Find best premium and Free Joomla templates at GetJoomlaTemplatesFree.com

Appendicostomy for Bowel Control in Children after Transanal Endorectal Pull-Through for Hirschsprung Disease

Christina Granéli, Anna Börjesson, Pernilla Stenström, Einar Arnbjörnsson

Department of Pediatric Surgery, Skåne University Hospital and Institution of Clinical research, Lund University, Lund, Sweden

 

Correspondence:

Einar Arnbjörnsson

Department of Pediatric Surgery

Skåne University Hospital and Institution of Clinical Research, Lund University

Lund, Sweden

E-mail: This email address is being protected from spambots. You need JavaScript enabled to view it.  

 

Abstract

AimTo evaluate the indications for, and outcome, of appendicostomy for patients with Hirschsprungs disease (HD) who underwent transanal endorectal pull-through (TERPT).

Method: Children with HD > 4 years old who received an appendicostomy between 2005 and 2011 at a tertiary pediatric surgery center were included. Pre- and post-appendicostomy bowel function was evaluated by a bowel function score. HD-patients not receiving an appendicostomy were the controls. The study was approved by an institutional ethics committee (2010/49).

Results: Seven of 37 HD-patients received an appendicostomy. Syndromes were present in 43% of the appendicostomy and 6% of the controls (controls reported absence of fecal accidents (p=0.297). Three (60%) with appendicostomy and 14 (47%) controls, respectively, reported absence of soiling or soiling RPT.

Keywords: Hirschsprungs disease, transanal endorectal pull through, bowel function score, antegrade continence enema, appendicostomy.

 

Introduction

Hirschsprung disease (HD) is a congenital disease in which the absence of ganglion cells in the intestinal muscular layer leads to aperistalsis of the affected bowel. A common surgical treatment used for HD is transanal endorectal pull-through (TERPT) [1]. However, absence of bowel control and fecal incontinence may follow. Furthermore, patients with Down syndrome or other disorders and HD may require additional attention to their bowel function [2-5].

Fecal incontinence, constipation, and bowel obstruction are symptoms often initially treated by dietary modification, medication, and training to instill regular toilet habits. Some patients will nonetheless be unable to achieve bowel control. The inability to control bowel movements may carry a negative social impact that can be severely limiting as the child grows older [6]. In some of these patients, rectal enema treatments could be effective for achieving improved bowel function. However, some patients do not tolerate rectal enemas or would prefer to be more autonomous, in which case antegrade colonic enemas (ACE) through an appendicostomy might be an option.

Since 1990, when appendicostomy was proposed for administering an antegrade continence enema [7], there have been several reports on its benefits and disadvantages for different diagnoses [8-12]. There are various appendicostomy techniques described [7, 11, 12-17]. Appendicostomy outcomes have been reported in children mainly with spinal bifida, intractable functional constipation, or anorectal malformation [9-11]. Reports on the long-term functional results of an appendicostomy in children with HD who have undergone TERPT are reported in mixed groups of diagnosis (REF med HD), but separate evaluation could be valuable for selecting patients with HD who might benefit from an appendicostomy. The aim of this study was to evaluate the indications and outcomes of appendicostomy for antegrade enemas in patients with HD who underwent TERPT. The secondary aim was to describe how appendicostomy is used in patients with HD and parents' satisfaction with appendicostomy.

Materials and Methods

Settings

The study was performed at a tertiary center for pediatric surgery that serves a region with 1.8 million residents and 22,000 births annually.

Patients and study setting

HD was diagnosed in all the patients by rectal biopsy and anography. The study patients included all patients with HD who underwent TERPT from 2005 to 2011 and subsequent appendicostomy until 2014. The control group included patients with HD who underwent TERPT, but not appendicostomy, from 2005 to 2011 and were aged older than 4 years at follow-up. The appendicostomy-patients’ bowel symptoms before appendicostomy were recorded retrospectively from medical charts. The patients’ guardians were asked if they would come in for follow up and would consent to an interview after the appendicostomy. In an outpatient setting, they were questioned about bowel symptoms, appendicostomy use, and satisfaction with the appendicostomy. Information on the bowel symptoms of the control patients was obtained from telephone interviews or during counseling at the outpatient clinic.

