Urethral diverticulum

DOI: 10.1111/tog.12192 The Obstetrician & Gynaecologist http://onlinetog.org 2015;17:125 9 Review Urethral diverticulum Rosemary Archer MB MS, a Jennifer Blackman MRCOG, a Mark Stott FRCS, b Julian Barrington MA MD FRCOG c, * a Specialist Registrar in Obstetrics and Gynaecology, Department of Obstetrics and Gynaecology, Torbay Hospital, Torquay, Devon TQ2 7AA, UK b Consultant Urologist, Department of Urology, Royal Devon and Exeter Hospital, Exeter, Devon EX2 5DW, UK c Consultant in Obstetrics and Gynaecology, Department of Obstetrics and Gynaecology, Torbay Hospital, Torquay, Devon TQ2 7AA, UK *Correspondence: Julian Barrington. Email: julian.barrington@nhs.net Accepted on 28 January 2015 Key content Urethral diverticulum (UD) is uncommon but is underdiagnosed. Imaging is essential before surgery is planned. Missed diagnosis may result in continued symptoms. Surgery for UD is challenging and complications are common. Learning objectives To review the symptomatic presentation of UD. To review the differential diagnosis of a mass in the urethra. To understand how imaging may help in the diagnosis of UD. To review the management of complications of UD. To review the surgical treatment of UD. Ethical issues If not performed adequately surgery may result in a worsening of symptoms especially urinary incontinence. Surgery is difficult and should be performed by specially trained individuals Keywords: complications / imaging / incontinence / urethral diverticulum / urethral-vaginal fistula Please cite this paper as: Archer R, Blackman J, Stott M, Barrington J. Urethral diverticulum. The Obstetrician & Gynaecologist 2015;17:125 9. Introduction A urethral diverticulum (UD) is defined as the presence of a sac opening from the urethra. It might be suspected by a lump (Figure 1) or tenderness along the line of the urethra or external urethral discharge on urethral massage. 1 It ranges in size from 3 mm to 4 cm. 2 It is an underdiagnosed condition and the prevalence is unknown because only approximately 1% to 6% of cases are reported. 3,4 It is often missed due to a protean presentation with symptoms common to numerous other gynaecological pathologies. 4 The time from presentation to diagnosis may range from 2 96 months (median 9.5 months). 5 The usual age at presentation is between 30 and 60 years. 6 Aetiology The urethra is a spongy tube sandwiched between two layers of urethral pelvic ligament. The periurethral glands (Skene s glands) lie along the whole length of the urethra in a posteriorlateral axis and most open into the distal third of the urethra. 6,7 The aetiology of UD remains largely unknown but it is thought it may be congenital or acquired in origin. Congenital cases may occur from remnants of Gartner s duct or abnormal union of primordial folds or persisting cell rests, especially M ullerian, but they are rarely found in children. 8 It is three times higher in black american compared with white american women suggesting a genetic susceptibility in this population. 9 Most cases are acquired and result from repeated infections and obstruction of the periurethral glands. These rupture into the urethral lumen and the cyst epithelialises and persists. 10 Traumatic childbirth, especially with assisted delivery, has been suggested as a cause of UD development, 11 but it may equally develop in nulliparous patients. 3 UD has also been reported following transurethral collagen injection for stress urinary incontinence. 12 The UD usually dissects within the urethral pelvic ligament with the orifice/neck just off centre at 4 and 8 o clock. Occasionally it may extend proximally beneath the bladder neck and trigonal area. Most UD have a single connection to the urethra and vary in size and shape. Complex patterns may occur with multiple ostia; UD may be multiple and loculated and may extend partially ( saddlebag or horseshoe ) or circumferentially around the urethra 13 and thereby compromise urethral sphincter function (Figure 2). They are usually lined by urothelium but squamous and glandular metaplasia can occur 14 and even leiomyoma. 15 Clinical presentation UD may present with multiple symptoms (Box 1). The historical classical triad of dysuria, post-void dribbling and dyspareunia is only seen in a minority of patients. Lower urinary tract symptoms (LUTS), namely frequency and urgency, are present in 40 100% of cases. 3 Other ª 2015 Royal College of Obstetricians and Gynaecologists 125

