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Modified Duhamel procedure for treatment of Hirschsprung's disease in infancy and childhood: Review of 41 consecutive cases
Modified Duhamel Procedure for Treatment of Hirschsprung's D i s e a s e in Infancy and Childhood: Review of 41 Consecutive Cases By Timothy G. Canty San Diego, Cafifornia 9 The original Duhamel procedure has undergone several important modifications over the years by Martin, Ravitch, Soper, Talbert, and others. Combining the best features of these various modifications, a m o d i f i e d Duhamel procedure has been developed and utilized in 41 consecutive infants and children with all types of Hirschsprung's disease, including three with long segment involvement. The procedure includes the use of a formal upper and lower anostomosis and division of the spur by the GIA stapling device. There were no deaths and no early complications. A t 2 - 7 yr all children are toilettrained, continent, and growing well. With appropriate modifications, the Duhamel operation is a very satisfactory definitive procedure for Hirschsprung's disease in infancy and childhood. INDEX WORDS: Agangiionic megacolon; sprung's disease; Duhamel operation.
Hirsch-
ONTROVERSY exists as to which is the
C optimum definitive procedure for treatment of aganglionic megacolon. Though the results of Swenson with his pullthrough procedure are legend, they have been duplicated by few.~ 3 Morbidity and mortality rates that were unacceptable in the hands of others led to the development of two other commonly used procedures in addition to the Swenson pullthrough: the Soave, or endorectal, procedure and its various modifications, and the Duhamel pullthrough and its modifications. ~'4-~2 All three of these procedures in use today successfully treat Hirschsprung's disease. Each has its own set of risks, complications, and advocates. The original Duhamel procedure was noted for problems with distention of the remaining rectal stump in spite of a variety of methods used to minimize the spur between the Stump and the pulled through colon. Martin in 1967, followed by Soper and Miller, modified the original Duham e l procedure to include both a formal upper and lower anastomosis, thereby completely eliminating the "blind pouch" problem, and used a crushing clamp to divide the common walls of the two bowel segments.7'~~ In 1968 this was then further modified and greatly simplified by Steichen and Ravitch and others by the introducJournal of Pediatric Surgery, Vol. 17, No. 6 (December), i 982
tion of the GIA stapling device. T M Combining these various modifications, the Duhamel procedure avoids the problems of stump impaction, pelvic dissection, and neuromuscular injury; moreover, it provides very satisfactory long-term results in regards to continence and constipation. This paper will report the results using these modifications of the Duhamel procedure in 41 consecutive infants and children with biopsyproven Hirschsprung's disease. CLINICAL MATERIAL Over the past 7 yr a modified Duhamel procedure has been used as the primary definitive treatment of Hirschsprung's disease in 41 consecutive infants and children. The series includes 30 males and 11 females. Twenty-eight patients presented symptoms leading to evaluation and diagnosis in the newborn period. Ten patients were diagnosed in the first year of life, and one at 15 mo of age. There were two older patients undiagnosed until ages 10 and 12 yr, On detailed questioning of the families of all 41 patients, all had difficulties with bowel habits and constipation since birth. Aganglionosis was localized to the low rectosigmoid in 38 patients. One male child had aganglionosis extending to the hepatic flexure and two children, one male and one female, had total colonic involvement. Diagnosis in all three children with long-segment disease was delayed. The diagnostic approach to Hirschsprung's disease in this group was as follows: after suggestive history and plain abdominal radiographs, a barium enema followed by suction rectal biopsy are performed. If these studies are not conclusive, full-thickness rectal biopsy is performed. Diagnostic accuracy utilizing barium enema approached 70% and increased to about 80% with suction biopsy. With fullthickness biopsy~ the diagnositc accuracy was 100%. The use of the suction biopsy capsule (Quinton Instruments, Seattle Wash.), which provides a small specimen containing mucosa and submucosa only, definitely involves a period of learning
From the Department of Surgery, Division of Pediatric Surgery, University of California, San Diego Medical Center, San Diego, Calif. Presented before the Thirteenth Annual Meeting of the American Pediatric Surgical Association, Phoenix, Arizona, May 29-June 1, 1982. Address reprint requests to Timothy G. Canty, M.D., Department of Surgery, Division of Pediatric Surgery, University of California, San Diego Medical Center, 225 Dickinson Street, San Diego, Calif. 92103. 9 1982 by Grune & Stratton, Inc. 0022-3468/82/1706~013501.00/0 773
774
TIMOTHY G. CANTY
by the pathologist. During the early part of this series, full-thickness biopsy was used in conjunction with the suction capsule for confirmation. As experience was gained, however, the accuracy of the capsule approached 100%. In the majority of cases, clinical symptomatology and the classic picture on barium enema are highly suggestive of aganglionosis. In all cases, however, the diagnosis is definitively proven by biopsy before a plan of operative therapy is instituted. The treatment program utilized has been routinized. In 38 infants and children with markedly dilated colons, definitive diagnosis was followed by loop colostomy; the modified Duhamel procedure was then performed as a second stage. In three older children, 15 mo, 10 yr, and 12 yr of age, a one-stage modified Duhamel procedure was performed primarily without preceding colostomy. The two-stage approach utilized in the majority involves a thorough initial exploration at the time of diagnosis, with placement of the colostomy just above the frozen section biopsy-proven transition zone, usually in the left lower quadrant (Fig. 1). At the time of the second stage, the colostomy is mobilized and brought down as the Duhamel pull-through segment. The operative technique utilized for the pull-through pro-
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Fig. 1. The first stage involves placement of a doublebarrelled colostomy at or above the biopsy-proven transition zone, usually in the left lower quadrant as shown here.
