Abstract
Unilateral renal agenesis and multicystic dysplastic kidney, resulting in a contralateral solitary functioning kidney (SFK), are part of the broad spectrum of congenital anomalies of the kidney and urinary tract (CAKUT). In girls with SFK, screening for asymptomatic Müllerian anomalies of uterus and vagina is not yet routinely performed, and therefore often overlooked until clinical complications in the menstrual cycle or fertility process occur. In this case series, we report on four teenagers with congenital SFK presenting with menstrual problems due to a Müllerian anomaly. Routine peri-menarchal screening for Müllerian anomalies in girls with SFK may provide timely counseling, surgical treatment and prevention of associated complications such as endometriosis, infertility and miscarriages.
Highlights
Unilateral renal agenesis (URA) and multicystic dysplastic kidney (MCDK), resulting in a contralateral solitary functioning kidney (SFK), are part of the broad spectrum of congenital anomalies of the kidney and urinary tract (CAKUT)
Even though the coincidence of congenital SFK and Müllerian anomalies has been described in many cases and studies, screening girls with SFK for uterine and vaginal anomalies has only been described in a recent recommendation [5], but may not yet be implemented in routine clinical care
Regardless of etiology, a SFK predisposes for the development of glomerular hyperfiltration injury, with long-term chronic kidney disease [1]
Summary
Unilateral renal agenesis (URA) and multicystic dysplastic kidney (MCDK), resulting in a contralateral solitary functioning kidney (SFK), are part of the broad spectrum of congenital anomalies of the kidney and urinary tract (CAKUT). Subsequent MRI demonstrated a left-sided non-communicating unicornuate uterus and a ipsilateral hematosalpinx She underwent a unilateral partial hysterectomy and salpingectomy, and oral contraceptives were started. The abdominal ultrasound was suspect of an uterine anomaly and the subsequent MRI revealed a uterus didelphys without vaginal anomalies. She is 12 years old, has started oral contraceptives and is awaiting surgical correction at the age of 16 years. She underwent an uncomplicated resection of the vaginal septum Her eGFR is 109 ml/min/1.73 m2, she has no proteinuria or hypertension, and ultrasound shows a solitary right kidney of 14.5 cm.
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