doi:10.1016/j.eururo.2006.01.034

C.R.J. Woodhouseab
published online 6 February 2006.

In 1809, Samuel Cooper devoted a whole chapter of his textbook on practical surgery to record that nothing could be done for infants born with spina bifida. Few children lived longer than a year, but for those who did, it was recognized that better prognosis was associated with lesions low in the spinal cord [1]. Even now, for all babies born with open spina bifida, the prognosis is poor. In underdeveloped countries, congenital anomalies, especially spina bifida, account for nearly 50% of infant deaths and the rate is inversely correlated with the per capita gross domestic product [2]. However, it is possible to improve matters but only by hard and lifelong work by doctors, nurses, parents, and patients. In the United Kingdom about 50% of children have survived to the age of 35 yr [3].

At all ages, renal failure is a common cause of death. In children, the risk of renal failure is strongly related to the sensory level (which may not be the same as the anatomic level in the spine or the level suggested by X-ray). Renal failure is rare with sensory levels at or below L4 and common at or above T10 [4]. However, renal failure can occur even with apparently minor neural tube defects such as occult spinal dysraphism.

In those who have reached adulthood, life expectancy is still shortened. In an unselected series of 695 adults in the United Kingdom, 56 were known to have died before their expected time. In the 30 whose cause of death could be determined, renal failure accounted for 33% although cardiorespiratory diseases were the most common [5].

Thus, although there has been progress in the last 200 yr, there is still room for improvement, especially in the prevention of renal failure. The relationship between renal disease and paraplegia has been recognized for at least 150 years, but initially it was thought that renal disease was a cause of paraplegia [6]. In children born with spina bifida, it was the pioneering work of McGuire that defined the pressure relationship between bladder hyperreflexia and renal damage. Bladder storage pressures >40cm water are associated with progressive renal damage [7].

Since then, management of the bladder has focused on the maintenance of a low-pressure, compliant bladder. The mainstay has been clean intermittent catheterization (CIC). Because this puts a heavy burden on the parents of infants and of older patients with inadequate manual dexterity, some pediatricians have been reluctant to instigate a CIC program unless renal dilatation has begun.

This brings us to the paper of Dik et al. [8]. The authors’ thesis is that “optimal” management of the lower urinary tract from early infancy protects the kidneys and maintains compliance of the bladder in the long term. Optimal management means starting CIC in the first few months of life, giving antimuscarinic drugs and, when necessary, bladder reconstruction. Subsequent management is dictated by repeated urodynamic studies. For example, 15% of children in their series were able to stop taking antimuscarinics on the basis of several near normal cystometrograms.

Their results are impressive. Eighty-six percent of children survived and no deaths were from a renal cause. With a mean follow-up of nearly 7 yr, no child is in renal failure. Ninety-two percent of kidneys are normal on ultrasound or dimercaptosuccinic acid (DMSA) scans. Seventy-seven percent of children over 6 yr old are continent. There are no problems with the performance of regular cystometrograms because the children are accustomed to the practice of CIC.

The present authors make no claim to be the first advocates for the benefits of early active management of the bladder. However, their protocol does appear to have been constructed prospectively so that there has been consistency since 1988.

The question then is whether it is justified to use the full spectrum of active management on all patients when some will do well on less intensive regimes. Dik et al. point out that the patients who came late to their unit did less well than those who presented at birth. Although this is true, it could have been that the late presenters were only referred because they were doing badly, whereas other patients in the referring units were progressing well on less intensive regimes.

Compared to historical controls, the patients discussed in Dik's paper have certainly done well. However, the follow-up is short for a condition in which progressive problems are present throughout life; where the hurdle of puberty has yet to come; and where the threat of death in early adulthood is still present. It is possible that they have deferred renal problems rather than prevented them.

Patients with spina bifida require lifelong and active supervision. Multidisciplinary care given in a dedicated clinic is associated with better outcomes as Dik et al. have discussed. Furthermore, a positive, hopeful, and supportive attitude in the family, such as would be encouraged by a specialist clinic, significantly improves outcomes [9,10].

Many studies have shown the devastating effects that the care of a child with spina bifida has on the life of the rest of the family. In particular, it has been shown that parents do not like CIC but accept it as a part of the management program [11]. Oxybutynin, which is, for the present, the mainstay of antimuscarinic therapy in children, has well known side effects. It is reasonable, therefore, to suggest that a means should be found to identify those babies for whom early active bladder management is essential and spare the others the burden of unnecessary intervention.

It is clear that specialist clinics practicing the type of active management described by Dik et al. have excellent results. Meticulous attention to every aspect of care is essential to allow children with spina bifida to realize their full potential. The next step is to try to refine the regime to avoid excessive interference.

Many authors finish their reviews and editorials by advocating the initiation of a controlled trial, knowing full well that it would, for diverse reasons, be impractical! There is a particular difficulty in getting ethical approval for trials in children. I am about to fall into this trap: I suggest that it would be ethical to have a randomized trial of immediate versus delayed initiation of CIC and antimuscarinics and that one or more of the bigger centers would have enough patients to make it possible.

References

1. Cooper S. Spina Bifida. The first lines of the practice of surgery. London: Richard Phillips; 1809;p. 543–6.
2. Rosano A, Botto LD, Botting B, Mastroiacovo P. Infant mortality and congenital anomalies from 1950 to 1994: an international perspective. J Epidemiol Community Health. 2000;54:660–666.
3. Hunt GM. Open spina bifida: outcome for a complete cohort treated unselectively and followed into adulthood. Dev Med Child Neurol. 2000;32:108–118.
4. Hunt GM, Lewin WS, Gleave J, Gairdner D. Predictive factors in open myelomeningocele with special reference to sensory level. BMJ. 1973;4:197–201.
5. Singhal B, Mathew KM. Factors affecting mortality and morbidity in adult spina bifida. Eur J Pediatr Surg. 1999;9(suppl 1):31–32.
6. Graves RJ. Paralysis. In: Neligan JM editors. Graves's clinical medicine. Dublin, Ireland: Fannin; 1864;.
7. Wang SC, McGuire EG, Bloom DA. A bladder pressure management system for myelodysplasia: clinical outcome. J Urol. 1988;140:1499–1502.
8. Dik P, Klijn AJ, van Gool JD, de Jong-de Vos van Steenwijk CCE, De Jong TPVM. Early start to therapy preserves kidney function in spina bifida patients. Eur Urol. 2006;49:908–913.
9. Kirpalani HM, Parkin PC, Willan AR, et al.. Quality of life in spina bifida: importance of parental hope. Arch Dis Child. 2000;83:293–297. CrossRef
10. Friedman D, Holmbeck GN, Jandaseck B, Zuckerman J, Abad M. Parent functioning in families of preadolescents with spina bifida: longitudinal implications for child adjustment. J Fam Psychol. 2004;18:609–619.
11. Borzyskowski M, Cox A, Edwards M, Owen A. Neuropathic bladder and intermittent catheterisation: social and psychological impact on families. Dev Med Child Neurol. 2004;46:160–167.

European Urology - 2006 05 (Vol. 49, Issue 5) p.777-778

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