extending to the Ieft flank. The finding of the junction of the diIated, hypertrophied segment with the dista1, narrowed segment (the transitional area) is the point at which bearings are taken. The superior hemorrhoida vessels are found and cIamped along the transitiona segment. The transitiona portion and a11 the Iarge bowe1 dista1 to it are now dissected free from a11 the structures attached to them. It must be constantIy kept in mind that in this dissection it is the absence of the gangIion ce1Is lying between the Iongitudina1 and circuIar muscuIatures that is the cause of this disease. Therefore, the removal of the tube of bowe1 where this deficiency exists without the incIusion of any attached structures is the desired dissection. Furthermore, since so many of these patients have poor innervation of the pelvic viscera, disturbance of any of the structures other than the bowe1 itseIf is highIy undesirabIe. The narrowed segment is, therefore, stripped down very cIoseIy aIong its outer coat to the insertion of the Ievator ani into the anus. Because the normal but diIated and hypertrophied portion has been propeIIing feces into the proxima1 part of the abnorma1 segment, some diIatation of a variabIe Iength of the proximal part of the
Iatter is present. For this reason it is necessary to resect a variabIe portion of the diIated segment orad to the transitiona area. More preciseIy, frozen sections of the muscuIaris taken at intervals aIong this part of the bowel wiI1 revea1 where the ganglia are present in norma number. This may, however, resuIt in undue proIongation of an aIready lengthy procedure. By pathoIogic examination 12 cm.21 and 20 crn.lO have been suggested as a sufIicientIy safe orad point of resection. In Iine with those suggestions the Iength of bowel resected will vary with the distance between the transitiona area and the anus. It may require anastomosing the spIenic flexure or even the transverse colon to the anus. The bIood suppIy to the unresected portion of the bowe1 must, of course, remain unimpaired. It may be found that the only thing preventing approximation of the point of resection of the bowe1 wall to the anus without tension is the inabihty of the blood vesseIs to stretch that distance. Where this is so, the mesentery must be cut cIose to its origin and the bIood vessels hindering this movement must be Iigated close to their origin, usuaIIy the inferior mesenteric vesseIs. With that done the arcades and the margina artery are preserved, permitting the vascuIar suppIy to remain intact and at the same time aIlowing the necessary mobiIity. This having been accomplished, attention IS now directed to the perineum. The anus is diIated, an AlIis clamp inserted from outside the body into the rectum and directed by a hand in the abdomen to as high a position as possible, the bowel is cIamped and is drawn down to the anus and out, intussuscepting the bowe1 through the anus. It is drawn out far enough so that the point which has been eIected for anastomosis to the anus is brought into proper position for the performance of the anastomosis on the perineum. A two-layer anastomosis may be done here, cutting away at the same time the diseased howeI. At the completion of this step the newly formed anastomosis is pushed back into the body through the anus. A drain may or may not be inserted into the presacraIretro-anastomotic area through a stab incision between the tip of the coccyx and the posterior margin of the ana ring. CASE REPORTS CASE I. D. L., a six year oId boy, was seen with the compIaints of abdomina1 distention, 22
CongenitaI
AgangIionic
Megacolon
extends completely across the abdomen to the Ieft side. No attempt was made to fill the proximal colon. Wide mucosal foIds are visible in this ditated intestine.” The patient was prepared for surgery b?means of daily enemas of physiologic saline until cIear return, administration of oral sulfasuxidine and miIk of magnesia and cascara cathartics. Orally the patient received daily multivitamin capsuIes and a Iow residue, high protein, high calorie diet. When the distention of the abdomen had been reduced to an almost norma appearance and the enema return showed soft stool without odor, the patient was subjected to the operation. His postoperative course was uneventful. He had a spontaneous bowe1 movement on the third postoperative day. OraI feedings were started that same day. Sutures were not removed until the tenth postoperative day and the patient was discharged on the thirteenth postoperative day. There has been no diarrhea and the patient has one to two formed, soft boweI movements daily. In the eighteen months since the operation the patient has shown a much more rapid rate of growth than previousIy. and he has put on weight in accordance with it. The treatment of the Perthes disease of the left hip was started four months after the operation under orthopedic care and is continuing. CASE II. C. hI., a two and a half year old girl, had had a few bowel movements immediately after birth but had aIso become distended abdominally within one month after birth. Treatment bv the various medical procedures was carried out in conjunction with enemas; and when the medication was haIted and the enemas aIone used, the child did as weII. AIthough enemas had been given daiIy there had always been present a marked degree of abdomina1 distention. No history of urinary dif&uIty was obtained. Physical examination revealed a two and one-half year old girl who did not appear to be behind the developmental pattern for her age. She appeared to be fairIy active. Her large protuberant abdomen, which in its state was symmetric, bulged up to the costal margins, causing the ribs to Aare. Her hemic component appeared to be normal. The abdomen was firm but not hard, not tender and no masses couId be felt. Neither liver nor spIeen was paIpabIe. No other abnormalities were noted. A barium enema reveaIed the findings
