Purpose: We report long-term results in 11 patients born with bladder exstrophy who under-went lower urinary tract reconstruction using a ceco-appendiceal unit.
Materials and Methods: Four boys and 2 girls underwent lower urinary tract reconstruction using an unaltered ceco-appendiceal unit. In 2 boys and 1 girl the bladder was replaced with a reservoir of terminal ileum, cecum and ascending colon, and the appendix was used as an orthotopic neourethra. In another 2 boys and 1 girl the bladder was augmented, while the appendix was used to create a stoma to the umbilicus. In 5 patients in whom the ceco-appendiceal junction was incompetent the cecum was plicated over the base of the appendix to reinforce the sphincteric mechanism. Four patients underwent augmentation with the appendix brought out as an umbilical stoma, and in 1 the bladder was replaced and the appendix was used as a neourethra.
Results: Six patients in whom the ceco-appendiceal junction was unaltered and 5 in whom it was plicated remain continent 5 to 11 and 2 to 7 years postoperatively, respectively. In the initial patient urinary incontinence developed due to high intraluminal pressure, which resolved after detubularization of the urinary reservoir. Another patient underwent revision of the abdominal stoma.
Conclusions: The ceco-appendiceal unit may be used for continent lower urinary tract reconstruction. Ceco-appendiceal junction competence can be tested intraoperatively and the sphincteric mechanism may be reinforced as necessary. The appendix may be ectopically or orthotopically placed and used for intermittent catheterization.
KEY WORDS: bladder; bladder exstrophy; appendix; urinary reservoirs, continent
Continent reservoirs provide much improved quality of life for pediatric patients who have long life expectancy. Bladder reconstruction providing a catheterizable abdominal wall stoma has certain advantages over native urethra. For the wheelchair bound female patient with a neurogenic bladder intermittent catheterization through the native urethra can be a cumbersome procedure. For male patients with bladder exstrophy the reconstructed urethra may be difficult to catheterize due to anatomical irregularity and discomfort from intact urethral sensation. In 1980 Mitrofanoff described appendicovesicostomy in which the appendix is implanted into the bladder through a submucosal tunnel and brought to the skin as a catheterizable stoma.1 Since then, use of the appendix as a catheterizable stoma has become routine with many variations described.
In 1986 Grunberger et al reported the first case of bladder and urethral replacement using an intact ceco-appendiceal unit.2 Continence was maintained by the intact ceco-appendiceal junction. Also, this case was the first in which in situ appendix was used to create an orthotopic neourethra. We report follow-up for that case and results of 10 others in which lower urinary tract reconstruction was performed using a ceco-appendiceal unit. Four of these 11 patients had an orthotopic neourethra. Long-term results of this technique are reviewed.
PATIENTS AND METHODS
Between 1985 and 1994, 11 patients 5 to 19 years old underwent lower urinary tract reconstruction using a ceco-appendiceal unit. The appendix remains in situ on the cecum and it is used as a conduit for intermittent catheterization. Seven patients underwent augmentation cecocystoplasty with creation of an appendiceal umbilical stoma. In 4 patients the bladder was unsuitable for augmentation and it was replaced with right colon, and the appendix was used as an orthotopic neourethra. In 6 patients an unaltered ceco-appendiceal junction provided continence, while in 5 it was plicated to reinforce the sphincteric mechanism.
At operation a urinary reservoir is created from terminal ileum, cecum and ascending colon based on the right and ileocolic vessels. The bowel is opened on the antimesenteric border and reconfigured as a U-shaped reservoir using a running 3-zero polyglycolic acid suture. This pouch is then anastomosed to the native bladder or, when native bladder is not used, the ureters are reimplanted in the cecal wall and the pouch is closed. The appendix remains unaltered on the cecum and it is positioned in such a way that it may be used as an umbilical stoma or orthotopic neourethra, depending on the type of reconstruction.
Intraoperatively the ceco-appendiceal junction is assessed for competence. A cystotomy tube is placed in the cecal part of the bladder and the tip of the appendix is then excised. The bladder is filled with normal saline through the cystotomy tube (fig. 1, A). Saline is infused via gravity at 60 cm. above bladder level to ensure watertight anastomosis and test the competence of the ceco-appendiceal sphincter. If saline leaks from the tip of the appendix, the cecum is plicated over the base of the appendix to reinforce the sphincteric mechanism (fig. 1, B). Appendiceal cecoplication is performed by passing a 3-zero silk suture medially through the seromuscular layer of the cecum, through the serosa of the appendix, and then again laterally through the seromuscular layer of the cecum, after which the suture is tied.3 Four plicating sutures are placed, burying the base of the appendix in the cecum.
