Does a standardized operative approach in cloacal reconstruction allow for preservation of a patent urethra?

BACKGROUND: Cloacal malformations are recognized as a particularly challenging congenital condition to manage and they present with a wide spectrum of anatomical configurations making surgical repair very complicated. Urethral necrosis or urethral loss is a known and devastating complication of cloacal repair. The surgical repair of these malformations has evolved over time and historically only common channel (CC) length was measured. More recently, it has been advocated that the urethral length and the CC are both important in determining surgical repair. The purpose of our study is to evaluate if this surgical approach allows for preservation of a patent urethra.

METHODS: A prospective database of all cloaca patients maintained with IRB approval (IRB# STUDY00000721) was retrospectively reviewed. We included any girl with cloacal malformation who underwent primary repair at our institution between May 2014 and December 2019. Standardized preop evaluation with endoscopy and 3-dimentional imaging to assess urethral length and CC length. These measurements were used to determine operative approach. Girls with CC < 1 cm undergo posterior sagittal anorectoplasty and introitoplasty (PSARP + I), those with CC measuring 1-3 cm and urethra > 1.5 cm undergo total urogenital mobilization (TUM) and those with CC > 3 cm or urethra < 1.5 cm undergo urogenital separation (UGS). Postoperative urethral patency was determined at the time of cystoscopy and exam under anesthesia (EUA) 4-6 weeks postoperatively by visualizing a viable and healthy urethra that is catheterizable.

RESULTS: A total of 59 patients met inclusion criteria with a median age of 11.6 months. Four girls underwent PSARP + I, 19 girls had a TUM and 36 girls underwent a UGS. All of the girls who had PSARP +I (n = 4) or TUM (n = 19) had a viable and patent urethra that was catheterizable at the cystoscopy and EUA 4-6 weeks postoperatively. Of the 36 girls who had UGS, all but 2 (5.6%) had a viable and patent urethra that catheterized without problems. Overall, 97% of girls in this cohort had a patent urethra after cloacal repair using this surgical protocol.

CONCLUSIONS: The use of a standard protocol that considers urethral and common channel length for cloacal repairs results in a viable and patent urethra in 97% of patients.

LEVEL OF EVIDENCE: Level II.