Hirschsprung’s disease (HD) is caused by the failure of ganglion cells to migrate cephalocaudally through the neural crest during weeks 4–12 of gestation 1. The disease occurs in one of 5000 births 2. Traditionally, surgical therapy for HD has consisted of a proximal defunctioning colostomy, followed months later by a definitive reconstructive pull-through procedure in which the aganglionic colon is resected and the normally innervated bowel is brought down and sutured to the area just above the anal sphincter 3. Over the past decade, the surgical management of HD has evolved. The previous gold standard three-staged procedure with a preliminary stoma was replaced by a two-staged procedure; one-stage pull-through is now advocated in many centers worldwide, with results as favorable as multistage procedures 4.
Swenson, Duhamel, and Soave (or endorectal pull-through) procedures were the most commonly performed operations for HD in North America until 1998 3, when transanal endorectal pull-through was first described 5–7.
Although there are many publications comparing different surgical procedures for the treatment of HD, little is known about parents’ satisfaction and the quality of life of children after different operations.
The aim of this study is to compare the surgical outcome as well as parents’ satisfaction and quality of life for children after the transanal and the Duhamel pull-through operations in a single-center experience.
Patients and methods
The preferred surgical procedure for HD was the two-stage Duhamel pull-through at the pediatric surgical department, Al Wasl Hospital (Dubai) until June 2002. The transanal endorectal pull-through has been performed in Al Wasl Hospital since that date to date.
A retrospective file review was carried out of all cases of HD treated surgically in our institution from June 1998 until June 2010.
Patients were classified into two groups according to the type of surgical procedure they underwent: group 1 (transanal endorectal pull-through) and group 2 (Duhamel pull-through).
Three questionnaires were used: one designed to evaluate bowel function for children 3 years of age or younger (*) and the other for children older than 3 years of age (**), the Wingspread scoring system 8 (3 years is the age at which the majority of children will have achieved day and night anal continence). The third questionnaire (***) measured the quality of life for patients as well as parents’/patients’ satisfaction and for the surgical management and outcome. Patients as well as parents were interviewed or contacted by phone to answer the questionnaire by an independent witness (social worker).
Demographic data, disease presentation, associated congenital anomalies, family history of HD, age at surgery, age at interview, type and stages of surgery, length of aganglionic segment, early postoperative complications (within 30 days), late postoperative complications, total number of enterocolitis attacks, stooling patterns (frequency of bowel motion and need for laxative and/or enemas), presence or absence of failure to thrive (according to growth charts that correlate height, weight, and age), need for redo surgery, length of follow-up were collected, tabulated, and analyzed statistically.
Sixty-nine patients were included in the study, 41 in group 1 and 28 in group 2. Figure 1 shows the sex distribution in both groups.
The median age at presentation was 20 days in group 1 and 16 days in group 2. The median age at surgery was 3 months in group 1 and 6 months in group 2. The median age at interview was 30 months in group 1 and 78 months in group 2.
The follow-up period ranged from 6 months to 4 years in group 1, mean 1.7±1.1 years, and from 1 to 8 years, mean 3.6±2.2 years, in group 2.
In group 1, 16.8% of patients had congenital anomalies in the form of neurological impairment (2.4%), cardiovascular, combined Down syndrome and heart anomalies, and Down syndrome (4.8% each).
In group 2, 17.9% of patients had congenital anomalies (10.7% of them had neurological impairment) (Fig. 2).
In group 1, 9.8% of patients had a positive family history of HD, whereas in group 2, only 7.1% of patients had a family history of HD (Fig. 3).
The most common presentation in group 1 was delayed passage of meconium and chronic constipation (61 and 22%, respectively). In group 2, the most common presentation was delayed passage of meconium and enterocollitis with distension (53.6 and 21.4%, respectively) (Fig. 4).
The most common segment for HD was the rectosigmoid segment in groups 1 and 2 (95.1 and 89.3%, respectively) (Fig. 5).
