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J Pediatr Urol. 2018 Apr;14(2):144-149. doi: 10.1016/j.jpurol.2018.01.011. Epub 2018 Feb 9.

Screening for Mullerian anomalies in patients with unilateral renal agenesis: Leveraging early detection to prevent complications.

Author information

1
Brown University, Department of Obstetrics and Gynecology, The Warren Alpert Medical School, Providence, RI, USA. Electronic address: mfriedman@wihri.org.
2
Brown University, Division of Pediatric Urology, Hasbro Children's Hospital, The Warren Alpert Medical School, Providence, RI, USA.
3
Brown University, The Warren Alpert Medical School, Providence, RI, USA.
4
Brown University, Department of Obstetrics and Gynecology, Division of Reproductive Endocrinology and Infertility, The Warren Alpert Medical School, Providence, RI, USA.

Abstract

BACKGROUND:

Mullerian anomalies have a known association with renal agenesis yet, to date, there are no formal recommendations for screening women with certain renal anomalies for associated genital tract disorders.

OBJECTIVE:

The objective of this study is to review current data regarding the association between renal and Mullerian anomalies, and propose screening recommendations.

STUDY DESIGN:

A comprehensive review of the literature was performed to identify relevant articles using the keywords "unilateral renal agenesis," "renal anomalies," and "Mullerian anomalies."

RESULTS:

Over 30% of patients with unilateral renal agenesis have an associated Mullerian anomaly. However, diagnosis is frequently delayed in this population until after menarche when complications of retrograde menstruation with obstructive anomalies lead to significant problems including endometriosis, pelvic inflammatory disease, and infertility. No clear guidelines exist for communication among the antenatal sonographer, the obstetrician, the parents, and the child's pediatrician, which creates a barrier to effective screening and follow-up. Further, no current guidelines exist for screening women with certain renal anomalies for Mullerian anomalies.

DISCUSSION:

The complications of Mullerian anomalies are easily preventable if identified early. We propose new guidelines for education and screening for Mullerian anomalies in patients with unilateral renal agenesis (URA) and multicystic dysplastic kidney (MCDK) to guide providers, patients, and parents on proper identification and management (Table).

CONCLUSIONS:

Screening young women with URA and MCDK for Mullerian anomalies has the potential to prevent long-term complications from untreated obstructive malformations. Identification of unilateral renal agenesis on antenatal ultrasound must be clearly articulated with parents and the child's pediatrician so that proper screening can be performed before menarche. Pelvic sonography is a low-cost, high-yield screening tool to identify these anomalies.

KEYWORDS:

Mullerian anomalies; OHVIRA syndrome; Unilateral renal agenesis

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