Urethral diverticulum imaging of upper tracts to exclude a duplex system is appropriate. A history of recurrent anterior vaginal wall prolapse repair may also alert the physician to the presence of a UD. 18 Figure 1. Typical presentation of a urethral diverticulum. common presentations include dysuria (30 70%), post micturition dribble (10 30%), dyspareunia (10 25%), urinary incontinence (35%), recurrent urinary tract infection (30 50%), tender vaginal mass (35%) or ulcer, 2 haematuria (10 25%), vaginal discharge (12%) and retention of urine (4%). 3 UD may therefore present in such a diverse variety of ways that a high index of suspicion is essential. Clinical examination may reveal a tender mass anteriorly 2 3 cm inside the introitus. Palpation of this mass may result in a purulent discharge from the external urethral meatus 6 but this other classic sign is only present in 25% of cases. 16 Malignant change or calculi should be considered if hardness or induration is found on palpation especially in the presence of blood. 17 Differential diagnosis includes interstitial cystitis, carcinoma in situ, overactive bladder, vaginal wall cysts, urethral caruncle and mucosa prolapse, Skene gland abnormality, vaginal leiomyoma and Gartner s duct cyst and abscess (Box 2). 3,6 Pelvic inflammatory disease and endometriosis should also be considered. Ectopic caecoureterocele may develop in the urethrovaginal septum so Investigations Suitable investigations to diagnose or confirm UD are listed in order of preference in Box 3. Urethroscopy using a 0 degree endoscope has traditionally been performed to investigate and locate a UD. A mucosal defect is found in 70% of patients 16 but concurrent inflammation may obscure the ostia. 19 However, concomitant pathology such as carcinoma in situ can be excluded by biopsy. Urodynamics are recommended in women where a UD is suspected. Sixty per cent of women with UD have associated urinary incontinence 19 and therefore urodynamics are essential to differentiate between stress incontinence, overactive bladder (OAB) and post micturition dribbling. Removal of the UD alone will resolve some cases with incontinence but concomitant treatment of stress urinary incontinence might be required. 10 Since 17% of women will develop incontinence post UD excision, baseline assessment of detrusor and urethral function is essential before surgery. Video urodynamics may help to identify women with coexistent obstructive voiding. 20 Magnetic resonance imaging (MRI) (Figure 2) has now become the recommended imaging study using T2 weighted images. 21 No radiation is used and a solid mass may be differentiated from a complex UD. The disadvantages of MRI scans are the increased cost and lack of availability, and they are time consuming. If radiological expertise is not available locally, ultrasound can be an appropriate alternative. Transvaginal ultrasound (TVUSS) has excellent sensitivity and anatomic delineation and is useful for UD that do not fill with contrast during imaging studies. 22 The size, number, location, structure, content and wall thickness may be Figure 2. MRI showing circumferential urethral diverticulum. 126 ª 2015 Royal College of Obstetricians and Gynaecologists

Archer et al. Box 1. Presentation of urethral diverticulum Dysuria Dribbling urine (post micturition) Dyspareunia Lower urinary tract symptoms (frequency/urgency) Incontinence Urinary tract infection Pain Vaginal mass/ulcer Haematuria Vaginal discharge Urinary retention Box 2. Differential diagnosis of urethral diverticulum Lower urinary tract symptoms Interstitial cystitis Carcinoma in situ Overactive bladder Mass Vaginal wall cysts Urethral mucosa prolapse Cystocele Skene gland abnormality Ectopic caeco-ureterocele Vaginal leiomyoma Urethral caruncle Gartner s duct cyst Gartner s duct abscess Pain Pelvic inflammatory disease Endometriosis Box 3. Investigations for urethral diverticulum Urethroscopy (Video) urodynamics Magnetic resonance imaging Ultrasound Computerised tomography Micturating cystogram Double balloon urethrogram obtained but the disadvantage of TVUSS is that the probe may directly compress the urethra. 22 Transabdominal ultrasound (TAUSS) is insensitive for UD less than 20 mm in diameter. 23 Transperineal ultrasound is more sensitive than TAUSS but is suboptimal for small diverticula. 24 Transrectal USS may improve visualisation of the periurethral area without urethral distortion. Conventional contrast-enhanced computerised tomography (CT) scan shows the UD as a cystic mass with wall thickening and enhancement at the level of the symphysis Figure 3. Micturating cystogram showing urethral diverticulum. pubis. Calculi and solid malignancies may also be detected. 23 The detection rate of UD may be enhanced by CT urethrography in which the bladder is filled with dilute contrast fluid. 25,26 A micturating cystogram or voiding cystourethrogram (VCUG) (Figure 3) will identify 85 95% of women with urethral diverticula. 8,27 This test will also help identify filling defects due to malignancy and calculi. It does, however, involve a dose of radiation, is uncomfortable and can be time consuming; the urethral catheterisation carries a risk of infection and women often have difficulty voiding in the standing position. The UD ostia may not be visualised if occluded by pus/debris and incomplete opacification may underestimate the size of the UD. The sensitivity of VCUG is improved when combined with other imaging studies. 28 A double balloon urethrogram used to be considered the gold standard investigation. A specialised catheter uses positive pressure to force contrast into the diverticular orifice. The accuracy of this test is 90% 29 but is technically difficult, uncomfortable for the patient and may result in urethral injury. Complications and management of urethral diverticulum Complications of UD (Box 4) include urinary tract infections, which are common and recurrent in 30% to 50%. 30 Fluid debris may be visible on imaging. Acute or chronic infection must be treated before any form of repair is contemplated. A UD may develop into an abscess, which presents with extreme tenderness in the anterior vaginal wall, and a mass. These should not be incised and drained because a urethral vaginal fistula may develop. The optimum treatment is ª 2015 Royal College of Obstetricians and Gynaecologists 127

