A case of hypospadias, anterior and posterior urethral valves

Abstract

This report outlines the case of a 3-year-old boy whose initial presentation was that of asymptomatic hypertension (lowest recording 148/90), found at preoperative check prior to stage 2-correction surgery for distal hypospadias. Upon diagnosis of true hypertension, an ultrasound of the child's renal tract showed evidence of marked hydronephrosis and calyceal dilatation. On the background of deteriorating renal function (Urea 25.5 and Creatinine 188), a Micturating Cystourethrogram was performed, demonstrating posterior urethral dilatation. With difficulties controlling blood pressure, the child was transferred to Urology care, where resection of a posterior urethral valve (PUV) was undertaken. Despite this, renal function deteriorated further and re-cystoscopy identified an anterior urethral valve (AUV), which was also resected. Renal function, although improved, remains poor and blood pressure is controlled with two anti-hypertensives. To the publisher's knowledge, the association between hypospadias, PUVs and AUVs is as yet undocumented.

Rare copy number variants implicated in posterior urethral valves

“…Although reports of hypospadias occurring in conjunction with PUV are rare [Bhagat et al, 2008; Carvell and Mulik, 2013; Ranawaka and Dickson, 2013], several gene variants, including BMP7 , that contribute to early urethral development have been shown to be associated with hypospadias risk in humans [Carmichael et al, 2013]. …”

Concomitant Anterior and Posterior Urethral Valves: A Comprehensive Review of Literature

“…30 However, the recent reports of concomitant AUV and PUV with hypospadias may further support this theory. 20,31,32 CONCOMITANT AUV/D AND PUV Available Studies From a statistical viewpoint, one may estimate the prevalence of concomitant AUV/D and PUV to be exceedingly rare by multiplying the incidence of AUV/D by that of PUV. However, this calculation method assumes that no biological or clinical relationship exists between the 2 entities that may not be essentially true.…”

“…5,20,21,[30][31][32][33][34][35][36][37][38][39] However, the true number would definitely be more than this because many older reports were not accessible for us and some authors did not focus on this finding in their series. It can be predicted that because of rarity of concomitant AUV/D and PUV compared with conditions like isolated AUV or PUV, many clinicians, especially in tertiary centers, have faced these patients but did not report them in the literature because of the small sample size.…”

“…In 1 patient with hypospadias, asymptomatic hypertension diagnosed preoperatively was the sole symptom that led to diagnosis of hydronephrosis and urologic workup. 31 The presenting symptoms in children with concomitant AUV/D and PUV may partially depend on the valve that causes more prominent obstructive symptoms. Although AUV and AUD may be associated with serious sequelae on the bladder and the upper urinary tract, it is generally believed that the outcomes are better compared with those of PUV.…”

“…41 Alternatively, the anterior anomaly may remain undiagnosed because the posterior valve impedes the adequate flow of urine beyond the proximal obstruction and does not permit bulging of the anterior urethra. 5,31 The same phenomenon may impede the anterior flap of AUD to develop completely to cause a valve effect until the proximal obstruction is resolved. 36 The valves may also remain unnoticed in retrograde urethrography because they may flatten against the urethral wall and remain open in response to retrograde flow.…”