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Historically, the accuracy of imaging teeth by computed tomography (CT) has been suboptimal and deemed inadequate for surgical planning of orthognathic procedures. However, recent advances in CT hardware and software have significantly improved the accuracy of imaging occlusal anatomy. This technical note describes a quantitative means of evaluating the accuracy of CT-based modeling of teeth. Three-dimensional models of the dentition were created from a CT scan obtained of a craniomaxillofacial skeleton. Multiple reconstruction algorithms and modeling parameters were applied. The dentition of the same skeleton was scanned using a handheld optical scanning device to serve as the "gold standard." Semi-automated registrations of CT and optically acquired models were performed and deviation analysis was conducted. On average, the deviation of the CT model with the optical scan measured 0.19 to 0.25 mm across the various reconstruction and modeling parameters, with a mean of 0.22 mm. Computed tomography underestimated contours at cusp tips, while overestimating contours in occlusal groves. The use of bone reconstruction algorithms and decreased model smoothing resulted in more accurate models, though greater surface noise. Future studies evaluating the clinical effectiveness of CT-based occlusal splints should take this finding into account.

The study aimed to describe a case of a 7-year-old Caucasian girl who developed isolated chorea in her right upper and lower extremities progressively increasing over 2 years. This study is a case report and conducted at tertiary care center. A 7-year-old Caucasian girl who gradually developed worsening choreiform movements in her right upper and lower extremities over the course of 2 years until medical attention was sought. Literature review of children presenting with chorea as the only manifestation in moyamoya disease. A 7-year-old right-handed Caucasian girl presented with progressively worsening choreiform movements in her right upper and lower extremities affecting her fine motor skills and gait impairment There was no weakness, hyperreflexia, or spasticity on her neurological exam. Neuroimaging studies showed “ivy sign,” asymmetric prominence of vessels within the subarachnoid spaces overlying the left cerebral hemisphere with corresponding serpiginous T2 fluid-attenuated inversion recovery abnormality, but no parenchymal volume loss or diffusion restriction. Magnetic resonance angiography showed focal moderate-to-severe stenosis at the junction of the paraophthalmic and supraclinoid segments of the left internal carotid artery, with poststenotic dilatation. Angiography demonstrated focal stenosis involving the ophthalmic segment of the left internal carotid artery with 50% stenosis. There was compensation through a medium sized left posterior communicating artery as well as posterior cerebral artery to middle cerebral artery and anterior cerebral artery collateralization. Brain single-photon emission computerized tomography scan showed no evidence of perfusion defects in the cerebral hemispheres, basal ganglia, or thalami. She underwent successful revascularization procedure (left pial synangiosis) with resolution of her choreiform movements and normalization of her gait. Our case demonstrates that moyamoya disease should be suspected when evaluating a child with hemichorea and describes resolution of symptoms after revascularization surgery. Neuroimaging and vascular studies should be obtained in children with unilateral movement disorder.

OBJECTIVEBiopsies of brainstem lesions are performed to establish a diagnosis in the setting of an atypical clinical or radiological presentation, or to facilitate molecular studies. A better understanding of the safety and diagnostic yield of brainstem biopsies would help guide appropriate patient selection.METHODSAll patients who underwent biopsy of a brainstem lesion during the period from January 2011 to June 2019 were reviewed. Demographic, radiological, surgical, and outcome data were collected.RESULTSA total of 58 patients underwent 65 brainstem biopsies during the study period. Overall, the median age was 7.6 years (IQR 3.9–14.2 years). Twenty-two of the 65 biopsies (34%) were open, 42 (65%) were stereotactic, and 1 was endoscopic. In 3 cases (5%), a ventriculoperitoneal shunt was placed, and in 9 cases (14%), a posterior fossa decompression was performed during the same operative session as the biopsy. An intraoperative MRI (iMRI) was performed in 28 cases (43%). In 3 of these cases (11%), the biopsy was off target and additional samples were obtained during the same procedure. New neurological deficits were noted in 5 cases (8%), including sensory deficits, ophthalmoparesis/nystagmus, facial weakness, and hearing loss; these deficits persisted in 2 cases and were transient in 3 cases. A pseudomeningocele occurred in 1 patient; no patients developed a CSF leak or infection. In 8 cases (13%) an additional procedure was needed to obtain a diagnosis.CONCLUSIONSBrainstem biopsies are safe and effective. Target selection and approach should be a collaborative effort. iMRI can be used to assess biopsy accuracy in real time, thereby allowing any adjustment if necessary.

Background The trochlear nerve (the fourth cranial nerve) is the only cranial nerve that arises from the dorsal aspect of the midbrain. The nerve has a lengthy course making it highly susceptible to injury. It is also the smallest cranial nerve and is often difficult to identify on neuroimaging. Evidence acquisition High-resolution 3-dimensional skull base MRI allows for submillimeter isotropic acquisition and is optimal for cranial nerve evaluation. In this text, the detailed anatomy of the fourth cranial nerve applicable to imaging will be reviewed. Results Detailed anatomic knowledge of each segment of the trochlear nerve is necessary in patients with trochlear nerve palsy. A systematic approach to identification and assessment of each trochlear nerve segment is essential. Pathologic cases are provided for each segment. Conclusions A segmental approach to high-resolution 3-dimensional MRI for the study of the trochlear nerve is suggested.

