Case 1: A 10-year-old boy sustained an accidental gunshot injury resulting in a shattered perineum. He had pelvic fracture with anorectal as well as urethral disruption. He underwent an emergency left transverse colostomy and a suprapubic cystostomy. Later, in another hospital, the ureters were re-implanted into the sigmoid colon that was used as an incontinent urinary reservoir. The upper end of descending colon (distal to the transverse colostomy) was obliterated with a non-absorbable ligature.
The child presented to us for urinary undiversion. Through Posterior sagittal approach anorectoplasty and perineal reconstruction was done. Urethral repair was also performed by end-to-end anastomosis. The ureters were re-implanted into the defunctionalised urinary bladder (Cohen’s method) and a Mitrofanoff procedure using appendiceal conduit was performed. Subsequently the colostomy was closed. The patient refrained from doing clean intermittent catheterization (CIC) through either conduits (urethra and appendicovesicostomy) in the sheer delight of being once again able to pass urine per urethra after 1 year of initial injury. Six months later, the Mitrofanoff appendicovesicostomy was excised on request. The child was lost to follow up.
Five years later, he presented with hypertension, chronic renal failure and right-sided optic nerve atrophy. There was bilateral hydroureteronephrosis on abdominal ultrasound. He was diagnosed to have neurogenic bladder (hypocontractile) with a large post-void residual urine volume. Voiding cystourethrogram revealed major degree vesicoureteric reflux on both sides. Cystourethroscopy demonstrated the urethra to be normal. He was advised CIC per urethra but was not accepted as it produced pain, being sensitive’ urethra.
Bilateral ureteral re-implantation was done. Since the appendix used for Mitrofanoff appendicovesicostomy had already been excised, a continent catheterizable stoma was fashioned using the Yang-Monti principle. A 2 cm segment of ileum was mobilized with a well vascularized mesentery. The ileal segment was divided longitudinally on its antimesenteric border. The opened bowel was then tubularized over a 12 F catheter along the long transverse axis, perpendicular to the mesentery. This was done in two layers, using fine absorbable sutures for mucosal approximation followed by a second serosal layer. The ends were closed with interrupted sutures while the middle part was closed with a running suture. The end result was a lengthened segment of bowel, about 7 cm long, with a perpendicular vascular pedicle (Fig. ). This tube was re-implanted into the bladder as in appendicovesicostomy.
Figure 1: Intra-operative photograph showing ileal graft appearing very similar to vascularized appendicular graft generally used for MACE or Mitrofanoff procedures.
The patient was advised CIC through Yang-Monti channel while awake and continuous bladder drainage at night. Though he religiously followed this advice, his compliance to other medical treatment was low. He was being treated for long time by religious leaders and quacks. This worsened his renal functions drastically. At the age of 19 years, he was advised renal transplantation, but there were no willing donors. For next two years, he required biweekly hemodialysis. At age 21, he died of chronic renal failure and its related complications.
Case 2: A boy with exstrophy bladder and double phallus was seen at birth. The phallus in continuity with the urinary bladder was found to be rudimentary. At the age of three months, he developed strangulation of right inguinal hernia, which led to resection of gangrenous ileo-cecal junction along with the appendix. He underwent primary bladder closure after six months.
At the age of four years, he was diagnosed to have major degree of vesicoureteric reflux on the left side with a small capacity bladder. As the appendix was lost at previous surgery, Mitrofanoff appendicovesicostomy could not be performed. Hence, Yang-Monti ileovesicostomy, using the same technique as described in case 1 was performed. At a later stage, genitoplasty was done. He was also advised daytime CIC and continuous bladder drainage at night. He has been doing well on follow-up as regards the renal functions and the social acceptability and the Yang-Monti channel has been complication-free since the last one decade.
Case 3: An eight-year old boy presented with repaired exstrophy bladder, done elsewhere, at the age of one year. He had been leaking urine through the wide penopubic fistula all these years. No attempt had been made to reconstruct his epispadiac penis. On investigations, he was diagnosed to have small capacity bladder (approximately 20cc) and preserved renal tracts. Bladder augmentation with colon, Young-Dees-Leadbetter bladder neck reconstruction along with Mitrofanoff procedure was done. Due to the small size of the native bladder and unusual configuration of the vermiform appendix, the latter was implanted in the bowel ‘augment’ and not the native bladder. The caecal end of the Mitrofanoff conduit was implanted in the ‘augment’ and the appendicular tip was brought out at the skin surface. The postoperative course was uneventful.
The patient returned after 8 months with stenosed Mitrofanoff channel. He had again started leaking from the penopubic fistula suggesting a failed bladder neck reconstruction. He was readmitted and prepared for the revision of Mitrofanoff channel, reconstruction/ closure of bladder neck and epispadias repair. At exploration, the capacity of the augmented bladder was satisfactory, but surprisingly the entire Mitrofanoff channel had disappeared. A continent catheterizable stoma with ileum was fashioned using the Yang-Monti principle. One important intra-operative complication worth mentioning here is that vascular pedicle of initial Yang-Monti channel was accidentally damaged by an assistant during surgery. A second Yang-Monti channel was similarly constructed with much ease. Bladder neck closure and Ransley’s repair of epispadias was done.
The child has been followed for 2 ½ years since the last surgery. He has been doing well on daytime 3 hourly CIC, daily bladder wash and night time bladder drainage through Yang-Monti channel.