Its a condition where uterus, vagina and kidney developments are at fault congenitally.

1. Dr. Chaduvula Suresh Babu

2.  What is Herlyn-Werner-Wunderlich
syndrome (HWW)?
 It is a rare congenital disorder characterized
by uterus didelphys, obstructed hemivagina,
and ipsilateral renal agenesis.

3.  Case Details
 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.

4.  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.

5.  Ultrasound evaluation showed a distended
endometrial cavity filled with complex fluid with
low-level internal echoes and nonvisualization of the
right kidney.
 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).

6.  Based on the observations, a provisional
diagnosis of uterus didelphys, hematometra,
hematocolpos, and agenesis of the right
kidney was made.

7.  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.

8. Thank You