Case 1. A 5-year-old boy born with classic bladder exstrophy was referred following multiple procedures elsewhere to close the bladder. At presentation he was incontinent of urine and had a short, broad phallus tethered upward with scar-ring around an epispadiac meatus at the penile pubic junction. The bladder was small and scarred with negligible musculature and grossly abnormal mucosa. A urinary reservoir was created from distal ileum, cecum and ascending colon. The ureters were reimplanted into the cecum through the taeniae coli in a nonrefluxing manner, as described by Leadbetter and Clarke.4 Dorsal chordee was corrected and the penis was lengthened by releasing the corpora from the pubic rami. The appendix was laid into a trough created on the dorsum of the penis and brought through the glans penis (fig. 2).
Postoperatively the patient was initially continent but incontinence with high intraluminal pressures developed after 1 year. At reoperation the bowel was detubularized and the patient remains continent 11 years after detubularization. (In all subsequent patients the bowel was detubularized at surgery.) Bladder calculi also developed 8 years postoperatively and cystoscopic electrohydraulic lithotripsy was performed without difficulty via the appendiceal neourethra. Cystography and excretory urography (IVP) 10 years after surgery showed satisfactory bladder capacity without reflux and normal upper tracts.
Case 2. A 19-year-old man was referred with a history of ureterosigmoidostomy for bladder exstrophy at age 6 years, and subsequent fecal and urinary incontinence. An Indiana pouch was constructed with the appendix left unaltered on the cecum. The appendix was placed dorsally between the penile crura and brought through the glans as a meatus. The ileal limb was plicated and the ileocecal junction was intussuscepted to reinforce the valvular mechanism. An umbilical stoma was created with the ileal limb. The patient has the option of catheterizing via the appendiceal neourethra or umbilical stoma (fig. 3). At 8 years of follow-up he remains dry on self-intermittent catheterization every 4 to 5 hours. Follow-up cystograms at 3 months and yearly thereafter demonstrated good bladder capacity without reflux. IVP at 6 months with annual sonograms thereafter revealed normal upper tracts.
Case 3. A 12-year-old girl born with bladder exstrophy who underwent functional closure during early childhood and ileal conduit creation at age 9 years for total urinary incontinence presented for urinary undiversion at age 12 years. An ileocecal reservoir was created and the small fibrotic bladder was excised. The ileal conduit was opened and used as an onlay graft onto the cecum. The ureters were reimplanted into the posterior cecal wall. The appendix was used as an orthotopic neourethra. (fig. 4). The patient remains dry on self-intermittent catheterization 10 years after surgery. Cystography revealed 750 ml. bladder capacity without evidence of reflux and IVP showed stable bilateral calicectasis (fig. 5).
Cases 4 to 6. Two boys and 1 girl underwent bladder augmentation. The terminal ileum, cecum and ascending colon were detubularized, and the ileum was anastomosed to the cecum. The ileocecal pouch was used to augment the bladder, while the in situ appendix was brought to the umbilicus as a catheterizable stoma. Each patient underwent bladder neck reconstruction with a gracilis or rectus sling (fig. 6). These patients may catheterize through the umbilical stoma or neourethra and each remains dry on self-intermittent catheterization.
Cases 7 to 11. In 5 patients the ceco-appendiceal junction was incompetent and it was plicated. In I girl born with bladder exstrophy who had an ileal conduit and presented for undiversion an appendiceal orthotopic neourethra was created. This case was similar to case 3. The remaining patients underwent bladder augmentation with terminal ileum, cecum and ascending colon. In each case the ceco-appendiceal junction was plicated and the appendix was brought to the anterior abdominal wall as an umbilical stoma. All 5 patients remain dry on self-intermittent catheterization.
Four of our 11 patients underwent orthotopic lower urinary tract reconstruction in which the appendix was fashioned into a neourethra. In 2 boys the appendiceal neourethra was placed dorsally between the crura and brought through the glans penis. In 2 girls the appendiceal neourethra was brought through the anterior vaginal wall. We have had no difficulty mobilizing the appendix to the orthotopic position and suturing it in place only at the urinary meatus using 5-zero chromic gut suture. When possible, the greater omentum is mobilized and used to obliterate the dead space around the neourethra. In only 1 of these 4 patients the ceco-appendiceal junction was plicated to reinforce continence. These patients are continent between catheterizations 5 to 10 years postoperatively.
Following surgery a suprapubic Malecot catheter (18 to 22F depending on patient size) remains in place for 2 to 3 weeks for drainage. In patients with bladder augmentation an 8F catheter is placed in the umbilical stoma, while in those with an appendiceal neourethra the catheter is placed in the neourethra. A Foley catheter or pediatric feeding tube is placed and then removed 10 to 14 days after surgery. We have used Penrose drains but now we prefer a Jackson-Pratt drain placed near the suture line. The drain is removed after a few days, when drainage is minimal.
In all patients cystography and IVP are done at least once following surgical reconstruction. Each patient undergoes a yearly upper urinary tract study IVP or sonography) and cystography every 2 years. Serum electrolytes, creatinine and blood urea nitrogen are determined annually. The patient who underwent ureterosigmoidostomy was acidotic pre-operatively and postoperatively, requiring oral bicarbonate therapy. No other patients have required treatment for electrolyte abnormalities. In the 4 patients in whom serum vitamin B12 was measured levels were within normal limits and none of our patients has had clinical evidence of vitamin B12 deficiency. One patient had diarrhea for several months post-operatively but it gradually resolved without therapy. Patients who moved elsewhere or reside abroad have been monitored by local physicians with our follow-up done by correspondence.
