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Quality of life and parents’ satisfaction for Duhamel’s versus transanal endorectal pull-through for treatment of Hirschsprung’s disease in children

Mustafawi AR. MD*, Mohamed E Hassan. MD**
*Pediatric surgery department, Al Wasl Hospital, Dubai, UAE
** Consultant pediatric surgery, Al Qassemi Hospital, Sharjah, UAE

 

Correspondence

Mohamed E Hassan, MD
P.O.Box 441103, Dubai, UAE
Tel: 00971502459456
Fax: 00971042887008
Email : This email address is being protected from spambots. You need JavaScript enabled to view it.

 

Abstract

Background: The aim of this study is to compare surgical outcome as well as parents’ satisfaction and quality of life for children after the transanal and the Duhamel pull through operations in single centre experience.

Materials and methods: A retrospective cohort file review of all cases of HD treated surgically in our institution was conducted. Patients were classified into, group 1 (transanal endorectal pull through), group 2 (Duhamel pull through). Three questionnaires designed. Demographic data, perioperative data, complications and length of follow up data were statistically analyzed.

Results: 69 patients were included. Medications were required postoperatively to 27 % in group 1 versus 60.7% in group 2. In group 1, 22.5% were fairly satisfied and 69% were satisfied, while in group 2, 31.8% of parents were poorly satisfied, 40.9% were fairly satisfied and 27.3% were satisfied. No patient older than 3 years had poor results in group 1 versus 33.3% in group 2.

Conclusions: Our experience with transanal pull through showed less incidence for postoperative enterocolitis, failure to thrive, redo surgery and need for anti-constipating medications than Duhamel pull through. Although anorectal scoring system showed better results in transanal pull through than Duhamel pull through in all age groups, it was statistically significant in age above 3 years. There was statistically significant better parents’ satisfaction and quality of life in transanal group than Duhamel pull through.

Key words: Hirschsprung’s disease, parents’ satisfaction, quality of life, children.

 

Introduction

Hirschsprung’s disease (HD) is caused by the failure of ganglion cells to migrate cephalocaudlly through the neural crest during week four to 12 of gestation [1]. The disease occurs in one out of 5,000 births [2]. Traditionally, surgical therapy for Hirschsprung’s disease 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, an evolution in the surgical management of HD has occurred. The previous gold standard three staged procedures with a preliminary stoma was replaced by two staged procedure. Recently one stage pull-through is advocated now 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 till 1998 [3], when transanal endorectal pull through (TERPT) was first described [5, 6, 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 surgical outcome as well as parents’ satisfaction and quality of life for children after the transanal and the Duhamel pull through operations in single centre experience.

Materials and methods

The preferred surgical procedure for HD was two stages Duhamel pull through in pediatric surgical department, al. Wasl Hospital, Dubai, till June 2002. The transanal endorectal pull through was started in our hospital since that date till now.

A retrospective file review of all cases of HD treated surgically in our institution from June 1998 till June 2010 was done. Patients were classified into 2 groups according to the type of surgical procedure. Group (1) -transanal endorectal pull through, group (2) - Duhamel pull through.

Three questionnaires were used, one designed to evaluate bowel function for children 3 years old or less (*), and the other for children older than 3 years (**), 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 independent witness (social worker).

Questionnaire 1.
(*) 3 Years of Age or younger bowel function estimation questionnaire

No

Question

Answer

Pointsscore

 

 

1

 

 

 

Stoolfrequencysixmonths postoperatively

 

Constipated(Notpassingevery 3daysspontaneously  /

veryhardstool).Morethan6timesdaily NormalFrequency

Alwayshisanusisstainedwithstool

(1) (1)

(0)

(2)

 

2

 

Stoolconsistency

Soft Fluid Hard

(0) (1) (1)

 

 

3

 

 

Frequencyofperianalexcoria- tion

 

Alwayspresent

Once/2-4weeksLessthanonceamonth None

 

(3) (2) (1) (0)

 

 

4

 

 

Useoforallaxatives

 

VeryFrequent(Every12days) Frequent(Every3-7days) Infrequent(Every8th  dayormore) None

 

(3) (2) (1) (0)

 

 

5

 

 

Frequencyofusing enemas

 

VeryFrequent(Every12days) Frequent(Every3-7days Infrequent(Every8th  dayormore) None

 

(3) (2) (1) (0)

 

6

 

Frequencyofabdominaldisten- tion+diarrhea+fever(entero- colitis)postoperatively

Alwayspresent

Once/2-4weeksLessthanonceamonth None

 

(3) (2) (1) (0)

 

 

7

 

 

Bowelhabitsofchild

 

Normal

Acceptable

Bad(ConstipatedalwaysorIncontinent)

 

(0) (1) (2)


Questionnaire2.

