Atypical Molecular Features of Pediatric Tectal Glioma: A Single Institutional Series.

We present 4 children (diagnosed between 1 and 8 y, 3 females and 1 male) with molecularly distinct tectal gliomas (2 KRAS mutant, 1 EGFR mutant, 1 SRGAP3-RAF-1 fusion) that contributes to the growing literature of this uncommonly biopsied tumor. The patient with EGFR R222C mutation had a more severe course, earlier diagnosis, subsequent leptomeningeal metastatic disease, required more aggressive therapies, and died 9 years after diagnosis. Patients with KRAS mutations and SRGAP3-RAF-1 fusion had a more indolent course. Our series expands the molecular phenotype of tectal glioma with the potential for leptomeningeal dissemination. Future studies on establishing genotypic/phenotypic correlation from those who undergo biopsy are needed.

Single-agent Bevacizumab in the Treatment of Symptomatic Newly Diagnosed and Recurrent/Refractory Pediatric Cervicomedullary Brainstem Low-grade Gliomas: A Single Institutional Experience.

Bevacizumab-based therapies have been utilized as single or combination therapy of refractory/recurrent pediatric low-grade gliomas. Its efficacy for symptomatic cervicomedullary low-grade gliomas (cmLGGs) in the upfront and the recurrent setting is less known. We report our retrospective single institutional experience from 2015 to 2021 with single-agent bevacizumab for symptomatic cmLGG. Six consecutive patients (4 female, ages 2 to 12 y) with newly diagnosed (n=3) and recurrent/refractory (n=3) symptomatic nondisseminated cmLGG (5/6 biopsy-proven, 2 BRAFV600E, 2 BRAF-KIAA1549) were treated with single-agent bevacizumab. All demonstrated radiographic response most pronounced on post-gadolinium T1-weighted magnetic resonance imaging (2 complete, 4 partial) at a median of 8 weeks (range: 2 to 12 wk). Clinical response was seen in all patients with improvement in cranial nerve abnormalities (3 recurrent/refractory, 1 newly diagnosed), strength (2 recurrent/refractory, 2 newly diagnosed), pain (2 recurrent/refractory), and anorexia (1 newly diagnosed). Four patients (2 recurrent/refractory, 2 newly diagnosed) experienced disease progression on subsequent adjunct therapies, 2 of which (the 2 newly diagnosed patients) are currently being rechallenged. At a mean follow-up of 7 months, all patients are clinically stable without disease progression. Single-agent bevacizumab may be effective in the management of symptomatic newly diagnosed and recurrent/refractory cmLGG and warrants further evaluation in a clinical trial setting.

Characterization of pediatric brain tumors using pre-diagnostic neuroimaging.

Purpose: To evaluate for predictive neuroimaging features of pediatric brain tumor development and quantify tumor growth characteristics in patients who had neuroimaging performed prior to a diagnosis of a brain tumor. Methods: Retrospective review of 1098 consecutive pediatric patients at a single institution with newly diagnosed brain tumors from January 2009 to October 2021 was performed to identify patients with neuroimaging prior to the diagnosis of a brain tumor. Pre-diagnostic and diagnostic neuroimaging features (e.g., tumor size, apparent diffusion coefficient (ADC) values), clinical presentations, and neuropathology were recorded in those patients who had neuroimaging performed prior to a brain tumor diagnosis. High- and low-grade tumor sizes were fit to linear and exponential growth regression models. Results: Fourteen of 1098 patients (1%) had neuroimaging prior to diagnosis of a brain tumor (8 females, mean age at definitive diagnosis 8.1 years, imaging interval 0.2-8.7 years). Tumor types included low-grade glioma (n = 4), embryonal tumors (n = 2), pineal tumors (n=2), ependymoma (n = 3), and others (n = 3). Pre-diagnostic imaging of corresponding tumor growth sites were abnormal in four cases (28%) and demonstrated higher ADC values in the region of high-grade tumor growth (p = 0.05). Growth regression analyses demonstrated R2-values of 0.92 and 0.91 using a linear model and 0.64 and 0.89 using an exponential model for high- and low-grade tumors, respectively; estimated minimum velocity of diameter expansion was 2.4 cm/year for high-grade and 0.4 cm/year for low-grade tumors. High-grade tumors demonstrated faster growth rate of diameter and solid tumor volume compared to low-grade tumors (p = 0.02, p = 0.03, respectively). Conclusions: This is the first study to test feasibility in utilizing pre-diagnostic neuroimaging to demonstrate that linear and exponential growth rate models can be used to estimate pediatric brain tumor growth velocity and should be validated in a larger multi-institutional cohort.

