Congenital anterior urethral valves and diverticulum: A case series and association with posterior urethral valves

Abstract

There is limited data regarding long-term results and associated complications in patients with anterior urethral valve (AUV) and diverticulum (AUD). We retrospectively reviewed AUV/AUD cases managed by us between the year 2002-2020. Presentation, investigations, management, concomitant posterior urethral valves (PUV) and pre-operative characteristics predisposing to long-term poor renal outcome were assessed. There were 27 patients [AUV (n=11); AUD (n=16)] with 5 having concomitant PUV. All presented with poor urinary stream and dribbling at a median age of 1-year (5 days-12 years). More patients with AUD (9 of 16, 56.3%) especially with concomitant PUV presented at ≤1-year-age as compared to those with AUV (4 of 11, 36.4%). Concomitant PUV and AUD cases (n=3) had characteristic micturating cystourethrography (MCUG) features. (Fig. 1A) Retrograde urethrography delineated the valve and distal urethra dimensions better (Fig.1B, C). Urethral hypoplasia distal to the valve (n=4) urethral duplication (n=1) were associated (Fig.1D). Syringocele was ruled out in proximal AUD by absence of filling defect on MCUG and appearance of urethral walls on urethrocystoscopy. Serum creatinine > 1mg/dL (n=5), trabeculated bladder (n=12), and vesicoureteral reflux (VUR) (n=12) was noted at presentation. Diverticulum/valve excision (n=13, 48.2%), fulguration alone (n=12, 44.4%) and primary urinary diversion (n=2, 7.4%) were performed. Follow up (range:3 mo-19 years), showed deranged renal function tests (n=6), VUR (n=4), impaired renal function on scans (n=8), and lower urinary tract dysfunction (n=7). Outcome with and without associated PUV was similar. Long term results were better in AUV compared to AUD. Pre-operative raised serum creatinine (>1mg/dL), trabeculated bladder, non-dilated posterior urethra on MCUG and bilateral impaired renal function on scans had significant association with follow up eGFR less than 60ml/kg/min. Co-existing PUV and AUD present earlier and have specific imaging findings. In proximal AUD, possibility of syringocele should be kept in mind, as they have similar presentation and imaging. Concomitant PUV did not alter prognosis. Secondary effects on bladder and renal function were more with AUD. Follow up eGFR less than 60ml/kg/min was associated with pre-operative elevated serum creatinine, trabeculated bladder, non-dilated posterior urethra, and bilateral impaired renal function on scans.