V7-05: Two-stage cloacal exstrophy closure in a male: a case from the multi-institutional bladder exstrophy consortium (MIBEC)
Cloacal exstrophy is a rare diagnosis that presents complex anatomical challenges and requires extensive surgical reconstruction. To increase experience and proficiency in the care of exstrophy patients, the Multi-Institutional Bladder Exstrophy Consortium (MIBEC) was formed in February 2013. This collaborative effort involves major reconstruction of rare diagnoses within the exstrophy-epispadias complex, including cloacal exstrophy. Our objective is to use one specific example of a prenatally diagnosed 46,XY male with cloacal exstrophy to serve as an overview of the complex reconstruction required and to highlight the outcome that can be achieved using a two-stage approach.
The surgical goals of the 1st stage in the neonate were to close the omphalocele, separate the gastrointestinal tract from the hemi-bladders, construct a single bladder plate from the hemi-bladders (i.e. convert the cloacal exstrophy to the anatomy of classic bladder exstrophy) and create a hindgut end-colostomy. The 2nd stage was performed following neurosurgical detethering of the spinal cord and good somatic growth. The surgical goals of the 2nd stage were to close the bladder and reconstruct the external genitalia. The 2nd stage required bilateral anterior iliac osteotomies with internal pins in order to reduce the wide pubic diastasis and stabilize the repair, respectively. The patient was immobilized with spica cast post-operatively.
The patient underwent successful 1st repair at 5 days of age and 2nd stage of reconstruction at 15 months of age. He recovered well without dehiscence, wound breakdown or infectious complication. Voiding cystourethrogram at four weeks post-operation documented an intact bladder, no vesicoureteral reflux and urination per urethra. Renal ultrasound revealed no new hydronephrosis in normal kidneys. Pubic diastasis decreased from 7.2cm preoperatively to 1.8cm immediately postoperatively, and the spica cast was removed 8 weeks after the 2nd stage.
Cloacal exstrophy is rare and complex. Familiarity with reconstructive surgical goals and techniques is paramount to optimal outcome. Bilateral iliac osteotomy is critical to reduce wide pubic diastasis, reconstruct pelvic floor soft tissue and bony anatomy, and to assist in successful bladder closure and reconstruction of the external genitalia.