Pediatr Surg Int (2014) 30:1135–1141 DOI 10.1007/s00383-014-3602-y
ORIGINAL ARTICLE
Antegrade continence enema (ACE): predictors of outcome in 111 patients S. Basson • A. Zani • S. McDowell • E. Athanasakos S. Cleeve • S. Phelps • P. Charlesworth
•
Accepted: 25 September 2014 / Published online: 7 October 2014 Ó Springer-Verlag Berlin Heidelberg 2014
Abstract Purpose The antegrade continence enema (ACE) is used as a means of managing faecal incontinence and constipation with varying outcomes. We aim to evaluate our outcomes of ACEs and identify predictors of outcome. Methods A retrospective case-note review of patients B16 years of age undergoing an ACE (March 2000–September 2013) was carried out. Data collected included: patient demographics, functional outcomes and complications. Data are quoted as median (range) and compared using Mann–Whitney and Fisher’s exact test. Univariate analysis was performed to identify predictors of successful outcomes. P \ 0.05 is significant. Successful outcome = total continence/occasional leakage and failed outcome = regular soiling and/or constipation. Results 111 patients with complete data sets underwent an ACE [59 % male, median age = 9.5 years (3.4–16 years)] and median follow-up = 48 months (4 months–11 years 4 months). Underlying diagnoses were idiopathic constipation (n = 68), anorectal malformation (n = 27), neuropathic bowel (n = 7), Hirschsprung disease (n = 5) and gastrointestinal dysmotility (n = 4). Social continence was achieved in 87/111 (78 %). Fifteen percent of patients underwent reversal of ACE due to resolution of symptoms. S. Basson A. Zani S. McDowell S. Cleeve S. Phelps P. Charlesworth (&) Department of Paediatric Surgery, The Royal London Hospital, Whitechapel, London E1 1BB, UK e-mail: [email protected] S. Basson e-mail: [email protected] E. Athanasakos Department of Gastrointestinal Physiology, University College Hospital, 235 Euston Road, London NW1 2BU, UK
There was no difference in outcomes related to diagnosis, gender, age or follow-up duration. Complication rate was 20.7 % (23/111). Conclusions The ACE is safe and effective in the management of intractable constipation and soiling. No predictors of outcome were identified. Keywords ACE MACE Appendicostomy Constipation Faecal soiling Children
Introduction The antegrade continence enema (ACE) has been used as a surgical means of managing faecal incontinence and constipation, for nearly 25 years [1] with the aim of providing predictability of faecal continence. Since its first description by Malone et al. [2], there have been many variations to the original technique and its use has spread across an increasing diverse population of patients [1, 3]. Over 600 ACEs have been performed and its acceptance has been based on individual units reporting favourable results without it having been subjected to a controlled, randomised study [1]. The ACE has been shown to greatly improve quality of life [4–7], with overall success rates as high as 90 % [8], with a continuing improvement in complication rates [1]. However, there continues to be variation in reported outcomes. Additionally, emerging long-term data on the ACE have shown a late ‘failure’ rate, with more than 40 % of patients discontinuing use of their ACE after a median of 11 years [9]. Predicting which patients are going to have good outcomes remains difficult. We have previously reported our early experience of the ACE [1]. The aim of this study was to evaluate the outcomes of ACEs at our institution with a view to identifying predictors of good
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outcomes with a larger cohort of patients and a longer duration of follow-up.
Materials and methods Patients and data collection A retrospective case-note review was carried out on all patients B16 years of age undergoing an ACE procedure for intractable constipation or faecal soiling between March 2000 and September 2013 at The Royal London Hospital Department of Paediatric Surgery, a tertiary referral centre. There were no predefined physiological, radiological or psychosocial criteria as indications for an ACE. Patient selection was based on clinical and subjective evaluation. Data collected included patient demographics, primary diagnosis, surgical technique, complications, follow-up duration and continence outcomes. Outcome was described as being successful when patients were totally clean or experienced the occasional leak and failed when patients described on going regular soiling and/or constipation. Surgical technique Prophylactic broad-spectrum antibiotics are administered at the time of anaesthetic induction. An orthoptic appendicostomy is the favoured approach. The appendix is mobilised through an incision in the right iliac fossa. The tip of the appendix is brought through a separate skin incision inferiorly, and the caecum imbricated around the appendiceal base. Since 2009 we have increasingly been performing this procedure laparoscopically. A 5 mm port is inserted at the umbilicus for 0° or 30° laparoscope and a 5 mm working port is introduced in the right iliac fossa at the site chosen for the appendicostomy, through which the appendix is grasped and delivered. A further 5 mm working port may be used to aid mobilisation of the appendix if required. In both procedures, the appendix is opened at the tip, spatulated and fashioned into a skin flap to create a wide cutaneous catheterisable stoma. Caecal imbrication is not performed in the laparoscopic technique. An indwelling 12F SILASTIC (Dow Coming, Midland, MI, USA) catheter is left in the stoma for 6 weeks, after which intermittent catheterisation is commenced. A small number of patients had a Chait tube (Cook Medical Inc, Bloominton, IN, USA) or a caecostomy button placed when the appendix was not available. ACE washouts are started on the second post-operative day and patients discharged the same day if the washout is successful and patients/carers are competent in administrating these.
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Initially alternate day washouts are commenced with Bisacodyl (0.5–5 mg) and/or KleanPrep (10–20 ml/kg). Glycerin (10–30 ml) is our second-line agent of choice. Removal of the SILASTIC (Dow Coming, Midland, MI, USA) catheter is performed in the surgical outpatient clinic 6-weeks post operatively. Patients are closely monitored by our paediatric nurse specialists and followed up in gastroenterology/surgical outpatient department to establish individualised washout regimens. The type, dosage and frequency of washouts are adjusted so that patients achieve a successful washout. Successful washout is defined as the evacuation of faeces shortly after a washout without retrograde leakage from the stoma or soiling in-between washouts, and no palpable faecalomas on abdominal examination. Statistics Data are quoted as median (range) and compared using Mann–Whitney and Fisher’s exact test as appropriate. Univariate analysis was performed to identify predictors of successful outcomes. P \ 0.05 was regarded as significant. The probability of reversal of ACE was calculated with Kaplan–Meier analysis. The 12 patients with failing ACE who underwent elective ACE closure and ileostomy creation were excluded from this analysis.
