01 What is Fowler’s Syndrome

If you are a young woman who has developed urinary retention you may have been told you have Fowler’s syndrome.

The key clinical features are shown below:

Female The condition has never been found in young men with retention
Aged between onset of start of periods (menarche) and menopause The peak age of onset is 26
No evidence of urological disease, gynaecological or neurological disease It is important that other possible causes of urinary retention have been excluded by a urologist and/or neurologist, ideally by a cystoscopy and an MRI of the brain and spinal cord
Retention with a volume in excess of >1000 ml Retention of volumes less than 1L are unlikely to turn out to be due to Fowler’s syndrome
No sense of urinary urgency despite high bladder volumes This is a striking feature of the condition, despite having more than 1L in the bladder these girls do not have the sense of urgency one might expect. Discomfort yes, but not urgency. The explanation for this is given later in the section “what has gone wrong”
Straining does not help emptying This is also a key feature of the condition. Most girls have tried “bearing down” but not found it helped empty
Sense of “something gripping” or difficulty on removing the catheter which has been used for urinary drainage This probably contributes to the discomfort of self catheterisation, which can be quite a problem for girls with Fowler’s syndrome
No history of urological abnormalities in childhood or associated abnormalities of the urinary tract A history of bladder troubles going back into childhood needing urethral re-implants etc. probably indicates some other underlying abnormality of the bladder and urinary system. Possibly a problem with the muscle of the urinary system – “a myopathy” but this has never been proved despite the research efforts of us and others
Association with polycystic ovarian syndrome and endometriosis A history of PCO was quite a prominent part of the history in some of the early cases, but is not an inevitable feature. However it was this that gave rise to the concept that the problem is hormonally determined

The following, expands on the information given above about the clinical history of girls with Fowler’s syndrome.