MSI, FDG-PET, and ictal SPECT each have clinical value in predicting seizure-free surgical outcome in epilepsy surgery candidates who typically require ICEEG.

Object The diagnosis and treatment of Chiari malformation Type I (CM-I) has evolved over the last few decades. The authors present their surgical experience of over 2 decades of treating children with this form of hindbrain herniation. Methods The authors conducted a retrospective review of their institutional experience with the surgical treatment of the pediatric CM-I from 1989 to 2010. Results The 2 most common presentations were headache/neck pain (40%) and scoliosis (18%). Common associated diagnoses included neurofibromatosis Type 1 (5%) and idiopathic growth hormone deficiency (4.2%). Spine anomalies included scoliosis (18%), retroversion of the odontoid process (24%), Klippel-Feil anomaly (3%), and atlantooccipital fusion (8%). Approximately 3% of patients had a known family member with CM-I. Hydrocephalus was present in 48 patients (9.6%). Syringomyelia was present in 285 patients (57%), and at operation, 12% of patients with syringomyelia were found to have an arachnoid veil occluding the fourth ventricular outlet. Fifteen patients (3%) have undergone reoperation for continued symptoms or persistent large syringomyelia. The most likely symptoms and signs to resolve following surgery were Valsalva-induced headache and syringomyelia. The average hospital stay and “return to school” time were 3 and 12 days, respectively. The follow-up for this group ranged from 2 months to 15 years (mean 5 years). Complications occurred in 2.4% of cases; there was no mortality. No patient required acute return to the operating room, and no blood transfusions were performed. Conclusions The authors believe this to be the largest reported series of surgically treated pediatric CM-I patients and hope that their experience will be of use to others who treat this surgical entity.

OBJECT Evidence in support of hemispherectomy stems from a multitude of retrospective studies illustrating individual institutions' experience. A systematic review of this topic, however, is lacking in the literature. METHODS A systematic review of hemispherectomy for the treatment of refractory epilepsy available up to October 2013 was performed using the following inclusion criteria: reports of a total of 10 or more patients in the pediatric age group (≤ 20 years) undergoing hemispherectomy, seizure outcome reported after a minimum follow-up of 1 year after the initial procedure, and description of the type of hemispherectomy. Only the most recent paper from institutions that published multiple papers with overlapping study periods was included. Two reviewers independently applied the inclusion criteria and extracted all the data. RESULTS Twenty-nine studies with a total of 1161 patients met the inclusion criteria. Seizure outcome was available for 1102 patients, and the overall rate of seizure freedom at the last follow-up was 73.4%. Sixteen studies (55.2%) exclusively reported seizure outcomes of a single type of hemispherectomy. There was no statistically significant difference in seizure outcome and type of hemispherectomy (p = 0.737). Underlying etiology was reported for 85.4% of patients with documented seizure outcome, and the overall distribution of acquired, developmental, and progressive etiologies was 30.5%, 40.7%, and 28.8%, respectively. Acquired and progressive etiologies were associated with significantly higher seizure-free rates than developmental etiologies (p < 0.001). Twenty of the 29 studies (69%) reported complications. The overall rate of hydrocephalus requiring CSF diversion was 14%. Mortality within 30 days was 2.2% and was not statistically different between types of hemispherectomy (p = 0.787). CONCLUSIONS Hemispherectomy is highly effective for treating refractory epilepsy in the pediatric age group, particularly for acquired and progressive etiologies. While the type of hemispherectomy does not have any influence on seizure outcome, hemispherotomy procedures are associated with a more favorable complication profile.

Objective-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 non-invasive test based on magnetoencephalography source localization, can supplement ICEEG by affecting electrode placement to improve sampling of the seizure onset zone(s).Methods-Of 298 consecutive epilepsy surgery candidates (between 2001-2006) 160 cases were prospectively enrolled on the basis of insufficient localization from seizure monitoring and MRI results. Prior to presenting MSI results, decisions were made as to whether to proceed with ICEEG, and if so, where to place electrodes such that the hypothetical seizure onset zone would be sampled. MSI results were then provided with allowance of changes to the original plan.Results-MSI indicated additional electrode coverage in 18 of 77 (23%) ICEEG cases. In 39% percent (95% CI: 16.4, 61.4) seizure onset ICEEG patterns involved the additional electrodes indicated by MSI. Sixty-two patients underwent surgical resection based on ICEEG recording of seizures. Highly localized MSI was significantly associated with seizure-free outcome (mean=3.4 years, minimum > 1 year) for the entire surgical population (n=62).Interpretation-MSI spike localization increases the chance that the seizure onset zone is sampled when patients undergo ICEEG for presurgical epilepsy evaluations. The clinical impact of this effect--improving diagnostic yield of ICEEG--should be considered in surgery candidates

MSI yields localizing information with a high positive predictive value in epilepsy surgery candidates who typically require ICEEG. This finding suggests that enough clinical validity exists for MSI to potentially replace ICEEG for seizure localization.

