XXXX : pitfalls in analysing the results of prophylactic surgery.

@entity362 @entity1 @entity5450 @entity1494
OBJECTIVES: Our objectives were to examine the assumption that the natural history of congenital @entity362 in @entity1 presenting early and those presenting late is the same, and to determine whether there are factors that might predict the post-operative outcome of surgery among the presenting features of @entity1 with @entity362 . METHODS: The authors present a clinical series of 50 consecutive cases of @entity362 that had been treated surgically. All @entity1 underwent both pre- and post-operative urological assessment and pre-operative imaging with MRI. The case notes, urology assessments and MRI films were studied to provide data on the clinical presentation, follow-up history and urological outcome of the @entity1 , as well as on the anatomical features of their @entity362 . RESULTS: Analysis of the data revealed a predominance in @entity1 and a bimodal distribution of age at presentation with peaks at 0-2 and 7-8 years. Skin stigmata were more frequent in @entity1 below 6 years of age than in older @entity1 (P=0.035). The MRI scans demonstrated that the conus lay within the sacral canal more often in @entity1 than @entity1 (P=0.025). Severe @entity1494 was significantly more frequent in those presenting at =3 years of age (P=0.017) and there were more normal outcomes in @entity1 operated upon before the age of 3 than in those operated upon at or after this age ( P<0.0001). Eight @entity1 who had been operated upon had severe urological problems at the time of their last assessment (median age of 8 years and 2 months). They were predominantly @entity1 with major pre-operative @entity5450 . CONCLUSIONS: Formal pre-operative urological assessment proved to be the strongest determinant of final urological outcome. Other predictors of poor urological outcome were @entity362 size and sacral position for the @entity362 . This series demonstrates how early and late presenting @entity1 may represent different @entity1 groups, follow different natural histories and may therefore not be reliable for comparison purposes. Future studies of prophylactic untethering need to follow cohorts beyond the age of deterioration found in untreated cases (8 years) and must report the salient MRI features that we suggest are predictive of poor outcome.
