Laparoscopic Findings and Pelvic Anatomy in Mayer-Rokitansky-Küster-Hauser Syndrome

Fedele, Luigi MD1; Bianchi, Stefano MD1; Frontino, Giada MD1; Ciappina, Nevio MD1; Fontana, Eleonora MD1; Borruto, Franco MD2

Obstetrics & Gynecology:
doi: 10.1097/01.AOG.0000260872.28368.46
Original Research
Abstract

OBJECTIVE: To describe analytically the anatomic variety and laparoscopic findings observed in patients with Rokitansky syndrome throughout an 11-year span.

METHODS: We analyzed the laparoscopic and chart records of 106 consecutive patients who underwent surgery for the creation of a neovagina, according to the modified laparoscopic Vecchietti procedure.

RESULTS: A hypoplastic vagina was observed in 61 women. Müllerian remnants, laterally displaced in the pelvis, were identified in 92 cases; 42 (25.9%, 95% confidence interval [CI] 19.2–33.3) of 162 müllerian remnants were cavitated and contained endometrial mucosa. Ovaries were extrapelvic in 17 (16.0%, 95% CI 9.6–24.4) cases. Anomalies of the urinary tract were identified in 32 (30.2%, 95% CI 21.7–39.9) patients; unilateral renal agenesis was the most frequent finding (18 cases; 18%, 95%CI 10.4–25.5).

CONCLUSION: Rokitansky syndrome has a wide variability of anatomic presentations, and, as the do other congenital anomalies of the female genital tract, it represents a continuum of embryonic malformations, which occur at different stages of development.

LEVEL OF EVIDENCE: III

In Brief

In Rokitansky syndrome there is a wide variability of pelvic anatomy representing a continuum of embryonic malformations, which occur at different stages of development.

Author Information

From the 1Department of Obstetrics, Gynecology and Neonatology, Fondazione “Policlinico-Mangiagalli-Regina Elena,” University of Milano, Milan, Italy; and 2Department of Obstetrics and Gynecology, University of Verona, Ospedale Borgoroma, Verona, Italy.

Corresponding author: Luigi Fedele, MD, Clinica Ostetrica e Ginecologica II, Università di Milano, Istituto Luigi Mangiagalli, Via della Commenda 12, 20122 Milano, Italy; e-mail: luigi.fedele@unimi.it.

© 2007 by The American College of Obstetricians and Gynecologists. Published by Wolters Kluwer Health, Inc. All rights reserved.