Fowler's syndrome

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Fowler's syndrome
Other namesUrethral sphincter relaxation disorder
Specialty Uro-neurology

Fowler's syndrome (urethral sphincter relaxation disorder) is a rare disorder in which the urethral sphincter fails to relax to allow urine to be passed normally in younger women with abnormal electromyographic activity detected. [1] [2] [3] [4]

Contents

Presentation

Urinary retention is a relatively uncommon presentation in young women. Fowler's syndrome primarily presents in women between menarche and menopause. The peak age of onset is 26 yrs. [5] It is seen in about one third of the women who experience urinary retention. The predominant complaint is the inability to urinate for a day or more with no urgency to urinate, in spite of a large bladder volume of more than 1 liters. Normally a person feels the need to urinate at a bladder volume of 400-500ml. The person usually has a progressively increasing lower abdominal pain. The condition can be associated with Polycystic ovary Syndrome and Endometriosis. [5]

Alternatively, women with Fowler's syndrome can present with impaired voiding, voiding difficulties with or without incomplete bladder emptying, may have increased urinary frequency and occasionally impairment in urination and increased frequency of urination, but rarely become incontinent. [5] [6]

Women with Fowler's syndrome often find catheterisation extremely painful. [7] [8]

Fowler's syndrome can be a disabling condition. 50% of women with Fowler's syndrome suffer from unexplained chronic pain, including chronic abdominopelvic, back, leg, or widespread pain. [9]

Women with Fowler's syndrome can suffer lifelong with debilitating effects on quality of life. [5]

Cause

The exact cause of Fowler's syndrome is not yet known. [4]

It may occur spontaneously, or following an event such as a surgical procedure or childbirth. Use of opiates also trigger urinary retention. [5]

There is not usually any prior history of urological abnormalities in childhood. [5]

One hypothesis is that it is due to an abnormality in muscle membrane, possibly hormonally dependent channelopathy. [10] causing excessive excitability of the external urethral sphincter which prevents the adequate relaxation of the muscle necessary for voiding . [11]

Another hypothesis in that Fowler's syndrome is due to an up-regulation of spinal cord enkephalins and that opiates may compound the functional abnormalities. [12]

It has also been hypothesised that there are both local pelvic floor and central neurological causations. [13]

Diagnosis

Urodynamic testing including Cystometry and Urethral Pressure Profilometry.

Women with Fowler's syndrome are often found to have an abnormally elevated urethral pressure profile, increased urethral sphincter volume. [2] [14]

The diagnosis is done by testing the Electromyography (EMG) of the external striated urethral sphincter.[ citation needed ]

Women with Fowler's syndrome characteristically show abnormal electromyography of the urethral sphincter. The usual findings are complex repetitive discharges without and with deceleration (decelerating bursts), suggesting an impairment in sphincter muscle relaxation. [15] [10]

Treatment

Sacral neuromodulation is the only treatment that has been found to restore voiding in women with Fowler's syndrome. It delivers an electric current to the neural reflexes associated with lower urinary tract function via stimulation of the S3 spinal nerve root. [4]

Although success rate is about 70%, there can be complications and it has a relatively high re-intervention rate. [7] [16] [17]

Sacral Neuromodulation is thought to work because the sensory parts of the brain (the periaqueductal grey) which receives sensory signals from the Lower Urinary Tract becomes activated in women with Fowler's syndrome when the device is switched on; the neuromodulation overriding the negative feedback from the sacral nerves. [18] [19]

Other treatment options are:

History

This disease was described first by Fowler et al. in 1985. [21]

Related Research Articles

<span class="mw-page-title-main">Interstitial cystitis</span> Medical condition

Interstitial cystitis (IC), a type of bladder pain syndrome (BPS), is chronic pain in the bladder and pelvic floor of unknown cause. It is the urologic chronic pelvic pain syndrome of women. Symptoms include feeling the need to urinate right away, needing to urinate often, and pain with sex. IC/BPS is associated with depression and lower quality of life. Many of those affected also have irritable bowel syndrome and fibromyalgia.

