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PEDIATRICS Vol. 109 No. 5 May 2002, pp. 914-918

REVIEW ARTICLE

Hirschsprung’s Disease: A Review

Orvar Swenson, MD

From Charleston, South Carolina


   INTRODUCTION
TOP
 INTRODUCTION
DIAGNOSIS
 OPERATIVE TREATMENT
 OPERATIVE MORTALITY
 OPERATIVE COMPLICATIONS
 OUTCOMES OF OPERATIVE TREATMENT...
 DISCUSSION
 REFERENCES
 
Because it is now more than 50 years since both the discovery of the cause and a treatment for Hirschsprung’s disease, it seems appropriate to assess outcomes for those unfortunate children who have this previously lethal disease. In 1948, the discovery of the cause of this disease was based on a series of clinical observations indicating that there was a defective segment of distal colon producing a partial bowel obstruction.1 Peristaltic tracings of the dilated proximal colon recorded progressive contractions; however, this peristaltic wave did not enter the more distal narrow segment. This was suggestive evidence of a physiologic defect in that distal segment. Therefore, removing this segment would prove the validity of these observations. An operative technique was devised and tested in the laboratory, and when it proved feasible, a patient’s distal narrow segment was removed and his intestinal continuity was restored with a pull-down and reanastomosis. Soon thereafter, the patient’s gastrointestinal system began to function normally. We concluded that this operation provided experimental evidence that proved our assumptions were correct.2


   DIAGNOSIS
TOP
 INTRODUCTION
 DIAGNOSIS
OPERATIVE TREATMENT
 OPERATIVE MORTALITY
 OPERATIVE COMPLICATIONS
 OUTCOMES OF OPERATIVE TREATMENT...
 DISCUSSION
 REFERENCES
 
It is possible to suspect the presence of Hirschsprung’s disease in the newborn by clinical criteria if the infant does not pass meconium in the first 48 hours or by the presence of vomiting and abdominal distention.3 In the older child, clinical criteria for diagnosis includes a history of severe constipation from birth, failure to thrive, abdominal distension and often an empty rectal ampulla on rectal examination. The disease occurs about once in 5000 live births.4 The incidence of this disease being transmitted to offspring is about 3%. In specific instances in females where the length of the aganglionic lesion is extensive, the percentage increases.4

As part of our investigation into the cause of this disease, a radiographic enema using a small amount of barium outlined an irregular and narrowed small colon beginning in the anal canal and extending proximally before it fanned out and connected with the more proximal dilated colon.5 Roentgenograms of this portion of the colon in this disease had never been previously published and became the definitive test to establish the diagnosis of congenital megacolon. Delayed clearance of barium from the colon after the enema was additional evidence suggesting Hirschsprung’s disease. It soon became apparent that this test was less sensitive for detecting short lesions, total colon aganglionosis, and the disease in the newborn. Therefore, studies were performed using the surgically removed distal segment. Strips of tissue were cut from the full length of the specimen extending down to the anal canal, and, for the first time, the whole segment was found to be devoid of Auerbach’s plexus.1 In 1957, this led us to recommend rectal biopsy as the most reliable test to establish the diagnosis even for those groups with a conflicting barium enema radiograph.6 This procedure required a general anesthetic and was likely to leave scarring, complicating the definitive operation. Mucosal biopsy and the application of histochemical staining eliminated these problems and such specimens were often obtained at the bedside by a suction cup or forceps.7 In a review of 1340 mucosal biopsies, there were 3 cases of bleeding requiring transfusion and 3 clinical perforations, 1 resulting in death.8 One caution in performing rectal biopsy for the diagnosis of Hirschsprung’s disease is the recognition that there is normally a 2-cm band in the distal rectum at the level of the internal anal sphincter devoid of ganglion cells.9


   OPERATIVE TREATMENT
TOP
 INTRODUCTION
 DIAGNOSIS
 OPERATIVE TREATMENT
OPERATIVE MORTALITY
 OPERATIVE COMPLICATIONS
 OUTCOMES OF OPERATIVE TREATMENT...
 DISCUSSION
 REFERENCES
 
