The management of the myelodysplastic child: a paradigm shift

Abstract

study these babies urodynamically and noted several unfavourable signs which predicted increased risk factors for urinary tract deterioration [18]. McGuire et al. [19] showed that leak-point pressures of >40 cmH 2 O lead to the development of hydronephrosis and reflux in 68% of cases. A review of infants followed expectantly after having undergone kidney and bladder imaging, and urodynamic studies in the newborn period, revealed that 71% with bladder external urethral sphincter dyssynergy, with or without high detrusor filling pressure, experienced deterioration of their urinary tract function within the first 3 years of life [20]. Several other paediatric urologists were able to detect similar rates of deterioration when dyssynergy between the bladder and sphincter was present in the newborn period [21]. Interestingly, reflux was present only in those neonates who had dyssynergy on their first urodynamic study, suggesting in utero deterioration from high bladder voiding pressures [22].In the early to mid-1980s these reproducible observations and accurate predictions of deterioration caused many to reconsider that perhaps watchful waiting was not the most practical approach for these babies. This prompted a new view about how they should be treated [16,17]. By then, CIC was becoming a readily acceptable means of emptying the bladder in older children, to achieve continence with no need for urinary diversion [23]. Its application to newborns and young infants whose urodynamic values put them at risk for urinary tract deterioration was a novel approach that required a period of observation to confirm its beneficence. Over time, concerns about urethral injury and difficulty in using CIC regularly proved to be moot [23]. However, within a short period the positive effect of regular bladder emptying with a clean but not sterile catheter, with and without the use of anticholinergic agents to lower detrusor filling pressure when present, became the new mainstay of treatment [24]. Controversy raged for a time until there was overwhelming proof that very few children had upper urinary tract deterioration when aggressively managed with CIC and anticholinergic medication [17,[25][26][27]. The contention that children could be followed and CIC started after changes were noted [28,29] became irrelevant. Indeed, it was noted that the incidence of VUR was directly related to the presence of dyssynergy between the bladder and the external sphincter. In addition, reflux was detected frequently and within 6 months in those children whose neurological condition changed, with the conversion from a synergic to a dyssynergic sphincter. Thus, serial urodynamic studies proved to be a pariah in preventing the occurrence of reflux [25].

Predictive value of initial urodynamic pattern on urinary continence in patients with myelomeningocele

“…However, it should be considered that in the present study also the very early implanted devices are included as we investigated patients with myelomenigocele treated since 1975 at our institution. Although some authors [Teichman et al, 1994;Hopps and Kropp, 2003] have questioned the value of urodynamics shortly after birth and serially thereafter, we agree with most authors [Bauer et al, 1984;Perez et al, 1992;Lais et al, 1993;Kurzrock and Polse, 1998;Bauer, 2003] that this is a prerequisite for an adequate treatment strategy in patients with myelomeningocele. The ¢ndings with thorough clinical investigations are speci¢c, but not sensitive enough, and also sophisticated means like magnetic resonance imaging cannot conclude from spinal cord lesions the consequences for urinary tract function.…”

“…The idea of watchful waiting has been replaced by proactive treatment to preserve both upper and lower urinary tract function, maintain neurological stability and achieve social acceptability [Bauer, 2003]. Although the primary goal in neurourological management of patients with myelomeningocele is the preservation of renal function, e¡orts to promote urinary continence have become important recognizing the impact on quality of life.…”

Early proactive management improves upper urinary tract function and reduces the need for surgery in patients with myelomeningocele

“…Although some authors [Teichman et al, 1994;Hopps and Kropp, 2003] have questioned the value of urodynamics shortly after birth and serially thereafter, we agree with most authors [Bauer et al, 1984;Perez et al, 1992;Lais et al, 1993;Kurzrock and Polse, 1998;Bauer, 2003] that this is a prerequisite for an adequate treatment strategy in patients with myelomeningocele. Only urodynamics together with the targeted clinical investigation (presence of anal/bulbocavernosus re£ex) allow de¢nition of the dysfunctional pattern of the lower urinary tract [Kessler et al, 2006] for detecting risk factors (low-bladder compliance, intravesical pressure >40 cmH 2 O, detrusor sphincter dyssynergia) in time before major changes in the upper urinary tract occur.…”

“…The idea of watchful waiting has been replaced by proactive treatment to preserve both upper and lower urinary tract function, maintain neurological stability, and achieve social acceptability [Bauer, 2003].…”

Urological Results after Fetal Myelomeningocele Repair in Pre-MOMS Trial Patients at the Children's Hospital of Philadelphia

“…Myelodysplasia is a classification describing the various abnormalities of the spinal cord and nerve roots, which include myelomeningocele (MMC), spinal dysraphism, sacral agenesis and caudal regression syndrome. The prognosis for survival of children who are born today with MMC has improved dramatically, with the more aggressive approach for early management contributing to a marked improvement in survival and quality of life [2]. From the perspective of the pediatric urologist, morbidity as the children become older is oftentimes due to renal compromise secondary to progressive hydronephrosis and/or recurrent episodes of pyelonephritis.…”