Chapter 42. Disorders of the Female Urethra

Distal Urethral Stenosis in Infancy and Childhood (Spasm of the External Urinary Sphincter) and Dysfunctional Voiding

There has been considerable confusion about the site of lower tract obstruction in young girls who have enuresis, a slow and interrupted urinary stream, recurrent cystitis, and pyelonephritis and who, on thorough examination, often exhibit vesicoureteral reflux. Treatment has been directed largely to the bladder neck on rather empiric grounds. Most of these children, however, have congenital distal urethral stenosis with secondary spasm of the striated external sphincter rather than bladder neck obstruction.

Lyon and Tanagho (1965) found that the distal urethral ring calibrates at 14F at age 2 and at 16F between the ages of 4 and 10. Even though from the hydrodynamic standpoint, such a stenotic area should not be obstructive, almost all observers agree that dilatation of the ring does relieve symptoms in these children and that it results in cure or amelioration of persistent infection or vesical dysfunction in 80% of cases (Kondo et al, 1994). Tanagho et al (1971) measured pressures in the bladder and in the proximal and mid urethra simultaneously in symptomatic girls and found high resting pressures, some as high as 200 cm of water (normal, 100 cm of water) in the midurethral segment. Attempts at voiding caused intravesical pressures as high as 225 cm of water (normal, 30–40 cm of water) to develop. Under curare, the urethral closing pressures dropped to normal (40–50 cm of water), proving that these obstructing pressures were caused by spasm of the striated sphincter muscle. If the distal urethral ring was treated and symptoms abated, repeat pressure studies showed normal midurethral and intravesical voiding pressures. It seems clear, therefore, that the likely cause of urinary problems in young girls is spasm of the external sphincter and not vesical neck stenosis (Smith, 1969).

In addition to recurrent urinary tract infections, these patients have hesitancy in initiating micturition and a slow, hesitant, or interrupted urinary stream. Enuresis and involuntary loss of urine during the day are common complaints. Abdominal straining may be required in order to void. Small amounts of residual urine are found, which impair the vesical defense mechanism. A voiding cystourethrogram may reveal an open bladder neck and ballooning of the proximal urethra secondary to spasm of the external sphincter (Figure 42–1).

The voiding cystourethrogram may reveal evidence of the distal ring, but typical findings are not always seen, particularly if the flow rate is slow. Definitive diagnosis is made by bougienage.

Historically, the simplest and least harmful treatment is overdilatation with sounds up to 32–36F). The ring “cracks” anteriorly, with some bleeding. Recurrence of the ring is rare. The results of internal urethrotomy were poor, since incising the urethra along its entire length does not cut the external sphincter (Kaplan et al, 1973).

In the past two decades, it has been recognized that many of these symptoms are due to functional, rather than neurologic or anatomic causes of obstruction. Children must achieve adult patterns of urinary control and disturbances, especially around the time of toilet training. This may cause a variety of symptoms that result from voiding against a voluntarily closed urethral sphincter (dysfunctional voiding). These range from severe functional obstruction with urinary retention, altered bladder anatomy, and vesicoureteral reflux (known as Hinman's syndrome) to less severe incomplete control of urination. They are often accompanied by bowel symptoms, such as constipation or encopresis. Treatment requires bladder retraining, including psychologic help and biofeedback, as well as restoration of normal bowel habits, including dietary changes and laxatives.

Labial Fusion (Synechia Vulvae)

Some children with recurring urinary infection are found to have fused labia minora, which are apt to obstruct the flow of urine, causing it to pool in the vagina. Local application of estrogen cream twice daily for 2–4 weeks usually causes spontaneous separation, with minimal side effects (Aribag, 1975; Leung et al, 2005). Forceful separation or dissection has its advocates (Christensen and Oster, 1971).

It is uncommonly seen as an acquired disease after puberty, caused by genital trauma (sexual abuse, vaginal delivery, surgery, etc) (Kumar et al, 2006). In cultures where “female circumcision” is performed, this may be a relatively common complication (Adekunle et al, 1999).

