Myelomeningocele operated in utero and the incontinent bladder pattern: Mid-term follow up of a prospective study

Introduction

In-utero myelomeningocele repair is the gold standard treatment after the publication of the MOMS trial. We have performed a retrospective analysis from our prospective in-utero myelomeningocele closure database (started in 2011), and selected only patients with the incontinent bladder pattern according to the Leal da Cruz categorization (Leal da Cruz, et al. J Urol 2015) to review mid-term clinical outcomes.

Material and methods

We identified 30 patients with leaking pressure under 40 cmH20 (incontinent pattern) at first urodynamic evaluation (UE) from the whole cohort of 129 patients who underwent in-utero myelomeningocele closure. We selected patients with a minimum active follow-up of 48 weeks (4 years) to provide mid-term data. Patients were followed according to the same protocol with the proposal of yearly sonogram and UE. All clinical and radiological data were reviewed.

Results

We found 11 patients, with a mean age of 10.2 years old, median age at diagnosis of 19 weeks, surgery performed at 25.6 weeks and birth at 33.2 weeks. The mean follow-up was 81.73 months (6.81 years). Mean age at first urological evaluation was 5 months, and UE was 5.6 months. Febrile UTI incidence in the whole observation period was 27.3%. The average initial DLPP was 30 cmH2O. 71.4% of the patients had bladder capacity less than 50% of the expected age. Bladder compliance could not be determined in 63.7% of cases due to leakage. A total of 5.7 urodynamic studies per patient were performed. Surgery was recommended for 8 patients and done in 4 (36.3%). Surgery consisted of Macedo catheterizable reservoir and Macedo-Malone ACE, associated with urethral sling (2 patients) and bladder neck closure (2). It took an average of 5 UE before the final surgical decision was confirmed. Last urodynamic study showed persistent leakage and low DLPP in 3 patients, normal bladder pressure in 2 (under CIC and anticholinergics), and 1 patient changed his bladder pattern into a high risk group. All operated patients are fully continent (urinary >4hs) and fecal.

Conclusion

Despite initially presenting a low risk for the most patients, we found surgery in 36.3% (4/11) and if we considered all cases with surgery indication proposed to treat urinary incontinence it would be even higher (72.7%).