FEATURE

The Mitrofanoff procedure: a continent revolution

BY RACHEL BARRATT, THERESA MARSDEN AND TAMSIN J GREENWELL

rior to 1980, surgeons had appendix has been the technique of choice closure or into a heterotopic neobladder been struggling to provide a for MACE formation, although the use of may offer symptom control in both catheterisable, continent channel MACE has significantly reduced of late. paediatric and adults with end-stage Pas an alternative to the native In this article we will discuss the incontinence who have failed to respond to urethra, primarily for paediatric patients indications, preoperative counselling, multiple conventional surgical interventions with congenital neuropathic bladder. In operative technique, alternatives and [13]. 1980, Professor Paul Mitrofanoff described surgical variations of the Mitrofanoff A Mitrofanoff channel is often the continent supravesical antireflux channel as well as its long-term constructed simultaneously with other appendicovesicostomy [1] in which the management and outcomes. forms of lower urinary tract reconstructions appendix is harvested on its vascular pedicle such as heterotopic neobladder and and refashioned into a catheterisable Indications enterocystoplasty, in order to allow continent channel passing in an antireflux The Mitrofanoff channel was originally continent storage and volitional emptying manner into the native bladder or described as a technique for the restoration of urine. The MACE has somewhat gone neobladder. This procedure provides a short, of continent bladder emptying in children out of surgical fashion but may be used straight and easily catheterisable channel with congenital neuropathic bladder to effect faecal continence in patients that has shown longevity and a relative lack dysfunction such as spinal dysraphism [1]. with neuropathic bowel dysfunction of surgical and metabolic complications. Since its inception it has developed a range (spinal dysraphism, cloacal exstrophy and Since then the Mitrofanoff principle has of applications in this form, in both adult imperforate anus). been expanded utilising many other tissues, and paediatric populations. These include most commonly reconfigured small bowel replacing a damaged, absent or structurally (Yang [2], Monti et al. [3]) but also utilising abnormal urethra in the following situations Preoperative work-up colon, ureter, stomach, fallopian tube and [1,8-10]: All patients being considered for a vas deferens [4,5]. In 1990 Malone et al. Mitrofanoff procedure should be discussed combined the Mitrofanoff principle with the • Traumatic loss (pelvic fracture or in a multidisciplinary team (MDT) meeting concept of an antegrade continence enema gunshot) with failed reconstruction and require input from a range of healthcare (MACE) to create the MACE for treatment • Congenitally absent urethra professionals to assess dexterity, patient of faecal incontinence and / or constipation • Urethrectomy for urethral malignancy motivation, psychosocial status and [6]. • Prune belly syndrome trajectory of any pre-existing neurological The appendix is the conduit of first • Cloacal exstrophy disease. Nutritional status, liver function choice because of ease of use and relative • Sacral agenesis and existing co-morbid conditions should be lack of complications and should be • Bladder exstrophy optimised preoperatively. If the Mitrofanoff used in preference for MACE formation if • Episapdias channel is to be anastomosed into a native synchronous MACE and Mitrofanoff / Monti • Posterior urethral valves bladder it is imperative that the bladder channel is planned [7]. Since 1997 in situ • Anorectal agenesis is compliant with low pressure filling • Multiple sclerosis and therefore video urodynamics are an • Spinal cord injury essential part of preoperative planning. Patients with a history of inflammatory Alternatively a Mitrofanoff channel can be bowel disease and pelvic radiotherapy “The patient must have tunnelled into a bowel neobladder to form are relative contraindications for the a continent urinary diversion following sufficient manual and Mitroffanoff procedure as these can cystectomy and reconstruction for a range threaten the viability of the anastomosis mental dexterity to perform of indications including [11,12]: and long-term function of the channel. stomal catheterisation • Carcinoma Patients and care-givers (if the patient’s and understand the need • End-stage interstitial cystitis upper limb function is impaired) should • Tuberculosis be educated and motivated to perform for life-long follow-up, • Ketamine bladder clean self-intermittent catheterisation via • Unreconstructable vesico-vaginal the channel. In addition, they should be and the pros and cons of fistula aware of the potential complications of the the procedure and the procedure, the need for long-term follow- Finally, a Mitrofanoff channel into native up and, in particular, the requirement for alternatives.” bladder when combined with bladder neck revision of the Mitrofanoff channel.