<span class="mw-page-title-main">Urology</span> Medical specialty

Urology, also known as genitourinary surgery, is the branch of medicine that focuses on surgical and medical diseases of the urinary-tract system and the reproductive organs. Organs under the domain of urology include the kidneys, adrenal glands, ureters, urinary bladder, urethra, and the male reproductive organs.

<span class="mw-page-title-main">Urethra</span> Tube that connects the urinary bladder to the external urethral orifice

The urethra is a tube that connects the mammalian urinary bladder to the urinary meatus. Male and female placental mammals release urine through the urethra during urination, but males also release semen through the urethra during ejaculation.

<span class="mw-page-title-main">Benign prostatic hyperplasia</span> Noncancerous increase in size of the prostate gland

Benign prostatic hyperplasia (BPH), also called prostate enlargement, is a noncancerous increase in size of the prostate gland. Symptoms may include frequent urination, trouble starting to urinate, weak stream, inability to urinate, or loss of bladder control. Complications can include urinary tract infections, bladder stones, and chronic kidney problems.

<span class="mw-page-title-main">Urination</span> Release of urine from the urinary bladder

Urination is the release of urine from the bladder to the outside of the body. Urine is released from the urethra through the penis or vulva in placental mammals and through the cloaca in other vertebrates. It is the urinary system's form of excretion. It is also known medically as micturition, voiding, uresis, or, rarely, emiction, and known colloquially by various names including peeing, weeing, pissing, and euphemistically going number one. The process of urination is under voluntary control in healthy humans and other animals, but may occur as a reflex in infants, some elderly individuals, and those with neurological injury. It is normal for adult humans to urinate up to seven times during the day.

<span class="mw-page-title-main">Urinary incontinence</span> Uncontrolled leakage of urine

Urinary incontinence (UI), also known as involuntary urination, is any uncontrolled leakage of urine. It is a common and distressing problem, which may have a large impact on quality of life. It has been identified as an important issue in geriatric health care. The term enuresis is often used to refer to urinary incontinence primarily in children, such as nocturnal enuresis. UI is an example of a stigmatized medical condition, which creates barriers to successful management and makes the problem worse. People may be too embarrassed to seek medical help, and attempt to self-manage the symptom in secrecy from others.

<span class="mw-page-title-main">Onuf's nucleus</span> Group of neurons

Onuf's nucleus is a distinct group of neurons located in the ventral part of the anterior horn of the sacral region of the human spinal cord involved in the maintenance of micturition and defecatory continence, as well as muscular contraction during orgasm. It contains motor neurons, and is the origin of the pudendal nerve. The sacral region of the spinal cord is the fourth segment of vertebrae in the spinal cord which consists of the vertebrae 26-30. While working in New York City in 1899, Bronislaw Onuf-Onufrowicz discovered this group of unique cells and originally identified it as “Group X.” “Group X” was considered distinct by Onufrowicz because the cells were different in size from the surrounding neurons in the anterolateral group, suggesting that they were independent.

<span class="mw-page-title-main">Urinary retention</span> Inability to completely empty the bladder

Urinary retention is an inability to completely empty the bladder. Onset can be sudden or gradual. When of sudden onset, symptoms include an inability to urinate and lower abdominal pain. When of gradual onset, symptoms may include loss of bladder control, mild lower abdominal pain, and a weak urine stream. Those with long-term problems are at risk of urinary tract infections.

<span class="mw-page-title-main">Bladder sphincter dyssynergia</span> Medical condition

Bladder sphincter dyssynergia is a consequence of a neurological pathology such as spinal injury or multiple sclerosis which disrupts central nervous system regulation of the micturition (urination) reflex resulting in dyscoordination of the detrusor muscles of the bladder and the male or female external urethral sphincter muscles. In normal lower urinary tract function, these two separate muscle structures act in synergistic coordination. But in this neurogenic disorder, the urethral sphincter muscle, instead of relaxing completely during voiding, dyssynergically contracts causing the flow to be interrupted and the bladder pressure to rise.