Results Demographics A total of 122 patients underwent an ACE procedure during the study period. Data sets were available for 111 patients (92 %). Of these 111, 66 (59 %) were male and median age at ACE was 9.5 years (3.4–16). Overall, 38 (34 %) patients were B7 years of age. Median follow-up was 48 months (4 months–11 years and 4 months). An ACE using the appendix was performed in 98 patients, four of which were performed laparoscopically, and 13 patients underwent a caecostomy. The underlying diagnoses were idiopathic constipation (IC) (n = 68), anorectal malformation (ARM) (n = 27), neuropathic bowel (n = 7), Hirschsprung disease (HD) (n = 5) and gastrointestinal (GI) dysmotility (n = 4) (Table 1). ARM was classified as rectourethral (5/27), rectovesical (1/27), rectovestibular (3/27), rectovaginal (2/27), cloaca (3/27), rectal atresia (3/27), and anterior stenotic anus (2/ 27). A further eight patients defined as having a ‘high’ ARM, had their primary surgery performed at external institutions and thus the precise anatomical ARM was not available. Sacral ratios were available for 18 patients with a median ratio of 0.56 (range 0.21–0.83). Five patients
Pediatr Surg Int (2014) 30:1135–1141 Table 1 Patient demographics
IC idiopathic constipation, ARM anorectal malformation, HD Hirschsprung disease, GI gastrointestinal
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Diagnosis
Idiopathic n = 68
ARM n = 27
Neuropathic n=7
HD n=5
GI dysmotility n=4
Age at ACE
9 years 8 months (3 years 2 months– 16 years)
9 years (4 years– 15 years 6 months)
11 years (6–15 years 6 months)
7 years (5–11 years– 7 months)
15 years (11 years 8 months– 15 years 8 months)
Male:female
41:27
16:11
4:3
4:1
1:3
(cloaca = 2, anterior stenotic anus = 2 and rectovestibular fistula = 1) presented with constipation and faecal soiling, refractory to maximal medical management. In the remaining 22/27 patients, an ACE was indicated for faecal incontinence. Three patients with GI dysmotility were all diagnosed with small bowel dysmotility, slow colonic transit and normal anorectum on small and large GI tract manometry studies. One patient was clinically diagnosed as pseudo-obstruction. All presented with symptoms of constipation, with or without overflow incontinence and an ACE procedure was offered to these patients as a last attempt to manage symptoms before formation of an ileostomy or colostomy. Patients with IC presented with longstanding constipation and varying degrees of faecal soiling. The majority of patients referred for consideration of ACE had previously been managed for many months and years under the care of the paediatric and gastroenterology teams. Those that had failed maximal conservative and medical therapy, as per NICE (National Institute for Health and Care Excellence) guidelines on management of constipation in children and young people [10], were considered for ACE stoma. Bowel evacuation programmes included oral and rectal laxatives, manual evacuation of faeces under general anaesthetic, anal intra-sphincteric Botulin toxin injection and rectal washout training. Six patients had previously undergone resection of megarectum. Continence Overall, the ACE procedure was successful in 87 patients (78 %), including 7 patients with a diagnosis of autism and behavioural problems. Of the 87 patients, only 2 (1.8 %) reported occasional soiling (once a month) whilst the remaining were completely clean. Of the 24 patients with ongoing soiling, 7 (29 %) were non-compliant with ACE washouts. Continence outcomes for each diagnostic are shown in (Fig. 1). Soiling and constipation resolved in all patients with Hirschsprung disease or neuropathic bowel. Conversely, 3 (75 %) patients with GI dysmotility still suffer from soiling. Finally, there was no significant difference in the success rate of the ACE procedure between the two major diagnostic groups, patients with IC (n = 54, 79 %) and those born with ARM (n = 20, 74 %; P = 0.59).
Fig. 1 Success rate of the ACE procedure according to the different diagnostic groups. ACE antegrade continence enema, ARM anorectal malformation, HD Hirschsprung disease, GI gastrointestinal
Thirty-eight (34 %) patients are able to open bowels spontaneously in-between washouts without soiling. ACE reversal was performed in 17/111 patients [median age 11.5 years (range 7–15.7 years), male = 12] following successful management of faecal soiling (12 = IC, 3 = neuropathic bowel, 1 = ARM, 1 = GI dysmotility), and 12/111 [median age 12.2 years (range 7.5–16 years), male = 3] for failed management of their symptoms with non-compliance or ineffectiveness of washouts (7 = IC, 3 = ARM, 2 = GI dysmotility). Resection of megarectum with ileostomy was performed in 7 out of 12 of these patients. In our series, the probability of an ACE being reversed for resolution of symptoms was 0.4 at 10 years (Fig. 2). At univariate analysis comparing patients with unsuccessful outcome with those with successful ACE, no differences were found in terms of age [11 (3–16) vs. 9 (3–16) years; P = 0.14], gender [11 male (46 %) vs. 55 (63 %); P = 0.16], or length of follow-up [52 (10–113) months vs. 44 (4–136); P = 0.14]. Complications Complications (n = 26) occurred in 23 patients (13 IC, 9 ARM, 1 HD) with an overall complication rate of 20.7 % (Table 2). Five out of twenty-three patients required stoma revision (stenosis = 3, stomal prolapse = 2). One patient with extensive granuloma required surgical excision. Local infection was treated with oral antibiotics. Remaining
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complications were managed conservatively. Complications occurred in 13/68 (19 %) patients with IC, 9/27 (33 %) patients with ARM and 1/5 (20 %) patients with HD. When complications were compared between the two largest diagnostic groups, IC and ARM, there were no significant association with gender [12 males with IC (92 %) vs. 6 with ARM (67 %); P = 0.26] or age [IC = 8 (3.2–16) years vs. ARM = 6.7 (4.2–10.4) years; P = 0.17] and incidence of complications. Complications were associated with younger age (8.5 ± 3.6 vs. 10.2 ± 3.2 years; P = 0.03) but not with gender [18 males with complications (78 %) vs. 48 males without complications (55 %); P = 0.06]. Of the 23 patients with complications, 20 had undergone an open appendicostomy 1/23 had a laparoscopic appendicostomy and 4/23 patients had a caecostomy sited. There was no significant association between open appendicostomy vs. laparoscopic appendicostomy (19 vs. 25 %; P = 1) or appendicostomy vs. caecostomy (19 vs. 31 %; P = 0.46) and complication rate.
