BritishJournal ojUrology (1995), 75, 386-389

Treatment of neuropathic urinary and faecal incontinence with synchronous bladder reconstruction and the antegrade continence enema procedure* J.P. ROBERTS, S. MOON andP.S. MALONE Department of Paediatric Urology, The Wessex Centre for Paediatric Surgery, Southampton General Hospital, Southampton, UK

Objectives To report our experience with synchronous bladder reconstruction and the antegrade continence enema (ACE) procedure in the management of neuro- pathic urinary and faecal incontinence.

Patients and methods Eight patients (four boys, four girls) with a median age of 9 years 10 months (range 4.5-1 7) were treated. Five had spina bifida, two high ano-rectal malformations and one had been success- fully treated for a spinal neuroblastoma. The appendix was used for the ACE procedure in five patients and a tubularized caecal or colonic flap in the other three. Seven patients had an augmentation cystoplasty and one a cystectomy with a continent diversion. A bladder neck reconstruction or an urethral lengthening pro- cedure was performed in five patients and the bladder outlet was closed in two. Six patients required some form of revision. Four patients underwent a simul- taneous Mitrofanoff procedure.

Results The median length of follow-up was 11.5 months (range 3-29). Five patients were completely clean and the remaining three experienced minor faecal soiling only. They were all delighted with the result. Six patients were dry day and night, one was wet at night only while the final patient was wet day and night.

Conclusions Synchronous surgical procedures to make patients with neuropathic incontinence both clean and dry is effective, but most patients require revision surgery. Patient motivation and selection and the availability of a nurse specialist is crucial in obtaining satisfactory results.

Keywords Urinary, faecal, incontinence, ACE, recon- struction

Introduction

The combination of urinary and faecal incontinence is common in patients with either congenital or acquired spinal cord lesions. In a national survey of patients with spina bifida, Malone et al. [ l ] found that only 24% of patients were reliably clean and dry while 30% were doubly incontinent. For many years reconstructive urol- ogists have actively treated the neuropathic bladder, with reported success rates in the region of 80% [2]. Indeed, in the spina bifida survey [l], 23% of patients were dry but soiled, some of whom had undergone bladder reconstruction. What a waste to perform a successful bladder reconstruction only to leave the patient with ongoing faecal incontinence!

In 1990 Malone et al. [3] described the ACE procedure (Antegrade Continence Enema). This is an adaptation of the Mitrofanoff principle and produces a continent

Accepted for publication 21 September 1994 *Based on a paper presented at BAUS Annual Meeting, Birmingham, June 19Y4

386

appendico-caecostomy through which antegrade enemas are administered to produce colonic emptying and thus keep the patient clean. Success rates in the order of 90% are reported for neuropathic patients [4,5]. The operative technique is almost identical to that of the Mitrofanoff procedure so there is no reason why the ACE should not be performed by the reconstructive urologist simultaneously with bladder reconstruction. In this way patients could be made both clean and dry, improving not only their quality of life, but also their independence and integration into society. We describe our early experiences with this combined approach.

Patients and methods

Eight patients who were undergoing synchronous blad- der reconstruction and the ACE procedure were prospec- tively studied (Table 1). The median age was 9 years 10 months (range 4.5-17); there were four boys and four girls. All had neuropathic bladders, demonstrated by videourodynamic studies, secondary to spina bifida (five patients), a treated neuroblastoma with a spinal

SYNCHRONOUS BLADDER RECONSTRUCTION AND ACE PROCEDURE 387

Table 1 Patients and operative details

Patirrit I IO. Agc (yearsj Sex Primary diagnosis Urolngiral prorcdurc Further procedures

1 1 5 M Spina bifida

7 - 11.5 F Neuroblastoma

3 4.5

9.7

6.8

17

M High ARA* llrethral stricture

Spina bifida

Spina biEda

Spina bifida

7 10 M High ARA Cerebral palsy

8 10 M Spina bifida

Clam ileocystoplasty Mitrofanoff Close bladder neck YDL bladder neck reconstruction Colocystoplasty

Clam ileocystoplasty Mitrofanoff

Pippi Salk urethral lengthening Ileocystaplasty Pippi Salle urethral lengthening Ureterocystoplasty Sigmoid colocystoplasty Mitrofanoff Closure of urethra Total cystectomy Colonic pouch and Mitrofanoff Pippi Salle urethral lengthening Colocystoplasty

Revision of Mitrofanoff stoma Division of urethra

Dilatation ACE Bladder neck injection Bladder neck injectiont Revision ACE,$ Mitrofanoff stomas Repeat Kropp

Colonic augmentation

Nil

Nil

Awaiting

* High ano-rectal anomaly. t Previous YDL bladder neck reconstruction. $Young-Dees-Leadbetter.

extension (one patient), high ano-rectal malformation with associated sacral anomalies and a chronic urethral stricture (one patient), and a high ano-rectal malfor- mation and cerebral palsy (one patient). Six patients were ambulant (two with the assistance of a frame or sticks) and two were wheelchair-bound.

