Few children with MM are born with and maintain normal bladder and sphincter function. Most urodynamic evaluations in the first months of life indicate abnormal innervation of these structures. Deterioration with the development of fixed external sphincter resistance, loss of detrusor compliance, and decreased capacity occurs in some patients within months to a few years of age. The DLPP helps distinguish patients at greater risk for subsequent upper tract damage from elevated intravesical pressure, despite the inherent imprecision of the tests. Nevertheless, some infants with low-risk assessments show upper tract deterioration over time. The optimal timing of initial and follow-up urodynamic studies remains controversial. Radiologic surveillance is considered by some an adequate alternative to early urodynamic studies, based on the observation that hydronephrosis and reflux detected by imaging most often resolve with medical management. The Boston group has focused attention on function of the external sphincter and the potential for denervation to occur over time, placing the bladder and upper tracts at increased risk. Their experience suggests that needle electrodes, rather than patches, should be used for EMG during urodynamic studies to best evaluate sphincter innervation. It remains unproved whether the early detection of external sphincter denervation with subsequent spinal cord detethering will improve long-term outcomes over the current management philosophy in other centers, which emphasizes control of elevated bladder pressure. Despite advances in the urologic care of children with MM, many basic questions regarding optimal evaluation and management remain unanswered. Regardless of whether management is primarily based on urodynamics (with preemptive intervention), radiologic imaging (with intervention for development of adverse changes), or a combination of the two, all children with MM require close surveillance for urologic problems, especially during the first few years of life. Goals to protect the bladder and upper tracts and eventually to attain continence, ideally by school age, are discussed with the family from the outset and reinforced during periodic follow-up visits.
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