IN SITU MALONE ANTEGRADE CONTINENCE ENEMA IN 127PATIENTS: A 6-YEAR EXPERIENCE

C. D. ANTHONY HERNDON, RICHARD C. RINK,* MARK P. CAIN, MICHELLE LERNER,MARTIN KAEFER, ELIZABETH YERKES AND ANTHONY J. CASALE

ABSTRACT

Purpose: The initial description of the Malone antegrade continence enema (MACE) relied ona reversed, tunneled and reimplanted appendix. In 1999 we reported our in situ technique thatuses windows developed in the appendiceal mesentery for imbrication. We present our long-termresults.Materials and Methods: From 1997 to 2003, 168 patients were identified who had undergone

a MACE procedure. An in situ technique was performed in 76 females and 51 males. Averagepatient age at the time of surgery was 9.6 years (range 2.9 to 28.4). Diagnoses includedmyelomeningocele in 116 cases, lipomeningocele in 6, spinal cord injury in 2, posterior urethralvalves in 1, sacral agenesis in 1 and functional constipation in 1.Results: Cecal plication/imbrication was performed in 100 patients, appendix intussusception

and imbrication in 24, and creation of tenia flaps in 3. The abdominal stoma was umbilical in 50cases, right lower quadrant in 74 and periumbilical in 3. Concomitant genitourinary reconstruc-tion was performed in 87% of patients. Mean followup was 26.9 months (range 0.7 to 68.1). Fecalcontinence was reported by 91% of the patients. Thirteen stomal revisions (stenosis 10, prolapse2 and leakage 1) were required in 11 patients. Major complications included a cecal volvulusrequiring a right hemicolectomy in 1 patient, small bowel obstruction in 2, and shunt infectionand/or malfunction in 2. Four patients have elected to no longer use the MACE for non-technicalreasons.Conclusions: The in situ MACE procedure has reliable long-term results for treating fecal

incontinence associated with neuropathic bowel.KEY WORDS: fecal incontinence, constipation, enema

In 1990 Malone et al revolutionized the treatment of in-tractable constipation in patients with neuropathic boweland/or anorectal malformations with the description of theantegrade continence enema.1 Since the initial description,the procedure has proven to be reproducible at multiple cen-ters.2–20 Moreover, a clear improvement in quality of life hasbeen seen in these patients who suffer from neuropathicbowel.7, 20The original description of the Malone antegrade conti-

nence enema (MACE) procedure involved amputation andreimplantation of the reversed appendix into the tenia of thecolon. The main impetus for this was fear that catheteriza-tion would be difficult if the appendix was folded back onitself and left in situ.1 Other centers tried an in situ tech-nique that involved imbricating the appendiceal base withinthe cecum.2 In 1999 we presented the initial results of our insitu technique which involved imbrication through the ap-pendiceal mesentery.8 We present our 6-year experience.

MATERIALS AND METHODS

From 1997 to 2003, the records of 168 patients who hadundergone a MACE procedure were reviewed in a retrospec-tive manner. An in situ technique was performed in 76 fe-males and 51 males. Average patient age at the time ofsurgery was 9.6 years (range 2.9 to 28.4). Diagnoses includedmyelomeningocele in 116 cases, lipomeningocele in 6, spinalcord injury in 2, posterior urethral valves in 1, sacral agen-esis in 1 and functional constipation in 1. The initial ap-proach to the neuropathic bowel involves treatment with

conservative measures, including bulking agents and MiraLax (Brain Tree Laboratories, Brain Tree, Massachusetts).Patients in whom these conservative measures fail are con-sidered candidates for the MACE procedure.The in situ technique is used as an isolated procedure or in

conjunction with genitourinary reconstruction. The patient isadmitted the day before surgery and a full antibiotic (ampi-cillin/naficillin and gentamicin) and mechanical bowel prep-aration (GOLYTELY, Brain Tree Laboratories) is instituted.A midline incision is made with care taken to leave a widefascial pedicle lateral to the umbilicus for possible stomalplacement. If present, the ventriculoperitoneal shunt is iso-lated and wrapped in an antibiotic soaked laparotomy pad.The cecum is then mobilized. After adequate length is ob-tained to bring the cecum to the level of the umbilicus or theright lower quadrant abdominal wall appendiceal integrity isassessed. A silk suture is placed in the tip of the appendix,and the tip is amputated and catheterized with a 12 or 14Frnonlatex catheter.Windows are developed in the appendiceal mesentery to

