Jeffrey P. Blount

Functional Imaging: II. Prediction of Epilepsy Surgery Outcome

To gain information on the value of magnetic source imaging (MSI), 2‐[18F]fluoro‐2‐deoxy‐D‐glucose positron emission tomography (FDG‐PET), and ictal single photon emission computed tomography (SPECT) to predict seizure‐free outcome following epilepsy surgery in patients who require intracranial electroencephalography (ICEEG).

Functional imaging: I. Relative predictive value of intracranial electroencephalography.

To gain information on the predictive and prognostic value of magnetic source imaging (MSI), 2‐[18F]fluoro‐2‐deoxy‐D‐glucose positron emission tomography (18FDG‐PET), and ictal single‐photon emission computed tomography (SPECT) as compared with intracranial electroencephalography (ICEEG) localization in epilepsy surgery.

Effect of epilepsy magnetic source imaging on intracranial electrode placement

Intracranial electroencephalography (ICEEG) with chronically implanted electrodes is a costly invasive diagnostic procedure that remains necessary for a large proportion of patients who undergo evaluation for epilepsy surgery. This study was designed to evaluate whether magnetic source imaging (MSI), a noninvasive test based on magnetoencephalography source localization, can supplement ICEEG by affecting electrode placement to improve sampling of the seizure onset zone(s).

Long-term control of hydrocephalus via endoscopic third ventriculostomy in children with tectal plate gliomas.

OBJECTIVE We report the control of hydrocephalus in children with presumed tectal plate gliomas by the use of endoscopic third ventriculostomy (ETV). METHODS The hospital records, office charts, and imaging studies from children who underwent ETV at the Children’s Hospital of Alabama were reviewed. Thirteen children with the diagnosis of tectal plate glioma and hydrocephalus were identified. ETV was the primary therapy instituted for all but one of these children. Successful treatment outcome was defined as shunt freedom, improvement in symptoms, and reduced ventricular size. RESULTS Thirteen children underwent a total of 15 ETVs, and all children were shunt-free at their most recent follow-up examinations. One child underwent successful secondary ETV, one child underwent shunt removal concomitant with the initial ETV, and one child underwent shunt removal concomitant with secondary ETV. Symptoms and signs resolved in all patients. All postoperative cranial imaging studies revealed normal or reduced ventricular size as compared with preoperative cranial imaging scans. The median follow-up period was 31 months. CONCLUSION In our surgical experience, ETV has been uniformly successful in the management of hydrocephalus caused by tectal plate gliomas in children. ETV should be considered the treatment of choice for hydrocephalus in pediatric patients with tectal plate gliomas.

Parameters of Care for Craniosynostosis

Background A multidisciplinary meeting was held from March 4 to 6, 2010, in Atlanta, Georgia, entitled “Craniosynostosis: Developing Parameters for Diagnosis, Treatment, and Management.” The goal of this meeting was to create parameters of care for individuals with craniosynostosis. Methods Fifty-two conference attendees represented a broad range of expertise, including anesthesiology, craniofacial surgery, dentistry, genetics, hand surgery, neurosurgery, nursing, ophthalmology, oral and maxillofacial surgery, orthodontics, otolaryngology, pediatrics, psychology, public health, radiology, and speech-language pathology. These attendees also represented 16 professional societies and peer-reviewed journals. The current state of knowledge related to each discipline was reviewed. Based on areas of expertise, four breakout groups were created to reach a consensus and draft specialty-specific parameters of care based on the literature or, in the absence of literature, broad clinical experience. In an iterative manner, the specialty-specific draft recommendations were presented to all conference attendees. Participants discussed the recommendations in multidisciplinary groups to facilitate exchange and consensus across disciplines. After the conference, a pediatric intensivist and social worker reviewed the recommendations. Results Consensus was reached among the 52 conference attendees and two post hoc reviewers. Longitudinal parameters of care were developed for the diagnosis, treatment, and management of craniosynostosis in each of the 18 specialty areas of care from prenatal evaluation to adulthood. Conclusions To our knowledge, this is the first multidisciplinary effort to develop parameters of care for craniosynostosis. These parameters were designed to help facilitate the development of educational programs for the patient, families, and health-care professionals; stimulate the creation of a national database and registry to promote research, especially in the area of outcome studies; improve credentialing of interdisciplinary craniofacial clinical teams; and improve the availability of health insurance coverage for all individuals with craniosynostosis.