The role of methylation profiling in histologically diagnosed neurocytoma: a case series.

PURPOSE: To highlight the clinical, neuroradiographic, neuropathologic, and molecular features of histologically identified neurocytoma in a pediatric cohort and highlight the evolving use methylation profiling in providing diagnostic clarity in difficult to diagnosis pediatric brain tumors. METHODS: Five consecutive children (ages 9-13, 2 girls 3 boys) were histologically diagnosed with neurocytoma at Rady Children's Hospital San Diego from 2012 to 2018. Clinical and molecular features were analyzed with regards to treatment course and outcome. RESULTS: Presenting symptoms included seizures (n = 2), syncope (n = 1), headache (n = 2), visual disturbances (n = 2) and emesis (n = 2). Tumor location included intraventricular (n = 2), intraventricular with parenchymal spread (n = 1), and extraventricular (n = 2). Magnetic resonance imaging demonstrated reduced diffusivity (2/5), signal abnormality on susceptibility-weighted sequences (3/5), and varying degrees of contrast enhancement (4/5). All patients underwent surgical resection alone. Recurrence occurred in four children that were treated with surgery (4/4), adjuvant radiation (2/4), and chemoradiation (1/4). Neuropathologic features included positivity for GFAP (4/5), synaptophysin (4/5), NSE (2/2), NeuN (4/4), and variable Ki-67 (< 1% to 15%). Next generation sequencing (3/5) and microarray (3/5) collectively were abnormal in four of five tumors. Methylation profiling was successfully performed on four of five samples which led to modification of diagnosis in two patients and the others were either unclassifiable or confirmatory with the histologic diagnosis. Mean time to follow up was 77 months (range 44-112 months). Mean progression free survival and overall survival were 24 months (range 6 to 52 months) and 100% respectively. CONCLUSION: Neurocytomas are a rare clinical entity that warrants further investigation into molecular and pathologic prognosticating features. Methylation profiling may aid in differentiation of neurocytoma from other difficult to diagnose tumors who share similar histologic features.

Clinical and Neuroimaging Features of Magnetic Resonance-Guided Stereotactic Laser Ablation for Newly Diagnosed and Recurrent Pediatric Brain Tumors: A Single Institutional Series.

OBJECTIVE: We describe our single-institutional experience with magnetic resonance-guided stereotactic laser ablation (SLA) for the treatment of newly diagnosed and recurrent pediatric brain tumors. METHODS: Eighteen consecutive ablation procedures were performed in 17 patients from March 2016-April 2020. Patient demographics, indications, procedures, neuroimaging features, and outcomes were reviewed retrospectively. RESULTS: Seventeen patients (mean age of 11.4 years, 11 boys, 6 girls) underwent SLA with a mean follow-up of 24 months (range: 3-45 months). Tumor histologies included pilocytic astrocytoma (n = 5), ganglioglioma (n = 3), low-grade glioma not otherwise specified (n = 4), glioblastoma (n = 2), meningioma (n = 1), medulloblastoma (n = 1), and metastatic malignant peripheral nerve sheath tumor (n = 1). SLA was first-line therapy in 10 patients. Mean procedure duration including anesthesia time was 328 minutes (range: 244-529 minutes), and mean postoperative length of stay was 1.5 days (range 1-5 days). The complication rate was 29%, which included 3 patients who experienced postoperative motor changes, which resolved within several weeks of surgery, 1 patient with self-limited intraoperative bradycardia and hypotension, and 1 patient who died postoperatively due to intracranial hemorrhage from a distant lesion. Twelve of 17 patients had a neuroimaging response after SLA (4 complete responses, 8 partial responses, 1 stable disease). Percentage of tumor shrinkage from baseline ranged from 33%-100% (mean 75%). Patients with low-grade glioma exhibited the best responses to SLA (range 3%-100% decrease; mean 90%; 36% complete response rate). CONCLUSIONS: SLA is a minimally invasive modality for the treatment of newly diagnosed and recurrent low-grade pediatric brain tumors. Low-grade glioma exhibited the best responses. Identification of ideal candidates for SLA, mitigation of perioperative complications, and demonstration of long-term outcomes need to be better defined in a clinical trial setting.

