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34 Continent Catheterizable Channels

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Contents in both children and adults, such as ARM, spina bifida, sacral agenesis, exstrophy complex, postradia- 34.1 Introduction . . . 427 tion cystitis, complex vesicovaginal fistulae, posterior 34.2 Indications . . . 427 urethral valves, prune-belly syndrome, cerebral palsy, 34.3 The Mitrofanoff Procedure . . . 428 sacrococcygeal teratoma, spinal-cord injury, perineal 34.3.1 Technical Details . . . 428 trauma, Hirschsprung’s disease, intractable consti- 34.4 The MACE Procedure . . . 430 pation, and different types of urethral failures, or in 34.4.1 Technical Details . . . 431 patients with perineal colostomy after rectal resec- 34.5 The Stoma Technique and Associated Problems . . . 432 tion [6–19]. In the original description of Mitrofanoff 34.6 Postoperative Management . . . 434 and antegrade colonic enema (ACE) procedures, the 34.7 Results . . . 435 conduit of choice is the appendix. In conditions in References . . . 435 which the appendix is not available, tubular struc- tures such as the ureter, fallopian tube, Meckel’s diverticulum, and the vas deferens, or tubes created from the ileum, colon, stomach, bladder, rectus 34.1 Introduction abdominis muscle, and prepuce can be used to create a Mitrofanoff channel. Ileal tubes are most commonly The concept of clean intermittent catheterization and preferred if the appendix is not available. The use of its efficacy in emptying urinary reservoirs was first in- the other organs except the ureters and bladder tubes troduced by Lapides in 1972 and is accepted as the first are either sporadic or experimental (Table 34.1) [20– step in the use of continent catheterizable channels 37]. (CCC). Subsequent important progress in CCC in- In ARM, the ACE procedure is used with two in- cludes performance of the first continent cystostomy dications: the treatment of fecal incontinence and/or by Mitrofanoff in the 1980s, acknowledgment of low- the treatment of postoperative intractable consti- pressure reservoirs from the beginning of the 1980s, pation and developing megarectum/megacolon in recognition of the importance and efficacy of colonic which conservative management frequently fails [38]. washouts in the management of fecal continence, and The presence of sacrospinal anomalies in ARM can the first continent cecostomy performed by Malone also lead to both urinary and fecal incontinence [39– in 1990 [1–4]. CCC are used in anorectal malforma- 42]. In this group of patients, the Mitrofanoff prin- tions (ARM) for problems involving the gastrointes- ciple is used to provide urinary continence. In some tinal and/or urinary systems. The aim is to overcome fecal and urinary incontinence concomitantly. These circumstances also lead to competition for the appen- dix, since this has been the favored channel for both Table 34.1 Organs used in the construction of continent chan- the Malone’s antegrade colonic enema (MACE) and nels using the Mitrofanoff principle Mitrofanoff procedures. These limitations have lead Tubular organs Tubularized organs to the creation of new techniques for these channels Appendix Ileum [5]. Ureter Colon Fallopian tubes Stomach 34.2 Indications Bladder Prepuce The Mitrofanoff and MACE procedures are widely Rectus abdominis muscle used alone or in combination for different problems 428 Yunus Söylet

patients with combined incontinence, the ACE and dix is anastomosed to the bladder with an antirefl ux Mitrofanoff procedures are used together. technique, there is no leak from the abdominal wall orifi ce, thus it is a continent vesicostomy. In this pro- cedure, a catheter is advanced through a conduit to 34.3 The Mitrofanoff Procedure empty the urinary reservoir in a clean and intermit- tent fashion. Th e Mitrofanoff procedure is also known as continent appendicovesicostomy according to the Mitrofanoff principle. Th e two indications for continent vesicos- 34.3.1 Technical Details tomy in ARM are the same as those for incontinent intestinal conduits: management of urinary inconti- In the majority of cases a Mitrofanoff channel is con- nence and preservation of renal function. ARM pa- structed through a midline infraumbilical incision. tients usually have neurogenic bladders secondary to If the patient will only have a continent vesicostomy sacrospinal anomalies. In a small group of patients constructed, the right laterovesical space is deeply that have urethral injury secondary to their ARM re- freed before opening the peritoneum. Th e cecum construction, the Mitrofanoff procedure may be help- and appendix are explored and the pedicle vessels ful in the long-lasting management of urethral stric- of the appendix are carefully mobilized. Th e appen- ture. Th is is the third indication for the Mitrofanoff dix is excised from the cecum with a cecal wall cuff . procedure, and has been reported in the management Th e aim in leaving the cecal cuff on the appendix is of urethral problems [43]. Th e Mitrofanoff procedure to enable a wide anastomosis on the abdominal wall. as the pioneer of CCC has helped to defi ne the pri- Once the cecal defect is closed, the tip of the appen- mary principles of these channels. Since the appen- dix is opened obliquely, the lumen is irrigated with