The original operation (the Swenson procedure) consisted of freeing the defective distal colon from within the pelvis by careful sharp extrarectal dissection down to 2 cm above the anal canal and performing an end-to-end anastomosis. Applying this technique, the defective aganglionic tissue is completely removed and the proximal ganglionated colon and anal canal are left in a normal anatomic position.2

In 1960, Duhamel10 published a modification of the original operation. He resected a portion of the distal defective segment as in the original operation and made a side-to-side anastomosis between the proximal normal colon and defective rectum. In 1963, Soave11 published another modification. It consisted of making a circumferential cut through the muscular coat of the colon at the pelvic peritoneal reflection. Working in an intramural plane, the mucosa is separated from the muscular coat down to the anal canal. The freed mucosa is excised and the proximal normal colon is pulled through this retained viable spastic aganglionic muscular sleeve. A telescoping type of anastomosis is made that adds an extra layer of colon wall at the anastomosis. Marks12 reported cutting the sleeve posteriorly, a practice now widely used. Boley13 further modified the method by making an extrapelvic anastomosis between the anal canal mucosa and the mucosa of the normal colon. Rehbein and von Zimmermann14 used an anterior approach to partially remove the defective aganglionic tissue leaving a considerable segment of aganglionic tissue in place. Some surgeons using this technique perform sphincterotomy as a routine part of the operation.15

During the past decade, there has been a trend to operate on infants in the first weeks of life without a preliminary colostomy. Carccassonne was a leader in introducing this change, and he reported results on 98 infants treated in this manner in whom all the defective tissue was removed (M. Carcassonne, personal communication). All the patients were under 3 months of age and there were no deaths. One anastomotic leak closed promptly when a colostomy was instituted (M. Carcassonne, personal communication). This strategy may prove to be an important change because it merges the period of incontinence with the normal period of training.

Contemporary laparoscopic surgery may also fit well with resecting patients with Hirschsprung’s disease, especially recognizing that the excised tissue can be removed through the anal canal. Wulkan and Georgeson16 have reported an experience with this technique; however, they leave the defective aganglionic muscular sleeve in place and follow with a prolonged program of daily rectal dilatations. Curran and Raffensperger,17 along with Arany et al,18 have demonstrated that it is easy to remove all the defective aganglionic tissue with laparoscopic technique with no need for daily rectal dilatations postoperatively. Still another new technique achieves the entire resection, leveling of ganglion cells, and definitive pull-through via the anal canal without an abdominal incision.19 Small series have been reported with reasonable complication rates.20

About 4% to 5% of patients with congenital megacolon have no ganglion cells in the entire colon.21 These smaller reported series have mortality rates after surgical treatment ranging from 13% to 23.5%.22 Martin23 suggested a technique that saves a portion of the defective colon, uniting it with the terminal ileum with a side-to-side anastomosis, thus providing additional surface for fluid absorption. Criticisms of this modified Duhamel technique have been reported because of poor emptying of the retained aganglionic colon.24,25 Better results in treating this entity have been reported by Sherman et al.26 Nineteen patients were treated with excision of the entire aganglionic colon with end-to-end anastomosis of the terminal ileum to the anal canal with no deaths. These patients were started on oral feedings shortly after operation; however, they went through a difficult postoperative period with frequent stools which at times produced perineal excoriation.27 With perseverance they all gained stool control. The first patient treated in this manner graduated from college, married, had several children and was doing well until 40 years after his operation when he became anemic with blood in his stool.28 At sigmoidoscopy the ileal mucosa was reddened. An ileostomy was performed and 6 months later he was no longer anemic, and his stool was negative for blood. A second patient went through the same experience. Some surgeons have modified this procedure by constructing a pouch to serve as a stool reservoir.29 A rare case of a colon transplant has been reported with a short follow-up.30