Discussion The prevalence of faecal incontinence in childhood ranges between 0.8 and 4.1 % in Western societies, and 7.8 %
Fig. 2 Probability of ACE reversal calculated by Kaplan–Meier analysis. ACE antegrade continence enema
globally [11]. Chronic constipation is the underlying cause in 90 % of cases [12], accounting for 25 % of all referrals to paediatric gastroenterology outpatient clinics [13], with one-third of children experiencing persisting symptoms into adulthood [14]. Irrespective of the underlying pathology, children with FI and constipation have a significantly lower quality of life and develop higher rates of behavioural and emotional problems [11]. Malone et al. [2] first described the ACE procedure in 1990 in patients with faecal incontinence secondary to neuropathic bowel. With evidence of increasing success rates, patient acceptance and reduced complication rates, the ACE has become established in the management of soiling and intractable constipation secondary to a range of underlying pathologies [1, 10, 15]. This is reflected in our cohort of patients, with idiopathic constipation and ARM constituting the largest diagnostic groups. Successful outcomes are achieved in 59–97 % of ACE procedures reported in the literature. [1, 15, 16]. Comparison between studies is difficult due to many factors including: patient selection, operative technique, washout regimes, definition of outcomes, complications and study design. Presently there are no universally agreed predictors of patient outcome. Establishing predictors of outcome would enable appropriate patient selection for ACE and allow for more informed counselling of patients and carers. Moreover it may lead to a reduction in complications and avoid unnecessary further surgical intervention. As the collective experience of using the ACE increases, clinicians are beginning to identify potential predictors of outcome. Historically, underlying aetiology was thought to predict outcome, with propulsive disorders (chronic idiopathic constipation or GI dysmotility) demonstrating a worse prognosis, with failure rates up to 40 % [17]. Patients with idiopathic constipation constitute the largest diagnostic group in our study, achieving a success rate of 79 %. This is comparable with success rates between 80 and 90 % in the recent literature [8, 18]. In addition to refinement of the ACE procedures over the years, improved outcomes in idiopathic constipation may be secondary to
Table 2 Complications Complication
Number
Diagnosis
ACE technique
Stomal stenosis
9
IC = 6, ARM = 2, HD = 1
OA = 9
Granulation tissue
7
IC = 3, ARM = 4
OA = 4, Caecostomy = 3
Local infection/leakage Stoma leakage
4 2
IC = 3, ARM = 1 IC = 1, ARM = 1
LA = 1, OA = 3 OA = 2 OA = 2
Stoma prolapse
2
IC = 1, ARM = 1
Creation of false passage
1
IC = 1
Caecostomy = 1
Incisional hernia
1
IC = 1
OA = 1
IC idiopathic constipation, ARM anorectal malformation, HD Hirschsprung disease, OA open appendicostomy, LA laparoscopic appendicostomy
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recognition of the importance of ongoing engagement with bowel management programmes with dedicated support from clinical nurse specialists. Long-term studies point towards a natural history of spontaneous improvement during adolescence in patients with idiopathic constipation [14, 19]. It is possible that there may be a time-related improvement unrelated to the use of an ACE bowel management system. All our patients with HD or neuropathic bowel had a successful outcome. Patients with GI dysmotility were the least successful. Accurate statistical subset analysis based on aetiology was not possible due to small numbers in some diagnostic groups. There was thus, no association between successful outcomes and underlying pathology in our cohort. Rodriguez et al. [20] have demonstrated that colonic motility improves after the ACE. Although the baseline colonic manometry in their group of selected patients did not predict the initial response to ACE, subsequent improvements in colonic manometry were used to predict successful decrease in the use of the ACE. Although a few of our patients underwent colonic manometry, this is not routinely performed in our institution, and therefore we were unable to evaluate its role as an outcome predictor. Siddiqui et al. [16] demonstrated successful response to medical therapy before the ACE placement, was a predictor of success with ACE irrigations. Pre-operative medical therapy varied widely with regards to type of laxatives, frequency and dosage in our cohort. Therefore we were unable to establish an association with ACE outcome. Curry et al. reported a significantly poorer outcome in patients \5 years of age, and suggested this to be secondary to poor compliance. Studies by Stenstro¨m et al. [21] and Masadeh et al. [22], showed an increased complication rate in patients \6 years of age and \7 years of age, respectively. Majority of these complications, however, were minor and success rates between the older and younger children were comparable. Conversely, Christison-Lagay et al. [23] reported age \12 years at time of ACE as a predictor of success. In our study, univariate analysis comparing patients with unsuccessful outcome with those with successful ACE, demonstrated no outcome differences in terms of age. However, there was a statistically significant association with an increased number of complications with younger age at time of ACE (P = 0.03). Although the majority of these complications were minor, it would be important to counsel parents and patients of this increased risk. We believe that in selected patients with highly motivated families, an ACE can be offered to young children with favourable outcomes unlike the study by Rodriguez et al. [20] and Jaffray et al. [24], there was no association between gender with outcomes or complications in our cohort.