allow imbrication of the cecum (fig. 1). Then 3 to 4 imbricat-ing sutures of 4-zero silk are placed through the appendicealmesenteric windows grasping serosa of cecum, appendix andserosa of cecum (fig. 2). The imbrication is performed withcare to avoid injury to the appendiceal blood supply. Afterconfirmation of ease of catheterization, attention is placed todeveloping the abdominal wall stoma.A V-flap is the technique of choice at our institution. The

site of the MACE stoma varies from umbilicus to right lowerquadrant. The site of the V-flap is based a point where thecolon easily reaches the anterior abdominal wall. The cecumis then secured to the anterior abdominal wall with nonab-sorbable sutures. Urinary reconstruction is completed at this

* Correspondence: Riley Hospital for Children, 702 Barnhill Drive,Indianapolis, Indiana 46202 (telephone: 317-274-7472; FAX: 317-274-7481; e-mail: rrink@inpui.edu).

0022-5347/04/1724-1689/0 Vol. 172, 1689–1691, October 2004THE JOURNAL OF UROLOGY® Printed in U.S.A.Copyright © 2004 by AMERICAN UROLOGICAL ASSOCIATION DOI: 10.1097/01.ju.0000138528.55602.20

1689

time if required. A 12Fr nonlatex catheter is left indwellingafter abdominal wall closure and remains for 3 weeks post-operatively.Irrigation of the colon is begun on postoperative day 4 with

50 cc tap water and increased by 50 cc a day until the desiredeffect is obtained. Some patients have required introductionof motility agents such as GOLYTLEY or mineral oil tofacilitate evacuation. Patients irrigate the colon in theevening and typically evacuate during a 1-hour period. Pa-tients are asked to intubate the channel twice daily eventhough irrigations are done nightly. In some situations weleave a catheter indwelling for a week.

RESULTS

Mean followup is 26.9 months (range 0.7 to 68.1). Cecalplication/imbrication was performed in 100 patients, appen-dix intussusception and imbrication in 24 and creation oftenia flaps in 3. The abdominal stoma was umbilical in 50cases, right lower quadrant in 74 and periumbilical in 3.Concomitant genitourinary reconstruction was performed in87% of patients. Fecal continence was reported to the physi-cian by 91% of the patients.Thirteen stomal revisions (stenosis 10, prolapse 2 and leak-

age 1) were required in 11 patients. Major complicationsincluded a cecal volvulus requiring a right hemicolectomy in1 patient, small bowel obstruction in 2, shunt infection and/ormalfunction in 2 and a stitch abscess in 1. Four patientselected to no longer use the MACE for nontechnical reasons(due to intractable abdominal cramping with irrigationwhich was refractory to pharmacological manipulation in 1).

DISCUSSION

Since the initial description, the MACE procedure has beensuccessfully used at numerous institutions.1–20 Before itsinception, some children who were made continent of urineremained in diapers due to intractable constipation and fecalsoiling. Malone et al revolutionized the care of these patientswith the introduction of the MACE procedure.1 A clear im-provement in quality of life has been demonstrated in pa-tients treated with the MACE procedure for neuropathicbowel.7, 20To simplify the technique, we elected to perform the pro-

cedure with an in situ appendix which was imbricatedthrough appendiceal mesenteric windows. Almost uniformlywe have not had the need to split the tenia and bury theappendix.8 In situations when the appendix is too short, wenow consider a stapled cecal extension technique as origi-nally described by Hensle et al.15The overall fecal continence rate after the MACE proce-

dure varies significantly in the literature. The fecal conti-nence rate in our series compares favorably to the literaturewhich ranges from 57% to 100% (see table). Factors thatappear to impact this rate are age and diagnosis.5 The largestcollective series reports a success rate of 79%.16 Intractableconstipation demonstrated the least benefit after the MACEprocedure with a 38% failure rate. In a blinded survey, witha response rate of 71%, patients reported a fecal continencerate of 77%. In our study continence was defined stringentlyas the absence of leakage during a 1-year interval.20The overall complication rate for our series was 13%. The

most common complication involved stomal revision whichoccurred in 9% of the patients. Critical stomal stenosis de-veloped in 6% of our population, and re-stenosis developed in

FIG. 1. Intraoperative photograph and line drawing demonstratewindows created in appendiceal mesentery.