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Contraindications Fortunately there appears to be similar rates The patient must have sufficient manual of continence and requirement for future and mental dexterity to perform stomal revision operations between Mitrofanoff catheterisation and understand the need for channels implanted in native bladder life-long follow-up, and the pros and cons of versus bowel augment or neobladder the procedure and the alternatives. They do providing a tunnelled anti-reflux technique not however require as much dexterity and is utilised [17]. The surgeon can choose mobility as is required to undertake urethral the best functional position to implant intermittent self-catheterisation (ISC) and the Mitrofanoff channel without being the Mitrofanoff technique has been utilised concerned about avoiding intestinal with good effect in quadriplegic patients. augmented sections. Patients must be highly motivated in order Conversely, in order to achieve to undertake a lifetime of ISC +/- MACE continence, some patients with large volume catheterisation and to cope with the revision bladders may require additional procedures surgery that is often required. to achieve urethral continence ranging from The appendix is generally the conduit of bulking agents and mid-urethral slings to choice but may be unavailable secondary artificial urinary sphincters and bladder neck to: appendicectomy, stenosis, short length, Figure 1. Mitrofanoff channel in situ. closure. short mesentery or congenital absence. The advantages of the Yang / Monti ileal channel Stoma formation are: availability, generally easy to mobilise, There are various techniques described for reliable vascularity, and only a small (2.5cm) to bladder) requiring revision (2- creating the stoma. It should be sited in the segment is required [3]. 10%), anastomotic leak (bowel and lower abdomen, away from any scar sites. It Mitrofanoff-bladder) (10%), incontinent is important to ensure that the appendiceal Consent channel (2-10%), false passage (0.4- mucosa is not exposed at skin level (in Consent is a dynamic process involving the 2%), difficult catheterisation (0.4-2%), contrast to the ileal conduit) as this can lead doctor, the patient and the other members stomal prolapse. to complications with bleeding during clean of the multidisciplinary team. If a patient is intermittent self-catheterisation (CISC) being considered for a Mitrofanoff procedure Operative technique and is cosmetically displeasing. Modern this should be agreed by the MDT. This techniques aim to provide a short length then begins a process of counselling and Mitrofanoff channel formation of skin conduit before anastomosing to the educating as outlined above. As part of A Pfannenstiel or lower midline incision appendix channel. Most commonly this is the consenting process the patient should is used to gain access to the bladder and achieved at the umbilicus. The umbilicus be offered all viable alternative surgical appendix / caecum although advances is detached from the rectus sheath and options. It may take several outpatient are being made in minimally invasive a flap is created. The intestinal conduit sessions to complete the consent process. approaches. The appendix is mobilised is spatulated on its posterior surface and Once the patient is consented appropriately on its mesentery and disconnected from anastomosed to the umbilical flap. Similar and judged motivated and educated the caecum (Figure 1). A 14-16Ch catheter techniques such as the tubularised skin flap sufficiently, a date for surgery can be set. is passed through the appendix channel and the VQZ flap have also been utilised if Consent is finalised on the day of surgery. to ensure it is patent and catheterisable. the stoma needs to be sited away from the As part of the discussion the following risks The reservoir end of the appendix is umbilicus [18]. should be outlined (risk percentages as per tunnelled into the bladder through a current BAUS figures) [14,15]: 3-4cm submucosal anti-reflux channel and Postoperative care anastomosed to the bladder urothelium. Postoperatively patients are left with a 12Ch • Generic complications of major The appendix may be anastomosed or 14Ch catheter in situ, often alongside a abdominal surgery – wound infection / posteriorly (studies have reported a higher urethral or suprapubic catheter, for four to dehiscence / hernia (2-10%), bleeding rate of urinary tract infections and stones six weeks until the anastomosis has healed. (requiring blood transfusion / drainage with anterior anastomosis) or anteriorly They are brought back and taught to CISC of haematoma) (2-10%), ileus, venous (which allows for a shorter channel and through the Mitrofanoff channel and once thromboembolism, anaesthetic less catheterisation difficulties) [16]. The established on this the urethral / suprapubic complications (0.4-2%). experience of the authors is that it is best catheter can be safely removed. • Complications associated with to anastomose the appendix in the position concomitant cystectomy or bladder which affords the shortest and most direct Alternative options and surgical reconstruction – anastomotic leak and straight channel. variations (2-10%), urine leak (2-10%), bowel obstruction (0.4-2%), enterocystoplasty Reservoir considerations Yang-Monti technique or neobladder rupture (2-10%), The bladder must be a low pressure One alternative to the appendix that has ureteric anastomotic stricture (0.4- reservoir for success and safety of the shown equivalent results is the Yang-Monti 2%), metabolic complications (if procedure and therefore additional procedure and can be used when the enterocystoplasty or neobladder) augmentation ileocystoplasty is appendix is absent, of insufficient length, (0.4-10%), long-term increased risk of concurrently performed in some patients. or has a poor blood supply. This procedure cancer (enterocystoplasty) (0.4-1%). Equally some patients will have undergone isolates a 2-2.5cm length of ileum which is • Complications specific to the urethrectomy and / or cystectomy for opened along its anti-mesenteric border and Mitrofanoff procedure – stenosis carcinoma or other indications and will is then re-tubularised along its longitudinal (skin level / channel / anastomosis therefore have a neobladder as the reservoir. axis in order to form a viable channel

