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The malone antegrade continence enema
The Malone Antegrade Continence Enema By D.M. Griffiths and P,S. Malone
Southampton, England • The previously reported Malone antegrade continence enema (MACE) was used in 21 children for intractable fecal incontinence or constipation. Twelve are completely clean, and three are much improved (71% success rate). However, only four of the 21 have not had some type of complication, either minor or major. Five of the 21 now have a colostomy, and one has abandoned the procedure.
Copyright © 1995by W.B. Saunders Company INDEX WORDS: Fecal incontinence.
INCONTINENCE or intractable constipa~ ECAL tion causes serious problems for children. If medical management with laxatives, suppositories, enemata, or the Shandling tube failed? a child's options were continuing incontinence or a colostomy. Results of preliminary studies of the Malone antegrade continence enema (MACE) 2,3 suggested that this procedure might be beneficial in the treatment of these children. Herein we report our results with a larger series. MATERIALS AND METHODS Between March 1991 and December 1992, 21 children have had a MACE for persistent soiling (Table 1). Medical treatment of their fecal incontinence or constipation had failed to achieve an acceptable result, and soiling persisted. This resulted in some being teased at school, and the older children often were withdrawn and socially immature. The age at time of operation was 18 months to 18 years (median, 12 years). After a routine complete bowel preparation, a continent appendicocecostomy was created with or without reversal of the appendix. The other end of th e appendix was brought through the anterior abdominal wall in the right iliac fossa (Figs 1 and 2). The angle of the V-flap is 100°, with sides of 3 cm in length and with optional extensions of 1 to 2 cm. The dorsal wall of the appendix was fishtailed (Fig 3), and the tip of the skin flap was inset and sutured with interrupted absorbable sutures (Fig 4) so that initially the flap resembled a gutter (Fig 5). Suturing of the flap to the appendix was continued around the appendiceal lumen to form a tube, and then continued so that a short ( < 1 cm) skin tunnel could be created to bury the appendiceal mucosa, thus avoiding the problems of exposed mucosa (Fig 6). The resulting skin defect was closed in two layers (Fig 7). The cecum was fixed to the anterior abdominal wall to avoid traction on the stoma and to ensure long-term ease of catheterization. Throughout the operation, the
From the Wessex Regional Centrefor Paediatric Surgery, Southampton General Hospital, Southampton, England. Date accepted: October 6, 1993. Address reprint requests to D.M. Griffiths, MCh, FRCS, Wessex Regional Centre for Paediatric Surgery, Southampton General Hospital, Tremona Rd, Southampton S09 4XY,, England. Copyright © 1995 by W..B. Saunders Company 0022-3468/95/3001-0018503. 00/0 68
ease of cecal catheterization was confirmed a t frequent intervals. An indwelling 8, 10, or 12F SILASTIC ® (Dow Coming, Midland, MI) Foley catheter was used postoperatively for 2 weeks. Three patients underwent simultaneous bladder reconstruction; the Mitrofanoff principle was used in two. 4 One patient's appendix was long enough (with favorable vascular anatomy) to divide it; half was used for the bowel and half for the bladder. The other patient's appendix was used for the bladder, and a tubularized transverse cecal flap was used for the MACE. 5 After the fourth day, when bowel peristalsis has recovered fully, the catheter was used for the insertion of a phosphate enema into the cecum (with or without a normal-saline flush). A period of trial-and-error is required to determine each patient's precise requirements in terms of the volume of phosphate enema and subsequent saline flushings. Most children empty their colon within 15 to 45 minutes, but any degree of distal obstruction will have caused a secondary megacolon, which may require greater volumes initially. After removal of the indwelling catheter (after 2 weeks in situ) the children catheterize themselves at least daily, with a stiff catheter (8 or 10F), even if they require enemata less frequently, to avoid stenosis of the anastamoses. After discharge on the fourth or fifth day, the children are monitored closely by a pediatric community nurse or by the surgical team (via phone) to assess the correct enema volume. The phosphate enema initiates defecation, and the normal saline flush ensures that the colon is completely clean. Some children require no flush, others require no phosphate enema, and others need large volumes of both. RESULTS
