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David McLone, David Frim, Richard Penn, Charles N. Swisher, Peter Heydemann, Kenneth M. Boyer, A. Gwendolyn Noble, Peter K. Rabiah, Shawn Withers, Kristen Wroblewski, Theodore Karrison, Samuel Hutson, Kelsey Wheeler, William Cohen, Joseph Lykins and Rima McLeod

OBJECTIVE

Hydrocephalus occurs in children with congenital toxoplasmosis and can lead to severe disability. In these cases, the decision to intervene is often influenced by the expectation of neurological recovery. In this study, clinical responses to neurosurgical intervention in children with hydrocephalus secondary to congenital toxoplasmosis are characterized.

METHODS

Sixty-five participants with hydrocephalus due to congenital Toxoplasma gondii infection were evaluated as part of the National Collaborative Chicago-based Congenital Toxoplasmosis Study, and their neuroradiographic findings were reviewed. Clinical outcomes were scored on the basis of cognition and motor skills through the use of IQ scores and Gross Motor Function Classification System (GMFCS) level. Outcomes were then analyzed in relation to approach to management, anatomy of hydrocephalus, and time from diagnosis of hydrocephalus to surgical intervention.

RESULTS

There was considerable variation in the outcomes of patients whose hydrocephalus was treated in early life, ranging from normal cognitive and motor function to profound developmental delay and functional limitation. Of the 65 participants included in the study, IQ and GMFCS level were available for 46 (70.8%). IQ and motor score were highly correlated (r = −0.82, p < 0.001). There were people with differing patterns of hydrocephalus or thickness of cortical mantle on initial presentation who had favorable outcomes. Time to neurosurgical intervention data were available for 31 patients who underwent ventriculoperitoneal (VP) shunt placement. Delayed shunt placement beyond 25 days after diagnosis of hydrocephalus was associated with greater cognitive impairment (p = 0.02). Motor impairment also appeared to be associated with shunt placement beyond 25 days but the difference did not achieve statistical significance (p = 0.13). Among those with shunt placement within 25 days after diagnosis (n = 19), the mean GMFCS level was 1.9 ± 1.6 (range 1–5). Five (29.4%) of 17 of these patients were too disabled to participate in formal cognitive testing, after excluding 2 patients with visual difficulties or language barriers that precluded IQ testing. Of the patients who had VP shunt placement 25 or more days after diagnosis (n = 12), the mean GMFCS level was 2.7 ± 1.4 (range 1–4). Of these, 1 could not participate in IQ testing due to severe visual difficulties and 8 (72.7%) of the remaining 11 due to cognitive disability.

CONCLUSIONS

VP shunt placement in patients with hydrocephalus caused by congenital toxoplasmosis can contribute to favorable clinical outcomes, even in cases with severe hydrocephalus on neuroimaging. Shunt placement within 25 days of diagnosis was statistically associated with more favorable cognitive outcomes. Motor function appeared to follow the same pattern although it did not achieve statistical significance.

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Brandon G. Rocque, Bonita S. Agee, Eric M. Thompson, Mark Piedra, Lissa C. Baird, Nathan R. Selden, Stephanie Greene, Christopher P. Deibert, Todd C. Hankinson, Sean M. Lew, Bermans J. Iskandar, Taryn M. Bragg, David Frim, Gerald Grant, Nalin Gupta, Kurtis I. Auguste, Dimitrios C. Nikas, Michael Vassilyadi, Carrie R. Muh, Nicholas M. Wetjen and Sandi K. Lam

OBJECTIVE

In children, the repair of skull defects arising from decompressive craniectomy presents a unique set of challenges. Single-center studies have identified different risk factors for the common complications of cranioplasty resorption and infection. The goal of the present study was to determine the risk factors for bone resorption and infection after pediatric cranioplasty.

METHODS

The authors conducted a multicenter retrospective case study that included all patients who underwent cranioplasty to correct a skull defect arising from a decompressive craniectomy at 13 centers between 2000 and 2011 and were less than 19 years old at the time of cranioplasty. Prior systematic review of the literature along with expert opinion guided the selection of variables to be collected. These included: indication for craniectomy; history of abusive head trauma; method of bone storage; method of bone fixation; use of drains; size of bone graft; presence of other implants, including ventriculoperitoneal (VP) shunt; presence of fluid collections; age at craniectomy; and time between craniectomy and cranioplasty.