   OPERATIVE MORTALITY
TOP
 INTRODUCTION
 DIAGNOSIS
 OPERATIVE TREATMENT
 OPERATIVE MORTALITY
OPERATIVE COMPLICATIONS
 OUTCOMES OF OPERATIVE TREATMENT...
 DISCUSSION
 REFERENCES
 
The overall mortality rate of Hirschsprung’s disease in 880 cases treated with the original Swenson operation was 2.5%; however, in the last 20 years the mortality has fallen to 1.25%.26 Soave31 in his own series of 271 patients reported a 4.5% mortality rate. In a recent paper reporting 260 patients treated with the Duhamel modification, the mortality rate was 6.2%.32 Carcassonne et al,33 Weizman et al,34 Shandhogue and Bianchi,35 Waldron and O’Donnell36 and Madonna et al37 in their papers describing their experience with the original operation reported no deaths.


   OPERATIVE COMPLICATIONS
TOP
 INTRODUCTION
 DIAGNOSIS
 OPERATIVE TREATMENT
 OPERATIVE MORTALITY
 OPERATIVE COMPLICATIONS
OUTCOMES OF OPERATIVE TREATMENT...
 DISCUSSION
 REFERENCES
 
It is difficult to make a precise comparison of complications between the original Swenson operation and the numerous modifications. For instance, Sherman et al26 reported a careful statistical analysis of 880 cases from seven children’s centers. A tissue diagnosis was obtained on all cases and all were treated with the original operation. There were no problems with urinary or sexual function. For comparison, there is a recent report of 2400 cases from multiple centers treated with the Duhamel modification and subjected to a 30-year follow-up.38 In a considerable proportion of the cases, there was no tissue diagnosis. The data were collected by questionnaire and treatment consisted of 8 different modifications of the Duhamel procedure. No data were reported on sexual function.

One of the most serious complications of a Hirschsprung’s disease pull-through is a leak at the anastomosis. In the Sherman series, 26 there was a 5.6% leak rate. Soave31 states that in his personal series there were no leaks. However, in the collection of 365 patients treated with his technique, the leak rate was 6.1%.39 In the survey on Hirschsprung’s disease of the members of the Surgical Section of the American Academy of Pediatrics, the leak rate after the Soave Boley modification was 6.9%.40 Harrison et al41 reported the leak rate of patients treated by the Duhamel and Soave modifications to be 7%.

Other additional complications characterize the modified procedures and they do not occur after the original Swenson procedure. The Duhamel modification is reported to have a 10% rectal pouch problem consisting of impactions and at times bleeding.42 Grosfeld et al43 reported an 8% rate and Baillie et al44 reported a 25% rate of pouch problems. Martin and Torres45 claim to have eliminated this problem with their series of modifications. In their review, Bourdelat et al38 list 9 modifications of the Duhamel technique. There also are reports of abscess formation between the retained aganglionic muscular sleeve, retraction of the pulled through normal colon, and mucosal prolapse after the Soave modification, complications that can occur years after the operation.46

Fortuna et al47 has reported secondary reoperation rates in patients treated by the Duhamel to be 29% and after the Soave to be 26%. The postoperative occurrence of enterocolitis was 19% to 20%. Sherman et al26 reported that in 880 Swenson procedure cases the secondary reoperation rate was 6%. This stemmed from an anastomotic leak rate of 5.6%, which required diverting colostomies, and, in rare cases, the need to surgically excise strictures. In a recent report on 97 patients treated with the original operation, the secondary operation rate was 6%.35 Throughout the 40-year study of the original operation, the postoperative rate of enterocolitis was 11%.26

There are several reports that some patients had such poor results from their pull-through that complete reoperation was required.48–50 A small number of these were for stricture or retained aganglionic tissue, the remainder were for recurrent enterocolitis, constipation, or soiling. The entire reoperative group had either a primary Duhamel or Soave modification. In a large series of Duhamel procedures, 4.9% of patients had such poor results that reoperations were required.38 Furthermore, Tariq et al51 reported such unrelenting postoperative constipation in 3.7% of their patients after treatment with the Soave modification that reoperations were required. Sherman et al,26 Carcassonne et al,33 Weizman et al,34 Shandhogue and Bianchi,35 Waldron and O’Donnell36 and Madonna et al37 all using the original Swenson operation have not encountered problems that required reoperation.