Neurogenic bladder dysfunction, often called by the shortened term neurogenic bladder, refers to urinary bladder problems due to disease or injury of the central nervous system or peripheral nerves involved in the control of urination. There are multiple types of neurogenic bladder depending on the underlying cause and the symptoms. Symptoms include overactive bladder, urinary urgency, frequency, incontinence or difficulty passing urine. A range of diseases or conditions can cause neurogenic bladder including spinal cord injury, multiple sclerosis, stroke, brain injury, spina bifida, peripheral nerve damage, Parkinson's disease, or other neurodegenerative diseases. Neurogenic bladder can be diagnosed through a history and physical as well as imaging and more specialized testing. In addition to symptomatic treatment, treatment depends on the nature of the underlying disease and can be managed with behavioral changes, medications, surgeries, or other procedures. The symptoms of neurogenic bladder, especially incontinence, can severely degrade a person's quality of life.

<span class="mw-page-title-main">Detrusor muscle</span> Muscle of the bladder which expels urine when it contracts

The detrusor muscle, also detrusor urinae muscle, muscularis propria of the urinary bladder and muscularis propria, is smooth muscle found in the wall of the bladder. The detrusor muscle remains relaxed to allow the bladder to store urine, and contracts during urination to release urine. Related are the urethral sphincter muscles which envelop the urethra to control the flow of urine when they contract.

<span class="mw-page-title-main">Overactive bladder</span> Condition where a person has a frequent need to urinate

Overactive bladder (OAB) is a common condition where there is a frequent feeling of needing to urinate to a degree that it negatively affects a person's life. The frequent need to urinate may occur during the day, at night, or both. Loss of bladder control may occur with this condition. This condition is also sometimes characterized by a sudden and involuntary contraction of the bladder muscles, in response to excitement or anticipation. This in turn leads to a frequent and urgent need to urinate.

<span class="mw-page-title-main">Retrograde urethrogram</span> Medical imaging of the urethra

A retrograde urethrography is a routine radiologic procedure used to image the integrity of the urethra. Hence a retrograde urethrogram is essential for diagnosis of urethral injury, or urethral stricture.

Sacral nerve stimulation, also termed sacral neuromodulation, is a type of medical electrical stimulation therapy.

Lower urinary tract symptoms (LUTS) refer to a group of clinical symptoms involving the bladder, urinary sphincter, urethra and, in men, the prostate. The term is more commonly applied to men – over 40% of older men are affected – but lower urinary tract symptoms also affect women. The condition is also termed prostatism in men, but LUTS is preferred.

Urologic diseases or conditions include urinary tract infections, kidney stones, bladder control problems, and prostate problems, among others. Some urologic conditions do not affect a person for that long and some are lifetime conditions. Kidney diseases are normally investigated and treated by nephrologists, while the specialty of urology deals with problems in the other organs. Gynecologists may deal with problems of incontinence in women.

<span class="mw-page-title-main">Overflow incontinence</span> Medical condition

Overflow incontinence is a concept of urinary incontinence, characterized by the involuntary release of urine from an overfull urinary bladder, often in the absence of any urge to urinate. This condition occurs in people who have a blockage of the bladder outlet, or when the muscle that expels urine from the bladder is too weak to empty the bladder normally. Overflow incontinence may also be a side effect of certain medications.

Dyssynergia is any disturbance of muscular coordination, resulting in uncoordinated and abrupt movements. This is also an aspect of ataxia. It is typical for dyssynergic patients to split a movement into several smaller movements. Types of dyssynergia include Ramsay Hunt syndrome type 1, bladder sphincter dyssynergia, and anal sphincter dyssynergia.

