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Early and late complications of the Swenson pull-through operation for Hirschsprung's disease
Early and Late Complications of the Swenson Pull-Through Operation for Hirschsprung’s Disease* California, AND ORLAND Fresno, California
EDWIN G. CLAUSEN, M.D., O&and,
From tbe Department of Surgery, tbe Children’s Hospital of tbe East Bay, Oakland, California and tbe Valley Children’s Hospital, Fresno, Calijornia.
IFTEEN years ago Swenson [I] introduced use of abdominaLperineal resection of the rectum for the treatment of Hirschsprung’s disease. This procedure has been accepted by most American surgeons. However, because of postoperative complications, some European and more recentIy American sursurgeons, its superiority over geons, have questioned other methods. A review of the comphcations of reported cases, as we11 as those seen by us at the Children’s HospitaI of the East Bay, in OakIand, the VaIIey ChiIdren’s HospitaI, Fresno, and the University of CaIifornia HospitaI, San Francisco, will be presented. The patients who were operated upon shortIy after Swenson introduced the puII-through procedure were mostIy those who had survived the earIy years of the disease. As a resuIt, a one stage operative procedure was done. Now the majority of cases are recognized during infancy. Since the high mortality rate is usuaIIy a result of obstruction and enterocoIitis a coIostomy is generaIIy advisabIe as soon as the diagnosis is made. Some of the patients with milder symptoms can be treated with diet, laxatives and enemas. Most surgeons agree that the puII-through procedure is too drastic for chiIdren under six months of age, and advise deferring the operation unti1 the chiId is tweIve to twenty months oId. As a resuIt, any of those patients who come to a definitive operation will have aIready had a proxima1 coIostomy.
F the
* Presented American
A coIostomy must be made in the part of the coIon which contains parasympathetic gangIion. Since in some newborn infants the Iine of demarcation of agangIionic bowe1 is ill defined, and since even in those in whom the demarcation is apparent the aganghonic area may extend proximaIIy in varying degrees, a frozen section must aIways be done to determine unequivocahy the area of normaI bowe1. Opinions differ as to the most desirabIe Iocation for coIostomy. In a transverse coIostomy a Ioop type in continuity is adequate. The Ioop coIostomy is not diffrcuIt to manage because of the fluidity of the stooIs; it is a simpIe procedure, and affords protection for the subsequent puII-through operation. On the other hand, this type of coIostomy does require an extra operative procedure to cIose it; occasionaIIy, it wiI1 interfere with mobiIization of the coIon to obtain the Iength for the puIIProIapse is 0ccasionaIIy through procedure. bothersome in transverse coIostomies. A more dista1 coIostomy affords more colon for absorption and can be eIiminated at the time of the puII-through procedure. However, because of its size and thickness, a Ioop coIostomy may present technica probIems. An end coIostomy would be more diffrcuIt to perform. At the time of the puII-through operation, contamination may occur if the coIostomy has to be taken down. RegardIess of the type of coIostomy, great care must be taken to prevent proIapse of smaI1 bowe1 around the coIostomy. This can be done by careful approximation of the peritoneum about the coIon. Deaths foIIowing the operation occur in the
at the annua1 meeting of the Pacific Coast SurgicaI Association, 3-6 1963.
Journal
of Surgery,
Volume
ro6,
August
rg6j
372
G. DAVIES, JR., M.D.,
PaIm Springs, California,
February,
PuII-Through CAUSES
OF DEATH
Operation
FOLLOWING
SWENSON
for Hirschsprung’s
TABLE I PULL-THROUGH
Disease
PROCEDURE
FOR HIRSCHSPRUNG’S
DISEASE
Totai Operation
Authors
T
Deaths
No.
T
EarIy Deaths
.-
Late Deaths
No.
‘er cent
No.
Cause
Three
Cardiac arrest, peritonitis, tracheobronchitis Tension pneumothorax from ruptured congenital
Cause
-_ Ehrenpreis
[9]
HaIIenback
Hays
[z].
Hiatt [6].
Sixty-nine
Four
5.8
d-j....
Thirty-four
Four
10.7
tOne
......
Nineteen
Six
31.5
Four
Sixty-seven
Five
7.4
,One
.........
Kostia [I I].
.......
Twenty-eight
Six
Langer [z7]. Swenson [r].
....... ......
Twenty-two Two hundred
Two Thirteen
9.1 6.5
Two Six
Nine
5.9
Six
Three
8.8
Thret
WyIIie [7] .........
. One hundred fifty-two
Authors
...........
Thirty-four
-
Three
21.4
cyst Shock, necrosis proximaI segment [9] PuImonary aspiration Not mentioned
One
Three
Two
Four Three
Peritonitis Peritonitis [8], small bowe1 obstruction, septicemia, biIatera1 adrena hemorrhage, gastroenteritis Shock [9], bowe1 necrosis [S], peritonitis EnterocoIitis, shock, sepsis
None Seven
Three
Puimonary aspiration after coIostomy closure EnterocoIitis
Postoperative obstruction, stricture of anastomosis EnterocoIitis Intestinal obstruction, encephalitis, died after second puII-through procedure
.. EnterocoIitis
IntestinaI obstruction [8], coIitis
None
...
-
immediate postoperative period and after the patient has Ieft the hospita1. The death rate has been Iowered to an acceptabIe IeveI now that experience has proved the operation to be inadvisabIe in infants, technica probIems have been eradicated and close attention has been paid postoperatively to the fluid and eIectroIyte balance, and to the possibility of enterocoIitis. Many of the earIy deaths were due to sepsis from a Ieak in the anastomosis or from septicemia. (TabIe I.) Many smaI1 infants died of shock or compIications reIative to any major operation. In Swenson’s series, four of the six patients who died were infants. Hays and Norris [2] reported three deaths in four infants operated upon in the first six months of Iife. Late deaths were usuaIIy due to rapid, fuIminating enterocoIitis, which caused death sometimes within twenty-four hours unIess
vigorousIy
treated.
reported eight who
patients, have
are IikeIy
BiII and
enterocoIitis eight
Chapman
[3] have
in twenty-four of whom
had
enterocoIitis
to have
it after
of forty-
died.
before operation
Patients operation
as weI1. To
must aIert the famiIy to the seriousness of abdomina1 distention, obstipation or diarrhea. Deaths aIso occurred after stranguIating bowe1 obstruction which was neglected because of faiIure to differentiate between simpIe functiona distention and vomiting, and a true stranguIating obstruction. ExceIIent descriptions of the technica aspects of this operation have been presented eIsewhere. We wish only to emphasize the saIient features which we beIieve have contributed to some of the major compIications. Leakage of the anastomotic site occurred in a11 reported series in which there were compIicaprevent
373
this,
one
CIausen and Davies
FIG. I. IIlustrates short left colic artery, ascending and descending branches with poor or absent marginal artery. Figure on right indicates preferred area of division of arteries to obtain Iength of mesentery.* * (Reproduced
by permission of C. N. Morgan
and J. D. Grifliths. Surg. Gynec. and Obst., 108: 641, 1959.)
branch forming the major margina artery, and consequentIy a very smaI1 artery may be near the coIon. (Fig. I.) ShouId either or both of these branches be divided the dista1 coIon may become ischemic. Genera1 carefu1 dissection must be done with specia1 reference to avoidance of injury to the margina artery, if dista1 bowe1 ischemia is to be prevented. ShouId the bowe1 be resected to the proxima1 descending coIon or transverse coIon, it wiI1 be necessary to divide the middIe colic artery and then bring the coIon down the right side of the abdomen with the hinge on the right coIic or iIeocoIic artery. Although as much coIon as possibIe should be saved, resection to more norma bowe1 is desirable and may prevent the pIeating which might resuIt from anastomosing dilated proxima1 bowe1 to smaIIer dista1 rectum. No suture line Ieakage of any consequence has been reported in patients who have had a proxima1 coIostomy. AIthough some cases of suture Iine leakage were treated successfuIIy by conservative means, a proxima1 coIostomy shouId be done as soon as the leakage is ascertained. This frequenty may not onIy be Iife saving, but may prevent Iong periods of hospitaIization, intractabIe fistuIa and stenosis. We do not ordinariIy drain the presacra1 space.
