The Malone Antegrade Continence Enema Procedurein the Management of Patients with Spina Bifida
Terry W Hensle, MD, FACS,* Elizabeth A Reiley, MD, and David T Chang, MD*
Background: In patients with spina bifida, traditionalbowel management programs such as suppositories, ret-rograde enemas, and manual disimpaction have beenlargely unsatisfactory. The Malone antegrade conti-nence enema (ACE) procedure has largely changed ourapproach to bowel management in this patient group.
Study Design: Over a 3-year period between January1994 and January 1997, 27 patients with spina bifidaunderwent the Malone ACE procedure at our institu-tions. At the time of their ACE procedure, four patientsunderwent simultaneous continent urinary diversionand three had simultaneous small-bowel bladder aug-mentation. All the patients were evaluated for 9 monthsor more after their procedure, and 10 of the patientshave been followed for more than 2 years.
Results: Postoperatively, predictable bowel control andcontinence were achieved in 19 of the 27 patients, but 6had some rectal soiling requiring a sanitary pad. Allpatients were out of diapers and none reported stomalleakage. Eighteen of the 27 patients were able to manageindependently and 9 required assistance. Two patientshad stopped using their ACE stoma despite good tech-nical results. The appendix was used as a catheterizablestoma in 15 of the 27 patients. The appendix was notavailable in 12 patients, so a tubularized cecal flap wasused in 9 and a small-bowel neoappendix was created in3. Complications included stomal stenosis in 5 patients,cecal-flap necrosis in 1, and stomal granulations in 3.
Conclusions: We believe that the ACE procedure pro-vides reliable colonic emptying and avoids fecal soilingin the majority of individuals, and we find it widely andenthusiastically accepted by patients with spina bifida.(J Am Coll Surg 1998;186:669674. 1998 by theAmerican College of Surgeons)
Uncertainties about bowel and bladder continenceappear to be the greatest impediments to emotionalgrowth in adolescents with meningomyelocele.1
This statement from the Journal of Pediatrics almost20 years ago is as true today as it was then. Bowel andbladder dysfunction in spina bifida patients causesnot only important medical concerns, but also emo-tional trauma of equal proportion. The emergence ofclean intermittent catheterization, along with an ar-ray of urinary continence procedures including con-tinent urinary diversion, has greatly decreased theproblem of urinary incontinence as an impedimentto patient socialization. It is unusual today for a pa-tient with spina bifida not to be able to achieve anacceptable level of urinary continence given the widerange of choices and procedures that can be offered.
Bowel dysfunction, on the other hand, has notreceived much interest or enthusiasm until relativelyrecently. Traditional bowel management programs,including the use of suppositories, retrograde enemaprograms, and manual disimpaction, have beenlargely unsatisfactory in the bowel management ofmost patients with spina bifida. Many adolescentsnever achieve independence and socialization be-cause of bowel dysfunction, and many remain depen-dent on a caretaker to deal with this problem. TheMalone antegrade continence enema (ACE) proce-dure (Fig. 1) has largely changed our attitude andapproach to bowel management in our populationwith spina bifida.
PatientsOver a 3-year period between January 1994 and
January 1997, 27 patients with spina bifida under-went the Malone ACE procedure at our institutions.The group included 17 males and 10 females whoranged in age from 10 to 31 years (mean age, 16years). Twenty-two of the individuals were bed- andwheelchair-bound, and five were ambulatory. Many
Received October 1, 1997; Revised December 17, 1997; Accepted January 5,1998.From the *Department of Urology, College of Physicians and Surgeons, Co-lumbia University, New York, NY; the Babies and Childrens Hospital of NewYork, New York, NY; and St. Josephs Hospital Medical Center, Paterson, NJ.Correspondence address: Terry W. Hensle, MD, FACS, Director of PediatricUrology, The Babies and Childrens Hospital of New York, 3959 Broadway,BHN Suite 219-N, New York, NY 10032.
669 1998 by the American College of Surgeons ISSN 1072-7515/98/$19.00Published by Elsevier Science Inc. PII S1072-7515(98)00091-X
had undergone major surgical procedures (Table 1).Twenty-three of the 27 patients had indwelling ven-triculoperitoneal shunts and 8 patients had under-gone continent urinary diversion. At the time of theirACE procedure, four patients underwent simulta-neous continent urinary diversion using the Mitro-fanoff principle, and three had simultaneous small-bowel bladder augmentation. We evaluated all of thepatients for 9 months or more after their procedureand followed up 10 of the patients for more than 2years.