(**)theWingspreadscoringsystemforchildrenolderthan3yearsofage.

 

No

Question

Pointsscore

 

1

Totallycontinent  orveryoccasionallystressrelatedstainingof underclothes withoutconstipation.

Toilettrainedwithnomedication.

 

Excellent/VeryGood

 

2

 

Rarelysoils exceptduringstressfulexercise. Constipationmanagedwithmedication.

 

Good

 

3

 

Intermittentsoiling,urgeincontinence.

Frequentloosestoolsor constipationwhichrequiresenemas.

 

Fair

 

4

 

Constantfecalsoilingandsmearing. Constipationonlyresponsetoenemas.

 

Poor


 

Questionnaire3.

(***) Parentssatisfaction/Qualityoflife

 

No

Question

Answer

Pointsscore

 

 

1

 

Explanationaboutprocedureandcomplica- tionsbeforesurgery

 

YesNo Partially

 

(2) (0) (1)

 

2

 

 

Postoperativecourseofthechildinthe hospital

 

VeryGood Good Fair

Bad

 

(3) (2) (1) (0)

 

 

3

 

Postoperativecourseofthechildafter discharge

VeryGood Good Fair

Bad

(3) (2) (1) (0)

 

4

 

Satisfactionwiththeresultofthesurgery

 

VeryGood Good Fair

Bad

 

(3) (2) (1) (0)

 

5

 

Recommendingthissurgeryto childsfriend ifhehadsamedisease

Yes

No

 

(1) (0)

 

 

6

 

 

Postoperativebowelhabits

 

VeryGood Good Fair

Bad

 

(3) (2) (1) (0)

 

7

 

Mentaldevelopment

 

VeryGood Good Fair

Bad

 

(3) (2) (1) (0)

 

 

8

 

 

Physicaldevelopment

VeryGood Good Fair

Bad

(3) (2) (1) (0)

 

 

9

 

 

Usingbowelwashorlaxatives

 

Regular Irregular None

 

(2) (1) (0)

 

10

Feelingthatthemanagementwasproperly done

Yes

No

(1) (0)

 

 

11

AbsenteeismfromschoolforHDrelated problem

 

None Infrequently Frequently

 

(0) (1) (2)

 

 

12

 

 

FrequencyofhospitalvisitsforHDrelated complaint

Nomorefollowup Onceamonth Once/3month Once/6month Onceayear

(0) (1) (2) (3) (4)

 

 

13

 

 

Childrelationshipwithhispeers

VeryGood Good Fair

Bad

(3) (2) (1) (0)


 

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 of laxative and/or enemas), presence or absence of failure to thrive ( according to growth charts that correlates height, weight and age), need for redo surgery, length of follow up were collected, tabulated and statistically analyzed .

Results

Sixty-nine patients were included in the study, 41 in group (1) versus 28 in group (2). Figure 1 shows the gender distribution in both groups. The median age at presentation in group (1) was 20 days while in group2 it was 16 days.

The median age at surgery in group1 was 3 months while in group (2) was 6 months. The median age at interview in group1 was 30 months while in group (2) it was 78 months.

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Figure 1: Gender distribution in both groups

Follow up period ranged from 6 months to 4 years in group (1) with a mean of 1.7 ± 1.1 years while in group (2) the follow up period ranged from 1-8 years with a mean of 3.6 ± 2.2 years. In group (1) 16.8% 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% had congenital anomalies (10.7% of them were neurologically impaired), (Figure 2). In group (1) 9.8% had positive family history of HD while 7.1% in group (2) only had family history (Figure 3). The most common presentation in group (1) was delayed passage of meconium and chronic constipation (61%, 22% respectively). In group (2) the most common presentation was delayed passage of meconium and enterocololitis with distension (53.6%, 21.4% respectively), (figure 4).

