<strong>Background:</strong> Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare type of female reproduction tract malformation, often accompanied by abnormal development of the ipsilateral urinary system, ...<strong>Background:</strong> Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare type of female reproduction tract malformation, often accompanied by abnormal development of the ipsilateral urinary system, which shows mostly double uterus and the absence of the kidney on the oblique side. <strong>Case Report:</strong> An 11-year-old patient presented with lower abdominal distension and pain for 2 days and was suspected of vaginal haematoma. <strong>Conclusion:</strong> Ipsilateral renal agenesis was confirmed by ultrasound during hospitalization, and the diagnoses of obstructed hemivagina and ipsilateral renal anomaly syndrome were established. The aim of this study was to present the diagnostic and therapeutic approaches in HWWS, and to draw attention to the challenges in its diagnosis, which can coexist with multiple complications.展开更多
Rationale:Herlyn-Werner-Wunderlich syndrome,also known as obstructed hemi-vagina with ipsilateral renal agenesis(OHVIRA)syndrome,is a rare congenital anomaly of the Müllerian and Wolffian ducts.It is often asympt...Rationale:Herlyn-Werner-Wunderlich syndrome,also known as obstructed hemi-vagina with ipsilateral renal agenesis(OHVIRA)syndrome,is a rare congenital anomaly of the Müllerian and Wolffian ducts.It is often asymptomatic until puberty and is usually misdiagnosed as infertility.Patient concerns:A 39-year-old woman desirous of pregnancy was referred to our radiodiagnosis unit for imaging evaluation on account of secondary infertility.Diagnosis:Incidental discovery of uterine bicornis bicollis and blind-ending hemivagina was noted at hysterosalpingography.Interventions:This necessitated the search for other genitourinary tract anomalies,and ipsilateral renal agenesis was also discovered.Outcomes:A diagnosis of Herlyn-Werner-Wunderlich syndrome was made.Lessons:Magnetic resonance imaging(MRI)is considered the gold standard imaging modality for the assessment of uterine malformations.However,in resource-poor centers where MRI is unavailable,a heightened clinical suspicion with the combination of available imaging modalities is important and reliable in making an accurate diagnosis,hence,successful management.展开更多
Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital anomaly characterized by <span style="font-family:Verdana;">triad</span><span style="font-family:Verdana;"> of uterus ...Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital anomaly characterized by <span style="font-family:Verdana;">triad</span><span style="font-family:Verdana;"> of uterus didelphys, obstructed </span><span style="font-family:Verdana;">hemi</span><span style="font-family:Verdana;"> vagina, and ipsilateral renal agenesis. The most common presentation is abdominal pain, dysmenorrhea, and abdominal mass secondary to </span><span style="font-family:Verdana;">hemi</span><span style="font-family:Verdana;">-</span><span style="font-family:Verdana;">hematometrocolpos</span><span style="font-family:Verdana;">. We report an emergency presentation of this syndrome during the pandemic of COVID-19 in Qatif Central Hospital, Saudi Arabia, April 2020. An 11-year-old Saudi girl presented to the emergency room with three months history of cyclical lower abdominal pain. The pain was progressive, continuous </span><span style="font-family:Verdana;">and</span><span style="font-family:Verdana;"> not relieved by analgesics. Abdominal examination revealed a tender abdominal mass mainly in the left iliac fossa, Ultrasound evaluation showed two uterine bodies. The left uterus was distended with complex fluid. Pelvic MRI findings consistent with Herlyn-Werner-Wunderlich (HWW) syndrome were found with uterine </span><span style="font-family:Verdana;">didelphys</span><span style="font-family:Verdana;"> and </span><span style="font-family:Verdana;">left sided</span><span style="font-family:Verdana;"> hematometra resulting from obstructed </span><span style="font-family:Verdana;">hemi</span><span style="font-family:Verdana;">-vagina and ipsilateral agenesis of the left kidney. Resection of the vaginal septum and drainage of hematometra was done. The patient recovered with normal cyclical menstruation. Cyclical or continuous lower abdominal pain with or without amenorrhea is the usual presentation of HWW syndrome during adolescence. Diagnosis is made by ultrasonography and MRI. Early diagnosis and accurate management can provide pain relief, prevent future complications, and preserve fertility.</span>展开更多
文摘<strong>Background:</strong> Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare type of female reproduction tract malformation, often accompanied by abnormal development of the ipsilateral urinary system, which shows mostly double uterus and the absence of the kidney on the oblique side. <strong>Case Report:</strong> An 11-year-old patient presented with lower abdominal distension and pain for 2 days and was suspected of vaginal haematoma. <strong>Conclusion:</strong> Ipsilateral renal agenesis was confirmed by ultrasound during hospitalization, and the diagnoses of obstructed hemivagina and ipsilateral renal anomaly syndrome were established. The aim of this study was to present the diagnostic and therapeutic approaches in HWWS, and to draw attention to the challenges in its diagnosis, which can coexist with multiple complications.