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Our complication rate was 20 %, which is comparable to published data. The 8 % incidence of stomal stenosis in this study compares favourably to reported rates of 14–50 % [16]. This may be due to our preferred technique of using a skin flap to create a wide cutaneous catheterisable stoma or due to under-reporting. The incidence of leakage was comparable with reported rates of 7–10 % [3, 16]. The role of caecal plication in reducing the rates of stomal leakage is controversial. Some studies have advocated its role [25, 26] whereas others have suggested that caecal plication is more invasive and complex and may lead to more complications [3, 15, 27, 28]. Caecal imbrication is performed in the open but not the laparoscopic method in our institution. Stomal leakage did not occur in any of our patients following laparoscopic ACE without caecal imbrication. Overall, there was no association between the surgical technique used to create an ACE and incidence of complications. Stoma reversal was performed in 29/111 (26 %) of our patients, 17/29 for successful resolution of symptoms [median age 11.5 years (range 7–15.7 years), male = 12] and 12/29 for disuse due to non-compliance or ineffectiveness of washouts [median age 12.2 years (range 7.5–16 years), male = 3]. This represents a ‘cure’ rate of 18 % (12/68) in patients with idiopathic constipation and 15 % across the whole cohort and is consistent with recent literature [8, 18, 24]. There was no statistical difference in demographics or diagnosis between patients who had a successful ACE reversal compared to the remaining cohort. Forty percent of patients stop using their ACE after a median of 11 years. This may partly be attributed to lack of patient support and inadequate transitional care arrangements on reaching adulthood [9]. Recent trends in bowel management of constipation and faecal incontinence refractory to medical therapy have seen the re-introduction of rectal irrigation systems. This represents a less-invasive alternative negating the need for surgical intervention. Despite high success quoted in the literature, this is only in small series with highly selected patients [29–31]. Electrical stimulation therapy for chronic constipation, both in adults and children is emerging either via the direct route in the form of sacral nerve stimulation or the indirect route with pad electrode placement on the skin. In combination with the ACE a significant reduction in laxative use has been demonstrated [32] in slow-transit constipation children. Due to its success and patient acceptance, ACE formation rate have subsequently fallen. Both of these represent interesting evolution of bowel management. Larger studies based on intention to treat will be valuable in the ongoing critical evaluation of these bowel management options. Despite the limitations of a retrospective study, where symptoms and complications are subject to observer bias,
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we have shown that the ACE is safe and effective in the management of chronic constipation and faecal incontinence at our institution with comparable outcomes and reduced complications. This may reflect; differences in referral patterns, socio-economic status, previous management, underlying pathology, co-morbidity, patient/parent motivation, patient selection criteria, differences in service provision, and variations in assessment of outcome and follow-up. With so many confounding factors, comparison can be speculative at best. The success of the ACE is heavily reliant on a multi-disciplinary team effort including an experienced team of clinical nurse specialists and clinical psychologists. Previous work at this institution suggests that pre-operative psychosocial assessment might help to refine patients that will have successful outcomes [33].
Conclusion This study shows that the ACE stoma is safe and effective in the management of children with faecal incontinence and constipation with a 78 % success rate. Fifteen percent of patients in this study underwent reversal of ACE due to resolution of symptoms. No association between underlying pathology, gender, age and duration of follow-up with outcomes was identified. Routine pre-operative psychosocial function, quality of life assessment and physiological assessment may refine patient selection but this would be associated with time, financial and compliance constraints. Acknowledgments The authors would like to thank Mr. Harry Ward and Continence Nurse Specialists Susan Broadhead and Claire Foster, for their contribution to the care and management of these patients. Conflict of interest of interest.
The authors declare that they have no conflict
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7. Tiryaki S, Ergun O, Celik A et al (2010) Success of Malone’s antegrade continence enema (MACE) from the patients’ perspective. Eur J Pediatr Surg 20:405–407 8. Peeraully MR, Lopes J, Wright A et al (2014) Experience of the MACE procedure at a regional paediatric surgical unit: a 15-year retrospective review. Eur J Pediatr Surg 24:113–161 9. Yardley IE, Pauniaho S, Baillie CT et al (2009) After the honeymoon comes divorce: long-term use of the antegrade continence enema procedure. J Paediatr Surg 44:1274–1277 10. National Institute for Health and Care Excellence (2010) Constipation in Children and Young People: NICE Guidance. National Institute for Health and Care Excellence, London, https://www.nice.org.uk/Guidance/qs62. Accessed 14 Sept 2014 11. Rajindrajitj S, Devanarayana NM, Benninga MA (2013) Review article: faecal incontinence in children: epidemiology, pathophysiology, clinical evaluation and management. Aliment Pharmacol Ther 37:37–48 12. Benninga MA, Voskuijl WP, Taminiau JA (2004) Childhood constipation: is there new light in the tunnel? J Pediatr Gastroenterol Nutr 39:448–464 13. Youssef NN, Di LC (2001) Childhood constipation: evaluation and treatment. J Clin Gastroenterol 33:199–205 14. van Ginkel R, Reitsma JB, Bu¨ller HA et al (2003) Childhood constipation: longitudinal follow-up beyond puberty. Gastroenterol 125:357–363 15. Curry JJ, Osborne A, Malone PS (1999) The MACE procedure: experience in the United Kingdom. J Pediatr Surg 34:338–340 16. Siddiqui