Cloacal Extrophy: A Unified Management Plan
S.Z. Soffer, N. Rosen, M. Alexianu and Alberto Peña
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Purpose
Despite a significant improvement in survival in the cloacal extrophy patient, quality of life is still poor and most children are left with an intestinal stoma.
The purpose of this paper is to describe a unified approach which optimizes the use of available bowel, minimizes the likelihood of long-term enterostomy, and allows these children to remain clean 24 hours a day.
Methods
The medical records of 23 patients treated for cloacal extrophy were reviewed retrospectively. In all patients bladder closure, omphalocele repair, and creation of an intestinal stoma were performed at birth.
Observation for at least one year allowed for colon maturation and assessment of the patient's ability to form solid stool through their stoma.
Capacity to form solid stool or response to a preoperative bowel management regimen through the stoma were considered indications for pull-through.
Timing and type of tissue used for genitourinary reconstruction was contingent upon the colorectal plan.
Results
All patients are alive. Three patients are fecally continent, four are continent with occasional soiling, ten remain clean with a bowel management regimen, two have permanent ileostomies and three are too young to assess.
One patient is urinary continent, twelve are dry with intermittent catheterization, eight await further urologic reconstruction and one is too young to assess. One patient is both urinary and fecally incontinent and refuses further treatment.
Conclusion
Cloacal extrophy patients possess a colon varying in size from virtually none at all to near normal length. During neonatal repair a colostomy should be formed incorporating all pieces of colon, regardless of size.
A pull-through should be undertaken if the patient has normal or near normal colonic length, or has demonstrated the ability to form solid stool preoperatively regardless of the quality of the sacrum or sphincters, allowing for successful bowel management even if the child is not primarily continent.
Decisions regarding genitourinary reconstruction should not be made until the gastrointestinal picture becomes clear.
A master plan must be conceived at birth so the pediatric surgeon and urologist do not inhibit each other's reconstructive options by focusing only on their particular system.
For more information or to request an appointment, please contact the Colorectal Center at Cincinnati Children's.
