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 Rokitansky syndrome there is a wide variability of pelvic anatomy representing a continuum of embryonic malformations, which occur at different stages of development.
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: firstname.lastname@example.org.