Surgical techniqueAll patients had preoperative mechanical bowel
cleanout, usually on an outpatient basis, and preop-erative intravenous antibiotics. Patient compliancepredictably altered the results of the outpatient bowelpreparation. In this series, the appendix was used as acatheter channel in 15 patients (Table 2). The proce-dure was performed as originally described by Ma-lone and associates,2 using the vascularized appendixtunneled into the cecum in nine of the patients (Fig.2). A cecal extension of the appendix, created using aGIA stapler (US Surgical Corporation, Norwalk,CT) as described by Sumfest and colleagues,3 wasused in six patients. Eight of the patients had had theappendix used previously as part of a continent uri-nary diversion, and in four patients the appendix wasfound to be stenotic and unusable. Nine of theseindividuals had a tubularized cecal flap based on theBoari principle.4 This was performed by creating a
Figure 1. The Malone antegrade continence enema procedure pro-vides a catheterizable channel through which antegrade colonicwashout can be performed. (Reprinted with permission from Ma-lone PSJ. Malone procedure for antegrade continence enemas. In:Spitz L, and Coran AG, eds. Rob & Smiths Operative Surgery:Pediatric Surgery. 5th ed. London: Chapman & Hall Medical;1995:459467.)
Figure 2. Appendix harvested with intact vascular supply has beentunneled into the cecum using the Mitrofanoff principle to providea nonrefluxing, catheter channel for antegrade enemas. (Reprintedwith permission from Malone PSJ. Malone procedure for ante-grade continence enemas. In: Spitz L, and Coran AG, eds. Rob &Smiths Operative Surgery: Pediatric Surgery. 5th ed. London:Chapman & Hall Medical; 1995:459467.)
Table 1. Previous Surgery in 27 Patients
Surgical procedure n
Ventriculoperitoneal shunt 23Continent urinary diversion 8Orthopaedic limb surgery 18Orthopaedic spine surgery 11
Table 2. Tissue Used for Catheterizable Channel
Tissue type n
Appendix 15Native 9Extended 6Cecal flap 9Small-bowel neoappendix 3
670 Hensle et al Malone Antegrade Continence Enema J Am Coll Surg
4 3 1.5-cm flap based on the lateral vessels and tu-bularized over an 8F catheter (Fig. 3). Three patientsunderwent creation of a neoappendix using a small-bowel tube following the description of Monti andcoauthors5 (Fig. 4). No matter what type of tube ischosen, it is very important to use an inlay of skin atthe cutaneous stomal anastomosis, as described byRoberts and coworkers,6 to reduce the occurrence ofpostoperative stomal stenosis (Fig. 5).
A catheter was left in place for an average of 10days when the appendix alone was used and 21 dayswhen an appendiceal extension, cecal flap, or small-bowel tube was created. The size of the catheterranged from 8F to 14F, depending on the size and ageof the patient, and antegrade irrigation was begun inall patients between postoperative days 5 and 7. Sa-line solution was used initially for irrigation in allpatients, beginning with a volume of 60 mL andincreasing incrementally until results were obtained.The amounts initially used ranged from 60 to 2,000mL, and the transit time varied from 10 to 120minutes.
RESULTSThe duration of followup for this group of patientsranged from 9 to 30 months. Eighteen of the 27patients performed both the catheterization and theirrigation independently, and 9 required assistance.All of the individuals requiring assistance were bed-and wheelchair-bound, and 6 had upper-extremityweakness and poor coordination. The other 3 pa-
Figure 3. Tubularized cecal flap. (A) Flap is cut from the anterior wall of the cecum and measures approximately 4 3 1.5cm. (B) Completed tubularized flap closed over 8F catheter. (Reprinted with permission from Malone PSJ. Maloneprocedure for antegrade continence enemas. In: Spitz L, and Coran AG, eds. Rob & Smiths Operative Surgery: PediatricSurgery. 5th ed. London: Chapman & Hall Medical; 1995:459467.)
Figure 4. Ileal neoappendix. (A) Ileal segment of 23 cm is isolatedon its blood supply and (B) detubularized halfway down on theanterior surface. The flap (C) is then tubularized (D, E) to create aneoappendix.
671Vol. 186, No. 6, June 1998 Hensle et al Malone Antegrade Continence Enema
tients were intellectually impaired. The volume re-quired for thorough irrigation and the transit timevaried widely in individual patients. In this group ofpatients, the average volume required for thoroughirrigation was 300 mL with a transit time of 30 min-utes. Nineteen of the 27 patients had complete andpredictable bowel control using the ACE program,and 6 had occasional rectal soiling requiring a sani-tary pad. Two patients had stopped using the ACEstoma. In both cases, the procedure was technicallysuccessful but the individuals, both teenage girls, re-fused to use the ACE stoma. We hope that this will bea temporary decision. No patients in this series re-ported stomal leakage.