FIG. 2. Intraoperative photograph and line drawing demonstrateimbrication of cecum within windows created in appendiceal mesen-tery.

Reported series of MACE

References No. Stomal Revisions/Total No. No. Fecal Continence/Total No.

Squire et al2 Stenosis 3/25, difficult catheterizing 1/25 Complete 17/25, partial 7/25Koyle et al3 Stenosis 2/20*, gas leak 3/20 Complete 17/22, partial 3/22Hill et al4 Stenosis 3/6 Complete 4/6, improved 2/6Griffiths and Malone5 Stenosis 5/21, breakdown 5/21, closed over 1/21 Complete 12/21, partial 3/21Roberts et al6 Stenosis 2/8 Complete 5/8, improved 3/8Ellsworth et al7 Necrosis 1/18, stomal stenosis 2/18, minor stomal narrowing 3/18 18/18Wilcox and Kiely9 Stomal stenosis 9/30* 28/36Levitt et al10 Leakage 1/13,* stomal stenosis 2/20 19/20Mor et al11 Stenosis 4/10 15/18‡Sheldon et al12 No complications 10/10Hensle et al15 Stenosis 5/27, stomal granulations 5/27 19/27Curry et al16 Stomal stenosis 30% Success 79%Tam17 No complications Excellent/good 12/12Van Savage and Yohannes18 Stomal stenosis 1 16/16Clark et al19 Stomal stenosis 19%† 19/20

* Includes 1 appendicocecostomy.† Includes bladder and cecal procedures.‡ Includes appendix and cecal flaps.

MALONE ANTEGRADE CONTINENCE ENEMA1690

2. Historically, stomal stenosis rates have ranged from 0% to40% (see table). The United Kingdom cohort reported a com-bined rate of 30% for stomal stenosis.16 However, not allpatients in that series had an in situ appendix MACE per-formed.16 It is our belief that 2 main factors have allowed usto avoid this complication in most patients. First, by perform-ing an in situ technique, preservation of the cecal bloodsupply to the base of the appendix is achieved, thereby lim-iting its dependence on an isolated appendiceal mesentery.Second, we have opted to perform a wide based V-flap for ourstoma reconstruction. If stenosis develops then we initiallyleave a catheter indwelling and if this fails we would proceedwith stomal revision. The other uncommon complication thatoccurred in our series was a cecal volvulus, a rare complica-tion that has been reported previously.5, 18 As a testament tothe improved quality of life with the MACE procedure, thisfamily and child elected to proceed with a transverse colonflap MACE channel. Although this complication is exceed-ingly rare, a high index of suspicion is warranted in allpatients who present with symptoms of gastrointestinal dis-tress.

CONCLUSIONS

In our experience the in situ MACE procedure is a reliablemeans to treat the neuropathic bowel that can be safelyperformed in conjunction with other major genitourinary re-constructive procedures. It achieves fecal continence in mostpatients, although infrequently it may require stomal revi-sion.

REFERENCES

1. Malone, P. S., Ransley, P. G. and Kiely, E. M.: Preliminaryreport: the antegrade continence enema. Lancet, 336: 1217,1990

2. Squire, R., Kiely, E. M., Carr, B., Ransley, P. G. and Duffy, P. G.:The clinical application of the Malone antegrade colonic en-ema. J Pediatr Surg, 28: 1012, 1993

3. Koyle, M. A., Kaji, D. M., Duque, M., Wild, J. and Galansky,S. H.: The Malone antegrade continence enema for neurogenicand structural fecal incontinence and constipation. J Urol,154: 759, 1995

4. Hill, J., Stott, S. and MacLennan, I.: Antegrade enemas for thetreatment of severe idiopathic constipation. Br J Surg, 81:1490, 1994

