Herlyn-Werner-Wunderlich syndrome: a case report
Session
Medicine and Nursing
Description
Herlyn-Werner-Wunderlich Syndrome is a congenital anomaly of mullerian ducts, characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis which can lead to progressive dysmenorrhea in post menarche, hematocolpos or hematometra.
The underlying cause behind this syndrome is not properly understood yet but it’s associated with impaired development of mesonephric and paramesonephric ducts also known as Wolffian and Mullerian ducts respectively. It has been observed that anomalous development of the caudal end of unilateral Wolffian duct is associated with same sided imperforated hemivagina and absence of kidney. Also lateral deviation of the Mullerian duct of the same side results in failure of fusion of the bilateral Mullerian ducts which leads to uterus dildelphys. Moreover, deviated paramesonephric duct cannot interact with the urogenital sinus properly, so instead of giving rise to a vagina, a blind pouch is developed forming obstructed hemivagina.
It is also called as OHVIRA which is an acronym that refers for obstructed hemivagina and ipsilateral renal anomaly. The true incidence ranges from 0.1-3%.
In this report we describe the clinical presentation and successful surgical management of a 17-year-old girl who presented with abdominal pain and painful menstruation. Further evaluation with pelvic ultrasound revealed 3 cystic formation. The diagnosis of Herlyn-Werner-Wunderlich Syndrome is confirmed by Pelvic MRI which showed hematocolpos, endometrioma, hematosalpinx and agenesis of right kidney in upper abdomen. Surgical management was done by iatrogenic defloration, vaginal septum incision, and Stressman metroplasty. The perioperative and postoperative periods went uneventful, and the patient recoverd without any significant complication. The cytological sample revealed absence of any malignant cells. The patient left the hospital with detailed instructions related to post-discharge care. The patient became asymptomatic with preservation of fertility.
Presentation of regular menstruation and non-specific abdominal pain causes misdiagnosis, so awareness is necessary to treat this disorder to prevent complications such as endometriosis and infertility.
Proceedings Editor
Edmond Hajrizi
ISBN
978-9951-550-50-5
Location
UBT Kampus, Lipjan
Start Date
29-10-2022 12:00 AM
End Date
30-10-2022 12:00 AM
DOI
10.33107/ubt-ic.2022.165
Recommended Citation
Ahmetgjekaj, Ilir; Kutllovci, Arben; and Bajraktari, Edita, "Herlyn-Werner-Wunderlich syndrome: a case report" (2022). UBT International Conference. 167.
https://knowledgecenter.ubt-uni.net/conference/2022/all-events/167
Herlyn-Werner-Wunderlich syndrome: a case report
UBT Kampus, Lipjan
Herlyn-Werner-Wunderlich Syndrome is a congenital anomaly of mullerian ducts, characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis which can lead to progressive dysmenorrhea in post menarche, hematocolpos or hematometra.
The underlying cause behind this syndrome is not properly understood yet but it’s associated with impaired development of mesonephric and paramesonephric ducts also known as Wolffian and Mullerian ducts respectively. It has been observed that anomalous development of the caudal end of unilateral Wolffian duct is associated with same sided imperforated hemivagina and absence of kidney. Also lateral deviation of the Mullerian duct of the same side results in failure of fusion of the bilateral Mullerian ducts which leads to uterus dildelphys. Moreover, deviated paramesonephric duct cannot interact with the urogenital sinus properly, so instead of giving rise to a vagina, a blind pouch is developed forming obstructed hemivagina.
It is also called as OHVIRA which is an acronym that refers for obstructed hemivagina and ipsilateral renal anomaly. The true incidence ranges from 0.1-3%.
In this report we describe the clinical presentation and successful surgical management of a 17-year-old girl who presented with abdominal pain and painful menstruation. Further evaluation with pelvic ultrasound revealed 3 cystic formation. The diagnosis of Herlyn-Werner-Wunderlich Syndrome is confirmed by Pelvic MRI which showed hematocolpos, endometrioma, hematosalpinx and agenesis of right kidney in upper abdomen. Surgical management was done by iatrogenic defloration, vaginal septum incision, and Stressman metroplasty. The perioperative and postoperative periods went uneventful, and the patient recoverd without any significant complication. The cytological sample revealed absence of any malignant cells. The patient left the hospital with detailed instructions related to post-discharge care. The patient became asymptomatic with preservation of fertility.
Presentation of regular menstruation and non-specific abdominal pain causes misdiagnosis, so awareness is necessary to treat this disorder to prevent complications such as endometriosis and infertility.