Cowper's syringocele: pathological manifestations and radiological aspects.

Transcription

1 Cowper's syringocele: pathological manifestations and radiological aspects. Poster No.: C-1468 Congress: ECR 2011 Type: Authors: Keywords: DOI: Educational Exhibit E. Guidi, B. Ginanni, N. Armillotta, D. Caramella, C. Bartolozzi; Pisa/IT Urinary Tract / Bladder /ecr2011/C-1468 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. Page 1 of 20

2 Learning objectives To illustrate clinical features and to describe radiological findings of Cowper's syringocele. Background Anatomy Cowper's glands are four small tubuloalveolar structures lying dorsally to the either side of the membranous urethra. They are two diaphragmatic and two bulbar or accessory glands: - the diaphragmatic glands are located between the fascial layers of the urogenital diaphragm (average diameter of 0,5 cm); - the accessory glands are smaller and located within the corpus spongiosum. Diaphragmatic gland ducts are 3 cm long, with an average diameter of 0,7 mm. They run through the inferior side of the urogenital trigonum and they open into the inferior side of the bulbar urethra. Bulbar gland ducts are short and thin, opening directly into the urethra or emerging with the main duct of the corresponding side (fig. 1). Epidemiology The term syringocele (from the Greek syrinx, tubular formation, and cele, swelling) was firstly used by Maizels in 1983 to define the congenital or acquired dilatation of Cowper's ducts. Page 2 of 20

3 Cowper's syringocele is uncommon: the real frequency is unknown since most of them are asymptomatic, with a reported rate of about 1,5% in pediatric cystourethrography and of 2,3% in autopsic studies (fig. 2). Classification Traditionally, in accordance with Maizels classification, based on the degree of dilatation, Cowper's syringocele has been divided into four types: a) simple syringocele with modestly dilated duct; b) imperforated or cystic syringocele with dilated duct that does not communicate with the urethra; c) perforated syringocele with pathological communication with the urethra; d) ruptured syringocele that leaves its covering membrane in the urethra and may cause obstructive voiding symptoms. A recent simplified classification, based on the configuration of the duct's orifice to the urethra, recognizes two variants of Cowper's syringocele and two clinical presentations, respectively: A) open (non-obstructing) syringocele; B) closed (obstructing) syringocele. Open syringocele is more common in adults and it presents with post-void dribbling, while closed syringocele cause obstructive symptoms (fig. 3). Etiopathogenesis The genesis of Cowper's syringocele is not completely clear. It is usually congenital and it may manifest in childhood or in adults because of spontaneous or iatrogenic duct's orifice opening (open syringocele). Page 3 of 20

4 Clinical manifestation Open syringoceles present with post-void dribbling, fever, urethral discharge, perineal pain, hematuria and urinary incontinence. Closed syringoceles is usually asymptomatic until it is not infected. In this case, obstructive voiding symptoms, dysuria, urinary retention and perineal pain can be present (fig. 4). Imaging The early evaluation of Cowper's syringocele typically involves a thorough voiding history and it may be suspected in young male patients with persistent post-void dribbling but normal uroflowmetry, excluding urethral strictures. Ultrasonography (US) visualizes closed syringocele as a cystic lesions in the anatomic region of Cowper's gland and it can also show open syringocele using normal saline solution to distend the urethra and see the dilatation. Retrograde and antegrade cystourethrography is the exam of choice to evaluate the pathology and it may be used to confirm US findings. It can show a cystic defect on the ventral portion of the urethra distal to prostate (closed syringocele), or an opacification of the dilated cavities of the excretory canals (open syringocele). When cystourethrography is contraindicated or more data are needed, cystourethroscopy, urodynamic studies, or magnetic resonance (MR) may be implemented. MR is particulary used in closed syringocele. Differential diagnoses The main differential diagnoses are with: - urethral fistulae (fistulae are irregular in contours with a tendency to enlarge); - contrast-media spillage and extravasation; - fake images; Page 4 of 20

5 - urethral duplications; - diverticula; - overlapping images. Treatment Usually, symptomatic syringoceles required surgical intervention. In non-obstructing syringoceles, we can use symptomatic medical treatment to resolve voiding symptoms. Obstructing syringoceles need endoscopic unroofing as first-choice option or open surgery. Images for this section: Page 5 of 20

