How to achieve a successful Malone antegrade continence enema

How To Achieve

a Successful

Malone

By J.I. Curry, A. Osborne, Southampton,

Background/Purpose:The Malone antegrade continence enema (MACE) has proved invaluable in the management of children with faecal incontinence. The authors have reviewed their experience in depth to try and ascertain why some MACES fail.

Methods: The

records of 31 children who had a MACE procedure between 1990 and 1995 were reviewed. Their preoperative bowel management, the operative procedure, the postoperative washout regime, and the outcome were assessed. Success was graded either as full, described as totally clean or minor rectal leakage on the night of the washout; partial, described as clean but significant stoma1 or rectal leakage, occasional major leaks, still wearing protection, and perceived improvement by the parents or child; or

A

FTER THE ORIGINAL description of the MACE procedure in 1990’ a number of studies have shown it to be useful in the managementof both faecal incontinence and chronic constipation.2-11 Failure rates of 0% to 50% are described, but it is not clear why the MACE fails. We have analysed our experience in 31 patients, paying particular attention to those in whom MACE failed, in an effort to define factors that might be responsiblefor failure and therefore ultimately achieve success. MATERIALS

AND METHODS

Thirty-one children who underwent a MACE procedure between December 1990 and July 1995 for faecal incontinence or constipation were analysed. A proforma was used to analyse the demographic and clinical data of the patients before and after surgery. Follow-up information was gained either from review of the clinical notes or from direct contact with the family by the clinical nurse specialist (AO). Success was classified as full, described as totally clean or minor rectal leakage on the night of the washout; partial, described as clean but significant stomal or rectal leakage, occasional major leak, still wearing protection, but perceived by the child or parent to be an improvement from previously; or failure, which occurred when regular soiling or constipation persisted. There was no perceived improvement, and the procedure was abandoned usually to perform a colostomy.

From the Department of Paediatric Urology, Southampton General Hospital, Southampton, England. Presented at the 44th Annual International Congress of the British Association of Paediatric Surgeons, Istanbul, Turkey, July 22-25, 1997. Address reprint requests to J.I. Curry, Department of Paediatric Urology, Southampton General Hospital, Tremona Rd, Southampton 509 4Xx England. Copyright 0 1998 by WB. Saunders Company 0022-3468/98/3301-0033$03.00/O 138

Antegrade

Continence

Enema

and P.S.J. Malone England

failure, described as regular soiling or constipation, ceived improvement, and the procedure abandoned. were 18 boys and 13 girls in the series. The mean survery was 8.4 years.

no perThere age at

Resu/ts:Overall success rate was 19 of 31 (61%) with a mean follow-up of 3.25 years. The diagnosis and success rates were: anorectal anomaly 11 (success, 8 of 11, 73%), neuropathic bowel 11 (8 of 11, 73%), chronic constipation 8 (3 of 8,

38%). J Pediatr Surg ders Company. INDEX ure.

WORDS:

33:138-141.

Malone

Copyright

antegrade

o 1998 by W.B.

continence

enema,

Saun-

fail-

RESULTS

There were 18 boys and 13 girls. Their diagnosesare shown in Table 1. The MACE was carried out as an isolated procedurein 23 and in combination with a major urinary tract reconstruction in eight. The mean age at operation was 8.4 years (range, 1.3 to 18.3 years). The mean follow-up time was 3.25 years (range, 1 to 5.75 years). The methods chosen for the construction of the MACE were appendix disconnection and reimplantation in 24, appendix straight with no reimplantation in two, tubularised caecal flap in four, and tubularised colonic flap in one. Notes were not available for review for one patient. Complications were common and occurred in 22 patients (7 1%). Somewere nonspecificto the MACE and related to abdominal surgery eg, adhesive obstruction, which occurred in four patients ( 13%). The main specific complications were stomal in 17 (55%), devascularisation of the channel in two (6%), phosphatetoxicity in three (lo%), and pain on irrigation in 18 patients (58%). In one child, technical problems were the main causeof failure. The tubularised caecal flap became gangrenous and pulled away from the abdominal wall. This occurred in an obesechild with spinabifida. The most common washout regimen used was that involving the administration of phosphate (Fletchers, Pharmax, UK) and saline. This was instituted when diet wasrestartedpostoperatively. The proceduretook a mean of 39 minutesto complete (range, 20 to 60 minutes). The children in whom the procedure was successfultook a meantime of 6.9 months(range, 12 days to 60 months)to achieve stabilisation ie, washout every 1 to 2 days with a Journal

of Pediatric

Surgery,

Vol33,

No 1 (January),

1998:

pp 138-141

MALONE

ANTEGRADE

CONTINENCE

Table

139

ENEMA

1. Diagnoses 12

Neuropathic Spina bifida Sacral Other

10 1

agenesis

Anorectal Chronic

anomaly constipation

1 10

(all high)