Fig. 34.1 (A–D) Submucous tunnel technique into the U-fl ap of thick-walled bladder for creating a Mitrofanoff channel 34 Continent Catheterizable Channels 429 an antiseptic solution, and the patency of the lumen In the majority of patients, augmentation cysto- is checked with a catheter. If the bladder has a small plasty is a part of this surgery. Augmentation pro- capacity and thick wall (as it has most of the time), vides a low-pressure reservoir, while the Mitrofanoff a U-shaped fl ap should be raised cranially for en- channel aids in emptying the reservoir regularly. Th e trance. With the help of this fl ap, the appendix can bladder neck should be constructed or closed so that be anastomosed to the bladder through a long tun- it does not leak. It has been shown in many cases that nel (Fig. 34.1). Th e tunnel should at least be 2.5–3 cm even though vesicoureteric refl ux may exist in the long. Once the conduit–bladder anastomosis is com- system, the creation of a low-pressure reservoir and pleted, if no further procedures such as bladder aug- guaranteeing regular emptying of the system will lead mentation, bladder-neck reconstruction, or ureteric to disappearance of the refl ux. Th us, in many patients reimplantation are necessary, the bladder is closed with small bladders and limited space for a ureteric and the conduit is anastomosed either to the right reimplantation, the reimplantation should not be car- lower quadrant or to the umbilicus. In the literature, ried out and morbidity is avoided [45]. two cases have been reported to have their appendix In patients with urethral injuries as a result of located into the left lower quadrant due to previous ARM repair, if a long and complicated management stomas created in the right lower quadrant. In these is necessary, or if autoaugmentation is indicated for cases the cecum and the ascending colon were widely a diff erent underlying pathology, it is appropriate to mobilized, the appendiceal vessels were dissected up construct an appendicovesicostomy without opening to their origin from the superior mesenteric artery, the bladder. In the extravesical Mitrofanoff technique and the conduit was moved to the left through a de- that we use in our patients, the abdomen is entered fect created in the bowel mesentery and anastomosed through a lower-abdominal midline incision, the to the left abdominal wall [44]. right laterovesical space is opened, and a Lich-Gre-