Fig. 2. The second stage begins with e generous curvilinear incision beginning across the midline in the pelvis and sweeping up into the left lower quadrant to include the previous stomas; the colostomy is mobilized and discarded. The proximal colon is dissected to allow it to reach the pelvis without tension. The distal rectal stump is mobilized just below the peritoneal reflection.
cedure involves several modifications of the original Duhamel technique. The colostomy is mobilized and then removed from the end of the proximal colon to be pulled through (Fig. 2). The distal colon is circumferentially dissected for 1-2 cm below the peritoneal reflection. The dissection is then carried to the pectinate line in the posterior midline only. A clamp is pouted into the posterior anal wall just above the dentate line and, via a transanal exposure, an opening is made at this point. The previously mobilized colon is pulled through this opening and a formal anastomosis between the two bowel segments is performed (Fig. 3). Non-absorbable sutures are used. The GIA stapling device is passed from the perineum so that one blade is in the rectal stump and one in the pulled through segment, and fired, forming a side-to-side anastomosis (Fig. 4A, B). From the abdomen, the upper end of the joined segment is localized, the bowel opened, and the excess stump proximal to the end of the staple line is removed; a formal upper anastomosis is performed between the rectal
DUHAMEL PROCEDURE FOR HIRSCHSPRUNG'S DISEASE
775
A Fig. 3. Dissection behind the rectal stump is continued in the midline to the pectinate line. The proximal colon is brought down to this point, pulled-through a posterior opening in the wall of the stump and anastomosed with non-absorbable sutures.
stump and the pulled through bowel (Fig. 5). In low segment disease, the anastomosis is no longer than one application of the stapling device, or 5 cm, as advocated by Talbert, et al. ~2 For long segment disease a longer anastomosis is used, utilizing 2-5 stapler applications. Utilizing the entire colon in an ultralong side-to-side anastomosis, as advocated by Martin, has not been employed. ~3 In the three older children in which a one-stage procedure was performed, the technique was similar: frozen section biopsy is used to determine the level of ganglion cells and this level is then brought down as the pulled through segment using the formal upper and lower anastomoses and the GIA stapler as described. The average operative time for this procedure is 3 hr; blood loss is minimal. The hospital stay for either the colostomy or the Duhamel pullthrough is 4-6 days; it has never been longer than 7 days. The postoperative course is remarkably smooth, with feedings starting on the third day in most cases.
RESULTS
With a 2-7 yr follow-up, there has been no mortality and no early morbidity, including wound infections, anastomotic leaks, etc. All 41 children are currently continent and toilettrained, with normal bowel habits. There was one interesting late complication. One youngster, a female with aganglionosis to the right transverse colon, developed marked constipation and colonic dilatation 2]/2 yr after pullthrough, after having been fully toilet-trained and continent. Attempts at conservative management were
B
Fig. 4. (A) T w o sutures from the lower anastomosis are left long to assist in pulling the walls of the stump and the pulled through colon well into the " j a w s " of the stapling device, greatly assisting in accurate placement of the stapled anastomosis. (B) This shows the correct position of the stapling device. Note opening in the pulled-through colon, which will be anastomosed to the open rectal stump after the stapler is fired. T h e excess rectal stump has been discarded.
unsuccessful. A transanal posterior "internal sphincterotomy" incising the bowel wall vertically up through the previous posterior anastomosis completely relieved the problem. This child has resumed normal bowel habits and has remained well for the past 3 yr. An interesting family is included in this series, consisting of five affected individuals. The firstborn female child had long segment disease; the second-born female and third-born male had rectosigmoid disease. The father and father's sister (not part of this series) also had short segment disease, treated by Swenson pullthrough procedures 25 and 23 yr ago, respectively.