Complications have included stomal stenosis infive patients, complete cecal-flap necrosis in one pa-tient, and false passage of a cecal flap in one patient(Table 3). Three of the patients with stomal stenosiswere managed with dilation in the office. The twoothers and the patient with necrosis of the tubular-ized cecal flap required reoperation. The patient with
a false passage was treated with prolonged intubationof the channel. Three patients had stomal granula-tions that were fulgurated as an office procedure.Twenty-five of the 27 patients reported substantialimprovement in their quality of life as a result ofdecreasing concerns about fecal soilage and bowelaccidents. Quality-of-life assessment was done aspart of a standard postoperative questionnaire thatincluded frequency of irrigation, volume of solutionused, transit time, stomal leakage, fecal soilage, andbowel accidents.
DISCUSSIONGreat strides have been made in the management ofthe urinary tract in individuals with spina bifida.There are very few individuals born in the past 1015years who cannot look forward to a reasonable degreeof urinary continence and a stable urinary tract,without the stigma of renal failure. By contrast, ad-vances in dealing with bowel dysfunction have notmatched the great strides made in managing prob-lems of the urinary tract. Bowel management pro-grams, including the use of suppositories, retrogradeenema programs, and manual disimpaction, havebeen largely unsuccessful. Most wheelchair-boundspina bifida patients are dependent on diapers as aresult of frequent and unanticipated episodes of fecalsoiling.
In 1990, the antegrade continence enema wasintroduced by Malone and colleagues2 and has rap-idly gained worldwide acceptance. Experience hasbeen reported both in Europe6-9 and in the UnitedStates.10,11 The laparoscopic approach to creating acecal tube has been presented by Cromie and associ-ates,12 and the use of the gastrostomy button as partof the ACE procedure has been reported by Fuku-naga and associates.13
In the present series, all the patients undergoingthe procedure had spina bifida, and the majority (22of 27) had a high spinal lesion requiring a bed-and-wheelchair existence. Only five were ambulatory, andall required diapers because of fecal soilage. In themajority of our patients, we were able to use theappendix as a catheterizable stoma much as originally
Figure 5. Stomal skin flap. (A) A chevron skin incision is madenorth to south, and the appendix is spatulated inferiorly. (B) Thelower edge of the spatulated appendix is sutured to the superiorskin edge. (C) The top edge of the stoma is sutured to the inferiorskin edge, and the lateral skin edges are then mobilized to cover theappendix circumferentially.
Table 3. Complications
Stomal stenosis 5Cecal-flap necrosis 1Stomal granulations 3Creation of false passage 1
672 Hensle et al Malone Antegrade Continence Enema J Am Coll Surg
described, although six needed the appendix elon-gated with a stapled cecal tube. The increased lengthof the tube was necessary in many of our patientsbecause of their pronounced lower abdominal girth.In several instances, we found it impossible to reachthe native appendix up to the skin without someform of elongation. When the appendix was eitherabsent or unusable, we initially turned to a tubular-ized cecal flap based on the Boari principle,4 or laterused a small-bowel neoappendix based on the de-scription of Monti and coauthors.5 At present, weprefer to create a small-bowel neoappendix as a cath-eterizable stoma rather than to rely on a long tubu-larized cecal flap. Much of the decision making isbased on the abdominal girth and width of the pan-niculus in the individual patient.
It is not unusual for patients with high spinabifida to require simultaneous procedures. Four ofthe patients in the series had simultaneous continenturinary diversion, in which the appendix was usedfor the continent urinary diversion and either a tu-bularized cecal flap or a small-bowel neoappendixwas used for the ACE procedure. In addition, three ofthe patients underwent simultaneous small-bowelbladder augmentation.
Our results showed that complete and predict-able bowel control was achieved in 19 of the 27 pa-tients (Table 4). Six patients in our series have occa-sional soiling and, unfortunately, two patients havestopped using their ACE stoma despite an adequatetechnical result. Complete and predictable bowelcontinence was not an immediate event in any of thepatients, and, in many individuals, particularly thosewho have been on longterm retrograde enema pro-grams, it will take time to achieve the desired results.Many individuals with particularly dilated transverseand descending colons will initially require excessiveamounts of fluid and will experience prolonged tran-sit times. Over time, both the amount of fluid re-quired to complete the irrigation and the transit timewill decrease.
Most patients in our series have been successful
using saline irrigation alone. Two patients have re-quired the addition of suppositories, and five requireintermittent use of phosphate-based enemas to en-sure bowel cleanout. We have tried to limit the use ofphosph...