Forming an antegrade continence enema (ACE) is a long-established way to treat children with difficulty stooling or faecal incontinence that is refractory to medical management, rectal suppositories or enemas. This is seen most commonly in the paediatric surgical patient population with Hirschsprung's disease, anorectal malformations, myelomeningocele, or chronic constipation.
The ACE procedure was first described by Malone et al (1990). Early technique descriptions included caecal imbrication to prevent ACE stoma site leakage (Levitt et al, 1997), but more recent studies concluded that a caecal wrap is not necessary (Curry et al, 1999; Koivusalo et al, 2006a). The introduction of minimally invasive surgery allowed the development of various laparoscopic techniques that demonstrated simplicity (Nanigian et al, 2008) and efficiency (Webb et al, 1997) over the traditional open surgery techniques.
All practitioners have to be aware that the lack of use of the ACE stoppers can lead to stenosis (Carnaghan et al, 2012), and the need for dilatation or potentially loss of the channel. ACE stoma stoppers have been developed to combat leakage and stenosis (Lopez et al, 2007).
Both the ACE procedure, and the use of stoppers, are designed to improve the quality of life of the patient and that of their family (Kim et al, 2006). When reviewing the efficacy of both interventions it is essential that the focus is on patient-reported outcomes, rather than hospital- or clinician-centric metrics (Henrichon et al, 2012). Assessment tools currently in use include the Hirschsprung's disease/Anorectal Malformation Quality of Life (HAQL) questionnaire (Hanneman et al, 2001), the Pediatric Quality of Life Inventory (PedsQL) (Har et al, 2013; Varni, 2020), and the Faecal Incontinence and Constipation Quality of Life (FICQOL) tool (Ok and Kurzrock, 2011).
The authors of the present study developed a ‘bowel care survey and stopper questionnaire’ that reflected the themes and formats of these validated surveys and focused particularly on factors related to the use of an ACE stopper.
The questions the authors sought to answer with this evaluation were:
- What is the impact of the ACE procedure on the patient's and family's quality of life?
- Are there any patient factors that predict which stopper will be most beneficial to them?
Material and methods
The study was a prospective cohort study using two time points of evaluation, conducted between June 2019 and August 2020. The authors registered this pilot project as a service evaluation locally with the Birmingham Children's Hospital clinical governance team, Clinical Audit Registration and Management System ID-30386.
All children in the study had a laparoscopic ACE formation with the appendix tip delivered through a chevron skin incision, before forming the appendicostomy. In this procedure, a Foley catheter is then inserted through the newly placed ACE and replaced after 2-3 weeks postoperatively with an ACE stopper device.
Patients were identified using a prospectively maintained list of those who were using an ACE, managed by the colorectal nurse specialists. Patients were contacted via telephone for verbal consent and sent covering letters for the evaluation. Demographics, operation details, and data on the patient's underlying condition were gathered from electronic clinic letters and operation records. A patient information sheet and baseline ‘bowel care survey and stopper questionnaire’ were sent to consenting patients. Patients' carers were then contacted by telephone by one of the colorectal nurse specialists to go through the survey to enable efficient data collection rather than relying on surveys being returned by post.
An alternative stopper device was then sent to patients to trial for a period of approximately 4 weeks, before a repeat stopper questionnaire was completed over the phone by one of the colorectal nurse specialists. Patients' carers or parents were contacted a third and final time to complete the final stopper questionnaire concerning the third ACE stopper. Questionnaire results were then collated, and data were analysed using Microsoft Excel, performing a two-tailed t-test for continuous variables, and chi square for categorical responses.
The ACE stoppers used were the Renew Medical's Aquaflush Stoma Stopper, Avanos Medical's CORSTOP™ ACE Stopper and Flexicare's Wystopper Stoma Stopper.
Results
Data were collected from 20 patients (Figure 1) with questionnaires that assessed baseline bowel management strategy, the impact of the ACE stopper, and their opinion on alternative stoppers available with different qualities. The questionnaires were completed by members of the colorectal team. Of the 20 patients, 7 were female and 13 male.
The underlying condition leading to the need for an ACE was anorectal malformation (60%), chronic constipation (30%), and Hirschsprung's disease (10%). The median age of procedure was 6.6 years. In total, 40% of patients had already been using transanal irrigation or an enema solution. Laxatives were ineffective in 75% of patients and transanal irrigations were not tolerated by patients in 70% of cases (Table 1).
