The “Rescue” Operation For Patients With Cloacal Exstrophy and Its Variants

Sunday, October 27, 2013: 9:52 AM
Windermere Ballroom W (Hyatt Regency Orlando, formerly the Peabody)
Andrea Bischoff, M.D., Giulia Brisighelli, M.D., Marc A. Levitt, M.D. and Alberto Peña, M.D., Colorectal Center for Children, Cincinnati Children's Hospital Medical Center, Cincinnati, OH

Purpose Patients with cloacal exstrophy have variable colonic length that ranges from normal to an almost absent colon.  A common error in the initial operative management is to create an ileostomy leaving the colon defunctionalized, and often still connected to the urinary tract. The negative implications of this are: a) the colon does not grow and b) the patient may suffer from hyperchloremic acidosis due to colonic absorption of urine.  An absent or a very short colon results in the incapacity to form solid stool due to lack of water absorption capacity and it disqualifies a patient from having a pullthrough.  Therefore, every effort must be made to preserve and use the available colon. Small colonic pieces will grow and improve their water absorption capacity, provided they are incorporated into the fecal stream.  These patients benefit from a “rescue” operation.  

Methods A search in our database was performed, looking for patients with cloacal exstrophy and its variants that underwent an inadequate diversion during the newborn period and required a “rescue” operation. 19 patients were identified. A retrospective review of the medical records of these patients was performed looking for gender, time of the “rescue” operation, colonic length rescued, symptoms present before the operation that improved after it, and follow up.

Results In all patients a piece of colon was rescued from the pelvis and incorporated into the fecal stream, the original stoma was closed and an end colostomy was created. 15 patients were female and 4 males. 13 had exstrophy variants and 6 had classic cloacal exstrophy. 18 patients received an ileostomy at birth, and 1 patient had a loop colostomy. The “rescue” operation was performed on average 26.9 months after birth (range 1 month – 12 years). The length of rescued colon ranged from 5.5 cm to 20 cm.  8 patients had symptoms that were present before and improved after the operation: acidosis (3), failure to thrive (2), sepsis (1), multiple urinary tract infection (1), and dehydration (1). On follow up, 10 patients still have their colostomies as we are waiting for continued colonic growth, 6 patients had a pullthrough after responding to our bowel management program through the stoma, 2 patients have a permanent stoma, and one patient expired.

Conclusion When patients with cloacal exstrophy or its variants incorrectly receive an ileostomy at birth, a rescue operation should be attempted in order to incorporate the colon into the fecal stream giving the colon the opportunity to grow enough to absorb water and form solid stool, which improves symptoms and makes the patient a candidate for a future colonic pullthrough.