<span class="mw-page-title-main">Artificial urinary sphincter</span> Medical device

An artificial urinary sphincter (AUS) is an implanted device to treat moderate to severe stress urinary incontinence, most commonly in men. The AUS is designed to supplement the function of the natural urinary sphincter that restricts urine flow out of the bladder.

Prof Clare Fowler CBE is a British physician and academic who created the subspecialty of uro-neurology, a medical field that combines urology and neurology. This work was done at the Institute of Neurology, University College London, where she is an emeritus professor.

References

  1. ↑ "2 Indications and current treatments | Sacral nerve stimulation for idiopathic chronic non-obstructive urinary retention | Guidance | NICE". www.nice.org.uk. 25 November 2015. Retrieved 29 March 2021.
  2. 1 2 Panicker, Jalesh N.; Pakzad, Mahreen; Fowler, Clare J. (2018). "Fowler's syndrome: a primary disorder of urethral sphincter relaxation". The Obstetrician & Gynaecologist. 20 (2): 95–100. doi:10.1111/tog.12448. ISSN   1744-4667. S2CID   79643408. Archived from the original on 14 November 2021. Retrieved 29 March 2021.
  3. ↑ Wein, Alan J. (2012), "Pathophysiology and Classification of Lower Urinary Tract Dysfunction", Campbell-Walsh Urology, Elsevier, pp. 1834–1846.e1, doi:10.1016/b978-1-4160-6911-9.00061-x, ISBN   978-1-4160-6911-9
  4. 1 2 3 "University College London Queens Square Institute of Neurology: Fowler's syndrome". www.ucl.ac.uk. 29 January 2018. Retrieved 29 March 2021.
  5. 1 2 3 4 5 6 Hussain, Dr R. (24 August 2016). "Fowler's syndrome Meeting 17th August 2016". Pennine GP Learning Group. Retrieved 29 March 2021.
  6. ↑ Panicker (2016). "Fowler's syndrome and Chronic Urinary Retention in Women: Fowler's syndrome- a review" (PDF). University College London.
  7. 1 2 3 Panicker, Jalesh N; Pakzad, Mahreen; Fowler, Clare J (April 2018). "Fowler's syndrome: a primary disorder of urethral sphincter relaxation" (PDF). The Obstetrician & Gynaecologist. 20 (2): 95–100. doi:10.1111/tog.12448. S2CID   79643408.
  8. ↑ Jn, Panicker; X, Game; S, Khan; Tm, Kessler; G, Gonzales; S, Elneil; Cj, Fowler (August 2012). "The Possible Role of Opiates in Women With Chronic Urinary Retention: Observations From a Prospective Clinical Study". The Journal of Urology. 188 (2): 480–4. doi:10.1016/j.juro.2012.04.011. PMID   22704100.
  9. ↑ Hoeritzauer, Ingrid; Stone, Jon; Fowler, Clare; Elneil‐Coker, Suzy; Carson, Alan; Panicker, Jalesh (2016). "Fowler's syndrome of urinary retention: A retrospective study of co-morbidity". Neurourology and Urodynamics. 35 (5): 601–603. doi:10.1002/nau.22758. ISSN   1520-6777. PMID   25865606. S2CID   1070270.
  10. 1 2 Tawadros, Cecile; Burnett, Katherine; Derbyshire, Laura F.; Tawadros, Thomas; Clarke, Noel W.; Betts, Christopher D. (September 2015). "External urethral sphincter electromyography in asymptomatic women and the influence of the menstrual cycle". BJU International. 116 (3): 423–431. doi: 10.1111/bju.13042 . PMID   25600712.