frequent episodes of iIIness and obstipation. Since the second month of his birth the parents could bareIy recaI1 when he had had a bowe1 movement spontaneously. Enemas and cathartics were used regularly to obtain bowe1 movements. There had been episodes when he was particuIarIy ill and when he wouId fInaIIy have a bowel movement after several attempts. At such times the excreta consisted of very firm, scybaIous balIs. Between such episodes the feces were very firm as weJl. The child went to school and was an average student. His mixture with his classmates was greatly limited because of his inability to keep up with their activity. He had been under the care of doctors who had treated him medicaIIy with very IittIe improvement above that obtained by enemas and cathartics aIone. Physica examination reveaIed a paIe, white boy who appeared his age of six but who definitely lacked the vigor seen in a we11 child of that age. His abdomen was the most prominent part of his anatomy because of its marked distention. It was symmetricaIIy distended and bulged the ribs outward, causing them to flare. Several Iarge veins coursed over the abdomen. On palpation the abd omen was firm but not hard. No tenderness was present unIess heavy and deep paIpation were done. No masses could be feIt. There was no rebound tenderness. The extremities were somewhat thinner in appearance than a norma chiId of the same age. He was paIe and the skin, as a resuIt of this and the absence of bIemishes, had an “ethereal” appearance to it. The only other abnormality of note was a Iimp in the Ieft lower extremity due to Perthes disease of the ieft hip. Barium enema reveaIed the typica findings in this disease. The radioIogist’s report foIIows: “Examination of the colon, by barium enema, demonstrates that the rectum and dista1 sigmoid did not distend to the degree that is ordinariIy seen in the course of a barium enema, but these structures were normaI in contour. The proxima1 rectum extends directIy supcriorIy in the midIine to a point anterior to the interspace between L 4 and 3 and then turns anteriorly and to the right. At the point of turn the lumen fairly abruptIy widens to about 3 times the caIiber of the sigmoid distal to this point. The sigmoid is very Iong and this dilated portion extends to the right to overIie the IateraI portion of the right ileum and then 23
AxeIrod typica of the disease. The radio1ogistY.s report foIIows: “A preIiminary fiIm of the abdomen shows severa markedIy diIated gas-fiIIed Ioops of intestine, undoubtedIy coIon, in the right abdomen and mid-line and severa Ioops of smaI1 intestine, not diIated, and containing onIy a IittIe gas, in the Ieft abdomen. Examination of the coIon by barium enema showed that the rectum and dista1 sigmoid were essentiaIIy normal in caIiber, but beginning at about the mid-sigmoid, there was very marked diIatation of the proxima1 coIon. The sigmoid extends upward and to the right so that the point of junction of non-diIated and diIated sigmoid is in the right upper quadrant. The post-evacuation fiIm shows that the dista1 sigmoid and rectum has expeIIed most of the barium from that area, but the proxima1 sigmoid and the coIon retained the barium. This examination shows evidence of megacoIon. The narrow segment of intestine begins at about the midsigmoid and involves the dista1 sigmoid and rectum.” After four days of preparation (morning and evening saIine enemas, miIk of magnesia with cascara daiIy, and suIfasuxidine combined with a low residue, high protein, high caIorie diet) the patient was taken to the operating room. The Iarge bowe1, extending orad from a point z cm. above the pectinate line (incIuding the entire narrowed segment) and 20 cm. cephaIad to the transitiona area, was dissected free, intussuscepted through the anus and resected as the anocoIonic anastomosis was performed on the perineum. The postoperative course was uneventfu1. On the second postoperative day the patient started to have bowe1 movements of gelatinous consistency and was aIIowed up and about freeIy. By the fifth postoperative day she was eating a reguIar diet, her bowel movements were onIy two per day and she was discharged. Since that time (sixteen months ago) she has had no diffIcuIty, her abdomen has receded to norma size and she has been norma in every respect for a chiId her age.
ease, have definitely estabIished the cause of this disease entity and resulted in a curative procedure. An attempt has been made to cIarify the steps in the deveIopment of the cIinica1 entity Iong noted in this disease. Two cases have been presented to iIIustrate the exceIIent resuIts that can be achieved by operative correction of this congenita1 anomaIy. REFERENCES I. ADAMSON, W. A. D. and AIRD, I. MegacoIon:
2.
3.
A. 7
5.
6.
7. 8.