Pre-operatively three patients were managed by an indwelling catheter and the rest were wet despite con- servative treatment. All were faecally incontinent.

In five patients the ACE procedure was performed using the reversed appendix [3]. In one of these the appendix was long, enabling it to be divided using one end for the ACE and the other for the Mitrofanoff. In the remaining three the appendix was needed for the Mitrofanoff and a tubularized flap of caecum or colon was employed (Fig. 1) [4]. This entailed creating a 4 x 2 cm flap based on the lateral vessels, and tubulariz- ing it snugly over an 8 F catheter. No anti-reflux pro- cedure was needed and leakage has not been a problem.

Post-operatively , five patients used alternate day wash- outs and the rest daily. S i x used a combination of phosphate enemas and saline and the other two used saline washouts only. The average time required for the ACE regimen was approximately 1 h. The ACE regimen eventually chosen was very personalized and was achieved mostly through trial and error.

Bladder reconstruction comprised an augmentation cystoplasty in seven patients: detubularized colonic pouch (three patients), clam ileocystoplasty (three

Pig. 1. Completed caecal flap stoma with appendix prepared for Mitrofanoff stoma.

patients), and ureterocystoplasty (one patient) [6] . One patient with a particularly small, thick-walled bladder had a cystectomy and a continent diversion using a detubularized ileocaecal segment with a Mitrofanoff. The bladder outlet was closed off in two patients, a Young- Dees-Leadbetter bladder neck reconstruction was per- formed in one, and a Pippi Salle urethral lengthening procedure in three [7]. The Pippe Salle procedure involves creating an 8 x 2cm full-thickness anterior blad- der-wall flap based on the bladder neck, and anastonios- ing it to a 1.5 cm wide strip of trigone and bladder in two layers (mucosa and muscle), over an 8 F catheter.

British ]oirrtml oJ Urohfly (1995). 75

388 J . P . ROBERTS. S . MOON and P.S. MALONE

The ureters need to be identified and may need to be reimplanted if they are lying close to the suture line. Bladder neck reconstruction and bladder augmentation was combined in four patients in whom urodynamic studies demonstrated a leak at a volume of 90-250 mL and a pressure of < 20 cm. A total of four patients had a simultaneous Mitrofanoff procedure using the appendix.

Five patients required some form of revision surgery and one will probably require it. In the patient with the ureterocystoplasty, a normal calibre ureter was used but persistent hyperreflexia necessitated a colonic pouch augmentation. Three patients needed dilatation or revision of the ACE or Mitrofanoff stoma. Three of the five patients with a bladder outlet procedure needed revision, with a repeat Kropp in one and bladder neck injection [ 81, using silicone particle suspension (macro- plastique), in two. One patient with bladder neck closure needed a subsequent urethral division. Follow-up results were assessed in out-patients and during domiciliary visits by a urology nurse specialist.

Results

The results are summarlzed in Table 2 . The median length of follow-up was 11.5 months (range 3-29). With the ACE procedure, five patients were completely clean while the other three suffered minor leakage only, usually involving washout fluid within a few hours of administration.

Six patients were completely dry on intermittent cath- eterization with an interval exceeding 3 h. One was dry by day with 3 hourly catheterization, but was wet at night. One patient was wet both day and night and is awaiting further evaluation and possible surgery.

Discussion Faecal incontinence associated with a neuropathic blad- der is common, but frequently is overlooked. Recent surveys have shown that around 84% of children with spina bifida and neuropathic bladders have abnormal

Table 2 Urinary and ACE washout results

CIC > 3 hourly Completely clean 5 Dry day and night 6

CIC= 3 hourly Occasional minor Dry by day soiling ( < l w e e k ) 3 wet at night 1

CIC = 3 hourly Duration of ACE (hour) Wet day and night 1 Range 0.5-1.5

Median (L) 1

CIC. Clean intermittent catheterization

bowel control [9], which frequently persists into adult life [I]. Up until recently there were no surgical options, other than a colostomy, if conservative management failed. The ACE procedure has changed this, and its effectiveness is again shown in this series.

The high rate of revision surgery is disappointing, but probably reflects the complexity of this surgery. The patient who had a ureterocystoplasty performed using a normal calibre ureter required a further augmentation. This has not been seen in previous series [6], and probably indicates that a mega-ureter is required for this procedure to be successful. Three patients required ACE/ Mitrofanoff stoma revision: this is a common feature of larger series [4,5]. However, after the initial revision, the stoma settles down and further surgery is generally not needed. We have recently modified the inlay cutaneous anastomosis by not closing the skin together superficially to reduce the incidence of stenosis (Fig. 2 ) .

