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Published on: October 12, 2017
Müllerian anomalies in girls with congenital solitary kidney
Laura Walawender1,2, Natasha Santhanam1, Benjamin Davies1
1Kidney and Urinary Tract Center, Abigail Wexner Research Institute at Nationwide Children's Hospital, 700 Children's Drive, Columbus, OH, 43205, USA.
Insights
Müllerian anomalies (MA) are common in girls with solitary functioning kidney (SFK). Routine pelvic ultrasounds are recommended for early diagnosis of obstructive MA in these patients.
Area of Science:
- Pediatric Urology
- Reproductive Medicine
- Medical Genetics
Background:
- Müllerian anomalies (MA) prevalence in patients with congenital solitary functioning kidney (SFK) is not well-defined.
- Delayed diagnosis of obstructive MA increases risks of adverse clinical outcomes.
- This study aimed to define MA prevalence in patients with congenital SFK.
Purpose of the Study:
- To determine the prevalence of Müllerian anomalies (MA) in girls diagnosed with congenital solitary functioning kidney (SFK).
- To identify the types of MA and their association with obstructive patterns.
- To evaluate the diagnostic timing of SFK and MA.
Main Methods:
- Retrospective review of patients with congenital SFK (unilateral renal agenesis or multicystic dysplastic kidney) within a hospital system.
- Exclusion of patients with complex urogenital pathology.
- Evaluation of renal anomaly, MA, pelvic evaluation details, and age at diagnosis.
Main Results:
- Congenital SFK occurred in 431 girls (209 URA, 222 MCDK).
- MA were diagnosed in 60 patients (24% of those aged ≥10 years), with 38% being obstructive.
- MA were five times more common in URA than MCDK; SFK was diagnosed before MA in 75% of cases.
Conclusions:
- Müllerian anomalies are prevalent in girls with congenital SFK, with a significant proportion being obstructive.
- Routine screening pelvic ultrasound in girls with congenital SFK is warranted for early MA detection.
- Early diagnosis of MA can mitigate risks associated with obstructive anomalies.
Background:
The prevalence of Müllerian anomalies (MA) among patients with congenital solitary functioning kidney (SFK) is not well defined. A delay in diagnosis of obstructive MA can increase the risk of poor clinical outcomes. This study describes the prevalence of MA 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 congenital SFK defined as either unilateral renal agenesis (URA) or multicystic dysplastic kidney (MCDK) and confirmed by chart review. Patients with complex urogenital pathology were excluded. Renal anomaly, MA, reason for and type of pelvic evaluation, and age of diagnosis of anomalies were evaluated.
Results:
Congenital SFK occurred in 431 girls due to URA (209) or MCDK (222). Pelvic evaluation, most commonly by ultrasound for evaluation of abdominal pain or dysmenorrhea, occurred in 115 patients leading to MA diagnosis in 60 instances. Among 221 patients ages 10 years and older, 104 underwent pelvic evaluation and 52 were diagnosed with an MA of which 20 were obstructive. Isolated uterine or combined uterine and vaginal anomalies were the most common MA. MA were five-fold more common in patients with URA compared to MCDK. In 75% of patients, the SFK was diagnosed prior to the MA.
Conclusions:
The prevalence of MA in patients with congenital SFK was 24% among those age 10 years or older, and 38% were obstructive. This justifies routine screening pelvic ultrasound in girls with congenital SFK to improve early diagnosis.
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