TIMOTHY G. CANTY
776
Fig. 5. Final result is a large pouch created by the formal upper and lower anastomoses and division of the septum with the GIA stapler. Note no remaining spur.
DISCUSSION
In our series no attempt was made at conservative management, utilizing rectal irrigations, etc. as proposed by Ehrenpries. 14 The blind right transverse colostomy was also not used in this series. Rather, the initial treatment in all but three cases involved locating the transition zone by frozen section biopsy and placing the colostomy at this point. The colostomy is then pulled through and excised as the second stage at or about 1 yr of age. In three patients definitive operation was performed in one stage without protective colostomy. The technique of definitive treatment used was a modification of the Duhamel procedure in all cases. The original Duhamel procedure was noted for difficulties with a retained rectal stump (spur) and the formation of large fecal impac-
tions in the blind pouch) Subsequent modifications by Martin, Soper, Ravitch, and others were combined in part for the technique used in this series. 7"t~2 As noted by Talbert, et al., the key feature of this technique involves the use of the upper and lower anastomoses and the use of one application of the G I A stapling device to completely divide the spur. 12 The caution by Dudgeon of septum reformation is of interest and can be prevented by frequent postoperative rectal exams.|5 The results in this series compare quite favorably with others using a similar procedure. 1't2:6 The absence of mortality and postoperative complications is a distinct advantage. This is most likely related to the ease of performance of this technique. Results of the Swenson and Soave procedures, though excellent in some hands, still have significant mortality and morbidity; postoperative enterocolitis is an important contributor to this) '3 There has been no postoperative enterocolitis encountered in this series. The one interesting family in this series again points to some mode of inheritance in Hirschsprung's disease in some instances. Though not clear as yet, a multifactorial form of inheritance seems most likely. Others have reported families with such complete penetrance) 7 In summary, the Duhamel procedure, with appropriate modifications to include use of upper and lower anastomoses and the G I A stapler, works quite well for all forms of Hirschsprung's disease in all ages. The technique is satisfactory as a primary procedure in older children if the colon is not markedly enlarged, or as a second stage at about 1 yr after colostomy in the newborn period. It is not necessary to use a protective colostomy (third stage) when utilizing this technique. The morbidity and mortality for this procedure should approach zero.
REFERENCES
1. Sieber WK: Hirschsprung's disease. Curr Prob Surg 15:1-93, 1978 2. Swenson O, Neuhauser EBD, Pickett LK: New concepts of etiology, diagnosis and treatment of congenital megacolon (Hirschsprung'sdisease). Pediatrics 4:201, 1949 3. Swenson O, Sherman JO, Fisher JH, et al: The treatment and postoperative complicationsof congenital megacoIon:A 25-year follow-up.Ann Surg 182:266-273, 1975 4. Boley SJ: An endorectal pull-through operation with
primary anastomosis for Hirschsprung'sdisease. Surg Gynecol Obstet 127:353-358, 1968 5. Duhamel B: Retrorectal and transanal pull-through procedure for the treatment of Hirschsprung's disease. Dis Col Rect 7:455, 1964 6. Duhamel B: A new operation for the treatment of Hirschsprung's disease. Arch Dis Child 35:28-39, 1960 7. Martin LW, Caudill DR: A method for elimination of the blind rectal pouch in the Duhamel operation for Hirschsprung's disease. Surgery62:951-953, 1967
DUHAMEL PROCEDURE FOR HIRSCHSPRUNG'S DISEASE
8. Soave F: A new surgical technique for treatment of Hirschsprung's disease. Surgery 56:1007 1013, 1964 9. Soper TR, Figueroa PR: Surgical treatment of Hirschsprung's disease: Comparison of modifications of the Duhamel and Soave operations. J Pediatr Surg 6:761-766, 1971 10. Soper TR, Miller FE: Modification of Duhamel procedure: Elimination of rectal pouch and colorectal septum. J Pediatr Surg 3:376-385, 1968 ll. Steichen FM, Talbert JL, Ravitch MM: Primary side-to-side colorectal anastomosis in the Duhamel operation for Hirschsprung's disease. Surgery 64:475-483, 1968 12. Talbert JL, Seashore JH, Ravitch MM: Evaluation of a modified Duhamel operation for correction of Hirschsprung's disease. Ann Surg 179:671-675, 1974
777
13. Martin LW: Surgical management of total colonic aganglionosis. Ann Surg 176:343-346, 1972 14. Ehrenpreis T: Hirschsprung's Disease. Chicago, Year Book, 1970 15. Dudgeon DL, Coran AG, Rosenkrantz JG: Septum reformation: A complication of the Duhamel procedure. Surgery 73:272-277, 1973 16. Grosfeld JL, Ballantine TVN, Csicsko JF: A critical evaluation of the Duhamel operation for Hirschsprung's disease. Arch Surg 113:454--460, 1978 17. Passarge E: The genetics of Hirschsprung's disease: Evidence for heterogeneous etiology and a study of sixtythree families. N Engl J Med 276:138-143, 1967
