The Value of the MACE (Malone Antegrade Colonic Enema) Procedure in Adult Patients Elmar W. Gerharz, MD, Viktor Vik, MD, Gregory Webb, FRCS, Rachel Leaver, P. Julian R. Shah, FRCS, and Christopher R.J. Woodhouse, FRCS ciples in children suffering from fecal incontinence. The Mitrofanoff nonrefluxing catheterizable channel (2) in its modification as appendicocecostomy allowed antegrade colonic washout (3) to produce complete colonic emptying and hence prevent soiling. Subsequently, the Malone antegrade colonic enema (MACE) has been applied in several pathologic conditions after all other attempts to control incontinence have failed and the only remaining option is a colostomy (4). In urology, the technique is used predominantly in children (1, 4 –7) who undergo simultaneous reconstruction of the lower urinary tract. We report our experience with the MACE procedure in adult patients and discuss its value outside of the realm of pediatric surgery.
Background: We report our experience with the Malone antegrade colonic enema (MACE) procedure in adult patients suffering from urinary incontinence and intractable constipation with or without fecal soiling. Study Design: Since June 1990, the MACE procedure was initiated in 4 female and 12 male patients 14 –54 years old (mean age, 29.9 years) with different pathologic conditions (myelodysplasia, n 5 7; anorectal anomaly, n 5 3; spinal cord lesion, n 5 4; neuropathic disease of unclear cause, n 5 2). Three surgical techniques were used: reversed and in situ appendix and tapered ileum). Complex simultaneous urologic continence procedures were performed in nine patients. Two patients had undergone previous operations in the lower urinary tract. Results: After 6.6 years of followup (average, 41.7 months), eight patients (50%) were still using the MACE successfully. They were completely clean day and night and were relieved of symptoms of constipation. Eleven complications related to the MACE procedure occurred in seven patients (44%). Eight patients abandoned the procedure for various reasons. The failure rate was higher in chronically constipated patients without fecal soiling.
Methods
We reviewed the records of 16 patients (4 women, 12 men; age at operation, 14 –54 years; mean, 29.9 years) who had undergone the MACE procedure at our institution between June 1990 and October 1996. A continent cecostomy was formed for intermittent catheterization of the cecum, allowing antegrade large-volume colonic irrigation while the patient sat on the toilet. In all patients, chronic constipation was intractable by standard conservative management (dietary and medical therapy, manual evacuation, high rectal washouts). Eight patients suffered from fecal soiling. Underlying pathology included myelodysplasia (n 5 7), anorectal abnormalities (n 5 3), spinal cord lesions (n 5 4), and neurogenic disorders of unclear cause (n 5 2). Subsequent information regarding the success of the procedure in terms of practicability (mode, frequency, enema administration interval) and patient satisfaction (continence status, independence) was obtained by patient interview or telephone call. Operative details on the cutaneous appendicocecostomy or its variations were extracted from
Conclusions: The MACE procedure is associated with a high failure rate when used in adults, but it may be possible to identify a subgroup of patients in whom the procedure could be beneficial. Success would depend on overcoming technical problems and difficulties with patient compliance. (J Am Coll Surg 1997;185: 544 –547. © 1997 by the American College of Surgeons)
Seven years ago, Malone and associates (1) published their preliminary report on the intriguing combination of two clinically well-established prinReceived July 1, 1997; Revised September 3, 1997; Accepted September 3, 1997. From the Institute of Urology and Nephrology, University College London Medical School, London, England. Correspondence address: Elmar W. Gerharz, MD, The Institute of Urology and Nephrology, University College London Medical School, 48 Riding House St., London W1P 7PN, England. © 1997 by the American College of Surgeons Published by Elsevier Science Inc.