tions. The proxima1 bowe1 must not be on tension; this frequentIy necessitates mobiIization of the spIenic ffexure for even a Iesion in the Iower sigmoid area. OrdinariIy about 50 per cent more bowe1 is needed for the tota pelvic pulLthrough procedure, which is measured from the promontory of the sacrum to the symphysis pubis. Since it is usuaIIy mesentery that prevents descent into the peIvis, the superior hemorrhoidal artery must aIways be sacrificed as we11 as the proxima1 sigmoid branches. If IocaI ischemia is to be avoided, the division of these vesseIs shouId be done near their base, thereby preserving as many arcades as possibIe. OccasionaIIy the Ieft coIic artery must aIso be divided, and the bIood supply to the coIon is then dependent on the margina artery associated with the middIe [4] have coIic artery. Morgan and Griffiths pointed out numerous anomalies of arteries to the coIon. In IOO cases studied no middIe or right coIic artery was present in two instances. The middIe coIic artery was absent in 22 per cent of the cases and the Ieft colic artery was absent in 6 per cent. In one instance there was no connection of the marginal artery between the middIe and Ieft coIic arteries. The Ieft coIic artery may be short with a proxima1 and dista1 374
PuII-Through
Operation for Hirschsprung’s TABLE
EARLY
COMPLICATIONS
FOLLOWING
II
SWENSON
HIRSCHPRUNG’S Anastomotic
Disease
PULL-THROUGH
PROCEDURE
Leak
Stricture
Author
Incomplete
No.
Ehrenpreis
[9].
...
Five
I-IaIIenback [,161..
. Nine
IIays
. Three
[2].
Kostia [II].
.....
Six
I-anger [r7].
......
Six
Swenson [I].
iVylIie [7].
Authors.
FOR
DISEASE
.......
.........
.
Five
None
Four
No.
Result
One died, no coIostomy, four responded to treatment coIostomy
seven
Five heaIed without cofour healed Iostomy, with coIostomy Died
Not mentionec
Five recovered, one died foIIowing operation for uretheraI IistuIa One died, four cIosed spontaneousIy, one required second operation
Six
Five
One necrosis of proxima1 Nine bowe1, one fistuIa deveIoped. two gross Ieak, one peIvic abscess, a11 responded to proxima1 coIostomy Seven . .
One permanent coIostomy, three responded to treatment, coIostomy
Four
ResuIts
411responded to dilatation
. Four responded to diIatation, one died Responded to diIatation
Five
Resection
Six patients, three reoperated upon, one constipated, one diarrhea, one norma Not mentioned
Two cases, corrected DuhameI operation
by
Two responded to diIatation, three under treatment by diIatation Eight responded to diIatation, one needed reoperation
One patient, reoperated upon and cured
Six responded to diIatation, one needed reoperation Responded to dilatation
Three cases, two ma1 bowe1, one rectum
None
proxidistaI
One case, reresection now we11
and
IncompIete resection of agangIionic bowe1 has been reported. (TabIe II.) In some instances this occurred whiIe treating earIy cases when the proxima1 bow1 was not studied by frozen section to determine the site of norma gangIion. The Iine of demarcation between the agangIionic and gangIionic areas cannot be de‘termined by gross examination; frozen section must aIways determine the presence of gangIia. However, recurrent constipation is not uncommon and wiI1 be discussed Iater. After the abdomina1 portion of the operation is compIeted a bimanua1 abdomina1 perinea1 determination of the adequacy of the peIvic dissection shouId be done. This wiII aIIow unrestricted eversion of the Iower segment and assures adequate dista1 bowe1 remova1. There must be
However, if there seems to be excessive and uncontroIIabIe oozing, drainage through the perineum wouId be advisabIe. Many surgeons have reported strictures at the site of anastomosis. (TabIe II.) Strictures are more common when a coIostomy is present than when the bowe1 is functioning. Repeated recta1 examination must be done even in the uncompIicated cases. However, when a major disruption occurs at the suture Iine, strictures wiI1 occur and must be treated vigorousIy by The second puII-through digita diIatation. procedure can be accomplished even if this stricture cannot be diIated so that a permanent coIostomy shouId not be made. DigitaI diIatations wiI1 cure most strictures, but it may be necessary to repeat them for many months. 375
CIausen
and
TABLE LATE
COMPLICATIONS
-
Author
FOLLOWING
SWENSON
Davies III
PULL-THROUGH
FOR HIRSCHSPRUNG’S
Diarrhea
Constipation
Incontinence
DISEASE
No. of IntestinaI Obstruction
-. Ehrenpreis
[9].
. Six cases, one treated by Four cases treated sphinctorotomy, cases late onset
HaIIenback
[I 61.
Three cases, relieved by enemas
Kostia [II]. Langer [r7].
Swenson [I]. WylIie [7]
Authors.
.
five
.
..
Three cases, treated by Iaxatives and enemas Three cases, treated by Iaxative and occasional enemas One case, treated by occasional enema Thirty-four cases, twothirds under three yr. of age, nine started after one yr., fourteen need two yr. or more of treatment Six cases, relieved with occasiona enema or cathartic
by
sphinctorotomy
Twenty-six cases, four we11 later, twenty-two cases persistent (eight bad) Two cases, mild
Four cases, two well after two yr., two remain problem Not mentioned Two cases
One
Not mentioned
Not mentioned
Two
Four cases, temporary
None
None
Not mentioned
Forty-one
Nine cases responded to repeated enemas, two required external sphincterotomy
Occasional case relieved with correction of diarrhea or constipation
Four
cases
Seven, two died
Three, reheved by operation
sufficient mucous membrane for proper sensation, and yet an excessive amount may resuIt in recurrent symptoms. The resuIts foIIowing resection are dramatic and most patients are we11 after a varying period of adjustment. Lee [5] has stressed the importance of carefu1 and proIonged foIIowup studies and Swenson [I] has emphasized the importance of performing the operation before the bowe1 training period. Hiatt [6] has discussed in detai1 the factors invoIved in deveIoping norma bowe1 habits. In most instances after operation, bowe1 habits are reIativeIy norma unti1 the training period. So much emphasis has been pIaced on having a bowe1 movement prior to operation that the chiId may rebel against anything to do with bowe1 training. On the other hand, the sudden Iack of attention may induce a functiona constipation. Many patients with a coIostomy have never had the sensation of a spontaneous bowe1 movement. The whoIe process is new and these patients must have bowel training by a we11 instructed, patient mother. Constipation often deveIops postoperatively, and varies from miId irreguIarity to recurrent
impactions. The diffIcuIty in evaIuating this symptom is indicated by its variabiIity in the reported cases. (TabIe III.) It is common for the patient to have normal daiIy bowel movements immediateIy postoperativeIy and for severa months, and not to experience constipation unti1 much later. WyIIie [7] reports nine patients in whom constipation deveIoped after one year of norma bowe1 movements. The acute obstructions which are associated with fever, toxicity and abdomina1 distention should be corrected by coIonic irrigations. The more chronic type of constipation wiI1 be reIieved by coIonic irrigations one to two times per week. It may be necessary to continue the irrigations for one to two years. With proper bowe1 training, diet, and judicious use of catharsis most of the more obstinate cases can be kept under controI unti1 the chiId is oId enough to be more cooperative. The physician and mother must work in cIose cooperation. If an adequate stoo1 is not produced in forty-eight hours, a miId Iaxative or enema shouId be given and impactions must be prevented. Postoperative diarrhea may be a more 376
PuII-Through
Operation