Table 1. Demographic of patients
| Sex | n |
|---|---|
| Female | 7 (35%) |
| Male | 13 (65%) |
| Diagnosis | |
| Anorectal malformation | 12 (60%) |
| Chronic constipation | 6 (30%) |
| Hirschsprung's disease | 2 (10%) |
| Age at procedure | |
| Median | 6.6 years |
| Range | 3.9–13.5 years |
| Standard deviation | 2.8 years |
| Indication for ACE | |
| Laxatives were ineffective | 15 (75%) |
| Washouts were ineffective | 11 (55%) |
| Washouts were not tolerated | 14 (70%) |
The qualitative data returned by parents about their child's bowel management on the bowel survey questionnaire was reviewed, looking for common themes. The questionnaire output was visually represented in a word cloud. Themes raised that affected individuals most included rectal washouts not being tolerated, followed by soiling, accidents and ineffective rectal washouts/laxatives.
After the ACE procedure, 85% of patients (17/20) reported that they had ‘no accidents’ or ‘occasional accidents’; 75% (15/20) reported that they now stooled only after the ACE washout, rather than sporadically throughout the day, or too infrequently.
With respect to the two key problems with an ACE – leaking and stenosis, 35% of patients (7/20) regularly experienced leaking and 50% of patients (10/20) regularly had difficulty catheterising the stoma. Only one patient required a surgical intervention to aid catheterisation. The remainder of this group either learned manipulative manoeuvres or used flushes to aid insertion of the catheter. When asked if the stopper helped with leakage, surprisingly only 20% (4/20) agreed (Table 2).
Table 2. Experiences with an antegrade continence enema (ACE)
| Difficulties experienced post-ACE procedure | n |
|---|---|
| Sometimes ACE site leaks | 4 (20%) |
| Regular leaks from ACE site | 7 (35%) |
| Difficulty catheterising | 10 (50%) |
| Stopper helps with leakage | 4 (20%) |
| Satisfaction with ACE procedure | |
| Median | 8.5 |
| Mean | 7.24 |
| Standard deviation | 1.36 |
| Would choose ACE again | 18 (90%) |
| Would not choose ACE again | 2 (10%) |
| Impact of ACE on quality of life | |
| Better school attendance | 12 (60%) |
| Bowel care does not stop child going out | 13 (65%) |
| No/slight effect on siblings | 16/19 (84%) |
| No/slight effect on parent/sibling relationship | 17/19 (85%) |
The overall satisfaction of the patients with the intervention of forming an ACE, was 8.5/10 (range 6-10, standard deviation 1.3). If given the choice to have the ACE procedure again 90% (18/20) would say yes (Table 2).
With regard to the impact on quality of life 60% of parents (12/20) reported a positive impact on school attendance since having the ACE procedure and 65% (13/20) said the patient's bowel care routine did not affect them going out and socialising. Out of the patients with siblings, 84% of parents (16/19) did not see an impact on the relationship between patient and sibling due to the ACE/bowel management, and 85% (17/19) stated it did not affect the parent's relationship with other siblings (Table 2).
As the evaluation trial advanced, 3 participants were unable to commit to completing the final questionnaire, resulting in 17 participants completing all three ACE stopper reviews. Reviewing the qualitative data returned by parents about the three ACE stopper choices shows a clear divide between people who prefer a rigid stopper (41%, 7/17) or a stopper that is more flexible (59%, 10/17). The retrospective results show the impact of the stopper on leakage from the ACE site might be coincidental as only 10%–17% experienced an improvement in leakage when trying alternative stoppers to their original. In total, 10% of parents/carers (2/20) experienced difficulties in catheterising after the transition from the flexible Aquaflush stopper to the more rigid CORSTOP or Wystopper. It is clear that preference is based on the material and design of the ACE stopper.
Finally, 59% (10/17) preferred the Aquaflush, 23% (4/17) preferred the Wystopper and 18% (3/17) preferred the CORSTOP. The CORSTOP and Wystopper are the more rigid stoppers and combining the results shows 41% (7/17) preferred the more rigid stopper and 59% (10/17) prefer the more flexible stopper.