RESULTS

A total of 359 patients met the inclusion criteria. The patients’ mean age was 8.4 years, and 51.5% were female. Thirty-eight cases (10.5%) were complicated by infection. In multivariate analysis, presence of a cranial implant (primarily VP shunt) (OR 2.41, 95% CI 1.17–4.98), presence of gastrostomy (OR 2.44, 95% CI 1.03–5.79), and ventilator dependence (OR 8.45, 95% CI 1.10–65.08) were significant risk factors for cranioplasty infection. No other variable was associated with infection.

Of the 240 patients who underwent a cranioplasty with bone graft, 21.7% showed bone resorption significant enough to warrant repeat surgical intervention. The most important predictor of cranioplasty bone resorption was age at the time of cranioplasty. For every month of increased age the risk of bone flap resorption decreased by 1% (OR 0.99, 95% CI 0.98–0.99, p < 0.001). Other risk factors for resorption in multivariate models were the use of external ventricular drains and lumbar shunts.

CONCLUSIONS

This is the largest study of pediatric cranioplasty outcomes performed to date. Analysis included variables found to be significant in previous retrospective reports. Presence of a cranial implant such as VP shunt is the most significant risk factor for cranioplasty infection, whereas younger age at cranioplasty is the dominant risk factor for bone resorption.

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Issam A. Awad, Javad Hekmatpanah and David Frim

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Nassir Mansour, Ruth-Mary deSouza, Christian Sikorski, Madelyn Kahana and David Frim

Barbiturates are widely used in the management of high intracranial pressure (ICP) caused by diffuse brain swelling. The cardiovascular, renal, and immunological side effects of these drugs limit them to last-line therapy. There are few published data regarding the role of barbiturates in focal brain lesions causing refractory elevated ICP and intraoperative brain swelling in the pediatric population. The authors here present 3 cases of nontraumatic, focally induced, refractory intracranial hypertension due to 2 tumors and 1 arteriovenous malformation, in which barbiturate therapy was used successfully to control elevated ICP. They focus on cardiovascular, renal, and immune function during the course of pentobarbital therapy. They also discuss the role of pentobarbital-induced hypothermia. From this short case series, they demonstrate that barbiturates in conjunction with standard medical therapy can be used to safely reduce postoperative refractory intracranial hypertension and intraoperative brain swelling in children with focal brain lesions.

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Vicko Gluncic, Michael Turner, Leonard Kranzler and David Frim

A case of atlanto-occipital dislocation (AOD) is presented to illustrate the importance of subtle imaging findings and the occipital condyle–C1 interval (CCI) measurement in the evaluation of high cervical spine injury. Although AOD is commonly considered to be fatal, recently there have been an increasing number of reports of children surviving this injury. Prompt recognition and treatment of AOD are crucial for survival.

The authors present a case of an 8-year-old boy who sustained a destabilizing injury without bone disruption but with ligamentous tears that rendered his cervical spine unstable from the occiput to the C-1 level. On admission, imaging findings were consistent with tectorial membrane damage, perimedullary subarachnoid hemorrhage, and extraaxial blood from the clivus to the C-2 level. Most standard cervical spine radiological indices were within normal limits except the CCI. After initial management in a cervical collar, the patient was placed in halo vest, and subsequently underwent occiput to C-3 fusion. Timely recognition of the injury and subsequent craniocervical stabilization with internal fixation resulted in full neurological recovery. This report supports CCI as a valuable index for the prompt recognition of AOD. It also supports recent literature suggesting that AOD is a survivable injury with the possibility for an excellent neurological recovery.

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Medina C. Kushen and David Frim

✓Subdural electrode arrays are placed to localize seizure foci for possible resection. The procedure is usually straightforward when an electrode grid array is placed on the brain convexity but can become complicated if the surface on which the grids are applied is not convex. Arachnoid cysts can be associated with seizures, but their topography presents a challenge to standard techniques for the placement of subdural grids. The authors report on a technique for electrode grid placement that successfully localized seizure foci in the depths of arachnoid cysts in two patients.

Subdural grids were placed to conform to the concave cyst cavity. They were held in place with rolled gelatin foam padding, which filled the arachnoid cyst. The padding was removed before removing the electrode grids and resecting the seizure focus.

Although arachnoid cysts present a technical challenge when seizure foci are located within the cyst cavity, the technique of packing the cyst cavity with gelatin foam provides good electrode contact on the concave cyst wall, allowing adequate seizure focus localization.