Herlyn-Weber-Wunderlich syndrome with ectopic ureter in prepubertal female
The present case report is about a 10-year-old prepubertal female, presented with complaints of vague abdominal pain and dysuria. On examination, there was a well-defined mass in the suprapubic region. The mass was tender to deep palpation, firm cystic in nature, had restricted mobility and its lower limit could not be reached. On examination of the external genitalia, there was a vulvar mass with a normal vaginal orifice and urethral orifice []. The vulvar mass increased in size on suprapubic pressure on the abdominal mass. On digital rectal examination, a cystic mass was palpable anteriorly. Ultrasound abdomen showed left renal duplication with hematocolpos. There was no mention of any uterine anomaly. To delineate the anatomy, a magnetic resonance imaging (MRI) was done. It showed the presence of uterine didelphys with hematocolpos (8.4 cm × 7.4 cm), with an ectopic left kidney and its ureter opening in the obstructed hemi vagina []. A Di-mercaptosuccinic acid scan showed poorly functioning left kidney with a differential renal function of 2% and a normal right kidney. A diagnosis of obstructed hemivagina with ipsilateral renal anomaly was made. On cystoscopy, there was a single ureteric orifice on the right side with a hemitrigone. The trigone on the left was not developed. A needle aspiration of the vulvar orifice showed thick hemorrhagic fluid []. The vaginal septum was divided and septoplasty was done to create a single vaginal orifice. A diagnostic laparoscopy was performed to confirm the anatomy described by MRI and to tackle the ureteric and renal remnant. This revealed uterine didelphys with normal ovary and fallopian tubes with left uterine horn dilated when compared with right. The ureter and ectopic kidney were not visualized. We explored the abdomen and found the ectopically inserting ureter in the obstructed hemi-vagina. The ectopic ureter (ER) continued blindly, superiorly, up to 8 cm from the pelvic brim, in a fibrotic renal remnant 1.4 cm in size []. The lower end was dissected and ligated at its entry point in the hemivagina. The anatomical details have been represented as a line diagram in . On histopathological examination, there was an oligonephric renal remnant with ureteric tissue. The patient on follow-up had no vaginal stenosis. An ultrasound abdomen done 2 months post-operatively showed normal hemiuterus.