CODE: 12.2 - Fowler syndrome - clinical characteristics
Article published in Urologia Polska 2006/59/Suplement 1.

authors

Piotr Radziszewski ¹, Włodzimierz Baranowski ², Andrzej Witek ³, Elżbieta Keller ¹, Andrzej Borkowski ¹, Maciej Czaplicki ¹, Agnieszka Bossowska ⁴, Mariusz Majewski ⁴

¹ Klinika Urologii AM w Warszawie
² Klinika Chorób Kobiecych Wojskowego Instytutu Medycznego MON w Warszawie
³ Klinika Położnictwa i Ginekologii ŚAM w Katowicach
⁴ Zakład Fizjologii Klinicznej, Katedra Morfologii Czynnościowej w Olsztynie

summary

Introduction. Etiology of voiding difficulties in young females may have different backgrounds. In case of Fowler syndrome, which is a co-occurrence of voiding difficulties in patients with polycystic ovary syndrome, the hypertrophy of the external urethral sphincter is considered to be the source of origin of voiding difficulties. Patients with this syndrome, described by Clare Fowler, had a complete urinary retention. It seems however that this manifestation concerns only the minority of the patients in the very advanced stage of the syndrome.

Objectives. We aimed our studies at performing lower urinary tract evaluation and symptoms characteristic in patients with Fowler syndrome.

Materials and methods. 200 patients with the polycystic ovary syndrome (mean age 20.4 years, range 18-33) were studied. Screening was targeted at disclosure of voiding disturbances in these patients (voiding difficulties, post void residual, urgency, frequency, pain during voiding). After identification of such patients a full urodynamic study and volumetry of the external urethral sphincter using transrectal ultrasound were performed. Fowler syndrome was diagnosed if the external urethral sphincter volume was bigger than 2 cm3 and the maximum urethral closure pressure (performed accordingly to Brown-Whickman) was bigger than 80 cmH2O.

Results. Fowler syndrome was diagnosed in 10 patients (mean age 19.5 years, range 18-33 years) out of 13 with polycystic ovary syndrome and voiding disturbances. No patient had complete urinary retention. All patients were complaining of pain in the lower abdomen, decrease of urinary flow and frequency. In the urodynamic examination urine flow in a characteristic "saw teeth" like shape was found (mean maximum flow rate 16.2 ml/s, range 12.4 to 22.8 ml/s). Maximum cystometric bladder capacity was 429 ml in the average (range 222 ml to 635 ml) and mean post void residual was 30 ml (range 20-200 ml). Maximum urethral closure pressure was 115 cmH2O in the average (range 87 to 128 cm H2O). In the transrectal ultrasound the mean external urethral sphincter volume was 2.8 cm3 (range 2.4 to 5.5 cm3).

Conclusions. Fowler syndrome is a rare disease, present in about 0.5% of patients with polycystic ovary syndrome. Its clinical manifestation is not homogenous and the complete urinary retention occurs very rarely (no such cases in examined patients). This diagnosis should be considered in young females with coexistence of voiding and menstruation disturbances.