544
ISSN 1072-7515/97/$17.00 PII S1072-7515(97)00125-7
Gerharz et al patient records. Different surgical approaches were used, including the technique originally described by Malone and associates (1) of a dismembered reimplanted appendicocecostomy (n 5 8) and, most recently, the submucosally embedded in situ appendix (n 5 1) (8). When the appendix was absent, not suitable for reconstructive operations, or used in another procedure (e.g., appendicovesicostomy), tapered ileum was used (n 5 7). Nine patients simultaneously underwent complex urologic reconstruction (appendicovesicostomy, augmentation cystoplasty, bladder neck closure) for urinary incontinence, and two had had previous operations in the lower urinary tract (augmentation cystoplasty). The MACE stoma was placed in a preselected area of the lower right quadrant of the abdomen. It was fashioned by either incorporating a triangleshaped skin flap into the spatulated distal end of the tube or using multiple skin flaps according to the V.Z.Q. technique (9). In all patients, the cecum was fixed to the anterior wall to avoid traction on the stoma and to ensure longterm ease of catheterization. A 12F silicone catheter was left in the stoma for $ 21 days postoperatively. Irrigations were instituted as soon as intestinal peristalsis had recovered fully (usually 4 – 6 days). Irrigations were begun with a phosphate enema (100 mL) diluted in an equal volume of saline. This solution was washed through with 1,000 –2,000 mL of saline. The content of the enema and the volume and frequency of irrigation were modified to determine the optimal regimen in each patient. The enema administration interval varied from daily to every 6 days, with the majority of patients administering an enema every second or third day. Apart from phosphate enemas, bisacodyl, arachis oil, and licorice root were used. The volume of saline required to complete the irrigation and successfully clear out the colon ranged from 1,000 to 2,000 mL. Instillation time was , 10 minutes in seven patients, and the interval until a result occurred was 10 – 60 minutes, and up to 20 minutes in the other patients. To avoid stomal stenosis, catheterization was performed daily using a
MACE PROCEDURE
Table 1. Malone Antegrade Colonic Enema Procedure: Complications Complication Stomal incontinence with fecal and gas leaks Stomal breakdown/complete obliteration/prolapse Stomal stenosis Fecal impaction Phosphate poisoning/nausea Pain with enema Wound infection Psychological problems
No. in literature
No. in institute
3
1
9 17 1 1 1 3 2
— 2 — 3 1 1 2
Jacques catheter, size 8 –12F. Six patients selfcatheterized and administered the enema alone. Results
No intraoperative complications occurred. Followup of our 16 patients ranged from 3 to 79.2 months (average, 41.7 months). At followup, eight patients (50%) still used the MACE routinely, with a mean duration of 40.1 months. They were completely clean day and night and reported a higher degree of independence. In the preoperatively continent patients, success was defined as facilitated and regular bowel emptying with relief of symptoms of constipation. Two patients combined the MACE with manual disimpaction. In all patients with a functioning antegrade colonic enema, the stoma was continent of stool and no mucus or gaseous discharge from the stoma has been recorded. No significant changes in serum electrolytes or renal function were observed at 3 months postoperatively. All patients were continent of urine. General surgical complications included deep vein thrombosis and acute respiratory distress syndrome with subsequent intensive care. Eleven complications related to the MACE procedure occurred in seven patients (44%), including nausea, wound infection, stomal stenosis, stomal leakage, and pain with enema administration (Table 1). Two complications required an additional surgical procedure.