Transcranial magnetic stimulation (TMS) is a newer noninvasive language mapping tool that is safe and well-tolerated by children. We examined the accuracy of TMS-derived language maps in a clinical cohort by comparing it against functional magnetic resonance imaging (MRI)–derived language map. The number of TMS-induced speech disruptions and the volume of activation during functional MRI tasks were localized to Brodmann areas for each modality in 40 patients with epilepsy or brain tumor. We examined the concordance between TMS- and functional MRI–derived language maps by deriving statistical performance metrics for TMS including sensitivity, specificity, accuracy, and diagnostic odds ratio. Brodmann areas 6, 44, and 9 in the frontal lobe and 22 and 40 in the temporal lobe were the most commonly identified language areas by both modalities. Overall accuracy of TMS compared to functional MRI in localizing language cortex was 71%, with a diagnostic odds ratio of 1.27 and higher sensitivity when identifying left hemisphere regions. TMS was more accurate in determining the dominant hemisphere for language with a diagnostic odds ratio of 6. This study is the first to examine the accuracy of the whole brain language map derived by TMS in the largest cohort examined to date. While this comparison against functional MRI confirmed that TMS reliably localizes cortical areas that are not essential for speech function, it demonstrated only slight concordance between TMS- and functional MRI–derived language areas. That the localization of specific language cortices by TMS demonstrated low accuracy reveals a potential need to use concordant tasks between the modalities and other avenues for further optimization of TMS parameters.

Homozygosity for nonsense variants in CEP55 has been associated with a lethal condition characterized by multinucleated neurons, anhydramnios, renal dysplasia, cerebellar hypoplasia, and hydranencephaly (MARCH syndrome) also known as Meckel-like syndrome. Missense variants in CEP55 have not previously been reported in association with disease. Here we describe seven living individuals from five families with biallelic CEP55 variants. Four unrelated individuals with microcephaly, speech delays, and bilateral toe syndactyly all have a common CEP55 variant c.70G>A p.(Glu24Lys) in trans with nonsense variants. Three siblings are homozygous for a consensus splice site variant near the end of the gene. These affected girls all have severely delayed development, microcephaly, and varying degrees of lissencephaly/pachygyria. Here we compare our seven patients with three previously reported families with a prenatal lethal phenotype (MARCH syndrome/Meckel-like syndrome) due to homozygous CEP55 nonsense variants. Our series suggests that individuals with compound heterozygosity for nonsense and missense variants in CEP55 have a different viable phenotype. We show that homozygosity for a splice variant near the end of the CEP55 gene is also compatible with life.

OBJECTIVEStenoocclusive cerebral vasculopathy is an infrequent delayed complication of ionizing radiation. It has been well described with photon-based radiation therapy but less so following proton-beam radiotherapy. The authors report their recent institutional experience in evaluating and treating children with radiation-induced cerebral vasculopathy.METHODSEligible patients were age 21 years or younger who had a history of cranial radiation and subsequently developed vascular narrowing detected by MR arteriography that was significant enough to warrant cerebral angiography, with or without ischemic symptoms. The study period was January 2011 to March 2019.RESULTSThirty-one patients met the study inclusion criteria. Their median age was 12 years, and 18 (58%) were male. Proton-beam radiation therapy was used in 20 patients (64.5%) and photon-based radiation therapy was used in 11 patients (35.5%). Patients were most commonly referred for workup as a result of incidental findings on surveillance tumor imaging (n = 23; 74.2%). Proton-beam patients had a shorter median time from radiotherapy to catheter angiography (24.1 months [IQR 16.8–35.4 months]) than patients who underwent photon-based radiation therapy (48.2 months [IQR 26.6–61.1 months]; p = 0.04). Eighteen hemispheres were revascularized in 15 patients. One surgical patient suffered a contralateral hemispheric infarct 2 weeks after revascularization; no child treated medically (aspirin) has had a stroke to date. The median follow-up duration was 29.2 months (IQR 21.8–54.0 months) from the date of the first catheter angiogram to last clinic visit.CONCLUSIONSAll children who receive cranial radiation therapy from any source, particularly if the parasellar region was involved and the child was young at the time of treatment, require close surveillance for the development of vasculopathy. A structured and detailed evaluation is necessary to determine optimal treatment.

Cortical stimulation mapping is the gold standard for presurgical language mapping; however, it cannot be reliably performed in very young patients. Language mapping using noninvasive modalities is also challenging in very young patients. Although utility of language mapping using power of high-gamma in electrocorticographic recordings was demonstrated in adults and older children, there is a gap of knowledge in the ability of this procedure for localizing language-specific cortex in very young patients. We describe a case of a 2-year-old patient who, to our knowledge, is the youngest person to undergo successful high-gamma electrocorticographic presurgical language mapping for localization of the expressive language cortex (Broca area). The surgical plan was to resect a cortical tuber within the left inferior frontal gyrus and there was a strong concern about postoperative language deficit after resection. Presurgical language mapping using noninvasive modalities were attempted without success. Cortical stimulation mapping was not feasible in this patient. Therefore, high-gamma electrocorticography was the only viable option for language mapping, and it successfully localized the expressive language cortex. The patient underwent surgery for resection of the IFG tuber based on results of high-gamma electrocorticography and had no postoperative language deficit. High-gamma electrocorticography can be used for localizing language-specific cortex, especially Broca’s area, in very young patients.

