title: The urodynamic profile of myelodysplasia in childhood with spinal closure during gestation.
abstract: OBJECTIVE
  Spinal dysraphism is the most common cause of neurogenic bladder dysfunction in newborns. Urodynamic findings in these patients include uninhibited bladder contractions, bladder areflexia, decreased compliance and detrusor-sphincter dyssynergia. Early urodynamic studies are recommended for spina bifida to help identify bladder characteristics that may cause a risk of upper tract deterioration. We recently evaluated a new early type of intervention involving closure of the neural tube defect during gestation in 25 patients at our institution. We hypothesize that this procedure results in decreased exposure of the spinal cord to amniotic fluid, which may improve neurological function. To date we have evaluated 16 of the 25 patients with video urodynamics. We compared the results to those in the literature on patients with myelomeningocele and without prenatal intervention.
  
  
  METHODS
  We performed urodynamic testing in 16 patients with a mean age of 6.5 months, including cystometrography, fluoroscopic evaluation of filling and voiding, pelvic floor electromyography and post-void residual urine measurement. In addition, we retrospectively reviewed renal ultrasound, voiding cystourethrography, catheterization need, number of urinary tract infections and medication in these cases.
  
  
  RESULTS
  Uninhibited detrusor contractions and an areflexic bladder were identified in 6% and 43% of patients, respectively, while 19% had decreased compliance and 75% had leak point pressure greater than 40 cm. water. Mean bladder capacity was 40 cc and 31% of patients had much lower capacity than expected for age. Previous renal ultrasound and voiding cystourethrography showed evidence of upper tract dilatation and reflux in 2 cases, respectively. Intermittent catheterization and anticholinergic therapy were required by 1 patient each and 1 had a significant urinary tract infection.
  
  
  CONCLUSIONS
  Urodynamic findings in this population are comparable to those previously reported in the literature in patients with spina bifida without prenatal closure of the spinal defect. The lower incidence of urinary tract infection and reflux in our study probably represents more aggressive early urological management rather than neurological improvement. These urodynamic studies were performed early in life and future evaluation may ultimately reveal improved bladder function compared with that in others with myelodysplasia. However, at this time we recommend that patients who undergo spinal cord defect closure during gestation be evaluated and treated in the same manner as those with myelomeningocele but without fetal intervention.
authors.affiliation: Divisions of Pediatric Urology, Pediatric Neurosurgery and Maternal-Fetal Medicine, Vanderbilt University Medical Center, Nashville, Tennessee, USA.
authors.forename: J
authors.initials: J
authors.lastname: Holzbeierlein
authors.forename: I V
authors.initials: IV
authors.lastname: Pope JC
authors.forename: M C
authors.initials: MC
authors.lastname: Adams
authors.forename: J
authors.initials: J
authors.lastname: Bruner
authors.forename: N
authors.initials: N
authors.lastname: Tulipan
authors.forename: J W
authors.initials: JW
authors.lastname: Brock
mesh_terms: D002648:Child; D004576:Electromyography; D005260:Female; D005315:Fetal Diseases / Q000601:surgery*; D005333:Fetus / Q000601:surgery*; D006801:Humans; D009436:Neural Tube Defects / Q000503:physiopathology* / Q000601:surgery*; D011184:Postoperative Period; D011247:Pregnancy; D011262:Pregnancy Trimester, Second; D016896:Treatment Outcome; D014563:Urodynamics