Fig. 34.2 (A–D) Extravesical Mitrofanoff technique 430 Yunus Söylet

goire-like extramucosal tunnel is created in the right Minimally invasive techniques, although not posterolateral wall of the bladder. The mucosa is en- widely popularized, have been used in the construc- tered close to the bladder neck and an appendicovesi- tion of continent urinary channel surgery. The first costomy performed. The seromuscular layer is closed laparoscopic appendiceal–vesical anastomosis with a with sutures, the tunnel is completed, and the appen- flap-valve mechanism has been reported by Hsu and dix is anastomosed to the skin (Fig. 34.2). In cases Shortliffe [57]. There is also a case report on laparo- where the appendix is not available or has been used scopic removal of a nonfuctioning kidney and anas- for the MACE procedure, the Mitrofanoff channel is tomosis of the distal ureter to the skin as a continent most commonly created using the Monti technique channel in a patient who had previously undergone [46]. Although this technique was described by Monti a ureteric reimplantation due to vesicoureteric reflux in 1997 [47], it was also described by Yang in 1993 [58]. [48]. When Yang reported this technique, however, he focused on the use of a needle as an aid for reimplants into the small bowel submucosa. In the Monti tech- 34.4 The MACE Procedure nique a 2.5-cm segment of the small bowel is detubu- larized longitudinally and retubularized transversally. Bowel management is necessary in the majority of A 2.5-cm segment of bowel provides a tube size of ARM in the postoperative period. Fecal incontinence about 18–20 Fr. The length of the Monti tube is de- in some patients and intractable constipation in termined by the diameter of the bowel segment. The others are the main problems. Initially, conservative length will be at least 8 cm when the ileum is used measures including aggressive potty training, dietary and 10–12 cm when the colon is used. The transverse management, different medications, daily retrograde tubularization should be carried out first with muco- enemas, and biofeedback therapy in suitable patients sal apposition and then with a second row of serosal are used to overcome these complications. Peña has sutures [48]. Problems such as kinking, diverticula reported a detailed bowel management program for formation, and catheterization problems observed ARM patients [59]. Reoperations for some patients after longitudinal tubularization of ileal segments can involve reconstructive surgical procedures such are rarely reported in Monti tubes [49]. Stomach and as gracilisplasty or, rarely, permanent stoma. Con- colonic tubes can be used for the same purpose [50]. servative measures should be initially undertaken in The common pedicled bowel segment can be used in all patients. Patients that have success with daily ret- patients in whom bladder augmentation needs to be rograde enemas are good candidates for the MACE combined with an ileal Monti. procedure. The most common cause of failure in Two Monti tubes can be anastomosed to each other the long term in patients who have success with or elongated in a spiral fashion, similar to the con- daily enemas is the dislike they develop for enemas struction of longer channels, creating a double Monti administered through the anus as they grow older. [51]. In addition, in both the Mitrofanoff and MACE Many patients, especially teenagers, find this method procedures, longer tubes may be created using combi- tedious and bothersome. In this group of patients nations of the appendix and Monti tube or appendix the antegrade colonic enema technique described by and cecal tube [52,53]. Rink created a continent cath- Malone in 1990 should be considered. The advantages eterizable vesicostomy by modifying Casale’s vesicos- are easy access to the colon compared to retrograde tomy with an antireflux technique. Stomal continence irrigations, on-way effective irrigation, smaller vol- was achieved in all patients with this technique, but umes for irrigation, psychological comfort, and 45% have required revision due to stomal steno- combined management of urinary incontinence. Suit- sis. Thus, the use of the technique has been limited able anatomy, which means sufficient length of colon to large bladders requiring continent vesicostomies and absence of distal stenosis, motivation of patients [54]. In a patient with microcolon-intestinal hypo- and caregivers, trial of all conservative measures peristalsis syndrome, a bladder tube created from the beforehand, technical success with rectal irrigations, posterior wall of a huge, adynamic bladder has been and location by hand of the stoma site by patients, used with success and no morbidity [55]. A salvage manipulation are factors influencing the success with continent vesicostomy has been created in five chil- the MACE procedure. Rigorous teaching and moti- dren with enterocystoplasties and no appendix, using vation before the procedure is mandatory [60–63]. a bladder tube created completely extraperitoneally, Antegrade enema through a continent cecostomy is and plicated with the bladder at its base in a Nissen- not a cure for intestinal problems with ARM, rather like fashion to add an antireflux property [56]. it is a more pleasant way for children to engage in a 34 Continent Catheterizable Channels 431