Introduction Flat panel imaging for emergent large vessel occlusion can be acquired prior to mechanical thrombectomy (MT). In this study, we examined patients undergoing MT with computed tomography angiography (CTA) to determine agreement on the site of occlusion and CTA collateral score (CS). Methods Flat Panel CTA (FP-CTA) was acquired before MT. Time between CTA and FP-CTA acquisition, site of occlusion, and CS were reported. Significant CS change was defined as >2-point change, or any change to/from a malignant profile (CS = 0 to CS > 0, or vice versa). Results Eleven patients (mean age, 60.8 years; NIHSS, 17; 55.0% female) were included; IV tPA was administered to 7. Intra-reader occlusion site, dichotomous CS, and continuous CS correlation between CTA and FP-CTA were 96.6%, 90.0%, and 86.6%, respectively. Inter-reader correlation for occlusion site was 93% for CTA and 100% for FP-CTA; dichotomous CS correlation was 87% for both CTA and FP-CTA; correlation of continuous CS was 77% for CTA and 87% for FP-CTA. Conclusion Standard CTA and FP-CTA have high intra and inter-reader correlation determining site of occlusion and CS in ELVO setting. This angiographic tool may have potential applications for both triage and patient selection.

Acute changes in mental status represent a broad collection of symptoms used to describe disorders in mentation and level of arousal, including the more narrowly defined diagnoses of delirium and psychosis. A wide range of precipitating factors may be responsible for symptom onset including infection, intoxication, and metabolic disorders. Neurologic causes that may be detected on neuroimaging include stroke, traumatic brain injury, nonconvulsive seizure, central nervous system infection, tumors, hydrocephalus, and inflammatory disorders. Not infrequently, two or more precipitating factors may be found. Neuroimaging with CT or MRI is usually appropriate if the clinical suspicion for an acute neurological cause is high, where the cause of symptoms is not found on initial assessment, and for patients whose symptoms do not respond appropriately to management. There was disagreement regarding the appropriateness of neuroimaging in cases where a suspected, nonneurologic cause is found on initial assessment. Neuroimaging with CT is usually appropriate for patients presenting with delirium, although the yield may be low in the absence of trauma or a focal neurological deficit. Neuroimaging with CT or MRI may be appropriate in the evaluation of new onset psychosis, although the yield may be low in the absence of a neurologic deficit. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

A palpable neck mass may be the result of neoplastic, congenital, or inflammatory disease. Older age suggests neoplasia, and a congenital etiology is more prevalent in the pediatric population. The imaging approach is based on the patient age, mass location, and clinical pulsatility. Underlying human papillomavirus-related malignancy should be considered in all age groups. Although the imaging appearance of some processes in the head and neck overlap, choosing the appropriate imaging examination may allow a specific diagnosis, or a limited differential diagnosis. Tissue sampling is indicated to confirm suspected malignancy. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

Nontraumatic neck pain is a leading cause of disability, with nearly 50% of individuals experiencing ongoing or recurrent symptoms. Radiographs are appropriate as initial imaging for cervical or neck pain in the absence of "red flag" symptoms or if there are unchanging chronic symptoms; however, spondylotic changes are commonly identified and may result in both false-positive and false-negative findings. Noncontrast CT can be complementary to radiographs for evaluation of new or changing symptoms in the setting of prior cervical spine surgery or in the assessment of extent of ossification in the posterior longitudinal ligament. Noncontrast MRI is usually appropriate for assessment of new or increasing radiculopathy due to improved nerve root definition. MRI without and with contrast is usually appropriate in patients with new or increasing cervical or neck pain or radiculopathy in the setting of suspected infection or known malignancy. Imaging may be appropriate; however, it is not always indicated for evaluation of cervicogenic headache without neurologic deficit. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