Acute Urethritis

Acute urethritis frequently occurs with gonorrheal (Neisseria gonorrhoeae) or trichomoniasis (Trichomonas vaginalis) infection in women, and it may less commonly occur with infection by Chlamydia trachomatis (approximately 25% of cases are symptomatic). Urinary symptoms are often present at the onset of the disease. Cultures and smears establish the diagnosis. Prompt cure can be achieved with antimicrobial drugs, usually to cover both gonorrhea and chlamydia, such as a combination of intramuscular ceftriaxone and oral azithromycin or doxycycline. Treatment is important, as 40% of women with untreated chlamydia infections will have pelvic inflammatory disease, which may lead to ectopic pregnancy, pelvic pain, and infertility (Simms and Stephenson, 2000).

The detergents in bubble bath and some spermicidal jellies may cause vaginitis and urethritis. Symptoms of vesical irritability may occur (Bass, 1968; Marshall, 1965).

Chronic Urethritis

Chronic urethritis is one of the most common urologic problems of females. The distal urethra normally harbors pathogens, and the risk of infection may be increased by wearing contaminated diapers, by insertion of an indwelling catheter, by spread from cervical or vaginal infections, or by intercourse with an infected partner. Urethral inflammation may also occur from the trauma of intercourse or childbirth, particularly if urethral stenosis, either congenital or following childbirth, is present.

Clinical Findings

The urethral mucosa is reddened, quite sensitive, and often stenotic. Granular areas are often seen, and polypoid masses may be noted just distal to the bladder neck.

Symptoms

The symptoms resemble those of cystitis, although the urine may be clear. Complaints include burning on urination, frequency, and nocturia. Discomfort in the urethra may be felt, particularly when walking.

Signs

Examination may disclose redness of the meatus, hypersensitivity of the meatus and of the urethra on vaginal palpation, and evidence of cervicitis or vaginitis. There is no urethral discharge.

Laboratory Findings

When the initial and midstream urine are collected in separate containers, the first glass contains pus and the second does not (Marshall et al, 1970). Ureaplasma urealyticum (formerly called T strains of mycoplasmas) is often identifiable in the first glass. These findings are similar to those of nongonococcal (chlamydial) urethritis in males. Clinically, the presence of white blood cells (leukocytes) in the absence of bacteria on a routine stain or culture suggests nongonococcal urethritis. In other cases, various bacteria (eg, Streptococcus faecalis, Escherichia coli) may be cultured from both the urethral washings and a specimen taken from the introitus.

Instrumental Examination

A catheter, bougie à boule, or sound may meet resistance because of urethral stenosis. Panendoscopy reveals redness and a granular appearance of the mucosa (Krieger, 1988). Inflammatory polyps may be seen in the proximal portion of the urethra. Cystoscopy may show increased injection of the trigone (trigonitis), which often accompanies urethritis.

Differential Diagnosis

Differentiation of urethritis from cystitis depends on bacteriologic study of the urine; panendoscopy demonstrates the urethral lesion. Both urethritis and cystitis may be present. Chronic noninflammatory urethritis may be a manifestation of psychic stressors. Patients with anxiety or other temporary or chronic psychologic disorders may present with symptoms that are very suggestive of urethritis. Alternatively, women with long-standing symptoms may have these symptoms as an adult version of childhood voiding dysfunction (see earlier discussion). It is important to understand that chronic dysuria in the absence of a real bacteriologic source is often a manifestation of chronic pelvic pain syndrome (ie, interstitial cystitis, painful bladder syndrome).

Treatment and Prognosis

Gradual urethral dilatations (up to 36F in adults) are indicated for urethral stenosis; this allows for some inevitable contracture. However, true urethral stenosis is not common in women, and diagnosis should be confirmed by cystoscopy and urethral calibration. If pelvic dysfunction is the underlying issue, calibration under anesthesia will show normal caliber urethra. Immergut and Gilbert (1973) prefer internal urethrotomy (Farrar, 1980). U. urealyticum and chlamydial urethritis usually respond to doxycycline or azithromycin.

