Axial MR Angiography in Evaluating Revascularization after Indirect Bypass Surgery for Moyamoya.

INTRODUCTION: At our institution, revascularization after indirect moyamoya surgery is routinely evaluated using magnetic resonance angiography (MRA) rather than catheter angiography. This study reviews how revascularization can be visualized on axial MRA versus catheter angiography and compares clinical outcomes of surgeries evaluated by routine postoperative MRA versus routine catheter angiography. METHODS: We reviewed the records of all patients treated at our institution who underwent unilateral encephaloduroarteriosynangiosis (EDAS)/pial synangiosis 2004-2021 at 1-21 years of age. Inclusion criteria included undergoing preoperative MRA within 18 months of surgery and postoperative MRA 3 to 30 months after surgery. Clinical outcome measures included postoperative stroke and transient ischemic attacks (TIAs), changes in symptoms (improved, unchanged, worsened), and new postoperative symptoms. Measures were compared between surgeries evaluated by routine postoperative MRA versus routine postoperative angiograms. For each surgery, we determined the ratios of the diameters and areas of the donor and contralateral corresponding vessels and the relative signal intensities of these two vessels on preoperative- and 3-to-30-month postoperative MRA. We did the same for the middle meningeal artery (MMA) ipsilateral to the donor artery and the contralateral MMA. We assessed changes from pre- to post-operation in diameter ratios, area ratios, relative signal intensity, ivy sign, and brain perfusion on arterial spin labeled (ASL) imaging. MRI and MRA measures of revascularization and flow were compared to Matsushima grades in patients who had postoperative catheter angiograms. RESULTS: Fifty-one operations were included. There were no significant differences in rates of strokes, TIAs, changes or new symptoms after surgeries evaluated by routine postoperative MRA versus catheter angiogram. Significant associations existed between greater collateralization on postoperative MRA and greater median increases in preoperative-to-postoperative ratios of donor-vessel-over-contralateral-vessel diameter (p=0.0461) and ipsilateral-MMA-over-contralateral-MMA diameter (p=0.0135). The median increase in the ratio of the donor-vessel-over-corresponding-contralateral-vessel diameters was significantly higher for Matsushima grade A versus B (p=0.036). The median increase in the ratio of the sum of donor-and-ipsilateral-MMA diameters over the sum of the contralateral vessel diameters was significantly higher for improved-versus-unchanged perfusion on ASL imaging (p=0.0074). There was a nonsignificant association between greater postoperative collateralization on MRA and Matsushima grade (p=0.1160) Conclusion: Cerebral revascularization after EDAS/pial synangiosis can be evaluated on axial MRA by comparing the diameter and/or signal intensity of the donor vessel and ipsilateral MMA to those of the corresponding contralateral vessels on postoperative-versus-preoperative MRA. The use of routine postoperative MRA rather than catheter angiography does not appear to negatively affect outcomes.

Radiation dose reduction using spectral shaping in pediatric non-contrast sinus CT.

BACKGROUND: CT is the standard imaging technique to evaluate pediatric sinuses. Given the potential risks of radiation exposure in children, it is important to reduce pediatric CT dose and maintain image quality. OBJECTIVE: To study the utility of spectral shaping with tin filtration to improve dose efficiency for pediatric sinus CT exams. MATERIALS AND METHODS: A head phantom was scanned on a commercial dual-source CT using a conventional protocol (120 kV) and a proposed 100 kV with a 0.4-mm tin filter (Sn100 kV) protocol for comparison. Entrance point dose (EPD) of eye and parotid gland region was measured by an ion chamber. Sixty pediatric sinus CT exams (33 acquired with 120 kV, 27 acquired with Sn100 kV) were retrospectively collected. All patient images were objectively measured for image quality and blindly reviewed by 4 pediatric neuroradiologists for overall noise, overall diagnostic quality, and delineation of 4 critical paranasal sinus structures, using a 5-point Likert scale. RESULTS: Phantom CTDIvol from Sn100 kV is 4.35 mGy, compared to CTDIvol of 5.73 mGy from 120 kV at an identical noise level. EPD of sensitive organs decreases in Sn100 kV (e.g., right eye EPD 3.83±0.42 mGy), compared to 120 kV (5.26±0.24 mGy). Patients in the 2 protocol groups were age and weight (unpaired T test P>0.05) matched. The patient CTDIvol of Sn100 kV (4.45±0.47 mGy) is significantly lower than 120 kV (5.56±0.48 mGy, unpaired T test P<0.001). No statistically significant difference for any subjective readers' score (Wilcoxon test P>0.05) was found between the two groups, indicating proposed spectral shaping provides equivalent diagnostic image quality. CONCLUSION: Phantom and patient results demonstrate that spectral shaping can significantly reduce radiation dose for non-contrast pediatric sinus CT without compromising diagnostic quality.

