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Shobhan Vachhrajani, Andrew Jea, John A. Rutka, Susan Blaser, Michael Cusimano and James T. Rutka

Meningiomas represent the most common benign intracranial neoplasm in adults, with a considerably lower incidence in children. The authors present the case of an intracranial meningioma with invasion of, and intraluminal extension into, the transverse and sigmoid sinuses, jugular bulb, and internal jugular vein, resulting in venous occlusion in a 14-year-old girl. Computed tomography scanning, MR imaging, and conventional angiography were performed preoperatively. The patient underwent a 2-stage resection: the supratentorial component was resected first, and the infratentorial and venous sinus and jugular vein components were subsequently removed using a combined skull base approach. Gross-total resection was achieved by opening the lateral dural sinus and removing the meningioma from within the transverse and sigmoid sinuses, the jugular bulb, and the internal jugular vein. The patient remained neurologically intact after the staged tumor resections. Postoperative imaging confirmed the gross-total resection. This case illustrates the unusual property of an intracranial meningioma to invade the intrasinusoidal space and extend into the jugular vein without adherence to the underlying venous endothelium of the jugular vein.

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Benjamin W. Y. Lo, Abhaya V. Kulkarni, James T. Rutka, Andrew Jea, James M. Drake, Maria Lamberti-Pasculli, Peter B. Dirks and Lehana Thabane

Object

Cephaloceles represent primary axial mesodermal defects, occurring in 0.8–4 per 10,000 live births. Prior studies have reported posterior location, hydrocephalus, microcephaly, seizure, and presence of brain tissue as poor prognostic markers for neurological outcome. However, these studies were small and the results were analyzed using univariate tests. The purpose of this study was to investigate the potential risk factors for the occurrence of developmental delay in patients with cephaloceles, using both univariate and multivariable regression techniques.

Methods

This is a retrospective cohort study of cephalocele cases treated at the Hospital for Sick Children between 1990 and 2006. Two independent investigators collected the data from the Hospital for Sick Children Encephalocele Database and hospital charts. Developmental assessments were made by general pediatricians and neuropsychologists. Both univariate analysis (α = 0.10) and multivariable logistic regression analysis (α = 0.05) were performed.

Results

Eighty-five cases of cephaloceles were identified. The patient group consisted of 48 boys and 37 girls. Sixty-eight lesions were encephaloceles and 17 were meningoceles. The distribution was as follows: frontal (40 lesions), occipital (33), and parietal (12). Associated conditions included hydrocephalus (23), seizure disorder (17), microcephaly (6), corpus callosal abnormalities (15), heterotopias (9), cerebral dysgenesis (11), and myelomeningocele (1). Evaluation of long-term development revealed that 41 patients (48%) had normal development, 9 (11%) had mild delay, 14 (16%) had moderate delay, and 21 (25%) had severe delay. Hydrocephalus, seizure disorder, microcephaly, presence of associated intracranial abnormalities, and presence of brain tissue were significantly associated with poor outcome on univariate analysis. Multivariable analysis revealed hydrocephalus and presence of intracranial abnormalities to be statistically significant predictors of developmental delay.

Conclusions

To the authors' knowledge, this is one of the largest North American cephalocele series documented. Unlike prior studies, location of the cephaloceles is not a significant predictor of outcome. The multivariable regression analysis demonstrates hydrocephalus and the presence of associated intracranial abnormalities as variables with cumulative predictive effects for developmental delay.

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Andrew Jea, Shobhan Vachhrajani, Keyne K. Johnson and James T. Rutka

Object

Although corpus callosotomy has been used effectively since the late 1930s to treat severe, medically intractable seizure disorders, particularly atonic or drop-attack seizures, controversy remains as to when, how, and how much surgery should be performed. Intraoperative determination of the extent of callosotomy, the need to stage the procedure, and the side of the interhemispheric approach represent technical issues that remain debatable. The authors report the 12-year experience of the senior author as well as surgical outcomes with corpus callosotomy using a frameless stereotactic neuronavigation system (ISG View Wand and BrainLab).

Methods

Thirteen consecutive children at The Hospital for Sick Children underwent single-stage corpus callosotomy for medically intractable seizures. The mean age was 10.3 years. Five children underwent partial callosotomy, and 8 underwent complete callosotomy. The side of operative approach to avoid large parasagittal bridging veins was determined by preoperative study of 3D MR imaging/MR venography reconstructed by the neuronavigation system. The extent of callosotomy was determined using intraoperative feedback from the neuronavigation system and postoperative MR imaging.

Results

The extent of callosotomy determined by intraoperative neuronavigation and postoperative MR imaging correlated closely in all cases. There were no operative deaths. There was no significant postoperative morbidity related to venous infarction. Four of 5 patients in the partial callosotomy cohort and 7 of 8 patients in the complete callosotomy cohort showed significant improvement in seizure control.

Conclusions

The use of frameless stereotactic neuronavigation is a safe, effective, and important surgical adjunct in the planning and execution of successful corpus callosotomy in children with intractable epilepsy.

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Andrew Jea, Michael D. Taylor, Peter B. Dirks, Abhaya V. Kulkarni, James T. Rutka and James M. Drake

✓The authors describe the novel use of C-1 lateral mass screws in four children 8 years of age or younger, in whom occipitocervical or atlantoaxial fusion was performed for trauma or os odontoideum. The authors retrospectively reviewed the demographics and procedural data of four children, ranging in age from 2 to 8 years, who required and underwent surgical fixation. Although C1–2 screw/rod constructs involving individual C-1 lateral mass screws and C-2 pars interarticularis or pedicle screws have been widely applied in adults, only C1–2 transarticular screw fixation has been reported in children less than 8 years of age. This report demonstrates the successful results of rigid occipitocervical and atlantoaxial fusion in four children in whom C-1 lateral mass screws were placed as part of a screw/rod construct. There was one instance of a vertebral artery injury, and the lessons learned from this complication are discussed.

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Douglas L. Brockmeyer