Table 2. Malone Antegrade Colonic Enema Procedure: Failures Underlying disorder
545
Failure rate
Cause of failure
Myelodysplasia/spina bifida
4/7
Spinal cord lesion Neuropathy of unknown cause
3/4 1/2
Nausea (in two patients); leakage, peristomal infection, cramps with phosphate; pain with enema Phobia to catheterization; did not work (in two patients) Problems with gas and leftover saline
546
J
AM COLL SURG
DECEMBER
1997
VOLUME
185:544 –547
In eight patients (50%), the MACE stoma was abandoned for various reasons (Table 2), including noncompliance despite a working system. In five of these patients, the MACE was applied successfully for . 3 months (0.5–7 months; mean, 3.8 months). Two patients had their MACE stoma resected. The failure rate was higher in chronically constipated patients without fecal soiling (five of eight). Failure was independent of the surgical technique. Discussion
Based on accepted surgical practice, Malone and associates (1) described in 1990 an adaptation of the Mitrofanoff procedure for treatment of the chronically constipated and fecally incontinent child, the antegrade continence enema (ACE). Complete colonic emptying was expected to relieve chronic constipation and prevent episodes of fecal soiling. Various surgical options currently are available to construct the antegrade colonic enema stoma, allowing this procedure to be performed on virtually all candidates (1, 4 – 8). Although the procedure is gaining popularity, its application has been confined almost exclusively to children. Since Malone and associates published their preliminary report (1) on five patients showing excellent results, four other series have been published with a total of 86 patients (average age, 9 years), with a followup ranging from 2 to 31 months (4 –7). Our series is the first to include adult patients only, with an average age of 29.9 years and a mean followup of almost 3.5 years. Although the MACE procedure can be extremely beneficial for the patient’s quality of life and the short- and longterm results are claimed to be excellent in the majority of pediatric patients (1, 4 –7), it is not universally successful or free of complications. Although 71–100% (mean, 90.5%) of all children use their MACE stoma with complete success (totally clean), in 57–100% (mean, 77.5%) (4 –7), half of our adult patients have abandoned their stoma for a variety of reasons. One could assume that the high rate of failure simply reflects the substantially longer followup in our series. This would imply that the procedure is always destined to fail in the medium or longterm and that the success rate in the pediatric population should drop when reassessed at a later stage. Although this tendency of a decreasing success rate with longer followup has been noticed by some surgeons (personal communication, P. S. Malone), it cannot be confirmed from our data. In the eight successful patients, the MACE has been
used for an average of 40.1 months, perfectly matching the mean followup of all patients. Lack of experience in patient selection is much more likely to account for some of the failures. With regard to psychological factors, there is a consensus that the level of commitment and compliance by the patient is critical to the success of the enema (4 –7). One should consider only highly motivated patients with reasonable intelligence and manual dexterity who are physically and emotionally capable of dealing with the regimen of intermittent catheterization. To discover a phobia to catheterize postoperatively is extremely unsatisfactory. To avoid disappointment, detailed and realistic preoperative education must emphasize that the individual regimen (i.e., the ultimate interval and volume) must be determined by trial and error in a period of occasionally stressful experimentation. Regarding the physical aspects of patient selection, the question arises whether the success of the procedure depends on the underlying pathologic process. The procedure failed in 8 of 13 patients with neurogenic disorders but was uniformly successful in anorectal anomalies. Although the number of patients in each subgroup was too small and the reasons documented for failure were too nonspecific to draw a meaningful conclusion, there may be factors predisposing to inefficacy. Long-standing constipation in adulthood changes the intestinal configuration with elongated, more distended bowel loops, and this renders efficient evacuation more difficult. Although the patients with myelodysplastic and anorectal anomalies by definition have an open anus at rest, the inability to relax the sphincter muscle in some of the other conditions contributed to the failure of the procedure. In three patients with spinal cord injury or demyelinizing disease, a combination of fluid trapping in largecapacity bowel loops and a closed anus led to complete failure of the procedure within the first weeks. In one patient, the anus had to be dilated to evacuate the instilled enema. In these patients, a high colonic lavage with a catheter placed under radiologic control should be performed preoperatively to mimick the ACE procedure. In our patients who have stopped using the MACE because of nausea or pain with administration, failure must be attributed to a lack of experience in adjustment of the regimen. In the meantime, a whole range of different approaches and substances have been added to the original phosphate-containing enemas. The complication rates reported in the literature are high, ranging from 22% to 81% (mean, 44.4%) (1, 4 –7; Ta-
Gerharz et al
MACE PROCEDURE
547
Table 3. Malone Antegrade Colonic Enema Procedure: Review of the Literature Age (y)
Followup (mo)
Author
Year
n
Median
Range
Median
Range
Complications (%)
In use (%)
Completely clean (%)
Squire et al* (7) Griffiths and Malone (4) Koyle et al (6) Ellsworth et al (5) Institute of Urology Total
1993 1995 1995 1995 1997
25 21 22 18 16 102
9 12 13 12 29.9 15
3–18 1.5–18 5–23 5–31 14–54
13 ? .4 6.6 41.7
2–31 — — 2–24 3–79.2 2–79.2
22 81 36 39 44 44.4
100 71 91 100 50 82.4
76 57 77 100 75 77
*Including Malone’s five original patients.
