What is Fowler’s Syndrome?

Fowler’s Syndrome is a specific cause of urinary retention that affects predominantly young women. First described by Professor Clare J. Fowler and her colleagues at the National Hospital for Neurology and Neurosurgery in London in 1985, the condition results from abnormal behaviour of the urethral sphincter muscle—the muscle that normally relaxes to allow urine to flow out of the bladder 1.

In women with Fowler’s Syndrome, the sphincter fails to relax appropriately when the bladder is full. Instead, it remains contracted, effectively creating a blockage that prevents normal urination. What makes this condition particularly unusual is that patients often lack the typical sensations of bladder fullness that would normally prompt someone to find a toilet. This absent urgency means the bladder can become extremely distended without the woman being aware of it.

The condition isn’t common, but it’s probably underdiagnosed. Many women experience symptoms for years before receiving an accurate diagnosis, often being told their problems are psychological or being misdiagnosed with other conditions. Recognition has improved since Fowler’s original description, though awareness among general practitioners remains variable.

Who Gets Fowler’s Syndrome?

The typical patient is a woman in her twenties or thirties, though cases have been reported across a broader age range from teenagers to women in their fifties. The condition almost exclusively affects women—male cases are exceedingly rare.

One striking observation from clinical studies is the association with polycystic ovary syndrome (PCOS). Approximately half of women diagnosed with Fowler’s Syndrome have polycystic ovaries on ultrasound examination 2. This association suggests a possible hormonal component to the condition, though the exact relationship remains incompletely understood. Some researchers have proposed that altered hormone levels might affect the sphincter muscle’s electrical activity, but this is still being investigated.

Women with Fowler’s Syndrome often report that their urinary symptoms began around a significant life event—surgery under general anaesthesia, childbirth, or another physical or emotional trigger. Whether these events actually cause the condition or simply unmask a pre-existing tendency is unclear. In some cases, there’s no obvious precipitating factor at all.

Recognising the Symptoms

The severity of Fowler’s Syndrome varies considerably between individuals. At one end of the spectrum, some women experience complete urinary retention—they simply cannot pass urine at all. At the other end, some women can void but incompletely, leaving a significant residual volume in the bladder after each attempt.

Complete retention often presents dramatically. A woman may suddenly find herself unable to urinate despite knowing her bladder must be full. The absence of the usual urgent sensation is disorienting—there’s fullness or discomfort rather than the familiar need to void. In some cases, women present to emergency departments unable to pass urine, and when catheterised, more than a litre of urine drains from the bladder.

Partial retention can be more insidious. Women may notice they urinate infrequently—sometimes only once or twice daily despite normal fluid intake. The urinary stream may be hesitant or intermittent rather than steady. There’s often a sense of incomplete emptying, though without the intense urgency that usually accompanies a full bladder. Over time, the bladder may become chronically overdistended.

Recurrent urinary tract infections plague many women with Fowler’s Syndrome. When urine pools in the bladder rather than being regularly flushed out, bacteria have an opportunity to multiply. Women may experience repeated infections requiring antibiotic treatment, sometimes monthly or even more frequently. These recurrent UTIs can be the presenting complaint that eventually leads to diagnosis.

Pain is variable. Some women experience significant lower abdominal discomfort or back pain as the bladder distends. Others have surprisingly little pain even with large retained volumes. Suprapubic tenderness—discomfort just above the pubic bone—is common when the bladder is palpably enlarged.

What Causes the Sphincter to Malfunction?

The underlying cause of Fowler’s Syndrome isn’t fully understood, even decades after its initial description. What we do know is that the urethral sphincter shows abnormal electrical activity when tested with a specialised technique called sphincter electromyography (EMG).

In healthy women, the urethral sphincter contracts to maintain continence and relaxes to allow urination. In Fowler’s Syndrome, EMG recordings reveal abnormal patterns characterised by complex repetitive discharges and decelerating bursts—essentially, the sphincter muscle is overactive and fails to switch off when it should. These electrical abnormalities seem to increase sphincter pressure beyond what normal relaxation mechanisms can overcome.

The association with polycystic ovaries points toward a possible hormonal influence. Opioid peptides (the body’s natural pain-regulating chemicals) may also play a role, as the sphincter muscle contains opioid receptors. Some researchers have suggested that altered sensitivity to these natural opioids could contribute to the sphincter’s abnormal behaviour.

What’s notable is that Fowler’s Syndrome is not a neurogenic bladder condition in the traditional sense—the nerves themselves appear to be intact. It’s also distinct from conditions like pelvic floor dysfunction where the problem lies with voluntary muscle coordination.

Getting a Diagnosis

Diagnosis requires a combination of clinical assessment and specialised testing. The clinical picture—young woman with urinary retention, absent or reduced bladder sensation, no obvious neurological disease—should prompt consideration of Fowler’s Syndrome.

Bladder ultrasound after attempting to void shows the post-void residual volume. Women with Fowler’s Syndrome typically retain substantial amounts—often several hundred millilitres or more. Serial measurements may show a pattern of chronic incomplete emptying.

Urodynamic studies assess bladder function during filling and attempted voiding. In Fowler’s Syndrome, these tests typically show a bladder that can hold large volumes without generating the normal urge to void. The detrusor muscle (the bladder’s muscular wall) may contract poorly or not at all, though this is thought to be secondary to chronic overdistension rather than a primary problem.