AA, Fishman SJ, Bauer et al (2011) Long-term followup of patients after antegrade continence enema procedure. Gastroenterol Nutr 52:1–16 17. Curry JI, Osborne A, Malone PS (1998) How to achieve a successful Malone antegrade continence enema. J Pediatr Surg 33:138–141 18. King SK, Sutcliffe JR, Southwell BR et al (2005) The antegrade continence enema successfully treats idiopathic slow-transit constipation. J Pediatr Surg 40:1935–1940 19. Procter E, Loader P (2003) A 6-year follow-up study of chronic constipation and soiling in a specialist paediatric service. Child Care Health Dev 29:103–109 20. Rodriguez L, Nurko S, Flores A (2012) Factors associated with successful decrease and discontinuation of antegrade continence enemas (ACE) in children with defecation disorders: a study evaluating the effect of ACE on colon motility. Neurogastroenterol Motil 25:e140–e181 21. Stenstro¨m P, Grane´li C, Salo¨ M et al (2013) Appendicostomy in preschool children with anorectal malformation: successful early bowel management with a high frequency of minor complications. Biomed Res Int 29:70–84 22. Masadeh MM, Krein M, Petersin J et al (2013) Outcome of antegrade continent enema (ACE) procedures in children and young adults. J Pediatr Surg 48:2128–2133 23. Christison-Lagay ER, Rodriguez L, Kurtza M et al (2010) Antegrade colonic enemas and intestinal diversion are highly effective in the management of children with intractable constipation. J Pediatr Surg 45:213–219 24. Jaffray B (2009) What happens to children with idiopathic constipation who receive an antegrade continent enema? J Pediatr Surg 44:404–407 25. Rangel SJ, LawalTA Bischoff A et al (2011) 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 46:1236–1342 26. Karpman E, Das S, Kurzrock EA (2002) Laparoscopic antegrade continence enema (Malone) procedure: description and illustration of technique. J Endourol 16:325–328
Pediatr Surg Int (2014) 30:1135–1141 27. Antao B, Ng J, Roberts J (2006) Laparoscopic antegrade continence enema using a two-port technique. J Laparoendosc Adv Surg Tech A 16:168–173 28. Koivusalo A, Pakarinen M, Rintala RJ (2006) Are cecal wrap and fixation necessary for antegrade colonic enema appendicostomy? J Pediatr Surg 41:323–326 29. Corbett P, Denny A, Dick K et al (2014) Peristeen integrated transanal irrigation system successfully treats faecal incontinence in children. J Pediatr Urol 10:219–222 30. Alenezi H, Alhazmi H, Trbay M et al (2014) Peristeen anal irrigation as a substitute for the MACE procedure in children who are in need of reconstructive bladder surgery. Can Urol Assoc J 8:E12–E15
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ORIGINAL ARTICLE
Antegrade continence enema (ACE): predictors of outcome in 111 patients S. Basson • A. Zani • S. McDowell • E. Athanasakos S. Cleeve • S. Phelps • P. Charlesworth
•
Accepted: 25 September 2014 / Published online: 7 October 2014 Ó Springer-Verlag Berlin Heidelberg 2014
Abstract Purpose The antegrade continence enema (ACE) is used as a means of managing faecal incontinence and constipation with varying outcomes. We aim to evaluate our outcomes of ACEs and identify predictors of outcome. Methods A retrospective case-note review of patients B16 years of age undergoing an ACE (March 2000–September 2013) was carried out. Data collected included: patient demographics, functional outcomes and complications. Data are quoted as median (range) and compared using Mann–Whitney and Fisher’s exact test. Univariate analysis was performed to identify predictors of successful outcomes. P \ 0.05 is significant. Successful outcome = total continence/occasional leakage and failed outcome = regular soiling and/or constipation. Results 111 patients with complete data sets underwent an ACE [59 % male, median age = 9.5 years (3.4–16 years)] and median follow-up = 48 months (4 months–11 years 4 months). Underlying diagnoses were idiopathic constipation (n = 68), anorectal malformation (n = 27), neuropathic bowel (n = 7), Hirschsprung disease (n = 5) and gastrointestinal dysmotility (n = 4). Social continence was achieved in 87/111 (78 %). Fifteen percent of patients underwent reversal of ACE due to resolution of symptoms. S. Basson A. Zani S. McDowell S. Cleeve S. Phelps P. Charlesworth (&) Department of Paediatric Surgery, The Royal London Hospital, Whitechapel, London E1 1BB, UK e-mail: [email protected] S. Basson e-mail: [email protected] E. Athanasakos Department of Gastrointestinal Physiology, University College Hospital, 235 Euston Road, London NW1 2BU, UK
There was no difference in outcomes related to diagnosis, gender, age or follow-up duration. Complication rate was 20.7 % (23/111). Conclusions The ACE is safe and effective in the management of intractable constipation and soiling. No predictors of outcome were identified. Keywords ACE MACE Appendicostomy Constipation Faecal soiling Children
Introduction The antegrade continence enema (ACE) has been used as a surgical means of managing faecal incontinence and constipation, for nearly 25 years [1] with the aim of providing predictability of faecal continence. Since its first description by Malone et al. [2], there have been many variations to the original technique and its use has spread across an increasing diverse population of patients [1, 3]. Over 600 ACEs have been performed and its acceptance has been based on individual units reporting favourable results without it having been subjected to a controlled, randomised study [1]. The ACE has been shown to greatly improve quality of life [4–7], with overall success rates as high as 90 % [8], with a continuing improvement in complication rates [1]. However, there continues to be variation in reported outcomes. Additionally, emerging long-term data on the ACE have shown a late ‘failure’ rate, with more than 40 % of patients discontinuing use of their ACE after a median of 11 years [9]. Predicting which patients are going to have good outcomes remains difficult. We have previously reported our early experience of the ACE [1]. The aim of this study was to evaluate the outcomes of ACEs at our institution with a view to identifying predictors of good
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outcomes with a larger cohort of patients and a longer duration of follow-up.
