Fowler's syndrome is a rare cause of urinary retention in young women. In these cases, there is no other neurological or anatomical abnormality to explain their inability to pass urine, and many may be wrongly diagnosed to have psychogenic urinary retention.
The real cause is the failure of the urethral sphincter to relax. The urethral sphincter is a circular muscle which controls the release of urine from the bladder through the urethra and out of the body. It is found that women with the metabolic syndrome called polycystic ovarian syndrome (PCOS) are far more likely to have this syndrome.
Almost half of Fowler's syndrome patients are suffering from PCOS.
Fowler's syndrome is typically found in women between 20-40 years. They report the absence of the usual sensation of bladder fullness, and very infrequent passage of urine.
The sensation of pain appears only when the bladder is severely over-distended. At this point, they are still not able to urinate. Finally they report to hospital and have to have their bladder drained via a urinary catheter, with at least a liter of urine being collected.
Other urinary symptoms such as low backache, pain just above the pelvic bone and burning or pain during urination occur during urinary infections, which are more frequent in these women.
There is a spectrum of severity as far as urinary retention is concerned. Thus some women are able to pass a little urine, while others experience complete retention.
Diagnosis and treatment
Diagnosis requires eliciting the characteristic history of inability to pass urine for a day or longer, comparatively young age, female gender, the absence of the urge to urinate despite the bladder over-distension, and increasing pain or discomfort low down in the abdomen.
To exclude other causes, tests are required to evaluate the bladder function and muscular contraction. The gold standard is a test called sphincter electromyography (EMG) where a fine electrode records the activity of the tiny urethral sphincter. A characteristic pattern of activity has been found to be diagnostic of Fowler's syndrome.
Treatment of this condition is aimed at ensuring bladder emptying. This relieves the pain, helps to prevent urinary and kidney infection, and keeps the bladder wall in normal condition.
In a few cases the woman recovers, but in many cases Fowler's syndrome remains a lifelong burden. The technique of sterile self-catheterization is a workaround for such women. Here, a clean tube is passed into the bladder at regular intervals, to drain the collected urine.
Sacral neuromodulation is an expensive and difficult treatment which involves stimulation of the sacral nerve. This is the only mode of treatment that has been found effective in restoring the voiding function, but has several disadvantages.
If this is not possible, other options include permanent catheter implantation, either through the urethra or through a small hole in the bladder made just above the pubic bone (suprapubic catheterization). This drains urine into a bag which can be emptied a couple of times a day. Other surgical options are risky and limited in scope.