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Purpose: The objective of this ICCS standardization document is to report the initial diagnostic evaluation and subse-
quent work-up of children with neuropathic bladder dysfunction. Materials and Methods: Due to a paucity of level I
or level II, ‘‘levels of evidence’’ publications, these recommendations are actually a compilation of best practices because
they seem to be effective and reliable, although not with any control. Results: Throughout the document, the
emphasis is on promoting early, comprehensive evaluation of lower urinary tract function that is thorough but with a
minimum of unnecessary testing. This includes what tests to order, when to order them and what to do with the
results. Some of the recommendations may not be practical in various worldwide locations but the suggested testing
should be considered the ideal approach to completely diagnosing and then promulgating treatments based on the full
knowledge of the condition and its effect on urinary tract function. Once the findings are delineated, those lower
urinary tract patterns of dysfunction that put the kidneys at risk for deterioration, that are barriers to attaining
eventual continence, and that have long-term consequence to the lower urinary track can be obviated by specific
management recommendations. The indications and timing of investigations to achieve these objectives are clearly
defined in each diagnostic category and during follow-up. Recommendations: This document should be used as a
basis for appropriate evaluation and timely surveillance of the various neuro-urologic conditions that affect children.
Neurourol. Urodynam. 31:610–614, 2012. ß 2012 Wiley Periodicals, Inc.
INTRODUCTION If untreated, death due to renal failure, in the first year of life,
can be as high as 20%. It is essential patients be followed from
Neuropathic bladder dysfunction (NBD) in children is an
early infancy in order to detect early signs of ureteral and
ever-evolving condition. The expansion of its understanding
renal dilation that could lead to ESRD.6 How and when these
and treatment over the last 50 years has been nothing short
patients should be followed has generally been left to the
of remarkable. In the mid 1950s, there were few insights and
discretion of individual practitioners. A review of the litera-
minimal alternatives to being in diapers or wearing an
ture reveals no unanimity on follow-up. We have attempted
appliance over an abdominal wall stoma. Starting with the
to provide a consensus of what appears to be best medical
development of adequate X-ray assessment and reliable
practice based on our understanding of the pathophysiology
urodynamic investigation (UDS),1 the advent of clean inter-
of NBD. We divide follow-up into stages of life since each
mittent catheterization (CIC),2 artificial sphincter implanta-
stage represents a different challenge for the child and the
tion,3 continent urinary conduits, a plethora of drug therapies
practitioner.
that modulate lower urinary tract function4 and a multitude
of rehabilitative surgical techniques, we have learned a great
deal about the pathophysiology, pathogenesis, and treatment
of these disorders and the evidenced-based ways to manage Conflicts of interest: none.
them. With the promise of tissue engineering and stem cell Christopher Chapple led the peer-review process as the Associate Editor responsi-
ble for the paper.
therapy, new vistas for treatment seem to be on the horizon. The review is produced in normal high ethical standards.
Since the introduction of proactive management of children *Correspondence to: Troels Munch Jorgensen, M.D., Faculty of Health, Institute of
with spina bifida,5 there has been a marked reduction in their Clinical Medicine, Aarhus University, Brendstrupgaardsvej 100, 8200 Aarhus N,
progression to end-stage renal disease (ESRD).6 If left untreat- Denmark. E-mail: tmj@dadlnet.dk
Received 3 March 2011; Accepted 5 March 2012
ed, neonates with detrusor sphincter dyssynergy (DSD) devel- Published online 24 April 2012 in Wiley Online Library
op upper urinary tract damage in the first year of life, starting (wileyonlinelibrary.com).
shortly after birth and in some cases even during fetal life.2,5,7 DOI 10.1002/nau.22247