OBJECTIVE An ischemic stroke following an elective craniotomy in a child is perceived to be a rare event. However, to date there are few papers on this topic. The purpose of this study was to investigate the occurrence of stroke following elective intracranial surgery at a children’s hospital. METHODS The authors performed a retrospective review of all patients who developed a perioperative stroke following an elective craniotomy from 2010 through 2017. Data were collected using an institutional database that contained demographic, medical, radiological, and outcome variables. RESULTS A total of 1591 elective craniotomies were performed at the authors’ institution during the study period. Of these, 28 (1.8%) were followed by a perioperative stroke. Radiographic diagnosis of the infarction occurred at a median of 1.7 days (range 0–9 days) from the time of surgery, and neurological deficits were apparent within 24 hours of surgery in 18 patients (62.5%). Infarcts tended to occur adjacent to tumor resection sites (86% of cases), and in a unilateral (89%), unifocal (93%), and supratentorial (93%) location. Overall, 11 (39.3%) strokes were due to a perforating artery, 10 (35.7%) were due to a large vessel, 4 (14.3%) were venous, and 3 (10.7%) were related to hypoperfusion or embolic causes. Intraoperative MRI (iMRI) was used in 11 of the 28 cases, and 6 (55%) infarcts were not detected, all of which were deep. CONCLUSIONS The incidence of stroke following an elective craniotomy is low, with nearly all cases (86%) occurring after tumor resection. Perforator infarcts were most common but may be missed on iMRI. ABBREVIATIONS ACA = anterior cerebral artery; AChA = anterior choroidal artery; ACS NSQIP-P = American College of Surgeons National Surgical Quality Improvement Program–Pediatric; CVA = cerebrovascular accident; DWI = diffusion weighted imaging; iMRI = intraoperative MRI; MCA = middle cerebral artery; mRS = modified Rankin Scale; PCA = posterior cerebral artery.

The authors describe a case of unilateral abducens nerve palsy following perinatal stroke of the middle cerebral artery. A 1-year-old boy presented with left eye esotropia but no other ocular abnormalities. The patient's history, examination, and diagnostic tests were consistent with abducens nerve palsy. He underwent left medial rectus recession of 5.5 mm and left lateral rectus resection of 7 mm followed by patching. At 15 months after surgery, primary gaze by prism alternate cover testing revealed a 4 prism diopter (PD) esophoria (small angle in left gaze with essentially no action of lateral rectus) and 2 PD right hyperphoria. Ophthalmologic management of abducens nerve palsy entails addressing neurological sequelae in a timely manner, treating the esotropia and strabismic amblyopia to optimize visual system development. [ J Pediatr Ophthalmol Strabismus . 2020;57:e30–e33.]

This article presents guidelines for imaging utilization in patients presenting with hearing loss or vertigo, symptoms that sometimes occur concurrently due to proximity of receptors and neural pathways responsible for hearing and balance. These guidelines take into account the superiority of CT in providing bony details and better soft-tissue resolution offered by MRI. It should be noted that a dedicated temporal bone CT rather than a head CT best achieves delineation of disease in many of these patients. Similarly, optimal assessment often requires a dedicated high-resolution protocol designed to assess temporal bone and internal auditory canals even though such a study will be requested and billed as a brain MRI. Angiographic techniques are helpful in some patients, especially in the setting of vertigo. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

Background Herpes Simplex Virus encephalitis (HSVE) is a devastating disease of all ages. Rigorous studies correlating viral load with neuroradiological and clinical severity have not been performed, particularly in neonates. Understanding these relationships may improve therapies. Objectives To correlate molecularly quantified HSV in cerebrospinal fluid (CSF) and disease severity. Study Design HSV loads (VL) were evaluated by real-time PCR from the CSF of 33 patients (20 neonates, 5 children, 8 adults) with HSVE. We studied relationships between CSF VL and structural and volumetric brain abnormalities (MRI); hospital morbidity; and discharge and long-term (>3 month) clinical outcomes. Results Initial CSF VL did not differ in neonates vs non-neonates (median 4.6 vs 5.1 log10 copies/mL, p = 0.75). Initial CSF VL was higher in neonates with HSV-2 vs HSV-1 (median 4.8 vs 3.2 log10 copies/mL, respectively, p = 0.02). Persistently detectable DNA in CSF despite acyclovir trended towards higher odds of unfavorable outcome at discharge for neonates [0.87 (CI 0.75–1), p = 0.07]. Initial VL correlated with higher CSF protein concentrations for the cohort and for neonates (p = 0.03 and 0.01, respectively), but not with lesion volume or subarachnoid exposure of involved brain (p all >0.05), hospital morbidity (p all >0.05), nor with higher odds of unfavorable discharge or long-term outcomes for the cohort [OR = 0.9(CI 0.5–1.6), p = 0.72; OR = 1.0(CI 0.5–1.8), p = 0.9] or for neonates [OR = 1.3(CI 0.5–3.3), p = 0.57; OR = 2.3(CI 0.7–8), p = 0.2]. Conclusions Initial HSV VL did not predict neuroradiological or clinical outcomes in patients with HSVE, suggesting host inflammatory factors contribute to disease in treated patients with good viral clearance.

Sinonasal imaging is performed in 2 major clinical scenarios: inflammatory rhinosinusitis or suspected mass lesion. Rhinosinusitis affects more than 16% of the US population annually. It poses an immense economic burden, accounting for more than 26 million outpatient visits annually and costing more than $4.3 billion annually in direct medical expenses. Most cases of uncomplicated acute and subacute rhinosinusitis are diagnosed clinically and should not require any imaging procedure. CT of the sinuses without contrast is the imaging method of choice in patients with recurrent acute sinusitis or chronic sinusitis. Sinusitis cannot be diagnosed on the basis of imaging findings alone. CT scan findings should be interpreted in conjunction with clinical and endoscopic findings. MRI is currently used for evaluation of sinus disease as a complementary study in cases of aggressive sinus infection with ocular/intracranial complications, potential invasive fungal sinusitis in immunocompromised patients or in the evaluation of a sinonasal mass. The ACR Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed every 2 years by a multidisciplinary expert panel. The guideline development and review include an extensive analysis of current medical literature from peer-reviewed journals and the application of a well-established consensus methodology (modified Delphi) to rate the appropriateness of imaging and treatment procedures by the panel. In those instances where evidence is lacking or not definitive, expert opinion may be used to recommend imaging or treatment.