Atrophic Urethritis

After physiologic (or surgical) menopause, hypoestrogenism occurs and atrophic changes take place in the vaginal mucosa, so it becomes dry and pale (Smith, 1972); atrophic vaginitis affects 20–30% of postmenopausal women. This is likely a significant underestimation because women are unwilling to report symptoms due to either embarrassment or lack of knowledge that there are treatments available (Johnston, 2004). Similar changes develop in the lower urinary tract, which arises from the same embryologic tissues as the female reproductive organs. Some eversion of the mucosa about the urethral orifice, from atrophy of the vaginal wall, is usually seen. This is commonly misdiagnosed as caruncle.

Clinical Findings

Symptoms

Many postmenopausal women have symptoms of vesical irritability (burning, frequency, urgency) and stress incontinence. Dysuria may occur due to urine contact with the inflamed atrophic tissues themselves or because of the increased incidence of urinary tract infections in these women. They may complain of vaginal and vulval itching or tightness, discharge, dyspareunia, and may have bloody vaginal spotting, especially after intercourse.

Signs

The vaginal epithelium is dry and pale, with a decrease in the rugae. The mucosa at the urethral orifice is often reddened and hypersensitive; eversion of its posterior lip from atrophy of the urethrovaginal wall is common. Atrophic vaginitis also increases the risk for urinary tract infections, and approximately 10–15% of women older than 60 years have frequent urinary tract infections.

Laboratory Findings

The urine is usually free of microorganisms. The diagnosis can be made by the following procedure: A dry smear of vaginal epithelial cells is stained with Lugol's solution. The slide is then washed with water and immediately examined microscopically while wet. In hypoestrogenism, the cells take up the iodine poorly and are therefore yellow. When the mucosa is normal, these cells stain a deep brown because of their glycogen content. The diagnosis may also be confirmed by a Papanicolaou smear. Postmenopausal status is associated with a higher vaginal pH, a decrease in vaginal lactobacillus colonization, and increased colonization with E. coli.

Instrumental Examination

Cystourethroscopy may demonstrate a reddened and granular urethral mucosa. Some urethral stenosis may be noted. More commonly, the mucosa at the meatus is reddened, but the remainder of the urethra is quite normal.

Differential Diagnosis

Atrophic urethritis is often mistaken for urethral caruncle. Eversion of the posterior lip of the urinary meatus is evident in both conditions; however, a hypersensitive vascular mass is not present in atrophic urethritis.

Treatment

Atrophic urethritis responds well to local estrogen treatment to the vagina (Sturdee, 2010). There are several formulations available: conjugated equine estrogen cream, a sustained-release intravaginal estradiol ring which delivers low level of estradiol over a 3-month period, or a low-dose estradiol tablet. Local application has the advantage of minimal change of systemic blood level and thus avoids the side effects associated with systemic hormone therapy. There is insufficient data to recommend annual endometrial surveillance in asymptomatic women using local estrogens. However, for those who have had breast or gynecologic cancers, consultation with their oncologist is important.

Prognosis

Topical estrogen vaginal cream is an effective treatment in postmenopausal women with recurrent infections (Quinlivan, 1965). In one study, patients treated with the estrogen cream averaged 0.5 infections per year, compared with about 6.0 infections per year in women who were not treated (Raz and Stamm, 1993).

Urethral Caruncle

Urethral caruncle is a benign, red, raspberry-like, friable vascular tumor involving the posterior lip of the external urinary meatus. It is rare before menopause. Microscopically, it consists of connective tissue containing many inflammatory cells and blood vessels and is covered by an epithelial layer (Lee, 1995).

Clinical Findings

Symptoms include pain on urination, pain with intercourse, and bloody spotting from even mild trauma. A sessile or pedunculated red, friable, tender mass is seen at the posterior lip of the meatus.