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complications concerning the Mitrofanoff channel include difficulty with intermittent self-catheterisation, quoted at up to 20.1%, and stomal prolapse quoted at 2% [23,24]. Revision rates of the Mitrofanoff channel are reported at up to 20% for any indication [9,17]. Re-operation rates (early and late complications) total up to 32% [23]. There appears to be a benefit in terms of stomal stenosis with VQ, VQQ or VQZ plasties but the umbilicus is often favoured over these techniques due to superior cosmesis [25,26]. Comparisons between Mitrofanoff and Yang-Monti channels have yielded conflicting results. Some authors have shown no difference in the incidence of complications between appendicovesicostomy and ileovesicostomy [27,28]. Narayanaswamy et al., however found that Yang-Monti Figure 2a. Double monti harvest. channels were more likely to have difficulty catheterising (60% of Yang-Monti vs. 26% Mitrofanoff) but that this was only due to stenosis in half of all cases in the Yang-Monti cohort. The remainder often had problems with a “pouch” in the channel or simply the channel was too long. Therefore a single Yang-Monti is recommended, where possible, compared to a double or spiral Monti [20]. Figure 2b. Double monti complete. Overall complications are common and re- operation rates high. However, this procedure has been shown to be well-tolerated and durable in the long-term despite the need (Figure 2). When required this technique Long-term management and can be adapted to increase the length of outcomes for revision surgery. The disadvantages of a Mitrofanoff procedure are outweighed for the channel in a double Monti procedure General complications of the procedure many patients by the high continence rates, in which two segments of bowel are include an early re-operative rate for improvement in quality of life and cosmesis isolated, de-tubularised and then joined significant postoperative complications of the procedure. together transversely. The resulting bowel requiring laparotomy of 8% [8]. The risk of plate is then tubularised to form a longer long-term recurrent urinary tract infections conduit. A spiral Monti uses a 3.5cm and stone formation appears to be reduced Conclusion segment of isolated ileum. It is partially with posterior implantation of the appendix Any patient undergoing a Mitrofanoff or transected in the centre. Resultant channel into the reservoir. However, the Yang-Monti procedure must accept that they segments are detubularised by incisions urinary tract infection risk long-term ranges will inevitably require long-term follow-up, close to the mesentery on opposite sides. from 9.5-40% and stone formation of 40% particularly if bladder reconstruction was The flap is retubularised transversely to [16,21]. The incidence of both conditions also performed. The rate of complications produce the conduit. is obviously increased in patients with and re-operation is high and patients must be Ileum is readily available, easy to concomitant augmentation cystoplasty / counselled, in particular, regarding the risks of mobilise and produces conduits of good neobladder. stomal stenosis and difficulties catheterising diameter and length. Resultant Yang- The great attraction of this procedure the channel requiring subsequent revision Monti channels are therefore commonly is the longevity of continence with studies surgery. However, for patients with end-stage used and convey continence to over 95% reporting continence ranging from 88- incontinence, neurogenic / end-stage bladder of patients [19]. Such outcomes rival the 98% at medium to long term follow-up dysfunction or disrupted / non-functional traditional Mitrofanoff conduit. However, [9,17,22]. However, traditionally the major urethrae, the Mitrofanoff procedure has Monti stenosis rates are reported as cause for re-operation involves problems provided a continent urinary conduit which is 5-10% and revision rates two to four with catheterising the channel including well tolerated and provides a good quality of times more common in double and spiral stenosis and false passages. Stomal stenosis life for our patients. Monti channels, respectively, relative to is reported in up to 13% at two years and as Almost 40 years since he first described the traditional Mitrofanoff conduit [20]. high as 54% at five years [11,23]. However, in this procedure, the Mitrofanoff procedure Of note, a high incidence of diverticular many, this can be managed conservatively continues to show superiority to other pouches within the double Monti channel with channel dilation or simple endoscopic continent conduits with the exception, may account for difficult catheterisation procedures. Bladder level stenosis is rarer perhaps, of the Yang-Monti conduit and is and failure of the conduit [20]. A single but often requires surgical intervention likely to continue to stay as an essential ileal channel is therefore preferable to with excision of the stenosed segment, part of the armamentarium of functional, double and spiral Monti conduits where lengthening of the channel (if required) and reconstructive and paediatric urologists for possible. repeat tunnelling and re-anastomosis. Other many years to come.