Twelve of the 21 children have become completely clean. Three others have had great improvement (95 % clean) and are prepared to cope with occasional leakage. Six children were not helped by the procedure (29%); five now have colostomies, and the sixth has refused to continue with the enemas and is still fecally incontinent. Four of the five children with a colostomy have definite or possible motility problems underlying the incontinence. One boy had Hirschsprung's disease, was fecally incontinent, and previously had a colostomy for the incontinence, which had been closed. Barium studies of the colon showed that the MACE enema reached the anus and then refluxed back into the transverse colon. One child had severe mental retardation, malrotation, adhesion obstruction, and an episode of phosphate poisoning. 6 Her parents could no longer cope with the MACE and its complications and requested a colostomy. One patient, an 18-month-old boy who was the product of an in vitro fertilization, had a high anorectal anomaly and was never clean despite massive colonic lavage, and his perianal area was raw. His MACE never worked satisfactorily, and we suspect a motility problem. He now has a right-sided colostomy. One boy with unJournalofPediatric Surgery, Vo130, No 1 (January), 1995: pp 68-71
MALONE ANTEGRADE CONTINENCE ENEMA
69
Table 1. Causes of Fecal Incontinence
Neuropathic Spina bifida Arthrogryposis multiplex congenita Spinal neuroblastoma Paraquat poisoning High anorectal anomaly Chronic constipation Hirschsprung's disease
9 6 1 1 1 8 3 1
diagnosed motility problems was given a colostomy as a toddler. Reconnection was requested, but he was incontinent. The MACE never cleansed him, and he now has another colostomy. A 10-year-old boy with a high anorectal anomaly was initially completely clean. Pain developed after 2 months, which became severe. A laparotomy showed that the cecum had fallen partially from the anterior abdominal wall and was potentially torting; it was resutured in place. However, the pain persisted, and a colostomy was requested. All five children with a colostomy are happy and coping well. The nine children with a neuropathic cause have done best (Table 1). Six are completely clean, two are more than 95% clean, and one has stopped using the MACE for psychological reasons. Two of the children with idiopathic constipation have colostomies, but the third is clean. Five of eight children with high anorectal anomalies are clean, one is much improved, and two have colostomies. The child with Hirschsprung's disease has a colostomy.
Fig 1.
Skin incisions.
. { i~
:;:,
!4"'\
.
. .
'"
Fig 2. The tip of the appendix is delivered into the wound.
The complications are listed in Table 2. The stoma has broken down in five, leaving the mucosa exposed on the surface. Five have required balloon dilatation for stenosis, and one closed over completely but was reopened with ease. One boy initially refused to catheterize himself but eventually coped with his psychological problems and is now clean. Three children required laparotomy (one of them twice) for obstruction or cecal torsion. A mentally retarded child who had colonic adhesion obstruction after Ladd's procedure was given three adult phosphate enemata (384 mL), which were not successful. She collapsed with hyperphosphatemia but recovered with appropriate treatment. 6 Some children had considerable pain when the phosphate enema was inserted,
Fig 3.
The dorsal wall of the appendix is fishtailed.
GRIFFITHS AND MALONE
70
! Fig 6. Continued suturing of the flap to the appendix creates a tube that is continued for 0.5 to 1.0 cm.
-
.
Fig 4.
-
.