  11. ↑ K, Jurkat-Rott; H, Lerche; N, Mitrovic; F, Lehmann-Horn (September 1999). "Teaching Course: Ion Channelopathies in Neurology". Journal of Neurology. 246 (9): 758–63. doi:10.1007/s004150050451. PMID   10525971. S2CID   18724264.
  12. ↑ Panicker, Jalesh N.; Game, Xavier; Khan, Shahid; Kessler, Thomas M.; Gonzales, Gwen; Elneil, Sohier; Fowler, Clare J. (1 August 2012). "The Possible Role of Opiates in Women with Chronic Urinary Retention: Observations from a Prospective Clinical Study". The Journal of Urology. 188 (2): 480–484. doi:10.1016/j.juro.2012.04.011. ISSN   0022-5347. PMID   22704100.
  13. ↑ Swash, Michael (2021). "Fowler's syndrome: What it is and what it's not" (PDF). Pelviperineology. 39 (4): 107–114. doi:10.34057/PPj.2020.39.04.002. S2CID   238703588.
  14. ↑ Wiseman, Oliver J.; Swinn, Michael J.; Brady, Ciaran M.; Fowler, Clare J. (March 2002). "Maximum Urethral Closure Pressure and Sphincter Volume in Women with Urinary Retention". Journal of Urology. 167 (3): 1348–1352. doi:10.1016/S0022-5347(05)65297-4. ISSN   0022-5347. PMID   11832729.
  15. ↑ Cj, Fowler; Rs, Kirby (February 1985). "Abnormal Electromyographic Activity (Decelerating Burst and Complex Repetitive Discharges) in the Striated Muscle of the Urethral Sphincter in 5 Women With Persisting Urinary Retention". British Journal of Urology. 57 (1): 67–70. doi:10.1111/j.1464-410x.1985.tb08988.x. PMID   4038618.
  16. ↑ Mj, Swinn; Nd, Kitchen; Rj, Goodwin; Cj, Fowler (October 2000). "Sacral Neuromodulation for Women With Fowler's syndrome". European Urology. 38 (4): 439–43. doi:10.1159/000020321. PMID   11025383. S2CID   46839550.
  17. ↑ Jk, Szymański; A, Słabuszewska-Jóźwiak; K, Zaręba; G, Jakiel (December 2019). "Neuromodulation - A Therapeutic Option for Refractory Overactive Bladder. A Recent Literature Review". Videosurgery and Other Miniinvasive Techniques. 14 (4): 476–485. doi:10.5114/wiitm.2019.85352. PMC   6939208 . PMID   31908692.
  18. ↑ Kavia, Rajesh; DasGupta, Ranan; Critchley, Hugo; Fowler, Clare; Griffiths, Derek (2010). "A functional magnetic resonance imaging study of the effect of sacral neuromodulation on brain responses in women with Fowler's syndrome". BJU International. 105 (3): 366–372. doi:10.1111/j.1464-410X.2009.08819.x. ISSN   1464-410X. PMID   19735259. S2CID   25646995.
  19. ↑ Hoeritzauer, I.; Phé, V.; Panicker, J. N. (1 January 2016), Hallett, Mark; Stone, Jon; Carson, Alan (eds.), "Chapter 38 - Urologic symptoms and functional neurologic disorders", Handbook of Clinical Neurology, 139, Elsevier: 469–481, doi:10.1016/B978-0-12-801772-2.00038-2, ISBN   9780128017722, PMID   27719863 , retrieved 31 March 2021
  20. ↑ Fenner, Annette (December 2015). "Botox injections are effective for Fowler's syndrome". Nature Reviews. Urology. 12 (12): 653. doi: 10.1038/nrurol.2015.258 . ISSN   1759-4820. PMID   26481577. S2CID   8058224.
  21. ↑ Cj, Fowler; Tj, Christmas; Cr, Chapple; Hf, Parkhouse; Rs, Kirby; Hs, Jacobs (3 December 1988). "Abnormal Electromyographic Activity of the Urethral Sphincter, Voiding Dysfunction, and Polycystic Ovaries: A New Syndrome?". BMJ. 297 (6661): 1436–1438. doi:10.1136/bmj.297.6661.1436. PMC   1835186 . PMID   3147005.
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