A rare congenital anomaly in a female child with acute urinary retention—female hypospadias: a case report

Abstract

Female hypospadias is a rare congenital anomaly and defined as a female urethral opening on the anterior vaginal wall. Diagnosis is challenging due to its rarity. Different anatomical variations exist depending on the location of the urethral opening. Clinical presentation can vary from asymptomatic until end-stage renal disease due to lower urinary tract obstruction. This case highlights the important clinical and radiological features of female hypospadias. Surgical treatment remains the mainstay management for this entity. We report a case of a 5-year-old girl presented with 3 days of lower abdominal pain, vomiting and difficulty in voiding. She was previously well. Abdominal examination revealed a palpable suprapubic mass arising from the pelvis up to the umbilicus. Perineal examination showed a single opening at the introitus. Ultrasound showed a grossly distended urinary bladder and bilateral mild hydroureteronephrosis. Catheterization of the opening failed to decompress the bladder, and a suprapubic catheter was inserted which drained 500 cc of clear urine. She also developed acute kidney injury which resolved after suprapubic drainage. Cystogram and genitogram failed to delineate the communication between the urinary bladder and vagina. However, cystovaginoscopy revealed a normal vagina opening, and the urethral meatus entered the anterior wall of the vagina distally. She underwent a meatoplasty and recovered well after surgery. Attention to important clinical and radiological features helps in the diagnosis of female hypospadias. Female hypospadias should be suspected in female patient with single opening at the introitus, acute urinary retention and failed urinary catheterization. Cystovaginoscopic examination is required to diagnose this condition. Urethroplasty is curative with good outcome for low type of female hypospadias.