Fetal Ultrasound and Magnetic Resonance Imaging Findings in Suspected Septo-Optic Dysplasia: A Diagnostic Dilemma.

OBJECTIVES: To investigate prenatal imaging findings supporting a diagnosis of suspected septo-optic dysplasia (SOD) by fetal ultrasound (US), magnetic resonance imaging (MRI), or both. METHODS: A retrospective review identified 11 patients with SOD: 9 had a clinical diagnosis of SOD postnatally, and 2 were terminated on the basis of suspicious prenatal imaging. Prenatal and neonatal imaging of the cavum septi pellucidi (CSP), frontal horns (FHs), and lateral ventricles was evaluated. RESULTS: The appearance of the CSP varied on US and MRI. Complete ("fused") FHs or partial absence of the CSP was reported in 6 of 11 patients by fetal US and 7 of 8 patients by fetal MRI. The diagnosis of SOD was prospectively suspected prenatally in 6 of 11 and in an additional 5 of 11 cases retrospectively. Fetal MRI incorrectly initially reported normal morphologic abnormalities for 2 cases with partial absence of the CSP, whereas US accurately identified the morphologic abnormalities in 1 of these cases before MRI. Imaging features were first suggested at anatomic US (4 patients) and follow-up prenatal US (2 patients). Neonatal imaging was concordant in all 9 live births: 5 completely absent CSP, 3 partially absent CSP, and 1 completely present CSP. Clinical manifestations included optic nerve hypoplasia (9 of 9), panhypopituitarism (5 of 9), and neurodevelopmental delays. CONCLUSIONS: Primary imaging features of SOD are "continuous" FHs with complete or partial absence of the CSP. Septo-optic dysplasia can be suspected in utero and can appear isolated but has substantial associated central nervous system anomalies identified on fetal MRI or after birth. Partial absence of the CSP can be a prenatal sign of suspected SOD, although fetal MRI lacked the spatial resolution to identify it accurately in all cases.

Undescended retropharyngeal parathyroid adenoma with adjacent thymic tissue in a 13-year-old boy with primary hyperparathyroidism.

We describe a rare presentation of a symptomatic parathyroid adenoma located in an ectopic retropharyngeal position in a 13-year-old boy. Preoperative CT scan and MRI demonstrated the ectopic location of the parathyroid adenoma. The patient underwent successful parathyroidectomy with cure of his hyperparathyroidism. On pathologic exam, the specimen was made up of a parathyroid adenoma and adjacent thymic tissue, indicating that it was likely an undescended lower parathyroid gland arising from the third pharyngeal pouch. Ectopic retropharyngeal parathyroid adenomas are very rare and to our knowledge, none have been previously described in adolescents.

Brain Magnetic Resonance Imaging Findings in Poorly Controlled Homocystinuria.

Homocystinuria is an inherited metabolic disorder most commonly caused by cystathionine ß-synthase deficiency. Severe cases can cause white matter abnormalities that can mimic other vascular, toxic and metabolic disorders on computed tomography and magnetic resonance imaging. We present such a case which demonstrates not only extensive white matter abnormalities on magnetic resonance imaging, but also previously unreported basal ganglia signal abnormalities and imaging manifestations of increased intracranial pressure, likely caused by elevated methionine and betaine therapy. We also review the literature and discuss the potential underlying biologic mechanisms of these imaging findings.

Single-agent bevacizumab in the treatment of recurrent or refractory pediatric low-grade glioma: A single institutional experience.

INTRODUCTION: Bevacizumab-based therapy has been demonstrated to be effective in the treatment of refractory or recurrent pediatric low-grade glioma (LGG); however its efficacy as a single agent is less understood. METHODS: We report our experience with single-agent bevacizumab for the treatment of recurrent or refractory LGG treated with either standard 2 week dosing (10 mg/kg/dose every 2 weeks) or with a standard 2 week dosing followed by an increased interval dosing (10 mg/kg/dose every 4 weeks). RESULTS: From 2012 to 2017, 15 patients (five males and 10 females) with recurrent/refractory LGG (nine suprasellar, three thalamic, two brainstem, and one intramedullary spinal cord) were treated with a total of 156 doses of bevacizumab (115 every 2 week dosing, 41 every 4 week dosing, median 10 doses). Patients were refractory to a median of one nonsurgical therapy (range 0-3) prior to treatment with bevacizumab. Twelve of 15 demonstrated radiographic response (three complete, nine partial, and three stable disease). Significant clinical responses including improved visual fields (four), cranial neuropathy (three3), strength (seven), and gait (two) were observed. Bevacizumab was discontinued in 12 patients (resolution, one; disease stability, seven; progression, two; toxicity, one; and other, one) and three patients continue to receive monthly bevacizumab. Eleven patients eventually had radiographic progression (median 5 months, range 0.5-31) without clinical progression, and four of five receiving bevacizumab rechallenge had lpartial response. CONCLUSION: Single-agent bevacizumab is efficacious in the management of recurrent or refractory pediatric LGG with radiographic and clinical responses similar to those reported for bevacizumab-based therapies.