Discussion Lester Martin (Cincinnati, Ohio): Our preferred technique is very similar to that described by Dr. Canty and has been employed in approximately 75 patients. Our previous experience included at least 15 patients, each with the Swenson operation, the Soave and the original Duhamel techniques. The long-term results, when you look at them several years later, from the various procedures are so similar that my own recommendations are for the surgeon to continue doing whichever technique he is the most familiar with and feels works the best in his hands. The early postoperative complaints are about the same in our experience in all groups and we have encountered minor complaints that probably would not have appeared in Dr. Canty's figures as complications. Things such as transient episodes of diarrhea, constipation, soiling, abdominal distention, cramps, delayed training, problems which last for a few months but at the end of a year or two year's time, seem to have all corrected themselves. Even with the blind pouch of the original Duhamel operation, after several years they no longer have problems. We feel that these symptoms are more related to the severity of the disease than they are to the type of operation performed. My own preference, like that of Dr. Canty, is for our own modification of the Duhamel procedure because the long Z-shaped anastomosis seems less likely to stricture, the pelvic nerves to the bladder and ejaculatory mechanism are not disturbed, and the pelvic dissection is kept at an absolute minimum. As he mentioned, blood loss is minimal; none of the patients require blood transfusion and rarely do they require postoperative catheter drainage of
the bladder. But again I would emphasize that the choice of operation for the individual surgeon is that procedure that works the best for him. The long term results from all the procedures seem to be really quite good. Since Dr. Canty does not use a colostomy to protect his extraperitoneal pelvic suture line, I am curious if he has experienced any pelvic infections secondary to leakage of the suture line; and if not, would he tell us what he considered the most important steps in the prevention of pelvic sepsis.
Jordan Weitzman (Los Angeles, California): In the abstract of Dr. Canty's paper there is a sentence which is reported in Dr. Canty's presentation. [t states, "'All children are continent, toilet trained, and growing well." Does this mean that all of the children are perfect, and are exactly the same as a comparable group of children who were born without Hirschsprung's disease? This disease has been an interest of mine since the beginning of my career, and I have concluded along with Lester Martin and many others, that the absence of surgical complications has more to do with the skill and experience of the surgeon than with the type of pullthrough performed. I also believe that no matter how complication-free a pullthrough is, there are always a few children who have some bowel problems that require long term followup to be resolved. Dr. Canty, if the gastrointestinal function and bowel habits of your 41 patients were compared to the gastrointestinal function and bowel habits of 41 normal children, of comparable ages, would it truly be impossible to tell in every instance which children had Hirschsprung's disease and which children did not?
778
Arnold Coran (Ann Arbor, Michigan): Dr. Canty, did any of your patients have enterocolitis prepullthrough, precolostomy and were there any cases of it post-Duhamel, and would you consider that a complication? Dr. Knutrud, would you like to make any comments? You are a proponent of the Duhamel procedure? Could you also define for us what you mean by continence? Ola Knutrud (Oslo, Norway): I have used the Duhamel procedure since 1979, since my friend Bernard Duhamel presented his first paper in the BAPS meeting in Liverpool, accumulating over 200 cases. I would like to agree with Dr. Martin that one does not change a winning horse. It is obvious that the follow-up of the late results that were presented some years ago, that the results of the three methods most commonly used for this anomaly, and our long-term follow-up of these children, indicate that although some have transient incontinence or constipation, most end up doing well. Timothy Canty (closing): I appreciate the comments of the discussors. Dr. Martin, your vast experience is certainly well recognized with this disease and with this operative treatment for it. In partial answer to your and Dr. Weitzman's questions, there are differences between these children and normal children who don't have
TIMOTHY G. CANTY
Hirschsprung's disease. One of them is a scar on the abdomen. In regards to episodes of diarrhea and constipation, these children do have occasional episodes, but I've been surprised with the infrequency following the Duhamel procedure, and this series is not unique. There are many others in this audience who have similar results, especially with these modifications. I have one child, in the family that I showed you, who had some trouble with constipation two years ago, with the first year after surgery, and it went away. As Dr. Martin points out this is not uncommon within the first year, and it usually resolves. Right now these children are indeed toilet trained and continent. By continent I mean they don't lose their stool either at night while asleep or while up and around during the day. By toilet trained I mean they go to the toilet and move their bowels. In regards to enterocolitis, Dr. Coran, we have not seen it, which may be a process of selection in our patients. Perhaps we are seeing a less severe form of Hirschsprung's disease, or else getting them early enough before this occurs. We have had no enterocolitis preoperatively and no enterocolitis postoperatively. I would definitely consider this a postoperative complication if it did occur.