bothersome and more serious symptom than constipation. Again diarrhea varies in severity and its incidence is diffIcuIt to assess accurateIy. (TabIe III.) StooI cuItures are not abnormal, and there is no specific change in the bowe1 waI1 as a ruIe. However, if untreated or negIected for any Iength of time, the coIon above the resected area appears on proctoIogic examination to present nonspecific ulcerative coIitis, and perforation may even occur. The cause of diarrhea is postuIated to be simiIar t.o that causing bowe1 obstruction. The point of obstruction is a spastic, aganglionic interna sphincter, resuIting in proxima1 coIonic diIat.ation with feca1 materia1 and fluid. Pressure is buiIt up and is suddenIy reIeased when the sphincter reIaxes. Vomiting, dehydration, fever and toxicity depend on the degree of obstruction. Death has been reported within a twentyfour hour period in patients with no abnormaIity of the bowe1 waI1 or bowe1 mucosa. ConsequentIy, the method of treatment is to reIease the ana obstruction by digita examirecta1 tubes and enemas. UsuaIIy nation, coIonic irrigations twice daiIy for three or four days wiI1 be effective. In more persistent, severe cases a tota externa1 sphincterotomy may be necessary. This operation does not result in incontinence. Incontinence may be associated with either constipation or diarrhea. Variation of reported cases (TabIe III) must depend on how criticaIIy t.he symptom is reviewed. UsuaIIy as the chiId gets aider, the frequency of soiling decreases. Loss of sphincter control is not a major probIem. With the correction of diarrhea, constipation and feca1 impaction, incontinence wiI1 cease accordingly. The incidence of intestina1 obstruction after a puII-through procedure may be higher than that foIIowing a simple Iaparotomy. An extensive dissection and mobilization of the coIon necessitates carefu1 reperitoneaIization, especially in the peIvis. However, if the proxima1 bowel is diIated, it may fiI1 the peIvis compIeteIy, making peritoneaIization virtuaIIy unnecessary. NevertheIess, severa deaths in the Iate postoperative period have been the result of unrecognized stranguIating obstruction. Since recurrent distention, vomiting and obstipation occur as a result of functiona a true stranguIation may be obstruction, overlooked. Impotence in maIe patients has been feared
for Hirschsprung’s
Disease
as one of the complications IikeIy to occur in the puII-through operation. However, Swenson [I] has operated upon tweIve patients who have compIete, norma sexua1 activity; apparentIy, this compIication is rare: A peIvic dissection must be done with care and precision. After division of the superior hemorrhoida vesseIs, a definite cIeavage pIane is readiIy entered, aIIowing the hypogastric pIexus to remain undisturbed. This pIane shouId then be carried posteriorly deep into the peIvis to the tip of the coccyx. AnteriorIy, the pIane between the seminal vesicles, prostate and urethra is narrow but we11 defined. This anterior IateraI dissection must be cIose to the bowe1 to prevent disturbance to the nerves entering the genitourinary tract, more anteriorIy. Since the peIvis is shaIIow, dissection is considerabIy easier than in the aduIt and may be performed under direct vision much of the time. Impotence has not been a probIem in the patients oId enough to be evaIuated in this series. Because of the compIications described previousIy, DuhameI [8] of Paris has advised a more simpIe procedure. He cIaims this procedure can be done in very young infants and with a very Iow mortaIity rate. In this procedure the bowel is divided at the rectosigmoid and the dista1 end is cIosed. After resection of the agangIionic area, a presacra1 dissection is performed and the proxima1 bowe1 is brought into this space, Ieaving the rectum essentiaIIy undisturbed anteriorIy and IateraIIy. By making a contmuous mucous membrane incision posteriorIy, the externa1 sphincter is separated from the rectum and the proxima1 bowe1 is brought down through this area, opened and sutured posteriorIy. The anterior aspect is opened into the rectum by the use of Kocher cIamps pIaced in a v-shaped manner. The main advantage of this procedure, according to DuhameI, is that it can be used in very smaI1 infants with reIativeIy IittIe shock. The norma proxima1 bowel then comes in contact with the anus and the norma externa sphincter. This section of the rectum is undisturbed and consequentIy there is no danger of disturbing sexua1 or urinary bIadder function. This procedure has been regarded with interest by some European surgeons [g-rz] and more recentIy by American surgeons [x3, 141. However, the incidence of incontinence, as reported by DuhameI, seems to be greater 377
CIausen and Davies than that associated with the Swenson procedure. More recentIy Martin and AItemeir [ 131 and Grob [IO] have reported a procedure which is similar to that of DuhameI. They aIso Ieave the rectum in pIace, but bring the norma bowe1 down to the IeveI of the interna sphincter and then perforate the rectum at this point, puIIing the proxima1 bower into the dista1 rectum, just proximal to the interna sphincter. Grob then sutures the proxima1 bowe1 to the rectal mucosa. Martin, however, uses a spur crushing cIamp which opens the entire dista1 rectum and Iower coIon into one Iarge cavity, haIf of which wiI1 have norma the foIIow-up periods in gangIia. However, these patients were so short that it cannot be ascertained how we11 this wiI1 function. We know that after an anterior resection of the Hartman type, patients do very we11 for a Iong period; however, eventuaIIy the proxima1 bowe1 begins to diIate, and in many instances and a further procedure is decompensate, necessary. State [15] has recentIy reviewed eighteen cases in which he has utiIized resection of the rectosigmoid, descending coIon and part of the transverse colon with anastomosis of the transverse coIon to the rectum. Resection of the proxima1 dilated bowe1 has been done many times before, with generaIIy poor results. However, State emphasizes the need to resect the entire descending coIon, the rectosigmoid, and part of the transverse coIon. This segmenta area is determined by the peristaItic activity at the time of the barium enema. His resuIts have been exceIIent, although coIon dilation has occurred in the tweIve cases that he restudied by roentgenograms, ten or more years Iater. However, the area of diIation appears to be emptying weI1. It is conceivabIe, however, that in time sufficient diIations couId occur resuIting in functiona disability. In spite of the serious nature of the Swenson puII-through operation it wouId appear to be the most physioIogic operation at this time. The procedure requires meticuIous attention to detail. If technica detaiIs are not faithfuIIy attended to, compIications of a serious nature may result. Postoperative care after a successfu1 primary operation must be continued for several months until the chiId has learned proper bowe1 habits. NevertheIess, the exact physioIogic defect is being questioned, and other operative procedures are being utiIized. 378
SUMMARY
A review of the compIications of Swenson’s puII-through operation for Hirschsprung’s disease reveaIs sequeIIae of varying severity. With increased technica experience, persistence in details, and proper foIIow-up care, these compIications can be kept to a minimum. However, if Iate resuIts confirm earIy reports, the retrorectal puII-through procedure or the segmenta1 resection of State, may, because of reIative simpIicity, prove to be the operation of choice. REFERENCES I. SWENSON, 0. FoIIow up on 200 patients
treated for Hirschsprung’s disease during a ten-year period. Ann. Surg., 146: 706, 1957. 2. HAYS, D. M. and NORRIS, W. J. CongenitaI agangIionic megacoIon. Calijornia Med., 84: 403, ‘956. 3. BILL, A. H., JR. and CHAPMAN, N. D. The entero-
4.
ij.
6.
7. 8.
g.
IO. I I.
12.
13.
14. 15.
16.