JPSS iunie 2013 Page 10 Image 0002

Figure 2: Associated congenital anomalies in both groups

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Figure 3: Family history for Hirschsprung’s disease in both groups

JPSS iunie 2013 Page 12 Image 0002

Figure 4: Clinical presentations in both groups

The most common segment for Hirscsprung’s disease was the recto sigmoid segment in group (1) and 2 (95.1% and 89.3% respectively), (Figure 5).

JPSS iunie 2013 Page 12 Image 0003

Figure 5: Affected segment of Hirschsprung’s disease in both groups

Early post-operative complications:

There was a significant difference between the two groups in occurrence of early post-operative complications. In group (1) 34% of patients suffered from early post-operative complications in the form of excoriations and soiling (29.2%, 4.8% respectively), while in group (2) only 10.7% suffered from early complications in the form of excoriation. (X2=4.92, P < .05)

Late post-operative complications:

There was no significant difference between the groups regarding the late post-operative complications (X2=0.337, P > .05). In group (1) 53.7% suffered from late complications most commonly excoriations and stricture (27%, 9.8% respectively), while in group (2) 60.7% suffered from late complications most commonly constipation and excoriation (32.1%, 14.3% respectively), ( Table 1).

Table I: Late postoperative complications for both groups

Late complications

 

Group

 

 

No

 

 

Excoriations

 

 

Constipation

 

 

Stricture

 

 

Soiling

 

Overflow incontinence

 

Adhesive obstructions

 

 

Total

 

Group(1)

 

19 (46.3%)

 

11 (27%)

 

3 (7.3%)

 

4 (9.8%)

 

2 (4.8%)

 

2 (4.8%)

 

0

 

41 (100%)

 

Group(2)

 

11 (39.3%)

 

4 (14.3%)

 

9 (32.1%)

 

0

 

1 (3.6%)

 

1 (3.6%)

 

2 (7.1%)

 

28 (100%)

Pre-operative enterocolitis:

There was no significant difference between the groups regarding the pre-operative enterocolitis. Table 2 shows that 14.6% versus 14.3% in group (1) and (2) respectively suffered from pre-operative enterocolitis.

Table II : Frequency of preoperative enterocolitis in both groups

Preoperative

enterocolitis

Group

 

No

 

Yes

 

Total

 

X2,p

 

Group(1)

35 (85.4%)

6 (14.6%)

41 (100%)

 

 

X2=0.002

P>0.05

 

Group(2)

24 (85.7%)

4 (14.3%)

28 (100%)

Postoperative enterocolitis:

No patients in group (1) developed postoperative enterocolitis versus 32.1% in group 2. There was a significant difference (X2=8.3, P< 0.05), (Table 3).

Table III : Frequency of postoperative enterocolitis in both groups

Postoperative enterocolitis

Group

 

 

No

 

 

Yes

 

 

x2,P

 

Group(1)

41 (100%)

 

0

 

x2=15.2

 

P<0.05

 

Group(2)

19 (67.9%)

9 (32.1%)

Colostomy:

All the patients in Group (2) required Colostomy, while only 14.6% in group (1) needed colostomy ( X2=48.5, P <0.05 ).

Frequency of bowel motion:

Analyzing the frequency of bowel motion at the time of questionnaire interview revealed no significance difference between the groups. In group (1) 53.8% had bowel movements 2-3 times /day compared to 53.6% in group (2), (Table 4).

Table IV: Frequency of bowel motion in both groups at time of last follow up visit before conducting the study

Bowelmovement

Group

4timesormore/

day

 

2-3/day

 

Once/3 days

Onceeverymore than4days

 

Total

 

Group(1)

15 (36.6%)

22 (53.8%)

2 (4.8%)

2 (4.8%)

41 (100%)

 

Group(2)

3 (10.7%)

15 (53.6%)

3 (10.7%)

7 (25%)

28 (100%)

Failure to thrive:

4.8% versus 28.6% in group (1) and (2) respectively showed postoperative failure to thrive that was statistically significant (x2=7.5, P <0.05).

