- Email: [email protected]
The Distal Left Malone Antegrade Continence Enema—Is it Better?
The Distal Left Malone Antegrade Continence EnemadIs it Better? Jonathan S. Ellison, A. Neil Haraway and John M. Park* From the University of Michigan, Ann Arbor, Michigan
Purpose: The Malone antegrade continence enema is used for refractory fecal dysfunction in the pediatric neuropathic population. While various locations along the colon have been described, the optimal site for achieving efficient enemas remains unknown. We reviewed our experience with the Malone antegrade continence enema procedure and investigated functional outcomes and complications associated with proximal and distal locations. Materials and Methods: We performed an institutional review board approved, retrospective chart review of 109 consecutive MACE procedures done by a single surgeon from 2000 to 2012. Preoperative patient characteristics, intraoperative techniques and postoperative outcomes were reviewed and statistical analysis was performed. Results: Included in analysis were 90 patients treated with a total of 109 MACE procedures. Average age at operation was 13.8 years. Myelomeningocele was the most common diagnosis. Most patients underwent simultaneous urinary reconstruction. Stomal complications were most common (49% of patients). We compared the outcomes of proximal (cecal) MACE in 48 cases and distal (sigmoid colon) MACE in 55. Location did not affect the overall efficiency of fecal continence. Flush time was shorter for distal than for proximal MACE stomas (37.2 vs 61.2 minutes, p <0.001). Of the patients 15 underwent a total of 20 complete MACE revisions, including 12 proximal and 6 distal MACEs. As a channel, appendix was associated with a higher but not statistically significant stomal complication rate compared to colon (42% vs 25%). Conclusions: A cecal, transverse or colonic location for MACE results in acceptable outcomes. Distal MACE is associated with significantly shorter flush time and possibly fewer stomal complications. Stomal complications remain a frustrating reality of the MACE procedure.
Abbreviations and Acronyms BMI ¼ body mass index MACE ¼ Malone antegrade continence enema Accepted for publication January 24, 2013. Study received institutional review board approval. * Correspondence: 1500 East Medical Center Dr., Urology 3875TC 0330, Ann Arbor, Michigan 48109 (telephone: 734-615-3038; FAX: 734-6153520; e-mail: [email protected]).
Key Words: colon, sigmoid; fecal incontinence; enema; reconstructive surgical procedures; surgical stomas
PEDIATRIC urologists have long assumed the responsibility of managing urinary and fecal incontinence in patients with neuropathy. In these cases management of bowel dysfunction includes laxatives, enemas, digital stimulation and in extreme cases enteral reconstruction with the ultimate goals of quality of life and independence.
The MACE, one such reconstructive option, was first described in 1990 to manage intractable fecal incontinence.1 The original technique relied on the appendicocecal junction with the distal tip of the appendix brought to the abdominal wall as a continent catheterizable stoma. This technique can be performed simultaneously with urinary reconstruction
0022-5347/13/1904-1529/0 THE JOURNAL OF UROLOGY® © 2013 by AMERICAN UROLOGICAL ASSOCIATION EDUCATION AND RESEARCH, INC.
http://dx.doi.org/10.1016/j.juro.2013.01.092 Vol. 190, 1529-1534, October 2013 Printed in U.S.A.
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with good results.2 It has high patient satisfaction in the spina bifida population.3 While most patients do well with the MACE procedure, a subset struggles with persistent incontinence, protracted enema transit time, severe colic or the need for stomal revision.4 Younger age at operation and poor patient compliance predict worse outcomes.5 However, other factors predictive of operative complications and enema function are poorly defined. Furthermore, the efficacy of distal left MACE was proposed but not fully explored compared to standard MACE using the proximal right appendicocecal junction.6 We describe our experience with MACE using traditional right vs left reconstruction to evaluate our hypothesis that left MACE may have functional advantages.
to a poor functional outcome of the initial procedure. 2) Distal MACE was created in patients with a history of severe chronic constipation and intraoperative findings of a dilated, tortuous distal colon. 3) A few patients had severe right colonic adhesions, making cecal MACE technically challenging. For appendicocecal MACE the appendix channel was imbricated in situ or detached and reimplanted using the tenia submucosal tunnel reimplantation technique for the continence mechanism. For distal transverse and sigmoid colon MACE a catheterization conduit was reconstructed using an ileal or colon Yang-Monti channel and implanted using the tenia submucosal tunnel. After the procedure, an indwelling catheter remained in the stoma for 3 weeks postoperatively. MACE flushing and instruction were initiated through the existing indwelling catheter by specialty urology nurses before patient discharge home.
Followup
METHODS Patient Accrual We performed an institutional review board approved, retrospective chart review of all patients treated with a MACE procedure by a single surgeon (JMP) from December 2000 to December 2012. Indications for surgery were neuropathic bowel with fecal incontinence and/or severe constipation with the overall aim of independent bowel management. Patient age and BMI, if available, at operation were recorded. We also documented the bowel regimen most recently performed before MACE reconstruction, defined as none, conservative (oral agents such as laxatives or stool softeners), aggressive (rectal stimulation via suppositories, enemas, digital stimulation and/or manual disimpaction) or previous MACE procedures. Postoperatively we assessed the volume of enema fluid, washout time and fecal continence. Data points were missing in 38 patient charts. The table shows the missing data points for enema function. The remaining missing data points pertained to BMI. Data points on stomal use and function were taken from the last relevant note in the chart on that stoma. In the case of complete MACE reconstruction separate data points were used so that the total data points for stomal use and function totaled 109 and not 90.