Table 34.2 Continent cecostomy techniques. MACE Malone’s the appendix is not available or has been previously antegrade colonic enema, LACE Left colonic antegrade colonic used as a Mitrofanoff channel, ileal, colonic, or gastric enema Monti channels can be used to create an ACE. Th e ce- Disconnection and reimplantation of the appendix cum, transverse colon, left colon, and stomach tubes Orthotopic appendicostomy (+/- divided appendix) have been used in ACE construction. Th e left colon antegrade continence enema (LACE) procedure has Tubularized cecal/colonic fl ap been reported to have advantages by providing grav- Transverse tubularized ileal tube (Monti) ity-assisted evacuation, avoidance of the right and Laparoscopic MACE (Appendicostomy only) transverse colon, which has a large-volume capacity, a Cecostomy button convenient stoma location in the left upper quadrant, shortening of the enema duration, a smaller fl uid vol- Percutaneous cecostomy catheter ume, and no ACE related abdominal pain. In the left LACE colon, laterally or medially based tubes are used with either in situ fl ap-valve mechanisms or placement of a Monti tube in a submucosal tunnel along the colon taenia to form an antirefl ux mechanism [64–69]. Th e bowel management protocol without the need for indications to perform a right or left colonic Monti- rectal enemas [8]. MACE procedure are: previous appendectomy, pre- vious appendicovesicostomy, inadequate mesentery associated with the appendix, previous ileocecal aug- 34.4.1 Technical Details mentation, and obliterated appendicocecostomy [70]. Other modifi cations are based on similar indications. In the classical MACE technique, following eff ective In the MACE procedure, diff erent antirefl ux tech- bowel preparation and under the protection of broad- niques are used. In cecal imbrication (cecal wrap tech- spectrum antibiotics, the appendix pedicle is mobi- nique) the appendix is not detached from its base. An lized, the blind tip is opened, the appendix is detached antirefl ux mechanism is developed in a similar fash- from the cecum, inverted, and is anastomosed to the ion to Nissen’s fundoplication around the in situ ap- cecal mucosa aft er being passed through a submuco- pendix (Fig. 34.3) [71]. In cases in which the ileocecal sal tunnel formed on the taenia. Th e seromuscular pouch reservoir has been created as a continent uri- layer is closed on top to form an antirefl ux mecha- nary diversion, a technique involving the reinforce- nism. Th e procedure is completed following cutane- ment of the imbrication of the in situ appendix with ous anastomosis. It is essential to suture the cecum a mesh has been reported [72]. In the extramucosal or colon to the back of the anterior abdominal wall seromuscular taenial tunnel technique, analogous to and not to leave a free intraperitoneal part of the con- the Lich-Gregoire technique, the seromuscular layer duit [4]. Multiple modifi cations of this original tech- of the taenia is incised without opening the mu- nique have been reported many times (Table 34.2). If cosa. On the distal part of the incision, the mucosa

Fig. 34.3 A, B Cecal imbrication around the in situ appendix 432 Yunus Söylet

Fig. 34.4 (A–D) Malone’s antegrade colonic enema (MACE) – extramucosal seromuscular taenial tunnel technique

is opened and the conduit is anastomosed to the ce- et al. have reported no stomal leakage in 16 laparo- cum. Th e antirefl ux mechanism is created by covering scopically performed procedures. Th eir statement is the conduit with the seromuscular layer (Fig. 34.4). that the continence mechanism is simply a function Th e application of the Monti channel to the ileocecal of the length of the appendix and the mucosal coapta- pouch involves similar technical aspects [73]. tion of the appendiceal lumen [75]. In the majority of Th e intraluminal reimplantation technique in- cases that develop leakage, a surgical revision is nec- volves the creation of a submucosal tunnel, similar essary. In our series, a mild leakage was managed by to Cohen’s reimplantation technique, and placement submucosal injection, and in Mitrofanoff ’s series an of the conduit into this tunnel. Th is technique is pre- artifi cial urinary sphincter application was benefi cial ferred in cases where bladder substitutes are con- in one case [76]. structed and the conduit needs to be anastomosed to the bowel (Fig. 34.5). Th e construction of a direct appendicostomy without supportive antirefl ux tech- 34.5 The Stoma Technique niques have been reported mostly by authors using and Associated Problems laparoscopic techniques, who state that it reduces the risk of conduit incontinence. On the contrary, Th e most common complication in CCC construction Malone has reported stomal incontinence for gas is the stomal orifi ce at the anterior abdominal wall. and feces in all six cases in whom he performed a di- Th e location of the stoma is related to the surgeon’s rect appendicostomy [62]. Lynch et al. report a 6.7% choice as well as the type of conduit used. In our se- leakage rate in 28 patients with laparoscopically per- ries, the fallopian tube and bladder stump were used formed continent cecostomy and state that this is not as channels in two cases and were located as perineal a higher rate compared to those who have an addi- stomas; this has provided technical applicability [77]. tional antirefl ux procedure [74]. Similarly, van Savage Duckett has also reported the use of perineal stomas 34 Continent Catheterizable Channels 433