Early postoperative complications
There was a significant difference between the two groups in the occurrence of early postoperative complications. In group 1, 34% of patients developed early postoperative complications in the form of excoriations and soiling (29.2 and 4.8%, respectively), whereas in group 2, only 10.7% of patients developed early complications in the form of excoriation (χ2=4.92, P<0.05).
Late postoperative complications
There was no significant difference between the groups in the late postoperative complications (χ2=0.337, P>0.05). In group 1, 53.7% of patients developed late complications, most commonly excoriations and stricture (27 and 9.8%, respectively), whereas in group 2, 60.7% of patients developed late complications, most commonly constipation and excoriation (32.1 and 14.3%, respectively) (Table 1).
There was no significant difference between the groups in the occurrence of preoperative enterocolitis. Table 2 shows that 14.6 versus 14.3% of patients in groups 1 and 2, respectively, had preoperative enterocolitis.
No patients in group 1 developed postoperative enterocolitis versus 32.1% of patients in group 2; there was a significant difference (χ2=8.3, P<0.05) (Table 3).
All the patients in group 2 required colostomy, whereas only 14.6% of patients in group 1 required colostomy (χ2=48.5, P<0.05).
Frequency of bowel motion
Analysis of the frequency of bowel motion at the time of the questionnaire interview showed no significance difference between the groups. In group 1, 53.8% of patients had bowel movements two to three times/day compared with 53.6% of patients in group 2 (Table 4).
Failure to thrive
4.8% of patients versus 28.6% of patients in groups 1 and 2, respectively, showed postoperative failure to thrive that was statistically significant (χ2=7.5, P<0.05).
Only one patient in group 1 (2.4%) required a redo operation compared with five patients (17.9%) in group 2, with no statistically significant difference.
Table 5 shows that there was a significant difference in the use of anticonstipation medications between both the groups (χ2=7.9, P<0.05).
Results of questionnaire interview
Thirty-six patients (88%) in group 1 versus 22 patients (78.5%) in group 2 could be reached for the questionnaire interview.
The anorectal function was rated using the Wingspread scoring system for patients older than 3 years of age.
In patients older than 3 years of age, there was a significant difference between the scores of both groups. In group 1, 20% of patients had an excellent function compared with 4.8% of patients in group 2 (t=2.8, P<0.05) (Table 6).
In patients 3 years of age or younger, in group 1, 65% of patients had good bowel function (score 0–6), 19% of patients had fair bowel function (score 7–12), and 16% of patients had poor bowel function (score 13–17).
In group 2, only one patient had fair function.
In terms of parents’ satisfaction, there was a significant difference between the two groups. In group 1, 69% of the parents were satisfied compared with 27.3% in group 2 (χ2=8.4, P<0.05) (Table 7).
Postoperative results in the surgical management of HD appear to be satisfactory. Despite this good overall outcome reported, many studies have shown a higher than anticipated incidence of problems after surgery for HD 9.
Quality of life remains a difficult concept to assess and is influenced by the physical, psychological, spiritual, functional, and social well-being of an individual. The discrepancies in the quality of life reported following surgical correction of HD may be attributed to the study design, the details of the investigations carried out, or the lack of an objective independent observer 9.
Recently, transendorectal pullthrough (TERPT) has become the most popular procedure for the treatment of HD, but overstretching of the internal anal sphincter remains a critical issue, which may impact the long-term continence outcome. Because TERPT is a relatively new procedure, there is only one report, to our knowledge, that compares the long-term outcome of TERPT with the conventional transabdominal pull-through 10.
Our current study is the first (to our knowledge) to compare TERPT with Duhamel pull-through in terms of the rates of complication as well as quality of life and parents’ satisfaction.
In our study, the most common length of aganglionic segments in both groups was rectosigmoid (95.1% of patients vs. 89.3% of patients in groups 1 and 2, respectively).
Although there were three cases of total colonic aganglionosis in group 2, early postoperative excoriation was higher in group 1 (29.2% of patients vs. 10.7% of patients in groups 1 and 2, respectively); overall, two cases had persistent soiling (a 2-year-old boy with down syndrome and a 5-year-old neurologically normal boy) in group 1. Langer et al. 3 have reported an incidence of 11% of early postoperative excoriation in his largest multicenter series for transanal pull-through.