9. IO.
evidence in favor of a neurogenic origin. Brit. J. Surg., 20: 220-233, 1932. BANCROFT, F. W. and WADE, P. A. SurgicaI Treatment of the Abdomen. PhiIadeIphia, 1947, J. B. Lippincott Co. BODIAN, M., STEPHENS, F. D. and WARD, B. C. H. Hirschsprung’s disease and idiopathic megaCOIOn.LQnCet, I : 6-I I, 1949. CAMERON. J. A. M. OesoDhaeectasia in chiId. Arch. Dis. Childhood, 2: 358-360, 1927. CAMERON, J. A. M. On aetiotogy of Hirschsprung’s disease. Arch. Dis. Childhood, 3: 210-211, 1928. DALLA VALLE, A. Richerche IstoIogiche su di un Cas di MegacoIon Congenito. Pediatria, 28: 74*752, 1920. DALLA VALLE, A. MegacoIon FamiIia. Pediatria, 32: 569-599. 1924. ETZEL, E. La Dilatation de1 Esofago Frente a Ias Lesiones de1 PIexo de Auerbach en eI Megaesofago. Bol. y trab., Sot. de cir. de Buenos Aires, 21: 131-148, 1937. HAWKINS, H. P. Remarks on idiopathic diIatation of the colon. Brit. hf. J., I: 477-483, 1907. HIA~, R. B. The pathologic physioIogy of congenitaI megacoIon. Ann. Surg., 133: 313-320, 1
-
195’. I I. HIATT, R. B. The surgica1 treatment of congenitaI megacoIon. Ann. Surg., 133: 321-329, 1951. 12. ISHIKAWA. N. ExperimenteIIe Untersuchungen iiber die Djckdarminnervation, Insbesondere des CoIon Descendens et Sigmoideum. Mitt. a. d. med. Fak. d. k. Univ. Kyusbu Fukuoka, 7: 295338, 1923. 13. ISHIKAWA, N. ExperimenteIIe und KIinische Untersuchungen uber die Pathogenese und das Wesen des MegakoIons. Mitt a. d. med. Fak. d. k. Univ. Kyusbu Fukuoka, 7: 339-400, 1923. 14. KLEINSCHMIDT, cited by BOCKUS, H. L. GastroEnteroIogy, vol. 2, p. 404, PhiIadeIphia, 1943. W. B. Saunders Co. 15. LADD, W. E. and GROSS, R. E. AbdominaI Surgery of Infancy and ChiIdhood. PhiIadeIphia, 1947. W. B. Saunders Co. 16. LAW, J. L. Treatment of megacoIon with acetyI beta methy choIine bromide. Am. J. Dis. Child., 60: 262, 1940. 17. MAUNSEL, H. W. A new method of excising the two upper portions of the rectum and the lower segment of the sigmoid ffexure of the coIon. Lancet, 2: 473, 1892.
CONCLUSIONS
Experimenta and pathoIogic observations over a Iong period of time, cuIminating in an operative procedure devised by Swenson to verify concIusions reached concerning the pathologic physioIogy of Hirschsprung’s dis24
CongenitaI
Aganglionic
I8. PEKROT,A. md DAIXOX,L. Obstruction IntestinaIe de Cause Rare, Chez un Nourison. Ann. d’anat. &J., 12: 157-165, 1935. 19. ROBERTSON, H. E. and KEKNOHAN,J. W. Myenteric megacolon. Proc. Staff pJtXUS in congenital *Meet., izIayo Clin., 13: 123-125, 1938. 20. SL\ENSOY,0. and BILL, A. H., JR. Resection of rectum and rectosigmoid with preservation of the sphincter for benign spastic lesions producing megacolon: experimenta study. Surgery, 24: 2 I 2-220, 1948.
Megacolon
SWENSON,0. A new surgica1 treatment for 1 lirschsprung’s disease. Surgery, 28: 371-383, 1950. 22. TITTLE, K. Ueber eine Augeborene Missbildung des Dickdarmes. Wien. klin. Wchnschr., r4: 903-907, ,901. 23. WEIK, R. F. An improved method of treating high-seated cancer of the rectum. J. A. ,2/1. A., 37: 801, 19or. 24. WHITEHOUSE,F. R. and KERNOHAY,.J. W. hlycnteric plexus in congenital megacolon; study of I I cases.Arch. IN. Med., 82: 75--1 I I, 1948. 2 I.
KINSELL has had a very favorabIe experience using hormone therapy in addition to antibiotics in acute inflammatory surgica1 cases. Of course the hormones he used (ACTH or cortisone) are known to increase the breakdown of protein tissue and the loss of chlorides and potassium. Therefore, these patients need increased quantities of proteins intravenously and potassium chloride. These hormones also cause an increase in body retention of sodium, so the use of intravenous sodium shouId be greatly limited. KinseII reaIizes there are certain contraindications to the use of these hormones, especiaIIy in patients with gastrointestiona1 uIceration, insuffrcient renaI function, congestive heart disease or tuberculosis. ManJ patients in a debiIitated condition who need non-emergency surgery wiI1 make a better convaIescence if treated by these hormones for two days prior to surgery and three days afterward. (Richard A. Leonardo, M.D.)