. •
The skin flap is inset into the fishtail•
but this has been resolved by diluting the phosphate in an equal volume of saline and warming it. Two children with neuropathic bladders were believed to be dry after conservative treatment, and were in nappies only because of fecal incontinence. However, now that they are clean and want to wear pants, it is apparent that both are wet and will need subsequent bladder reconstructive surgery to address this. DISCUSSION
To convert an introverted, fecally incontinent child in a nappy into a normally adjusted, clean child in pants, without the need of a bag, is immensely satisfying. However, all operations have risks, and the MACE procedure is no exception. One child nearly
died of massive hyperphosphatemia. Only four of the 21 have not had some sort of complication, ranging from minor wound infection or stenosis to stomal breakdown and the need for laparotomy. Five of the 21 now have a colostomy, and one other has stopped using the MACE. If a child is socially continent with a regimen of enemata, suppositories, or washouts, it is probably inappropriate to risk the complications of surgery unless it could make them independent. Older children who are wheelchair-bound with spina bifida can perform intermittent cecal catheterization but cannot administer rectal enemata; thus, the procedure might be beneficial. Construction of a stoma with burial of the appendiceal tip avoids all the problems of exposed mucosa (bleeding, mucus discharge, and the need for dressing) and is an integral part of the procedure. Children who have appendixes at skin level are not as pleased as those whose appendixes are buried. With the Mitrofanoff stoma, the appendix is used as a conduit for bladder catheterization; hence it is dilated four hourly. Initially we did not realize the importance of daily catheterisation in avoiding stenosis, even if an enema is required only on alternate days. We now recommend daily or twice-daily catheterization to keep the stoma patent, and problems with stomal stenosis have been reduced. Experience suggests that the appendix does not
":"2 :.,',,
•
•
.
..
Fig 5.
%%; : \
,
Initially the flap resembles a gutter.
Fig 7.
The skin edges are approximated as in a V-Y flap.
MALONE ANTEGRADE CONTINENCE ENEMA
71
Table 2. Complications Laparotomy Stoma breakdown Stomal stenosis Stoma closed over Wound infection Pain with enema Psychological problems Phosphate overdose
3 5 5 1 1 4 2 1
need to be reimplanted in the cecum to prevent fecal leakage; simply bringing out the tip of the appendix apparently works as well, as in did in our two patients. If the appendix has been removed or is needed for a simultaneous Mitrofanoff procedure, a transverse cecal flap can be created. This is working well in one of our patients. If a patient with a neuropathic bladder has not had success with conservative treatment, it is best to perform bladder reconstruction simultaneously. This requires accurate preoperative assessment and planning. We did not recognize the degree of urinary incontinence in two patients who must now undergo additional surgery. With more accurate assessment in the future and the realization that the degree of
urinary incontinence can be easily underestimated in the fecally incontinent child, this problem will be avoided. If incurable problems remain after the MACE is performed, it is always possible to use the older surgical approach of colostomy, without prejudicing its chance of success. We suggest that in the future, the treatment of children with high anorectal anomalies will change. They have a very poor chance of continence with no treatment, despite newer surgical techniques. By the time they go to school, they will have had 2 years in nappies and 2 to 3 years of suppositories, enemas, and washouts to see if they can be socially continent. If not, a MACE could be performed before they start school. Because they are not otherwise externally handicapped, they are expected to be normal, and unless they are reliably clean by 8 years of age, some can have major psychological morbidity. Now it is possible to avoid this. The treatment of constipation and soiling in adults by subtotal colectomy has the advantage of no stoma, but it produces frequent bowel actions. The MACE is an alternative procedure that would not preclude subtotal colectomy if it failed.
REFERENCES 1. Shandling B, Gilmour RF: The enema continence catheter in spina bifida; Successful bowel management. J Pediatr Surg 22:271273, 1987 2. Malone PS, Ransley PG, Keily EM: Preliminary report; The antegrade continence enema. Lancet 336:1217-1218, 1990 3. Wheeler RA, Malone PS: The use of the appendix in reconstructive surgery: A case against incidental appendicectomy. Br J Surg 78:1283-1285, 1991 4. Mitrofanoff P: Cystostomie Continente Trans-Appendiculare
Dans Le Tratemente Des Vessies Neurologiques. Chir Pediatr 21:29%305, 1980 5. Squire R, Kiely EM, Carr B, et al: The clinical application of the Malone antegrade colonic enema. J Pediatr Surg 28:1012-1015, 1993 6. Hunter M, Ashton MR, Griffiths DM, et al: Hyperphosphataemia after enemas in childhood; Prevention and treatment. Arch Dis Child 68:233-234, 1993
Southampton, England • The previously reported Malone antegrade continence enema (MACE) was used in 21 children for intractable fecal incontinence or constipation. Twelve are completely clean, and three are much improved (71% success rate). However, only four of the 21 have not had some type of complication, either minor or major. Five of the 21 now have a colostomy, and one has abandoned the procedure.