Procedure An in-house or home preoperative bowel preparation was prescribed. The choice of bowel segment for catheterization conduit (appendix, ileum or colon) and stomal location (cecum, or transverse or sigmoid colon) were determined intraoperatively at attending surgeon discretion based on the need for additional urinary reconstruction and other anatomical considerations. Since the study was a retrospective review, no prospective algorithms were routinely followed intraoperatively to determine whether distal or proximal MACE would be performed. The attending surgeon preferentially created distal MACE in several instances. 1) A few cases of initial cecal MACE were intentionally converted to distal MACE due
Patients were followed annually with assessment of enema use and function, including patient reported complaints, continence, enema frequency and volume, flush time and enema additives, and stomal examination. Complications were recorded as described by Dindo et al.7 They were further subdivided into minor and major complications (Clavien score 2 or less and 3 or greater), and early and late (before and after 30 days, respectively). Stomal stenosis was defined as stenosis requiring operative intervention, superficial dilation or revision in the operating room, or complete MACE revision. We further analyzed cases of complete MACE revision.
Statistical Analysis All reported averages are the mean unless otherwise indicated. Subanalysis was performed to compare the type of bowel regimen before surgery, enema type and location, and patients undergoing revision. Continuous data were analyzed using the 2-tailed unpaired t test or ANOVA, as appropriate. Categorical data were analyzed using the chi-square test. All statistical analysis was hypothesis driven and performed using PrismÒ, version 5.0.
RESULTS In 90 patients a total of 109 MACE stomas were created. Presence of a ventriculoperitoneal shunt, ability to self-catheterize, ambulatory status, simultaneous urinary reconstruction, preoperative bowel program or use of inpatient bowel preparation did not vary significantly by MACE location (data not shown). Overall, most patients had a diagnosis of myelomeningocele (74), while the remainder had imperforate anus (4), sacral agenesis (2), spinal cord injury (4) or another condition, including spinal cord aneurysm, posterior urethral valves, cloacal extrophy and bladder extrophy (6). This distribution did not vary by MACE location (data not shown). Patients who received a right cecal stoma were younger at operation than those who received
DISTAL LEFT MALONE ANTEGRADE CONTINENCE ENEMA
transverse and sigmoid colon stomas (12.1 vs 17.9 and 14.9 years, respectively, p ¼ 0.005). Functional outcomes included patient reported fecal continence, flush time and volume, and enema agent and schedule. Incomplete functional outcomes were recorded in 38 of 109 medical records, such as flush time, or enema type or volume, including the charts of 6 patient with a right MACE and 3 with a left MACE who reported not using the enema due to discomfort, inconvenience or difficulty. Average flush time was 48 minutes and the average volume used was 886 ml. Subanalysis comparisons were made by organizing groups by bowel regimen before surgery, segment type used for the catheterization conduit (appendix, ileum or colon) and MACE colonic site. The table lists outcomes stratified by MACE location. Flush time was notably shorter for sigmoid vs transverse vs cecal stomas (p ¼ 0.002). Enema frequency and additives did not vary significantly among these groups (see table). Comparison of the type of conduit (appendix, ileum or colon) mirrored MACE site results since appendicocecal stomas are always on the right side and colonic stomas always on the left side. Results did not significantly differ by preoperative bowel regimen (data not shown). Followup was longer in the proximal MACE group (see table). At least 1 complication developed in 54 patients for a total of 70 complications (see table). Reoperation was indicated for stomal stenosis, stomal leakage, enterocutaneous fistula, failed prolonged elimination, bowel obstruction, incisional hernia, MACE conduit false passage and wound abscess. The single Clavien grade 4a complication was a perioperative aspiration event requiring postoperative intensive care monitoring. Stomal complications, including stomal stenosis, incontinence via the stoma, perforation, fistula and prolapse, developed in 34 cases. The distribution of the specific type of stomal complication was similar among the groups. Average time to initial stomal stenosis was 19.7 months (range 3 to 48). Right stomas showed a higher rate of stomal complications, although this did not attain statistical significance. Distal left MACE stomas had a higher rate of minor complications than others (p ¼ 0.04, see table). Notably, there was a higher rate of stomal stenosis for the detached appendicocecostomy technique than for the in situ appendicocecostomy technique, although again this did not attain statistical significance (7 patients or 41% vs 5 or 26%, p ¼ 0.48). Included in these complications were 15 patients who underwent a total of 20 complete MACE revisions. The Appendix lists revision sites and indications. Of the patients 12 (25%) treated with proximal right MACE required revision for
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Functional outcomes and complications by MACE location MACE Location Cecum No. pts No. continued fecal incontinence (%) No. MACE stomal leakage (%) No. no longer using MACE (%) Mean SD flush time (mins) Mean SD enema vol (ml) No. enema schedule (%): Daily Every other day Other Not available No. agent used (%): Tap water only Soapsuds enema Tap water þ other Not available Mean/median followup (mos) No. pts with complications (%) No. complication type (%): Stomal Infectious Gastrointestinal Pulmonary Incisional Other No. Clavien grade (%):* 1 2 3b 4a No. timing (%): Early Late
48 2
(4)
Transverse Colon 6 2 (33)
2 (4) 0 6 (13) 0 61.2 35.8 45 21.2 815 328 990 317 12 22 7 6
(25) (46) (15) (12.5)
3 (50) 1 (17) 0 2 (33)
Sigmoid Colon 55 1 (2) 3 (6) 3 (6) 37.2 16.4 938 625 27 18 4 6
(49) (33) (7) (11)
34 (71) 0 8 (17) 6 (12) 68.9/70.2 24 (50)
5 (83) 0 0 1 (17) 47.9/24.7 2 (33)
46 (84) 1 (2) 2 (3) 6 (11) 30.4/31.3 28 (51)
20 (42) 4 (8) 9 (19) 1 (2) 0 1 (2)
0 0 4 (67) 0 2 (33) 0
14 8 11 0 0 4
4 (13) 4 (13) 23 (71) 1 (3)
0 0 2 (100) 0
2 (6) 14 (38) 20 (56) 0
9 (28) 23 (72)
0 2 (100)
19 (53) 17 (47)
(25) (15) (20) (7)
* No patient had Clavien grade 3a, 4b or 5 complications.
prolonged elimination in 6, stomal stenosis in 5 and incontinence from MACE in 1. Six patients (11%) treated with distal left MACE required revision due to incontinence from MACE (3), fistula (1), stomal stenosis (1) and incomplete emptying (1). Comparison of conversions required for proximal and distal MACE trended toward significance in favor of distal left MACE (p ¼ 0.07). One patient currently has a distal and a proximal MACE, given the ineffectiveness of each location alone. Age at operation, preoperative diagnosis, BMI and bowel segment type or site did not appear to influence the need for reoperation (data not shown).