Fig. 34.5 (A–H) Reimplantation of Monti-MACE to the large bowel 434 Yunus Söylet

in three cases that had ureter and ileal conduit con- rare in our experience. In most cases, granulations struction [78]. can be managed with silver nitrate and prolapse with Another group reports no difference in compli- surgical revision. cations between umbilical and right lower quadrant Cecostomy tubes may be placed by either a percu- abdominal stomas and prefers umbilical stomas due taneous or an open approach, providing a comparable to better cosmesis, limiting lower quadrant stomas to outcome to that of the original Malone procedure. ureteric conduits [79]. The right and left lower quad- This technique may be preferred not only in cases rants and umbilicus are preferred stomal sites. There with a missing appendix, but also in those for whom is no significant difference in complications between the appendix needs to be reserved for urinary recon- umbilical and extraumbilical sites. On the other hand struction, or to avoid a laparotomy in cases who do the umbilical stomas have superior cosmetic results not require simultaneous reconstruction. [80–82]. The disadvantage is the presence of a permanent Skin anastomosis techniques can vary. Multiple catheter or button on the anterior abdominal wall. flap techniques aiming to avoid stenosis are fre- Shandling published his initial percutaneous cecos- quently used. Mitrofanoff suggests maintaining an tomy tube experience [98], only to notice later the exteriorized small cuff of cecal mucosa to overcome difficulty of carrying a tube attached to the body the stomal stenosis complication reported as the most and balloon tube complications. Thus, he suggested common problem (5–45%) [76]. V and Y type skin the insertion of a plastic trapdoor cecostomy tube plasties [83,84], VQ, WQQ, and VQZ techniques and 6–8 weeks after the procedure, which was better tol- similar modifications all aim to create a more cos- erated by patients. He reported no complications metic and functional stoma [85]. in his first eight-case series using plastic trapdoor Among the two most common CCC, the Yang- cecostomy tubes [99]. Chait et al., in a series of 163 Monti channels and appendiceal channels are most percutaneous cecostomy tubes, reported minor com- frequently compared. Narayanaswamy has reported plications in 60% of cases and mentioned that they 60% catheterization difficulty and 28% pouch-like were easily managed with conservative measures. The dilatation in Monti tubes in his series [86]. Studies failure rate was 10%, and 90% of patients reported an involving similar comparisons among two conduits improved quality of life [100]. report no difference in the results. Obesity increases A different technique involving colonoscopy assis- complication rates, as in any surgical procedure tance for the insertion of a percutaneous cecostomy [87–90]. Bladder tube complication rates are around tube using the principles of percutaneous endoscopic 30–40% and are higher in comparison to appendiceal gastrostomy (PEG) has been reported [101]. In the and Monti channels [63,91]. five-case cecostomy button series of Duel and Gon- Laterally based colonic conduits appear to have a zales, three cases had localized infection and one pa- higher rate of stomal stenosis compared to medially tient required intravenous antibiotic administration based colonic tubes (40% versus 12%), which may be due to cellulitis [102]. attributed to the local blood supply properties of the colon [92]. MACE channels are more prone to stenosis com- 34.6 Postoperative Management pared to Mitrofanoff channels. The most possible cause may be less frequent catheterization. To over- Catheters are left in situ for 2–3 weeks in the post- come this problem, simultaneous catheterization of operative period of urinary and intestinal continent the Mitrofanoff and continent cecostomy channel is channels. Catheterization is initiated after this period. recommended [77]. Prevention of stomal stenosis can If the patient has a simultaneous augmentation cys- be overcome by skin plasties as well as prevention of toplasty, the reservoirs are continuously drained at catheter trauma. Snodgrass suggests parastomal Tri- nighttime for 2–3 months. Twenty-four-hour urinary amcinolone injection to prevent stenosis in Mitro- output, and day and night urine volumes help to iden- fanoff stomas [93]. tify the frequency of catheterization. Appendicitis can lead to channel obliteration and Colonic enemas can start at between 1 and 30 days loss of appendiceal channels [94,95]. Perforations postoperatively. Colonic enemas may involve tap wa- have been reported in gastric and bladder tubes ter, isotonic saline, salty water, phosphate, polyethyl- [96,97]. Mucosal prolapse and granulation of the tis- ene glycol electrolyte, glycerine, mannitol, lactulose, sue around the skin opening leading to a continuous or mineral oil, either alone or in combination. The bloody discharge and staining of underwear are not volume of enemas will depend on the composition 34 