Research Article| Volume 18, ISSUE 5, P676.e1-676.e7, October 2022

Should we screen for Müllerian anomalies following diagnosis of a congenital renal anomaly?



      Despite the well-established embryological relationship in the development of renal and Müllerian structures, no clear guidelines exist regarding screening for Müllerian anomalies (MA) in the setting of a renal anomaly (RA). Delayed diagnosis of MA can have significant reproductive consequences.


      To investigate the prevalence of coexisting MA in patients with congenital RA.

      Study design

      This is a retrospective cohort study of females age 12–35 years with a diagnosis of RA, identified by diagnosis codes, who were followed for care between 2013 and 2020. Data were collected on demographics, medical history, clinical presentation, and imaging studies. Descriptive statistics were used to summarize the data. This study was IRB approved.


      A total of 465 patients were included in this study, of whom 326 patients (70.3%) had a pelvic evaluation during the study period. Of these 326 patients, 125 (38.3%) were found to have coexistent MA. About one-third of patients who underwent pelvic evaluation due to pain were found to have MA.
      For 69.6% (87/125) of patients with MA, the RA was diagnosed prior to the MA. The average age at time of RA diagnosis was 6.4 ± 8.8 years and the average age of MA diagnosis was 16.4 ± 6.9 years. Forty-eight (38.4%) patients had obstructive anomalies. Of the Müllerian obstructions, 93.8% were treated with urgent surgery and the remainder started on hormonal suppression.
      The prevalence of MA was dependent on the RA diagnosis (Figure). Of patients with a solitary kidney, 67.1% were diagnosed with MA. For other parenchymal RA, the prevalence of MA was 20–23%. In patients with solitary kidney, uterus didelphys was the most common MA (52.1%). Thirty percent of patients with a solitary kidney were diagnosed with an obstructive MA.


      Summary Figure
      Graphical AbstractPrevalence of Müllerian anomaly diagnoses (obstructive and non-obstructive) related to renal anomaly diagnoses. MA, Müllerian anomaly; RA, renal anomaly. aPatients could have more than one RA diagnosis. Includes those with only duplicated collecting system, ureteropelvic junction obstruction, megaureter, ectopic ureter, ureterocele, or vesicoureteral reflux.


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