Fowler's syndrome
| Fowler's Syndrome | |
|---|---|
| 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]
Presentation
Urinary retention is a relatively uncommon presentation in young women. Fowler's syndrome primarily presents in women under the age of 30 years. It is seen in about one third of women having complaints of 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.
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.
Women with Fowler's Syndrome often find catheterisation extremely painful. [5] [6]
Cause
The exact cause of Fowler's syndrome is not yet known. A possible cause is an abnormality in muscle membrane, possibly due to a hormonally dependent channelopathy.[7] This may cause an excessive excitability of the external urethral sphincter which prevents the adequate relaxation of the muscle necessary for voiding .[8]
Diagnosis
Women with Fowler's syndrome are often found to have an abnormally elevated urethral pressure profile, increased urethral sphincter volume and characteristically abnormal electromyography of the urethral sphincter [9]
Urodynamic testing Cystometry shows bladder capacity and sensations during the filling phase. The maximum urethral closure pressure (MUCP) is raised.[10]
The diagnosis is done by testing the electromyographic (EMG) activity of external striated urethral sphincter. The usual findings are complex repetitive discharges without and with deceleration (decelerating bursts), suggesting an impairment in sphincter muscle relaxation.[11][7]
Treatment
Sacral neuro modulation is the commonly practiced treatment for restoration of normal micturition. This technique involves modulation of micturition reflex by stimulating S3 nerve root.[12][13]
History
This disease was described first by Fowler et al in 1985.[14]
References
- ^ "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.
- ^ 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.
- ^ 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
- ^ "Uro-Neurology : University College London Hospitals NHS Foundation Trust". www.uclh.nhs.uk. Retrieved 2021-03-29.
- ^ 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.
- ^ 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.
- ^ 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.
- ^ 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.
- ^ 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.
- ^ 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.
- ^ 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.
- ^ 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.
- ^ 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.
- ^ 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.