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References 15. BAUS. Mitrofanoff procedure (Creation of a 1. Mitrofanoff P. [Trans-appendicular continent catheterisable urinary stoma): Information about your cystostomy in the management of the neurogenic procedure from The British Association of Urological Surgeons (BAUS). 2017. www.baus.org.uk/_userfiles/ bladder]. Chir Pediatr 1980;21(4):297-305. pages/files/Patients/Leaflets/Mitrofanoff.pdf “The Mitrofanoff procedure 2. Yang WH. Yang needle tunneling technique in Accessed 26 November 26 2017 creating antireflux and continent mechanisms.J Urol has provided a continent 16. Berkowitz J, North AC, Tripp R, et al. Mitrofanoff 1993;150(3):830-4. continent catheterizable conduits: top down or urinary conduit which is 3. Monti PR, Lara RC, Dutra MA, de Carvalho JR. New bottom up? J Pediatr Urol 2009;5(2):122-5. techniques for construction of efferent conduits 17. Franc-Guimond J, Gonzalez R. Effectiveness of well tolerated and provides based on the Mitrofanoff principle.Urology implanting catheterizable channels into intestinal 1997;49(1):112‑5. segments. J Pediatr Urol 2006;2(1):31-3. a good quality of life for our 4. Jung P, Jakse G. The choice of continence mechanism 18. Woodhouse C. Continent Urinary Diversion. In: in continent (supra)vesical urinary diversion. Urol Int Frank J, Gearhart J, Snyder H, eds. Operative Pediatric patients.” 1996;57(3):175-9. Urology. Churchill Livingstone; 2002:79-108. 5. Bihrle R, Adams MC, Foster RS. Adaptations of the 19. Wagner M, Bayne A, Daneshmand S. Application of Mitrofanoff principle in adult continent urinary the Yang-Monti channel in adult continent cutaneous reservoirs. Tech Urol 1995;1(2):94-101. urinary diversion. Urology 2008;72(4):828-31. 6. Malone PS, Ransley PG, Kiely EM. Preliminary 20. Narayanaswamy B, Wilcox DT, Cuckow PM, et al. The report: the antegrade continence enema. Lancet Yang-Monti ileovesicostomy: a problematic channel? 1990;336(8725):1217-8. BJU Int 2001;87(9):861-5. 21. Fishwick JE, Gough DC, O’Flynn KJ. The Mitrofanoff 7. Wedderburn A, Lee RS, Denny A, et al. Synchronous procedure: does it last? BJU Int 2000;85(4):496-7. bladder reconstruction and antegrade continence 22. Thomas JC, Dietrich MS, Trusler L, et al. Continent enema. J Urol 2001;165(6 Pt 2):2392-3. catheterizable channels and the timing of their 8. Gowda BDR, Agrawal V, Harrison SCW. The continent, complications. J Urol 2006;176(4 Pt 2):1816-20. AUTHORS catheterizable abdominal conduit in adult urological 23. Jacobson DL, Thomas JC, Pope J 4th, et al. Update on practice. BJU Int 2008;102(11):1688-92. Continent Catheterizable Channels and the Timing of 9. Harris CF, Cooper CS, Hutcheson JC, Snyder HM 3rd. their Complications. J Urol 2017;197(3 Pt 2):871-6. Appendicovesicostomy: the mitrofanoff procedure-a 24. Suzer O, Vates TS, Freedman AL, et al. Results of the 15-year perspective. J Urol 