Sphincter EMG is the key diagnostic test. Using a specialised concentric needle electrode inserted into the urethral sphincter, clinicians can record the muscle’s electrical activity. The characteristic finding is complex repetitive discharges and decelerating bursts—patterns that sound like whale song when amplified through a speaker. This EMG pattern is highly specific to Fowler’s Syndrome and helps distinguish it from other causes of retention 3.

MRI scanning of the spine and brain is usually performed to rule out neurological conditions that could cause similar symptoms, such as spinal cord problems or multiple sclerosis. In Fowler’s Syndrome, these scans are normal.

Treatment Options

Management depends on the severity of symptoms and the impact on quality of life.

When Intervention Isn’t Needed

For women with mild incomplete emptying who aren’t experiencing infections or significant discomfort, watchful waiting may be appropriate. Regular monitoring ensures any deterioration is caught early, but active treatment isn’t always necessary if the bladder is coping reasonably well.

Intermittent Self-Catheterisation

For women with significant retention, clean intermittent self-catheterisation (CISC) is often the initial management approach. This involves passing a thin catheter through the urethra to drain the bladder, typically four to six times daily. While it sounds daunting initially, most women can learn the technique and incorporate it into their routines.

CISC prevents the bladder from becoming dangerously overdistended, reduces the risk of urinary tract infections (by ensuring stagnant urine doesn’t accumulate), and protects kidney function. It’s effective but doesn’t treat the underlying sphincter problem—it’s a management strategy rather than a cure.

Some women find CISC difficult or unacceptable as a long-term solution, which has driven interest in more definitive treatments.

Sacral Nerve Stimulation

The most exciting development in Fowler’s Syndrome treatment has been sacral nerve stimulation (also called sacral neuromodulation). This involves implanting a small device—similar to a pacemaker—that delivers mild electrical impulses to the sacral nerves controlling bladder function.

The treatment was initially developed for overactive bladder but was found to be remarkably effective for urinary retention in Fowler’s Syndrome. Success rates in carefully selected patients are impressive—studies show that 70-80% of women with Fowler’s Syndrome can regain the ability to void spontaneously after sacral nerve stimulation 2.

The procedure typically involves two stages. First, a trial stimulation period using temporary electrodes placed near the sacral nerves. If this trial shows significant improvement in bladder function (usually assessed over one to two weeks), the permanent stimulator is implanted. The device sits beneath the skin, usually in the upper buttock area, and can be adjusted externally to optimise its effect.

Exactly how sacral nerve stimulation works in Fowler’s Syndrome isn’t entirely clear. It may help by modulating the abnormal sphincter activity, by restoring bladder sensation, or through some combination of effects. What matters to patients is that it often works when other treatments haven’t.

Other Treatments

Alpha-blocker medications, which relax smooth muscle in the urinary tract, are sometimes tried but generally have limited effectiveness in Fowler’s Syndrome. Botulinum toxin injections into the urethral sphincter have been attempted with variable results—some women improve, but the effect is temporary and the procedure has to be repeated.

Urethral dilation and other mechanical interventions aren’t appropriate for Fowler’s Syndrome, as the problem is functional rather than anatomical.

Living with Fowler’s Syndrome

Beyond the physical aspects of treatment, living with Fowler’s Syndrome can be challenging. The condition often affects women at a time of life when they’re establishing careers, relationships, and perhaps considering starting families. The need for regular catheterisation or the presence of an implanted device requires adjustment.

Support from specialist continence services can be invaluable. These teams include nurses experienced in teaching catheterisation techniques, providing psychological support, and helping women navigate the practical aspects of managing their condition.

Pregnancy and childbirth require special consideration for women with Fowler’s Syndrome. Sacral nerve stimulators are typically turned off during pregnancy as a precaution, and delivery plans need to account for potential bladder management issues. Most women can have successful pregnancies with appropriate planning and monitoring.

When to Seek Medical Help

Young women experiencing urinary retention or difficulty emptying their bladder should seek medical evaluation. Symptoms that should prompt assessment include:

Infrequent urination (less than four times daily) despite normal fluid intake
Weak or hesitant urinary stream
Sense of incomplete bladder emptying
Recurrent urinary tract infections
Lower abdominal discomfort or swelling
Complete inability to pass urine (this requires urgent assessment)

If you’re experiencing these symptoms, asking your GP for referral to a urologist or urogynaecologist with experience in female voiding dysfunction is worthwhile. Fowler’s Syndrome remains underdiagnosed, and many women benefit from assessment at specialist centres familiar with the condition.

Summary

Fowler’s Syndrome represents an important and treatable cause of urinary retention in young women. Though relatively rare, it’s probably more common than diagnostic rates suggest. The condition results from abnormal urethral sphincter behaviour rather than nerve damage, and the characteristic EMG findings help distinguish it from other causes of retention.

Treatment has advanced considerably since the condition was first described. While intermittent self-catheterisation remains an effective management option, sacral nerve stimulation offers the possibility of restored spontaneous voiding for many women. With appropriate diagnosis and treatment, most women with Fowler’s Syndrome can achieve good bladder function and quality of life.

References

Fowler CJ, Christmas TJ, Chapple CR, et al. Abnormal electromyographic activity of the urethral sphincter, voiding dysfunction, and polycystic ovaries: a new syndrome? BMJ. 1988;297(6661):1436-1438. PubMed
De Ridder D, Ost D, Bruyninckx F. The presence of Fowler’s syndrome predicts successful long-term outcome of sacral nerve stimulation in women with urinary retention. Eur Urol. 2007;51(1):229-234. PubMed
NHS. Urinary retention - Overview. National Health Service. NHS