Hirsch-
ONTROVERSY exists as to which is the
C optimum definitive procedure for treatment of aganglionic megacolon. Though the results of Swenson with his pullthrough procedure are legend, they have been duplicated by few.~ 3 Morbidity and mortality rates that were unacceptable in the hands of others led to the development of two other commonly used procedures in addition to the Swenson pullthrough: the Soave, or endorectal, procedure and its various modifications, and the Duhamel pullthrough and its modifications. ~'4-~2 All three of these procedures in use today successfully treat Hirschsprung's disease. Each has its own set of risks, complications, and advocates. The original Duhamel procedure was noted for problems with distention of the remaining rectal stump in spite of a variety of methods used to minimize the spur between the Stump and the pulled through colon. Martin in 1967, followed by Soper and Miller, modified the original Duham e l procedure to include both a formal upper and lower anastomosis, thereby completely eliminating the "blind pouch" problem, and used a crushing clamp to divide the common walls of the two bowel segments.7'~~ In 1968 this was then further modified and greatly simplified by Steichen and Ravitch and others by the introducJournal of Pediatric Surgery, Vol. 17, No. 6 (December), i 982
tion of the GIA stapling device. T M Combining these various modifications, the Duhamel procedure avoids the problems of stump impaction, pelvic dissection, and neuromuscular injury; moreover, it provides very satisfactory long-term results in regards to continence and constipation. This paper will report the results using these modifications of the Duhamel procedure in 41 consecutive infants and children with biopsyproven Hirschsprung's disease. CLINICAL MATERIAL Over the past 7 yr a modified Duhamel procedure has been used as the primary definitive treatment of Hirschsprung's disease in 41 consecutive infants and children. The series includes 30 males and 11 females. Twenty-eight patients presented symptoms leading to evaluation and diagnosis in the newborn period. Ten patients were diagnosed in the first year of life, and one at 15 mo of age. There were two older patients undiagnosed until ages 10 and 12 yr, On detailed questioning of the families of all 41 patients, all had difficulties with bowel habits and constipation since birth. Aganglionosis was localized to the low rectosigmoid in 38 patients. One male child had aganglionosis extending to the hepatic flexure and two children, one male and one female, had total colonic involvement. Diagnosis in all three children with long-segment disease was delayed. The diagnostic approach to Hirschsprung's disease in this group was as follows: after suggestive history and plain abdominal radiographs, a barium enema followed by suction rectal biopsy are performed. If these studies are not conclusive, full-thickness rectal biopsy is performed. Diagnostic accuracy utilizing barium enema approached 70% and increased to about 80% with suction biopsy. With fullthickness biopsy~ the diagnositc accuracy was 100%. The use of the suction biopsy capsule (Quinton Instruments, Seattle Wash.), which provides a small specimen containing mucosa and submucosa only, definitely involves a period of learning
From the Department of Surgery, Division of Pediatric Surgery, University of California, San Diego Medical Center, San Diego, Calif. Presented before the Thirteenth Annual Meeting of the American Pediatric Surgical Association, Phoenix, Arizona, May 29-June 1, 1982. Address reprint requests to Timothy G. Canty, M.D., Department of Surgery, Division of Pediatric Surgery, University of California, San Diego Medical Center, 225 Dickinson Street, San Diego, Calif. 92103. 9 1982 by Grune & Stratton, Inc. 0022-3468/82/1706~013501.00/0 773
774
TIMOTHY G. CANTY
by the pathologist. During the early part of this series, full-thickness biopsy was used in conjunction with the suction capsule for confirmation. As experience was gained, however, the accuracy of the capsule approached 100%. In the majority of cases, clinical symptomatology and the classic picture on barium enema are highly suggestive of aganglionosis. In all cases, however, the diagnosis is definitively proven by biopsy before a plan of operative therapy is instituted. The treatment program utilized has been routinized. In 38 infants and children with markedly dilated colons, definitive diagnosis was followed by loop colostomy; the modified Duhamel procedure was then performed as a second stage. In three older children, 15 mo, 10 yr, and 12 yr of age, a one-stage modified Duhamel procedure was performed primarily without preceding colostomy. The two-stage approach utilized in the majority involves a thorough initial exploration at the time of diagnosis, with placement of the colostomy just above the frozen section biopsy-proven transition zone, usually in the left lower quadrant (Fig. 1). At the time of the second stage, the colostomy is mobilized and brought down as the Duhamel pull-through segment. The operative technique utilized for the pull-through pro-
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7....,.... .*~
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.............-:..-...-.r.... ,.,; t" - . .j,.- ~ ../j ~ .-'. 9 \...