Re-do operation:

Only 1 patient in Group 1 (2.4%) required a re-do operation compared to 5 patients (17.9%) in Group 2 with no statistical significant difference.

Anti constipation medications:

Table 5 shows that there was a significant difference in the use of anti constipation medications between both groups (x2=7.9, P< 0.05).

Table V: The need for laxatives and or enema in both groups

Medications

Group

 

 

No

 

 

Yes

 

 

x2,P

 

Group(1)

30 (73%)

11 (27%)

 

x2=7.9

 

P<0.05

 

Group(2)

11 (39.3%)

17 (60.7%)

 

Results of Questionnaires interview:

36 patients (88%) in group (1) versus 22 patients (78.5%) in group (2) could be reached for questionnaire interview. The anorectal function was rated using the Wingspread Scoring System for patients older than 3 years. In patients more than 3 years, there was a significant difference between the scores of both groups. In group (1) 20% had an excellent function compared to 4.8% in group (2) (t=2.8, P < 0.05), ( Table 6).

Table VI: Bowel functions in children above 3 years old in both groups using Wingspread scoring system

 

 

Bowel Function >3 yrs
Group

 

Poor

 Fair

 

Good

      Very good/ Excellent

 

Number of patients above 3 years interviewed

t, P

 

Group(1)

0

2
(40%)

2 (40%)

1 (20%)

5

   t=2,8

   P< 0,05

 

Group(2)

9
(42.9%)

10
(47.6%)

1
(4.8%)

1
(4.8%)

21

 

 

In patients 3 years old or less, in group (1) 65% had good bowel function (score 0-6), 19% had fair (score 7-12) and16% had poor (score 13-17). In group (2), there was only 1 patient with fair function.

Regarding the parents’ satisfaction there was a significant difference between the two groups. In group (1) 69% of the parents were satisfied compared to 27.3% in group2 (x2=8.4, P l< 0.05), (Table 7).

Table VII : Parents satisfaction Quality of life in both groups

 

Parents’

satisfaction

Group

 

 

Poorlysatisfied

 

 

Fairlysatisfied

 

 

Satisfied

 

 

 

x2,P

 

Group(1)

 

3 (8.5%)

 

8 (22.5%)

 

25 (69%)

 

 

x2=8.4

 

P<0.05

 

Group(2)

 

7 (31.8%)

 

9 (40.9%)

 

8 (27.3%)

 

Discussion:

Post operative results in the surgical management of HD appear to be satisfactory. Despite this reported good overall outcome, many studies show 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 physical, psychological, spiritual, functional and social well being of the individual. Possible reasons for discrepancies in the quality of life reported following surgical correction of HD may lie in study design, the depth of the investigations undertaken or the lack of an objective independent observer [9].

Recently, 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 compare long term outcome of TERPT with the conventional transabdominal pull through [10].

Our current study represents the first study (to our knowledge) that compares TERPT with Duhamel pull through from the aspects of complication rates as well as quality of life and parent’s satisfaction. In our study, the most common length of aganglionic segments in both groups was recto sigmoid (95.1% versus 89.3% in group (1) and group (2) respectively).

Although there was 3 cases of total colonic aganglionosis in group (2), early post operative excoriation was higher in group (1) (29.2% versus 10.7% in group (1) and (2) respectively), over all there were two cases suffered from persistent soiling (2 year old Down syndrome boy and 5 year old neurologically normal boy) in group1. Langer et al. [3] reported incidence of 11% of early post operative 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%), then constipation (7.3%). Langer et al.[3] series showed 4% incidence of postoperative stricture. The 4 cases of strictures developed in our early experience with transanal pull through, but after we adopted a protocol for 3 months of postoperative dilatation and we didn’t encounter any more strictures. In group (2) constipation was the most common late postoperative complications (32.1%) followed by excoriation (14.3%). El-Sawaf et al. [10] study reported also more incidence of constipation in abdominal than transanal pull through group (38.1% versus 20% respectively). Moore et al. [9] showed constipation in 26% versus 9% in Duhamel versus transabdominal Soave procedures respectively.