Scoring of bowel symptoms

Bowel function was assessed by a bowel function scoring system with bowel function scores (BFS’s) ranging from worse to better function: 1 to 20 [18].

Bowel management program and indications for appendicostomy

All children who undergo TERPT for HD are routinely offered an individualized bowel management program at our center. Each patient’s bowel habits and symptoms are regularly checked and scored, and a BFS is recorded. The patient and family are counseled, and recommendations for obtaining improved bowel function are provided if needed.

If the patient requires enemas but neither the family nor the patient accept the rectal approach, appendicostomy is evaluated as an alternative. Another reason for appendicostomy is patient desire for greater autonomy.

The pediatric surgeon and the colorectal nurse perform repeated evaluations prior to a final decision for performing appendicostomy. The operation is only performed after a comprehensive discussion with patients and their families, which includes possible complications and failure in bowel control despite the administration of antegrade continence enemas (ACEs) through an appendicostomy. Throughout participation in the bowel management program, the family and patient regularly receive counseling by a colorectal pediatric surgeon and with a colorectal nurse as needed. The family and patient are also routinely offered psychological consultation according to the local follow-up program.

Surgical techniques: TERPT and appendicostomy

The TERPT procedure was performed in accordance with the technique described in 1998, with rectal mucosectomy, colectomy of the aganglionic segment, and normoganglionic colon pull-through performed through the anus [1]. The length of the muscular cuff was 2–3 centimeters. Focus was put on not to damage the anal canal during the operation. Colonic resection was extended to include the transition zone, and any dilated bowel was resected, along with the aganglionic bowel. The end of the proximal bowel had to demonstrate a normal frequency of mature ganglionic cells, without any signs of nerve hypertrophy, according to the pathologists’ report on a frozen section obtained during the operation. The final pathology report included a statement on positive staining for calretinin.

At our center, a laparoscopic approach was used for appendicostomy, which was carried out by 2 experienced pediatric surgeons. The laparoscopic procedure involved open access at the umbilicus with a 5-mm 30-degree laparoscope. A 5-mm laparoscopy port was introduced at the right lateral inguinal fossa, at the point selected for the appendicostomy stoma. A grasper was introduced through the port to grab the appendix and pull it out through the port hole. The tip of the appendix was then opened, and 5-10 mm of it was removed. The wall of the appendix was sutured to the skin using a V-Y plastic with absorbable sutures. Until 2009, a Foley catheter® was left in the stoma and removed after 6 weeks. After 2009, a Chait button® was placed in the stoma and either removed after 6 weeks or left in place to avoid repeat catheterizations of the stoma in very young or active children. The Chait button® was then replaced every 6 months.

Appendicostomy – complications and use

Peri- and post-operative complications of appendicostomy were retrospectively recorded from medical records. Infection was defined as a positive culture from a specimen obtained from the stoma site in combination with clinical signs of infection. A questionnaire on the use of and satisfaction with the appendicostomy was administered during follow-up counseling sessions. The questionnaire had been used in a previous study [19] and includes questions regarding time needed to administer enemas, time until the bowel was completely empty after enema administration, type of enema used, and degree of satisfaction with the appendicostomy.

Statistical analysis

Nonparametric statistics were used, since data could be skewed because of the small number of patients. This is based on the following observations: 1. Analyzing paired data as unpaired does not increase the type 1 error for the t test. 2. The WMW test is the nonparametric equivalent of the t test. P values < 0.05 were considered significant. The Fischer exact probability test 2×4 with the Freeman-Halton extension yielded the same results and was also used. All statistical analysis was performed by a statistician.