coIitis of Hirschsprung’s disease. Its natural history and treatment. Am. J. Surg., 103: 70, 1962. MORGAN, C. N. and GRIFFITHS, J. D. High ligation of the inferior mesenteric artery during operation for carcinoma of the dista1 coIon and rectum. Surg. Gynec. &+Obst., 108: 641, rggg. LEE. C. M., JR. Exnerience with the extended nostoperative care of congenita1 megacolon. ‘Am. Surgeon, 32: 705, 1956. HIATT, R. B. A further description of the pathologic physiology of congenital megacoIon and the resuIts of surgica1 treatment. Pediatrics, 21: 825, 1958. WYLLIE, G. G. Treatment of Hirschsprung’s disease by Swenson’s operation. Lancer, I : 850, 1957. DUHAMEL, B. A new operation for the treatment of Hirschsprung’s disease. Arch. Dis. Cbildbood, 35: 38, 1960. EHRENPREIS, T. Long-term results of rectosigmoidectomy for Hirschsprung’s disease, with a note on Duhamel’s operation_Surgery, 49: 701, 1961. GROB. M. IntestinaI obstruction in the newborn infant. Arch. Dis. Cbikibood, 35: 40, rg6o. Kos~~A, J. ResuIts of surgical treatment in Hirschsprung’s disease. Arch. Dis. Cbildbood, 37: 167, 1962. Louw, J. H. The DuhameI operation for Hirschsprung’s disease. Soutb African M. J., 35: 1053, 1961. MARTIN, L. W. and ALTEMEIR, W. A. CIinicaI experience with a new operation (modified DuhameI procedure) for Hirschsprung’s disease. Ann. Surg., 156: 678, 1962. SNYDER, W. PersonaI communication. STATE, D. SegmentaI coIon resection in the treatment of congenita1 megacoIon (Hirschsprung’s disease). Am. J. Surg., 105: 93, 1963. HALLENBECK,G. A., BROWN, P. M., WAUGH, J. M. and STICKLER, G. B. SurgicaI treatment of
PuII-Through
Operation
Hirschsprung’s disease. A review of 40 cases. Arch Surg., 83: 928, 1961. 17. LANCER, B. and THOMSON, S. Hirschsprung’s disease. Nine years’ experience at the HospitaI for Sick Children, Toronto, Canada. Surgery, 2: 123, ‘959. DISCUSSION WILLIAM H. SNYDER, JR. (Los AngeIes, Calif.): This exceIIent paper by Clausen and Davies is a fair and exhaustive evatuation of the Swenson procedure. DuhameI’s operation and those of others, which wiI1 foIIow, shouId, I beIieve, be considered as modifications of the technica approach to the correction of the basic defect in Hirschsprung’s disease, which Orvar Swenson set forth in 1948. Thus, a11 procedures utilizing this concept shouId bear his name. We, too, at ChiIdrens HospitaI of Los Angeles have experienced simiIar compIications to those described by the authors. Because of this, about a year ago, we began utiIizing the Swenson-Duhamel technic. Thus far, we have performed tweIve such procedures. These tweIve have been compared with twentythree pull-through operations performed during the previous three years. During the last year, we have had no deaths, no anastomotic separation, peIvic infections, or any major complication. Among the twenty-three patients operated on during the preceding three years, there was one death; five peIvic infections, three of which were severe requiring drainage and a protracted hospita1 stay. In one, the infection extended to the right gutter with eventual Iung abscess. There was one stricture. With these compIications weighing heavily on our minds, we switched to the SwensonDuhamel technic. Discussing this worthwhite paper has been a pleasure. ‘H. GLENN BELL (San Francisco, Calif.): I am perhaps one of the oIdest members here and it has been interesting to me to see how the treatment of Hirschsprung’s disease has changed over the past thirty years. EarIy in the rg3o’s, we performed lumbar sympathectomies to treat this disease; this was usually unsuccessfuI. We then started to do anterior resections, removing most of the Ieft coIon and particularly the Iarge diIated thick-waIled portion down to the upper end of the rectum. We then performed an end to end anastomosis. Of course at that time, we were not considering whether or not there were ganglion ceIIs in the bowel wal1. Many of these patients did very we11 after anterior resection. Recentiy, one of the patients operated upon in the middIe 1930’s was admitted to the CIinic for a simpIe inguina1 hernia repair; it was Iearned that he was having no difficulty with his bowe1 function. A barium enema showed that the colon. was apparcntIy compIeteIy normal.
for Hirschsprung’s
Disease
NOWthat .we are on the Iookout for agangIionic sections of the bowel, a new technica procedure has been deveIoped which has just been described. PersonaIIy, I think we ail Iearn from our mistakes and I wouId Iike to tell you about a little patient with Hirschsprung’s disease who came under my care three years ago. He was in such poor condition that I decided the only thing that couId be done was a transverse colostomy, which wouId improve his status enough so I could perform a Swenson pull-through procedure. Much to my surprise, the coIostomy did not function. I had failed to remove a specimen of the bowe1 for biopsy in order to determine whether or not it contained ganglion ceIIs. However, it obviousIy did not. The chiId was reoperated upon and I had to progress to the hepatic Aexure in order to find normai gang&on celis. This coIostomy functioned well. Later another procedure was performed in which the right colon was turned down and anastomosed to the rectum by the Swenson pull-through operation. When last seen, which was recentIy, the patient was doing very weI1. I wouId Iike to ask for a consuItation about another patient who was referred to me about a year and a half ago. This boy had been operated upon elsewhere and a Swenson puII-through procedure had been done. UnfortunateIy, a peIvic abscess from the anastomosis deveIoped. The anastomosis had to be taken down; the coIon was brought out as a singIe barre1 colostomy. The rectal stump was left open with the thought that it would drain the peIvic abscess. When I first saw the boy, it was obvious that he had a smaI1 bowe1 fistuIa to the recta1 stump. He was nearIy moribund; I hesitated to attempt any kind of treatment. Nevertheless, an operation was performed in which practically no anesthetic was given. We were able to free the small bowel fistuIa, cIose it and bring the coIon out as a more satisfactory functioning coIostomy. My question to the authors is as foIIows: Since this boy stiI1 has a recta1 stump 6 cm. Iong, when and how shouId another attempt be made to put his bowel back in continuity? BERNARD P. MULLEN (SeattIe, Wash.): I want to report a case, possibIy the oldest case in medica Iiterature, on which a Swenson puII-through operation was done. These patients aImost never Iive to aduIt Iife without some surgica1 intervention. I saw the boy for the first time a number of years before with a tremendousIy distended abdomen. That was before the sgangiionic segment was discovered as a cause of this disease; I did an anterior resection of a Iarge part of the sigmoid and descending coIon. His heaIth was fairly good foIlowing this procedure. When he was forty-three years of age, more symptoms deveIoped; he had aImost a complete
379
CIausen and Davies wouId appear to be. The diffzcuhies that have arisen, of course, have been matters of technic; I think those who have done this operation reaIize that it is like anything eIse. The more one does it, the simpIer it becomes; one is able to correct errors that have been made in previous operations. As a resuIt of these compIications, new procedures have been devised. Just whether or not these wiI1 continue to be satisfactory wiI1 take a Iong time to teI1. The anterior resections that were done, took many years to prove that this was not a wise procedure. The case Dr. Be11 just toId about, perhaps does not fall into the right category. I do not know whether or not that case was a true Hirschsprung’s disease since pathologic studies are incomplete. State stiI1 prefers the anterior procedure, and he resects ‘way back into the transverse coIon, and claims that one has to Iook at the peristalsis under the fluoroscope. The fluoroscopist wiI1 point out where the peristaItic activity seems to terminate; then, he actuaIIy resects a11 that bowel distaIIy. However, it is rather interesting that of these eighteen cases he has foIIowed up for more than and restudied by roentgenograms ten years, (tweIve of them), in each patient the bowe1 has diIated. However, it is interesting that the dilated bowel stiI1 is functioning very weII. The modification of the DuhameI procedure sounds to me Iike it might be a good procedure. One wiI1 have essentiaIIy haIf of the bowe1 that wiI1 be agangIionic, and the other part wiI1, of course, have ganglion. One of the rea1 advantages of this procedure is that a normal sensation of the rectum wilt be maintained; this is the part in the Swenson procedure which is a IittIe probIem, because if too much recta1 mucosa is left, then symptoms seem to recur. As far as Dr. BeII’s other case is concerned, it would seem to me that if a peIvic resection had already been done, and particularIy if an abscess was present, that probabIy the DuhameI or the Grob-Martin modification wouId seem to be the one that wouId be the simpIest and the one that would most IikeIy succeed. We appreciate having had the opportunity to present this paper.
obstruction from time to time; he had to take daiIy enemas, and the so-called coIonic flushing. Thus, I decided to operate on him. We took roentgenograms and found the contracted aganglionic segment, and decided to do the Swenson puIIthrough procedure. The problem that I faced was the tremendous distension of this greatiy hypertrophied boweI; I wondered, if in removing the aganglionic segment, and in puIIing the Iarge bowe1 down to do the Swenson operation, wouId that bowe1, as it contracted, puI1 the anastomosis apart. When the bowel contracts to its norma size, it not only contracts in diameter but also in length. This did not prove to be the case, and one can be reassured that that would not be a complication if the patient is an ad&. There was one patient who lived to an oIder age. The operation was done at the Mayo CIinic a few years before, and I found the report in the Bulletin of the Mayo Clinic. First, they removed most of the Iarge howeI and Ieft the agangIionic segment. At a second operation they removed the entire coIon and anastomosed the iIeum to the anus, but the patient continued to have troubIe. I have not seen further report on the patient’s condition. He was a doctor’s son and evidently had been pampered, until about age fifty-three. The patient of mine whom I mentioned previously is in good health and doing very weI1. I removed the agangIionatic segment. He was a married man and I worried about his impotence. As soon as his anastomosis was we11 healed, I urged him to go home and try it; which he did. He came back and happiIy reported that everything was aII right. EDWIN G. CLAUSEN (cIosing): I believe that this condition and the deveIopment of Swenson’s operation shows pretty we11 what happens when one deveIops a highIy technical procedure. By 1950 certainIy everyone had decided that this was the operation of choice, and certainly it fitted we11 physioIogicaIIy. However, it took about ten years and many complications before peopIe started Iooking for an easier procedure. I do not think there is any doubt that from the knowledge we have at the present time the Swenson puII-through operation is a proper procedure, or
380
EDWIN G. CLAUSEN, M.D., O&and,
From tbe Department of Surgery, tbe Children’s Hospital of tbe East Bay, Oakland, California and tbe Valley Children’s Hospital, Fresno, Calijornia.