文摘Rationale:Herlyn-Werner-Wunderlich syndrome,also known as obstructed hemi-vagina with ipsilateral renal agenesis(OHVIRA)syndrome,is a rare congenital anomaly of the Müllerian and Wolffian ducts.It is often asymptomatic until puberty and is usually misdiagnosed as infertility.Patient concerns:A 39-year-old woman desirous of pregnancy was referred to our radiodiagnosis unit for imaging evaluation on account of secondary infertility.Diagnosis:Incidental discovery of uterine bicornis bicollis and blind-ending hemivagina was noted at hysterosalpingography.Interventions:This necessitated the search for other genitourinary tract anomalies,and ipsilateral renal agenesis was also discovered.Outcomes:A diagnosis of Herlyn-Werner-Wunderlich syndrome was made.Lessons:Magnetic resonance imaging(MRI)is considered the gold standard imaging modality for the assessment of uterine malformations.However,in resource-poor centers where MRI is unavailable,a heightened clinical suspicion with the combination of available imaging modalities is important and reliable in making an accurate diagnosis,hence,successful management.
文摘Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital anomaly characterized by <span style="font-family:Verdana;">triad</span><span style="font-family:Verdana;"> of uterus didelphys, obstructed </span><span style="font-family:Verdana;">hemi</span><span style="font-family:Verdana;"> vagina, and ipsilateral renal agenesis. The most common presentation is abdominal pain, dysmenorrhea, and abdominal mass secondary to </span><span style="font-family:Verdana;">hemi</span><span style="font-family:Verdana;">-</span><span style="font-family:Verdana;">hematometrocolpos</span><span style="font-family:Verdana;">. We report an emergency presentation of this syndrome during the pandemic of COVID-19 in Qatif Central Hospital, Saudi Arabia, April 2020. An 11-year-old Saudi girl presented to the emergency room with three months history of cyclical lower abdominal pain. The pain was progressive, continuous </span><span style="font-family:Verdana;">and</span><span style="font-family:Verdana;"> not relieved by analgesics. Abdominal examination revealed a tender abdominal mass mainly in the left iliac fossa, Ultrasound evaluation showed two uterine bodies. The left uterus was distended with complex fluid. Pelvic MRI findings consistent with Herlyn-Werner-Wunderlich (HWW) syndrome were found with uterine </span><span style="font-family:Verdana;">didelphys</span><span style="font-family:Verdana;"> and </span><span style="font-family:Verdana;">left sided</span><span style="font-family:Verdana;"> hematometra resulting from obstructed </span><span style="font-family:Verdana;">hemi</span><span style="font-family:Verdana;">-vagina and ipsilateral agenesis of the left kidney. Resection of the vaginal septum and drainage of hematometra was done. The patient recovered with normal cyclical menstruation. Cyclical or continuous lower abdominal pain with or without amenorrhea is the usual presentation of HWW syndrome during adolescence. Diagnosis is made by ultrasonography and MRI. Early diagnosis and accurate management can provide pain relief, prevent future complications, and preserve fertility.</span>