Ethics

The regional research committee approved the study (registration number 2010/49). Approval from each patient´s guardians was obtained before including the child in the study. Intention to treat was the main diagnostic strategy used for all patients. All evaluations, treatments, and procedures described in this report were the standard of care. All data were maintained as confidential in the hospital file system and were coded in the study investigators’ computers.

Results

Indications for appendicostomy

A total of 7 children with HD who underwent TERPT subsequently received an appendicostomy. Their median age at appendicostomy was 5 (2-8) years. Other patient characteristics and details on the procedure are shown in Table 1. All 7 patients had been using rectal enemas. The rectal enemas had a satisfactory effect on individual bowel management problems, but the treatment had failed in 5 of the 7 because of the following issues: low compliance secondary to anal pain (n=3); patient intolerance to enema administration (n=4), and both parent and patient psychological intolerance to rectal enemas (n=5). Indications for appendicostomy for the other 2 patients were desire for increased autonomy and easier administration and emptying while sitting on a toilet (n=2) (Table 1).

Table 1: Characteristics of study patients with Hirschsprung disease who had undergone transanal endorectal pull-through (TERPT) and received an appendicostomy. Median (range)

 

Group (n = 7)

Gender

Male, 4 (57%)

Female, 3 (43%)

Birth weight, g

3460 (2355-2965)

Comorbidity

Down syndrome, 2 (29%)

Translocation syndrome, 1 (14%)

 

 

Age at TERPT, days

86 (16-262)

Length of bowel removed by TERPT, cm

27 (20-35)

Age at appendicostomy, years

5 (2 - 7)

Follow-up time after appendicostomy, months

112 (8-46)

Age at follow-up, years

5 (4 – 8)

Time between TERPT and appendicostomy, years

4 (1 - 6)


Complications associated with TERPT and appendicostomy

There were no recorded intraoperative complications during either the TERPT or the appendicostomy procedures. A laparoscopic appendicostomy was converted to open surgery for 1 patient because of intra-abdominal adhesions. Post operative no additional pull-through interventions were required, although 1 patient developed a rectourethral fistula and therefore underwent additional surgery.

A total of 6 postoperative complications after appendicostomy occurred in 4(57%) patients and included granuloma (n=1), local infection (n=2), and irritation at the site of the stoma (n=3). No pain at the site of the appendicostomy was reported. All infections were treated by oral antibiotics and/or sterile washing, and no additional surgical interventions were needed because of infection. No one still using the appendicostomy at the follow-up reported localized pain associated with the stoma.

Outcome and use of the appendicostomy

At the time of follow-up, 2 of the 7 children no longer used their appendicostomy. Both had received colostomy at 8 and 10 months respectively after the appendicostomy procedure. The reasons for colostomy were development of a recto urethral fistula in the one patient who needed a reoperation, and the other child, who also had Down syndrome, had colostomy because of severe obstructive symptoms and a painful anal stricture. The five patients still using their appendicostomy used it daily as follows: 2 children used it in the morning and evening, and 3 other children used it in the evening only. Four children used a Chait button because they preferred to avoid catheterization. The other used intermittent catheterization. All of the parents would recommend appendicostomy to other families in the same situation (Table 2).

Two children were still using diapers, 1 because of late maturity and urinary incontinence due to Down syndrome and the other because of a rare chromosomal translocation syndrome which led to concomitant urinary outlet symptoms.

Table 2: Guardians’ answers to questionnaire on the use of the appendicostomy. The guardians to the 5 patients who were still using their appendicostomy completed the questionnaire.