Tinnitus is the perception of sound in the absence of an external source. It is a common symptom that can be related to hearing loss and other benign causes. However, tinnitus may be disabling and can be the only symptom in a patient with a central nervous system process disorder. History and physical examination are crucial first steps to determine the need for imaging. CT and MRI are useful in the setting of pulsatile tinnitus to evaluate for an underlying vascular anomaly or abnormality. If there is concomitant asymmetric hearing loss, neurologic deficit, or head trauma, imaging should be guided by those respective ACR Appropriateness Criteria® documents, rather than the presence of tinnitus. Imaging is not usually appropriate in the evaluation of subjective, nonpulsatile tinnitus that does not localize to one ear. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

MRI without and with contrast is the most accurate imaging method to determine whether a process is intrinsic or extrinsic to a nerve of the brachial or lumbosacral plexus. However, there are no Current Procedural Terminology codes to correspond to imaging studies of the brachial or lumbar plexus discretely. This assessment uses "MRI of the brachial plexus" or "MRI of the lumbosacral plexus" as independent entities given that imaging acquisition for the respective plexus differs in sequences and planes compared with those of a routine neck, chest, spine, or pelvic MRI, yet acknowledges the potential variability of ordering practices across institutions. In patients unable to undergo MRI, CT offers the next highest level of anatomic evaluation. In oncologic patients, PET/CT imaging can identify the extent of tumor involvement and be beneficial to differentiate radiation plexitis from tumor recurrence but provides limited resolution of the plexus itself. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer-reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

BACKGROUND Pineoblastomas are rare, supratentorial, primitive neuroectodermal tumors. OBJECTIVE To document outcomes with multimodal therapy and evaluate the impact that the degree of surgical resection has on outcome. METHODS A departmental brain tumor database was queried to identify all patients with pathologically proven pineoblastoma who were treated from January 1997 to June 2015 at St. Jude Children's Research Hospital. For each patient, we recorded demographic, pathological, radiological, surgical, and clinical follow-up data. The effect of degree of surgical resection on survival outcomes was analyzed. RESULTS Forty-one patients (21 male, 20 female) treated for pineoblastoma were identified. The median age at diagnosis was 5.5 years (range 0.4-28.1) and the median follow-up was 34.5 months. Nineteen patients experienced tumor relapse with a median progression-free survival of 11.3 months, and 18 ultimately succumbed to their disease. Patients who died or experienced treatment failure were younger (median, 2.69 vs 6.5 years, P = .026) and more likely to have metastatic disease at diagnosis (12 [63.2%] vs 5 [22.7%], P = .012). When analyzing only patients 5 years of age or older with focal disease at presentation, those who had a gross total resection or near-total resection-compared with subtotal resection or biopsy-had greater overall survival (75.18 vs 48.57 months), with no patients dying as a result of their cancer. CONCLUSION Poor prognostic variables for children with pineoblastoma include young age, metastatic disease at presentation, and tumor relapse. For patients older than 5 years with focal disease, maximal tumor resection should be the goal.

Neonatal head ultrasound has a key role in triaging neonates with antenatal imaging or postnatal clinical concerns. This article will discuss key features of various intracranial pathologies of concern in term infants. It will also illustrate various congenital malformations.

Cerebellar tumors are the most common group of solid tumors in children. MR imaging provides an important role in characterization of these lesions, surgical planning, and postsurgical surveillance. Preoperative imaging can help predict the histologic subtype of tumors, which can provide guidance for surgical planning. Beyond histology, pediatric brain tumors are undergoing new classification schemes based on genetic features. Intraoperative MR imaging has emerged as an important tool in the surgical management of pediatric brain tumors. Effective understanding of the imaging features of pediatric cerebellar tumors can benefit communication with neurosurgeons and neuro-oncologists and can improve patient management.

Background Three-dimensional rotational angiography (3DRA) offers more detailed anatomic information than 2D digital acquisition (2DDA). Concerns over potentially higher contrast and radiation doses have limited its routine use. Objective The primary objective of this study was to compare radiation doses required to obtain 3DRA using a customized low dose radiation protocol with 2DDA. The secondary objective was to compare total procedural radiation in pediatric cardiac catheterization procedures utilizing 3DRA to those that do not. Study Design Phantom studies were conducted to establish customized 3DRA protocols for radiation reduction. Comparison of 3DRA and non-3DRA procedures in age-, size- and diagnosis-matched controls was performed. Radiation doses were indexed to body surface area (BSA) to account for differing body habitus as validated from the phantom study. Results Study (n = 100) and control (n = 100) groups were matched for age (10.2 vs. 9.98 years; P = .239) and BSA (1.23 vs. 1.09 m2; P = .103). The dose area product (DAP) to acquire a 3DRA was similar to a 5 s, 15 frames/second 2DDA (278 vs. 241 cGy/cm2; P = .14). Despite the 3DRA group consisting of more complex interventions, no difference was found in the total procedural Air Kerma and DAP indexed to BSA (244 vs. 249 mGy/m2; P = .79 and 3348 vs. 3176 cGy/cm2/m2; P = .48, respectively). The contrast volume to acquire a 3DRA compared to a 2DDA was greater (1.59 vs. 1.01 mL/kg; P

