Posterior urethral valves and Cowper's syringocele: A rare association causing voiding dysfunction.

Urethral syringoceles are cystic dilatations of paired bulbourethral glands of Cowper. They can cause voiding dysfunction in male children and usually occur in isolation. We report a rare association of Cowper's syringocele with posterior urethral valves in a 4-year-old child, which was successfully managed endoscopically.

INTRODUCTION

Posterior urethral Valves (PUV) are the most common cause of bladder outlet obstruction in a male child. The other rarer causes are anterior urethral valves/diverticula, urethral duplications, Cowpers’ syringocele etc. It is rare to have double obstruction in both anterior as well as posterior urethra and we describe a case of syringocele in association with posterior urethral valves as a cause of double obstruction.

CASE REPORT

A 4-year-old boy presented with right-sided inguinal hernia and voiding dysfunction. He had straining, frequency, and prolonged micturition for the past 1 year. Apparently the child had a weak urinary stream since birth, which was ignored as being normal. The child never had a urinary infection. On investigations his urine examination, renal functions, and renal tract ultrasound (US) were normal. A micturating cystourethrogram (MCU) showed dilated posterior urethra suspicious of posterior urethral valves and a definite filling defect in the proximal end of anterior urethra [Figure 1].

Figure 1

Micturating cystourethrogram showing dilated posterior urethra suggestive of posterior urethral valves and a filling defect in proximal bulbar urethra

At cystoscopy, the bladder was minimally trabeculated with prominent bladder neck and type 1 posterior urethral valves. There was also an elongated translucent cystic swelling in the posterior wall of proximal portion of bulbar urethra [Figure 2], which corresponded to the filling defect seen on MCU. A diagnosis of posterior urethral valves with Cowper's syringocele was made.

Figure 2

Cystoscopy showing nonperforate syringocele

The valves were fulgurated at 5 and 7 O’ clock positions and the syringocele was deroofed with a bugbee, which decompressed the syringocele. The hernia was also repaired at the same time. Postoperatively, the child was on an indwelling Foley's catheter, which was removed after 3 days. The child at discharge was passing urine with good stream but still had increased frequency of micturition, which resolved after a few weeks of treatment with oral oxybutynin.

A repeat cystoscopy, done after 6 weeks, showed no evidence of syringocele and there were no residual valves. The MCU showed no filling defect seen previously and the posterior urethral dilatation had resolved. The child is on regular followup, symptomfree, and the recent US was normal.

DISCUSSION

Syringoceles are cystic dilatations of Cowper's gland duct within the bulbous urethra.[1] Maizel's et al. classified them into 4 types: simple syringocele, perforate syringocele, imperforate syringocele (the type which was seen in our case), and ruptured syringocele. These are rare lesions seen in boys with a reported incidence of 1.5% on cystograms.[2] Most are asymptomatic but occasionally they may cause urinary infection, hematuria, dysuria, and obstructive voiding symptoms. Only a quarter of children with syringocele need surgical intervention.[3] The diagnosis is confirmed on MCU and cystourethroscopy. Imaging with US or rarely computed tomography and magnetic resonance imaging may aid in diagnosis.[4]

Although syringoceles occur as isolated entities, they may be associated with a variety of other urethral anomalies, such as urethral diverticulum, anterior urethral valves, posterior urethral valves, ureterocoele, acontractile bladder, and Cobb's collar.[5] Symptomatic syringoceles in children are successfully managed by transurethral endoscopic deroofing. Large syringoceles may occasionally need an open operation through perineal approach.[4] This case is reported here due its rarity and its association with posterior urethral valves.