Diagnosis and Treatment in patient with Herlyn-Werner-Wunderlich Syndrome: A case causing pelvic pain


Norman Aji Triantoro(1), Nuring Pangastuti(2*)

(1) Departemen Obstetri dan Ginekologi, Fakultas Kedokteran, Kesehatan Masyarakat dan Keperawatan (FKKMK) UGM
(2) Departemen Obstetri dan Ginekologi, Fakultas Kedokteran, Kesehatan Masyarakat dan Keperawatan (FKKMK) UGM
(*) Corresponding Author


Background: Herlyn-Werner-Wunderlich (HWW) syndrome is a rare variant of Mullerian duct anomalies. The characteristic triad of this syndrome includes didelphys uterus, obstructed hemivagina, and ipsilateral renal agenesis, recently known as Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) syndrome. The most common presentation is abdominal and pelvic pain, dysmenorrhea, and abdominal mass secondary to hematocolpos, hematosalping or hematometra.

Objective: to determine the diagnosis and operative management of HWW syndrome case with pelvic pain as the chief complaint. Method: case examination and tracing medical records at Dr Sardjito Hospital Yogyakarta.

Results and Discussion: Fourteen-year-old female, presented with pelvic pain. Physical examination revealed a cystic mass in the abdomen inferior, palpable up to 3 cm inferior to the umbilicus. From the Intravenous Pyelography (IVP) examination, it was found normal anatomy and function of the right kidney, but there was no left kidney. On a contrast-enhanced abdominal CT scan, magnetic resonance imaging (MRI) and laparoscopy showing a complete duplication of the uterus from the horn to the cervix with no connection between the two uterine cavities, both ovaries were normal, the right fallopian tube was normal, the left tube was enlarged, attached to the uterus and the left ovary, no left kidney was found. Operative management of the vaginal septectomy procedure was performed.

Conclusion: MRI is most accurate for providing details regarding the altered anatomy and for identifying associated hematocolpos,hematosalping or hematometra for HWW syndrome cases. Surgical intervention by vaginal septectomy is performed to relieve symptoms, provide better reproductive and sexual functions.
Keywords: Herlyn-Werner-Wunderlich syndrome; Mullerian disgenesis; pelvic pain.


Herlyn-Werner-Wunderlich syndrome; Mullerian disgenesis; pelvic pain

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DOI: https://doi.org/10.22146/jkr.64831

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