"r.:"
Fig. 1. The first stage involves placement of a doublebarrelled colostomy at or above the biopsy-proven transition zone, usually in the left lower quadrant as shown here.
Fig. 2. The second stage begins with e generous curvilinear incision beginning across the midline in the pelvis and sweeping up into the left lower quadrant to include the previous stomas; the colostomy is mobilized and discarded. The proximal colon is dissected to allow it to reach the pelvis without tension. The distal rectal stump is mobilized just below the peritoneal reflection.
cedure involves several modifications of the original Duhamel technique. The colostomy is mobilized and then removed from the end of the proximal colon to be pulled through (Fig. 2). The distal colon is circumferentially dissected for 1-2 cm below the peritoneal reflection. The dissection is then carried to the pectinate line in the posterior midline only. A clamp is pouted into the posterior anal wall just above the dentate line and, via a transanal exposure, an opening is made at this point. The previously mobilized colon is pulled through this opening and a formal anastomosis between the two bowel segments is performed (Fig. 3). Non-absorbable sutures are used. The GIA stapling device is passed from the perineum so that one blade is in the rectal stump and one in the pulled through segment, and fired, forming a side-to-side anastomosis (Fig. 4A, B). From the abdomen, the upper end of the joined segment is localized, the bowel opened, and the excess stump proximal to the end of the staple line is removed; a formal upper anastomosis is performed between the rectal
DUHAMEL PROCEDURE FOR HIRSCHSPRUNG'S DISEASE
775
A Fig. 3. Dissection behind the rectal stump is continued in the midline to the pectinate line. The proximal colon is brought down to this point, pulled-through a posterior opening in the wall of the stump and anastomosed with non-absorbable sutures.
stump and the pulled through bowel (Fig. 5). In low segment disease, the anastomosis is no longer than one application of the stapling device, or 5 cm, as advocated by Talbert, et al. ~2 For long segment disease a longer anastomosis is used, utilizing 2-5 stapler applications. Utilizing the entire colon in an ultralong side-to-side anastomosis, as advocated by Martin, has not been employed. ~3 In the three older children in which a one-stage procedure was performed, the technique was similar: frozen section biopsy is used to determine the level of ganglion cells and this level is then brought down as the pulled through segment using the formal upper and lower anastomoses and the GIA stapler as described. The average operative time for this procedure is 3 hr; blood loss is minimal. The hospital stay for either the colostomy or the Duhamel pullthrough is 4-6 days; it has never been longer than 7 days. The postoperative course is remarkably smooth, with feedings starting on the third day in most cases.
RESULTS
With a 2-7 yr follow-up, there has been no mortality and no early morbidity, including wound infections, anastomotic leaks, etc. All 41 children are currently continent and toilettrained, with normal bowel habits. There was one interesting late complication. One youngster, a female with aganglionosis to the right transverse colon, developed marked constipation and colonic dilatation 2]/2 yr after pullthrough, after having been fully toilet-trained and continent. Attempts at conservative management were
B
Fig. 4. (A) T w o sutures from the lower anastomosis are left long to assist in pulling the walls of the stump and the pulled through colon well into the " j a w s " of the stapling device, greatly assisting in accurate placement of the stapled anastomosis. (B) This shows the correct position of the stapling device. Note opening in the pulled-through colon, which will be anastomosed to the open rectal stump after the stapler is fired. T h e excess rectal stump has been discarded.
unsuccessful. A transanal posterior "internal sphincterotomy" incising the bowel wall vertically up through the previous posterior anastomosis completely relieved the problem. This child has resumed normal bowel habits and has remained well for the past 3 yr. An interesting family is included in this series, consisting of five affected individuals. The firstborn female child had long segment disease; the second-born female and third-born male had rectosigmoid disease. The father and father's sister (not part of this series) also had short segment disease, treated by Swenson pullthrough procedures 25 and 23 yr ago, respectively.
TIMOTHY G. CANTY
776
Fig. 5. Final result is a large pouch created by the formal upper and lower anastomoses and division of the septum with the GIA stapler. Note no remaining spur.
