Complete Genito-urinary Reconstruction in a 9 year old boy for failed Ectopia Vesicae Epispadias Complex repair. | DesiMD

Complete Genito-urinary Reconstruction in a 9 year old boy for failed Ectopia Vesicae Epispadias Complex repair.



Bladder exstrophy is the most common of a larger group of birth defects called the bladder exstrophy-epispadias complex more common in males than in females. Classic bladder exstrophy is a rare malformation of the genitourinary tract and its incidence is around 1 case to 30.000 to 40.000 live births. Bladder exstrophy itself also has a range of severity and, in addition to the abnormalities in the bladder, genitals and pelvic bones, may include defects in the intestines and reproductive organs.

Children with bladder exstrophy will also have a condition called vesicoureteral reflux, in which urine flows the wrong way — from the bladder back up into the tubes that connect to the kidneys (ureters). Children with bladder exstrophy also have epispadias.

These defects are treated through surgical procedures that repair the affected organs, muscles and bones.

The management of children with bladder exstrophy remains a challenge and despite the choice of approach (staged versus one-stage reconstruction), patients have to undergo several procedures in order to attain goals of surgical treatment such as: urinary continence, preservation of upper urinary tract and genital function and cosmesis.

A nine year boy from Iraq presented with urinary incontinence since birth and walking disability due to left hip advanced flexion deformity due to congenital dislocation of hip and scoliosis of the spine with severely deformed pelvis  pubic diastasis. His bowel was constipated. He had undergone surgery for exstrophy with epispadias complex at the age of 3 year, which unfortunately failed with multiple urethrocutaneous fistulae and urinary incontinence. On clinical examination he had gross pubic diastasis with deformed asymmetrical pelvis and had multiple urethrocutaneous fistulae on dorsal aspect of penis. His perineal skin was sore due to urine contact dermatitis. He underwent radiological and urodynamic evaluation along with other blood tests, MCU showed bilateral gross vesicoureteric reflux Grade 5 (in reflux urine goes back in to kidney) with small capacity bladder. Urodynamic study hypocontractile (weak) bladder with capacity of 100 ml. CT urogram showed bilateral gross hydroureteronephrosis with dilated ureters and  functioning kidneys. This was a very challenging case in view of complex genitourinary reconstruction. Patient’s father was counselled about the supra-major reconstructive surgery of the genitourinary tract. His father was also warned that he may not be able to pass urine through urethra. He may have to empty his bladder periodically (CIC). In child it is difficult task to evacuate bladder by putting catheter through urethra as urethra is sensate many children are not co-operative.

I took up this challenge and performed the most complex surgery of 13 hours duration under 3.5X magnification- Augmentation cystoplasty, Bladder neck reconstruction (Young-Dees  Leadbetter) with epispadias repair, Left to right transureteroureterostomy with left ureteric reimplantation and Mitrofanoff’s procedure ( appendico-vesicostomy for draining the urine by clean intermittent catheterization as urethra is sensate). The intra-op and postoperative period were uneventful. Boy was managed well in pediatric ICU in post op period. This surgery could be possible only due to great help of OT staffs, anesthetist and excellent infrastructure at Fortis Hospital and great pediatric ICU backup. It came as a surprise that boy is passing urine through natural urine passage (urethra). He is dry now and no urinous smell coming from body. The boy and his father are very happy and went back home. Has been called for follow up after 6 months.

Dr Suresh Kumar Bhagat is one of the best Urologist in mumbai

Dr Suresh K. Bhagat is a Senior Consultant Urologist and Renal Transplant Surgeon in mumbai with more than 20 years of experience.