- Incidence: 1:50000
- Initial management
- Cover bladder with Saran wrap; change with each diaper change and irrigate bladder surface with sterile saline to keep moist
- Goal of surgical repair
- Primary bladder closure with protection of upper tracts
- Eventual voided continence
- Kelly repair - modern staged repair of exstrophy (MSRE) - 1970s
- Newborn – Closure of the bladder, posterior urethra, and abdominal wall defect results in the creation of complete (penopubic) epispadias
- Six months to one year of age – Epispadias repair is performed
- Four to five years of age – Bladder neck reconstruction (BNR) and bilateral ureteral reimplantation if needed
- Mainz approach - complete primary repair of exstrophy (CPRE) - 1990s
- Completes all components of repair in a single surgery
- Often performed right after birth
- No direct comparison between the two methods (rare cases; generally one center will perform all cases the same way)
- Requirement for iliac osteotomies - <72 hours old, very wide (> 4 cm) diastasis
- Will need traction x 4 weeks afterwards
- Postop - SPT for 4 weeks, ureteral stents x 2-4 weeks
- Failed repair - dehisence, prolapse, outlet obstruction, vesicocutaneous fistula
- Important to completely dissect the urogenital diaphragm fibers
- In general continence rates ~ 70%
author:
last edited: May 25, 2022, 3:11 p.m. | pk: 196
| unpublished