8

Idiopathic Other Hirschsprung’s

6 2 1

disease

good result. Once a steady state was achieved, we perceived no benefit from changing the regimen to try to reduce its frequency in an effort to reduce the burden for the parents and child; it often resulted in reoccurrence of constipation or further soiling. Twelve MACES failed (39%). We have analysed these cases in detail, and the causes are listed in Table 2. The mean duration from surgery to failure was 1.5 years (range, 12 days to 55 months). We have found it important to ensure that the children have adequate bowel preparation to ensure the colon is empty before surgery and to facilitate the first MACE washout. Washout failure was the most common overall cause for failure. This was defined as failure to pass any or very little of the enema from the rectum within 1 or 2 hours of lavage. The child with Hirschsprung’s disease only ever had one successful washout, but in the other children this developed weeks to months after surgery. Phosphate toxicity occurred three times, and this was in children with washout failure. If the cause of washout failure was thought to be constipation, then a Fletchers’ arachis oil Retention Enema (Pharmax, UK) was instilled into the MACE and left for a period of time (ensuring there was no known peanut allergy). This continues to be a useful avoidance technique for some children in the successful group. If washout failure occurred, the parents were advised to contact the ward or the nurse specialist before the administration of any further washout. Continued rectal leak was a factor in five children. Some degree of leak within the first few hours of washout was common to most children regardless of success or failure, but unlike the successes, this was not ameliorated by manipulation of the regimen, which took the form of Table Reason

Washout Isolated Combined

2. Causes

for Failure

pain

of MACE

Number

failure

with

of Failure

or leak

Diagnosis

9 5

Chronic

4

Hirschsprung’s ARM (n = 3)

constipation

(n = 4) disease

(n = 1)

or lack of compliance Lack of compliance associated with leak

2

Sacral agenesis (n = 1) ARM In = 1) Chronic constipation (n = 1)

Technical

1

Spina

bifida

changing the volume of either the enema or the saline flush. The amount of leakage can improve with time, and the parents and children need strong reassurance in the early days and weeks. Pain was encountered in 15 of the children within 3 months of their MACE formation. In three children this persisted and became a contributory reason for their failure. Two children who now have a successfully functioning MACE had their regimen changed to one containing liquorice root with resolution of their pain (hypematraemia can be a complication of this treatment, K. Parashar, personal correspondence). In the other children who underwent successful procedures, pain either improved with time or responded to antispasmodic medication in the form of colofac (Solvay, UK) at a dose of 5 to 15 mL given before their enema (50 mg/5 mL). Of the three children in whom lack of compliance compounded failure of the procedure, one was a girl with Down’s syndrome who refused to allow further washouts, another was a boy going through a rebellious puberty who was also being tested for minor rectal leak by his school mates, and the third was a boy with no obvious predisposition to poor compliance. DISCUSSION

Reports now exist that lay testamentto the successof the MACE procedure in the management of some children and young adults with intractable constipation and faecal soiling. Its successhashelped to revolutionise the lives of many children and their families and helped them to achieve a level of independencethat they could never have previously hoped for. It has for some, however, become another unsuccessfuloperation in the life of a child all to familiar with the operating theatre. In this in-depth analysis, we have attempted to answer the question “what can be done to improve the selection of children who will have a successfuloutcome?” The one pure technical failure in our seriesresulted from not having an appendix to perform the MACE. In those circumstances in which either the appendix is absentor it is usedfor a Mitrofanoff procedure, two new techniquesmay be used. The first is the use of a button caecostomy as describedby Shandling,6and the second involves the use of tubularised small bowel.12 This procedure involves taking a 2-cm length of small bowel that is detubularisedand retubularisedtransversely over a stent. This can create a channel of up to four times longer than the original length of small bowel. There are no long-term resultsasyet with this procedure, but the initial resultsare promising. Newer techniques used in fashioning the stomahave led to a decrease in the complications of stenoses. Multiple flap techniques asdescribedby Kajbafzedeh et alI3 have reduced the complications causedby the stoma

140

CURRY,

from 59% if the appendix is anastamosed flush with the skin to 15% if a multiple flap technique is employed. It is also important to perform frequent cathaterisation of the stoma to ensure no stenosis develops because some of these patients may only washout once every 2 days compared with 4 hourly catheterisations of a Mitrofanoff channel. The MACE technique has been applied to faecal soiling and constipation regardless of the initial diagnosis. This diagnosis has not previously been attributed to the success or failure of the MACE. Our figures, although too small for statistical significance, suggest that those children with propulsive disorders (chronic idiopathic constipation and Hirschsprung’s disease) did worse than the other groups. A possible explanation is their lack of normal colonic peristalsis, which would be needed to propagate the enemata along the colon, and, washout failure was a contributing factor in five of these children. Two of these children still have major problems because of their undiagnosed gastrointestinal dysmotility disorder. Those children with a localised abnormality, such as an anorectal anomaly or neuropathic sphincter abnormalities, would be expected to do well. Those patients did well in our series (70% and 75% success rates, respectively). Colonic dysmotility has not been equated with a poor result after the MACE procedure, but our figures suggest this may be the case. Other centres have reported good success with the Hirschsprung’s group, but because there was only one such child in our series, we are unable to comment.* Continued leakage can be a difficult problem to overcome. Changing the washout regime, for example increasing the enema content and decreasing the lavage, can help overcome leakage. The parents and the patients should be warned that a small amount of leak, especially on the night of the washout, is to be expected, and that protection may need to be worn. The amount of leakage can improve with time, and one should be cautious about declaring a failure too soon. A degree of functional anal stenosis may prove to be of useful benefit for children with either neuropathic anal sphincters or an anorectal anomaly, but in the extreme, can result in simple mechanical obstruction to the flow of the enema and the lavage. It is useful to ensure this is not the case before surgery.