Questionnaires

Answer

Median (range)

Number

Total volume of enema used at each treatment (ml)

 

500 (120-700)

5

Type of enema

Saline (0.9%)

Sorbitol

 

 

4

1

Time needed to administer enema (min)

 

5 (5-10)

 

Time needed to finish a bowel movement (min)

 

40 (15-60)

 

Frequency of appendicostomy use

Once a day

Twice a day

Every other day

 

2

2

1

Would recommend appendicostomy to others in the same situation

Yes

No

 

5

0

Type of catheter

Chait

Intermittent catheterization

 

4

1


None of the children with appendicostomy had been treated with botulinum toxin A injections before the procedure, because the treatment had not yet been introduced in our department at that time. Three children, all with some syndromes, had obstructive symptoms that were treated by botulinum toxin A injections after the appendicostomy. Two of them were successfully treated by the combination of ACE through the appendicostomy plus botulinum toxin A, while the third child needed a colostomy.

Bowel function scores before and after appendicostomy

The pre- and postoperative BFS of the children who received an appendicostomy are shown in Table 3, which shows that the bowel scores were significantly increased for the patients who still used their appendicostomy.

Table 3: Pre- and postoperative bowel symptoms after an appendicostomy

Evaluation of bowel control

Score

Appendicostomy

P-value*

 

 

Before

After

 

 

 

N =7

N =5**

 

Ability to hold back defecation

 

 

 

 

 

0.001

-          Always

3

0

5

-          Problems <1/week

2

0

0

-          Weekly problems

1

0

0

-          No voluntary control

0

7

0

Feels/reports the urge to defecate

 

 

 

 

 

0.028

-          Always

3

0

2

-          Most of the time

2

0

2

-          Uncertain

1

0

1

-          Absent

0

7

0

Frequency of defecation

 

 

 

 

 

0.001

-          Every other day to twice a day

2

0

5

-          More often

1

5

0

-          Less often

1

2

0

Soiling

 

 

 

 

 

0.005

-          Never

3

0

1

-          Staining <1/week, no change of underwear required

2

0

3

-          Frequent staining, change of underwear often required

1

2

1

-          Daily soiling, requires protective aids

0

5

0

Faecal accidents

 

 

 

 

 

0.001

-          Never

3

1

5

-          Fewer 1/week

2

0

0

-          Weekly, requires protective aids

1

0

0

-          Daily, requires protective aids day and night

0

6

0

Constipation***

 

 

 

 

 

0.005

-          No constipation

3

0

4

-          Manageable with diet

2

0

0

-          Manageable with laxatives

1

2

1

-          Manageable with enemas

0

5

0

Social problems

 

 

 

 

 

0.001

-          No social problems

3

0

4

-          Sometimes

2

0

0

-          Problems restricting social life

1

0

1

-          Severe social/psychosocial problems

0

7

0


*Fisher Exact Probability Test 2 × 4 or 2 × 3 with Freeman-Halton extension (unpaired exact Wilcoxon-Mann-Whitney test)

**2 patients stopped using their appendicostomy

*** Feeling of urgency, capacity to verbalize, and holding bowel movements


Control patients without appendicostomy

During the study period a total of 32 patients with HD were operated on with TERPT but not appendicostomy. After the exclusion of 2 patients who had migrated 30 children without appendicostomy were included in the study as controls. Their median age at the time of the study was 5.5 (4-9) years. There were no intra- or postoperative complications associated with TERPT. At follow-up, 5 patients used rectal enemas regularly because of obstructive symptoms and 2 of them also fecal incontinence. Two of these 5 patients also received botulinum toxin A injections.

Comparison of BFS between appendicostomy patients and controls

The BFS of each symptom of the children needing appendicostomy were significantly lower before appendicostomy than the BFS of the control patients (Table 4). At follow up after appendicostomy; there was no difference between the BFS of the 5 children with appendicostomy and the BFS of the control patients. Some of the BFS outcomes of the children with appendicostomy were better (Table 5).

Table 4: Bowel function scores of children with Hirschsprung disease before appendicostomy compared with controls.