6 Fig. 1: Cowper's syringocele: anatomy of Cowper's glands. Page 6 of 20

7 Fig. 2: Cowper's syringocele: epidemiology. Page 7 of 20

8 Fig. 3: Cowper's syringocele: classification. Page 8 of 20

9 Fig. 4: Cowper's syringocele: clinical features. Page 9 of 20

10 Imaging findings OR Procedure details In all patients, retrograde and antegrade cystourethrography was performed to study low urinary tract (fig. 1). In all cases cystourethrograms show open syringocele; contrast-medium reflux can reach the Cowper's gland ducts or the glands. It is usually unilateral. Cystourethrography shows cystic dilatation as a lacuna on the ventral portion of the urethra, or opacification of the dilated cavities of the excretory canals which have been spontaneously broken or opened by endoscopic manoeuvres. In all cases the reflux of iodinated contrast-medium in Cowper's gland duct is already visible during the filling phase, especially in the oblique view, and it may become more evident, reaching the gland, during the voiding phase (fig. 2-8). The opacification of these cavities leads often to the erroneous interpretation of "diverticula" or "incomplete duplication of the urethra", however the opacified ducts and Cowper's glands are oriented in an anterior-posterior direction respect to the urethra and lead back and away from it. Ultrasound (US) was performed during the retrograde cystourethrography to confirm a previously absent cystic lesion or after urethra distension by normal saline solution (in order to distend the urethra and see better the dilatation). It shows an anechoid doublechannel tubular structure, the nearest to the probe, representing the dilated Cowper's duct, the more distal the urethra (fig. 5, 6, 8). Images for this section: Page 10 of 20

11 Fig. 1: Clinical features. Page 11 of 20

12 Fig. 2: Case 1: a 69 years-old man with previous prostatectomy for prostate carcinoma with post-void dribbling, hematuria and urinary incontinence. Voiding cystourethrogram shows reflux in a minimally dilated Cowper's duct. Page 12 of 20

13 Fig. 3: Case 2: a 47 years-old man with post-void dribbling and hematuria. Oblique view of retrograde cystourethrography shows a thin septum that form a little pocket on the ventral surface of bulbar urethra. Contrast-medium fills it when urethra is distend (left image). Voiding cystourethrogram shows Cowper's syringocele (right image). Page 13 of 20

14 Fig. 4: Case 3: a 75 years-old man with prostate hypertrophy, post-void dribbling and urinary tract infection. Oblique view shows opacification of the dilated left Cowper's gland duct (left image). Voiding cystourethrogram shows the reflux in the left Cowper's gland duct (right image). Page 14 of 20

15 Fig. 5: Case 4: a 50 years-old man with post-void dribbling. Oblique view shows large syringocele under membranous urethra (upper image). Longitudinal (left and central images) and axial (right image) US-scans show an anechoid double-channel tubular structure. Page 15 of 20

16 Fig. 6: Case 5: a 45 years-old man with post-void dribbling and hematuria. Oblique view shows a thin radio-opaque image that runs parallel to the inferior edge of urethra (left image). Longitudinal US-scan shows an anechoid double-channel tubular structure (right image). Page 16 of 20

17 Fig. 7: Case 6: a 46 years-old man with post-void dribbling and hematuria. Oblique view shows a thin radio-opaque image that runs parallel to the ventral edge of urethra (left image). Voiding cystourethrogram shows the dilatation of the Cowper's gland duct that form a little pocket (right image). Page 17 of 20

18 Fig. 8: Case 7: a 46 years-old man with post-void dribbling and hematuria. Oblique view shows large syringocele under membranous urethra. Voiding cystourethrogram shows the large syringocele. Longitudinal (left and central images) and axial (right image) USscans show an anechoid double-channel tubular structure. Page 18 of 20

19 Conclusion Cowper's syringocele is fairly common but often misunderstood and it may be suspected in male patients with persistent post-void dribbling with normal uroflowmetry, that excludes urethral strictures. Detection of Cowper's ducts and glands dilatation in patients without signs of urethral anomalies is a rare finding during cystourethrography, which is considered a variant. On the contrary, it can be more frequently observed when a urethral stenosis is present. Therefore it is very important correlate US and cystourethrography imaging with clinical symptoms, because, however Cowper's syringocele is often asymptomatic, it can be a possible cause of voiding disorders. Personal Information Elisa Guidi, MD elisaguidi83@hotmail.it Department of Oncology, Transplants and New Technologies in Medicine Division of Diagnostic and Interventional Radiology University Hospital of Pisa ITALY References Beluffi G, Fiori P, Pietrobono L, Romano P: Cowper's glands and ducts: radiological findings in children. Radiol med 2006; 111: Bevers RFM, Abbekerk EM, Boon TA: Cowper's Syringocele: symptoms, classification and treatment of an unappreciated problem. The Journal of Urology 2000; 163: Page 19 of 20

20 Campobasso P,Schieven E,Fernandes EC: Cowper's syringocele: an analysis of 15 consecutive cases. Archives of Disease in Childhood 1996; 75:71-73 Maizels M, Stephens FD, King LR, et al.: Cowper's Syringocele: a classification of dilatations of Cowper's gland duct based upon clinical characteristic of 8 boys. The Journal of Urology 1983; 129: Melquist J, Sharma V, Sciullo D, et al.: Current Diagnosis and Management of Syringocele: A Review. International Braz J Urol 2010; 36(1): 3-9 Selli C, Nesi G, Pellegrini G, Bartoletti R, et al.: Cowper's Gland Duct Cyst in an Adult Male. Radiological and Clinical Aspects. Sand J Urol Nephrol 1996; 31: Page 20 of 20