Ophthalmic manifestations of ruptured arachnoid cysts in children.

BACKGROUND: Intracranial arachnoid cysts are relatively common in the pediatric population. Rarely, they rupture, leading to acute subdural fluid collections, which can cause a sudden increase in intracranial pressure. The purpose of this study was to characterize ophthalmic sequelae in a large cohort of these patients. METHODS: The medical records of all children treated for ruptured arachnoid cysts who presented at a single tertiary pediatric hospital for initial assessment between 2009 and 2021 were reviewed retrospectively. RESULTS: Of 35 children treated for ruptured arachnoid cysts during the study period, 30 received ophthalmological examination. Papilledema was found in 57% of these children, abducens palsy in 20%, and retinal hemorrhages in 10%. Of the 30 children, 22 were seen in outpatient follow-up, of whom 5 had a best-corrected visual acuity of 20/40 or worse in one or both eyes at most recent follow-up. Cranial nerve palsies resolved in all cases without strabismus surgery. CONCLUSIONS: Given high rates of papilledema, cranial nerve palsies, and vision loss, all children with ruptured arachnoid cysts should be evaluated by pediatric ophthalmologists.

Use of the Posterior Auricular Artery for Indirect Bypass in Moyamoya: A Pediatric Case Series.

INTRODUCTION: Encephaloduroarteriosynangiosis (EDAS) for moyamoya is predominantly performed using a branch of the superficial temporal artery (STA) as the donor artery. At times, other branches of the external carotid artery are better suited for EDAS than is the STA. There is little information in the literature concerning using the posterior auricular artery (PAA) for EDAS in the pediatric age-group. In this case series, we review our experience using the PAA for EDAS in children and adolescents. CASE PRESENTATIONS: We describe the presentations, imaging, and outcomes of 3 patients in whom the PAA was used for EDAS, as well our surgical technique. There were no complications. All 3 patients were confirmed to have radiologic revascularization from their surgeries. All patients also had improvement of their preoperative symptoms, and no patient has had a stroke postoperatively. CONCLUSION: The PAA is a viable option for use as a donor artery in EDAS for the treatment of moyamoya in children and adolescents.

Fetal MRI Neuroradiology: Indications.

Fetal MRI is a safe, noninvasive examination of the fetus and placenta, a complement to ultrasonography. MRI provides detailed CNS evaluation, including depicting parenchymal architecture and posterior fossa morphology, and is key in prenatal assessment of spinal dysraphism, neck masses, and ventriculomegaly. Fetal MRI is typically performed after 22 weeks gestation, and ultrafast T1 and T2-weighted MRI sequences are the core of the exam, with advanced sequences such as diffusion weighted imaging used for specific questions. The fetal brain grows and develops rapidly, and familiarity with gestational age specific norms is essential to MRI interpretation.

Imaging of Congenital Spine Malformations.

Congenital malformations of the spine and spinal cord are a large and diverse group of diagnoses, which are often broadly referred to as spinal dysraphisms (SDs). Derived from the Greek words dys (bad) and raphe (suture), the term dysraphism describes missteps in the process of forming a midline seam during the zipper-like fusion of the neural folds in primary neurulation. As such, the term "spinal dysraphism" is a designation that should technically be reserved for malformations resulting from aberrations in primary neurulation. In medical practice, however, it is a catch-all designation regularly used to describe any of the numerous abnormalities demonstrating incomplete midline closure of mesenchymal, osseous, and nervous tissue, occurring at any point during embryologic development. For the sake of clarity and completeness, this article will also include that breadth in the discussion of congenital abnormalities of the spine.

