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Following a strict renal protection protocol in cloacal malformations: A descriptive analysis
Shruthi Srinivas1, Jacqueline P Morin2, Katherine C Bergus1
1Department of Pediatric Colorectal and Pelvic Reconstructive Surgery, Nationwide Children's Hospital, Columbus, OH, USA.
Insights
A strict renal protection protocol significantly reduced progressive renal dysfunction in children with cloacal malformations, with only 2.9% progressing to this stage. Most patients with renal dysfunction had pre-existing kidney issues at presentation.
Area of Science:
- Pediatric Urology
- Nephrology
- Congenital Malformations
Background:
- Cloacal malformations pose a significant risk of renal dysfunction in children, with historical incidence rates up to 50%.
- Effective management strategies are crucial to mitigate renal impairment in this vulnerable population.
- The long-term impact of a dedicated renal protection protocol on renal function in these patients remains to be fully elucidated.
Purpose of the Study:
- To evaluate the incidence of renal dysfunction in children with cloacal malformations managed under a strict renal protection protocol.
- To assess the effectiveness of the protocol in preventing or delaying the progression of renal impairment.
Main Methods:
- A prospective database of 105 children with cloacal malformations managed since the implementation of a renal protection protocol was reviewed.
- The protocol included regular laboratory evaluation, appropriate surgical approach selection, proactive imaging, and early catheterization.
- Renal dysfunction was defined as CKD grade 3b or higher, or need for renal replacement therapy (RRT) or transplantation; GFR was calculated using the Schwartz formula.
Main Results:
- The median follow-up was 4.2 years, with 5.7% of children experiencing renal dysfunction at the most recent follow-up.
- Only 2.9% of children progressed from normal renal function at initial evaluation to renal dysfunction.
- No child with initially normal renal function progressed to require dialysis or transplantation.
Conclusions:
- A strict, multi-disciplinary renal protection protocol is associated with a low incidence (2.9%) of progressive renal dysfunction in children with cloacal malformations.
- The majority of children who developed renal dysfunction had pre-existing kidney impairment at presentation.
- This protocol suggests that preservation of renal function in early childhood is achievable.
Background:
In children with cloacal malformations, renal dysfunction is a constant concern, with reported incidence as high as 50%. Multiple factors exist that may impair renal function. Our institution follows a strict renal protection protocol in this population. Incidence of renal dysfunction in these patients is unknown.
Objective:
We aimed to evaluate incidence of renal dysfunction while implementing this protocol in a cohort of children with cloacal malformation.
Study Design:
We reviewed a prospectively collected database of children with cloacal malformations managed at a single institution since implementation of a renal protection protocol. This involves regular laboratory evaluation, appropriate selection of total urogenital mobilization or urogenital separation, proactive imaging in patients with signs of impending renal dysfunction or urinary retention, and early catheterization teaching and implementation if necessary. Glomerular filtration rate (GFR) was calculated with the Schwartz formula and CKD grades assigned per standard definitions. Renal dysfunction was defined as CKD grade 3b or higher, need for renal replacement therapy (RRT) or transplantation. Descriptive statistics were computed.
Results:
A total of 105 children were managed under this protocol with a median follow-up of 4.2 years [IQR: 2.0-5.9]. Six children (5.7%) had renal dysfunction at most recent follow-up; of these children, only three (2.9%) progressed from normal renal function at initial evaluation to renal dysfunction (Table). No child with normal presenting renal function thus far has progressed to require dialysis or transplantation.
Discussion:
Previous literature estimated rates of renal dysfunction in cloaca patients as high as 50%; in contrast, we demonstrate a rate of progression to renal dysfunction of 2.9% in girls following a strict renal protection protocol. Most children who developed renal dysfunction had dysfunctional kidneys on presentation. This suggests that preservation of renal function may be possible in early childhood with a strict, multi-disciplinary renal protection protocol.
Conclusion:
In our cohort of patients with cloacal malformations following a strict renal protection protocol, incidence of progressive renal dysfunction is low at 2.9%. Most who go on to renal dysfunction present with impaired renal function.
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