Accurate assessment of motor function is critical, particularly in the context of presurgical functional mapping. There are a few noninvasive tools available for preoperative motor mapping in older children and adults undergoing surgery for brain tumor or refractory epilepsy including fMRI, magnetoencephalography (MEG), and transcranial magnetic stimulation (TMS).1,2 However, assessing motor function is challenging in very young children. Direct cortical stimulation, a standard procedure in adults, is associated with a high incidence of intraoperative seizures, and is often unsuccessful in young children.3,4 Alternately, noninvasive motor mapping using MEG and fMRI attempted during sleep or under sedation primarily localize the somatosensory cortex from which the location of motor cortex is inferred.5,6 Furthermore, these methods have a low success rate (∼25%) and have risks associated with sedation.6 However, TMS has the advantage of mapping the motor cortex directly without requiring sedation. We have previously reported on successful mapping of the motor cortex in young children aged between 17 and 35 months.7 Here we describe a case of an 11-month-old infant who is, to our knowledge, the youngest person to undergo successful presurgical motor mapping with TMS.

Nontraumatic neck pain is a leading cause of disability, with nearly 50% of individuals experiencing ongoing or recurrent symptoms. Radiographs are appropriate as initial imaging for cervical or neck pain in the absence of "red flag" symptoms or if there are unchanging chronic symptoms; however, spondylotic changes are commonly identified and may result in both false-positive and false-negative findings. Noncontrast CT can be complementary to radiographs for evaluation of new or changing symptoms in the setting of prior cervical spine surgery or in the assessment of extent of ossification in the posterior longitudinal ligament. Noncontrast MRI is usually appropriate for assessment of new or increasing radiculopathy due to improved nerve root definition. MRI without and with contrast is usually appropriate in patients with new or increasing cervical or neck pain or radiculopathy in the setting of suspected infection or known malignancy. Imaging may be appropriate; however, it is not always indicated for evaluation of cervicogenic headache without neurologic deficit. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

Acute changes in mental status represent a broad collection of symptoms used to describe disorders in mentation and level of arousal, including the more narrowly defined diagnoses of delirium and psychosis. A wide range of precipitating factors may be responsible for symptom onset including infection, intoxication, and metabolic disorders. Neurologic causes that may be detected on neuroimaging include stroke, traumatic brain injury, nonconvulsive seizure, central nervous system infection, tumors, hydrocephalus, and inflammatory disorders. Not infrequently, two or more precipitating factors may be found. Neuroimaging with CT or MRI is usually appropriate if the clinical suspicion for an acute neurological cause is high, where the cause of symptoms is not found on initial assessment, and for patients whose symptoms do not respond appropriately to management. There was disagreement regarding the appropriateness of neuroimaging in cases where a suspected, nonneurologic cause is found on initial assessment. Neuroimaging with CT is usually appropriate for patients presenting with delirium, although the yield may be low in the absence of trauma or a focal neurological deficit. Neuroimaging with CT or MRI may be appropriate in the evaluation of new onset psychosis, although the yield may be low in the absence of a neurologic deficit. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

Background and importance Presurgical mapping of eloquent cortex in young patients undergoing neurosurgery is critical for timely intervention, surgical planning, and minimizing postoperative deficits. However, invasive direct cortical stimulation has limited success in young children and noninvasive modalities, such as magnetoencephalography and functional MRI, require sedation, often precluding localization of critical language cortices. Transcranial magnetic stimulation (TMS), a noninvasive brain stimulation technique, is well suited to evaluate language areas in young children because it does not require the patient to remain still during mapping. Clinical presentation A 4-yr and 11-mo-old female patient diagnosed with epilepsia partialis continua of the right arm and face and right-sided weakness was evaluated at our institution. MRI findings and clinical examination led to the diagnosis of Rasmussen encephalitis involving left frontal lobe and insula. Language cortices were successfully identified in both hemispheres using TMS. The TMS findings aided in discussing with the family the risks of postsurgical deficits of left functional hemispherectomy, the definitive treatment for Rasmussen encephalitis. Postoperatively, the patient had intact speech and was seizure free. Conclusion We illustrate the feasibility and utility of TMS as a noninvasive functional mapping tool in this young child. The preoperative demonstration of bilateral language organization indicated a greater likelihood of preserved language functions postsurgery. We demonstrate that TMS is a safe and noninvasive tool to map language cortices in young children with serious epilepsy syndromes.

