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. urology news | JANUARY/FEBRUARY 2018 | VOL 22 NO 2 | www.urologynews.uk.com FEATURE 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