Materials and methods Patients and data collection A retrospective case-note review was carried out on all patients B16 years of age undergoing an ACE procedure for intractable constipation or faecal soiling between March 2000 and September 2013 at The Royal London Hospital Department of Paediatric Surgery, a tertiary referral centre. There were no predefined physiological, radiological or psychosocial criteria as indications for an ACE. Patient selection was based on clinical and subjective evaluation. Data collected included patient demographics, primary diagnosis, surgical technique, complications, follow-up duration and continence outcomes. Outcome was described as being successful when patients were totally clean or experienced the occasional leak and failed when patients described on going regular soiling and/or constipation. Surgical technique Prophylactic broad-spectrum antibiotics are administered at the time of anaesthetic induction. An orthoptic appendicostomy is the favoured approach. The appendix is mobilised through an incision in the right iliac fossa. The tip of the appendix is brought through a separate skin incision inferiorly, and the caecum imbricated around the appendiceal base. Since 2009 we have increasingly been performing this procedure laparoscopically. A 5 mm port is inserted at the umbilicus for 0° or 30° laparoscope and a 5 mm working port is introduced in the right iliac fossa at the site chosen for the appendicostomy, through which the appendix is grasped and delivered. A further 5 mm working port may be used to aid mobilisation of the appendix if required. In both procedures, the appendix is opened at the tip, spatulated and fashioned into a skin flap to create a wide cutaneous catheterisable stoma. Caecal imbrication is not performed in the laparoscopic technique. An indwelling 12F SILASTIC (Dow Coming, Midland, MI, USA) catheter is left in the stoma for 6 weeks, after which intermittent catheterisation is commenced. A small number of patients had a Chait tube (Cook Medical Inc, Bloominton, IN, USA) or a caecostomy button placed when the appendix was not available. ACE washouts are started on the second post-operative day and patients discharged the same day if the washout is successful and patients/carers are competent in administrating these.
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Initially alternate day washouts are commenced with Bisacodyl (0.5–5 mg) and/or KleanPrep (10–20 ml/kg). Glycerin (10–30 ml) is our second-line agent of choice. Removal of the SILASTIC (Dow Coming, Midland, MI, USA) catheter is performed in the surgical outpatient clinic 6-weeks post operatively. Patients are closely monitored by our paediatric nurse specialists and followed up in gastroenterology/surgical outpatient department to establish individualised washout regimens. The type, dosage and frequency of washouts are adjusted so that patients achieve a successful washout. Successful washout is defined as the evacuation of faeces shortly after a washout without retrograde leakage from the stoma or soiling in-between washouts, and no palpable faecalomas on abdominal examination. Statistics Data are quoted as median (range) and compared using Mann–Whitney and Fisher’s exact test as appropriate. Univariate analysis was performed to identify predictors of successful outcomes. P \ 0.05 was regarded as significant. The probability of reversal of ACE was calculated with Kaplan–Meier analysis. The 12 patients with failing ACE who underwent elective ACE closure and ileostomy creation were excluded from this analysis.
Results Demographics A total of 122 patients underwent an ACE procedure during the study period. Data sets were available for 111 patients (92 %). Of these 111, 66 (59 %) were male and median age at ACE was 9.5 years (3.4–16). Overall, 38 (34 %) patients were B7 years of age. Median follow-up was 48 months (4 months–11 years and 4 months). An ACE using the appendix was performed in 98 patients, four of which were performed laparoscopically, and 13 patients underwent a caecostomy. The underlying diagnoses were idiopathic constipation (IC) (n = 68), anorectal malformation (ARM) (n = 27), neuropathic bowel (n = 7), Hirschsprung disease (HD) (n = 5) and gastrointestinal (GI) dysmotility (n = 4) (Table 1). ARM was classified as rectourethral (5/27), rectovesical (1/27), rectovestibular (3/27), rectovaginal (2/27), cloaca (3/27), rectal atresia (3/27), and anterior stenotic anus (2/ 27). A further eight patients defined as having a ‘high’ ARM, had their primary surgery performed at external institutions and thus the precise anatomical ARM was not available. Sacral ratios were available for 18 patients with a median ratio of 0.56 (range 0.21–0.83). Five patients
Pediatr Surg Int (2014) 30:1135–1141 Table 1 Patient demographics
IC idiopathic constipation, ARM anorectal malformation, HD Hirschsprung disease, GI gastrointestinal
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Diagnosis
Idiopathic n = 68
ARM n = 27
Neuropathic n=7
HD n=5
GI dysmotility n=4
Age at ACE
9 years 8 months (3 years 2 months– 16 years)
9 years (4 years– 15 years 6 months)
11 years (6–15 years 6 months)
7 years (5–11 years– 7 months)
15 years (11 years 8 months– 15 years 8 months)
Male:female
41:27
16:11
4:3
4:1
1:3
(cloaca = 2, anterior stenotic anus = 2 and rectovestibular fistula = 1) presented with constipation and faecal soiling, refractory to maximal medical management. In the remaining 22/27 patients, an ACE was indicated for faecal incontinence. Three patients with GI dysmotility were all diagnosed with small bowel dysmotility, slow colonic transit and normal anorectum on small and large GI tract manometry studies. One patient was clinically diagnosed as pseudo-obstruction. All presented with symptoms of constipation, with or without overflow incontinence and an ACE procedure was offered to these patients as a last attempt to manage symptoms before formation of an ileostomy or colostomy. Patients with IC presented with longstanding constipation and varying degrees of faecal soiling. The majority of patients referred for consideration of ACE had previously been managed for many months and years under the care of the paediatric and gastroenterology teams. Those that had failed maximal conservative and medical therapy, as per NICE (National Institute for Health and Care Excellence) guidelines on management of constipation in children and young people [10], were considered for ACE stoma. Bowel evacuation programmes included oral and rectal laxatives, manual evacuation of faeces under general anaesthetic, anal intra-sphincteric Botulin toxin injection and rectal washout training. Six patients had previously undergone resection of megarectum. Continence Overall, the ACE procedure was successful in 87 patients (78 %), including 7 patients with a diagnosis of autism and behavioural problems. Of the 87 patients, only 2 (1.8 %) reported occasional soiling (once a month) whilst the remaining were completely clean. Of the 24 patients with ongoing soiling, 7 (29 %) were non-compliant with ACE washouts. Continence outcomes for each diagnostic are shown in (Fig. 1). Soiling and constipation resolved in all patients with Hirschsprung disease or neuropathic bowel. Conversely, 3 (75 %) patients with GI dysmotility still suffer from soiling. Finally, there was no significant difference in the success rate of the ACE procedure between the two major diagnostic groups, patients with IC (n = 54, 79 %) and those born with ARM (n = 20, 74 %; P = 0.59).
