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Prospective multicenter studies in pediatric hydrocephalus

JNSPG 75th Anniversary Invited Review Article

John R. W. Kestle and Jay Riva-Cambrin

Prospective multicenter clinical research studies in pediatric hydrocephalus are relatively rare. They cover a broad spectrum of hydrocephalus topics, including management of intraventricular hemorrhage in premature infants, shunt techniques and equipment, shunt outcomes, endoscopic treatment of hydrocephalus, and prevention and treatment of infection. The research methodologies include randomized trials, cohort studies, and registry-based studies. This review describes prospective multicenter studies in pediatric hydrocephalus since 1990. Many studies have included all forms of hydrocephalus and used device or procedure failure as the primary outcome. Although such studies have yielded useful findings, they might miss important treatment effects in specific subgroups. As multicenter study networks grow, larger patient numbers will allow studies with more focused entry criteria based on known and evolving prognostic factors. In addition, increased use of patient-centered outcomes such as neurodevelopmental assessment and quality of life should be measured and emphasized in study results. Well-planned multicenter clinical studies can significantly affect the care of children with hydrocephalus and will continue to have an important role in improving care for these children and their families.

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Shobhan Vachhrajani, Abhaya V. Kulkarni and John R. W. Kestle

In the era of evidence-based medicine, clinical practice guidelines (CPGs) have become an integral part of many aspects of medical practice. Because practicing neurosurgeons rarely have the time or, in some cases, the methodological expertise, to assess and assimilate the totality of primary research, CPGs can in theory provide a vehicle through which neurosurgeons could more efficiently integrate the most current evidence into patient management. Clinical practice guidelines have been met with some skepticism, however, particularly within the neurosurgical community. Some have expressed concerns that the promise of CPGs has not been matched by the reality. Others who oppose CPGs fear that they hinder the art of medicine, and limit physician and patient autonomy. The purpose of this paper is to provide the practicing neurosurgeon with an up-to-date review of CPGs. The authors discuss some of the complexities and recent advancements in CPG development, appraisal, and publication. An overview of the various systems for grading medical evidence and issuing CPG recommendations, each of which has its advantages and disadvantages, is included, and the current knowledge on the impact of CPGs in 2 important realms, patient care and medicolegal issues, is discussed.

The purpose of this review is to provide a balanced, current synopsis of what CPGs are, how they are developed, and what they can and cannot do. The authors hope that this will allow neurosurgeons to make more informed decisions about the many CPGs that will inevitably become an essential component of medical practice in the years to come.

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John R. W. Kestle, Abhaya V. Kulkarni and Benjamin C. Warf

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Todd D. McCall, James K. Liu and John R. W. Kestle

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John R. W. Kestle

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Paul Klimo Jr., John R. W. Kestle and Meic H. Schmidt


Spinal cord compression is one of the most dreaded complications of metastatic cancer. It can lead to a number of sequelae, including pain, spinal instability, neurological deficits, and a reduction in the patient's quality of life. Except in selected circumstances, treatment is palliative. Treatment options include surgery, radiation, and chemotherapy. The goal of this study was to summarize the existing data on the outcomes of various treatment methods for metastatic spinal epidural disease and to make appropriate recommendations for their use.


The authors used a search strategy that included an electronic database search, a manual search of journals, analysis of bibliographies in relevant review papers, and consultation with the senior author. There is good evidence, including Class I data, that steroid drugs constitute a beneficial adjunctive therapy in patients with myelopathy from epidural compression. Historically, conventional radiation therapy has been viewed as the first-line treatment because it has been shown to be as effective as a decompressive laminectomy, with a lower incidence of complications (Class II data). Nevertheless, in the last 20 years there has been remarkable progress in surgical techniques and technology. Currently, the goals of surgery are to achieve a circumferential decompression of the spinal cord, and to reconstruct and immediately stabilize the spinal column. Results in a large body of literature support the belief that surgery is better at retaining or regaining neurological function than radiation and that surgery is highly effective in relieving pain. Most of the data on the treatment of metastatic spinal disease are Class II or III, but the preliminary results of a well-designed, randomized controlled trial in which surgery is compared with standard radiation therapy represents the first Class I data.


As the number of treatment options for metastatic spinal disease has grown, it has become clear that effective implementation of these treatments can only be achieved by a multidisciplinary approach.

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Richard C. E. Anderson, Marion L. Walker, John M. Viner and John R. W. Kestle

✓ Inadvertent adjustments and malfunctions of programmable valves have been reported in cases in which patients have encountered powerful electromagnetic fields such as those involved in magnetic resonance imaging, but the effects of small magnetic fields are not well known. The authors present a case in which a child playing with a collection of commercially available toy magnets altered the pressure setting of an implanted valve and may have caused its permanent malfunction.

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Douglas Kondziolka, L. Dade Lunsford, John C. Flickinger and John R. W. Kestle

✓ The benefits of radiosurgery for cavernous malformations are difficult to assess because of the unclear natural history of this vascular lesion, the inability to image malformation vessels, and the lack of an imaging technique that defines “cure.” The authors selected for radiosurgery 47 patients who harbored a hemorrhagic malformation in a critical intraparenchymal location remote from a pial or ependymal surface. Of these, 44 patients had experienced at least two hemorrhages before radiosurgery. The mean patient age was 39 years; six patients had previously undergone attempted surgical removal. The malformation was located in the pons/midbrain in 24 cases, the medulla in three, the thalamus in nine, the basal ganglia in three, deep in a parietal lobe in four, and deep in a temporal lobe in four. Patients had sustained initial hemorrhages from 0.5 to 12 years prior to radiosurgery (mean 4.12 years). In these patients, who were not typical of the majority of patients with cavernous malformations, there were 109 bleeds before radiosurgery in 193 prior observation-years, for a 56.5% annual hemorrhage rate (including the first hemorrhage), or an annual rate of 32% subsequent to the first hemorrhage.

The mean follow-up period after radiosurgery was 3.6 years (range 0.33–6.4 years). The proportion of patients with hemorrhage after radiosurgery was significantly reduced (p < 0.0001), as was the mean number of hemorrhages per patient (p = 0.00004). In the first 2 years after radiosurgery, there were seven bleeds in 80 observation-years (8.8% annual hemorrhage rate). In the 2- to 6-year interval after radiosurgery, the annual rate decreased to 1.1% (one bleed). After radiosurgery, 12 patients (26%) sustained neurological worsening that correlated with imaging changes. In eight patients these deficits were temporary; two underwent surgical resection and died. Two patients had new permanent deficits (4%). A significant reduction was observed in the hemorrhage rate after radiosurgery in patients who had deep hemorrhagic cavernous malformations, especially after a 2-year latency interval. This evidence provides further support to the belief that radiosurgery is an effective strategy for cavernous malformations, especially when located within the parenchyma of the brainstem or diencephalon.

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