DISCUSSION
In our series no attempt was made at conservative management, utilizing rectal irrigations, etc. as proposed by Ehrenpries. 14 The blind right transverse colostomy was also not used in this series. Rather, the initial treatment in all but three cases involved locating the transition zone by frozen section biopsy and placing the colostomy at this point. The colostomy is then pulled through and excised as the second stage at or about 1 yr of age. In three patients definitive operation was performed in one stage without protective colostomy. The technique of definitive treatment used was a modification of the Duhamel procedure in all cases. The original Duhamel procedure was noted for difficulties with a retained rectal stump (spur) and the formation of large fecal impac-
tions in the blind pouch) Subsequent modifications by Martin, Soper, Ravitch, and others were combined in part for the technique used in this series. 7"t~2 As noted by Talbert, et al., the key feature of this technique involves the use of the upper and lower anastomoses and the use of one application of the G I A stapling device to completely divide the spur. 12 The caution by Dudgeon of septum reformation is of interest and can be prevented by frequent postoperative rectal exams.|5 The results in this series compare quite favorably with others using a similar procedure. 1't2:6 The absence of mortality and postoperative complications is a distinct advantage. This is most likely related to the ease of performance of this technique. Results of the Swenson and Soave procedures, though excellent in some hands, still have significant mortality and morbidity; postoperative enterocolitis is an important contributor to this) '3 There has been no postoperative enterocolitis encountered in this series. The one interesting family in this series again points to some mode of inheritance in Hirschsprung's disease in some instances. Though not clear as yet, a multifactorial form of inheritance seems most likely. Others have reported families with such complete penetrance) 7 In summary, the Duhamel procedure, with appropriate modifications to include use of upper and lower anastomoses and the G I A stapler, works quite well for all forms of Hirschsprung's disease in all ages. The technique is satisfactory as a primary procedure in older children if the colon is not markedly enlarged, or as a second stage at about 1 yr after colostomy in the newborn period. It is not necessary to use a protective colostomy (third stage) when utilizing this technique. The morbidity and mortality for this procedure should approach zero.
REFERENCES
1. Sieber WK: Hirschsprung's disease. Curr Prob Surg 15:1-93, 1978 2. Swenson O, Neuhauser EBD, Pickett LK: New concepts of etiology, diagnosis and treatment of congenital megacolon (Hirschsprung'sdisease). Pediatrics 4:201, 1949 3. Swenson O, Sherman JO, Fisher JH, et al: The treatment and postoperative complicationsof congenital megacoIon:A 25-year follow-up.Ann Surg 182:266-273, 1975 4. Boley SJ: An endorectal pull-through operation with
primary anastomosis for Hirschsprung'sdisease. Surg Gynecol Obstet 127:353-358, 1968 5. Duhamel B: Retrorectal and transanal pull-through procedure for the treatment of Hirschsprung's disease. Dis Col Rect 7:455, 1964 6. Duhamel B: A new operation for the treatment of Hirschsprung's disease. Arch Dis Child 35:28-39, 1960 7. Martin LW, Caudill DR: A method for elimination of the blind rectal pouch in the Duhamel operation for Hirschsprung's disease. Surgery62:951-953, 1967
DUHAMEL PROCEDURE FOR HIRSCHSPRUNG'S DISEASE
8. Soave F: A new surgical technique for treatment of Hirschsprung's disease. Surgery 56:1007 1013, 1964 9. Soper TR, Figueroa PR: Surgical treatment of Hirschsprung's disease: Comparison of modifications of the Duhamel and Soave operations. J Pediatr Surg 6:761-766, 1971 10. Soper TR, Miller FE: Modification of Duhamel procedure: Elimination of rectal pouch and colorectal septum. J Pediatr Surg 3:376-385, 1968 ll. Steichen FM, Talbert JL, Ravitch MM: Primary side-to-side colorectal anastomosis in the Duhamel operation for Hirschsprung's disease. Surgery 64:475-483, 1968 12. Talbert JL, Seashore JH, Ravitch MM: Evaluation of a modified Duhamel operation for correction of Hirschsprung's disease. Ann Surg 179:671-675, 1974
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13. Martin LW: Surgical management of total colonic aganglionosis. Ann Surg 176:343-346, 1972 14. Ehrenpreis T: Hirschsprung's Disease. Chicago, Year Book, 1970 15. Dudgeon DL, Coran AG, Rosenkrantz JG: Septum reformation: A complication of the Duhamel procedure. Surgery 73:272-277, 1973 16. Grosfeld JL, Ballantine TVN, Csicsko JF: A critical evaluation of the Duhamel operation for Hirschsprung's disease. Arch Surg 113:454--460, 1978 17. Passarge E: The genetics of Hirschsprung's disease: Evidence for heterogeneous etiology and a study of sixtythree families. N Engl J Med 276:138-143, 1967
