Congenital vesicovaginal fistula with or without menouria: a literature review.

Congenital vesicovaginal fistula (CVVF) is a rare, complex female genital malformation that is difficult to diagnose, classify and treat. Symptoms include menouria, cyclical haematuria and urinary incontinence from birth. The aim of this review was to highlight the importance of correct diagnosis and treatment of this congenital anomaly. A comprehensive literature review was conducted to identify articles on CVVF with and without menouria. Forty-one articles were found, of which 31 described 37 original cases of congenital fistula (30 CVVF, six uterovesical fistula and one urethrovaginal fistula): 14 in girls and 23 in adults. The cases were classified according to clinical, diagnostic and therapeutic parameters in order to unify concepts and terms. Menouria occurred in 21 of the 23 adults: 14 cases were CVVF, six cases were congenital uterovesical fistula, and one case was congenital urethrovaginal fistula in a young woman without congenital adrenal hyperplasia. Sixteen (53%) of the 30 patients with CVVF had urinary incontinence or hydrocolpometra, and 14 (47%) had menouria. There were associated anomalies in 91% of cases, 29 (48%) of which involved the urinary tract. Various diagnostic and therapeutic methods were used, and there was confusion surrounding the aetiopathogenesis in most cases. CVVF should be suspected in any girl with urinary incontinence, urinary tract infections from birth, vaginal swelling or hydrocolpometras, as well as in adults with menouria. Diagnosis should be based on physical examination and imaging (cystoscopy during menouria, ultrasound and magnetic resonance imaging). Surgical treatment should be based on correction of the vaginal defects (obstructive problem opening or vaginal reconstruction) and CVVF closure. The embryological origin of CVVF lies in the abnormal persistence of the urogenital sinus due to lack of formation and caudal growth of the urogenital wedge, combined with distinct degrees of agenesis or hypoplasia of the entire urogenital ridge or the mesonephric ducts (which affects development of the Müllerian ducts).