Long-term urologic outcome in patients with caudal regression syndrome, compared with meningomyelocele and spinal cord lipoma

J Pediatr Surg. 2008 Mar;43(3):530-3. doi: 10.1016/j.jpedsurg.2007.10.036.

Abstract

Background/purpose: The long-term urologic outcome in a large series of patients with neural tube defects was evaluated.

Methods: The following clinical parameters in 398 patients ranging from 1 to 37 years of age--69 with caudal regression syndrome (CRS), 244 with meningomyelocele (MMC), and 85 with spinal lipoma (SL)--were studied: congenital renal anomalies, renal function, vesico-ureteric reflux, upper tract dilatation, urodynamic pattern, and urinary continence.

Results: Single kidney was much more frequent in CRS (20.3%), compared with MMC (1.2%) and SL (0%). Vesico-ureteric reflux was found in 37.7% of patients with CRS, 43.0% of MMC, and 21.2% of SL. Patients with CRS had a higher risk of impaired renal function (8.7%), compared with MMC (5.3%) and SL (1.2%). Neuropathic bladder was found in 61% of patients with CRS, 98% of MMC, and 42% of SL. Among them, clean intermittent catheterization and drugs allowed 30% of patients with CRS, 45% of MMC, and 71% of SL to be dry for more than 4 hours.

Conclusions: Diagnosis influences the urologic outcome in neural tube defect. In CRS, the incidence of renal agenesis and vesico-ureteric reflux was unexpectedly high. The risk of renal damage and, in those with neuropathic bladder, of urinary incontinence, was similar to patients with MMC.

Publication types

  • Comparative Study

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Child, Preschool
  • Cohort Studies
  • Follow-Up Studies
  • Humans
  • Infant
  • Kidney Function Tests
  • Lipoma / diagnosis
  • Lipoma / epidemiology
  • Lipoma / surgery*
  • Meningomyelocele / diagnosis
  • Meningomyelocele / epidemiology
  • Meningomyelocele / surgery*
  • Probability
  • Risk Assessment
  • Severity of Illness Index
  • Spina Bifida Occulta / diagnosis
  • Spina Bifida Occulta / epidemiology
  • Spina Bifida Occulta / surgery*
  • Spinal Cord Neoplasms / diagnosis
  • Spinal Cord Neoplasms / epidemiology
  • Spinal Cord Neoplasms / surgery*
  • Treatment Outcome
  • Urinary Bladder, Neurogenic / epidemiology
  • Urinary Bladder, Neurogenic / physiopathology*
  • Urinary Bladder, Neurogenic / therapy
  • Urinary Catheterization
  • Urodynamics
  • Vesico-Ureteral Reflux / diagnosis*
  • Vesico-Ureteral Reflux / epidemiology