Chronic Urinary Retention due to Fowler's Syndrome

Abstract

Fowler's syndrome (FS) is a rare cause of chronic urinary retention in teenage girls and young women. We present a case of a 14-year-old girl who presented at our hospital 2 weeks after uncomplicated laparoscopic appendectomy. The girl complained of reduced urinary frequency and prolonged micturition time. Following an acute cystitis 2 months later, she completely lost her ability to void. A comprehensive set of investigations to assess the cause of her urinary retention including a cerebral and spinal magnetic resonance imaging (MRI), and videourodynamics were performed. The diagnostic workup revealed polycystic ovaries and an asensitive and hypotonic bladder with capacity up to 1200 mL and high maximum urethral pressure of 120 cm of water. She did not tolerate clean intermittent catheterization; therefore, a suprapubic catheter was placed. Under this treatment, she suffered recurrent urinary tract infections. Two years later, she was diagnosed with FS on the basis of the medical history, clinical symptoms, and urodynamic findings. Finally, the implantation of a S3 neurostimulator restored her ability to void.

Keywords

urinary retention - Fowler's syndrome - sacral neuromodulation

• 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 (6661): 1436-1438
• 2 Osman NI, Chapple CR. Fowler's syndrome--a cause of unexplained urinary retention in young women?. Nat Rev Urol 2014; 11 (02) 87-98
• 3 Panicker JN, Emmanuel A, Fowler CJ. Urinary retention. Pelvic Organ Dysfunct Neurol Dis Clin Manag Rehabil 2010; 293-306
• 4 Karmarkar R, Abtahi B, Saber-Khalaf M, Gonzales G, Elneil S. Gynaecological pathology in women with Fowler's syndrome. Eur J Obstet Gynecol Reprod Biol 2015; 194: 54-57
• 5 Gatti JM, Perez-Brayfield M, Kirsch AJ, Smith EA, Massad HC, Broecker BH. Acute urinary retention in children. J Urol 2001; 16 (03) 918-921
• 6 Austin PF, Bauer SB, Bower W. , et al. The standardization of terminology of lower urinary tract function in children and adolescents: update report from the Standardization Committee of the International Children's Continence Society. J Urol 2014; 191 (06) 1863-1865.e13
• 7 Chase J, Austin P, Hoebeke P, McKenna P. ; International Children's Continence Society. The management of dysfunctional voiding in children: a report from the Standardisation Committee of the International Children's Continence Society. J Urol 2010; 183 (04) 1296-1302
• 8 Deindl FM, Vodusek DB, Bischoff C, Hofmann R, Hartung R. Dysfunctional voiding in women: which muscles are responsible?. Br J Urol 1998; 82 (06) 814-819
• 9 Fowler CJ, Kirby RS. Electromyography of urethral sphincter in women with urinary retention. Lancet 1986; 1 (8496): 1455-1457
• 10 Kavia R, Dasgupta R, Critchley H, Fowler C, Griffiths D. A functional magnetic resonance imaging study of the effect of sacral neuromodulation on brain responses in women with Fowler's syndrome. BJU Int 2010; 105 (03) 366-372