Copyright © 1995by W.B. Saunders Company INDEX WORDS: Fecal incontinence.
INCONTINENCE or intractable constipa~ ECAL tion causes serious problems for children. If medical management with laxatives, suppositories, enemata, or the Shandling tube failed? a child's options were continuing incontinence or a colostomy. Results of preliminary studies of the Malone antegrade continence enema (MACE) 2,3 suggested that this procedure might be beneficial in the treatment of these children. Herein we report our results with a larger series. MATERIALS AND METHODS Between March 1991 and December 1992, 21 children have had a MACE for persistent soiling (Table 1). Medical treatment of their fecal incontinence or constipation had failed to achieve an acceptable result, and soiling persisted. This resulted in some being teased at school, and the older children often were withdrawn and socially immature. The age at time of operation was 18 months to 18 years (median, 12 years). After a routine complete bowel preparation, a continent appendicocecostomy was created with or without reversal of the appendix. The other end of th e appendix was brought through the anterior abdominal wall in the right iliac fossa (Figs 1 and 2). The angle of the V-flap is 100°, with sides of 3 cm in length and with optional extensions of 1 to 2 cm. The dorsal wall of the appendix was fishtailed (Fig 3), and the tip of the skin flap was inset and sutured with interrupted absorbable sutures (Fig 4) so that initially the flap resembled a gutter (Fig 5). Suturing of the flap to the appendix was continued around the appendiceal lumen to form a tube, and then continued so that a short ( < 1 cm) skin tunnel could be created to bury the appendiceal mucosa, thus avoiding the problems of exposed mucosa (Fig 6). The resulting skin defect was closed in two layers (Fig 7). The cecum was fixed to the anterior abdominal wall to avoid traction on the stoma and to ensure long-term ease of catheterization. Throughout the operation, the
From the Wessex Regional Centrefor Paediatric Surgery, Southampton General Hospital, Southampton, England. Date accepted: October 6, 1993. Address reprint requests to D.M. Griffiths, MCh, FRCS, Wessex Regional Centre for Paediatric Surgery, Southampton General Hospital, Tremona Rd, Southampton S09 4XY,, England. Copyright © 1995 by W..B. Saunders Company 0022-3468/95/3001-0018503. 00/0 68
ease of cecal catheterization was confirmed a t frequent intervals. An indwelling 8, 10, or 12F SILASTIC ® (Dow Coming, Midland, MI) Foley catheter was used postoperatively for 2 weeks. Three patients underwent simultaneous bladder reconstruction; the Mitrofanoff principle was used in two. 4 One patient's appendix was long enough (with favorable vascular anatomy) to divide it; half was used for the bowel and half for the bladder. The other patient's appendix was used for the bladder, and a tubularized transverse cecal flap was used for the MACE. 5 After the fourth day, when bowel peristalsis has recovered fully, the catheter was used for the insertion of a phosphate enema into the cecum (with or without a normal-saline flush). A period of trial-and-error is required to determine each patient's precise requirements in terms of the volume of phosphate enema and subsequent saline flushings. Most children empty their colon within 15 to 45 minutes, but any degree of distal obstruction will have caused a secondary megacolon, which may require greater volumes initially. After removal of the indwelling catheter (after 2 weeks in situ) the children catheterize themselves at least daily, with a stiff catheter (8 or 10F), even if they require enemata less frequently, to avoid stenosis of the anastamoses. After discharge on the fourth or fifth day, the children are monitored closely by a pediatric community nurse or by the surgical team (via phone) to assess the correct enema volume. The phosphate enema initiates defecation, and the normal saline flush ensures that the colon is completely clean. Some children require no flush, others require no phosphate enema, and others need large volumes of both. RESULTS