Evaluation of bowel control

Score

Before appendicostomy

Controls

P-value*

 

 

 

 

 

 

 

N =7

N=30

 

Ability to hold back defecation

 

 

 

 

 

<0.001

-          Always

3

0

7

-          Problems <1/week

2

0

15

-          Weekly problems

1

0

7

-          No voluntary control

0

7

1

Feels/reports the urge to defecate

 

 

 

 

 

<0.001

-          Always

3

0

8

-          Most of the time

2

0

12

-          Uncertain

1

0

9

-          Absent

0

7

1

Frequency of defecation

 

 

 

 

 

<0.001

-          Every other day to twice a day

2

0

19

-          More often

1

5

11

-          Less often

1

2

0

Soiling

 

 

 

 

 

 

<0.001

-          Never

3

0

2

-          Staining <1/week, no change of underwear required

2

0

12

-          Frequent staining, change of underwear often required

1

2

10

-          Daily soiling, requires protective aids

0

5

6

Fecal accidents

 

 

 

 

 

<0.001

-          Never

3

1

21

-          Fewer 1/week

2

0

4

-          Weekly, requires protective aids

1

0

3

-          Daily, requires protective aids day and night

0

6

2

Constipation**

 

 

 

 

 

<0.001

-          No constipation

3

0

22

-          Manageable with diet

2

0

4

-          Manageable with laxatives

1

2

5

-          Manageable with enemas

0

5

0**

Social problems

 

 

 

-          No social problems

3

0

25***

 

 

<0.001

-          Sometimes

2

0

5

-          Problems restricting social life

1

0

0

-          Severe social/psychosocial problems

0

7

0

*Statistical method: the Freeman-Halton extension of the Fisher’s exact probability test 2 × 4 or 2 × 3, two-tailed.

**Five patients used enemas including Klyx® regularly or as needed. Not because of constipation but because of leakage and the inability to empty the bowel.

***Two of these had severe neuropsychological disabilities (autism) and were difficult to evaluate.

 

Table 5: Bowel function scores of children with Hirschsprung disease after appendicostomy compared with controls.

Evaluation of bowel control

Score

After appendicostomy

Controls

P-value*

 

 

 

 

 

 

 

N =5**

N=30

 

Ability to hold back defecation

 

 

 

 

 

<0.01

-          Always

3

5

7

-          Problems <1/week

2

0

15

-          Weekly problems

1

0

7

-          No voluntary control

0

0

1

Feels/reports the urge to defecate

 

 

 

 

 

0.874

-          Always

3

2

8

-          Most of the time

2

2

12

-          Uncertain

1

1

9

-          Absent

0

0

1

Frequency of defecation

 

 

 

 

 

0.157

-          Every other day to twice a day

2

5

19

-          More often

1

0

11

-          Less often

0

0

0

Soiling

 

 

 

 

 

 

0.500

-          Never

3

1

2

-          Staining <1/week, no change of underwear required

2

3

12

-          Frequent staining, change of underwear often required

1

1

10

-          Daily soiling, requires protective aids

0

0

6

Fecal accidents

 

 

 

 

 

1.0

-          Never

3

5

21

-          Fewer 1/week

2

0

4

-          Weekly, requires protective aids

1

0

3

-          Daily, requires protective aids day and night

0

0

2

Constipation***

 

 

 

 

 

1.0

-          No constipation

3

4

22

-          Manageable with diet

2

0

4

-          Manageable with laxatives

1

1

5

-          Manageable with enemas

0

0

0**

Social problems

 

 

 

 

 

0.156

-          No social problems

3

4

25****

-          Sometimes

2

0

5

-          Problems restricting social life

1

1

0

-          Severe social/psychosocial problems

0

0

0

*Statistical method: The Freeman-Halton extension of the Fisher exact probability test 2 × 4 or 2 × 3, two-tailed.

**2 patients stopped using the appendicostomy.

***Five patients used enemas, including Klyx®, regularly or as needed; but not because of constipation, but because of leakage and inability to empty the bowel.

