Welcome to the Department of Uro-neurology information page on Fowler’s Syndrome. This website was setup in response to requests for information to be made available to patients with this condition.
There is continuous research going on into Fowler’s syndrome but apart from academic medical journals and some urological centres there is no easy way to communicate what is known about this condition to patients.
This website was setup by two research registrars at the National Hospital of Neurology and Neurosurgery Drs. Sam Datta and Rajesh Kavia, and put together by Sidhartha Datta. The aim of this website is to provide information about Fowler’s Syndrome and how to cope with the diagnosis.
Professor Clare J Fowler, MB, BS MSc FRCP is a Professor of Uro-neurology at the National Hospital of Neurology & Neurosurgery and first described the syndrome in 1985, she has continued to work on patients’ problems ever since.
She has unique first hand experience in the diagnosis and treatment of this challenging condition, but acknowledges that there is still a lot to understand.
What is Fowler’s Syndrome?
First described in 1985, it is a cause of urinary retention (inability to pass water normally) in young women. Urinary retention in young women is not common but can be quite debilitating. The abnormality lies in the urethral sphincter (the muscle that keeps you continent). The problem is caused by the sphincter’s failure to relax to allow urine to be passed normally. There is no neurological disorder associated with the condition. Up to half the women have associated polycystic ovaries.
What sort of symptoms do patients present with?
The typical woman who is seen with the condition is in her 20-30s and may infrequently pass urine with an intermittent stream. The normal sensation of urinary urgency expected with a full bladder are not present but as the bladder reaches capacity there may be pain and discomfort, and she finds that she is not able to pass urine. This can happen spontaneously or following an operative procedure (gynaecological, urological or even ENT) or following childbirth.
Classically, the woman presents to the hospital as they have been unable to pass urine for many hours and a catheter (tube that drains the bladder) is inserted, and usually over a litre is drained with consequent relief of the pain.
If the retention occurs after an operation in hospital, urinary retention may occur during the night after the operation when the patient develops pain over their bladder.
Initial hospital management is carried out by the urology team at the local hospital but if the symptoms do not resolve, the patient maybe referred on.
There is a spectrum in the severity of the condition, with some patients being able to pass urine with difficulty but leaving significant amounts, and some not being able to pass any (complete retention).
Many women who are not in complete retention, may present to they doctors complaining of recurrent cystitis (bladder infections) or even kidney infections.
What causes these symptoms?
Most of symptoms of Fowler’s Syndrome are caused by inability to empty the urine that is stored in the bladder.
Some women may experience back pain, suprapubic pain (pain over the bladder) or dysuria (discomfort/burning whilst passing urine) due the urinary infections.
The cause and process which gives rise to Fowler’s Syndrome is not known and is still under research.
How do you diagnose the condition?
The key diagnostic test for the condition is a Sphincter Electromyogram (EMG). However, this is somewhat uncomfortable since a needle must be used to record from the sphincter and needs specialist expertise and equipment. Other tests that may be carried out which indicate the diagnosis is likely include flow rate, residual volume, urethral pressure profile and ultrasound sphincter volume. These tests depend on whether you pass urine.
What are the tests? What happens? What do they tell us?
If you can pass urine, you sit normally as you would at home but on a special toilet that measures the speed of your stream and how long it takes you to pass urine. The computer measures the rate of flow of urine and draws a graph. We can use this to see if your stream is interrupted and how bad it is. The test is easy to perform and is totally non-invasive.
This test, carried out using an ultrasound scanner, gives useful information on how well the bladder is emptying. This scan measures the amount of urine left in your bladder after you have been to the toilet. Normally, the bladder should be completely empty after passing urine.
This test, sometimes also called “cystometry” is more useful if you cannot pass urine and involves placing two small catheters (tubes), one in the bladder and one in the rectum (back passage). The bladder is slowly filled with saline (salt water) and is monitored for any irregular spasms. Once, the bladder is full, you are asked to pass urine with the catheters in. This gives information on what pressure the bladder muscle generates for a particular urine flow rate.
This test takes 30-40 minutes and may cause a little discomfort on insertion of the catheters. However once the catheters are inserted, it is fairly painless.
Urethral Pressure Profile
Whilst you lie on your back, a catheter is inserted into the urethra (similar to that used in the cystometry) and saline is infused slowly through the catheter. The catheter is then withdrawn and re-inserted (six times) into the bladder whilst the pressure of the urethral sphincter is measured. This test gives information on how much pressure is generated by the sphincter, and thus how overactive the muscle is.
Ultrasound Sphincter Volume Measurement
The volume of the urethral sphincter (the muscle that keeps one continent in the normal state) is measure using ultrasound. A small probe is placed in the vagina, and the sphincter is identified. Measurements are then made and the volume calculated. It can be a little uncomfortable on insertion of the probe, but once the sphincter is found, most patients do not find it too bothersome. An overactive sphincter may enlarge due to continuous ‘muscle activity’.
Sphincter Electromyogram (EMG)
This is the gold standard test for Fowler’s syndrome, and is done to confirm our other findings. Professor Fowler usually performs this test within the department.
With the patient lying on their back, local anaesthetic is injected into the sphincter region. A small needle is then used to take recordings from the sphincter. The area from which the needle takes the recording is very small (1mm³). (It is quite a complex and tricky test sometimes).
Characteristic waveforms and sounds can be identified using this technique. The abnormality in Fowler’s syndrome is a complex repetitive discharge and decelerating bursts, but to the non specialist, it is the sound of ‘helicopters’ and ‘whales’.
What happens to me once the diagnosis is made?