Fig. 1 Success rate of the ACE procedure according to the different diagnostic groups. ACE antegrade continence enema, ARM anorectal malformation, HD Hirschsprung disease, GI gastrointestinal
Thirty-eight (34 %) patients are able to open bowels spontaneously in-between washouts without soiling. ACE reversal was performed in 17/111 patients [median age 11.5 years (range 7–15.7 years), male = 12] following successful management of faecal soiling (12 = IC, 3 = neuropathic bowel, 1 = ARM, 1 = GI dysmotility), and 12/111 [median age 12.2 years (range 7.5–16 years), male = 3] for failed management of their symptoms with non-compliance or ineffectiveness of washouts (7 = IC, 3 = ARM, 2 = GI dysmotility). Resection of megarectum with ileostomy was performed in 7 out of 12 of these patients. In our series, the probability of an ACE being reversed for resolution of symptoms was 0.4 at 10 years (Fig. 2). At univariate analysis comparing patients with unsuccessful outcome with those with successful ACE, no differences were found in terms of age [11 (3–16) vs. 9 (3–16) years; P = 0.14], gender [11 male (46 %) vs. 55 (63 %); P = 0.16], or length of follow-up [52 (10–113) months vs. 44 (4–136); P = 0.14]. Complications Complications (n = 26) occurred in 23 patients (13 IC, 9 ARM, 1 HD) with an overall complication rate of 20.7 % (Table 2). Five out of twenty-three patients required stoma revision (stenosis = 3, stomal prolapse = 2). One patient with extensive granuloma required surgical excision. Local infection was treated with oral antibiotics. Remaining
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complications were managed conservatively. Complications occurred in 13/68 (19 %) patients with IC, 9/27 (33 %) patients with ARM and 1/5 (20 %) patients with HD. When complications were compared between the two largest diagnostic groups, IC and ARM, there were no significant association with gender [12 males with IC (92 %) vs. 6 with ARM (67 %); P = 0.26] or age [IC = 8 (3.2–16) years vs. ARM = 6.7 (4.2–10.4) years; P = 0.17] and incidence of complications. Complications were associated with younger age (8.5 ± 3.6 vs. 10.2 ± 3.2 years; P = 0.03) but not with gender [18 males with complications (78 %) vs. 48 males without complications (55 %); P = 0.06]. Of the 23 patients with complications, 20 had undergone an open appendicostomy 1/23 had a laparoscopic appendicostomy and 4/23 patients had a caecostomy sited. There was no significant association between open appendicostomy vs. laparoscopic appendicostomy (19 vs. 25 %; P = 1) or appendicostomy vs. caecostomy (19 vs. 31 %; P = 0.46) and complication rate.
Discussion The prevalence of faecal incontinence in childhood ranges between 0.8 and 4.1 % in Western societies, and 7.8 %
Fig. 2 Probability of ACE reversal calculated by Kaplan–Meier analysis. ACE antegrade continence enema
globally [11]. Chronic constipation is the underlying cause in 90 % of cases [12], accounting for 25 % of all referrals to paediatric gastroenterology outpatient clinics [13], with one-third of children experiencing persisting symptoms into adulthood [14]. Irrespective of the underlying pathology, children with FI and constipation have a significantly lower quality of life and develop higher rates of behavioural and emotional problems [11]. Malone et al. [2] first described the ACE procedure in 1990 in patients with faecal incontinence secondary to neuropathic bowel. With evidence of increasing success rates, patient acceptance and reduced complication rates, the ACE has become established in the management of soiling and intractable constipation secondary to a range of underlying pathologies [1, 10, 15]. This is reflected in our cohort of patients, with idiopathic constipation and ARM constituting the largest diagnostic groups. Successful outcomes are achieved in 59–97 % of ACE procedures reported in the literature. [1, 15, 16]. Comparison between studies is difficult due to many factors including: patient selection, operative technique, washout regimes, definition of outcomes, complications and study design. Presently there are no universally agreed predictors of patient outcome. Establishing predictors of outcome would enable appropriate patient selection for ACE and allow for more informed counselling of patients and carers. Moreover it may lead to a reduction in complications and avoid unnecessary further surgical intervention. As the collective experience of using the ACE increases, clinicians are beginning to identify potential predictors of outcome. Historically, underlying aetiology was thought to predict outcome, with propulsive disorders (chronic idiopathic constipation or GI dysmotility) demonstrating a worse prognosis, with failure rates up to 40 % [17]. Patients with idiopathic constipation constitute the largest diagnostic group in our study, achieving a success rate of 79 %. This is comparable with success rates between 80 and 90 % in the recent literature [8, 18]. In addition to refinement of the ACE procedures over the years, improved outcomes in idiopathic constipation may be secondary to
Table 2 Complications Complication
Number
Diagnosis
ACE technique
Stomal stenosis
9
IC = 6, ARM = 2, HD = 1
OA = 9
Granulation tissue
7
IC = 3, ARM = 4
OA = 4, Caecostomy = 3
Local infection/leakage Stoma leakage
4 2
IC = 3, ARM = 1 IC = 1, ARM = 1
LA = 1, OA = 3 OA = 2 OA = 2
Stoma prolapse
2
IC = 1, ARM = 1
Creation of false passage
1
IC = 1
Caecostomy = 1
Incisional hernia
1
IC = 1
OA = 1
IC idiopathic constipation, ARM anorectal malformation, HD Hirschsprung disease, OA open appendicostomy, LA laparoscopic appendicostomy
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recognition of the importance of ongoing engagement with bowel management programmes with dedicated support from clinical nurse specialists. Long-term studies point towards a natural history of spontaneous improvement during adolescence in patients with idiopathic constipation [14, 19]. It is possible that there may be a time-related improvement unrelated to the use of an ACE bowel management system. All our patients with HD or neuropathic bowel had a successful outcome. Patients with GI dysmotility were the least successful. Accurate statistical subset analysis based on aetiology was not possible due to small numbers in some diagnostic groups. There was thus, no association between successful outcomes and underlying pathology in our cohort. Rodriguez et al. [20] have demonstrated that colonic motility improves after the ACE. Although the baseline colonic manometry in their group of selected patients did not predict the initial response to ACE, subsequent improvements in