Spinal injury caused in children mostly by myelomeningocele results in urinary bladder and anal canal dysfunction (1). The lack of sensation and lower urinary tract control can lead to upper urinary tract damages and consequently to renal failure. Therefore the prevention of the upper urinary tract deterioration is the most important aim of the management of neurogenic bladder. Urinary and fecal continence as well as adequate voluntary bladder and bowel emptying are equally important for school and kindergarten age children. These aims can be achieved in the majority of children by means of intermittent catheterization and the use of anticholinergic medications (1-3). This treatment should be introduced immediately after birth, especially in children with high-pressure bladder and detrusor-sphincter dyssynergia, because renal damage starts often within the first half year of life (2, 4). The anticholinergic drug used since the infant age is oxybutinine chloride in the dosage of 0.3-0.4 mg/kg body weight, divided over 3-4 doses per day (4, 5). Besides that, the children are put on prophylaxis of urinary tract infections, mostly furaginum 1-2 mg/kg body weight per day. Intermittent catheterization, oxybutinine and prophylaxis of infections since early infancy allow obtaining normal bladder capacity, its compliance and consequently preventing progressive renal damage. Moreover, this approach has significantly reduced the need for bladder augmentation in these children (2). Small children are catheterized by their parents and other caregivers. Since about 6 years of life, sufficiently intellectually and manually dexterous children are taught to catheterize themselves. A creation of the continent appendico-vesicostoma (Mitrofanoff procedure) is an optional solution in children in whom self-catheterization through their native urethra is difficult e.g. in girls with severe deformations of lower limbs (2). Malone antegrade continence enema through the appendicular stoma is an efficient management option for children with severe constipations and bowel incontinence due to neurogenic dysfunction of anal canal (6).

The aim of the paper is the assessment of the appendicular stomas in treatment of children with neurogenic dysfunction of bladder and anal canal.

The Mitrofanoff stoma is constructed utilizing the appendix which is detached from the cecum but its vascularization is preserved. Distal end of the appendix is sutured through the submucosal tunel into the bladder. The other end of the appendix is connected to the umbilicus or to the skin in the right lower quadrant. Till 2009 we created a V-shape skin flap which was anastomosed into an incised proximal part of the appendix. Since 2010 we have used VQZ plasty for the connection of the appendix into the skin. In this technique, besides a V-flap, a parallel quadrilateral flap is created from the skin. This flap is inverted and anastomosed to the edge of the appendix and to the V-flap. With this technique the stoma is more aesthetic and its mucosa is hidden in the skin tunnel. Currently we keep catheter in the stoma for 6 weeks after the procedure. After this period, the stoma is catheterized every 3 hours.

We create the Malone stoma using the appendix which is not detached from the cecum. We do a window in the mesentery of the base of the appendix. Then the cecum is wrapped around the base of the appendix to form the antireflux mechanism. The distal end of the appendix, incised along its anti-mesenteric border, is connected to the V-shape skin flap or with VQZ technique. We keep catheter in the stoma also for 6 weeks after this procedure. The antegrade enemas are done through the catheter left in the stoma beginning from the second day after the operation. After 6 weeks the stoma is catheterized once a day for performing enema.

In years 2002-2012 in our institution catheterizable appendicular stomas were constructed in 34 children. In this number 23 children had neurogenic dysfunction of urinary bladder and anal canal. The indications for Mitrofanoff procedure in the other children were: bladder extrophy syndrome (8 children), prune belly syndrome (1 child), persistent cloaca (1 child), isolated urogenital sinus (1 child) and urethral atresia (1 child). The causes of neurogenic dysfunctions of the bladder and the anal canal in 23 operated children were: meningomyelocele (21 children), anal atresia and sacral agenesis (1 child), Currarino triad (1 child). The age of the operated children was 11-18 years. 19 Mitrofanoff stomas and 7 Malone stomas were constructed (tab. 1). There were three children in whom the appendix was divided and both Mitrofanoff and Malone stomas were done. Different techniques of bladder augmentations were performed simultaneously with catheterizable stoma in 8 children; autoaugmentation (detrusorectomy) was done in four children and augmentation of the bladder by ileocystoplasty or colocystoplasty was performed in four children. The catheter was kept in the stoma for 2 weeks in early patients and since 2010 – for 6 weeks. The catheter left in the bladder after autoaugmentation was clamped for 2-3 hours and after these periods the bladder was emptied. The aim of this approach is the prevention of bladder collapse and scar formation in the place of detrusorectomy.