Imaging of Macrocephaly.

Macrocephaly is a common diagnosis in the pediatric population, particularly in the infantile time period. There is a wide range of causes of macrocephaly, from benign to malignant, for which imaging plays a key role in the diagnosis and clinical guidance. Our aim is to review the distinct and prevalent neuroimaging findings in the evaluation of the macrocephalic infant.

Use of Magnetic Resonance Imaging in Neuroprognostication After Pediatric Cardiac Arrest: Survey of Current Practices.

BACKGROUND: Use of magnetic resonance imaging (MRI) as a tool to aid in neuroprognostication after cardiac arrest (CA) has been described, yet details of specific indications, timing, and sequences are unknown. We aim to define the current practices in use of brain MRI in prognostication after pediatric CA. METHODS: A survey was distributed to pediatric institutions participating in three international studies. Survey questions related to center demographics, clinical practice patterns of MRI after CA, neuroimaging resources, and details regarding MRI decision support. RESULTS: Response rate was 31% (44 of 143). Thirty-four percent (15 of 44) of centers have a clinical pathway informing the use of MRI after CA. Fifty percent (22 of 44) of respondents reported that an MRI is obtained in nearly all patients with CA, and 32% (14 of 44) obtain an MRI in those who do not return to baseline neurological status. Poor neurological examination was reported as the most common factor (91% [40 of 44]) determining the timing of the MRI. Conventional sequences (T1, T2, fluid-attenuated inversion recovery, and diffusion-weighted imaging/apparent diffusion coefficient) are routinely used at greater than 97% of centers. Use of advanced imaging techniques (magnetic resonance spectroscopy, diffusion tensor imaging, and functional MRI) were reported by less than half of centers. CONCLUSIONS: Conventional brain MRI is a common practice for prognostication after CA. Advanced imaging techniques are used infrequently. The lack of standardized clinical pathways and variability in reported practices support a need for higher-quality evidence regarding the indications, timing, and acquisition protocols of clinical MRI studies.

"Growing cerebellum" requiring operative decompression following perinatal ventriculoperitoneal shunting.

INTRODUCTION: While cases of acquired Chiari I malformation following ventriculoperitoneal shunting for posthemorrhagic hydrocephalus have been reported, true disproportionate cerebellar growth is rare, with no previous cases requiring posterior fossa decompression reported. CLINICAL PRESENTATION: We present a premature neonate who underwent ventriculoperitoneal shunt placement for suspected posthemorrhagic hydrocephalus. He subsequently developed a symptomatic Chiari I malformation with volumetric measurements demonstrating disproportionate growth of the cerebellum. He did not demonstrate thickening of the supratentorial or posterior fossa cranium. The patient underwent an extradural posterior fossa decompression, with resolution of symptoms. OUTCOME AND CONCLUSIONS: We review the extant literature regarding the development of Chiari malformation type I as a manifestation of craniocerebral disproportion (CCD) following shunt placement for posthemorrhagic hydrocephalus of prematurity. Most previous reports reflect a mechanism that includes underdevelopment of the intracranial posterior fossa (or supratentorial) volume. The case presented in this report, as well as one additional case, indicates that there may exist a variant mechanism, characterized by rapid growth of the cerebellum itself, in the absence of one of the rare syndromes associated with primary macrocerebellum. While this case was effectively managed with extradural posterior fossa decompression, previous reports indicate that supratentorial cranial expansion procedures are preferable in some cases. As such, pediatric neurosurgeons should be able to distinguish the patterns of craniocerebral disproportion when considering treatment options for these patients. Further investigation regarding these uncommon patients may better describe the underlying mechanisms.

Ocular Findings in Pontine Tegmental Cap Dysplasia.

