Fowler's syndrome and pregnancy

Background

Fowler's syndrome is a relatively rare disorder with significant implications during pregnancy due to difficulty in maintaining adequate bladder emptying and the risk of recurrent urinary tract infections with their associated morbidity to the mother and fetus.

Case presentation

We report the successful outcome of a pregnancy in a 32-year-old patient with Fowler's syndrome. Mrs P was diagnosed with the condition 10 years prior to the index pregnancy, following recurrent urinary tract infections associated with symptoms of voiding dysfunction and difficulty with defecation. Although she was initially managed with intermittent self-catheterisation and anti-cholinergic agents, followed by the long-term insertion of suprapubic catheters, the results were disappointing. She finally had placement of a sacral nerve stimulator (SNS) at the National Hospital for Neurology and Neurosurgery (NHNN) in London with a good clinical response.

Mrs P's first presentation to our Obstetric Department was at 7 weeks gestation with a urinary tract infection and an infected suprapubic catheter site. Her SNS had been deactivated at the NHNN due to concerns about the safety of the device during pregnancy. Mrs P responded well to medical management, and she was discharged on a low-dose-maintenance regimen of antibiotic to cover the remainder of her pregnancy.

Mrs P had multiple admissions under the care of the urology and obstetric teams during the second and third trimester with recurrent urinary tract infections associated with significant pelvic pain. These required parenteral antibiotic treatment and changes of her suprapubic catheter. The suprapubic pain was difficult to manage, with Mrs P requiring opioid analgesia to stabilise her symptoms, initially as an inpatient but subsequently in the community.

With the onset of the third trimester Mrs P's urinary symptoms worsened, resulting in more frequent and prolonged admissions. At 31 weeks gestation she was administered a course of betamethasone to promote fetal lung maturation, and an elective caesarean section was planned for 36 weeks. Serial ultrasound examinations demonstrated normality of fetal growth and liquor volume.

At her request her SNS was reactivated by the NHNN at 32 weeks gestation. However, Mrs P chose to leave it in its non-functional state until she had delivered despite the numerous admissions, courses of systemic antibiotics, and insertions of central venous access and doses of analgesics that this approach entailed.

Outcome...