There were 2 cases of adhesive bowel obstruction in group (2) which required surgical release. Incidence of incontinence and overflow incontinence were nearly similar in both groups. Although there was nearly same percentage of cases in both groups who suffered preoperative enterocolitis, there was statistically significant difference in the postoperative enterocolitis incidence (nil in group (1) versus 32.1% in group (2). El-Sawaf et al. [10] reported incidence of postoperative enterocolitis 45% versus 61.9% in transanal versus abdominal pull through groups. Langer et al. [3] study showed 6% incidence of postoperative enterocolitis for transanal pull through as well as Moore et al. [9] for abdominal pull through.

Although we prescribed oral metronidazole for all patients in group (1) postoperatively for one month, we are still not sure that this is the contributing factor to decrease the number of postoperative enterocolitis in group (1) patients. Another possible explanation may be under reporting of cases of enterocolitis to our hospital, in which parents seek medical advice in other hospitals.

Regarding bowel movement, 53.8% versus 53.6%% in group (1) and (2 )respectively had 2-3 normal bowel movements per day at the time of questionnaire interview, Langer et al. [3] reported 80.5% of children post transanal pull through to have normal bowel function as reported by their parents or care giver, El-Sawaf et al. [10] study revealed data suggesting the longer the postoperative period is for transanal versus abdominal pull through , both groups will eventually achieve similar continence outcomes.

In our study there was more incidence of constipation in group (2) (25%) than in group (1) (4.8%), meanwhile, group (1) showed (36.6%) versus (10.7%) in group (2) who had bowel movements 4 times or more per day at the time of questionnaire interview.

There was statistically significant differences regarding incidence of failure to thrive between group (1) and 2 respectively (4.8% versus 28.6%) during the follow up period in our study. 39% of the Moore et al. [9] study patients were over 50th percentile regarding weight for age (WA), most of the patients whose WA was below 3rd percentile were in the younger age group. They concluded that normal WA is regained with time after surgical correction of HD. One case (2.4%) 6 years old female in group (1) required redo surgery for persistent anastomotic stricture, in group (2), 5 cases (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 6 cases (14.6%) required redo surgery in El-Sawaf et al. [10] study (5 for abdominal pull through and 1 for transanal pull through), their results showed significantly poorer long term outcome in the redo group than none redo group. Follow up of our redo cases showed only two cases in group (2) who still suffering from persistent constipation although no residual spur and no residual aganglionic segment in the pull through segment.

Regarding need of laxatives and/or enemas, 27% in group (1) versus 60.7% in group (2) still they do require to use medications for management of their constipation. The results were statistically significant. Langer et al. [3] study revealed 9.3% still they do require medications to manage their constipation. El-Sawaf et al. [10] study showed 19.5%, Moore et al. [9] 13% of the study groups still require medications to manage their constipation.

Quality of life and patients’/ parents’ 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 Wingspread Scoring system. Although the same results were obtained in patients 3 years old or younger, but it wasn’t statistically significant. The total stooling score in El-Sawaf et al. [10] study showed better results in the trans anal pull through group, (12.75± 8.07) in comparison to the abdominal pull through group (11.28 ±7.75), although the results were not statistically significant.

Analyzing the degree of parents’ satisfaction/ quality of life in our study, there was statistically significant more parents’ satisfaction in group (1) versus group (2). Bai et al. [11] study for quality of life after Swenson procedure showed 40% had good quality of life, 46.7% had fair quality of life and 13.3% had poor quality of life. In Bai et al. study there was 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 post Duhamel pull through , their study concluded that the majority of patient have impaired anorectal function after Duhamel’s operation and that there was no proof that this impairment improved with time. In Moore et al. [9] study the majority of patients (94%) appeared to be well adjusted members of the society.

Conclusion:

Our experience with transanal pull through showed less incidence for postoperative enterocolitis, failure to thrive, redo surgery and need for anti-constipating medications than Duhamel pull through. Although anorectal scoring system showed better results in transanal pull through than Duhamel pull through in all age groups, it was statistically significant in age groups above 3 years. There was statistically significant better parents’ satisfaction and quality of life in transanal group than Duhamel pull through.

 

 

 

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