This article presents guidelines for imaging utilization in patients presenting with hearing loss or vertigo, symptoms that sometimes occur concurrently due to proximity of receptors and neural pathways responsible for hearing and balance. These guidelines take into account the superiority of CT in providing bony details and better soft-tissue resolution offered by MRI. It should be noted that a dedicated temporal bone CT rather than a head CT best achieves delineation of disease in many of these patients. Similarly, optimal assessment often requires a dedicated high-resolution protocol designed to assess temporal bone and internal auditory canals even though such a study will be requested and billed as a brain MRI. Angiographic techniques are helpful in some patients, especially in the setting of vertigo. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

A 14-year-old girl with a history of mid-line defects, basal encephalocele and morning glory disc anomaly presented with untreated growth hormone deficiency, pubertal delay and hypothyroidism. She was found to have a large craniopharyngeal canal based on MRI scan. Craniopharyngeal canal is an uncommon condition that has not been well described in the pediatric population. Consideration of craniopharyngeal canal in the differential diagnosis for basal encephaloceles and understanding its presentation can impact medical decision making and follow-up for patients.

OBJECTMalignant tumors of the brainstem, excluding classic diffuse intrinsic pontine gliomas (DIPGs), are a very rare, heterogeneous group of neoplasms that have been infrequently described in the literature. In this paper, the authors present their experiences with treating these unique cancers.METHODSA retrospective chart review was conducted to identify eligible cases over a 15-year period. All tumors involving the pons were, by consensus, felt not to be DIPGs based on their neuroimaging features. Demographic information, pathological specimens, neuroimaging characteristics, surgical and nonsurgical management plans, and survival data were gathered for analysis.RESULTSBetween January 2000 and December 2014, 29 patients were identified. The mean age at diagnosis was 8.4 years (range 2 months to 25 years), and 17 (59%) patients were male. The most common presenting signs and symptoms were cranial neuropathies (n = 24; 83%), hemiparesis (n = 12; 41%), and ataxia or gait disturbance (n = 10; 34%). There were 18 glial and 11 embryonal tumors. Of the glial tumors, 5 were radiation-induced and 1 was a malignant transformation of a previously known low-grade tumor. Surgical intervention consisted of biopsy alone in 12 patients and some degree of resection in another 15 patients. Two tumors were diagnosed postmortem. The median overall survival for all patients was 196 days (range 15 to 3999 days). There are currently 5 (17%) patients who are still alive: 1 with an anaplastic astrocytoma and the remaining with embryonal tumors.CONCLUSIONSIn general, malignant non-DIPG tumors of the brainstem carry a poor prognosis. However, maximal cytoreductive surgery may be an option for select patients with focal tumors. Long-term survival is possible in patients with nonmetastatic embryonal tumors after multimodal treatment, most importantly maximal resection.

Background Weakness of the sixth cranial nerve is the most common cause of an ocular motor cranial nerve palsy. It is often difficult to identify a corresponding abnormality on neuroimaging to correlate with the clinical examination. Evidence acquisition High-resolution 3D skull base magnetic resonance imaging (MRI) allows for visualization of the sixth nerve along much of its course and may increase sensitivity for abnormalities in regions that previously were challenging to evaluate. In this review, the authors share their experience with high-resolution imaging of the sixth nerve. Results For each segment, anatomic features visible on high-resolution imaging are described along with relevant pathologic entities. Conclusions We present a segmental approach to high-resolution 3D MRI for evaluation of the sixth nerve from the nuclear to the orbital segment.

OBJECT Cervicomedullary tumors (CMTs) represent a heterogeneous group of intrinsic neoplasms that are typically low grade and generally carry a good prognosis. This single-institution study was undertaken to document the outcomes and current treatment philosophy for these challenging neoplasms. METHODS The charts of all pediatric patients with CMTs who received treatment at St. Jude Children’s Research Hospital between January 1988 and May 2013 were retrospectively reviewed. Demographic, surgical, clinical, radiological, pathological, and survival data were collected. Treatment-free survival and overall survival were estimated, and predictors of recurrence were analyzed. RESULTS Thirty-one children (16 boys, 15 girls) with at least 12 months of follow-up data were identified. The median age at diagnosis was 6 years (range 7 months-17 years) and the median follow-up was 4.3 years. Low-grade tumors (Grade I or II) were present in 26 (84%) patients. Thirty patients underwent either a biopsy alone or resection, with the majority of patients undergoing biopsy only (n = 12, 39%) or subtotal resection (n = 14, 45%). Only 4 patients were treated solely with resection; 21 patients received radiotherapy alone or in combination with other treatments. Recurrent tumor developed in 14 children (45%) and 4 died as a result of their malignancy. A high-grade pathological type was the only independent variable that predicted recurrence. The 5- and 10-year treatment-free survival estimates are 64.7% and 45.3%, respectively. The 5- and 10-year overall survival estimate is 86.7%. CONCLUSIONS Children with CMTs typically have low-grade neoplasms and consequently long-term survival, but high risk of recurrence. Therapy should be directed at achieving local tumor control while preserving and even restoring neurological function.