It is generally believed that all postoperative Hirschsprung’s disease patients, regardless of the original operative procedure, improved with time. This is challenged by Postuma and Corkery,52 who observed that this was true for a period of time, but that those treated by the modifications subsequently had an increase in troubles. Mishalany et al53 and Heij et al54 have expressed the same concern.


   OUTCOMES OF OPERATIVE TREATMENT FOR HIRSCHSPRUNG’S DISEASE
TOP
 INTRODUCTION
 DIAGNOSIS
 OPERATIVE TREATMENT
 OPERATIVE MORTALITY
 OPERATIVE COMPLICATIONS
 OUTCOMES OF OPERATIVE TREATMENT...
DISCUSSION
 REFERENCES
 
There have been concerns about how end results are evaluated. The commonly used method is to list results as excellent, good, fair, and poor. This method involves judgment and in collected series, the evaluation is made by the operating surgeon to whom parents are reluctant to report problems. In an attempt to enhance objectivity, the results can be reported by the normalcy of bowel function. Duthrie55 has estimated the normal pattern of bowel function is one movement or more each 3 days. Drossman et al56 studied 800 normal adults with no gastrointestinal complaints and determined a normal range was at least 1 movement every three days and no more than 3 movements per day. Sherman et al26 avoided these criticisms because he was the Swenson procedure surgeon in only a small number of the total patients he interviewed about bowel function and continence. Using the normal pattern of bowel function to determine outcomes, he found that 96% of the patients fell into the normal pattern and were continent. In an attempt to determine the quality of life the patients experienced, he asked each patient if they considered their bowel habits normal. Affirmative answers were received from 94%. Rescorla in his report on 260 patients treated with the Duhamel modification adhered to the same standards. Only 103 patients were available for long-term follow-up and the outcomes in 67% were very satisfactory, 27% required occasional enemas or stool softeners and 8% had constipation or soiling.32 Heij et al54 reported the results on 75 patients treated with the Duhamel modification. Ten of the 49 patients >4 years old were continent without constipation, 22 had soiling and/or constipation, and 17 were incontinent.54 In his review of collected cases treated with his modification, Soave31 reported 83% as good and 17% as unsatisfactory. Quinn et al57 investigated the long-term incidence of constipation after operation. The incidence was 54% after the Duhamel, 43% after the Soave, and 4% after the original Swenson operation.


   DISCUSSION
TOP
 INTRODUCTION
 DIAGNOSIS
 OPERATIVE TREATMENT
 OPERATIVE MORTALITY
 OPERATIVE COMPLICATIONS
 OUTCOMES OF OPERATIVE TREATMENT...
 DISCUSSION
REFERENCES
 
The last 50 years have seen the successful application of operative therapy to the management of Hirschsprung’s disease, but controversy continues over the issue of what is the best operation. The major deficit after the Duhamel modification is constipation and poor emptying. Martin and Altemeier58 insist that the rectum should be left in place so that it can perform its normal function. It is difficult to comprehend how an aganglionic rectum can function normally. It is true that with a series of modifications, the troublesome pouch problem may have been eliminated. However, half of the rectum and some of the rectosigmoid wall are aganglionic tissue with limited peristaltic contraction. Additional evidence that when half the circumference of intestine is made up of aganglionic tissue there is functional disruption and stasis is suggested by the results of the Martin procedure used in patients with total colon agangliosis.24,25