ble 3). In our series, the majority of complications were not surgical but related to the administration of the enema, emphasizing that compliance is an important prerequisite for success. A fair judgment of the complication rate must consider that the MACE procedure is a last resort and that the affected population is often psychologically and physically traumatized by the underlying condition and multiple operations. A complication that should be expected in the application of the Mitrofanoff principle is impaired catheterization due to a gradual outlet stenosis (11) potentiated by less frequent catheterization of the antegrade colonic enema. Stomal stenoses usually can be corrected with minor procedures on an outpatient basis. To minimize the incidence of outlet stenosis, which typically occurs at the skin level, we incorporated a V-shaped skin flap into the distally spatulated ileal tube or used the V.Z.Q. technique, with good cosmetic results (9). The fact that the MACE procedure is reversible appears to be advantageous, but it may prevent some patients from trying hard to achieve an optimal outcome. The effort to cope with the procedure may be related to the severity of symptoms, which could explain why the failure rate was higher in patients suffering from chronic constipation without the stigma of fecal soiling. If intractable problems remain after the MACE is performed, it is always possible to use the older surgical approach of colostomy without prejudicing its chances of success. In conclusion, the MACE has revolutionized bowel care in a subset of highly motivated patients with chronic constipation and fecal incontinence. It is of particular benefit to patients who can administer the enema by themselves and thus become completely independent. The procedure
can be performed in isolation or combined with reconstruction of the lower urinary tract in patients with double incontinence. In the latter case, it adds little to operative time or hospital stay and obviates the need for additional anesthesia. Although there is no difference concerning the rather high complication rates, the success rate in the reported adult population appears to be lower than in children. Increasing experience with the various surgical options, modes of enema administration, and patient selection may reduce the rates of both failure and complications. References 1. Malone PS, Ransley PG, and Kiely EM. Preliminary report: the antegrade continence enema. Lancet 1990;336:1217– 1218. 2. Mitrofanoff P. Cystostomie continente trans-appendiculaire dans le traitement des vessies neurologiques. Chir Ped 1980; 21:297–305. 3. Radcliffe AG, and Dudley HAF. Intraoperative antegrade irrigation of the large intestine. Surg Gynecol Obstet 1983; 156:721–723. 4. Griffiths DM, and Malone PS. The Malone antegrade continence enema. J Pediatr Surg 1995;30:68 –71. 5. Ellsworth PI, Webb HW, Crump JM, et al. The Malone antegrade colonic enema enhances the quality of life in children undergoing urological incontinence procedures. J Urol 1996;155:1416 –1418. 6. Koyle MA, Kaji DM, Duque M, et al. The Malone antegrade continence enema for neurogenic and structural fecal incontinence and constipation. J Urol 1995;154:759 –761. 7. Squire R, Kiely EM, Carr B, et al. The clinical application of the Malone antegrade colonic enema. J Pediatr Surg 1993; 28:1012–1015. 8. Gerharz EW, Vik V, Webb G, and Woodhouse CRJ. The in situ-appendix in the MACE (Malone antegrade continence enema)-procedure for faecal incontinence. Br J Urol 1997; 79:985–986. 9. Mor Y, Quinn FMJ, Carr B, et al. Combined Mitrofanoff and antegrade continence enema procedures for urinary and fecal incontinence. J Urol 1997;158:192–195. 10. Woodhouse CRJ, and MacNeily AE. The Mitrofanoff principle: expanding upon a versatile technique. Br J Urol 1995; 74:447– 453. 11. Gerharz EW, Ko¨hl U, Weinga¨rtner K, et al. Complications related to different continence mechanisms in ileocecal reservoirs. J Urol 1997;158:1709 –1713.