Fowler's syndrome

Fowler's syndrome

• first described in 1988 as a syndrome which occurs in teenage girls and young women (1)
  • is characterized by a large bladder capacity, reduced sensation, increased maximal urethral closure pressure, and detrusor underactivity (2)
  • is described only in hormonally active women between menarche and menopause, never in males, with a peak incidence between 20 and 30 years of age
    • epidemiological studies estimate that its incidence in young women ranges from 3 cases per 100,000 per year to 0.3% after the exclusion of other causes, such as postoperative, postpartum, gynecological, urological, rectal, and psychiatric causes (2)
  • characterized by chronic urinary retention with the history of general anesthesia for varying reasons, prolonged use of opiates, or delivery
    • most of the patients reveal a trigger medical event in their history, such as gynecological surgery or other surgical procedures, childbirth, and acute medical conditions (2)
    • pathognomonic clinical signs are (1):
    • unexplained urinary retention of more than 1 litre of sterile urine on catheterization,
    • an asensitive bladder with loss of urge to void, and
    • no help of straining, poor tolerance of clean intermittent self-catheterization
      • difficulties of self-catheterization are typically described as a sense of "something gripping" the catheter inside the bladder as it is being pulled out of the bladder
  • etiology of Fowler's syndrome remains unclear (2)
    • in around 50% of the patients, there is an association with polycystic ovaries or endometriosis
  • functional retention is caused by external urethral sphincter (EUS) spasm due to vicious circle of autonomous excitation of sphincter muscle cells and failure to relax
    • EUS hyperactivity is followed by an asensitive and hypotonic bladder with permanent loss of the ability to void

Reference:

• Trachta J, Wachter J, Kriz J. Chronic Urinary Retention due to Fowler's Syndrome. European J Pediatr Surg Rep. 2018 Jan;6(1):e77-e80. doi: 10.1055/s-0038-1672147. Epub 2018 Oct 18. PMID: 30473987; PMCID: PMC6193800.
• Szymanski JK et al. Fowler's Syndrome-The Cause of Urinary Retention in Young Women, Often Forgotten, but Significant and Challenging to Treat. Int J Environ Res Public Health. 2021 Mar 23;18(6):3310. doi: 10.3390/ijerph18063310. PMID: 33806865; PMCID: PMC8005021.