Orbital Perivenous Abscess Complicating the Diagnosis and Management of Orbital Cellulitis.

An 11-year-old female presented with orbital cellulitis, bacterial sinusitis, enlarged left superior ophthalmic vein, dural venous sinuses, and internal jugular vein. The patient underwent endoscopic sinus surgery and was started on intravenous antibiotics and anticoagulation with limited improvement in orbital signs and symptoms. A magnetic resonance imaging/magnetic resonance venography of the orbits and brain revealed a dilated left superior ophthalmic vein with absence of flow but no clearly discernible orbital abscess. Intravenous corticosteroids resulted in dramatic improvement of pain, hypoglobus, proptosis, and extraocular motility, all of which rapidly recurred on discontinuation. Serial imaging revealed progression of what eventually manifested as a well-defined, rim-enhancing peri-superior ophthalmic vein abscess, which was incised and drained with prompt resolution of orbital cellulitis and complete visual recovery.

Atypical Teratoid Rhabdoid Tumor in a Teenager with Unusual Infiltration Into the Jugular Foramen.

BACKGROUND: Atypical teratoid rhabdoid tumor is a rare malignant neoplasm that represents 1%-2% of all pediatric central nervous system tumors. Immunohistochemistry plays an important role in establishing the diagnosis with a loss of INI-1 staining in tumor cells. In this case report, we describe a teenager with an unusual presentation and pattern of infiltration of the tumor. CASE DESCRIPTION: A 13-year-old boy presented with a history over several months of progressive nausea, weight loss, and hoarseness of voice associated with multiple lower cranial nerve palsies on neurologic examination. Magnetic resonance imaging revealed a large heterogeneously enhancing extra-axial neoplasm with extension and bony expansion of the jugular foramen. After near total resection, neuropathology demonstrated the absence of INI-1 expression consistent with a diagnosis of atypical teratoid rhabdoid tumor. CONCLUSIONS: This case highlights the diverse clinical presentation and infiltrative potential of atypical teratoid rhabdoid tumors, thus expanding the differential diagnosis of extra-axial tumors invading the jugular foramen.

FDG PET of the brain in pediatric patients: imaging spectrum with MR imaging correlation.

Positron emission tomography (PET) of the brain is an important problem-solving tool in pediatric neuroimaging, neurology, and neurosurgery. Fluorine 18 fluorodeoxyglucose (FDG) PET or dual-modality PET and computed tomographic (CT) imaging (PET/CT), with magnetic resonance (MR) imaging correlation, can be used to evaluate childhood epilepsy and pediatric brain tumors, areas in which PET adds value in patient management. FDG PET has been widely used in pediatric temporal lobe epilepsy, most commonly manifesting as mesial temporal sclerosis, which demonstrates hypometabolism at interictal PET and hypermetabolism during seizures. Recently, FDG PET has shown added value for patients with extratemporal epilepsy, in whom FDG PET can help identify cortical foci of interictal hypometabolism that are undetectable or difficult to detect with MR imaging. These findings can then guide additional investigations and surgery. FDG PET also enhances medical decision making in children with brain tumors, in whom FDG PET can be used to (a) improve the diagnostic yield of stereotactic biopsies by detecting metabolically active areas of tumor, (b) help guide the surgeon in achieving total tumor resection, and (c) increase detection of residual or recurrent tumor. Technologic advances in the past decade have allowed fusion of PET and MR images, combining the high resolution of MR imaging with the low-resolution functional capability of PET. As dual-modality integrated PET/MR imaging systems become available, CT coregistration for PET can be eliminated, thus reducing patient radiation exposure. Increasing familiarity with normal and abnormal appearances of FDG PET brain images correlated with MR images can enhance diagnostic yield and improve the care of children with epilepsy and brain tumors.