DISCUSSION The pediatric urologist often treats neurogenic bladder as part of the overall care for elimination disorders using laxatives, enemas, suppositories and in severe cases the creation of an antegrade enema. The MACE seeks to provide an easily accessible stoma for antegrade enema administration, although techniques vary among surgeons. Since the first description of appendicocecostomy by
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Malone et al,1 multiple methods of conduit creation have been described as well as different locations along the course of the colon. Others suggested that distal MACE may provide shorter elimination time.5,8e10 While patient and family satisfaction is high,11 complications exist, especially those involving the stoma and treatment failure. Patient age, compliance and enema location affect enema performance, although comparisons are limited to case series of a single enema technique.2,5,6 We took advantage of the change from proximal to distal MACE at our institution by comparing our experience with the 2 techniques. In our series the distribution of distal and proximal MACE was approximately equal. All except 9 of the 90 patients (90%) still used the MACE regularly at a median followup of 42 months. Stomal complications developed most frequently at about a third of the time. Distal stomas were associated with shorter flush time. Of the original proximal right MACE cases 25% were converted to a distal location due to ineffective enema results. This is consistent with the observations of Curry et al that 9 of 31 patients with appendicocecostomy were troubled with washout failure.12 In our experience these patients are well served by conversion from proximal to distal MACE. Patient characteristics or physiological studies to aid in the preoperative identification of patients who may benefit from primary distal left MACE are lacking. As noted, abundant right lower quadrant adhesions, a severely dilated distal colon or previously ineffective proximal MACE were considerations for distal MACE creation. Our subanalysis of the impact of a preoperative bowel regimen as a surrogate indicator of colonic motility did not provide a useful clue. The limitations of our study deserve mention. Selection and followup biases are unavoidable in retrospective reviews. The study group was heterogeneous, although heterogeneity appeared evenly distributed among MACE sites. Data on the specific preoperative bowel regimen and the evaluation of enema function were limited to patient reports in the chart review with complete information not always available. Standardized methods of reporting continence outcomes are lacking. As such, continence was based on self-reported or parent reported outcomes without preoperative or postoperative physiological evaluation. Intraoperative decisions involving stomal location and type were based on various patient and anatomical factors, as described. Such decisions are case specific and possibly not generalizable. These limitations aside, we believe that our experience adds to a growing body of evidence
supporting MACE in select individuals with neuropathic bowel. Our findings reinforce the high continence rates previously reported in the literature.13 Our study includes a fairly even distribution of proximal right cecal vs distal left MACE locations, allowing for comparison between the methods. Right appendicocecostomy MACE is a less complicated procedure, which may account for its lower rate of early postoperative complications. However, this observation is limited by the fact that most cases were done with simultaneous urinary reconstruction, adding extra complexity to the procedure. As reported in other studies,6,14 distal stomas had a shorter flush time than proximal stomas. While stomal complications did not differ statistically between the groups, there was a trend toward a lower rate of stomal complications for left distal MACE. The use of detached appendicocecostomy may contribute to a higher stomal stenosis rate, although the difference between the 2 techniques for appendicocecostomy did not attain statistical significance. Appendicocecal stomas may be more susceptible to minor wound infections progressing to stenosis due to the fecal bacterial content in that bowel area. However, we cannot exclude followup bias with a shorter mean followup in patients with left distal MACE. To our knowledge the impact of preoperative bowel regimens has not been studied in relation to postoperative MACE function. Indications for the MACE procedure include the failure of adequate bowel programs. However, the appropriate aggressiveness of bowel programs before MACE reconstruction has yet to be defined. The concomitant need for complex urological reconstruction may drive the decision for patients with borderline bowel function to undergo a MACE procedure. In our series most patients had undergone trials of enemas, suppositories or digital stimulation without satisfaction. However, many patients had been involved in no formal bowel regimen or in a bowel regimen consisting of only oral stool softeners or laxatives. Of such patients those who had received only oral agents appeared to have a lower complication rate postoperatively, suggesting a potential role for early surgical intervention in those requiring an aggressive bowel program via the rectum. Patients who received rectal agents may have had more severe fecal incontinence and constipation, complicating analysis. Ideally, a prospective study would eliminate these confounding variables, although the feasibility of such a study remains limited.
CONCLUSIONS The MACE procedure is generally successful. It remains a useful adjunct in the management of
DISTAL LEFT MALONE ANTEGRADE CONTINENCE ENEMA
neurogenic fecal incontinence and severe constipation. Patient selection, close postoperative followup and continued education by well informed clinical staff are essential to appropriate MACE maintenance, and patient and family satisfaction. Proximal right and distal left MACE locations result in acceptable outcomes, although left sigmoid stomas are associated with a shorter flush time and may have fewer stomal complications. Up to 20% of patients with a proximal right stoma experience prolonged elimination and abdominal cramping, and may benefit from conversion to a distal left MACE. Stomal complications, especially stomal stenosis, remain a frustrating reality of the MACE procedure. While these issues often present within the first several years after enema creation, they may present later, underscoring the need for continued followup of these patients by a knowledgeable practitioner.
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APPENDIX Complete MACE revisions with location change, if any, and indication Pt. No.
Location (revision No.)
Indication (revision No.)