Another concern expressed about operative procedures for Hirschsprung’s disease is data on sexual function. In the review of 2400 patients treated with the Duhamel modification, no data on this subject are included.38 In their report on the Duhamel operation, Rescorla et al32 do not mention sexual function. Polley et al59 stated that the advantage of the Soave modification over the Swenson operation was that the rectal intramural dissection ensured that no damage would be done to the pelvic neural structures that might result in urinary and fecal incontinence and possibly damage sexual function. The report of Sherman et al26 demonstrated that these hypothetical fears are groundless and that all the defective tissue can be removed during extrarectal dissection without these postulated damages. In contrast, Sherman et al26 state that no defect in urinary or sexual function occurred in his review of a large number of adult postoperative patients treated with the Swenson operation. In 1 report of patients treated with the Swenson operation, 80 patients were married and collectively there were 146 children.60

The Soave modification is complicated by recurrent enterocolitis and constipation. Holschneider et al61 found in his large review that the Soave modification had the highest incidence of postoperative enterocolitis followed by the Duhamel. The least incidence occurred after the Swenson operation.60,61 Kimura et al62 found persistent rectal achalasia in postoperative patients treated with the Soave modification and subjected them to posterior rectal myectomy. They believe that the cause of this was the telescoping type of anastomosis associated with this Soave technique, which incorporates an extra layer into the anal canal. Abbas Banani and Forootan63 reported a series of 37 patients who underwent a Soave modification and 6 had signs and symptoms similar to their preoperative state. Conservative therapy did not improve their condition. On examination, they had high anal canal pressures. After anorectal myectomy, there was a reduction in anal canal pressure and improvement in their symptoms. Blair et al64 reported on 10 postoperative patients who were not relieved of their symptoms. They had been treated with both the Soave modification and the Duhamel modification. All were treated with posterior internal sphincterotomies. It is important to note that Wulkan and Georgeson’s patients treated with a telescoping anastomosis all required daily rectal dilatations for prolonged periods, and Soave dilated all his patients postoperatively.16,31 Postuma and Corkery52 believed that the long aganglionic spastic muscular sleeve that the normal colon is pulled through limited rectal capacity and contributed to the persistent diarrhea he observed. Rescorla et al32 state that the variability in long-term results might be related to the varying amounts of aganglionic tissue left in place. Touloukian, in discussing these papers, wonders if consideration should be given to going back to the concept of removing all the defective tissue.

The concept that removing the aganglionic rectum obliterates a reflex that causes relaxation of the sphincter as Varma and Stephens65 reported is seriously questioned. A series of reports has concluded that the sensation of rectal fullness is mediated from sensory endings in the levator and puborectalis sling rather than the rectum.66–68 Adults treated by the original operation with removal of the aganglionic rectum insist that they can detect rectal fullness.

The Duhamel and Soave modifications are less attractive than the original Swenson operation because of the greater numbers of complications and the poorer long-term outcomes. Surgeons who use these modifications are meticulous, resorting to frozen sections to determine that at the oral end all of the aganglionic colon is completely removed; yet at the pelvic end they are content to leave varying amounts of aganglionic tissue in place. Sherman et al26 have reported outcomes that can be achieved for Hirschsprung’s disease patients when treated with the Swenson operation in a children’s center staffed with experienced pediatric surgeons backed by pediatric pathologists, radiologists, and pediatricians. It is important to realize that the surgical procedure is only the first step in curing these patients; and there is a variable period for the patient and parent to be guided to overcome enterocolitis and to gain fecal control. To ensure optimal operative outcomes, ease of operative performance of the operative technique is secondary. Rather, the selection of the course of treatment should be based on proven long-term results and outcomes.


   FOOTNOTES
 
Received for publication May 11, 2001; Accepted Jan 14, 2002.

Reprint requests to (O.S.) 1 Gadsen Way, Apartment 322, Charleston, SC 29412-3574


   REFERENCES
TOP
 INTRODUCTION
 DIAGNOSIS
 OPERATIVE TREATMENT
 OPERATIVE MORTALITY
 OPERATIVE COMPLICATIONS
 OUTCOMES OF OPERATIVE TREATMENT...
 DISCUSSION
 REFERENCES
 

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PEDIATRICS (ISSN 1098-4275). ©2002 by the American Academy of Pediatrics

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