2000;163(6):1922-6. Mitrofanoff procedure in urinary tract reconstruction 10. Liard A, Seguier-Lipszyc E, Mathiot A, Mitrofanoff in children. Br J Urol 1997;79(2):279-82. P. The Mitrofanoff procedure: 20 years later. J Urol 25. Kajbafzadeh AM, Chubak N. Simultaneous Malone 2001;165(6 Pt 2):2394-8. antegrade continent enema and Mitrofanoff 11. Sahadevan K, Pickard RS, Neal DE, Hasan TS. Is principle using the divided appendix: report of a new continent diversion using the Mitrofanoff principle a technique for prevention of stoma complications. viable long-term option for adults requiring bladder J Urol 2001;165(6 Pt 2):2404-9. replacement? BJU Int 2008;102(2):236-40. 26. Van Savage JG, Khoury AE, McLorie GA, Churchill Rachel Barratt, BM. Outcome analysis of Mitrofanoff principle 12. Ordorica R. The continent bladder: indications and ST5 Urology Trainee, Whipps Cross Hospital, London. applications using appendix and ureter to techniques for the continent catheterizable segment. umbilical and lower quadrant stomal sites. J Urol Curr Opin Urol 2004;14(6):345-50. 1996;156(5):1794-7. 13. Woodhouse CR, Gordon EM. The Mitrofanoff principle 27. Lemelle JL, Simo AK, Schmitt M. Comparative study of for urethral failure. Br J Urol 1994;73(1):55-60. the Yang-Monti channel and appendix for continent 14. BAUS. Enlargement of the bladder with a piece of diversion in the Mitrofanoff and Malone principles. Bowel (Enterocystoplasty) : Information about your J Urol 2004;172(5 Pt 1):1907-10. procedure from The British Association of Urological 28. Castellan MA, Gosalbez R, Labbie A, et al. Outcomes of Surgeons (BAUS). 2017. www.baus.org.uk/_userfiles/ continent catheterizable stomas for urinary and fecal pages/files/Patients/Leaflets/Enterocystoplasty.pdf incontinence: comparison among different tissue Accessed 26 November 2017. options. BJU Int 2005;95(7):1053-7.

Theresa Marsden, TAKE HOME MESSAGE CT1, Core Surgical Trainee, Frimley Park Hospital.

• The Mitrofanoff procedure creates a continent appendicovesicostomy for patients with disrupted / non-functional urethrae, end-stage incontinence / detrusor overactivity or after cystectomy for benign or malignant causes.

• If a patient is having a concurrent MACE and Mitrofanoff procedure the appendix should be used for the MACE procedure and a Monti substitution for the bladder conduit.

• The Yang-Monti procedure is a suitable variation for patients with an absent or non-utilisable appendix but may have slightly higher complication rates. Tamsin Greenwell,

• Patients must be closely counselled about the risks of channel complications and Consultant Urologist, University College London Hospital. the need for future revision surgery. E: [email protected]

• Continence rates are excellent and despite the high revision rates the procedure is

well-accepted by patients Declaration of competing interests: None declared.

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