PURPOSE: To describe the ocular complications experienced by patients with pontine tegmental cap dysplasia (PTCD) and the management strategies used to care for these children. METHODS: Subjects with PTCD were recruited through social media advertisement and completed a survey gathering information on potential ocular problems related to the patient's PTCD disease and any current or previous treatments. RESULTS: Twenty-two patients or guardians completed the survey. Neurotrophic cornea was the most common ocular diagnosis (82%), followed by facial palsy (59%), dry eye syndrome (59%), and blepharitis (55%). Other diagnoses included cortical visual impairment (27%), strabismus (27%), amblyopia (18%), and nystagmus (18%). Common treatment modalities included lubricating eye drops (59%) or ointment (50%), contact lenses (14%), punctal plugs (27%), glasses (45%), and patching (18%). The most common surgical interventions were temporary or permanent tarsorrhaphy (64%) and amniotic membrane grafts (23%). In total, 68% of families reported self-injury to eyes and 91% reported the child to be primarily a visual learner. CONCLUSIONS: PTCD is a newly described, very rare disorder with a variety of vision-threatening ocular manifestations. It is essential that the ophthalmologist be aware of the potential for neurotrophic cornea because timely treatment could prevent corneal scarring, perforation, and blindness.

COL4A1 mutations in two infants with congenital cataracts and porencephaly: an ophthalmologic perspective.

COL4A1 mutations present with a spectrum of clinical phenotypes often involving the cerebrovascular and ophthalmic systems. We report 2 cases of COL4A1 mutations that presented with congenital cataracts and porencephaly. Both patients had posterior cortical cataracts and radiographically defined bilateral posterior lenticonus. Considering the long-term clinical implications of these mutations, posterior cortical cataracts, bilateral posterior lenticonus, and porencephaly should raise clinical suspicion for COL4A1 mutations.

Outcomes of Colorado children with acute flaccid myelitis at 1 year.

OBJECTIVE: We describe long-term functional, neurodiagnostic, and psychosocial outcomes of a cohort of 12 children from Colorado diagnosed with acute flaccid myelitis (AFM) in 2014. METHODS: Children were assessed every 3 months for 1 year or until clinical resolution. Assessments included neurologic examination, MRI, EMG/nerve conduction studies (NCS), functional measures (Assisting Hand Assessment, Hammersmith Functional Motor Scale), and Patient-Reported Outcomes Measurement Information System questionnaires. RESULTS: Eight of 12 children completed the study. Six of 8 had persistent motor deficits at 1 year; 2 demonstrated full recovery. Four were not enrolled, 2 of whom reported full recovery. The 6 affected were weakest in proximal muscles, showing minimal to no improvement and significant atrophy at 1 year. All patients improved in distal muscle groups. Cranial nerve dysfunction resolved in 2 of 5 and improved in all. Four of 5 showed progressive functional improvement at 6 and 12 months. Two of 8 reported pain at 1 year. Three of 8 reported depressive symptoms. Repeat MRI was performed in 7 of 8 children a median of 7 months after onset and showed significant improvement or normalization in all but one child. Repeat EMG/NCS was performed on 4 children a median of 8 months after onset and showed ongoing denervation and chronic reinnervation in 3 children with persistent deficits. CONCLUSIONS: At 1 year, children with AFM demonstrated functional gains but weakness persisted. EMG changes correlated with persistent deficits better than imaging. Despite improvements, AFM had substantial long-term functional effects on affected children.

Pediatric Brain: Repeated Exposure to Linear Gadolinium-based Contrast Material Is Associated with Increased Signal Intensity at Unenhanced T1-weighted MR Imaging.