Twelve of the 21 children have become completely clean. Three others have had great improvement (95 % clean) and are prepared to cope with occasional leakage. Six children were not helped by the procedure (29%); five now have colostomies, and the sixth has refused to continue with the enemas and is still fecally incontinent. Four of the five children with a colostomy have definite or possible motility problems underlying the incontinence. One boy had Hirschsprung's disease, was fecally incontinent, and previously had a colostomy for the incontinence, which had been closed. Barium studies of the colon showed that the MACE enema reached the anus and then refluxed back into the transverse colon. One child had severe mental retardation, malrotation, adhesion obstruction, and an episode of phosphate poisoning. 6 Her parents could no longer cope with the MACE and its complications and requested a colostomy. One patient, an 18-month-old boy who was the product of an in vitro fertilization, had a high anorectal anomaly and was never clean despite massive colonic lavage, and his perianal area was raw. His MACE never worked satisfactorily, and we suspect a motility problem. He now has a right-sided colostomy. One boy with unJournalofPediatric Surgery, Vo130, No 1 (January), 1995: pp 68-71
MALONE ANTEGRADE CONTINENCE ENEMA
69
Table 1. Causes of Fecal Incontinence
Neuropathic Spina bifida Arthrogryposis multiplex congenita Spinal neuroblastoma Paraquat poisoning High anorectal anomaly Chronic constipation Hirschsprung's disease
9 6 1 1 1 8 3 1
diagnosed motility problems was given a colostomy as a toddler. Reconnection was requested, but he was incontinent. The MACE never cleansed him, and he now has another colostomy. A 10-year-old boy with a high anorectal anomaly was initially completely clean. Pain developed after 2 months, which became severe. A laparotomy showed that the cecum had fallen partially from the anterior abdominal wall and was potentially torting; it was resutured in place. However, the pain persisted, and a colostomy was requested. All five children with a colostomy are happy and coping well. The nine children with a neuropathic cause have done best (Table 1). Six are completely clean, two are more than 95% clean, and one has stopped using the MACE for psychological reasons. Two of the children with idiopathic constipation have colostomies, but the third is clean. Five of eight children with high anorectal anomalies are clean, one is much improved, and two have colostomies. The child with Hirschsprung's disease has a colostomy.
Fig 1.
Skin incisions.
. { i~
:;:,
!4"'\
.
. .
'"
Fig 2. The tip of the appendix is delivered into the wound.
The complications are listed in Table 2. The stoma has broken down in five, leaving the mucosa exposed on the surface. Five have required balloon dilatation for stenosis, and one closed over completely but was reopened with ease. One boy initially refused to catheterize himself but eventually coped with his psychological problems and is now clean. Three children required laparotomy (one of them twice) for obstruction or cecal torsion. A mentally retarded child who had colonic adhesion obstruction after Ladd's procedure was given three adult phosphate enemata (384 mL), which were not successful. She collapsed with hyperphosphatemia but recovered with appropriate treatment. 6 Some children had considerable pain when the phosphate enema was inserted,
Fig 3.
The dorsal wall of the appendix is fishtailed.
GRIFFITHS AND MALONE
70
! Fig 6. Continued suturing of the flap to the appendix creates a tube that is continued for 0.5 to 1.0 cm.
-
.
Fig 4.
-
.