IFTEEN years ago Swenson [I] introduced use of abdominaLperineal resection of the rectum for the treatment of Hirschsprung’s disease. This procedure has been accepted by most American surgeons. However, because of postoperative complications, some European and more recentIy American sursurgeons, its superiority over geons, have questioned other methods. A review of the comphcations of reported cases, as we11 as those seen by us at the Children’s HospitaI of the East Bay, in OakIand, the VaIIey ChiIdren’s HospitaI, Fresno, and the University of CaIifornia HospitaI, San Francisco, will be presented. The patients who were operated upon shortIy after Swenson introduced the puII-through procedure were mostIy those who had survived the earIy years of the disease. As a resuIt, a one stage operative procedure was done. Now the majority of cases are recognized during infancy. Since the high mortality rate is usuaIIy a result of obstruction and enterocoIitis a coIostomy is generaIIy advisabIe as soon as the diagnosis is made. Some of the patients with milder symptoms can be treated with diet, laxatives and enemas. Most surgeons agree that the puII-through procedure is too drastic for chiIdren under six months of age, and advise deferring the operation unti1 the chiId is tweIve to twenty months oId. As a resuIt, any of those patients who come to a definitive operation will have aIready had a proxima1 coIostomy.
F the
* Presented American
A coIostomy must be made in the part of the coIon which contains parasympathetic gangIion. Since in some newborn infants the Iine of demarcation of agangIionic bowe1 is ill defined, and since even in those in whom the demarcation is apparent the aganghonic area may extend proximaIIy in varying degrees, a frozen section must aIways be done to determine unequivocahy the area of normaI bowe1. Opinions differ as to the most desirabIe Iocation for coIostomy. In a transverse coIostomy a Ioop type in continuity is adequate. The Ioop coIostomy is not diffrcuIt to manage because of the fluidity of the stooIs; it is a simpIe procedure, and affords protection for the subsequent puII-through operation. On the other hand, this type of coIostomy does require an extra operative procedure to cIose it; occasionaIIy, it wiI1 interfere with mobiIization of the coIon to obtain the Iength for the puIIProIapse is 0ccasionaIIy through procedure. bothersome in transverse coIostomies. A more dista1 coIostomy affords more colon for absorption and can be eIiminated at the time of the puII-through procedure. However, because of its size and thickness, a Ioop coIostomy may present technica probIems. An end coIostomy would be more diffrcuIt to perform. At the time of the puII-through operation, contamination may occur if the coIostomy has to be taken down. RegardIess of the type of coIostomy, great care must be taken to prevent proIapse of smaI1 bowe1 around the coIostomy. This can be done by careful approximation of the peritoneum about the coIon. Deaths foIIowing the operation occur in the
at the annua1 meeting of the Pacific Coast SurgicaI Association, 3-6 1963.
Journal
of Surgery,
Volume
ro6,
August
rg6j
372
G. DAVIES, JR., M.D.,
PaIm Springs, California,
February,
PuII-Through CAUSES
OF DEATH
Operation
FOLLOWING
SWENSON
for Hirschsprung’s
TABLE I PULL-THROUGH
Disease
PROCEDURE
FOR HIRSCHSPRUNG’S
DISEASE
Totai Operation
Authors
T
Deaths
No.
T
EarIy Deaths
.-
Late Deaths
No.
‘er cent
No.
Cause
Three
Cardiac arrest, peritonitis, tracheobronchitis Tension pneumothorax from ruptured congenital
Cause
-_ Ehrenpreis
[9]
HaIIenback
Hays
[z].
Hiatt [6].
Sixty-nine
Four
5.8
d-j....
Thirty-four
Four
10.7
tOne
......
Nineteen
Six
31.5
Four
Sixty-seven
Five
7.4
,One
.........
Kostia [I I].
.......
Twenty-eight
Six
Langer [z7]. Swenson [r].
....... ......
Twenty-two Two hundred
Two Thirteen
9.1 6.5
Two Six
Nine
5.9
Six
Three
8.8
Thret
WyIIie [7] .........
. One hundred fifty-two
Authors
...........
Thirty-four
-
Three
21.4
cyst Shock, necrosis proximaI segment [9] PuImonary aspiration Not mentioned
One
Three
Two
Four Three
Peritonitis Peritonitis [8], small bowe1 obstruction, septicemia, biIatera1 adrena hemorrhage, gastroenteritis Shock [9], bowe1 necrosis [S], peritonitis EnterocoIitis, shock, sepsis
None Seven
Three
Puimonary aspiration after coIostomy closure EnterocoIitis
Postoperative obstruction, stricture of anastomosis EnterocoIitis Intestinal obstruction, encephalitis, died after second puII-through procedure
.. EnterocoIitis
IntestinaI obstruction [8], coIitis
None
...
-
immediate postoperative period and after the patient has Ieft the hospita1. The death rate has been Iowered to an acceptabIe IeveI now that experience has proved the operation to be inadvisabIe in infants, technica probIems have been eradicated and close attention has been paid postoperatively to the fluid and eIectroIyte balance, and to the possibility of enterocoIitis. Many of the earIy deaths were due to sepsis from a Ieak in the anastomosis or from septicemia. (TabIe I.) Many smaI1 infants died of shock or compIications reIative to any major operation. In Swenson’s series, four of the six patients who died were infants. Hays and Norris [2] reported three deaths in four infants operated upon in the first six months of Iife. Late deaths were usuaIIy due to rapid, fuIminating enterocoIitis, which caused death sometimes within twenty-four hours unIess
vigorousIy
treated.
reported eight who
patients, have
are IikeIy
BiII and
enterocoIitis eight
Chapman
[3] have
in twenty-four of whom
had
enterocoIitis
to have
it after
of forty-
died.
before operation
Patients operation
as weI1. To
must aIert the famiIy to the seriousness of abdomina1 distention, obstipation or diarrhea. Deaths aIso occurred after stranguIating bowe1 obstruction which was neglected because of faiIure to differentiate between simpIe functiona distention and vomiting, and a true stranguIating obstruction. ExceIIent descriptions of the technica aspects of this operation have been presented eIsewhere. We wish only to emphasize the saIient features which we beIieve have contributed to some of the major compIications. Leakage of the anastomotic site occurred in a11 reported series in which there were compIicaprevent
373
this,
one
CIausen and Davies
FIG. I. IIlustrates short left colic artery, ascending and descending branches with poor or absent marginal artery. Figure on right indicates preferred area of division of arteries to obtain Iength of mesentery.* * (Reproduced
by permission of C. N. Morgan
and J. D. Grifliths. Surg. Gynec. and Obst., 108: 641, 1959.)
branch forming the major margina artery, and consequentIy a very smaI1 artery may be near the coIon. (Fig. I.) ShouId either or both of these branches be divided the dista1 coIon may become ischemic. Genera1 carefu1 dissection must be done with specia1 reference to avoidance of injury to the margina artery, if dista1 bowe1 ischemia is to be prevented. ShouId the bowe1 be resected to the proxima1 descending coIon or transverse coIon, it wiI1 be necessary to divide the middIe colic artery and then bring the coIon down the right side of the abdomen with the hinge on the right coIic or iIeocoIic artery. Although as much coIon as possibIe should be saved, resection to more norma bowe1 is desirable and may prevent the pIeating which might resuIt from anastomosing dilated proxima1 bowe1 to smaIIer dista1 rectum. No suture line Ieakage of any consequence has been reported in patients who have had a proxima1 coIostomy. AIthough some cases of suture Iine leakage were treated successfuIIy by conservative means, a proxima1 coIostomy shouId be done as soon as the leakage is ascertained. This frequenty may not onIy be Iife saving, but may prevent Iong periods of hospitaIization, intractabIe fistuIa and stenosis. We do not ordinariIy drain the presacra1 space.
tions. The proxima1 bowe1 must not be on tension; this frequentIy necessitates mobiIization of the spIenic ffexure for even a Iesion in the Iower sigmoid area. OrdinariIy about 50 per cent more bowe1 is needed for the tota pelvic pulLthrough procedure, which is measured from the promontory of the sacrum to the symphysis pubis. Since it is usuaIIy mesentery that prevents descent into the peIvis, the superior hemorrhoidal artery must aIways be sacrificed as we11 as the proxima1 sigmoid branches. If IocaI ischemia is to be avoided, the division of these vesseIs shouId be done near their base, thereby preserving as many arcades as possibIe. OccasionaIIy the Ieft coIic artery must aIso be divided, and the bIood supply to the coIon is then dependent on the margina artery associated with the middIe [4] have coIic artery. Morgan and Griffiths pointed out numerous anomalies of arteries to the coIon. In IOO cases studied no middIe or right coIic artery was present in two instances. The middIe coIic artery was absent in 22 per cent of the cases and the Ieft colic artery was absent in 6 per cent. In one instance there was no connection of the marginal artery between the middIe and Ieft coIic arteries. The Ieft coIic artery may be short with a proxima1 and dista1 374
PuII-Through
Operation for Hirschsprung’s TABLE
EARLY
COMPLICATIONS
FOLLOWING
II
SWENSON
HIRSCHPRUNG’S Anastomotic
Disease
PULL-THROUGH
PROCEDURE
Leak
Stricture
Author
Incomplete
No.