Fowler’s syndrome is a condition which is slowly being understood. There is no absolute cure for the condition yet. The aim of treatment is to try and ensure bladder emptying.
Bladder function may spontaneously recover in some patients, especially in the group in whom the problems started after childbirth.
In patients with little recovery, it can be a lifelong condition which can cause significant impact to quality of life.
At the National Hospital, we have a specialist team of doctors, nurses and continence advisors to help manage your condition long term. There are various treatments that are used to regain control and overcome the symptoms.
Are there any treatments?
Currently the treatments for Fowler’s syndrome are being researched and developed. Depending on the severity of the condition, there are various but limited options.
Often patients have a poor urine stream but can still void almost normally. In these patients, we monitor their residual volume. If they are low, no intervention is necessary.
Some patients have a large residual volume which gives rise to urinary infections and a large bladder. These patients are helped by regular clean intermittent catherisation (putting a sterile catheter into the bladder at regular intervals to empty the bladder).
The most severe patients, those in complete retention may be candidates for sacral nerve stimulation, which is the only treatment shown to restore voiding. However this requires major efforts by the patient, is expensive, often troubled by operative difficulties and cannot be regarded as a "good fix".
What is intermittent self catheterisation?
This is where you as the patient will insert a catheter (tube) into your bladder to empty it. This is done at regular intervals to ensure there is not a stagnant volume of urine, which can give rise to infections. Our continence advisors can teach this technique, and suggest ways to make it as easy as possible. They also can help arrange to put you in contact with suppliers of the most suitable catheters for your convenience.
The procedure is not too uncomfortable, however many women do complain that although it is easy to insert the catheter, removal is painful and that the catheter gets ‘stuck’ when attempting to withdraw it.
What is Sacral Nerve Stimulation?
Sacral nerve stimulation (SNS) is a process whereby small electrical pulses stimulate nerves in the lower back (just above the tail bone [sacrum]). The nerves stimulated are those involved in control of bladder sensation. How the stimulation works at restoring voiding is still under research.
If you are deemed eligible for SNS, then the first stage is to test stimulation, peripheral nerve evaluation (PNE). A temporary lead is inserted into the 3rd sacral foramen (naturally occurring hole in the lower spine) using an external stimulator (looks similar to pager) and the response is assessed for 3 to 7 days. You will need to keep a diary to record your symptoms. In approximately two thirds of the women there will be a restoration of voiding.
If bladder function is improved then the patient will be placed on the waiting list for a staged SNS implant. The implant is the InterStim Therapy® produced by Medtronic® (more information about the InterStim implant is available at www.medtronic.com).
The staged operation involves the placement of a permanent electrode (see right), either under local or general anaesthetic. This is connected to an external stimulator source and the response assessed for 4 weeks. If the lead placement results in good voiding with minimal side effects then the stimulator is internalised. It is thought that this second longer period of test stimulation with the permanent lead should reduce the future complications and improve success rates. The stimulator is usually placed in the buttock or abdomen.
Why may I not be considered for SNS?
If you live a great distance form the National Hospital or you find the journey to the hospital difficult then we may not consider you for the treatment, as on average you would be required to be seen at the hospital four times a year.
You may not be considered for SNS if the test stimulation is unsuccessful.
What options have I got if SNS is not possible and I cannot self catheterise?
If you are unable to catheterise and SNS is not an option, then a long term catheter may be required. This may be a permanent tube that drains your bladder either via your urethra or via a suprapubic catheter (a tube placed below you belly button, into your bladder). For more information see attached article.
Very occasionally, if you are in complete retention you may be offered more radical procedures, such as removal of the bladder (these may sound attractive but are not short of complications or suitable for all).
This technique has showed some good results with many not needing to catheterise. However it is not always easy to get a good result and some patients have found over time that they have run into problems with batteries needing replacement, reduced effect and needing to catheterise or pain down the leg.
Who can I see for help?
- General Practitioner – will be able to advise or refer patients.
- Continence Adviser – teach clean self intermittent catherisation techniques, advice on catheters, moral support.
- Consultant Urologist – local urologist may have experience on Fowler’s Syndrome may refer on to Specialist unit such as National Hospital.
- National Hospital for Neurology & Neurosurgery – referral for diagnosis and help with management can be obtained here.
Fowler's Syndrome website - http://www.fowlersyndrome.co.uk/
Fowler’s Forum – Link to patient’s only forum on fowler’s syndrome.
Web Link to Medtronic site
Web link to Bladder and Bowel Foundation
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Anaesthesia: A substance that prevents pain from being felt, given before an operation.
Anus: The opening of the rectum where solid waste leaves the body.
Bladder: The muscular bag in the lower abdomen where urine is stored.
Catheter: A tube inserted through the urethra to the bladder in order to drain urine from the body.
Cystoscope: A tube-like instrument used to view the interior of the bladder.
Hormone: A substance that stimulates the function of a gland.
Incontinence: The inability to control urination.
Obstruction: A clog or blockage that prevents liquid from flowing easily.
Rectum: The last part of the large intestine (colon) ending in the anus, often termed ‘back passage’.
Reproductive system: The bodily systems that allow men and women to have children.
Retropubic: Behind the pubic bone.
Suprapubic: Above the pubic bone.
Ultrasound: A type of test in which sound waves too high to hear are aimed at a structure to produce an image of it.
Urinary tract: The path that urine takes as it leaves the body. It includes the kidneys, ureters, bladder, and urethra.
Urination: Discharge of liquid waste from the body.
Urethra: The canal through which urine passes through as it leaves the body.