5. Griffiths, D. M. and Malone, P. S.: The antegrade continenceenema. J Pediatr Surg, 30: 68, 1995

6. Roberts, J. P., Moon, S. and Malone, P. S.: Treatment of neuro-

pathic urinary and faecal incontinence with synchronous blad-der reconstruction and the antegrade continence enema pro-cedure. Br J Urol, 75: 386, 1995

7. Ellsworth, P. I., Webb, H. W., Crump, J. M., Barraza, M. A. andStevens, P. S.: The Malone antegrade colonic enema enhancesthe quality of life in children undergoing urological inconti-nence procedures. J Urol, 155: 1416, 1996

8. Rink, R. C., Casale, A. J., Cain, M. P. and Kind, S. J.: In situimbricated appendix: experience with simple MACE tech-nique. J Urol, suppl., 161: 589, 1999

9. Wilcox, D. T. and Kiely, E. M.: The Malone (antegrade conti-nence enema) procedure: early experience. J Pediatr Surg, 33:204, 1998

10. Levitt, M. A., Soffer, S. Z. and Pena, A.: Continent appendicos-tomy in the bowel management of fecally incontinent children.J Pediatr Surg, 32: 1630, 1997

11. Mor, Y., Quinn, F. M. J., Carr, B., Mouriquand, P. D., Duffy,P. G. and Ransley, P. G.: Combined Mitrofanoff and antegradecontinence enema procedures for urinary and fecal inconti-nence. J Urol, 158: 192, 1997

12. Sheldon, C. A., Minevich, E., Wacksman, J. and Lewis, A. G.:Role of the antegrade continence enema in the management ofthe most debilitating childhood rectourogenital malforma-tions. J Urol, 158: 1277, 1997

13. Graf, J. L., Strear, C., Bratton, B., Housley, H. T., Jennings,R. W., Harrison, M. R. et al: The antegrade continence enemaprocedure: a review of the literature. J Pediatr Surg, 33: 1294,1998

14. Gerharz, E. W., Vik, V., Webb, G. and Woodhouse, C. R. J.: Thein situ appendix in the Malone antegrade continence enemaprocedure for faecal incontinence. Br J Urol, 79: 985, 1997

15. Hensle, T. W., Reiley, E. A. and Chang, D. T.: The Maloneantegrade continence enema procedure in the management ofpatients with spina bifida. J Am Coll Surg, 186: 669, 1998

16. Curry, J. I., Osborne, A. and Malone, P. S.: The MACE proce-dure: experience in the United Kingdom. J Pediatr Surg, 34:338, 1999

17. Tam, P. K. H.: Y-appendicoplasty: a technique to minimizestomal complications in antegrade continence enema. J Pedi-atr Surg, 34: 1733, 1999

18. Van Savage, J. G. and Yohannes, P.: Laparoscopic antegradecontinence enema in situ appendix procedure for refractoryconstipation and overflow fecal incontinence in children withspina bifida. J Urol, 164: 1084, 2000

19. Clark, R., Pope, J. C., Adams, M. C., Wells, N. and Brock, J. W.:Factors that influence outcomes of the Mitrofanoff and Maloneantegrade continence enema reconstructive procedures in chil-dren. J Urol, 168: 1537, 2002

20. Yerkes, E. B., Cain, M. P., King, S., Brei, T., Kaefer, M., Casale,A. J. et al: The Malone antegrade continence enema procedure:quality of life and family perspective. J Urol, 169: 320, 2003

DISCUSSION

Dr. Mark Cain. Since the cecum is a right lower quadrant structure, how do you decide where to put the stoma,the umbilicus versus the right lower quadrant? Why not just put them all in the right lower quadrant?

Dr. Anthony Herndon. At Indiana it is by surgeon preference. In our series there was no difference incomplications whether the location was umbilical or the right lower quadrant.

Doctor Cain. Most of those patients have a Malone procedure performed at the time of reconstruction withbladder augmentation and Rink taught us all to open the bladder and put the Mitrofanoff down in the right lowerquadrant. The bladder is opened as a clam shell, the suprapubic tube is placed in the left lower quadrant andmost of the MACE stomas are being placed in the umbilicus because of that.

MALONE ANTEGRADE CONTINENCE ENEMA 1691