****Two of these had severe neuropsychological disabilities (autism) and were difficult to evaluate.


Discussion

This study showed that ACE through an appendicostomy stoma can achieve increased bowel control for selected children with HD who have undergone TERPT. Our patients who were considered for appendicostomy and had favorable outcomes after the procedure were those with very poor bowel function, those who had low compliance with rectal enemas, and those with a syndrome. The children with an appendicostomy who continued to use it to administer ACE achieved a level of bowel control similar to the bowel control of children with HD without an appendicostomy.

To our knowledge, there are no published reports on this select group of patients with appendicostomy. Prior studies have reported data from mixed cohorts of patients with a variety of conditions, including anorectal malformations, HD, idiopathic constipation, neurological disorders such as spine bifida, and post-traumatic disorders with various indications [9-12, 13, 20]. Our aim was to clarify the indications for appendicostomy in children with HD who have undergone TERPT. The results confirmed the findings of similar studies that were performed on other types of patients; namely, that ACE through an appendicostomy is effective and improves fecal continence and patient autonomy [14, 20].

The long-term complication rate of children undergoing an appendicostomy was high (57%) but was in line with previous studies on appendicostomy in mixed diagnosis groups reporting 23–100% complications [19, 20-23]. Previously, the most frequently reported complications associated with appendicostomy were stenosis, pain during catheter insertion, and leakage [8, 21, 23]. It has been suggested that the complication rate might be decreased using a Chait cecostomy regarding infections and fistula formation [24]. It has also been speculated that younger children might have fewer complications with their appendicostomy because either the appendicular valve is continent in young children or the Chait in the appendix, which is used more frequently in young children and might be protective [19, 25]. In our study, the median age was low and the frequency of Chait button use was high, which might explain why minor complications were reported. None of our patients developed stenosis, although previous reports have indicated that this is one of the major problems emerging during the years following an appendicostomy in 15–20% of older children [8, 15]. It is possible that stoma stenosis did not develop in our patients because we left a catheter in place for at least 6 weeks after surgery, and most of these children continued to use the Chait button.

ACE had been proposed with the main purpose of improving personal control and hygiene, but it has primarily been studied in older patients [19]. However, the timing of appendicostomy and bowel control problems have not been evaluated in children with HD. Appendicostomy for ACE may seem to be an aggressive treatment for bowel management in preschool children. At our institution, we chose to perform appendicostomy in select patients with HD, independent of age, who had severe bowel management issues and who did not obtain sufficient management with conventional regimens such as dietary modifications, oral medications, and rectal enemas. This requires detailed knowledge not only of the child, but also of the child’s family. In the study cohort, all families were well informed of available treatment options and insisted upon a solution for their children’s problems. A positive finding of our study was that all guardians indicated that they would recommend appendicostomy to the family of a child with HD who had the same bowel management issues. This result agrees with a study of children with anorectal malformations who underwent the same operation [19]. Results from that study led the authors to suggest that patients should receive an appendicostomy before they reach school age to provide them with early autonomy and the possibility of being clean during the school day.

However, there have not been any valid studies that have identified the best age to perform an appendicostomy. Additional studies are required as to decide whether it is beneficial for a child to have an appendicostomy for ACE, instead of continuing rectal enemas despite a child’s dislike of the procedure. In our study, it was difficult to interpret whether improved bowel control was an effect of older age versus ACE. This issue is a consideration as the patients were older when they were assessed after ACE initiation than they were when the appendicostomy was performed. It has been suggested that many patients with HD develop improved bowel control with age. A recent study [26] has shown that after TERPT, incontinence scores, but not constipation scores, are positively correlated with follow-up duration. This result indicates that improved continence could be expected over time. Moreover, for patients with HD, appendicostomy might be used as a tool during the worst years and stopped at a later time.