1 2 3*
Distal to distal (1 + 2) Distal to distal Distal to proximal (1), proximal to distal (2) Proximal to distal (1), distal to proximal (2) Proximal to distal (1), distal to proximal (2) Proximal to distal Proximal to distal Proximal to distal Proximal to distal Proximal to distal Proximal to distal Proximal to proximal Proximal to proximal Proximal to transverse Transverse to transverse (1 + 2)
Fistula (1), MACE incontinence (2) Stomal stenosis Incomplete emptying (1), MACE incontinence (2) Prolonged elimination (1), MACE incontinence (2) Stomal stenosis (1), MACE incontinence (2) Stomal stenosis Prolonged elimination Stomal stenosis Prolonged elimination Prolonged elimination Prolonged elimination Prolonged elimination Stomal stenosis Stomal stenosis Ventral hernia obstructing MACE (1 + 2)
4 5 6 7 8 9 10 11 12 13 14 15
* Sequentially proximal and distal MACE.
REFERENCES 1. Malone PS, Ransley PG and Kiely EM: Preliminary report: the antegrade continence enema. Lancet 1990; 336: 1217.
and Malone antegrade continence enema reconstructive procedures in children. J Urol 2002; 168: 1537.
10. Malone PS, Curry JI and Osborne A: The antegrade continence enema procedure why, when and how? World J Urol 1998; 16: 274.
2. Roberts JP, Moon S and Malone PS: Treatment of neuropathic urinary and faecal incontinence with synchronous bladder reconstruction and the antegrade continence enema procedure. Br J Urol 1995; 75: 386.
6. Blackburn SC, Fishman JR, Geoghegan N et al: The first 5-year follow up of distal antegrade continence enema stomas. J Pediatr Urol 2012; 8: 17.
11. Yerkes EB, Cain MP, King S et al: The Malone antegrade continence enema procedure: quality of life and family perspective. J Urol 2003; 169: 320.
7. Dindo D, Demartines N and Clavien PA: Classification of surgical complications: a new proposal with evaluation in a cohort of 6336 patients and results of a survey. Ann Surg 2004; 240: 205.
12. Curry JI, Osborne A and Malone PS: How to achieve a successful Malone antegrade continence enema. J Pediatr Surg 1998; 33: 138.
3. Hensle TW, Reiley EA and Chang DT: The Malone antegrade continence enema procedure in the management of patients with spina bifida. J Am Coll Surg 1998; 186: 669. 4. Griffiths DM and Malone PS: The Malone antegrade continence enema. J Pediatr Surg 1995; 30: 68. 5. Clark T, Pope JC 4th, Adams MC et al: Factors that influence outcomes of the Mitrofanoff
8. Diamond DA and Pohl HG: Use of a colon based tubularized flap for an antegrade continence enema. J Urol 2003; 169: 324.
13. Herndon CD, Rink RC, Cain MP et al: In situ Malone antegrade continence enema in 127 patients: a 6-year experience. J Urol 2004; 172: 1689.
9. Liloku RB, Mure PY, Braga L et al: The left MontiMalone procedure: preliminary results in seven cases. J Pediatr Surg 2002; 37: 228.
14. Sinha CK, Butler C and Haddad M: Left Antegrade Continent Enema (LACE): review of the literature. Eur J Pediatr Surg 2008; 18: 215.
EDITORIAL COMMENT This review of proximal vs distal MACE procedures is unique in that it compares a single surgeon technical outcome together with a single institutional management protocol to compare overall success. The authors report several advantages for considering distal MACE. Several factors may influence the poorer results of right colon MACE. 1) Flush time duration is almost double in this group. There is no mention of additives that might decrease transit time, eg glycerin or sorbitol. We have found that the aggressive use of additives improves outcomes and shortens
flush time duration.1 2) The stool burden tends to increase with time despite active MACE irrigation. It may influence the duration and amount of irrigant required (right colon followup twice as long). 3) Only 25% of patients with proximal MACE flushed daily compared to almost 50% with distal MACE. This may increase the stool burden in those with proximal MACE and increase irrigation time. 4) Although the in situ vs the detached appendix did not show a significant difference in the stenosis rate, it clearly increased the overall stenosis rate for proximal MACE due to the expected
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DISTAL LEFT MALONE ANTEGRADE CONTINENCE ENEMA
higher complication rate associated with detaching the appendix from the colonic blood supply. 5) There is no mention of using the L stent,2 the MACE stopper or topical steroids to decrease the risk of stomal stenosis. Each could potentially lower the stenosis rate and improve the perceived outcome of proximal MACE. In a large series of more than 225 patients with MACE Bani-Hani et al
found a 14% stomal stenosis rate for the in situ appendix when these measures were aggressively used.3 Mark P. Cain Division of Pediatric Urology Indiana University Riley Hospital for Children at Indiana University Health Indianapolis, Indiana
REFERENCES 1. Bani-Hani AH, Cain MP, King K et al: Tap water irrigation and additives to optimize success with the Malone antegrade continence enema: the Indiana University algorithm. J Urol 2008; 180: 1757. 2. Michelson JJ, Yerkes EB, Meyer T et al: L stent for stomal stenosis in catheterizable channels. J Urol 2009; 182: 1786. 3. Bani-Hani AH, Cain MP, Kaefer M et al: The Malone antegrade continence enema: single institutional review. J Urol 2008; 180: 1106.
REPLY BY AUTHORS In our practice saline or polyethylene glycol is rarely added to the enema solution. One of these additives was added to 8 proximal and 2 distal MACEs. Enema regimens are determined jointly by parents, patient and providers, and are subject to variability. We acknowledge the challenges of comparing proximal to distal MACE in real world practice, where multiple variables in enema administration may confound results. It is unclear to what degree these differences in enema administration affected our
results, although the optimal MACE outcome likely depends on multiple factors. In addition, we agree that distal MACE followup is shorter than that of classic proximal MACE in our series. This is a product of introducing the former procedure more recently in the literature. Future studies may help better elucidate these issues. Finally, stomal stenosis is a real, frustrating challenge of the MACE procedure. MACE stoppers, L stents and steroids are not routinely used in our practice.
