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Pelvic Ectopic Kidney Prevalence and Pressure Changes During Cloacal Exstrophy (Omphalocele-Exstrophy-Imperforate
Ted Lee1, Elizabeth Roth2, Aseem Shukla3
1Boston Children's Hospital, Department of Urology, Boston, MA.
Insights
Pelvic ectopic kidneys (PEK) were found in 30% of patients with Omphalocele-Exstrophy-Imperforate Anus-Spinal Defects Syndrome (OEIS). Renal parameters showed higher changes in PEK compared to orthotopic kidneys during surgical repair.
Area of Science:
- Urology
- Pediatric Surgery
- Medical Imaging
Background:
- Pelvic ectopic kidney (PEK) is a congenital anomaly where the kidney is located in the pelvic cavity.
- The Omphalocele-Exstrophy-Imperforate Anus-Spinal Defects Syndrome (OEIS) is a complex condition that can include PEK.
- Surgical repair of OEIS, particularly pubic bone approximation, may impact renal parameters.
Purpose of the Study:
- To determine the prevalence of PEK in patients with OEIS.
- To compare renal parameters, including renal pelvis pressure (RPP), peak systolic velocity (PSV), and resistive index (RI), between PEK and orthotopic kidneys after pubic bone approximation.
- To assess the implications of these changes for perioperative management.
Main Methods:
- A retrospective review of 80 OEIS patients was conducted to determine PEK prevalence.
- In four OEIS patients with both PEK and orthotopic kidneys, RPP, PSV, and RI were measured before and after pubic bone approximation during second-stage repair.
- Paired t-tests were used for statistical analysis of renal parameters.
Main Results:
- The prevalence of PEK among OEIS patients was 30% (24 out of 80), with 4% having a solitary PEK.
- Following pubic bone approximation, the mean increase in RPP was higher in PEK (+26.0 mmHg) compared to orthotopic kidneys (+10.3 mmHg).
- Mean changes in PSV and RI also showed distinct patterns between PEK and orthotopic kidneys, though not statistically significant (P=0.09 and P=0.29, respectively).
Conclusions:
- Pelvic ectopic kidneys are common in OEIS patients.
- Renal parameters like RPP, PSV, and RI exhibit greater changes in PEK compared to orthotopic kidneys during surgical repair.
- Real-time RPP monitoring may be beneficial for managing OEIS patients, especially those with solitary PEK, during the perioperative period.
Objectives:
To assess the prevalence of pelvic ectopic kidneys (PEK) and compare renal parameters of the PEK to the orthotopic kidney following pubic bone approximation.
Methods:
In four Omphalocele-Exstrophy-Imperforate Anus-Spinal Defects Syndrome (OEIS) patients undergoing second-stage repair with known pelvic and orthotopic kidneys, changes in the renal pelvis pressure (RPP), peak systolic velocity (PSV), and resistive index (RI) were measured in the pelvic and orthotopic kidneys following pubic bone approximation. A paired t-test was performed for analysis. Prevalence was tabulated using a multi-institutional retrospective review.
Results:
Mean rise in RPP was +26.0 mmHg in the PEK and +10.3 mmHg in the orthotopic kidney (P = .55). One patient had immediate increase in RPP of 66 mmHg in the PEK (7 mmHg in the orthotopic kidney) which decreased to +17 mmHg in the PEK upon release of the pubic approximation stitch. Mean change in PSV was +67.7 cm/s in the PEK compared to - 25.7 cm/s in the orthotopic kidney (P = .09). Mean change in renal RI was + 0.06 in PEK compared to - 0.01 in the orthotopic kidney (P = .29). Among 80 OEIS patients, 24 (30%) had a PEK. 3 (4%) had a solitary PEK.
Conclusion:
During second-stage OEIS closure, we witnessed patterns of higher change in RPP, PSV, and RI of the PEK compared to the orthotopic kidney. Furthermore, PEKs were found in nearly a third of our OEIS patients. Real-time RPP monitoring, especially those with solitary PEK, may aid in the management of OEIS patients during the perioperative period.
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