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Kidney Week

Abstract: FR-PO635

Müllerian Anomalies in Girls with Solitary Functioning Kidney

Session Information

  • Pediatric Nephrology - II
    November 03, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
    Abstract Time: 10:00 AM - 12:00 PM

Category: Pediatric Nephrology

  • 1900 Pediatric Nephrology

Authors

  • Walawender, Laura E., Nationwide Children's Hospital, Columbus, Ohio, United States
  • Santhanam, Natasha, The Ohio State University Wexner Medical Center, Columbus, Ohio, United States
  • Davies, Benjamin, The Ohio State University Wexner Medical Center, Columbus, Ohio, United States
  • Fei, Yueyang Frances, Nationwide Children's Hospital, Columbus, Ohio, United States
  • Becknell, Brian, Nationwide Children's Hospital, Columbus, Ohio, United States
  • Mcleod, Daryl J., Nationwide Children's Hospital, Columbus, Ohio, United States
Background

Patients with Müllerian anomalies have a 30-50% increased prevalence of congenital renal anomalies and solitary functioning kidney (SFK) is the most common. However, the prevalence of Müllerian anomalies among patients with SFK is not well defined. A delay in diagnosis of obstructive Müllerian anomalies can increase the risk of poor clinical outcomes. This study describes the prevalence of Müllerian anomalies in patients with congenital SFK.

Methods

A retrospective review was performed of patients within the Nationwide Children’s Hospital system with ICD9 or ICD10 diagnostic codes for SFK. Analysis focused on girls with congenital SFK defined as either unilateral renal agenesis (URA) or multicystic dysplastic kidney (MCDK). Patients with complex urogenital pathology, such as, cloaca, urogenital sinus, or bladder exstrophy were excluded. Renal anomaly, Müllerian anomaly, reason for and type of pelvic evaluation, and age of diagnosis of anomalies were evaluated.

Results

Diagnostic codes identified 400 patients; 97 were excluded for complex urogenital anatomy. SFK was confirmed in 204 girls and 169 (82%) were congenital. There were 115 patients with URA and 54 with MCDK. Additional anomalies of the SFK were pelvic/ectopic (4), dysgenesis/hypoplasia (8), cystic (2), and other (19). Of patients with congenital SFK, 85 (50%) had a pelvic evaluation, most frequently in response to abdominal pain/dysmenorrhea (39%). Ultrasound was the most common imaging modality (74%). 49 (29%) patients had a Müllerian anomaly with the majority having combined uterine and vaginal anomalies (28) or isolated uterine anomaly (18). In 59% of patients, the renal anomaly was diagnosed prior to the Müllerian anomaly, at a median age of 6.5 years. In 24 patients, an obstructive Müllerian anomaly was found, including 11 who had a known renal anomaly.

Conclusion

The prevalence of Müllerian anomalies in patients with congenital SFK was 29%. Only half of patients with a congenital SFK had a pelvic evaluation. A Müllerian anomaly was found in about 60% of patients evaluated and it was obstructive in about one third of cases. Over half of the girls with Müllerian anomalies had a prior renal anomaly diagnosis. The high prevalence of Müllerian anomalies in patients with congenital SFK justifies routine screening pelvic ultrasound to improve early diagnosis.