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A renal anomaly? Look for a Müllerian anomaly as well

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Contemporary PEDS Journal, July 2022,

A retrospective study found that postmenarchal women with a renal anomaly were also at risk of having a Müllerian anomaly.

A retrospective study found that young women (postmenarchal) with a renal anomaly (RA) also were at risk of having a Müllerian anomaly (MA). The study group included 326 patients with an RA in whom a pelvic examination revealed a coexisting MA in 125 (38.3%). Of those who underwent the pelvic exam because of pain, about one-third were found to have an MA. The average age for RA diagnosis was 6.4 years and for MA diagnosis 16.4 years. Of the patients with obstructive MAs, 93.8% underwent urgent surgery, with the others started on hormonal suppression.

Patients with what investigators termed “solitary kidney” (true renal agenesis and involuted early multicystic dysplastic kidney [MCDK]) were most likely also to have an MA. In these patients, MA prevalence was 67% and included a 30% risk of an obstructive anomaly. Solitary kidney was the most common RA diagnosis in the study group, seen in 37.6%, but persistent MCDK also posed a 20% to 23% risk of an MA.

These findings support routine screening for an MA in patients with an RA, especially those with a congenital solitary kidney, the authors noted.

Thoughts From Dr. Farber
There has been controversy over the years about kidney disease and associated conditions (should we image the kidneys in children with isolated preauricular pits?), but this looks to have a more solid evidence base. For technical reasons, screening is best done shortly after menarche. Note that hydronephrosis is not one of the renal disorders that predisposes to MAs.

Reference:

Fei YF, Smith YR, Wan J, Dendrinos M, Winfrey OK, Quint EH. Should we screen for Müllerian anomalies following diagnosis of a congenital renal anomaly? J Pediatric Urol. 2022;S1477-5131(22)001164-4. doi:10.1016/j.jpurol.2022.04.017