Agenesis of Urinary Bladder with Solitary Kidney, Ectopic Ureter, and Unicornuate Uterus in an Adult Patient

A 20-year-old female presented to the Urology Department at Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER) hospital with persistent urine leak since birth. She had no history of normal voiding of urine and had no recurrent urinary tract infection (UTI). Developmentally she was normal. She had abnormal gait with inability to flex right hip and knee joint. On examination, she had separate urethral and vaginal openings appearing stenosed with labial adhesions. Dribbling of urine was noted from the urethral opening and not vaginal opening.

Ultrasound of abdomen and pelvis performed with Esaote, My Laboratory 60, Genoa, Italy showed left solitary kidney without dilatation of the pelvicalyceal system or ureter in normal location with nonvisualization of urinary bladder. Contrast-enhanced computed tomography (CT) abdomen was performed on a 64 slice CT scanner (Somatom Sensation, Siemens, Erlangen, Germany). Plain imaging was performed from domes of diaphragm to proximal thigh, followed by corticomedullary phase (30-second delay), nephrographic phase (100-second delay), and excretory phase (10-minute delay) after the administration of contrast medium (iohexol 300 mg/mL iodine concentration—Omnipaque, GE Healthcare, Marlborough, MA, United States), through a 18 G catheter secured in antecubital vein, at a dose of 1.5 mL/kg (80 mL), rate of 3.5 mL/s. Saline flush was done with 20 mL of normal saline following contrast at same flow rate. Multiplanar reformation and volume rendering of the images were performed as needed in excretory phase. Imaging showed absent right kidney with compensatory hypertrophy of the left kidney without hydronephrosis. Urinary bladder was absent and also distal left ureter was dilated ([Fig. 1A]). The ureter was seen ectopically opening and delayed imaging in excretory phase showed contrast extravasation anterior to the vaginal wall ([Fig. 1B]). Other anomalies noted include scoliosis, dysplasia of right hip ([Fig. 2]), unicornuate uterus.

Fig. 1 Contrast-enhanced computed tomography abdomen in excretory phase showing absent urinary bladder and dilated distal left ureter (white arrow in A), ectopic location of distal end of left ureter (star in A). Thin streak of contrast is seen extravasating through the vulva (black arrow in B).

Fig. 2 Contrast-enhanced computed tomography abdomen showing dysplasia of right hip (star), solitary left kidney (black arrow), and bowel loops occupying the right renal area (white arrow).

Magnetic resonance imaging (MRI) was performed on 1.5 Tesla scanner (Magnetom Avanto, Siemens, Erlangen, Germany). T2-weighted sagittal, axial images, T1-weighted axial, short tau inversion recovery coronal of abdomen and pelvis, heavily T2 weighted MR urography coronal and maximum intensity projection to delineate the course of ureter were acquired. MRI confirmed absent urinary bladder with ectopic ureteric insertion, left solitary kidney, and unicornuate uterus ([Fig. 3]). Both the ovaries were normal showing follicles. Cystoscopy showed left ureter opening 1 cm proximal to external urethral meatus with no urinary bladder in between.

Fig. 3 Magnetic resonance imaging short tau inversion recovery coronal image showing unicornuate uterus (white arrow). Right hip dysplasia (star).

She underwent cut down vaginoplasty with adhesiolysis and laparoscopic Mainz 2 urinary pouch, which is a continent urinary pouch. She was discharged and advised to follow up after 1 month. No significant complications observed at 1-month follow-up imaging that showed functioning left kidney with ureter draining into the surgical pouch ([Fig. 4]).

Fig. 4 Volume rendered image of follow-up computed tomography in delayed phase showing functioning left kidney (short arrow) with ureter draining into the surgical pouch (long arrow).

• References

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