The patient's condition did not appear to impact on which stopper is preferred. The sex of the child did not seem to make much impact on which colour stopper they preferred. The Aquaflush is available in blue, the CORSTOP and Wystopper are both translucent.
Discussion
This study is the first description of ACE stopper properties and their place in the management of complex colorectal paediatric patients. The choice of stopper not only helps with comfort and aesthetics but also allows the child to be their own advocate in their care. The results of this evaluation suggest that having an ACE procedure is beneficial compared to the patients being on ineffective transanal irrigation or enemas. These results are similar to others (Curry et al, 1999; Church et al, 2017; Grabski et al, 2018). It is important to note that patient satisfaction with the ACE is high (Table 2) but this might be increased if patients are given a choice of stopper in the first instance in line with what the above results highlight. It was interesting to explore why two candidates would not have the ACE procedure again but scored highly on the satisfaction scale; one patient required further surgery due to adhesions and after surgical revision to correct ACE complications they are much happier; the other patient has a very complex condition meaning 100% continence is not achievable. However, the satisfaction score was 8.5 overall.
The quality-of-life data also proves that having the ACE procedure has minimal impact on family life, can increase school attendance and most families report minimal impact on their socialising or family relationships. This is not dissimilar to the results of a study by Ok and Kurzrock (2011). The present study has also highlighted that, if a patient experiences leakage, changing the stopper does not appear to improve this problem. Other methods such as using a Deflux injection seem to have a more noticeable impact on leakage as documented by Koivusalo et al (2006b). Other methods to prevent stenosis include the use of a button device in the ACE, as documented by Grabski et al (2018). The majority of parents reported a good understanding of why a stopper is needed, to keep the ACE site patent and therefore preventing stenosis in the future.
A common theme as to why the Aquaflush ACE stopper was preferred was due to its flexibility and low profile; whereas the CORSTOP and Wystopper were preferred by some as they are more rigid. These specific preferences highlight the need for a choice of ACE stopper, as every patient/parent's preference is different, and this could improve patient compliance and experience.
ACE stoppers are widely used to prevent or reduce stenosis rates (Halleran et al, 2019). The diameter of the stent remains important to prevent stenosis, allowing easy catheterisation. This is similar to the findings of Carnaghan et al (2012), who found that use of an ACE stopper was highly successful in reducing patients' rates of stenosis. This study also emphasises the point that, for a device to be successful, it needs to be accepted by the patient, reinforcing the need for a choice of stopper. Carnaghan et al (2012) found that ‘stomal reflux is not a significant complication’. Unfortunately, in the present study, some patients experienced either occasional or regular leaks, therefore the authors feel this point is arguable.
In the future, it may be beneficial to liaise with manufacturers to suggest reducing the thickness of the external proportion of the CORSTOP stopper and potentially slightly increasing the stiffness of the Aquaflush stopper to make them easier for patients to insert. The size of the Aquaflush stopper being used initially was not reviewed in some patients; it is worth noting that the greater the diameter of the stopper, the more rigid it is.
Despite the limitation of a small sample size, the authors of this evaluation have gained a better understanding in the use of different ACE stopper devices in children. Although these findings cannot be generalised they are useful to develop a prospective study investigating the use of ACE stopper devices in greater detail.
Conclusion
The data from this study shows that there is a place for providing a choice of ACE stopper for patients. This offers options for patients due to the various different benefits unique to each type of stopper. The study found that the ACE procedure has a positive impact on patient and family quality of life.
KEY POINTS
- The antegrade continence enema (ACE) procedure can have a positive impact on both the patient's and their family's quality of life
- There is no strong evidence for one of the ACE stoppers being superior to the other ACE stopper options
- Preference of ACE stopper is personal, and patients favour the different stoppers for their unique reasons (eg flexibility of stopper stem, low profile of stopper top, etc)
- Allowing patient choice of ACE stopper could improve compliance
CPD reflective questions
- Think about a patient you have cared for with an antegrade continence enema (ACE) stopper. What were the main complaints about the stopper and how could this influence the choice of ACE stopper recommended?
- Are there any aspects of ACE stoppers that could be changed to enhance the stopper and make it more suitable?
- Is your current team able to give patients with an ACE stopper a choice? If not, how could you make that possible?
- Are there other aspects of patient care in which giving patients a choice could help improve their compliance with care?