Purpose To determine whether repeated exposure of the pediatric brain to a linear gadolinium-based contrast agent (GBCA) is associated with an increase in signal intensity (SI) relative to that in GBCA-naive control subjects at unenhanced T1-weighted magnetic resonance (MR) imaging. Materials and Methods This single-center, retrospective study was approved by the institutional review board and compliant with HIPAA. The authors evaluated 46 pediatric patients who had undergone at least three GBCA-enhanced MR examinations (30 patients for two-group analysis and 16 for pre- and post-GBCA exposure comparisons) and 57 age-matched GBCA-naive control subjects. The SI in the globus pallidus, thalamus, dentate nucleus, and pons was measured at unenhanced T1-weighted MR imaging. Globus pallidus-thalamus and dentate nucleus-pons SI ratios were calculated and compared between groups and relative to total cumulative gadolinium dose, age, sex, and number of and mean time between GBCA-enhanced examinations. Analysis included the Wilcoxon signed rank test, Wilcoxon rank sum test, and Spearman correlation coefficient. Results Patients who underwent multiple GBCA-enhanced examinations had increased SI ratios within the dentate nucleus (mean SI ratio ± standard error of the mean for two-group comparison: 1.007 ± 0.0058 for GBCA-naive group and 1.046 ± 0.0060 for GBCA-exposed group [P < .001]; mean SI ratio for pre- and post-GBCA comparison: 0.995 ± 0.0062 for pre-GBCA group and 1.035 ± 0.0063 for post-GBCA group [P < .001]) but not the globus pallidus (mean SI ratio for two-group comparison: 1.131 ± 0.0070 for GBCA-naive group and 1.014 ± 0.0091 for GBCA-exposed group [P = .21]; mean SI ratio for pre- and post-GBCA comparison: 1.068 ± 0.0094 for pre-GBCA group and 1.093 ± 0.0134 for post-GBCA group [P = .12]). There was a significant correlation between dentate nucleus SI and total cumulative gadolinium dose (r = 0.4; 95% confidence interval [CI]: 0.03, 0.67; P = .03), but not between dentate nucleus SI and patient age (r = 0.23; 95% CI: -0.15, 0.56; P = .22), sex (mean SI ratio: 1.046 ± 0.0072 for boys and 1.045 ± 0.0110 for girls; P = .88), number of contrast-enhanced examinations (r = 0.13; 95% CI: -0.25, 0.48; P = .49), or time between contrast-enhanced examinations (r = -0.06; 95% CI: -0.42, 0.32; P = .75). Conclusion SI in the pediatric brain increases on unenhanced T1-weighted MR images with repeated exposure to a linear GBCA. © RSNA, 2016.

A cluster of acute flaccid paralysis and cranial nerve dysfunction temporally associated with an outbreak of enterovirus D68 in children in Colorado, USA.

BACKGROUND: Clusters of acute flaccid paralysis or cranial nerve dysfunction in children are uncommon. We aimed to assess a cluster of children with acute flaccid paralysis and cranial nerve dysfunction geographically and temporally associated with an outbreak of enterovirus-D68 respiratory disease. METHODS: We defined a case of neurological disease as any child admitted to Children's Hospital Colorado (Aurora, CO, USA) with acute flaccid paralysis with spinal-cord lesions involving mainly grey matter on imaging, or acute cranial nerve dysfunction with brainstem lesions on imaging, who had onset of neurological symptoms between Aug 1, 2014, and Oct 31, 2014. We used Poisson regression to assess whether the numbers of cases during the outbreak period were significantly greater than baseline case numbers from a historical control period (July 31, 2010, to July 31, 2014). FINDINGS: 12 children met the case definition (median age 11·5 years [IQR 6·75-15]). All had a prodromal febrile illness preceding neurological symptoms by a median of 7 days (IQR 5·75-8). Neurological deficits included flaccid limb weakness (n=10; asymmetric n=7), bulbar weakness (n=6), and cranial nerve VI (n=3) and VII (n=2) dysfunction. Ten (83%) children had confluent, longitudinally extensive spinal-cord lesions of the central grey matter, with predominant anterior horn-cell involvement, and nine (75%) children had brainstem lesions. Ten (91%) of 11 children had cerebrospinal fluid pleocytosis. Nasopharyngeal specimens from eight (73%) of 11 children were positive for rhinovirus or enterovirus. Viruses from five (45%) of 11 children were typed as enterovirus D68. Enterovirus PCR of cerebrospinal fluid, blood, and rectal swabs, and tests for other causes, were negative. Improvement of cranial nerve dysfunction has been noted in three (30%) of ten children. All ten children with limb weakness have residual deficits. INTERPRETATION: We report the first geographically and temporally defined cluster of acute flaccid paralysis and cranial nerve dysfunction in children associated with an outbreak of enterovirus-D68 respiratory illness. Our findings suggest the possibility of an association between enterovirus D68 and neurological disease in children. If enterovirus-D68 infections continue to happen in an endemic or epidemic pattern, development of effective antiviral or immunomodulatory therapies and vaccines should become scientific priorities. FUNDING: National Center for Advancing Translational Sciences, National Institutes of Health.