Purpose: The Malone antegrade continence enema is used for refractory fecal dysfunction in the pediatric neuropathic population. While various locations along the colon have been described, the optimal site for achieving efficient enemas remains unknown. We reviewed our experience with the Malone antegrade continence enema procedure and investigated functional outcomes and complications associated with proximal and distal locations. Materials and Methods: We performed an institutional review board approved, retrospective chart review of 109 consecutive MACE procedures done by a single surgeon from 2000 to 2012. Preoperative patient characteristics, intraoperative techniques and postoperative outcomes were reviewed and statistical analysis was performed. Results: Included in analysis were 90 patients treated with a total of 109 MACE procedures. Average age at operation was 13.8 years. Myelomeningocele was the most common diagnosis. Most patients underwent simultaneous urinary reconstruction. Stomal complications were most common (49% of patients). We compared the outcomes of proximal (cecal) MACE in 48 cases and distal (sigmoid colon) MACE in 55. Location did not affect the overall efficiency of fecal continence. Flush time was shorter for distal than for proximal MACE stomas (37.2 vs 61.2 minutes, p <0.001). Of the patients 15 underwent a total of 20 complete MACE revisions, including 12 proximal and 6 distal MACEs. As a channel, appendix was associated with a higher but not statistically significant stomal complication rate compared to colon (42% vs 25%). Conclusions: A cecal, transverse or colonic location for MACE results in acceptable outcomes. Distal MACE is associated with significantly shorter flush time and possibly fewer stomal complications. Stomal complications remain a frustrating reality of the MACE procedure.
Abbreviations and Acronyms BMI ¼ body mass index MACE ¼ Malone antegrade continence enema Accepted for publication January 24, 2013. Study received institutional review board approval. * Correspondence: 1500 East Medical Center Dr., Urology 3875TC 0330, Ann Arbor, Michigan 48109 (telephone: 734-615-3038; FAX: 734-6153520; e-mail: [email protected]).
Key Words: colon, sigmoid; fecal incontinence; enema; reconstructive surgical procedures; surgical stomas
PEDIATRIC urologists have long assumed the responsibility of managing urinary and fecal incontinence in patients with neuropathy. In these cases management of bowel dysfunction includes laxatives, enemas, digital stimulation and in extreme cases enteral reconstruction with the ultimate goals of quality of life and independence.
The MACE, one such reconstructive option, was first described in 1990 to manage intractable fecal incontinence.1 The original technique relied on the appendicocecal junction with the distal tip of the appendix brought to the abdominal wall as a continent catheterizable stoma. This technique can be performed simultaneously with urinary reconstruction
0022-5347/13/1904-1529/0 THE JOURNAL OF UROLOGY® © 2013 by AMERICAN UROLOGICAL ASSOCIATION EDUCATION AND RESEARCH, INC.
http://dx.doi.org/10.1016/j.juro.2013.01.092 Vol. 190, 1529-1534, October 2013 Printed in U.S.A.
www.jurology.com
j
1529
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with good results.2 It has high patient satisfaction in the spina bifida population.3 While most patients do well with the MACE procedure, a subset struggles with persistent incontinence, protracted enema transit time, severe colic or the need for stomal revision.4 Younger age at operation and poor patient compliance predict worse outcomes.5 However, other factors predictive of operative complications and enema function are poorly defined. Furthermore, the efficacy of distal left MACE was proposed but not fully explored compared to standard MACE using the proximal right appendicocecal junction.6 We describe our experience with MACE using traditional right vs left reconstruction to evaluate our hypothesis that left MACE may have functional advantages.
to a poor functional outcome of the initial procedure. 2) Distal MACE was created in patients with a history of severe chronic constipation and intraoperative findings of a dilated, tortuous distal colon. 3) A few patients had severe right colonic adhesions, making cecal MACE technically challenging. For appendicocecal MACE the appendix channel was imbricated in situ or detached and reimplanted using the tenia submucosal tunnel reimplantation technique for the continence mechanism. For distal transverse and sigmoid colon MACE a catheterization conduit was reconstructed using an ileal or colon Yang-Monti channel and implanted using the tenia submucosal tunnel. After the procedure, an indwelling catheter remained in the stoma for 3 weeks postoperatively. MACE flushing and instruction were initiated through the existing indwelling catheter by specialty urology nurses before patient discharge home.
Followup
METHODS Patient Accrual We performed an institutional review board approved, retrospective chart review of all patients treated with a MACE procedure by a single surgeon (JMP) from December 2000 to December 2012. Indications for surgery were neuropathic bowel with fecal incontinence and/or severe constipation with the overall aim of independent bowel management. Patient age and BMI, if available, at operation were recorded. We also documented the bowel regimen most recently performed before MACE reconstruction, defined as none, conservative (oral agents such as laxatives or stool softeners), aggressive (rectal stimulation via suppositories, enemas, digital stimulation and/or manual disimpaction) or previous MACE procedures. Postoperatively we assessed the volume of enema fluid, washout time and fecal continence. Data points were missing in 38 patient charts. The table shows the missing data points for enema function. The remaining missing data points pertained to BMI. Data points on stomal use and function were taken from the last relevant note in the chart on that stoma. In the case of complete MACE reconstruction separate data points were used so that the total data points for stomal use and function totaled 109 and not 90.
