Unusual variant of inverted Y ureteral duplication with an ipsilateral seminal vesicle cyst and renal dysgenesis

Abstract

Inverted Y malformation is a rare variant of ureteral duplication with a marked female predominance. We describe a case of inverted Y ureteral duplication with concurrent ectopic ureteral insertion into a seminal vesicle cyst, a ureterocoele and renal dysgenesis, which occurred in a 29-year-old man with lower urinary tract symptoms, haematospermia and postcoital discomfort. To our knowledge, this is the first reported case with this constellation of urogenital abnormalities. Case historyA 29-old-year man presented with urgency, dysuria, haematospermia and postcoital discomfort of 2 months' duration with left abdominal pain. There was no history of fever or haematuria. His medical history included a left renal agenesis and treated bilateral cryptorchidism. Physical examination revealed a palpable, soft and tender intra-abdominal lump occupying the left lumbar region. Genitourinary examination was unremarkable. Laboratory tests were within normal limits. Abdominal ultrasonography demonstrated a left retroperitoneal cystic mass with a hypoechoic intravesical lesion. Computed tomography (CT) showed a cyst measuring 70mm × 66mm × 57mm, expanding from the left renal fossa to a large filling defect in the left bladder base (Fig 1). A ureterocoele with a hydronephrotic kidney was suspected. A cystic lesion of the ipsilateral seminal vesicle was also noted on CT.On cystoscopy, there was severe elevation of the left hemitrigone with bladder neck obstruction, raising the suspicion of a ureterocoele. The ipsilateral ureteric orifice was not visualised. Magnetic resonance imaging (MRI) demonstrated a seminal vesicle cyst (SVC), which measured 30mm × 18mm × 15mm, and a ureter-like tube expanding from the lower ureter and opening into the cyst (Fig 2).In order to investigate further, transrectal ultrasonography guided puncture of the ureterocoele was performed with aspiration of 260ml of chocolate coloured fluid and injection of contrast inside (Fig 3). Fluoroscopy and x-ray showed opacification of the left vas deferens (Fig 4). The aspirated chocolate coloured fluid contained spermatozoa.A diagnosis of an inverted Y ureter with a stenotic ureterocoele and ureteral ectopia into a SVC was considered. The patient underwent an open midline suprapubic, extraperitoneal approach. The specimen was dissected extravesically and resected completely (Fig 5). Microscopically, the ureterlike tube was lined by urothelial epithelium. There were glomeruli and renal tubules with fetal characteristics in the wall of the cystic kidney, suggesting renal dysgenesis. During the six months following resection, he experienced neither haematospermia nor coital discomfort and denied any voiding difficulties.

Diagnosis and surgical outcomes of ectopic ureters in adults: A case series and literature review

“…Ectopic ureters are a rare diagnosis in adults, and symptoms are often nonspecific. As such, diagnosis remains a significant challenge for clinicians, requiring a combination of detailed history and clinical examination, contrast‐enhanced cross sectional imaging and cystoscopy of the urinary tract …”

“…a combination of detailed history and clinical examination, contrast-enhanced cross sectional imaging and cystoscopy of the urinary tract. [4][5][6] The published literature focuses primarily on surgical techniques, with limited data on presentation, diagnostics and functional outcomes. We describe a series of 10 adult patients referred to a tertiary unit for urinary incontinence with subsequent assessment confirming an ectopic ureter.…”