CASE REPORT Blind hemibladder, ectopic ureterocele, or Gartner’ s duct cyst in a woman with Müllerian malformation and supposed unilateral renal agenesis: a case report Pedro Acién & Maribel Acién & Jesús Romero-Maroto Received: 3 March 2009 / Accepted: 21 June 2009 / Published online: 14 July 2009 # The International Urogynecological Association 2009 Abstract Genital anomalies associated with unilateral renal agenesis are generally due to agenesis or hypoplasia of the entire urogenital ridge or distal mesonephric aberrations. However, renal adysplasia could also occur in association with anomalies of the ventral urogenital sinus. The patient presented didelphys uterus in the superior uterine segment, a septate cervix, and a simple vagina. After transvaginal puncture and injection of a contrast agent into the bulge observed in the right vaginal wall, a filled sac or cavity was detected, possibly a hemibladder. This structure continued upward with a possible dilated tortuous ureter that filled retrogradely. Magnetic resonance imaging also showed the presence of the right blind paravaginal sac. Right hemi- trigone and ureteral orifice were absent in the cystourethro- scopy. No right kidney was found, despite the use of multiple imaging techniques. Blind hemibladder, ectopic ureterocele, and Gartner ’ s duct cyst seem to be a possible diagnosis associated to Müllerian malformations and supposed unilateral renal agenesis. Therefore, Müllerian anomalies without combined mesonephric alteration could be associated with conditions of the ventral urogenital sinus, including blind hemibladder or ectopic ureterocele with secondary renal dysplasia. Keywords Unilateral renal agenesis . Female genital malformations . Müllerian anomalies . Ectopic ureterocele . Hemibladder . Gartner ’ s duct cysts Introduction According to previous studies [1, 2], unilateral renal agenesis is embryologically associated with genital, and sometimes extragenital, malformations. The associated genital malformations are due to agenesis of all derivatives of the ipsilateral urogenital ridge (with a unicornuate uterus on the other side) or distal mesonephric anomalies. An alternative embryologic sequence resulting in unilateral renal agenesis without Müllerian anomalies could be an involution or secondary renal atrophy. In other cases presenting with Müllerian anomalies but probably without mesonephric aberrations, there can also be atrophy or severe secondary renal adysplasia when the Müllerian anomaly is associated with anomalies of the cloaca/ urogenital sinus. Table 1 shows the adult derivatives and male and female abnormalities of the embryonic urogenital structures according to our observations [1, 2]. This case was a genital malformation seen as an isolated Müllerian anomaly (bicornuate uterus, or didelphys in the superior uterine segment, with septate cervix and simplex vagina) that also showed what we interpreted as a blind right P. Acién : M. Acién Service of Obstetrics and Gynecology, University Hospital of San Juan, Alicante, Spain P. Acién (*) : M. Acién Department/Division of Gynecology, Facultad de Medicina de la Universidad ‘Miguel Hernández’, Campus de San Juan, 03550 Alicante, Spain e-mail: acien@umh.es J. Romero-Maroto Service of Urology, University Hospital of San Juan, Alicante, Spain J. Romero-Maroto Department of Surgery/Division of Urology, Facultad de Medicina de la Universidad ‘Miguel Hernández’, Campus de San Juan, 03550 Alicante, Spain Int Urogynecol J (2010) 21:365–369 DOI 10.1007/s00192-009-0952-4