Procedure An in-house or home preoperative bowel preparation was prescribed. The choice of bowel segment for catheterization conduit (appendix, ileum or colon) and stomal location (cecum, or transverse or sigmoid colon) were determined intraoperatively at attending surgeon discretion based on the need for additional urinary reconstruction and other anatomical considerations. Since the study was a retrospective review, no prospective algorithms were routinely followed intraoperatively to determine whether distal or proximal MACE would be performed. The attending surgeon preferentially created distal MACE in several instances. 1) A few cases of initial cecal MACE were intentionally converted to distal MACE due
Patients were followed annually with assessment of enema use and function, including patient reported complaints, continence, enema frequency and volume, flush time and enema additives, and stomal examination. Complications were recorded as described by Dindo et al.7 They were further subdivided into minor and major complications (Clavien score 2 or less and 3 or greater), and early and late (before and after 30 days, respectively). Stomal stenosis was defined as stenosis requiring operative intervention, superficial dilation or revision in the operating room, or complete MACE revision. We further analyzed cases of complete MACE revision.
Statistical Analysis All reported averages are the mean unless otherwise indicated. Subanalysis was performed to compare the type of bowel regimen before surgery, enema type and location, and patients undergoing revision. Continuous data were analyzed using the 2-tailed unpaired t test or ANOVA, as appropriate. Categorical data were analyzed using the chi-square test. All statistical analysis was hypothesis driven and performed using PrismÒ, version 5.0.
RESULTS In 90 patients a total of 109 MACE stomas were created. Presence of a ventriculoperitoneal shunt, ability to self-catheterize, ambulatory status, simultaneous urinary reconstruction, preoperative bowel program or use of inpatient bowel preparation did not vary significantly by MACE location (data not shown). Overall, most patients had a diagnosis of myelomeningocele (74), while the remainder had imperforate anus (4), sacral agenesis (2), spinal cord injury (4) or another condition, including spinal cord aneurysm, posterior urethral valves, cloacal extrophy and bladder extrophy (6). This distribution did not vary by MACE location (data not shown). Patients who received a right cecal stoma were younger at operation than those who received
DISTAL LEFT MALONE ANTEGRADE CONTINENCE ENEMA
transverse and sigmoid colon stomas (12.1 vs 17.9 and 14.9 years, respectively, p ¼ 0.005). Functional outcomes included patient reported fecal continence, flush time and volume, and enema agent and schedule. Incomplete functional outcomes were recorded in 38 of 109 medical records, such as flush time, or enema type or volume, including the charts of 6 patient with a right MACE and 3 with a left MACE who reported not using the enema due to discomfort, inconvenience or difficulty. Average flush time was 48 minutes and the average volume used was 886 ml. Subanalysis comparisons were made by organizing groups by bowel regimen before surgery, segment type used for the catheterization conduit (appendix, ileum or colon) and MACE colonic site. The table lists outcomes stratified by MACE location. Flush time was notably shorter for sigmoid vs transverse vs cecal stomas (p ¼ 0.002). Enema frequency and additives did not vary significantly among these groups (see table). Comparison of the type of conduit (appendix, ileum or colon) mirrored MACE site results since appendicocecal stomas are always on the right side and colonic stomas always on the left side. Results did not significantly differ by preoperative bowel regimen (data not shown). Followup was longer in the proximal MACE group (see table). At least 1 complication developed in 54 patients for a total of 70 complications (see table). Reoperation was indicated for stomal stenosis, stomal leakage, enterocutaneous fistula, failed prolonged elimination, bowel obstruction, incisional hernia, MACE conduit false passage and wound abscess. The single Clavien grade 4a complication was a perioperative aspiration event requiring postoperative intensive care monitoring. Stomal complications, including stomal stenosis, incontinence via the stoma, perforation, fistula and prolapse, developed in 34 cases. The distribution of the specific type of stomal complication was similar among the groups. Average time to initial stomal stenosis was 19.7 months (range 3 to 48). Right stomas showed a higher rate of stomal complications, although this did not attain statistical significance. Distal left MACE stomas had a higher rate of minor complications than others (p ¼ 0.04, see table). Notably, there was a higher rate of stomal stenosis for the detached appendicocecostomy technique than for the in situ appendicocecostomy technique, although again this did not attain statistical significance (7 patients or 41% vs 5 or 26%, p ¼ 0.48). Included in these complications were 15 patients who underwent a total of 20 complete MACE revisions. The Appendix lists revision sites and indications. Of the patients 12 (25%) treated with proximal right MACE required revision for
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Functional outcomes and complications by MACE location MACE Location Cecum No. pts No. continued fecal incontinence (%) No. MACE stomal leakage (%) No. no longer using MACE (%) Mean SD flush time (mins) Mean SD enema vol (ml) No. enema schedule (%): Daily Every other day Other Not available No. agent used (%): Tap water only Soapsuds enema Tap water þ other Not available Mean/median followup (mos) No. pts with complications (%) No. complication type (%): Stomal Infectious Gastrointestinal Pulmonary Incisional Other No. Clavien grade (%):* 1 2 3b 4a No. timing (%): Early Late
48 2
(4)
Transverse Colon 6 2 (33)
2 (4) 0 6 (13) 0 61.2 35.8 45 21.2 815 328 990 317 12 22 7 6
(25) (46) (15) (12.5)
3 (50) 1 (17) 0 2 (33)
Sigmoid Colon 55 1 (2) 3 (6) 3 (6) 37.2 16.4 938 625 27 18 4 6
(49) (33) (7) (11)
34 (71) 0 8 (17) 6 (12) 68.9/70.2 24 (50)
5 (83) 0 0 1 (17) 47.9/24.7 2 (33)
46 (84) 1 (2) 2 (3) 6 (11) 30.4/31.3 28 (51)
20 (42) 4 (8) 9 (19) 1 (2) 0 1 (2)
0 0 4 (67) 0 2 (33) 0
14 8 11 0 0 4
4 (13) 4 (13) 23 (71) 1 (3)
0 0 2 (100) 0
2 (6) 14 (38) 20 (56) 0
9 (28) 23 (72)
0 2 (100)
19 (53) 17 (47)
(25) (15) (20) (7)
* No patient had Clavien grade 3a, 4b or 5 complications.
prolonged elimination in 6, stomal stenosis in 5 and incontinence from MACE in 1. Six patients (11%) treated with distal left MACE required revision due to incontinence from MACE (3), fistula (1), stomal stenosis (1) and incomplete emptying (1). Comparison of conversions required for proximal and distal MACE trended toward significance in favor of distal left MACE (p ¼ 0.07). One patient currently has a distal and a proximal MACE, given the ineffectiveness of each location alone. Age at operation, preoperative diagnosis, BMI and bowel segment type or site did not appear to influence the need for reoperation (data not shown).