Differential Diagnosis

Carcinoma of the urethra may involve the urethral meatus. Palpation reveals definite induration. Biopsy establishes the true diagnosis. Atrophic urethritis is often associated with a polypoid reaction of the urinary meatus and in fact is the most common cause of masses in this region. The diagnosis can be made by verifying the patient's hypoestrogenic status and by demonstrating a favorable response to estrogen replacement therapy. Biopsy should be done if doubt exists (Neilson, 1989; Young, 1996).

Thrombosis of the urethral vein presents as a bluish, swollen, tender lesion involving the posterior lip of the urinary meatus. It has the appearance of the thrombosed hemorrhoid. It usually subsides without treatment; however, persistence or pain will require excision to control symptoms.

Treatment

Local excision is indicated only if symptoms are troublesome.

Prognosis

True caruncle is usually cured by excision, but in a few instances, it does recur.

Prolapse of the Urethra

Prolapse of the female urethra is not common. It usually occurs only in children or in paraplegics suffering from a lower motor neuron lesion. The protruding urethral mucosa presents as an angry red mass that may become gangrenous if it is not reduced promptly (Kleinjan, 1996). When a young girl has a protruding mass, urethral prolapse must be differentiated from prolapse of a ureterocele (Fernandes et al, 1993; Valerie et al, 1999).

After reduction, cystoscopy should be done to rule out ureterocele. Recurrences are rare following reduction; the accompanying inflammation probably “fixes” the tissue in place as healing progresses. If the prolapsed urethra cannot be reduced or if it recurs, an indwelling catheter should be inserted, traction placed on it, and a heavy piece of suture material tightly tied over the tissue and catheter just proximal to the mass. The tissue later sloughs off. Using this same technique, the tissue can be resected, preferably with an electrosurgical cautery (Devine, 1980).

Urethrovaginal Fistula

Urethrovaginal fistulas may follow local injury secondary to fracture of the pelvis or obstetric or surgical injury. A common cause in the industrial world is accidental trauma to the urethra or its blood supply in the course of surgical repair of a cystocele or excision of urethral diverticula. In the developing world, obstructed and prolonged labor is the most common cause (Elkins, 1994). Other causes may include radiation therapy for pelvic malignancy, trauma/pelvic fracture, and vaginal neoplasms. Diagnosis can usually be made on physical examination and urethroscopy. Vaginal urethroplasty is indicated, and these surgeries may be very challenging due to poor quality local tissue; often, two or more procedures may be necessary (Webster et al, 1984). A Martius labial fat flap can be used as interposition layer to augment repair and prevent recurrence of fistula.

Urethral Diverticulum

The incidence of diverticulation of the urethral wall is between 0.6% and 5% (Andersen, 1967; Davis and Robinson, 1970). Diverticula are at times multiple or lobulated. Most cases are probably secondary to obstetric urethral trauma or severe urethral infection. A few cases of carcinoma in such diverticula have been reported (Kato, 1998; Marshall, 1977; Nakamura et al, 1995). Urethral diverticula are usually associated with recurrent attacks of cystitis, irritative voiding symptoms, and urethral pain. A mnemonic is the three Ds: dribbling, dyspareunia, and dysuria. Purulent urethral discharge is sometimes noted as the infected diverticulum empties. On occasion, the diverticulum may be large enough to be discovered by the patient.

The diagnosis is usually made on feeling a rounded cystic mass in the anterior wall of the vagina that leaks pus from the urethral orifice when pressure is applied. Urethroscopy may reveal the urethral opening, although the openings are often very difficult to locate. The postvoiding film of an excretory urographic series may demonstrate the lesion. It may be possible to introduce a small catheter through which radiopaque fluid can be instilled. Appropriate x-ray films are then exposed (Figure 42–2). The plain film may show a stone in the diverticulum (Presman, 1964). If these methods fail, the following procedures can be used:

1. Empty the diverticulum manually. Via a catheter, instill 5 mL of indigo carmine and 60 mL of contrast medium into the bladder. Remove the catheter and have the patient begin to void. Occlude the meatus with a finger. This maneuver usually causes the diverticulum to fill with the test solution. Take appropriate x-rays, and perform urethroscopy to look for leakage of blue dye from the mouth of the diverticulum.

