Introduction Neurogenic bladder dysfunction in children is an ever-evolving condition. The expansion of its understanding and treatment over the past 50 years has been just remarkable. In the mid 1950s there were few insights and minimal alternatives to the child’s being in diapers or wearing an appliance over an abdominal wall stoma. Starting with the development of adequate X-ray assessment and reliable urodynamic investigation, the advent of clean intermittent catheterization, artificial sphincter implantation, continent urinary conduits and a plethora of drug therapies that modulate lower urinary tract function, we have learned a great deal about the pathophysiology, pathogenesis and treatment of this disorder and the evidence specific ways to manage it. With the promise of tissue engineering and stem cell therapy, new vistas for treatment seem to be on the horizon. 1 4 Journal of Urology 5 Urodynamic studies 6 2 6 2 2 2 5 6 7 Neurospinal dysraphism Myelomeningocele 1 8 Table 1 Spinal bony level of myelomeningocele (uppermost vertebral abnormality) Location Incidence (%) Cervical-high thoracic 2 Low thoracic 5 Lumbar 26 Lumbosacral 47 Sacral 20 9 2 9 10 13 14 15 16 17 18 20 11 12 20 2 2 19 21 22 Table 2 IVP ECHO UDS VCUG RNC Sphincter activity Recommended tests Frequency Intact-synergic Post-void residual volume Every 4 months IVP or renal ECHO Every 12 months UDS Every 12 months a IVP or renal ECHO Every 12 months UDS Every 12 months b Every 12 months Partial denervation Post-void residual volume Every 4 months IVP or renal ECHO Every 12 months c Every 12 months b Every 12 months Complete denervation Post-void residual volume Every 6 months Renal ECHO Every 12 months a b c When vesicoureteral reflux is present, CIC effectively lowers the intravesical emptying pressure when the bladder is drained. In addition, anticholinergic medication can be added to lower detrusor filling pressure, increasing compliance without fear of causing urinary retention when combined with CIC. The lowered filling and emptying pressures has proven to be very beneficial; in 30–50% of children reflux is resolved within 2–3 years of its discovery and initiation of therapy. 23 24 16 25 Credé voiding is not an efficacious form of bladder emptying in children with myelodysplasia, especially if the urethral sphincter is partially or fully innervated. Because most children have intact motor function above L1, any increase in abdominal pressure from a Credé maneuver can lead to a reflexive increase in urethral sphincter activity, thus producing an increase in bladder outlet resistance resulting in “high voiding pressure”. This can be particularly noxious in children with moderate or severe grades of reflux. In addition, as the child grows, the bladder resides more in the pelvis and not intra-abdominally, further reducing the effectiveness of the Credé maneuver. 9 26 A plethora of surgical procedures has been designed to increase bladder outlet resistance in those children with a level that is insufficient to maintain continence between catheterizations, that include implantation of an artificial urinary sphincter, bladder neck tightening, using adjacent tissue, a fascial sling, and various bulking agents. All can provide more resistance, but no one procedure is ideally suited for every patient. Creating a catheterizable urinary stoma has become fashionable in those children with intractable urethral incontinence (with obliteration of the bladder neck) or inability to catheterize their urethra easily due to obesity, poor eye–hand coordination or caretaker issues surrounding genital organ privacy. Long-term success has been achieved that provides the individual with a degree of independence, but problems with stomal stenosis can occur. Occult spinal dysraphism 27 28 28 31 32 33 34 35 33 36 37 33 37 39 28 37 39 Sacral agenesis 40 41 42 43 44 45 46 40 47 48 47 49 47 Associated conditions Imperforate anus 3 Table 3 Wingspread classification of anorectal malformations Female Male High High  Anorectal agenesis  Anorectal agenesis   With rectovaginal fistula   With rectourethral (prostatic) fistula   Without fistula  Without fistula  Rectal atresia  Rectal atresia Intermediate Intermediate   Rectovestibular fistula   Rectovestibular urethral fistula   Rectovaginal fistula   Anal agenesis without fistula   Anal agenesis without fistula Low Low  Anovestibular fistula  Anocutaneous fistula  Anocutaneous fistula  Anal stenosis  Anal stenosis  Rare malformation Cloacal malformation  Rare malformation  V A C TE R L 50 51 52 49 53 54 Central nervous system disorders Cerebral palsy 55 56 4 57 Table 4 61 UMN LMN Factor UMN (no. of patients) LMN (no. of patients) Prematurity 10 1 Respiratory distress/arrest/apnea 9 2 Neonatal seizures 5 – Infection 5 1 Traumatic birth 5 – Congenital hydrocephalus 3 – Placenta previa/abruption 2 2 Hypoglycemia seizures 2 – Intracranial hemorrhage 2 – Cyanosis at birth 1 3 No specific factor noted 15 – 58 59 60 61 5 6 Table 5 Lower urinary tract function in cerebral palsy Type Number Upper motor neuron lesion 49 Mixed upper + lower motor neuron lesion 5 Incomplete lower motor neuron lesion 1 No urodynamic lesion 2 Table 6 Urodynamics findings in cerebral palsy (some patients had more than one finding) Type of Lesion No. of Patients Upper motor neuron (detrusor or sphincter) Detrusor overactivity 35 Detrusor sphincter dyssynergy 7 Overactive sacral reflexes 6 No voluntary control 3 Smaller than expected bladder capacity 2 Poorly compliant 2 Lower motor neuron (abnormal motor unit potentials) Excessive polyphagia of sphincter 5 ↑ Amplitude + ↑ duration potentials 4 Trauma Traumatic injuries to the spine 62 63 62 64 67 68 65 s c i w o r a 69 70 71 72 73 74 76 77 78 79 Conclusions Neurogenic bladder dysfunction in children takes in a very wide spectrum of conditions that include congenitally acquired conditions that may even be preventable today, conditions that are associated with specific anatomic abnormalities, and acquired conditions that may occur perinatally or from accidents or sports or motor vehicle related injuries. Despite the etiology, the guiding principles for management are similar; insuring and maintaining an adequate sized, normally compliant, reservoir that evacuates urine completely, at a relatively low pressure, is the key to maintaining a healthy environment for the kidneys. A plethora of methods has come into existence, especially since the advent of clean intermittent catheterization and the advancement of pharmacologic understanding and manipulation. Future prospects look bright for affected children, with the overall health of the individual the most paramount goal to be achieved.