DISCUSSION The pediatric urologist often treats neurogenic bladder as part of the overall care for elimination disorders using laxatives, enemas, suppositories and in severe cases the creation of an antegrade enema. The MACE seeks to provide an easily accessible stoma for antegrade enema administration, although techniques vary among surgeons. Since the first description of appendicocecostomy by
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Malone et al,1 multiple methods of conduit creation have been described as well as different locations along the course of the colon. Others suggested that distal MACE may provide shorter elimination time.5,8e10 While patient and family satisfaction is high,11 complications exist, especially those involving the stoma and treatment failure. Patient age, compliance and enema location affect enema performance, although comparisons are limited to case series of a single enema technique.2,5,6 We took advantage of the change from proximal to distal MACE at our institution by comparing our experience with the 2 techniques. In our series the distribution of distal and proximal MACE was approximately equal. All except 9 of the 90 patients (90%) still used the MACE regularly at a median followup of 42 months. Stomal complications developed most frequently at about a third of the time. Distal stomas were associated with shorter flush time. Of the original proximal right MACE cases 25% were converted to a distal location due to ineffective enema results. This is consistent with the observations of Curry et al that 9 of 31 patients with appendicocecostomy were troubled with washout failure.12 In our experience these patients are well served by conversion from proximal to distal MACE. Patient characteristics or physiological studies to aid in the preoperative identification of patients who may benefit from primary distal left MACE are lacking. As noted, abundant right lower quadrant adhesions, a severely dilated distal colon or previously ineffective proximal MACE were considerations for distal MACE creation. Our subanalysis of the impact of a preoperative bowel regimen as a surrogate indicator of colonic motility did not provide a useful clue. The limitations of our study deserve mention. Selection and followup biases are unavoidable in retrospective reviews. The study group was heterogeneous, although heterogeneity appeared evenly distributed among MACE sites. Data on the specific preoperative bowel regimen and the evaluation of enema function were limited to patient reports in the chart review with complete information not always available. Standardized methods of reporting continence outcomes are lacking. As such, continence was based on self-reported or parent reported outcomes without preoperative or postoperative physiological evaluation. Intraoperative decisions involving stomal location and type were based on various patient and anatomical factors, as described. Such decisions are case specific and possibly not generalizable. These limitations aside, we believe that our experience adds to a growing body of evidence
supporting MACE in select individuals with neuropathic bowel. Our findings reinforce the high continence rates previously reported in the literature.13 Our study includes a fairly even distribution of proximal right cecal vs distal left MACE locations, allowing for comparison between the methods. Right appendicocecostomy MACE is a less complicated procedure, which may account for its lower rate of early postoperative complications. However, this observation is limited by the fact that most cases were done with simultaneous urinary reconstruction, adding extra complexity to the procedure. As reported in other studies,6,14 distal stomas had a shorter flush time than proximal stomas. While stomal complications did not differ statistically between the groups, there was a trend toward a lower rate of stomal complications for left distal MACE. The use of detached appendicocecostomy may contribute to a higher stomal stenosis rate, although the difference between the 2 techniques for appendicocecostomy did not attain statistical significance. Appendicocecal stomas may be more susceptible to minor wound infections progressing to stenosis due to the fecal bacterial content in that bowel area. However, we cannot exclude followup bias with a shorter mean followup in patients with left distal MACE. To our knowledge the impact of preoperative bowel regimens has not been studied in relation to postoperative MACE function. Indications for the MACE procedure include the failure of adequate bowel programs. However, the appropriate aggressiveness of bowel programs before MACE reconstruction has yet to be defined. The concomitant need for complex urological reconstruction may drive the decision for patients with borderline bowel function to undergo a MACE procedure. In our series most patients had undergone trials of enemas, suppositories or digital stimulation without satisfaction. However, many patients had been involved in no formal bowel regimen or in a bowel regimen consisting of only oral stool softeners or laxatives. Of such patients those who had received only oral agents appeared to have a lower complication rate postoperatively, suggesting a potential role for early surgical intervention in those requiring an aggressive bowel program via the rectum. Patients who received rectal agents may have had more severe fecal incontinence and constipation, complicating analysis. Ideally, a prospective study would eliminate these confounding variables, although the feasibility of such a study remains limited.
CONCLUSIONS The MACE procedure is generally successful. It remains a useful adjunct in the management of
DISTAL LEFT MALONE ANTEGRADE CONTINENCE ENEMA
neurogenic fecal incontinence and severe constipation. Patient selection, close postoperative followup and continued education by well informed clinical staff are essential to appropriate MACE maintenance, and patient and family satisfaction. Proximal right and distal left MACE locations result in acceptable outcomes, although left sigmoid stomas are associated with a shorter flush time and may have fewer stomal complications. Up to 20% of patients with a proximal right stoma experience prolonged elimination and abdominal cramping, and may benefit from conversion to a distal left MACE. Stomal complications, especially stomal stenosis, remain a frustrating reality of the MACE procedure. While these issues often present within the first several years after enema creation, they may present later, underscoring the need for continued followup of these patients by a knowledgeable practitioner.
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APPENDIX Complete MACE revisions with location change, if any, and indication Pt. No.
Location (revision No.)
Indication (revision No.)