In assessing the long-term results of the treatment, the cosmetic effect, as well as the complications, which have a serious impact on the functioning of the stoma were taken into consideration (tab. 2).

An injury and complete obstruction of the appendicular stoma making its catheterization impossible, was observed in three children operated on before 2010. These children are still catheterized through the urethrae. The urine leakage through the stoma at the maximal bladder filling is observed in one child. The Malone stoma stenosis requiring reoperation was found in two children. Currently the stomas in these children are easily catheterized. An unaesthetic eversion of the mucosa of the appendix is observed in 6 children. Five of them were operated before the introduction of the VQZ technique. Since 2010 this type of complication has been found only in one child.

The introduction of the intermittent catheterization (7) in the 70’s of the 20th century was a major breakthrough in the treatment of the neurogenic bladder dysfunction. Catheterizing children by guardians, mainly parents, is a relatively simple procedure. Also, children who are intellectually and manually dexterous are able to catheterize themselves. However, for some children self-catheterization can be a big inconvenience, especially for girls in wheelchairs or boys with narrowed urethrae, resulting from long-term catheterization. For such patients, the best solution is to create an alternative, easier way to catheter the bladder by using the method suggested in 1980 by Paul Mitrofanoff (8). In 1990 Padraig Malone inspired by the idea of Mitrofanoff, described a method of creating appendicular stoma in order to perform antegrade continence enema (9). In both types of surgery the appendix is used to create a continent catheterizable stoma. Sometimes, however, the child does not have an appendix or the appendix is too short. In such cases, the most common solution is an application of the Monti method involving the use of a detubularized and transversely re-tubularized 2-cm-long ileal segment (8). The length of a tube created in this way may be increased by implementing a slightly longer fragment of the intestine and retubularizing it along the diagonal flap (10). In cases where there is a need to produce two stomas: for bladder catheterization and antegrade continence enema, the appendix can be divided (11). A Mitrofanoff stoma is constructed using distal part of the appendix and the proximal part of the appendix is used for Malone stoma. An essential condition is the suitable length of the appendix, which must be no less than 9 cm. In our center we have simultaneously performed Mitrofanoff and Malone stomas on three children. According to data from the literature the risk of complications after the appendicular stoma contruction is 19-28% (12, 13). Apart from the unaesthetic appearance of the stoma, serious complications in our group occurred in 6 children (26%). The most serious complication in our patients was an injury of the stoma and consequently its occlusion. These complications occurred in obese children operated on before 2010, when the catheter was kept in the stoma relatively short after the operation. Since the catheter is kept in the stoma for 6 weeks, no such complication has been observed.

We connected Mitrofanoff stoma to the skin at the umbilicus or at the right lower quadrant whereas Malone stoma was always constructed at the right lower abdomen. The location of the stoma is decided based on the anatomical conditions and the preferences of the patient. The location of the stoma has no influence on the complication rate (14, 15). When the lower right abdomen was chosen, the proximal part of the appendix was incised lengthwise and it was anastomosed to the V flap of the skin. The purpose of this was decreasing the risk of narrowing the stoma. Nonetheless the stoma frequently took on the form of an unaesthetic, everted mucosa. In the year 2010 we introduced a new tactic of stoma construction. We leave the catheter inside the stoma for a period of 6 weeks, and we connect the appendix with the skin utilizing VQZ technique (7, 13). As a result the number of complications has decreased and the stoma is more aesthetic (fig. 1). Both all children who are earnestly and regularly catheterizing themselves via the Mitrofanoff stoma and/or performing enemas via Malone stoma and their parents emphasize an improvement in the quality of their life. Data from literature confirm our observations (16). The children feel more independent while the parents gain a bit more free time in their already task filled lives.

The construction of the appendicular stoma both for intermittent catheterization of the bladder and for antegrade continence enema in children with a neurogenic dysfunction of the bladder and the anal canal is a surgical procedure worth of recommendation. Children efficiently catheterize themselves through the stoma, in this way decreasing the amount of their guardians’ duties, while teenagers become increasingly happy as a result of their growing independence.