Oculoauriculofrontonasal syndrome: case series revealing new bony nasal anomalies in an old syndrome.

Frontonasal Dysplasia (FND) and Oculo-auriculo-vertebral spectrum (OAVS) are two well-recognized clinical entities. With features of both FND and OAVS, the term oculoauriculofrontonasal syndrome (OAFNS) was coined in 1981. The OAFNS phenotype combines elements of abnormal morphogenesis of the frontonasal and maxillary process (derived from forebrain neural crest) with abnormal development of the first and second branchial arches (derived from hindbrain neural crest). We present a case series of 33 children with OAFNS ascertained from a comprehensive review of the literature and report an additional retrospective series of eight patients displaying features consistent with OAFNS. Notably, in a subset of our cases, we have observed abnormalities in nasal ossification and bony structures of the maxilla that have not previously described in OAFNS and are not seen in either FND or OAVS. We present the phenotype and novel naso-maxillary findings and explore potential etiologic and developmental pathways for OAFNS. We highlight the differences in phenotypic characteristics of OAFNS compared to OAVS and FND. These observations support the classification of OAFNS as a discrete syndrome. Further phenotypic refinements of OAFNS are needed to understand pathogenesis of this syndrome and the newly described nasal malformation may help identify the etiology.

Making the diagnosis: metopic ridge versus metopic craniosynostosis.

INTRODUCTION: The metopic suture is the only calvarial suture which normally closes during infancy. Upon closure, a palpable and visible ridge often forms which can be confused with metopic craniosynostosis. Metopic ridging (MR) is treated nonsurgically while metopic craniosynostosis (MCS) is treated surgically. Differentiating between the two is paramount; however, consensus is lacking about where a clear diagnostic threshold lies. The goal of this study is to describe the physical examination and CT scan characteristics which may help to differentiate between physiological closure of the metopic suture with ridging (MR) and MCS. METHODS: A retrospective chart review of all patients seen at Seattle Children's Hospital between 2004 and 2009 with the diagnosis of either MCS or MR (n = 282) was performed. Physical examination characteristics described by diagnosing practitioners were analyzed. Clinical photos were assessed by 3 expert raters to determine the importance of these characteristics. CT scan findings were abstracted and compared between the two diagnoses. RESULTS: The "classic" triad of narrow forehead, biparietal widening, and hypotelorism was present in only 14% of patients with MCS. Ninety-eight percent of patients in both groups had a palpable metopic ridge. The photographic finding of narrow forehead and pterional constriction was present in all patients with MCS, but only in 11.2% and 2.8% of patients with MR. On CT scan, the presence of 3 or more MCS findings was diagnostic of MCS in 96% of patients. Patients with MCS were more likely to present before 6 months of age (66% vs. 32%). CONCLUSIONS: Patients with MCS tend to present earlier than those with MR. Upon physical examination, the relationship between the lateral frontal bone and the lateral orbit is important in distinguishing between the two diagnoses. A CT scan can be helpful in making the diagnosis not to confirm a closed suture but to identify 3 or more MCS characteristics.

Image-guided cerebrospinal fluid shunting in children: catheter accuracy and shunt survival.

OBJECT: Cerebrospinal fluid shunt placement has a high failure rate, especially in patients with small ventricles. Frameless stereotactic electromagnetic image guidance can assist ventricular catheter placement. The authors studied the effects of image guidance on catheter accuracy and shunt survival in children. METHODS: Pediatric patients who underwent placement or revision of a frontal ventricular CSF shunt were retrospectively evaluated. Catheters were placed using either anatomical landmarks or image guidance. Preoperative ventricular size and postoperative catheter accuracy were quantified. Outcomes of standard and image-guided groups were compared. RESULTS: Eighty-nine patients underwent 102 shunt surgeries (58 initial, 44 revision). Image guidance was used in the placement of 56 shunts and the standard technique in 46. Shunt failure rates were not significantly different between the standard (22%) and image-guided (25%) techniques (p = 0.21, log-rank test). Ventricular size was significantly smaller in patients in the image-guided group (p < 0.02, Student t-test) and in the surgery revision group (p < 0.01). Small ventricular size did not affect shunt failure rate, even when controlling for shunt insertion technique. Despite smaller average ventricular size, the accuracy of catheter placement was significantly improved with image guidance (p < 0.01). Shunt accuracy did not affect shunt survival. CONCLUSIONS: The use of image guidance improved catheter tip accuracy compared with a standard technique, despite smaller ventricular size. Failure rates were not dependent on shunt insertion technique, but an observed selection bias toward using image guidance for more at-risk catheter placements showed failure rates similar to initial surgeries.

