Long-term urologic outcome in patients with caudal regression syndrome, compared with meningomyelocele and spinal cord lipoma.
OBJECTIVE
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.
Torre
Michele
M
Paediatric Surgery, Istituto Giannina Gaslini, 16148 Genoa, Italy. micheletorre@hotmail.com
Buffa
Piero
P
Jasonni
Vincenzo
V
Cama
Armando
A
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007677:Kidney Function Tests; D008067:Lipoma / Q000175:diagnosis / Q000453:epidemiology / Q000601:surgery*; D008591:Meningomyelocele / Q000175:diagnosis / Q000453:epidemiology / Q000601:surgery*; D011336:Probability; D018570:Risk Assessment; D012720:Severity of Illness Index; D016136:Spina Bifida Occulta / Q000175:diagnosis / Q000453:epidemiology / Q000601:surgery*; D013120:Spinal Cord Neoplasms / Q000175:diagnosis / Q000453:epidemiology / Q000601:surgery*; D016896:Treatment Outcome; D001750:Urinary Bladder, Neurogenic / Q000453:epidemiology / Q000503:physiopathology* / Q000628:therapy; D014546:Urinary Catheterization; D014563:Urodynamics; D014718:Vesico-Ureteral Reflux / Q000175:diagnosis* / Q000453:epidemiology