Progeroid disorders overlapping with De Barsy syndrome (DBS) are collectively denoted as autosomal-recessive cutis laxa type 3 (ARCL3). They are caused by biallelic mutations in PYCR1 or ALDH18A1, encoding pyrroline-5-carboxylate reductase 1 and pyrroline-5-carboxylate synthase (P5CS), respectively, which both operate in the mitochondrial proline cycle. We report here on eight unrelated individuals born to non-consanguineous families clinically diagnosed with DBS or wrinkly skin syndrome. We found three heterozygous mutations in ALDH18A1 leading to amino acid substitutions of the same highly conserved residue, Arg138 in P5CS. A de novo origin was confirmed in all six probands for whom parental DNA was available Using fibroblasts from affected individuals and heterologous overexpression, we found that the P5CS-p.Arg138Trp protein was stable and able to interact with wild-type P5CS but showed an altered sub-mitochondrial distribution. A reduced size upon native gel electrophoresis indicated an alteration of the structure or composition of P5CS mutant complex. Furthermore, we found that the mutant cells had a reduced P5CS enzymatic activity leading to a delayed proline accumulation. In summary, recurrent de novo mutations, affecting the highly conserved residue Arg138 of P5 CS, cause an autosomal-dominant form of cutis laxa with progeroid features. Our data provide insights into the etiology of cutis laxa diseases and will have immediate impact on diagnostics and genetic counseling.

Despite the frequent occurrence of autism spectrum disorder (ASD) in tuberous sclerosis complex (TSC), the neu- rophysiological factors that distinguish children with TSC from children with TSC presenting with ASD symptoms remain unspecified. Growing evidence suggests that ASD may be characterized by atypical structural and functional connectivity be- tween specific cortical regions. In this exploratory study, we utilized magnetoencephalography to derive resting brain connec- tivity patterns, in an attempt to identify neurophysiological markers that may differentiate TSC children with ASD (n = 2) from TSC children without ASD (n = 2) and typically developing children (n = 2). Connectivity pattern analysis revealed that TSC children presenting with ASD symptoms can be distinguished from TSC and typically developing children by the presence of long-range, medial, anterior-posterior connections previously observed in adolescents with ASD. While preliminary in nature, our findings support the notion that altered functional connectivity may be a constituent characteristic of ASD and may enable prediction of which TSC children are likely to develop ASD and facilitate early behavioral and medical intervention.

Moyamoya disease and syndrome are typically described by their imaging features on cerebral angiography, however advances in noninvasive imaging has allowed improved anatomic and physiologic characterization of this vascular abnormality. Advanced imaging complements the role of cerebral angiography, and when used appropriately can provide valuable information regarding the diagnosis, treatment planning, and surveillance in patients with moyamoya. Techniques to be aware of include computed tomography, CT-angiography, Magnetic Resonance Imaging, MR Angiography (time-of-flight and dynamic contrast enhancemed angiography), and perfusion imaging (CT perfusion, arterial spin labeling perfusion, and nuclear medicine perfusion). With an appropriate understanding and implementation of noninvasive techniques, this disease process can be better understood allowing for improved patient management.

The development of high-quality intraoperative MRI (iMRI) capability has offered a major advance in the care of patients with complex intracranial disease. To date, this technology has been limited by the need for pin fixation of the calvaria. The authors report their preliminary experience with an MRI-compatible horseshoe headrest that allows for the following: 1) iMRI in patients too young for pin fixation; 2) iMRI in patients with large calvarial defects; 3) the ability to move the head during iMRI surgery; and 4) the use of neuronavigation in such cases. The authors report 2 cases of infants in whom the Visius Surgical Theatre horseshoe headrest (IMRIS Inc.) was used. Image quality was equivalent to that of pin fixation. The infants suffered no skin issues. The use of neuronavigation with the system remained accurate and could be updated with the new iMRI information. The Visius horseshoe headrest offers a technical advance in iMRI technology for infants, for patients with cranial defects or prior craniotomies in whom pin fixation may not be safe, or for patients in whom the need to move the head during surgery is required. The image quality of the system remains excellent, and the ability to merge new images to the neuronavigation system is helpful.

The incidence of primary malignant and nonmalignant central nervous system (CNS) tumors in children and adolescents aged 0–19 years in the US is 5.42 per 100,000, and approximately 4620 new cases are expected to be diagnosed in the US in 2015 (Ostrom et al. 2014). There is a rich variety of brain tumors found in children which is primarily a function of the patient’s age and location of origin, with the overall most common being pilocytic astrocytoma (Ostrom et al. 2014). It has been traditionally taught that approximately 60% of pediatric brain tumors are infratentorial, but the actual ratio of supratentorial to infratentorial pediatric tumors is dependent on the specific age group (Ostrom et al. 2015). Tumors can be broadly categorized as glial (e.g., astrocytomas, ependymomas), embryonal (e.g., medulloblastomas, pineoblastoma), germ cell (e.g., germinoma, teratoma), and other (e.g., choroid plexus tumors, craniopharyngiomas).

Identifying the normal relationship of the orbital rims to the globes is critical in planning surgical correction of craniofacial deformities affecting the orbit. This article illustrates a technical proof of principle and mathematical basis for a computed tomography-based measurement of the sagittal orbit-globe relationship. The technique does not require subject cooperation and is, therefore, optimal for pediatric craniofacial surgical treatment planning and outcome evaluation.