We chose to use BFS because this score has been previously used for patients with HD and because it seems to consider many potential problems. However, the term constipation might be misleading and must be used and analyzed with caution. The real problem might be obstruction and bowel emptying issues. A physiological obstruction can sometimes be successfully treated by botulinum toxin A [27]. For treating obstructive symptoms, current bowel management includes botulinum toxin A injection as the first option before an appendicostomy or a colostomy.

One limitation of this study is that a small study group was assessed, with 7 of the study patients receiving an appendicostomy. Another potential limitation is that bowel function was assessed at a visit comprising an interview. The results could have been skewed to obtain a better outcome given the closeness of the patients and their families. We attempted to diminish this bias by having an independent person, who had not been previously involved in patient care, assess the BFS and appendicostomy use. However, strength of this study is that no patients were lost to follow-up and that the patients were treated at the same center where all information was also collected. All residents have access to health care free of charge at the time of need and any dropout due to socioeconomic factors is unlikely.

The bowel management program used to day differs from that used during the period of the study. The first step in the bowel management program is dietary counseling, which includes dietary modifications. If bowel symptoms are not improved, and if constipation or obstruction is the main problem, oral medications such as Loperamid are prescribed. If it turns out that the patient’s main symptom is outlet obstruction, botulinum toxin A is injected into the anal sphincter. If the patient continues to have fecal incontinence and/or constipation, the patient is introduced to the use of rectal enemas.

In conclusion, ACE administered through an appendicostomy stoma appears to improve the bowel control in some children with HD who had undergone TERPT. Using a standardized method for evaluating and selecting the patients most likely to benefit from an appendicostomy is important.

Acknowledgments

We are grateful to Fredrik Nilsson, biostatistician at the Competence Centre for Clinical Research, Skåne University Hospital, LUND, Sweden, for statistical advice. This manuscript has been edited by native English-speaking medical experts from BioMed Proofreading LLC.


References:

1. De la Torre-Mondragón L, Ortega-Salgado JA. Transanal endorectal pull-through for Hirschsprung’s disease. J Pediatr Surg 1998;8:1283-1286.

2. Rintala RJ, Pakarinen MP. Long-term outcomes of Hirschsprungs’s disease. Semin Ped Surg 2012;21:336-343.

3. Chumpitazi BP, Nurko S Defecation disorders in children after surgery for Hirschsprung disease. J Pediatr Gastr Nutr 2011;53:75-79.

4. Ruttenstock E, Puri P. Systematic review and meta-analysis of enterocolitis after one-stage transanal pull-through procedure for Hirschsprung’s disease. Pediatr Surg Int 2010;26:1101-1105.

5. Van Kuyk EM, Brugman-Boezeman AT, Wissink-Essink M, Oerlemans HM, Severijnen RS, Bleijenberg G. Defecation problems in children with Hirschsprung’s disease: a prospective controlled study of a multidisciplinary behavioral treatment. Acta Pediatr 2001;90:153-159.

6. Hartman EE, Oort FJ, Aronson DC, Sprangers MA. Quality of life and disease-specific functioning of patients with anorectal malformation or Hirschsprungs’s disease: a review. Arch Dis Child 2011;96:398-406.

7. Malone PS, Ransley PG, Kiely EM. Preliminary report: the antegrade continence enema. The Lancet 1990;336:1217-1218.

8. Hoekstra LT, Kuijper CF, Bakx R, Heij HA, Aronson DC, Benninga MA. The Malone antegrade continence enema procedure: the Amsterdam experience. J Pediatr Surg 2011;46:1603-1608.

9. Becmeur F, Demarche M, Lacreuse I, Molinaro F, Kauffmann I, Moog R, Donnars F, Rebeuh J. Cecostomt button for antegrade enemas: survey of 29 patients. J Pediatr Surg 2008;43:1853-1857.

10. Wong AL, Kravarusic D, Wong SL. Impact of cecostomy and antegrade colonic enemas on management of fecal incontinence and constipation Ten years of experience in pediatric population. J Pediatr Surg 2007;43:1445-1451.