Ehrenpreis
[9].
...
Five
I-IaIIenback [,161..
. Nine
IIays
. Three
[2].
Kostia [II].
.....
Six
I-anger [r7].
......
Six
Swenson [I].
iVylIie [7].
Authors.
FOR
DISEASE
.......
.........
.
Five
None
Four
No.
Result
One died, no coIostomy, four responded to treatment coIostomy
seven
Five heaIed without cofour healed Iostomy, with coIostomy Died
Not mentionec
Five recovered, one died foIIowing operation for uretheraI IistuIa One died, four cIosed spontaneousIy, one required second operation
Six
Five
One necrosis of proxima1 Nine bowe1, one fistuIa deveIoped. two gross Ieak, one peIvic abscess, a11 responded to proxima1 coIostomy Seven . .
One permanent coIostomy, three responded to treatment, coIostomy
Four
ResuIts
411responded to dilatation
. Four responded to diIatation, one died Responded to diIatation
Five
Resection
Six patients, three reoperated upon, one constipated, one diarrhea, one norma Not mentioned
Two cases, corrected DuhameI operation
by
Two responded to diIatation, three under treatment by diIatation Eight responded to diIatation, one needed reoperation
One patient, reoperated upon and cured
Six responded to diIatation, one needed reoperation Responded to dilatation
Three cases, two ma1 bowe1, one rectum
None
proxidistaI
One case, reresection now we11
and
IncompIete resection of agangIionic bowe1 has been reported. (TabIe II.) In some instances this occurred whiIe treating earIy cases when the proxima1 bow1 was not studied by frozen section to determine the site of norma gangIion. The Iine of demarcation between the agangIionic and gangIionic areas cannot be de‘termined by gross examination; frozen section must aIways determine the presence of gangIia. However, recurrent constipation is not uncommon and wiI1 be discussed Iater. After the abdomina1 portion of the operation is compIeted a bimanua1 abdomina1 perinea1 determination of the adequacy of the peIvic dissection shouId be done. This wiII aIIow unrestricted eversion of the Iower segment and assures adequate dista1 bowe1 remova1. There must be
However, if there seems to be excessive and uncontroIIabIe oozing, drainage through the perineum wouId be advisabIe. Many surgeons have reported strictures at the site of anastomosis. (TabIe II.) Strictures are more common when a coIostomy is present than when the bowe1 is functioning. Repeated recta1 examination must be done even in the uncompIicated cases. However, when a major disruption occurs at the suture Iine, strictures wiI1 occur and must be treated vigorousIy by The second puII-through digita diIatation. procedure can be accomplished even if this stricture cannot be diIated so that a permanent coIostomy shouId not be made. DigitaI diIatations wiI1 cure most strictures, but it may be necessary to repeat them for many months. 375
CIausen
and
TABLE LATE
COMPLICATIONS
-
Author
FOLLOWING
SWENSON
Davies III
PULL-THROUGH
FOR HIRSCHSPRUNG’S
Diarrhea
Constipation
Incontinence
DISEASE
No. of IntestinaI Obstruction
-. Ehrenpreis
[9].
. Six cases, one treated by Four cases treated sphinctorotomy, cases late onset
HaIIenback
[I 61.
Three cases, relieved by enemas
Kostia [II]. Langer [r7].
Swenson [I]. WylIie [7]
Authors.
.
five
.
..
Three cases, treated by Iaxatives and enemas Three cases, treated by Iaxative and occasional enemas One case, treated by occasional enema Thirty-four cases, twothirds under three yr. of age, nine started after one yr., fourteen need two yr. or more of treatment Six cases, relieved with occasiona enema or cathartic
by
sphinctorotomy
Twenty-six cases, four we11 later, twenty-two cases persistent (eight bad) Two cases, mild
Four cases, two well after two yr., two remain problem Not mentioned Two cases
One
Not mentioned
Not mentioned
Two
Four cases, temporary
None
None
Not mentioned
Forty-one
Nine cases responded to repeated enemas, two required external sphincterotomy
Occasional case relieved with correction of diarrhea or constipation
Four
cases
Seven, two died
Three, reheved by operation
sufficient mucous membrane for proper sensation, and yet an excessive amount may resuIt in recurrent symptoms. The resuIts foIIowing resection are dramatic and most patients are we11 after a varying period of adjustment. Lee [5] has stressed the importance of carefu1 and proIonged foIIowup studies and Swenson [I] has emphasized the importance of performing the operation before the bowe1 training period. Hiatt [6] has discussed in detai1 the factors invoIved in deveIoping norma bowe1 habits. In most instances after operation, bowe1 habits are reIativeIy norma unti1 the training period. So much emphasis has been pIaced on having a bowe1 movement prior to operation that the chiId may rebel against anything to do with bowe1 training. On the other hand, the sudden Iack of attention may induce a functiona constipation. Many patients with a coIostomy have never had the sensation of a spontaneous bowe1 movement. The whoIe process is new and these patients must have bowel training by a we11 instructed, patient mother. Constipation often deveIops postoperatively, and varies from miId irreguIarity to recurrent
impactions. The diffIcuIty in evaIuating this symptom is indicated by its variabiIity in the reported cases. (TabIe III.) It is common for the patient to have normal daiIy bowel movements immediateIy postoperativeIy and for severa months, and not to experience constipation unti1 much later. WyIIie [7] reports nine patients in whom constipation deveIoped after one year of norma bowe1 movements. The acute obstructions which are associated with fever, toxicity and abdomina1 distention should be corrected by coIonic irrigations. The more chronic type of constipation wiI1 be reIieved by coIonic irrigations one to two times per week. It may be necessary to continue the irrigations for one to two years. With proper bowe1 training, diet, and judicious use of catharsis most of the more obstinate cases can be kept under controI unti1 the chiId is oId enough to be more cooperative. The physician and mother must work in cIose cooperation. If an adequate stoo1 is not produced in forty-eight hours, a miId Iaxative or enema shouId be given and impactions must be prevented. Postoperative diarrhea may be a more 376
PuII-Through
Operation