Children with Growth Hormone Deficiency and Chiari I Malformation: A Morphometric Analysis of the Posterior Cranial Fossa

Objective: The posterior fossa (PF) has been found to be small in various forms of Chiari malformation. Explanations involving a connection between growth hormone deficiency (GHD) and Chiari I malformation (CIM) have been proposed. However, to date, no quantitative analysis of the PF of patients with CIM and GHD has been performed. Our study was performed to determine the geometry of the PF in children with GHD and CIM. Methods: Morphometric analysis of the PF was performed in 10 children with GHD and CIM (group 1), 20 children with GHD and no CIM (group 2) and 50 controls. Results: PF volumes for group 1 ranged from 128 to 259 ± 33 ml, and for group 2, they ranged from 115 to 186.2 ± 25.4 ml. Lengths of the foramen magnum for groups 1 and 2 had means of 36 and 38 mm, respectively. The mean basiocciput length and tentorial angle for groups 1 and 2 were 20 and 19 mm and 89 and 87.5°, respectively. Conclusions: We have determined that children with GHD with or without CIM have no significant difference in their PF volume compared to controls. However, our data demonstrate significant underdevelopment of portions of the bony PF in both patients with GHD alone and in patients with GHD and CIM. Tentorial angles were elevated in noncontrol groups. We propose that this association is not due to an increased rate of ‘midline’ defects seen in GHD but rather a structurally distorted PF that is not capacious enough to house the entire developing rhombencephalon. These data will hopefully aid in the further understanding of the pathophysiology of CIM.

Acute Hydrocephalus following a Chiari I Decompression

Complications of posterior cranial fossa decompression for Chiari malformations are uncommon. We present three patients who developed infratentorial supracerebellar hygromas causing acute hydrocephalus after posterior cranial fossa decompression.

Endoscopic third ventriculostomy for hydrocephalus secondary to central nervous system infection or intraventricular hemorrhage in children.

Objective: We review our experience of endoscopic third ventriculocisternostomy (ETV) in children with hydrocephalus from central nervous system (CNS) infection or intraventricular hemorrhage to better elucidate success rates and predictors of success in these children. Methods: We performed a retrospective review of 12 children less than 21 years of age with ETV treated from 1999 to 2002 with a minimum follow-up of 12 months. Children selected for surgery had been diagnosed with hydrocephalus from perinatal germinal matrix hemorrhage or CNS infection and had neuroimaging consistent with obstruction of the aqueduct, fourth ventricular outlets or both at the time of ETV. Charts and imaging studies were reviewed to determine clinical outcomes and predictors of successful ETV. An ETV was deemed successful if the child did not require placement or replacement of a ventriculoperitoneal shunt. Results: Thirteen ETV were performed in 12 patients (7 boys and 5 girls; mean age 9.2 years, range 3.8–21 years), with an overall success rate of 60% for those with CNS infections and 71% for those with perinatal intraventricular hemorrhage at initial presentation. There were no significant complications from the procedure. Conclusions: ETV is a safe procedure, which in carefully selected children with etiologies of hydrocephalus thought to be ‘communicating’ in nature carries a satisfyingly high success rate. Further application and study of this modality in larger groups of patients with these causes of hydrocephalus is warranted.

Parameters of care for craniosynostosis: craniofacial and neurologic surgery perspectives.

Background: A multidisciplinary meeting was held from March 4 through 6, 2010, in Atlanta, Georgia, to conceptualize, map out, and operationalize the variables most relevant to the care of patients with craniosynostosis. In this article, the authors highlight the development of the craniofacial and neurologic surgery parameters of care. Methods: Fifty-two conference attendees, representing a broad range of expertise in craniosynostosis care and 16 professional societies, participated in this working group. Literature and expert opinion were used to identify the variables important for the care of patients with craniosynostosis. In an iterative manner, specialty-specific data were compiled into draft recommendations and presented to all conference attendees. Cross-discipline analysis was used to assess the completeness of the data, facilitate exchange of ideas, and achieve consensus. Results: Consensus was reached among the 52 conference attendees and two post hoc reviewers in 18 areas of craniosynostosis care. Longitudinal parameters of care were developed for the diagnosis, treatment, and management of craniosynostosis in each of the specialty areas of care, from prenatal evaluation to completion of care in adulthood. Conclusions: To our knowledge, this is the first multidisciplinary effort to develop parameters of care and best practices for craniosynostosis. In conjunction with patient input, these parameters may help to heuristically develop core outcome sets that are important not only to patients and their families, but also to health care providers and health services and outcome researchers. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, V.

Advances in intracranial monitoring

Intracranial monitoring using electroencephalography (IC-EEG) continues to play a critical role in the assessment of patients with medically intractable localization-related epilepsy. There has been minimal change in grid or electrode design in the last 15-20 years, and the surgical approaches for implantation are unchanged. Intracranial monitoring using EEG allows detailed definition of the region of ictal onset and defines the epileptogenic zone, particularly with regard to adjacent potentially eloquent tissue. Recent developments of IC-EEG include the coregistration of functional imaging data such as magnetoencephalography to the frameless navigation systems. Despite significant inherent limitations that are often overlooked, IC-EEG remains the gold standard for localization of the epileptogenic cortex. Intracranial electrodes take a variety of different forms and may be placed either in the subdural (subdural strips and grids, depth electrodes) or extradural spaces (sphenoidal, peg, and epidural electrodes). Each form has its own advantages and shortcomings but extensive subdural implantation of electrodes is most common and is most comprehensively discussed. The indications for intracranial electrodes are reviewed.