Urethral diverticulum Box 4. Complications of urethral diverticulum Urinary tract infection Abscess Calculi Incontinence Neoplasm aspiration and appropriate antibiotics and delayed definitive surgical management once the abscess has resolved. A calculus may be present in 1.5% to 10% of patients with a UD. 10,31 Urine stasis and infection may promote stone formation. Management of a calculus is removal at the time of excision of the UD. Concomitant urinary incontinence may be stress incontinence which may coexist with the UD or be the result of a complex UD with weakening of the urethral sphincter. Urinary incontinence may also develop from post micturition dribble leaking from the UD, especially if the diverticulum is in the distal third of the urethra. Urethral neoplasms are rare (6 9%) 32,33 and are mainly adenocarcinomas (40 60%) 15 and there is scant evidence for best management. If suspected, a biopsy should be considered if conservative surgical management is planned. For confirmed cases, partial 34 or complete urethrectomy 35 or even anterior exenteration is usually required. 36 Urethral diverticular malignancies characteristically present late with early metastases and therefore there is a high risk of recurrence after surgery. 15 Surgery for urethral diverticulum Asymptomatic diverticula and patients with minor symptoms may be monitored and treated with antibiotics. 7,36 Due to the complexity of UD excision, surgery is only indicated for persistent symptoms and complications of UD after full informed consent. The surgery should only be carried out by urogynaecologists and/or urologists who have wide and extensive experience of these reconstructive procedures. The three main surgical options to treat UD are discussed below. Endoscopic re-roofing or transurethral incision Endoscopic incision converting a narrow into a wide diverticular neck and thereby allowing it to drain freely has been described. 37 This is mainly used for recurrent UD arising in the distal urethra. If used for mid to proximal UD, there may be iatrogenic damage to the urethral sphincter, which does not allow concomitant stress incontinence surgery. Marsupialisation of the urethral diverticulum sac Transvaginal marsupialisation involves an incision through the diverticulum to its urethral orifice. 38 The vaginal and urethral epithelium are then co-opted using dissolvable sutures. The diverticular cavity may be packed with oxidised cellulose or similar agents to encourage fibrosis. Complications of marsupialisation include a splayed stream and there is a high risk of urethro-vaginal fistulae since the UD usually extends through all layers of the urethra. This procedure should again only be done with UD in the distal third due to the high risk of damage to the urethral sphincter. Marsupialisation should therefore only be considered if the patient is frail, elderly or too infirm to undergo diverticulectomy. Diverticulectomy In a diverticulectomy procedure, the UD is incised and a well vascularised vaginal wall flap is mobilised towards the bladder neck. The peri-urethra fascia and rhabdosphincter should be preserved with complete excision of the diverticulum wall and neck. The resultant large urethral defect should be repaired in a multi-layered non-overlapping closure with absorbable sutures 33 but it may be difficult to adequately close without compromising the urethral lumen. A cure rate of approximately 70% may be expected. 30 If stress incontinence is evident pre-operatively, a concomitant procedure with an autologous sling can be considered. Due to risks of erosion and infection, synthetic slings especially polypropylene should be avoided. A simpler technique involves excision of the UD wall and ligation of the urethral orifice with the interposition of a Martius graft 39,40 to revascularise the often found poor quality tissues. The Martius graft may help prevent urinary incontinence due to its bulking effect but if not, a delayed anti incontinence procedure may be carried out. Complications of UD surgery are common (Box 5) with recurrence rates of up to 36% due to incomplete resection. Stress incontinence may develop in up to 17% of patients due to damage to the urethral rhabo-sphincter or a urethral stricture may develop. A urethral vaginal fistula may develop in up to 6% of patients. 4 Conclusion Diagnosis of UD requires a high index of suspicion especially in the presence of certain symptoms such as urgency, frequency and dribbling incontinence. Appropriate investigations to define the extent and delineation of the Box 5. Complications of surgery for urethral diverticulum Recurrence/incomplete excision Stress incontinence Urethral stricture Urethro-vaginal fistula 128 ª 2015 Royal College of Obstetricians and Gynaecologists

Archer et al. lesion are essential. UD surgery is difficult due to the vascular and friable condition of the tissues and difficulty in identifying the defect, especially if the urethral diverticulum is complex and therefore should only be done by an appropriately trained urogynaecologist or urologist with experience in female reconstruction surgery. Strict adherence to surgical principals may reduce concomitant complications. Contribution of authorship R Archer and J Barrington conceived the initial idea and design of the article; conducted the literature search, and drafted the article. They approved the final version to be published. J Blackman and M Stott revised the article and approved the final version to be published. Disclosure of interests There are no disclosures by R Archer, J Blackman and M Stott. J Barrington has received training and travel grants from Astellas, Boston Scientific and CR Bard. 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