. •
The skin flap is inset into the fishtail•
but this has been resolved by diluting the phosphate in an equal volume of saline and warming it. Two children with neuropathic bladders were believed to be dry after conservative treatment, and were in nappies only because of fecal incontinence. However, now that they are clean and want to wear pants, it is apparent that both are wet and will need subsequent bladder reconstructive surgery to address this. DISCUSSION
To convert an introverted, fecally incontinent child in a nappy into a normally adjusted, clean child in pants, without the need of a bag, is immensely satisfying. However, all operations have risks, and the MACE procedure is no exception. One child nearly
died of massive hyperphosphatemia. Only four of the 21 have not had some sort of complication, ranging from minor wound infection or stenosis to stomal breakdown and the need for laparotomy. Five of the 21 now have a colostomy, and one other has stopped using the MACE. If a child is socially continent with a regimen of enemata, suppositories, or washouts, it is probably inappropriate to risk the complications of surgery unless it could make them independent. Older children who are wheelchair-bound with spina bifida can perform intermittent cecal catheterization but cannot administer rectal enemata; thus, the procedure might be beneficial. Construction of a stoma with burial of the appendiceal tip avoids all the problems of exposed mucosa (bleeding, mucus discharge, and the need for dressing) and is an integral part of the procedure. Children who have appendixes at skin level are not as pleased as those whose appendixes are buried. With the Mitrofanoff stoma, the appendix is used as a conduit for bladder catheterization; hence it is dilated four hourly. Initially we did not realize the importance of daily catheterisation in avoiding stenosis, even if an enema is required only on alternate days. We now recommend daily or twice-daily catheterization to keep the stoma patent, and problems with stomal stenosis have been reduced. Experience suggests that the appendix does not
":"2 :.,',,
•
•
.
..
Fig 5.
%%; : \
,
Initially the flap resembles a gutter.
Fig 7.
The skin edges are approximated as in a V-Y flap.
MALONE ANTEGRADE CONTINENCE ENEMA
71
Table 2. Complications Laparotomy Stoma breakdown Stomal stenosis Stoma closed over Wound infection Pain with enema Psychological problems Phosphate overdose
3 5 5 1 1 4 2 1
need to be reimplanted in the cecum to prevent fecal leakage; simply bringing out the tip of the appendix apparently works as well, as in did in our two patients. If the appendix has been removed or is needed for a simultaneous Mitrofanoff procedure, a transverse cecal flap can be created. This is working well in one of our patients. If a patient with a neuropathic bladder has not had success with conservative treatment, it is best to perform bladder reconstruction simultaneously. This requires accurate preoperative assessment and planning. We did not recognize the degree of urinary incontinence in two patients who must now undergo additional surgery. With more accurate assessment in the future and the realization that the degree of
urinary incontinence can be easily underestimated in the fecally incontinent child, this problem will be avoided. If incurable problems remain after the MACE is performed, it is always possible to use the older surgical approach of colostomy, without prejudicing its chance of success. We suggest that in the future, the treatment of children with high anorectal anomalies will change. They have a very poor chance of continence with no treatment, despite newer surgical techniques. By the time they go to school, they will have had 2 years in nappies and 2 to 3 years of suppositories, enemas, and washouts to see if they can be socially continent. If not, a MACE could be performed before they start school. Because they are not otherwise externally handicapped, they are expected to be normal, and unless they are reliably clean by 8 years of age, some can have major psychological morbidity. Now it is possible to avoid this. The treatment of constipation and soiling in adults by subtotal colectomy has the advantage of no stoma, but it produces frequent bowel actions. The MACE is an alternative procedure that would not preclude subtotal colectomy if it failed.
REFERENCES 1. Shandling B, Gilmour RF: The enema continence catheter in spina bifida; Successful bowel management. J Pediatr Surg 22:271273, 1987 2. Malone PS, Ransley PG, Keily EM: Preliminary report; The antegrade continence enema. Lancet 336:1217-1218, 1990 3. Wheeler RA, Malone PS: The use of the appendix in reconstructive surgery: A case against incidental appendicectomy. Br J Surg 78:1283-1285, 1991 4. Mitrofanoff P: Cystostomie Continente Trans-Appendiculare
Dans Le Tratemente Des Vessies Neurologiques. Chir Pediatr 21:29%305, 1980 5. Squire R, Kiely EM, Carr B, et al: The clinical application of the Malone antegrade colonic enema. J Pediatr Surg 28:1012-1015, 1993 6. Hunter M, Ashton MR, Griffiths DM, et al: Hyperphosphataemia after enemas in childhood; Prevention and treatment. Arch Dis Child 68:233-234, 1993