bothersome and more serious symptom than constipation. Again diarrhea varies in severity and its incidence is diffIcuIt to assess accurateIy. (TabIe III.) StooI cuItures are not abnormal, and there is no specific change in the bowe1 waI1 as a ruIe. However, if untreated or negIected for any Iength of time, the coIon above the resected area appears on proctoIogic examination to present nonspecific ulcerative coIitis, and perforation may even occur. The cause of diarrhea is postuIated to be simiIar t.o that causing bowe1 obstruction. The point of obstruction is a spastic, aganglionic interna sphincter, resuIting in proxima1 coIonic diIat.ation with feca1 materia1 and fluid. Pressure is buiIt up and is suddenIy reIeased when the sphincter reIaxes. Vomiting, dehydration, fever and toxicity depend on the degree of obstruction. Death has been reported within a twentyfour hour period in patients with no abnormaIity of the bowe1 waI1 or bowe1 mucosa. ConsequentIy, the method of treatment is to reIease the ana obstruction by digita examirecta1 tubes and enemas. UsuaIIy nation, coIonic irrigations twice daiIy for three or four days wiI1 be effective. In more persistent, severe cases a tota externa1 sphincterotomy may be necessary. This operation does not result in incontinence. Incontinence may be associated with either constipation or diarrhea. Variation of reported cases (TabIe III) must depend on how criticaIIy t.he symptom is reviewed. UsuaIIy as the chiId gets aider, the frequency of soiling decreases. Loss of sphincter control is not a major probIem. With the correction of diarrhea, constipation and feca1 impaction, incontinence wiI1 cease accordingly. The incidence of intestina1 obstruction after a puII-through procedure may be higher than that foIIowing a simple Iaparotomy. An extensive dissection and mobilization of the coIon necessitates carefu1 reperitoneaIization, especially in the peIvis. However, if the proxima1 bowel is diIated, it may fiI1 the peIvis compIeteIy, making peritoneaIization virtuaIIy unnecessary. NevertheIess, severa deaths in the Iate postoperative period have been the result of unrecognized stranguIating obstruction. Since recurrent distention, vomiting and obstipation occur as a result of functiona a true stranguIation may be obstruction, overlooked. Impotence in maIe patients has been feared
for Hirschsprung’s
Disease
as one of the complications IikeIy to occur in the puII-through operation. However, Swenson [I] has operated upon tweIve patients who have compIete, norma sexua1 activity; apparentIy, this compIication is rare: A peIvic dissection must be done with care and precision. After division of the superior hemorrhoida vesseIs, a definite cIeavage pIane is readiIy entered, aIIowing the hypogastric pIexus to remain undisturbed. This pIane shouId then be carried posteriorly deep into the peIvis to the tip of the coccyx. AnteriorIy, the pIane between the seminal vesicles, prostate and urethra is narrow but we11 defined. This anterior IateraI dissection must be cIose to the bowe1 to prevent disturbance to the nerves entering the genitourinary tract, more anteriorIy. Since the peIvis is shaIIow, dissection is considerabIy easier than in the aduIt and may be performed under direct vision much of the time. Impotence has not been a probIem in the patients oId enough to be evaIuated in this series. Because of the compIications described previousIy, DuhameI [8] of Paris has advised a more simpIe procedure. He cIaims this procedure can be done in very young infants and with a very Iow mortaIity rate. In this procedure the bowel is divided at the rectosigmoid and the dista1 end is cIosed. After resection of the agangIionic area, a presacra1 dissection is performed and the proxima1 bowe1 is brought into this space, Ieaving the rectum essentiaIIy undisturbed anteriorIy and IateraIIy. By making a contmuous mucous membrane incision posteriorIy, the externa1 sphincter is separated from the rectum and the proxima1 bowe1 is brought down through this area, opened and sutured posteriorIy. The anterior aspect is opened into the rectum by the use of Kocher cIamps pIaced in a v-shaped manner. The main advantage of this procedure, according to DuhameI, is that it can be used in very smaI1 infants with reIativeIy IittIe shock. The norma proxima1 bowel then comes in contact with the anus and the norma externa sphincter. This section of the rectum is undisturbed and consequentIy there is no danger of disturbing sexua1 or urinary bIadder function. This procedure has been regarded with interest by some European surgeons [g-rz] and more recentIy by American surgeons [x3, 141. However, the incidence of incontinence, as reported by DuhameI, seems to be greater 377
CIausen and Davies than that associated with the Swenson procedure. More recentIy Martin and AItemeir [ 131 and Grob [IO] have reported a procedure which is similar to that of DuhameI. They aIso Ieave the rectum in pIace, but bring the norma bowe1 down to the IeveI of the interna sphincter and then perforate the rectum at this point, puIIing the proxima1 bower into the dista1 rectum, just proximal to the interna sphincter. Grob then sutures the proxima1 bowe1 to the rectal mucosa. Martin, however, uses a spur crushing cIamp which opens the entire dista1 rectum and Iower coIon into one Iarge cavity, haIf of which wiI1 have norma the foIIow-up periods in gangIia. However, these patients were so short that it cannot be ascertained how we11 this wiI1 function. We know that after an anterior resection of the Hartman type, patients do very we11 for a Iong period; however, eventuaIIy the proxima1 bowe1 begins to diIate, and in many instances and a further procedure is decompensate, necessary. State [15] has recentIy reviewed eighteen cases in which he has utiIized resection of the rectosigmoid, descending coIon and part of the transverse colon with anastomosis of the transverse coIon to the rectum. Resection of the proxima1 dilated bowe1 has been done many times before, with generaIIy poor results. However, State emphasizes the need to resect the entire descending coIon, the rectosigmoid, and part of the transverse coIon. This segmenta area is determined by the peristaItic activity at the time of the barium enema. His resuIts have been exceIIent, although coIon dilation has occurred in the tweIve cases that he restudied by roentgenograms, ten or more years Iater. However, the area of diIation appears to be emptying weI1. It is conceivabIe, however, that in time sufficient diIations couId occur resuIting in functiona disability. In spite of the serious nature of the Swenson puII-through operation it wouId appear to be the most physioIogic operation at this time. The procedure requires meticuIous attention to detail. If technica detaiIs are not faithfuIIy attended to, compIications of a serious nature may result. Postoperative care after a successfu1 primary operation must be continued for several months until the chiId has learned proper bowe1 habits. NevertheIess, the exact physioIogic defect is being questioned, and other operative procedures are being utiIized. 378
SUMMARY
A review of the compIications of Swenson’s puII-through operation for Hirschsprung’s disease reveaIs sequeIIae of varying severity. With increased technica experience, persistence in details, and proper foIIow-up care, these compIications can be kept to a minimum. However, if Iate resuIts confirm earIy reports, the retrorectal puII-through procedure or the segmenta1 resection of State, may, because of reIative simpIicity, prove to be the operation of choice. REFERENCES I. SWENSON, 0. FoIIow up on 200 patients
treated for Hirschsprung’s disease during a ten-year period. Ann. Surg., 146: 706, 1957. 2. HAYS, D. M. and NORRIS, W. J. CongenitaI agangIionic megacoIon. Calijornia Med., 84: 403, ‘956. 3. BILL, A. H., JR. and CHAPMAN, N. D. The entero-
4.
ij.
6.
7. 8.
g.
IO. I I.
12.
13.
14. 15.
16.
