A New Case of Herlyn-Werner-Wunderlich Syndrome: Uterine Didelphys with Unilateral Cervical Dysgenesis, Vaginal Agenesis, Cervical Distal Ureteral Remnant Fistula, Ureterocele, and Renal Agenesis in a Patient with Contralateral Multicystic Dysplastic Kidney.

Jin-Hee Yu,Heayeon Choi,Byung-Moon Kang,Kun-Suk Kim,Sa-Ra Lee

doi:10.3390/diagnostics12010083

Jin-Hee Yu, Heayeon Choi + Show 3 more

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https://doi.org/10.3390/diagnostics12010083

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Journal: Diagnostics	Publication Date: Dec 30, 2021
Citations: 3	License type: CC BY 4.0

Affiliation: Ulsan College, University of Ulsan, Asan Medical Center

Abstract

The aim of this study was to present a new case of congenital Herlyn–Werner–Wunderlich syndrome, a rare anomaly of the female reproductive tract, and review the related literature. A 12-year-old girl presented with severe dysmenorrhea since menarche and magnetic resonance imaging showing a bicornuate uterus, double cervix, right hematometra, and hematosalpinx with ipsilateral renal agenesis, accompanied by a remnant distal ureter with hydroureter. A diagnostic cystoscopy and a reduced-port robot-assisted laparoscopy with chromopertubation were performed in order to identify the anomaly. Uterine didelphys and right cervical dysgenesis with ipsilateral vaginal agenesis, cervical distal ureteral remnant fistula, ureterocele, and renal agenesis were diagnosed on the basis of histopathologic findings, and she subsequently underwent a robotic unilateral right total hysterectomy with salpingectomy. This case report reinforces the importance of the intraoperative biopsy for an accurate diagnosis, despite magnetic resonance imaging being considered the gold-standard diagnostic tool.

Highlights

Herlyn–Werner–Wunderlich (HWW) syndrome is characterized by the triad of uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis [1,2,3]
We present a new category of HWW syndrome through a case of uterine didelphys combined with biopsy-proven unilateral cervical dysgenesis, vaginal agenesis, cervical distal ureteral remnant fistula, ureterocele, and renal agenesis in a girl with a contralateral multicystic dysplastic kidney (MCDK) and a history of severe dysmenorrhea since menarche
HWW syndrome is a rare genito-urinary anomaly that is mostly reported as uterine didelphys, blind hemivagina, and ipsilateral renal agenesis [3,4]; its clinical presentations are complex and diverse, including different combinations of uterine anomalies, such as unilateral cervicovaginal obstruction and ipsilateral renal anomalies, including paravaginal cystic structures and/or ureteral remnants [3,4]

Summary

Introduction

Herlyn–Werner–Wunderlich (HWW) syndrome is characterized by the triad of uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis [1,2,3]. HWW syndrome can present with a bicornuate uterus. We present a new category of HWW syndrome through a case of uterine didelphys combined with biopsy-proven unilateral cervical dysgenesis, vaginal agenesis, cervical distal ureteral remnant fistula, ureterocele, and renal agenesis in a girl with a contralateral multicystic dysplastic kidney (MCDK) and a history of severe dysmenorrhea since menarche. The aim of this study was to present a new case of congenital HWW syndrome, a rare anomaly of the female reproductive tract, and review the related literature. Magnetic resonance imaging (MRI) showed a bicornuate uterus, double cervix, right hematometra, and hematosalpinx with ipsilateral renal agenesis, accompanied by a remnant distal ureter with hydroureter. MCDK was noted, despite the patient’s renal function being within normal limits as determined by the blood urea nitrogen (BUN) level of 17 mg/dL and the creatinine level of 0.47 mg/dL

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