The high failure rate for bladder neck reconstruction and urethral lengthening exceeds that seen in other series [2,10]. Initial experience with the Pippi Salle urethral lengthening showed one of three to be success- ful, with another awaiting further investigations. Similar initial experience has been reported [ 71. The high failure rate may also reflect attempts to preserve urethral access to the bladder and keep the appendix for the ACE. when under normal circumstances the bladder outlet would be closed. Alternative strategies would be to use the distal ureter (combined with transureteroureterostomy ), a tubularized segment of ileum, the fallopian tube or even the vas for the bladder conduit [ll], abandoning the urethra in unfavourable situations, and leaving the appendix for the ACE. A further possibility would be to use an artificial urinary sphincter. However, with the success of the tubularized caecal or colonic flap, used in the latter stages of this study, we would recommend

Fig. 2. Modified antegrade continence enema/Mitrofanoff stoma.

British Journal of Urology (1995). 75

SYNCHRONOUS BLADDER RECONSTRUCTION AND ACE PROCEDURE 389

using the appendix for the Mitrofanoff and not striving to preserve it for the ACE stoma.

There is little doubt that, with revision surgery, the majority of patients ( 7 of 8) can be made both dry and clean, an expectation not previously achievable. Patient selection is of vital importance to ensure a successful outcome; they need to be motivated and have a clear understanding that multiple procedures may be needed and that a lifelong committment to catheterization and washouts is essential. With the presence of two stomas on the abdominal wall it is vital to educate the patient to ensure colonic washouts are delivered into the correct stoma. We have found the constant encouragement and advice that can be offered to these patients by a urology nurse specialist to be invaluable.

There is no worthier goal than to make a doubly incontinent patient both clean and dry. This can be achieved by a synchronous bladder reconstruction and ACE procedure. As the ACE procedure is essentially an adaptation of an urological procedure (the Mitrofanoff) we believe that it is the reconstructive urologist who is ideally situated to provide this service.

References Malone PS, Wheeler RA, Williams JE. Continence in spina bifida patients: long term results. Arch Dis Child 1994;

Nurse DE, Britton JP, Munday AR. Relative indication for orthotopic lower urinary tract reconstruction, continent urinary diversion and conduit urinary diversion. Br J Urol

Malone PS, Ransley PG. Preliminary report: the antegrade continence enema. Lancet 1990; 336: 1217-8

70: 107-10

1993; 71: 562-65

4 Squire R, Kiely EM, Carr B, Ransley PG, Duffy PG. The clinical application of the Malone antegrade colonic enema. J Pedintr Surg 1993; 28: 1012-5

5 Griffiths DM, Malone PS. The Malone antegrade continence enema (MACE). J Pediatr Surg 1995; 30: 68-71

6 Hitchcock RJI, Duffy PG, Malone PS. Ureterocystoplasty: the ‘biadder’ augmentation of choice. Br J Urol 1994: in press

7 Pippi Salle JL, de Fraga JCS, Silvera ML, Lambertz M, Schmid M. Urethral lengthening with anterior bladder wall flap for urinary incontinence: a new approach. American Academy ofpediatrics, Washington 1993: 105-6 (Abstract)

8 Caoni P. Lais A, De Gennaro M, Lapozza N. Gluteraldehyde cross-linked bovine collagen in exstrophy/epispadias com- plex. ] Urol 1993; 150: 631-3

9 Lie HR, Lagergren J, Rasmussen F et al. Bowel and bladder control of children with myelomeningocele: a Nordic study. Dev Med Child Neurology 1991; 33: 1053-61

10 Jones JA, Mitchell ME, Rink RC. Improved results using a modification of the Young-Dees-Leadbetter bladder neck repair. Br ] Urol 1993; 71: 555-61

11 Dykes EH, Du@ PG, Ransley PG. The use of the Mitrofanoff principle in achieving clean intermittent catheterisation and urinary continence in children. ] Pediatr Surg 1991; 26: 535-8

Authors J.P. Roberts MS, FRCS(PAED), Senior Registrar. S . Moon, RGN, RSCN. Urology Nurse Specialist. P.S. Malone, MCh, FRCSI, Consultant Paediatric Urologist. Correspondence: Mr P.S. Malone. Department of Paediatric Urology, The Wessex Centre for Paediatric Surgery, Southampton General Hospital, Tremona Road, Southampton S0164XY, UK.

British Journal o/ Urology (1995). 75