1 2 3*
Distal to distal (1 + 2) Distal to distal Distal to proximal (1), proximal to distal (2) Proximal to distal (1), distal to proximal (2) Proximal to distal (1), distal to proximal (2) Proximal to distal Proximal to distal Proximal to distal Proximal to distal Proximal to distal Proximal to distal Proximal to proximal Proximal to proximal Proximal to transverse Transverse to transverse (1 + 2)
Fistula (1), MACE incontinence (2) Stomal stenosis Incomplete emptying (1), MACE incontinence (2) Prolonged elimination (1), MACE incontinence (2) Stomal stenosis (1), MACE incontinence (2) Stomal stenosis Prolonged elimination Stomal stenosis Prolonged elimination Prolonged elimination Prolonged elimination Prolonged elimination Stomal stenosis Stomal stenosis Ventral hernia obstructing MACE (1 + 2)
4 5 6 7 8 9 10 11 12 13 14 15
* Sequentially proximal and distal MACE.
REFERENCES 1. Malone PS, Ransley PG and Kiely EM: Preliminary report: the antegrade continence enema. Lancet 1990; 336: 1217.
and Malone antegrade continence enema reconstructive procedures in children. J Urol 2002; 168: 1537.
10. Malone PS, Curry JI and Osborne A: The antegrade continence enema procedure why, when and how? World J Urol 1998; 16: 274.
2. Roberts JP, Moon S and Malone PS: Treatment of neuropathic urinary and faecal incontinence with synchronous bladder reconstruction and the antegrade continence enema procedure. Br J Urol 1995; 75: 386.
6. Blackburn SC, Fishman JR, Geoghegan N et al: The first 5-year follow up of distal antegrade continence enema stomas. J Pediatr Urol 2012; 8: 17.
11. Yerkes EB, Cain MP, King S et al: The Malone antegrade continence enema procedure: quality of life and family perspective. J Urol 2003; 169: 320.
7. Dindo D, Demartines N and Clavien PA: Classification of surgical complications: a new proposal with evaluation in a cohort of 6336 patients and results of a survey. Ann Surg 2004; 240: 205.
12. Curry JI, Osborne A and Malone PS: How to achieve a successful Malone antegrade continence enema. J Pediatr Surg 1998; 33: 138.
3. Hensle TW, Reiley EA and Chang DT: The Malone antegrade continence enema procedure in the management of patients with spina bifida. J Am Coll Surg 1998; 186: 669. 4. Griffiths DM and Malone PS: The Malone antegrade continence enema. J Pediatr Surg 1995; 30: 68. 5. Clark T, Pope JC 4th, Adams MC et al: Factors that influence outcomes of the Mitrofanoff
8. Diamond DA and Pohl HG: Use of a colon based tubularized flap for an antegrade continence enema. J Urol 2003; 169: 324.
13. Herndon CD, Rink RC, Cain MP et al: In situ Malone antegrade continence enema in 127 patients: a 6-year experience. J Urol 2004; 172: 1689.
9. Liloku RB, Mure PY, Braga L et al: The left MontiMalone procedure: preliminary results in seven cases. J Pediatr Surg 2002; 37: 228.
14. Sinha CK, Butler C and Haddad M: Left Antegrade Continent Enema (LACE): review of the literature. Eur J Pediatr Surg 2008; 18: 215.
EDITORIAL COMMENT This review of proximal vs distal MACE procedures is unique in that it compares a single surgeon technical outcome together with a single institutional management protocol to compare overall success. The authors report several advantages for considering distal MACE. Several factors may influence the poorer results of right colon MACE. 1) Flush time duration is almost double in this group. There is no mention of additives that might decrease transit time, eg glycerin or sorbitol. We have found that the aggressive use of additives improves outcomes and shortens
flush time duration.1 2) The stool burden tends to increase with time despite active MACE irrigation. It may influence the duration and amount of irrigant required (right colon followup twice as long). 3) Only 25% of patients with proximal MACE flushed daily compared to almost 50% with distal MACE. This may increase the stool burden in those with proximal MACE and increase irrigation time. 4) Although the in situ vs the detached appendix did not show a significant difference in the stenosis rate, it clearly increased the overall stenosis rate for proximal MACE due to the expected
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DISTAL LEFT MALONE ANTEGRADE CONTINENCE ENEMA
higher complication rate associated with detaching the appendix from the colonic blood supply. 5) There is no mention of using the L stent,2 the MACE stopper or topical steroids to decrease the risk of stomal stenosis. Each could potentially lower the stenosis rate and improve the perceived outcome of proximal MACE. In a large series of more than 225 patients with MACE Bani-Hani et al
found a 14% stomal stenosis rate for the in situ appendix when these measures were aggressively used.3 Mark P. Cain Division of Pediatric Urology Indiana University Riley Hospital for Children at Indiana University Health Indianapolis, Indiana
REFERENCES 1. Bani-Hani AH, Cain MP, King K et al: Tap water irrigation and additives to optimize success with the Malone antegrade continence enema: the Indiana University algorithm. J Urol 2008; 180: 1757. 2. Michelson JJ, Yerkes EB, Meyer T et al: L stent for stomal stenosis in catheterizable channels. J Urol 2009; 182: 1786. 3. Bani-Hani AH, Cain MP, Kaefer M et al: The Malone antegrade continence enema: single institutional review. J Urol 2008; 180: 1106.
REPLY BY AUTHORS In our practice saline or polyethylene glycol is rarely added to the enema solution. One of these additives was added to 8 proximal and 2 distal MACEs. Enema regimens are determined jointly by parents, patient and providers, and are subject to variability. We acknowledge the challenges of comparing proximal to distal MACE in real world practice, where multiple variables in enema administration may confound results. It is unclear to what degree these differences in enema administration affected our
results, although the optimal MACE outcome likely depends on multiple factors. In addition, we agree that distal MACE followup is shorter than that of classic proximal MACE in our series. This is a product of introducing the former procedure more recently in the literature. Future studies may help better elucidate these issues. Finally, stomal stenosis is a real, frustrating challenge of the MACE procedure. MACE stoppers, L stents and steroids are not routinely used in our practice.