Discussion Lester Martin (Cincinnati, Ohio): Our preferred technique is very similar to that described by Dr. Canty and has been employed in approximately 75 patients. Our previous experience included at least 15 patients, each with the Swenson operation, the Soave and the original Duhamel techniques. The long-term results, when you look at them several years later, from the various procedures are so similar that my own recommendations are for the surgeon to continue doing whichever technique he is the most familiar with and feels works the best in his hands. The early postoperative complaints are about the same in our experience in all groups and we have encountered minor complaints that probably would not have appeared in Dr. Canty's figures as complications. Things such as transient episodes of diarrhea, constipation, soiling, abdominal distention, cramps, delayed training, problems which last for a few months but at the end of a year or two year's time, seem to have all corrected themselves. Even with the blind pouch of the original Duhamel operation, after several years they no longer have problems. We feel that these symptoms are more related to the severity of the disease than they are to the type of operation performed. My own preference, like that of Dr. Canty, is for our own modification of the Duhamel procedure because the long Z-shaped anastomosis seems less likely to stricture, the pelvic nerves to the bladder and ejaculatory mechanism are not disturbed, and the pelvic dissection is kept at an absolute minimum. As he mentioned, blood loss is minimal; none of the patients require blood transfusion and rarely do they require postoperative catheter drainage of
the bladder. But again I would emphasize that the choice of operation for the individual surgeon is that procedure that works the best for him. The long term results from all the procedures seem to be really quite good. Since Dr. Canty does not use a colostomy to protect his extraperitoneal pelvic suture line, I am curious if he has experienced any pelvic infections secondary to leakage of the suture line; and if not, would he tell us what he considered the most important steps in the prevention of pelvic sepsis.
Jordan Weitzman (Los Angeles, California): In the abstract of Dr. Canty's paper there is a sentence which is reported in Dr. Canty's presentation. [t states, "'All children are continent, toilet trained, and growing well." Does this mean that all of the children are perfect, and are exactly the same as a comparable group of children who were born without Hirschsprung's disease? This disease has been an interest of mine since the beginning of my career, and I have concluded along with Lester Martin and many others, that the absence of surgical complications has more to do with the skill and experience of the surgeon than with the type of pullthrough performed. I also believe that no matter how complication-free a pullthrough is, there are always a few children who have some bowel problems that require long term followup to be resolved. Dr. Canty, if the gastrointestinal function and bowel habits of your 41 patients were compared to the gastrointestinal function and bowel habits of 41 normal children, of comparable ages, would it truly be impossible to tell in every instance which children had Hirschsprung's disease and which children did not?
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Arnold Coran (Ann Arbor, Michigan): Dr. Canty, did any of your patients have enterocolitis prepullthrough, precolostomy and were there any cases of it post-Duhamel, and would you consider that a complication? Dr. Knutrud, would you like to make any comments? You are a proponent of the Duhamel procedure? Could you also define for us what you mean by continence? Ola Knutrud (Oslo, Norway): I have used the Duhamel procedure since 1979, since my friend Bernard Duhamel presented his first paper in the BAPS meeting in Liverpool, accumulating over 200 cases. I would like to agree with Dr. Martin that one does not change a winning horse. It is obvious that the follow-up of the late results that were presented some years ago, that the results of the three methods most commonly used for this anomaly, and our long-term follow-up of these children, indicate that although some have transient incontinence or constipation, most end up doing well. Timothy Canty (closing): I appreciate the comments of the discussors. Dr. Martin, your vast experience is certainly well recognized with this disease and with this operative treatment for it. In partial answer to your and Dr. Weitzman's questions, there are differences between these children and normal children who don't have
TIMOTHY G. CANTY
Hirschsprung's disease. One of them is a scar on the abdomen. In regards to episodes of diarrhea and constipation, these children do have occasional episodes, but I've been surprised with the infrequency following the Duhamel procedure, and this series is not unique. There are many others in this audience who have similar results, especially with these modifications. I have one child, in the family that I showed you, who had some trouble with constipation two years ago, with the first year after surgery, and it went away. As Dr. Martin points out this is not uncommon within the first year, and it usually resolves. Right now these children are indeed toilet trained and continent. By continent I mean they don't lose their stool either at night while asleep or while up and around during the day. By toilet trained I mean they go to the toilet and move their bowels. In regards to enterocolitis, Dr. Coran, we have not seen it, which may be a process of selection in our patients. Perhaps we are seeing a less severe form of Hirschsprung's disease, or else getting them early enough before this occurs. We have had no enterocolitis preoperatively and no enterocolitis postoperatively. I would definitely consider this a postoperative complication if it did occur.