Should urethral pain upon catheter insertion or withdrawal be considered pathognomonic in women with Fowler’s syndrome?

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LETTER

Hippokratia 2022, 26(1): 47

Al-Shaiji TF
Urology Unit (Head), Department of Surgery, Jaber Al-Ahmad Al-Jaber Al-Sabah Hospital, Kuwait City, Kuwait

Keywords: Fowler’s syndrome, urethral pain, clinical sign, pathognomonic

Corresponding author: Dr Tariq F. Al-Shaiji, Urology Unit (Head), Department of Surgery, Jaber Al-Ahmad Al-Jaber Al-Sabah Hospital, PO Box 34489, Adelliah, Kuwait City, Al-Asima 73255, Kuwait, tel: +96525305000, e-mail: tshaiji@gmail.com

Dear Editor,

Urinary retention can be challenging to diagnose and treat in women. Fowler et al described in 1987 complete urinary retention with a specific abnormal pattern of electromyographic (EMG) activity in young women with clinical features of polycystic ovaries, as Fowler’s syndrome (FS)1. It has been suggested that these patients can have severe urethral pain upon catheter insertion or removal as a distinct phenomenon. However, the subjectivity of pain makes this sign hard to assess or call. This short opinion describes the utility of this clinical sign in women with Flower’s syndrome.

FS is a rare entity with an estimated incidence of two cases per 10,00,000 subjects per year, and it uniquely affects females with an average age of 20-30 years2. Although the diagnosis of FS can occasionally be a classical presentation, it is a dilemma for urologists and urogynecologists. From a clinical standpoint, it was reported that those women with FS often find the urethral catheterization process excruciating, especially catheter withdrawal, with several complaining of a “something gripping” sensation3. In the era of specialized testing, this clinical sign may well be overlooked; however, not all physicians have access to these tests or resources nor have the expertise to diagnose. Subsequently, they will merely depend on clinical diagnosis.

Can this “something gripping” pain sensation be considered pathognomonic for FS? Through anecdotal experience, several FS patients have been observed experiencing distinct pain upon catheter insertion and removal compared to others, which is rational given that the etiology of FS is caused by failure of the sphincter to relax. Nevertheless, this is still a non-specific observation. It would be more reliable if all female patients presenting with urinary retention, irrespective of the underlying pathology, were encountered and observed simultaneously to document this phenomenon. There are also inherited limitations. Those index young women with urinary retention may not necessarily have the classic FS, namely Fowler’s-like syndrome, further complicating the matter. Urethral irritation (urethral syndrome) secondary to hormonal imbalances, injury, or infection can lead to the same phenomena due to an ongoing inflammatory process. The condition is uncommon, and pain is subjective. In addition, if we attempt to retrospectively study this sign using tools such as a numerical rating pain scale, this would be amenable to recall bias since the finding is self-reported, using broad pain level assessment via an observation study. As with rare diseases, selection and chronological bias could affect the outcome. This is an intriguing area to open research channels carrying out studies with a precise assessment of urethral pain using validated scores. An anatomical explanation for this sign with a high positive predictive value could eventually make it a classical finding or pathognomonic assessment of these patients.

Observing urethral pain upon catheter insertion or removal in women with FS is commonplace. Nevertheless, the subjective nature of this clinical sign puts its credibility under question. More research using validated tools to define pain in a larger cohort is warranted.

Conflict of interest

Author declares no conflict of interest.

References

  1. Fowler CJ, Christmas TJ, Chapple CR, Parkhouse HF, Kirby RS, Jacobs HS. Abnormal electromyographic activity of the urethral sphincter, voiding dysfunction, and polycystic ovaries: a new syndrome? BMJ. 1988; 297: 1436-1438.
  2. Lapointe SP, Rivet C, Goulet O, Fékété CN, Lortat-Jacob S. Urological manifestations associated with chronic intestinal pseudo-obstructions in children. J Urol. 2002; 168:1768-1770.
  3. Swinn MJ, Wiseman OJ, Lowe E, Fowler CJ. The cause and natural history of isolated urinary retention in young women. J Urol. 2002; 167: 151-156. Erratum: J Urol. 2002; 167: 1805.