Continent Catheterizable Channels 435 of the material used and other personal factors, and functional after 20 years and have stated that the tech- will vary between 30 and 1000 ml. The evacuation nique has long-lasting efficiency. They have found period may last between 10 and 120 min. Enema no catheterization problems during pregnancy and frequency may also vary from once a day to once a have reported successful delivery by cesarean sec- week. Dietary management, including medications tion 16 years after the Mitrofanoff procedure. Patients that slow intestinal motility, is necessary as adjuvant requiring transplantation have not encountered any therapy in almost all patients. If fecal incontinence catheterization or reservoir problems during or af- continues despite conservative measures, the volume ter surgery. Late stomal stenosis has been attributed of enema should be increased, and the frequency to the cessation of urine output in patients with renal decreased. Anal stenosis should be considered in insufficiency [76]. The first normal, complication- cases with frequent but small-volume incontinence. free vaginal delivery following MACE procedure was Colicky abdominal pain is initially noted in 50% of reported by Wren et al. [107]. An 8- to 10-year fol- cases, but this resolves spontaneously within a couple low-up study of nine cases has reported intact chan- of months. Antispasmodic drugs have been found to nels in all and has estimated approximately 20,000 be effective in these cases [103]. Some patients have catheterizations without any problems. All patients reported spontaneous initiation of colonic motility except one (who was experiencing adolescent prob- simply with catheter insertion and no washouts [84]. lems) reported that they were happy with their qual- Concentrated enemas leading to colonic evacuation ity of life [108]. In another study reporting a 15-year through bowel irritation may lead to hyperphospha- experience of 50 patients, 96% continue catheteriza- temia, while high-volume nonirritable tap water and tion, 10% have developed stomal stenosis, and 16% salt water enemas may cause fatal metabolic and elec- have required surgical revision due to stomal leakage; trolyte imbalances [62,104,105]. continence was achieved in 98% [109]. Simultaneous combined Mitrofanoff and ACE procedures are re- ported to provide 80% dual continence [110,111]. 34.7 Results The main role of these long-term patent chan- nels is to improve the quality of life for patients. Al- There are two important scoring systems for MACE though some patients find CCC difficult, they report procedures. The first belongs to the Southampton an increase in self-esteem, happiness, and social ac- group and the criteria are as follows: (1) complete ceptance. Great support should be given to increase success, where the patient is clean or has minor leak- patient motivation, and patient groups should be or- age with only irrigations, and no further measures are ganized to meet together at least once a year to pre- required; (2) partial success, where there is occasional vent those patients who find the procedure lengthy leakage from the stoma or anus, and further measures and painful from abandoning their channels, despite are needed, but both child and family are satisfied; the advantages [77,112–114]. (3) unsuccessful, where there is continuous serious leakage. According to these criteria, a success rate of 62–98% is reported in the literature. A success rate of References 89% in ARM drops to a level of 62% in intractable constipation. A second group of unsuccessful results 1. Lapides J, Diokno AC, Silber SJ, Lowe BS (1972) Clean in- have been reported in children younger than 5 years termittent self catheterisation in the treatment of urinary of age. These facts stress the importance of patient tract disease. J Urol 107:458–461 education and motivation [103]. 2. Mitrofanoff P (1980) Cystostomie continente trans-ap- Another scoring system has been suggested by the pendiculaire dans le traitement des vessies neurologiques. Melbourne group in patients with slow-transit con- Chir Pediatr 21:297–305 stipation, wherein the scoring criteria are: the soiling 3. Shandling B, Gilmour RF (1987) The enema continence score, pain frequency, pain severity, appetite score, catheter in spina bifida: successful bowel management. J and mood score prior to and after the MACE pro- Pediatr Surg 22:271–273 cedure. Evaluation also involves diagnostic tests and 4. Malone PS, Ransley PG, Kiely EM (1990) Prelimi- nary report: the antegrade continence enema. Lancet clinical findings [106]. 336:1217–1218 In a study evaluating their 20-year, long-term ex- 5. Cain MP (1999) Urologic reconstruction for total confi- perience, Mitrofanoff’s team does seem to have found dence – the MACE procedure and newer options for cre- an answer for many important questions. They have ating a Mitrofanoff channel. Dialogues Pediatr Urol 22:1 established that all continent cystostomies remain 436 Yunus Söylet

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