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Fowler's syndrome

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Fowler's Syndrome
SpecialtyUro-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]

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 complain about 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]

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 [14].[15]

The diagnosis is done by testing the Electromyography (EMG) of the external striated urethral sphincter

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.[16][10]

Treatment

Sacral neuromodulation is the only treatment that has been found to restore voiding in women with Fowler's Syndrome. It delivers an electrical 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][17][18]

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. [19] [20]

Other treatment options are:

  • Sphincter injections of botulinum toxin [21]
  • Catheterisation. Women with Fowler's Syndrome may report difficulties in performing self catheterisation therefore an indwelling catheter such as a suprapubic catheter may be required. [7]
  • Women with Fowler's Syndrome can suffer lifelong with debilitating effects on quality of life.[5]

History

This disease was described first by Fowler et al in 1985.[22]

References

  1. ^ "2 Indications and current treatments | Sacral nerve stimulation for idiopathic chronic non-obstructive urinary retention | Guidance | NICE". www.nice.org.uk. Retrieved 2021-03-29.
  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.
  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. ^ a b c "University College London Queens Square Institute of Neurology: Fowler's Syndrome". www.ucl.ac.uk. Retrieved 2021-03-29.{{cite web}}: CS1 maint: url-status (link)
  5. ^ a b c d e f Hussain, Dr R. (2016-08-24). "Fowler's Syndrome Meeting 17th August 2016". Pennine GP Learning Group. Retrieved 2021-03-29.
  6. ^ Panicker (2016). "Fowler's Syndrome and Chronic Urinary Retention in Women: Fowler's Syndrome- a review" (PDF). University College London.{{cite web}}: CS1 maint: url-status (link)
  7. ^ a b c 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.
  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.
  10. ^ a b 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. (2012-08-01). "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 (2020). "Fowler's Syndrome: What it is and what it's not" (PDF). Pelviperineology. 39(4): 107–114.
  14. ^ 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.
  15. ^ 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.
  16. ^ 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.
  17. ^ 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.
  18. ^ 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.
  19. ^ 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.
  20. ^ Hoeritzauer, I.; Phé, V.; Panicker, J. N. (2016-01-01), Hallett, Mark; Stone, Jon; Carson, Alan (eds.), "Chapter 38 - Urologic symptoms and functional neurologic disorders", Handbook of Clinical Neurology, Functional Neurologic Disorders, vol. 139, Elsevier, pp. 469–481, retrieved 2021-03-31
  21. ^ Fenner, Annette (2015-12). "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. {{cite journal}}: Check date values in: |date= (help)
  22. ^ Cj, Fowler; Tj, Christmas; Cr, Chapple; Hf, Parkhouse; Rs, Kirby; Hs, Jacobs (1988-12-03). "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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