Excoriation was still the most common late postoperative complications in group 1 (27%) followed by stricture (9.8%) and constipation (7.3%). The Langer et al.3 series reported a 4% incidence of postoperative stricture. The four cases of strictures developed in our early experience with transanal pull-through, but after we adopted a protocol of postoperative dilatation for 3 months, Moreno cases of strictures were found.
In group 2, constipation was the most common late postoperative complication (32.1%), followed by excoriation (14.3%). El-Sawaf et al.’s 10 study also reported a higher incidence of constipation in the abdominal than the transanal pull-through group (38.1 vs. 20%, respectively). Moore et al.9 reported constipation in 26% of patients versus 9% of patients who underwent Duhamel versus transabdominal Soave procedures, respectively.
There were two cases of adhesive bowel obstruction in group 2 that required surgical release. The incidences of incontinence and overflow incontinence were almost similar in both the groups.
Although there was almost the same percentage of patients who had preoperative enterocolitis in both the groups, there was a statistically significant difference in the incidence of postoperative enterocolitis (nil in group 1 vs. 32.1% in group 2). El-Sawaf et al.10 reported an incidence of postoperative enterocolitis of 45 versus 61.9% in the transanal versus abdominal pull-through groups. Both Langer et al.3 and Moore et al.9 studies reported a 6% incidence of postoperative enterocolitis for transanal pullthrough and abdominal pullthrough respectively.
Although we prescribed oral metronidazole for all patients in group 1 postoperatively for 1 month, it is still not clear whether this led to a decrease in the incidence of postoperative enterocolitis in the patients in group 1. Another possible explanation may be under-reporting of cases of enterocolitis in our hospital as parents seek medical advice in other hospitals.
In terms of bowel movement, 53.8 of patients in group 1 versus 53.6% of patients and in group 2, respectively, had two to three normal bowel movements per day at the time of the questionnaire interview. Langer et al.3 reported that 80.5% of children had normal bowel function after undergoing transanal pull-through as reported by their parents or care givers. EL Sawaf et al.10 study revealed similar continence outcomes in transanal and abdominal pullthrough although the postoperative period was longer in transanal group.
In our study, there was a higher incidence of constipation in group 2 (25%) than in group 1 (4.8%); however, 36.6% of patients in group 1 versus 10.7% of patients in group 2 had bowel movements four times or more per day at the time of the questionnaire interview.
There were statistically significant differences in the incidence of failure to thrive between groups 1 and 2, respectively (4.8 vs. 28.6%) during the follow-up period in our study. Thirty-nine percent of the patients in Moore et al.’s 9 study were over 50th percentile in terms of weight for age (WA); most of the patients whose WA was below the 3rd percentile were in the younger age group. Moore et al. 9 concluded that normal WA regained with time after surgical correction of HD.
One patient (2.4%), a 6-year-old female in group 1, required redo surgery for persistent anastomotic stricture; in group 2, five patients (17.9%) required redo surgery in the form of division of persistent pouch spur. This difference between both groups was statistically significant. A total of six patients (14.6%) required redo surgery in the El-Sawaf et al.10 study (five for abdominal pull-through and one for transanal pull-through); their results indicated significantly poorer long-term outcome in the redo group. Follow-up of our redo cases indicated that only two patients in group 2 still had persistent constipation, although they had no residual spur and no residual aganglionic segment in the pull-through segment.
In terms of the need for laxatives and/or enemas, 27% of patients in group 1 versus 60.7% of patients in group 2 still require medications for the management of their constipation; the results were statistically significant. The Langer et al.3 study reported that 9.3% of patients still require medications to manage their constipation. The El-Sawaf et al.10 study showed that 19.5% of patients still require medications to manage their constipation; Moore et al.9 reported that 13% of patients of the study groups still require medications to manage their constipation.
