Imaging Stewardship: Triage for Neuroradiology MR During Limited-Resource Hours.

OBJECTIVES: To decrease call burden on pediatric neuroradiologists, we developed guidelines for appropriate use of MR overnight. These guidelines were implemented using triage by in-house generalist pediatric radiologists. Process measures and balancing measures were assessed during implementation. METHODS: For this improvement project, interdepartmental consensus guidelines were developed using exploratory mixed-methods design. Implementation of triage used plan-do-study-act cycles. Process measures included reduction in the number of telephone calls, frequency of calls, triage decisions, and number and type of examinations ordered. Balancing measures included burden of time and effort to the generalist radiologists. Differences in examination orders between implementation intervals was assessed using Kruskal-Wallis, with significance at P < .05. RESULTS: Consensus defined MR requests as "do," "defer," or "divert" (to CT). Guidelines decreased neuroradiologist calls 74% while adding minimal burden to the generalist radiologists. Most nights had zero or one triage request and the most common triage decision was "do," and the most common examination was routine brain MR. Number of MR ordered and completed overnight did not significantly change with triage. DISCUSSION: Multidisciplinary consensus for use of pediatric neurological MR during limited resource hours overnight is an example of imaging stewardship that decreased the burden of calls and burnout for neuroradiologists while maintaining a comparable level of service to the ordering clinicians.

Summary of the 2022 ACR Intersociety Meeting.

The ACR Intersociety Committee meeting of 2022 (ISC-2022) was convened around the theme of "Recovering From The Great Resignation, Moral Injury and Other Stressors: Rebuilding Radiology for a Robust Future." Representatives from 29 radiology organizations, including all radiology subspecialties, radiation oncology, and medical physics, as well as academic and private practice radiologists, met for 3 days in early August in Park City, Utah, to search for solutions to the most pressing problems facing the specialty of radiology in 2022. Of these, the mismatch between the clinical workload and the available radiologist workforce was foremost-as many other identifiable problems flowed downstream from this, including high job turnover, lack of time for teaching and research, radiologist burnout, and moral injury.

A retrospective study of the impact of supraglottic airway devices on the appearance of neck masses in children undergoing serial magnetic resonance imaging.

BACKGROUND: General anesthesia and sedation are used routinely for magnetic resonance imaging (MRI) studies in children to optimize image quality. Airway devices such as supraglottic airways (SGAs) can alter the appearance of cervical soft tissue regions on an MRI and increase the risk of misdiagnosis. This phenomenon has not been well described in vivo. AIMS: We conducted this retrospective study to determine how often SGAs affected the appearance of neck masses in children who received multiple anesthetics for MRIs with and without an SGA. METHODS: We retrieved data on children 17 years old and younger who had multiple MRIs between January 2005 and January 2015. Inclusion criteria were patients with neck masses who had a SGA for at least one MRI and either a natural airway or endotracheal tube (ETT) for another MRI. We reviewed MRI images and imaging reports to determine if SGAs affected the appearance of neck masses. RESULTS: Twelve of the 921 patients who received anesthesia for neck MRIs during the study period met the inclusion criteria. SGAs affected the appearance of the neck mass in 11 of the 12 patients. CONCLUSIONS: Supraglottic airways can significantly alter the appearance of neck masses in children undergoing MRIs and affect radiologists' ability to assess those masses. Communication with the radiologist prior to the induction of anesthesia is crucial when using supraglottic devices in this patient population. It may be more prudent to use a different airway device and/or anesthetic technique when MRIs of these neck masses are undertaken.

Massive cortical reorganization is reversible following bilateral transplants of the hands: evidence from the first successful bilateral pediatric hand transplant patient.

In this repeated measures case study, we show that sensory deafferentation after limb amputation leads to changes in cortical somatotopic maps which are reversible after restoration of sensory input. Using magnetoencephalography (MEG), we observed in a child with bilateral hand transplants large-scale shifts in somatosensory lip cortical representation from anatomic hand area to anatomic face region. After recovery of tactile sensation in the digits, responses to finger stimulation were localized to orthotopic sensory cortex, but with atypical electrophysiologic features (amplitude and frequencies).

Neuromagnetic responses to tactile stimulation of the fingers: Evidence for reduced cortical inhibition for children with Autism Spectrum Disorder and children with epilepsy.

The purpose of this study was to compare somatosensory responses from a group of children with epilepsy and a group of children with autism spectrum disorder (ASD), with age matched TD controls. We hypothesized that the magnitude of the tactile "P50m" somatosensory response would be reduced in both patient groups, possibly due to reduced GABAergic signaling as has been implicated in a variety of previous animal models and in vivo human MRS studies. We observed significant (~ 25%) decreases in tactile P50m dipole moment values from the source localized tactile P50m response, both for children with epilepsy and for children with ASD. In addition, the latency of the tactile P50m peak was observed to be equivalent between TD and ASD groups but was significantly delayed in children with epilepsy by ~ 6 ms. Our data support the hypothesis of impaired GABAergic signaling in both children with ASD and children with epilepsy. Further work is needed to replicate these findings and directly relate them to both in vivo measures of GABA via e.g. magnetic resonance spectroscopy and psychophysical assessments of somatosensory function, and behavioral indices.

