Researchers reported a rare case of Herlyn-Werner- Wunderlich syndrome presenting with dysmenorrhea.
What is Herlyn-Werner-Wunderlich syndrome (HWW)?
It is a rare congenital disorder characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis.
A 15-year-old girl presented to the ED with complaints of increasing pain in the right lower abdomen for three months. The patient experienced severe, colicky pain in the right lower abdomen with the onset of menstruation.
She had no history of any medical condition. She had menarche three months earlier and had a regular menstrual cycle with dysmenorrhea and cyclical abdominal pain. For dysmenorrhea, she was prescribed analgesics from a local pharmacy, which resulted in transient improvement of the symptoms.
She was born at the term of an uncomplicated pregnancy and had no family history of any congenital diseases.
The patient was not sexually active and did not take contraceptive pills or hormone therapy.
While admission, she was afebrile, and her vital signs were stable except for mild anemia.
An abdominal examination found a tender mass on the right iliac fossa, and Per rectal examination revealed a mass in the pouch of Douglas. She was admitted to the gynecology department, where she was given drugs like omeprazole and paracetamol for relief of symptoms until an MRI scan was obtained, and corrective surgery was planned.
Ultrasound evaluation showed a distended endometrial cavity filled with complex fluid with low-level internal echoes and nonvisualization of the right kidney.
Based on the observations, a provisional diagnosis of uterus didelphys, hematometra, hematocolpos, and agenesis of the right kidney was made.
Pelvic MRI showed two separate endometrial stripes surrounded by endometrium and muscular layer. The right endometrial cavity and cervix were distended with blood, possibly owing to obstructed right hemivagina. The right kidney was absent.
An MRI finding was suggestive of uterine didelphys with right-sided hematometra resulting from obstructed hemivagina with ipsilateral agenesis of the right kidney (HWW syndrome).
IVU revealed an absent or nonexcreting right kidney and normal excreting left kidney. Identification and resection of the vaginal septum were done and reached up to the right cervix for the drainage of tarry blood.
Thus the vaginal canal was reconstructed. There were no perioperative or postoperative complications. She was discharged five days after surgery.
The patient first came for a follow-up of 7 days after discharge. She had no health issues or complaints. Vaginal examination revealed a healthy wound with no adhesion of the vaginal wall. Thus, her recovery was uneventful.
Later, the patient informed that she had started regular menstruation without any pain 30 days after the surgery. Later, during follow-up visits, she confirmed that she had no dysmenorrhea or any other complaints.
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