Rokitansky Syndrome: Clinical Experience and Results of Sigmoid Vaginoplasty in 23 Young Girls

Abstract

The Mayer-Rokitansky-Kuster-Hauser syndrome (Rokitansky syndrome) is a frequently misdiagnosed congenital anomaly of the female genital tract. Of several surgical treatments sigmoid vaginoplasty is among the few that provide a functional self-lubricating neovagina. We evaluated the results of sigmoid neovagina in girls affected by the Rokitansky syndrome. We followed 26 patients with the Rokitansky syndrome between 1990 and 2005. Diagnosis was based on clinical examination, normal ovarian hormones and pelvic ultrasound or magnetic resonance imaging. Associated anomalies were detailed. Vaginoplasty was performed in 23 patients. Functional results and complications were assessed. Renal anomalies were found in 11 patients (42%) and skeletal anomalies in 6 (23%). Six girls (23%) had a family history of the Rokitansky syndrome and/or renal agenesis. Vaginoplasty was performed at a mean age of 16 years (range 10.3 to 18.8). Median postoperative followup was 3.4 years. Postoperative complications included lower extremity compartment syndrome (1 patient), pelvic hematoma (1), mucosal prolapse (2), cystitis (2) and introital stenosis (1). Of the 23 patients undergoing surgery 9 (39%) had an active sex life postoperatively. Sigmoid vaginoplasty is a valuable procedure in girls with the Rokitansky syndrome. We recommend reconstruction during adolescence because the local conditions are excellent and it allows adaptation of the anatomy to physical development.