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Hum Reprod. 2013 Jun;28(6):1580-3. doi: 10.1093/humrep/det081. Epub 2013 Mar 26.

Double uterus with obstructed hemivagina and ipsilateral renal agenesis: pelvic anatomic variants in 87 cases.

Author information

1
Department of Obstetrics and Gynecology, Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, University of Milan, Milan 20122, Italy.

Abstract

STUDY QUESTION:

What are the anatomic variants (and their frequencies) of double uterus, obstructed hemivagina and ipsilateral renal agenesis?

SUMMARY ANSWER:

Most cases examined (72.4%) were of the classic anatomic variant of the Herlyn-Werner-Wunderlich syndrome (with didelphys uterus, obstructed hemivagina and ipsilateral renal agenesis) but the 27.6% of cases are of a rare variant of the syndrome (with uterus septum or cervical agenesis), showing relevant clinical and surgical implications.

WHAT IS KNOWN ALREADY:

The extreme variability of anatomic structures involved in this syndrome (both uterus, cervico-vaginal and renal anomalies) is well known, even if a complete and uniform analysis of all its heterogeneous presentations in a large series is lacking.

STUDY DESIGN, SIZE, DURATION:

This is a retrospective study with 87 patients referred to our third level referral center between 1981 and 2011.

PARTICIPANTS/MATERIALS, SETTING, METHODS:

We analyzed the laparoscopic and chart records of 87 women, who referred to our institute with double uterus, unilateral cervico-vaginal obstruction and ipsilateral renal anomalies.

MAIN RESULTS:

Sixty-three of 87 patients had the more classic variant of didelphys uterus with obstructed hemivagina; 10/87 patients had septate bicollis uterus with obstructed hemivagina; 9/87 patients had bicornuate bicollis uterus with obstructed hemivagina; 4/87 patients had didelphys uterus with unilateral cervical atresia; 1/87 patients had bicornuate uterus with one septate cervix and unilateral obstructed hemivagina.

LIMITATIONS:

This is a retrospective study with a long enrolling period (30 years).

WIDER IMPLICATIONS OF THE FINDINGS:

New insights in the anatomic variants of this rare syndrome with their relevant surgical implications.

KEYWORDS:

Herlyn-Werner-Wunderlich syndrome; cervical agenesis; didelphys; double uterus; obstructed hemivagina

PMID:
23532323
DOI:
10.1093/humrep/det081
[Indexed for MEDLINE]

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