Quality of life and patient/parent satisfaction discussion
In patients older than 3 years of age, there was a statistically significant better bowel function in group 1 than in group 2 using the Wingspread scoring system. Although the same results were obtained in patients 3 years of age or younger, it was not statistically significant.
The total stooling score in the El-Sawaf et al.10 study was higher in the transanal pull-through group (12.75±8.07) in comparison with that of the abdominal pull-through group (11.28±7.75), although the results were not statistically significant.
Analysis of the degree of parent’s satisfaction/quality of life in our study indicated that there was statistically significant more parent satisfaction in group 1 versus group 2. The Bai et al.11 study of quality of life after the Swenson procedure showed that 40% of patients had good quality of life, 46.7% had fair quality of life, and 13.3% had poor quality of life. In the Bai et al.11 study, there was a strong association between fecal continence and the quality of life in patients.
Heij et al.12 used a questionnaire on anorectal function and quality of life after Duhamel pull-through surgery; they concluded that the majority of patients have impaired anorectal function after Duhamel’s operation and that there was no indication that this impairment improved with time. In the Moore et al.9 study, the majority of patients (94%) appeared to be well-adjusted members of the society.
Our experience with transanal pull-through showed lower incidence of postoperative enterocolitis, failure to thrive, redo surgery, and need for anticonstipation medications than that with Duhamel pull-through.
Although the anorectal scoring system showed better results in transanal pull-through than Duhamel pull-through in all age groups, it was statistically significant in patients older than 3 years of age.
There was a statistically significant better parent satisfaction and quality of life in the transanal group than the Duhamel pull-through group.
Conflicts of interest
There are no conflicts of interest.
1. Parisi MA, Kapur RP. Genetics of Hirschsprung disease. Curr Opin Pediatr. 2000;12:610–617
2. Amiel J, Lyonnet S. Hirschsprung disease, associated syndromes, and genetics: a review. J Med Genet. 2001;38:729–739
3. Langer JC, Durrant AC, De La Torre L, Teitelbaum DH, Minkes RK, Caty MG, et al. One-stage transanal Soave pullthrough for Hirschsprung disease: a multicenter experience with 141 children. Ann Surg. 2003;238:569–576
4. Hackam DJ, Superina RA, Pearl RH. Single-stage repair of Hirschsprung’s disease: a comparison of 109 patients over 5 years. J Pediatr Surg. 1997;32:1028–1032
5. De La Torre-Mondragón L, Ortega-Salgado JA. Transanal endorectal pull-through for Hirschsprung’s disease. J Pediatr Surg. 1998;33:1283–1286
6. Langer JC, Minkes RK, Mazziotti MV, Skinner MA, Winthrop AL. Transanal one-stage Soave procedure for infants with Hirschsprung’s disease. J Pediatr Surg. 1999;34:148–152
7. Coran AG, Teitelbaum DH. Recent advances in the management of Hirschsprung’s disease. Am J Surg. 2000;180:382–387
8. Stephens FD, Smith ED. Classification, identification, and assessment of surgical treatment of anorectal anomalies. Pediatr Surg Int. 1986;1:200–205
9. Moore SW, Albertyn R, Cywes S. Clinical outcome and long-term quality of life after surgical correction of Hirschsprung’s disease. J Pediatr Surg. 1996;31:1496–1502
10. El-Sawaf MI, Drongowski RA, Chamberlain JN, Coran AG, Teitelbaum DH. Are the long-term results of the transanal pull-through equal to those of the transabdominal pull-through? A comparison of the 2 approaches for Hirschsprung disease. J Pediatr Surg. 2007;42:41–47
11. Bai Y, Chen H, Hao J, Huang Y, Wang W. Long-term outcome and quality of life after the Swenson procedure for Hirschsprung’s disease. J Pediatr Surg. 2002;37:639–642
12. Heij HA, De Vries X, Bremer I, Ekkelkamp S, Vos A. Long-term anorectal function after Duhamel operation for Hirschsprung’s disease. J Pediatr Surg. 1995;30:430–432