Although surgical procedures are often performed over the posterior head and neck, surgical landmarks for avoiding the cutaneous nerves in this region are surprisingly lacking in the literature. Twelve adult cadaveric specimens underwent dissection of the cutaneous nerves overlying the posterior head and neck, and mensuration was made between these structures and easily identifiable surrounding bony landmarks. All specimens were found to have a third occipital nerve (TON), lesser occipital nerve (LON), and greater occipital nerve (GON), and we found that the TON was, on average, 3 mm lateral to the external occipital protuberance (EOP). Small branches were found to cross the midline and communicate with the contralateral TON inferior to the EOP in the majority of sides. The mean diameter of the main TON trunk was 1.3 mm. This trunk became subcutaneous at a mean of 6 cm inferior to the EOP. The GON was found to lie at a mean distance of 4 cm lateral to the EOP. On all but three sides, a small medial branch was found that ran medially from the GON to the TON approximately 1 cm superior to a horizontal line drawn through the EOP. The GON was found to pierce the semispinalis capitis muscle on average 2 cm superior to the intermastoid line. The mean diameter of the GON was 3.5 mm. The GON was found to branch into medial and lateral branches on average 0.5 cm superior to the EOP. The LON was found to branch into a medial and lateral component at approximately the midpoint between a horizontal line drawn through the EOP and the intermastoid line. The main LON trunk was found on average 7 cm lateral to the EOP. In specimens with a mastoid branch of the great auricular nerve (GAN), this branch was found at a mean of 9 cm lateral to the EOP. The main trunk of this branch of the GAN was found to lie on average 1 cm superior to the mastoid tip. Easily identifiable bony landmarks for identification of the cutaneous nerves over the posterior head and neck can aid the surgeon in more precisely identifying these structures and avoiding complications. Although the occipital nerves were found to freely communicate with one another, avoiding the main nerve trunks could lessen postoperative or postprocedural morbidity. Moreover, clinicians who need to localize the occipital nerves for the treatment of occipital neuralgia could do so more reliably with better external landmarks.

Lipomas of the spinal cord are among the most fascinating lesions encountered by the pediatric neurosurgeon. An understanding of spinal lipomas may, however, be difficult because the terminology used to describe the accumulations of spinal fat is confusing, inconsistently applied, and at times contradictory. An anatomical characterization of lipomas may assist in understanding these lesions. Lipomas of the spinal cord are very rare and cause symptoms related to mass effect and secondary compressive myelopathy. Lipomas of the conus medullaris (or lipomyelomeningocele) are the most common form of fatty masses in the spine and can be divided into dorsal, caudal, and transitional forms. These lesions are a manifestation of occult spinal dysraphism and a common cause of the tethered cord syndrome (TCS). The natural history of untreated lipomyelomeningocele, although incompletely understood, appears to be progressive neurological deterioration with loss of bladder control. Timely, careful surgical intervention may prevent significant neurological deterioration and progressive disability in the majority of children harboring these lesions. In surgical intervention the surgeon seeks to disrupt the connection between the fibrofatty mass and underlying cord as well as to reestablish normal anatomical planes. Several intraoperative video segments illustrating lipomyelome-ningocele resection are included in this paper. Lipomas of the terminal filum (fatty filum) are truly occult and are also associated with TCS. Surgical treatment of filum lipomas carries significantly lower risk than that for lipomas of the conus medullaris. Again, the goal of surgery is to disrupt the connection between the abnormal fibrofatty tissue and the underlying spinal cord.

Conclusively positive MSI has a high predictive value for seizures localized with ICEEG. Diagnostic gain may be achieved with addition of either PET or ictal SPECT to MSI. Diagnostic values for imaging tests are lower than "true values" because of the limitations of ICEEG as a gold standard.