Fowler’s syndrome is a rare condition causing urinary retention, the inability to pass urine. It is a functional disorder where the bladder’s operations are affected, not its physical structure. First described in 1985 by Professor Clare Fowler, the condition primarily impacts young women. Because its symptoms can be misunderstood, it is sometimes incorrectly assumed to have psychological origins, but the issue lies with the muscle that controls urine release.
Primary Symptoms and Patient Profile
The defining characteristic of Fowler’s syndrome is a sudden and often painless inability to urinate. This may lead women to go a full day or longer without emptying their bladder, resulting in a visit to an emergency room. A feature is the lack of the typical sensation of bladder fullness, even as it becomes severely stretched. When drained by a catheter, it is common for over a liter of urine to be collected.
This condition presents in women between the ages of 20 and 40, often without warning. While some experience a complete inability to urinate, others are left with significant residual urine. This incomplete emptying can lead to secondary symptoms like lower back pain, recurrent urinary tract infections, and pelvic pain that can be worsened by movement.
Physiological Causes and Associated Conditions
The direct cause of urinary retention in Fowler’s syndrome is the failure of the urethral sphincter muscle to relax. This muscle acts as a valve at the bladder’s exit, and in this condition, it remains contracted, blocking the passage of urine. This is not due to a physical obstruction but an abnormality in the muscle’s activity.
The underlying trigger is not fully understood, but research has identified a link with Polycystic Ovary Syndrome (PCOS). Nearly half of the women diagnosed with Fowler’s syndrome also have PCOS, a metabolic and hormonal condition. One hypothesis suggests that hormonal changes from PCOS may affect the stability of the muscle membrane in the urethra, though this connection is still being researched.
While the association with PCOS is documented, other factors may play a role, and a specific trigger is often never identified. The condition can appear after events such as a surgical procedure, childbirth, or a significant infection. The core problem lies in abnormal electrical signals within the sphincter muscle itself, which prevent it from receiving the signal to relax.
The Diagnostic Process
Diagnosing Fowler’s syndrome begins by excluding other potential causes of urinary retention. A physician will investigate for neurological conditions or physical blockages in the urinary tract. This process rules out more common issues before considering a rarer functional disorder.
The definitive diagnostic test is a procedure called urethral sphincter electromyography (EMG). This test uses a fine needle electrode to measure the electrical activity of the urethral sphincter muscle. In patients with Fowler’s syndrome, the EMG reveals a characteristic abnormal pattern of electrical discharges, which confirms the diagnosis. This signature is sometimes described as a “whistling” sound on the EMG machine’s audio output.
Urodynamic studies are also performed to assess bladder function, including its capacity, internal pressure, and how it senses fullness. These studies often show a large bladder capacity with reduced sensation, which aligns with the symptoms. An ultrasound may also be used to measure the volume of the sphincter muscle, which can be enlarged.
Treatment and Management Strategies
The primary goal of management is to ensure the bladder is emptied regularly to prevent complications like kidney damage and recurrent infections. The most common technique is Intermittent Self-Catheterization (ISC). This procedure involves the patient inserting a clean, thin tube into the bladder at regular intervals to drain urine. ISC is an effective way to manage retention and maintain bladder health.
A more permanent solution that can restore urination is Sacral Nerve Stimulation (SNS). This therapy involves surgically implanting a small device, often called a “bladder pacemaker,” under the skin in the upper buttock area. The device sends mild electrical pulses through a thin wire to the sacral nerves that control the bladder and urethral sphincter.
These electrical pulses help modulate nerve signals, allowing the urethral sphincter to relax and enabling urination. This treatment restores voiding function in a high percentage of patients, with studies reporting success rates over 70%. The procedure can be expensive and may require revisions. Other options, like medications or pelvic floor physiotherapy, may also be considered.