coIitis of Hirschsprung’s disease. Its natural history and treatment. Am. J. Surg., 103: 70, 1962. MORGAN, C. N. and GRIFFITHS, J. D. High ligation of the inferior mesenteric artery during operation for carcinoma of the dista1 coIon and rectum. Surg. Gynec. &+Obst., 108: 641, rggg. LEE. C. M., JR. Exnerience with the extended nostoperative care of congenita1 megacolon. ‘Am. Surgeon, 32: 705, 1956. HIATT, R. B. A further description of the pathologic physiology of congenital megacoIon and the resuIts of surgica1 treatment. Pediatrics, 21: 825, 1958. WYLLIE, G. G. Treatment of Hirschsprung’s disease by Swenson’s operation. Lancer, I : 850, 1957. DUHAMEL, B. A new operation for the treatment of Hirschsprung’s disease. Arch. Dis. Cbildbood, 35: 38, 1960. EHRENPREIS, T. Long-term results of rectosigmoidectomy for Hirschsprung’s disease, with a note on Duhamel’s operation_Surgery, 49: 701, 1961. GROB. M. IntestinaI obstruction in the newborn infant. Arch. Dis. Cbikibood, 35: 40, rg6o. Kos~~A, J. ResuIts of surgical treatment in Hirschsprung’s disease. Arch. Dis. Cbildbood, 37: 167, 1962. Louw, J. H. The DuhameI operation for Hirschsprung’s disease. Soutb African M. J., 35: 1053, 1961. MARTIN, L. W. and ALTEMEIR, W. A. CIinicaI experience with a new operation (modified DuhameI procedure) for Hirschsprung’s disease. Ann. Surg., 156: 678, 1962. SNYDER, W. PersonaI communication. STATE, D. SegmentaI coIon resection in the treatment of congenita1 megacoIon (Hirschsprung’s disease). Am. J. Surg., 105: 93, 1963. HALLENBECK,G. A., BROWN, P. M., WAUGH, J. M. and STICKLER, G. B. SurgicaI treatment of
PuII-Through
Operation
Hirschsprung’s disease. A review of 40 cases. Arch Surg., 83: 928, 1961. 17. LANCER, B. and THOMSON, S. Hirschsprung’s disease. Nine years’ experience at the HospitaI for Sick Children, Toronto, Canada. Surgery, 2: 123, ‘959. DISCUSSION WILLIAM H. SNYDER, JR. (Los AngeIes, Calif.): This exceIIent paper by Clausen and Davies is a fair and exhaustive evatuation of the Swenson procedure. DuhameI’s operation and those of others, which wiI1 foIIow, shouId, I beIieve, be considered as modifications of the technica approach to the correction of the basic defect in Hirschsprung’s disease, which Orvar Swenson set forth in 1948. Thus, a11 procedures utilizing this concept shouId bear his name. We, too, at ChiIdrens HospitaI of Los Angeles have experienced simiIar compIications to those described by the authors. Because of this, about a year ago, we began utiIizing the Swenson-Duhamel technic. Thus far, we have performed tweIve such procedures. These tweIve have been compared with twentythree pull-through operations performed during the previous three years. During the last year, we have had no deaths, no anastomotic separation, peIvic infections, or any major complication. Among the twenty-three patients operated on during the preceding three years, there was one death; five peIvic infections, three of which were severe requiring drainage and a protracted hospita1 stay. In one, the infection extended to the right gutter with eventual Iung abscess. There was one stricture. With these compIications weighing heavily on our minds, we switched to the SwensonDuhamel technic. Discussing this worthwhite paper has been a pleasure. ‘H. GLENN BELL (San Francisco, Calif.): I am perhaps one of the oIdest members here and it has been interesting to me to see how the treatment of Hirschsprung’s disease has changed over the past thirty years. EarIy in the rg3o’s, we performed lumbar sympathectomies to treat this disease; this was usually unsuccessfuI. We then started to do anterior resections, removing most of the Ieft coIon and particularly the Iarge diIated thick-waIled portion down to the upper end of the rectum. We then performed an end to end anastomosis. Of course at that time, we were not considering whether or not there were ganglion ceIIs in the bowel wal1. Many of these patients did very we11 after anterior resection. Recentiy, one of the patients operated upon in the middIe 1930’s was admitted to the CIinic for a simpIe inguina1 hernia repair; it was Iearned that he was having no difficulty with his bowe1 function. A barium enema showed that the colon. was apparcntIy compIeteIy normal.
for Hirschsprung’s
Disease
NOWthat .we are on the Iookout for agangIionic sections of the bowel, a new technica procedure has been deveIoped which has just been described. PersonaIIy, I think we ail Iearn from our mistakes and I wouId Iike to tell you about a little patient with Hirschsprung’s disease who came under my care three years ago. He was in such poor condition that I decided the only thing that couId be done was a transverse colostomy, which wouId improve his status enough so I could perform a Swenson pull-through procedure. Much to my surprise, the coIostomy did not function. I had failed to remove a specimen of the bowe1 for biopsy in order to determine whether or not it contained ganglion ceIIs. However, it obviousIy did not. The chiId was reoperated upon and I had to progress to the hepatic Aexure in order to find normai gang&on celis. This coIostomy functioned well. Later another procedure was performed in which the right colon was turned down and anastomosed to the rectum by the Swenson pull-through operation. When last seen, which was recentIy, the patient was doing very weI1. I wouId Iike to ask for a consuItation about another patient who was referred to me about a year and a half ago. This boy had been operated upon elsewhere and a Swenson puII-through procedure had been done. UnfortunateIy, a peIvic abscess from the anastomosis deveIoped. The anastomosis had to be taken down; the coIon was brought out as a singIe barre1 colostomy. The rectal stump was left open with the thought that it would drain the peIvic abscess. When I first saw the boy, it was obvious that he had a smaI1 bowe1 fistuIa to the recta1 stump. He was nearIy moribund; I hesitated to attempt any kind of treatment. Nevertheless, an operation was performed in which practically no anesthetic was given. We were able to free the small bowel fistuIa, cIose it and bring the coIon out as a more satisfactory functioning coIostomy. My question to the authors is as foIIows: Since this boy stiI1 has a recta1 stump 6 cm. Iong, when and how shouId another attempt be made to put his bowel back in continuity? BERNARD P. MULLEN (SeattIe, Wash.): I want to report a case, possibIy the oldest case in medica Iiterature, on which a Swenson puII-through operation was done. These patients aImost never Iive to aduIt Iife without some surgica1 intervention. I saw the boy for the first time a number of years before with a tremendousIy distended abdomen. That was before the sgangiionic segment was discovered as a cause of this disease; I did an anterior resection of a Iarge part of the sigmoid and descending coIon. His heaIth was fairly good foIlowing this procedure. When he was forty-three years of age, more symptoms deveIoped; he had aImost a complete
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CIausen and Davies wouId appear to be. The diffzcuhies that have arisen, of course, have been matters of technic; I think those who have done this operation reaIize that it is like anything eIse. The more one does it, the simpIer it becomes; one is able to correct errors that have been made in previous operations. As a resuIt of these compIications, new procedures have been devised. Just whether or not these wiI1 continue to be satisfactory wiI1 take a Iong time to teI1. The anterior resections that were done, took many years to prove that this was not a wise procedure. The case Dr. Be11 just toId about, perhaps does not fall into the right category. I do not know whether or not that case was a true Hirschsprung’s disease since pathologic studies are incomplete. State stiI1 prefers the anterior procedure, and he resects ‘way back into the transverse coIon, and claims that one has to Iook at the peristalsis under the fluoroscope. The fluoroscopist wiI1 point out where the peristaItic activity seems to terminate; then, he actuaIIy resects a11 that bowel distaIIy. However, it is rather interesting that of these eighteen cases he has foIIowed up for more than and restudied by roentgenograms ten years, (tweIve of them), in each patient the bowe1 has diIated. However, it is interesting that the dilated bowel stiI1 is functioning very weII. The modification of the DuhameI procedure sounds to me Iike it might be a good procedure. One wiI1 have essentiaIIy haIf of the bowe1 that wiI1 be agangIionic, and the other part wiI1, of course, have ganglion. One of the rea1 advantages of this procedure is that a normal sensation of the rectum wilt be maintained; this is the part in the Swenson procedure which is a IittIe probIem, because if too much recta1 mucosa is left, then symptoms seem to recur. As far as Dr. BeII’s other case is concerned, it would seem to me that if a peIvic resection had already been done, and particularIy if an abscess was present, that probabIy the DuhameI or the Grob-Martin modification wouId seem to be the one that wouId be the simpIest and the one that would most IikeIy succeed. We appreciate having had the opportunity to present this paper.
obstruction from time to time; he had to take daiIy enemas, and the so-called coIonic flushing. Thus, I decided to operate on him. We took roentgenograms and found the contracted aganglionic segment, and decided to do the Swenson puIIthrough procedure. The problem that I faced was the tremendous distension of this greatiy hypertrophied boweI; I wondered, if in removing the aganglionic segment, and in puIIing the Iarge bowe1 down to do the Swenson operation, wouId that bowe1, as it contracted, puI1 the anastomosis apart. When the bowel contracts to its norma size, it not only contracts in diameter but also in length. This did not prove to be the case, and one can be reassured that that would not be a complication if the patient is an ad&. There was one patient who lived to an oIder age. The operation was done at the Mayo CIinic a few years before, and I found the report in the Bulletin of the Mayo Clinic. First, they removed most of the Iarge howeI and Ieft the agangIionic segment. At a second operation they removed the entire coIon and anastomosed the iIeum to the anus, but the patient continued to have troubIe. I have not seen further report on the patient’s condition. He was a doctor’s son and evidently had been pampered, until about age fifty-three. The patient of mine whom I mentioned previously is in good health and doing very weI1. I removed the agangIionatic segment. He was a married man and I worried about his impotence. As soon as his anastomosis was we11 healed, I urged him to go home and try it; which he did. He came back and happiIy reported that everything was aII right. EDWIN G. CLAUSEN (cIosing): I believe that this condition and the deveIopment of Swenson’s operation shows pretty we11 what happens when one deveIops a highIy technical procedure. By 1950 certainIy everyone had decided that this was the operation of choice, and certainly it fitted we11 physioIogicaIIy. However, it took about ten years and many complications before peopIe started Iooking for an easier procedure. I do not think there is any doubt that from the knowledge we have at the present time the Swenson puII-through operation is a proper procedure, or
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