We have performed repeat surgery in 2 cases. As noted previously, the initial patient required detubularization of the urinary reservoir because of urinary incontinence with increased intraluminal pressure. In another patient stomal stenosis developed 5 years postoperatively and stomal YV-plasty was successfully done on an outpatient basis.
In 1986 Grunberger et al reported the first use of a ceco-appendiceal unit for replacing the scarred exstrophy bladder of a 5-year-old boy.2 An orthotopic neourethra was created from the appendix, and the remnants of the bladder and urethra were wrapped around the ceco-appendiceal junction in an attempt to create a neobladder neck. In subsequent cases it became apparent that continence is maintained by the ceco-appendiceal junction itself.
In 1991 Hübner et al reported experimental evidence confirming our clinical observation of an anatomical and functional sphincter at the ceco-appendiceal junction.5 They measured the thickness of the circular muscle layer of the cecum, ceco-appendiceal junction and appendix, and found that the circular muscle is thickest at the ceco-appendiceal junction. They also measured in situ pressure profiles of the ceco-appendiceal junction and demonstrated that the junction remains competent at 37 to 112 cm. water.
As discussed, we test the competence of the ceco-appendiceal junction by filling the cecum. with saline via gravity. Although we have not done so, intraoperative manometric measurement of intracecal pressure can be used to determine leak point pressure of the ceco-appendiceal junction. Bissada measured appendiceal leak point pressure in adults undergoing continent diversion and he augmented the ceco-appendiceal junction when pressure was below 75 to 80 cm. water.6 However, using our simple method of ceco-appendiceal junction assessment and plication all patients have had continence between catheterizations 2 to 11 years after surgery. The technique of cecal plication around the base of the appendix is similar to the Nissen fundoplication of the gastroesophageal junction.7 This technique has been used to modify the antegrade continence enema procedure for our patients with fecal incontinence.3
In 1988 Maggio et a] presented experimental data derived from a canine study demonstrating the feasibility of ileocecal augmentation using an appendiceal neourethra.8 Their experimental observations provide credence to our contention that the lower urinary tract can be replaced in its entirety. Indeed, in 4 patients the appendix proved to be a reliable neourethra for up to 11 years. We have performed ileocecal augmentation with creation of an appendiceal neourethra in 4 patients. The 19-year-old man who also has an umbilical ileal stoma initially preferred to catheterize via the appendiceal neourethra but now he prefers to catheterize via the umbilical stoma. Our other patients have no difficulty catheterizing through the orthotopic neourethra and none has required revision of the appendiceal neourethra. Admittedly, it is easier to catheterize an umbilical stoma than an orthotopic neourethra, particularly for patients with limited dexterity or those confined to a wheelchair. However, for select patients the orthotopic neourethra is a viable alternative to the umbilical stoma, providing a near normal body image.
Lower urinary tract reconstruction may be performed using a ceco-appendiceal unit. The cecum may be used to augment or replace the native bladder. The appendix may then be brought out to the anterior abdominal wall or used to create an orthotopic neourethra. An anatomical and functional sphincteric mechanism exists at the ceco-appendiceal junction, which may be reinforced as necessary. This method of lower urinary tract reconstruction is useful for the pediatric population as well as adults.
1. Mitrofanoff, P.: Cystotomie continente transappendiculaire dans le traitment des vessies neurologiques. Chir. Ped., 21: 297, 1980.
2. Grunberger, I., Catanese, A. and Hanna, M. K.: Total replacement of bladder and urethra by cecum and appendix in bladder exstrophy. Urology, 28: 497, 1986.
3. Stock, J. A. and Hanna, M. K.: Appendiceal cecoplication: a modification of the Malone antegrade colonic enema procedure. Tech. Urol., 2: 40, 1996.
4. Leadbetter, W. F. and Clarke, B. G.: Five years' experience with uretero-enterostomy by the "combined" technique. J. Urol., 73: 67, 1955.
5. Hübner, W. A., Porpaczy, P. and Hartung, R.: Coeco-appendiceal junction: a neo-bladder neck? J. Urol., part 2, 145: 393A, abstract 722, 1991.
6. Bissada, N. K.: Characteristics and use of the in situ appendix as a continent catheterization stoma for continent urinary diversion in adults. J. Urol., 150: 151, 1993.
7. Nissen, R.: Eine einfache Operation zur Beeinflussung der Refluxoesophagitis. Schweiz Med. Wochenschr., 86: 590, 1956.
8. Maggio, M. I., King, L. R. and Kim, K. S.: Ileocecal bladder replacement utilizing appendix as neourethra. J. Urol., part 2, 139: 233A, abstract 284, 1988.