11. Levitt MA, Soffer SZ, Pena A. Continent appendicostomy in the bowel management of fecally incontinent children. J Pediatr Surg 1997;32:1630-1.

12. Yagmurlu A, Harmon CM, Georgeson KE. Laparoscopic cecostomy button placement for the management of fecal incontinence in children with Hirschsprung’s disease and anorectal anomalies. Surg Endosc 2006;20:624-627.

13. Lawal TA, Rangel SJ, Bischoff A, Pena A, Levitt MA. Laparoscopic- assisted Malone appendicostomy in the management of fecal incontinence in children. J Laparoendoscopic and advanced surg techniques. 2011;21:455-459.

14. Curry JI, Osborne A, Malone PSJ. The MACE procedure: Experience in the UK. J Pediatr Surg 1999;34:338-340.

15. Warner Webb H, Barraza MA, Crump JM. Laparoscopic appendicostomy for management of fecal incontinence. J Pediatr Surg 1997;32:457-458.

16. Robertson RW, Lynch AC, Beasley SW, Morreau PN. Early experience with the laparoscopic ace procedure. Aust N Z J Surg 1999;69:308-310.

17. Stanton MP, Shin YM, Hutson JM. Laparoscopic placement of the chait cecostomy device via appendicostomy. J Pediatr Surg 2002;37:1766-1767.

18. Jarvi K, Laitakari EM, Koivusalo A, Rintala RJ, Pakarinen MP. Bowel function and gastrointestinal quality of life among adults operated for Hirschsprung disease during childhood: a population-based study. Ann Surg. 2010;252(6):977-981.

19. Stenström P, Granéli C, Salö M, Hagelsteen K, Arnbjörnsson E. Appendicostomy in preschool children with anorectal malformation: successful early bowel management with a high frequency of minor complications. Biomed Res Int. 2013;2013:297084.

20. Rangel SJ, Lawal TA, Bischoff A, Chatoorgoon K, Louden E, Peña A, Levitt MA. The appendix as a conduit for antegrade continence enemas in patients with anorectal malformations. Lessons learned from 163 cases treated over 18 years. J Pediatr Surg 2011;46:1236- 1242.

21. Kim J, Beasley SW, Maoate K. Appendicostomy stomas and antegrade colonic irrigation after laparoscpic antegrade continence enema. J Laparoendosc Adv Surg Tech A. 2006;16:400-3.

22. Freeman JJ, Simha S, Jarboe MD, Ehrlich PF, Teitelbaum DH. Antegrade continent enema procedures performed prior to starting school may improve functional stooling and quality of life. Pediatr Surg Int 2014;30:715-722.

23. Mattix KD, Novotny NM, Shelley AA, Rescorla FJ. Malone antegrade continence enema (MACE) for fecal incontinence in imperforate anus improves quality of life. Pediatr Surg Intern 2007;23:1175- 1177.

24. De Peppo F, Iacobelli BD, De Gennaro M, Colajacomo M, Rivosecchi M. Percutaneous endoscopic cecostomy for antegrade colonic irrigation in fecally incontinent children. Endoscopy. 1999;31:501-503.

25. Yardley IE, Pauniaho SL, Baillie CT, Turnock RR, Coldicutt P, Lamont GL, Kenny SE. After the honeymoon comes divorce: long-term use of the antegrade continence enema procedure. J Pediatr Surg 2009;44:1274-1277.

26. Aworanti OM, McDowell DT, Martin IM, Quinn F. Does Functional Outcome Improve with Time Postsurgery for Hirschsprung Disease? Eur J Pediatr Surg. 2015; 2015 Feb 2.

27. Wester T, Granström AL. Botulinum toxin is efficient to treat obstructive symptoms in children with Hirschsprung disease. Pediatr Surg Int. 2015;31:255-259.