We describe a 3-week-old girl with a genital prolapse. She was seen postnatally because a prenatal ultrasound had shown a cystic dilation in the right kidney. Ultrasound showed two duplex kidneys, with hydronephrosis of the upper pole of the right kidney and a ureterocele in the bladder. Antibiotic prophylaxis was started while further investigations were awaited. At 3 weeks of age the patient presented to the emergency department with fever. Physical examination showed a moderately ill girl, with no obvious signs to explain the origin of the fever. Genital examination showed a blue cystic mass extruding from the vulva, most likely a ureterocele (figure 1). Urine culture was positive for Klebsiella oxytoca. Dimercaptosuccinic acid-scintigraphy showed an almost non-functioning upper pole of the right kidney. The patient was treated with intravenous antibiotics and made a good recovery. Voiding cystogram showed right-sided vesicoureteral reflux grade one to the lower pole.
The girl was referred to a paediatric urologist for incision of the ureterocele. At the age of 12 months a resection of the right upper pole will be performed.
A ureterocele is a dilation of the distal ureter that is located within the bladder; if a portion is located at the bladder neck or urethra, it is termed ectopic or a cecoureterocele. Ureteroceles have an incidence of 1:500–1:4000.1 2 However, a ureterocele presenting as a vulval mass is rather rare and occurs in only 5–10% of all ureteroceles.1 3 When found, it is advisable to rule out other anomalies, such as duplex kidneys, since they frequently (80–90%) coincide.1 2