Summary
Introduction
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.
Objective
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.
Results
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.
Conclusions

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.
Keywords
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Article info
Publication history
Published online: April 28, 2022
Accepted:
April 21,
2022
Received in revised form:
April 12,
2022
Received:
August 23,
2021
Identification
Copyright
© 2022 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.