colonic manometry were used to predict successful decrease in the use of the ACE. Although a few of our patients underwent colonic manometry, this is not routinely performed in our institution, and therefore we were unable to evaluate its role as an outcome predictor. Siddiqui et al. [16] demonstrated successful response to medical therapy before the ACE placement, was a predictor of success with ACE irrigations. Pre-operative medical therapy varied widely with regards to type of laxatives, frequency and dosage in our cohort. Therefore we were unable to establish an association with ACE outcome. Curry et al. reported a significantly poorer outcome in patients \5 years of age, and suggested this to be secondary to poor compliance. Studies by Stenstro¨m et al. [21] and Masadeh et al. [22], showed an increased complication rate in patients \6 years of age and \7 years of age, respectively. Majority of these complications, however, were minor and success rates between the older and younger children were comparable. Conversely, Christison-Lagay et al. [23] reported age \12 years at time of ACE as a predictor of success. In our study, univariate analysis comparing patients with unsuccessful outcome with those with successful ACE, demonstrated no outcome differences in terms of age. However, there was a statistically significant association with an increased number of complications with younger age at time of ACE (P = 0.03). Although the majority of these complications were minor, it would be important to counsel parents and patients of this increased risk. We believe that in selected patients with highly motivated families, an ACE can be offered to young children with favourable outcomes unlike the study by Rodriguez et al. [20] and Jaffray et al. [24], there was no association between gender with outcomes or complications in our cohort.
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Our complication rate was 20 %, which is comparable to published data. The 8 % incidence of stomal stenosis in this study compares favourably to reported rates of 14–50 % [16]. This may be due to our preferred technique of using a skin flap to create a wide cutaneous catheterisable stoma or due to under-reporting. The incidence of leakage was comparable with reported rates of 7–10 % [3, 16]. The role of caecal plication in reducing the rates of stomal leakage is controversial. Some studies have advocated its role [25, 26] whereas others have suggested that caecal plication is more invasive and complex and may lead to more complications [3, 15, 27, 28]. Caecal imbrication is performed in the open but not the laparoscopic method in our institution. Stomal leakage did not occur in any of our patients following laparoscopic ACE without caecal imbrication. Overall, there was no association between the surgical technique used to create an ACE and incidence of complications. Stoma reversal was performed in 29/111 (26 %) of our patients, 17/29 for successful resolution of symptoms [median age 11.5 years (range 7–15.7 years), male = 12] and 12/29 for disuse due to non-compliance or ineffectiveness of washouts [median age 12.2 years (range 7.5–16 years), male = 3]. This represents a ‘cure’ rate of 18 % (12/68) in patients with idiopathic constipation and 15 % across the whole cohort and is consistent with recent literature [8, 18, 24]. There was no statistical difference in demographics or diagnosis between patients who had a successful ACE reversal compared to the remaining cohort. Forty percent of patients stop using their ACE after a median of 11 years. This may partly be attributed to lack of patient support and inadequate transitional care arrangements on reaching adulthood [9]. Recent trends in bowel management of constipation and faecal incontinence refractory to medical therapy have seen the re-introduction of rectal irrigation systems. This represents a less-invasive alternative negating the need for surgical intervention. Despite high success quoted in the literature, this is only in small series with highly selected patients [29–31]. Electrical stimulation therapy for chronic constipation, both in adults and children is emerging either via the direct route in the form of sacral nerve stimulation or the indirect route with pad electrode placement on the skin. In combination with the ACE a significant reduction in laxative use has been demonstrated [32] in slow-transit constipation children. Due to its success and patient acceptance, ACE formation rate have subsequently fallen. Both of these represent interesting evolution of bowel management. Larger studies based on intention to treat will be valuable in the ongoing critical evaluation of these bowel management options. Despite the limitations of a retrospective study, where symptoms and complications are subject to observer bias,
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we have shown that the ACE is safe and effective in the management of chronic constipation and faecal incontinence at our institution with comparable outcomes and reduced complications. This may reflect; differences in referral patterns, socio-economic status, previous management, underlying pathology, co-morbidity, patient/parent motivation, patient selection criteria, differences in service provision, and variations in assessment of outcome and follow-up. With so many confounding factors, comparison can be speculative at best. The success of the ACE is heavily reliant on a multi-disciplinary team effort including an experienced team of clinical nurse specialists and clinical psychologists. Previous work at this institution suggests that pre-operative psychosocial assessment might help to refine patients that will have successful outcomes [33].
Conclusion This study shows that the ACE stoma is safe and effective in the management of children with faecal incontinence and constipation with a 78 % success rate. Fifteen percent of patients in this study underwent reversal of ACE due to resolution of symptoms. No association between underlying pathology, gender, age and duration of follow-up with outcomes was identified. Routine pre-operative psychosocial function, quality of life assessment and physiological assessment may refine patient selection but this would be associated with time, financial and compliance constraints. Acknowledgments The authors would like to thank Mr. Harry Ward and Continence Nurse Specialists Susan Broadhead and Claire Foster, for their contribution to the care and management of these patients. Conflict of interest of interest.
The authors declare that they have no conflict
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