Female hypospadias leading to delayed end stage renal disease - prenatal and neonatal correlation

Case contributed by Fabien Ho
Diagnosis certain


Isolated urinary anomaly detected by antenatal ultrasound. Lack of neonatal follow-up from day 3 until 5 months, when presented with hemodynamic shock and hyperkalemia complicated by cardiac arrest and requiring emergency hemodialysis. At ER, parents reported that urination had been impossible for 3days.

Patient Data

Age: Embryo to fetus to 5 months of age (delivery at term)
Gender: Female

Prenatal US from 11 to 35 wk gestational age

Age of gestation (AG) is shown on the top left corner.

At 11 weeks gestation (WG), kidneys and bladder were normal.
At 17 WG, we found a mild bilateral pyelic dilation, with normal kidneys parenchymas, ureters, and bladder. There were no associated malformation or aneuploidy signs.
Upon follow-up, the mild dilation remained stable at 20, 25, 28 and 32 WG. Bowel, anal muscles, spine vertebra, and cord were normal. Amniocentesis was proposed, but not performed.
At 35 WG, the left pyelic dilation worsened, reaching 15 mm versus 10 mm on the right side.

US performed at day of life 3 showed right hydroureteronephrosis, and mild pyelic dilation on the left side. Bladder was full, and ureteral urine stream was visible on color Doppler.

Postnatal Uro-MRI at 5 mth while in ICU for renal failure

MRI revealed bilateral megaureters with renal parenchymal thinning.  No other associated genital or neurologic anomaly.

Anterograde cystography through suprapubic catheter lower urinary tract catherization failure

Bladder catheterization was impossible, hence the pediatric surgeon had to use a suprapubic cathether. Anterograde cystography through a suprapubic catheter revealed an urethral abnormal tract leading to vaginal opacification.

Case Discussion

Clinical examination revealed an abnormal urethral meatus on the anterior vaginal wall, consistent with female hypospadias.

This case demonstrates the classic delayed clinical course of female hypospadias leading to obstructive renal failure. The prenatal findings of renal pelviectasis are mild and nonspecific, and yet the later presentation was dramatic and critical.

Female hypospadias, defined as a female urethra opening on the anterior vaginal wall, is a sneaky congenital urological condition. It is hard to diagnose clinically, and frequently asymptomatic until end-stage renal disease (ESRD) due to lower urological tract obstruction. There have been case reports of female hypospadias diagnosed in teenagers at ESRD.

To our knowledge, this is the first report of prenatal and postnatal correlation.

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