OSBORNE,

AND

MALONE

Fifteen of the 31 children (48%) experienced pain in the first 3 months of the MACE procedure. This pain was in the form of colic and occurred during the actual washout. Antispasmodic medication was effective in some of the children, but there was a natural tendency for improvement. Because of pain, two children converted to liquorice root as the main ingredient of their washout with success. Liquorice root is a demulcent and an antiinflammatory agent, which may explain this effect. It may be necessary to alter the regimen if pain persists, usually in the form of reducing the amount of enema and/or the lavage and also increasing the time taken to wash the enema and flush through. With increasing knowledge of washout regimes, this should become an infrequent cause of failure. We have found a 70% failure rate in those children operated on under the age of 5 years but only a 24% failure rate in those above. This was not diagnosis specific. This should not be surprising because we know that the child’s perception of an abnormality in terms of incontinence or constipation takes time to develop, and they may not wish to embark on major surgery if they are happy in nappies or some other aid. Also a child under 5 years may not tolerate an hour out of his day spent on the toilet to achieve an adequate washout. Other units also adopt this policyS4 Certainly, if major urinary tract reconstruction is planned to make the child continent, then the operations can be successfully be performed at the same time.3x9J3Their cooperation, understanding, and motivation will be vital to the success of the operation, and this can take months and years. The involvement of experienced nurse specialists is vital also to provide day to day support for the parents and child and to reinforce the pre- and postoperative management. All other conservative methods of treating these conditions should be exhausted before undertaking such a procedure because it is not without its complications. A frank discussion with parents and child over many months or even years will be necessary to ensure they are aware of its benefits and also the time and effort required to be sure of continued success. Young age at surgery and a diagnosis of gastrointestinal dysmotility or propulsive disorder may be a bad prognostic factor in terms of success.

REFERENCES 1. Malone PSJ, Ransley PG, Kiely EM: Preliminary report: The antegrade continence enema. Lancet 336:1217-1218, 1990 2. Koyle MA, Kaji DM, Duque M, et al: The Malone antegrade continence enema for neurogenic and structural faecal incontinence and constipation. .I Urol 154:759-761,1995 3. Ellsworth PI, Webb HW, Crump JM, et al: The Malone antegrade continence enema enhances the quality of life in children undergoing urological incontinence procedures. J Ural 155:1416-141X, 1996 4. Squire R, Kiely EM, Cam B, et al: The clinical application of the Malone antegrade colonic enema. J Pediatr Surg 28:1012-1015, 1993

5. Marsh PJ, Kiff ES: Ileocaecostomy: An alternative surgical procedure for antegrade colonic enema. Br J Surg 83507-508, 1996 6. Shandling B, Chait PG, Richards HF: Percutaneous caecostomy: A new technique in the management of faecal incontinence. J Pediatr Surg 31534.537,1996 7. Dick AC, McCallion WA, Brown S, et al: Antegrade colonic enemas. Br .I Surg 83:642-643. 1996 8. Toogood GJ, Bryant PA, Dudley NE: Control of faecal incontinence using the Malone antegrade continence enema procedure: A critical appraisal. Pediatr Surg Int 10:37-39, 1995

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9. Koyle MA, Waxman SW, Duque M, et al: Applications and modification of the Malone antegrade continence enema with coincidental urinary tract reconstruction-The dual role of the appendix. Presentation ESPU 8th Annual Meeting, Rome, April 1997 10. Goepel M, Sperling H, Stohrer M, et al: Continent caecal stoma and antegrade colonic enema: Treatment option in children with faecal incontinence. Presentation ESPU 8th Annual Meeting, Rome, April I997 11. Gerharz EW, Vik Y Webb G, et al: The M.A.C.E. (Malone Antegrade Colonic Enema) procedure in adult patients with double incontinence or chronic constipation. Symposium on Reconstructive Urology. London, England, May 14-16, 1997, p 6 (abstr)

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12. Monti PR, Lara RC, Dutra MA, et al: New techniques for construction of efferent conduits based on the Mitrofanoff principle. Urology49:112-115, 1997 13. Kajbafzedeh AM, Duffy PG, Carr B, et al: A review of 100 Mitrofanoff stomas and report of the VQZ technique for the prevention of complications at stoma level. Presentation ESPU 6th Annual Meeting, Toledo, April 1995 14. Roberts JP, Moon S, Malone PSI: Treatment of neuropathic urinary and faecal incontinence with synchronous bladder reconstruction and the antegrade continence enema procedure. Br .I Urol75:386389,1995