Hedgehog pathway inhibitor saridegib (IPI-926) increases lifespan in a mouse medulloblastoma model.

The Sonic Hedgehog (Shh) pathway drives a subset of medulloblastomas, a malignant neuroectodermal brain cancer, and other cancers. Small-molecule Shh pathway inhibitors have induced tumor regression in mice and patients with medulloblastoma; however, drug resistance rapidly emerges, in some cases via de novo mutation of the drug target. Here we assess the response and resistance mechanisms to the natural product derivative saridegib in an aggressive Shh-driven mouse medulloblastoma model. In this model, saridegib treatment induced tumor reduction and significantly prolonged survival. Furthermore, the effect of saridegib on tumor-initiating capacity was demonstrated by reduced tumor incidence, slower growth, and spontaneous tumor regression that occurred in allografts generated from previously treated autochthonous medulloblastomas compared with those from untreated donors. Saridegib, a known P-glycoprotein (Pgp) substrate, induced Pgp activity in treated tumors, which likely contributed to emergence of drug resistance. Unlike other Smoothened (Smo) inhibitors, the drug resistance was neither mutation-dependent nor Gli2 amplification-dependent, and saridegib was found to be active in cells with the D473H point mutation that rendered them resistant to another Smo inhibitor, GDC-0449. The fivefold increase in lifespan in mice treated with saridegib as a single agent compares favorably with both targeted and cytotoxic therapies. The absence of genetic mutations that confer resistance distinguishes saridegib from other Smo inhibitors.

MRI of pediatric patients: Part 1, normal and abnormal cartilage.

OBJECTIVE: Cartilage development has a profound impact on musculoskeletal growth. The objective of this article is to offer insights about the maturation of hyaline cartilage through MRI. We begin by briefly describing the molecular make up of hyaline cartilage. We will then follow with a discussion of the basic principles to apply to optimize hyaline cartilage imaging. The remainder of the article will focus on the MR appearances of the distinct histologic types of hyaline cartilage, normal variations in cartilage development, and the sequelae of cartilage injury on normal skeletal development. CONCLUSION: Knowledge of the normal and abnormal appearances of hyaline cartilage on MRI of pediatric patients will allow readers to avoid mistaking the changes associated with skeletal maturation for pathologic findings.

MRI of pediatric patients: Part 2, normal variants and abnormalities of the knee.

OBJECTIVE: The purpose of this article is to discuss MRI of the pediatric knee and familiarize the reader with conditions encountered in the pediatric population. Clinical scenarios are included to convey important concepts and to orient the learner to normal variants and abnormalities of the pediatric knee. The conditions discussed include, but are not limited to, distal femoral metaphysial irregularity, isolated popliteus tendon avulsion, juvenile idiopathic arthritis, and discoid meniscus. CONCLUSION: The knee is the joint that is most commonly imaged by MRI in children. Injury patterns and signs of other pathologic processes seen in skeletally immature patients are different from those seen in adults. Interpreting pediatric knee MRI studies may be a challenge for those unfamiliar with the evolving patterns of normal development and of the signs of conditions that are more prevalent in children. Through case scenarios, this article describes and provides images that depict conditions commonly encountered in the pediatric knee. Most of the described normal findings and abnormalities are more prevalent in the pediatric population than in adults, and a few of the conditions are, in fact, unique to pediatric patients.

Neuroimaging of migrational disorders in pediatric epilepsy.

Seizures in children are common and represent a final pathway for a variety of brain insults. Although most children with seizures do not require imaging, when indicated, imaging plays an important role in the clinical workup. Imaging in the pediatric seizure population is reserved for a particular subset of patients depending on factors, such as age of onset, symptomatology, physical examination findings, and specific electroencephalography changes to name a few. The etiologies of seizures are extensive and include disorders of cortical migration and organization. Cortical migration and organization disorders are multifactorial and complex and a major cause of seizure disorders. Although magnetic resonance imaging is the most common imaging modality used to identify the seizure focus, positron emission tomographic and/or diffusion tensor imaging are beginning to provide complementary information about the involved areas. Early and accurate detection is key to better treatment and overall improved patient prognosis.