Laparoscopic findings and pelvic anatomy in Mayer-Rokitansky-Küster-Hauser syndrome

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.