TITLE:
Herlyn-Werner-Wunderlich Syndrome Presenting with Abdominal Pain: A Case Report
AUTHORS:
Hayat Al-Jumah, Rashida Sadiq, Noor Al-Muslem, Fathia E. Al-Jama, Raghad Aljishi, Salah Abohelaika
KEYWORDS:
Herlyn-Werner-Wunderlich Syndrome, Obstructed Hemi-Vagina, Hematometrocolpos, Mullerian Anomaly, Vaginoplasty
JOURNAL NAME:
Open Journal of Obstetrics and Gynecology,
Vol.11 No.7,
July
23,
2021
ABSTRACT: Herlyn-Werner-Wunderlich
(HWW) syndrome is a rare congenital anomaly characterized by triad of uterus didelphys, obstructed hemi vagina, and ipsilateral renal agenesis.
The most common presentation is abdominal pain, dysmenorrhea, and abdominal
mass secondary to hemi-hematometrocolpos. 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 and 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 didelphys and left sided hematometra resulting from obstructed hemi-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.