2. Insert a Davis-TeLinde catheter (aka double balloon catheter). This looks like a Foley catheter but is surrounded by a second movable balloon. Pass the catheter to the bladder and inflate the proximal balloon. While exerting tension on the catheter, slide the second balloon against the urinary meatus and inflate it. Then inject contrast medium into the catheter. The radiopaque fluid will exit from the catheter through a hole between the balloons and will fill the urethra and diverticulum, after which x-rays can be exposed. Occasionally, urethral diverticulum is elusive and difficult to visualize. Transvaginal ultrasonography (Baert, 1992; Mouritsent, 1996; Siegel et al, 1998; Vargas-Serrano et al, 1997) or pelvic magnetic resonance imaging (MRI) (Chaudhari, 2010; Debaere et al, 1995; Kim, 1993) can be helpful in diagnosis; MRI is reported to be much more sensitive in detecting diverticulae than double-balloon urethrography (Neitlich et al, 1998), especially when an endoluminal coil is placed in the vagina to enhance the periurethral imaging (Dwarkasing, 2011). MRI is useful to assess urethral diverticula but there are limitations. A study by Chung et al (2010) revealed 24% discrepancy between MRI and surgical findings. Examples include misdiagnosis of the diverticulum as Bartholin's cyst, a sterile abscess incorrectly diagnosed as a diverticulum, and missed detection of cancer within the diverticulum.

Treatment consists of removal of the sac through an incision in the anterior vaginal wall and care being taken not to injure the urethral sphincteric musculature. Incision is carried down to the diverticular mucosa, and the plane of cleavage is followed all around to the neck of the diverticulum. The diverticular sac is completely excised and the defect in the urethra repaired. A suprapubic cystostomy or urethral catheter should be left in place for 2–3 weeks following surgical excision of the diverticulum.

The outcome is usually good unless the diverticulum is so situated that its excision injures the external urinary sphincter mechanism. In a few cases, urethrovaginal fistula may develop. If the fistula does not close with adequate drainage, surgical repair will be necessary 2–3 months later.

Urethral Stricture

True organic stricture of the adult female urethra is not common. (Functional urethral obstruction is more common, due to pelvic floor dysfunction.) It may be congenital or acquired. The trauma of intercourse and especially of childbirth may lead to periurethral fibrosis with contracture, or the stricture may be caused by the surgeon during vaginal repair. It may develop secondary to acute or chronic urethritis.

Persistent hesitancy in initiating urination and a slow urinary stream are the principal symptoms of stricture. Burning, frequency, nocturia, and urethral pain may occur from secondary urethritis or cystitis. If secondary infection of the bladder is present, pus and bacteria will be found in the urine. A fairly large catheter (22F) may pass to the bladder only with difficulty. Urethroscopy may demonstrate the point of narrowness and disclose evidence of urethritis. Cystoscopy often reveals trabeculation (hypertrophy) of the bladder wall.

Chronic cystitis may cause similar symptoms, but urinalysis reveals evidence of infection. Cancer of the urethra causes progressive narrowing of the urethra, but induration and infiltration of the urethra are found on vaginal examination. Panendoscopy with biopsy establishes the diagnosis. Bladder tumor involving the bladder neck causes hesitancy and impairment of the urinary stream. Cystoscopy and biopsy is definitive. Chronic urethritis commonly accompanies urethral stenosis; either may be primary. Recurrent or chronic cystitis is often secondary to stenosis.

Treatment consists of gradual urethral dilatation (up to 36F) at weekly intervals. Slight overstretching is necessary, since some contracture will occur after therapy is discontinued. Measures to combat urethritis and cystitis also must be employed. Internal urethrotomy has its proponents (Essenhigh, 1968). With proper overdilatation of the urethra and specific therapy of the urethritis that is usually present, the prognosis is good. Formal urethroplasty can be performed with high success rates in cases that do not respond to urethral dilations (Onol, 2011).

Bibliography