Anatomical features of the cervical spinal canal in Chiari I deformity with presyrinx: A case-control study.

Purpose The relationship between syringomyelia and presyrinx, characterized by edema in the spinal cord, has not been firmly established. Patients with syringomyelia have abnormal spinal canal tapering that alters cerebrospinal fluid flow dynamics, but taper ratios in presyrinx have never been reported. We tested the hypothesis that presyrinx patients have abnormal spinal canal tapering. Materials and methods At six medical institutions, investigators searched the PACS system for patients with Chiari I and spinal cord edema unassociated with tumor, trauma, or other evident cause. In each case taper ratios were calculated for C1 to C4 and C4 to C7. In two age- and gender-matched control groups, Chiari I patients with no syringomyelia and patients with normal MR scans, the same measurements were made. Differences between groups were tested for statistical significance with t tests. Results The study enrolled 21 presyrinx patients and equal numbers of matched Chiari I and normal controls. C4 to C7 taper ratios were positive and steeper in presyrinx patients than in the normal controls ( p = 0.04). The upper cervical spine, C1 to C4, tapered negatively in cases and controls without significant differences between the groups. The difference in degree of tonsillar herniation was statistically significant between presyrinx patients and Chiari I controls ( p = 0.01). Conclusions Presyrinx patients have greater than normal positive tapering in the lower cervical spine and greater degree of tonsillar herniation than the controls.

Reduced Radiation in Children Presenting to the ED With Suspected Ventricular Shunt Complication.

BACKGROUND: Ventricular shunt complications in children can be severe and life-threatening if not identified and treated in a timely manner. Evaluation for shunt obstruction is not without risk, including lifetime cumulative radiation as patients routinely receive computed tomography (CT) scans of the brain and shunt series (multiple radiographs of the skull, neck, chest, and abdomen). METHODS: A multidisciplinary team collaborated to develop a clinical pathway with the goal of standardizing the evaluation and management of patients with suspected shunt complication. The team implemented a low-dose CT scan, specifically tailored for the detection of hydrocephalus and discouraged routine use of shunt series with single-view radiographs used only when specifically indicated. RESULTS: There was a reduction in the average CT effective dose (millisievert) per emergency department (ED) encounter of 50.6% (confidence interval, 46.0-54.9; P ≤ .001) during the intervention period. There was a significant reduction in the number of shunt surveys obtained per ED encounter, from 62.4% to 5.32% (P < .01). There was no significant change in the 72-hour ED revisit rate or CT scan utilization rate after hospital admission. There were no reports of inadequate patient evaluations or serious medical events. CONCLUSIONS: A new clinical pathway has rapidly reduced radiation exposure, both by reducing the radiation dose of CT scans and eliminating or reducing the number of radiographs obtained in the evaluation of patients with ventricular shunts without compromising clinical care.

18-month outcomes of heterologous bilateral hand transplantation in a child: a case report.

BACKGROUND: Although heterologous vascular composite allotransplantation has become a burgeoning treatment option for adult amputees, there have been no successful cases previously reported in children. Here, we describe the surgical, immunological, and neurorehabilitation details with functional outcomes 18 months after heterologous bilateral hand and forearm transplantation in an 8-year-old child with quadrimembral amputations and a previous kidney transplant. METHODS: 2 years of extensive preparation by medical and surgical teams preceded the hand-forearm transplantation of this child. The initial immunosuppressive protocol included thymoglobulin, tacrolimus, prednisone, and mycophenolate mofetil. In July, 2015, our vascularised composite allotransplantation team did the first bilateral hand and forearm transplantation in a child, an 8-year-old boy with previous living-related kidney transplantation. The surgery included four teams working simultaneously on the donor and recipient limbs, aided by customised cutting guides that aimed to reduce ischaemia time. Following an extended length of time in hospital, skin biopsies and close monitoring of renal function and drug concentrations occurred weekly for the first 3 months and were slowly tapered to monthly, and then quarterly. Skin biopsies were also done when tissue rejection was suspected. Paediatric-specific rehabilitation techniques were applied to promote patient engagement during rehabilitation. Progress was assessed by monthly sensory and motor function tests during routine clinic visits and with serial functional brain imaging studies, including structural brain MRI, magnetoencephalography and transcranial magnetic stimulation. FINDINGS: The surgery lasted 10 h and 40 min. Vascular revision of the ulnar artery was required a few hours postoperatively. There were no further immediate postsurgical complications. Rejection episodes occurred throughout the first year but were reversed. An increase in serum creatinine led to the addition of sirolimus at 3 months after transplantation with concomitant reduction in tacrolimus targets. Sensibility to light touch was present by 6 months after transplantation. Intrinsic hand muscle innervation was present by 7-10 months after transplantation. At 18 months, the child had exceeded his previous adapted abilities. As of 18 months after transplantation surgery he is able to write and feed, toilet, and dress himself more independently and efficiently than he could do before transplantation. He remains on four immunosuppressive medications and functional neuroimaging studies have shown motor and somatosensory cortical reorganisation. INTERPRETATION: Hand transplantation in a child can be surgically, medically, and functionally successful under carefully considered circumstances. Long-term data on the functional trajectory, neurological recovery, psychological sequelae, and the potential late effect of immunosuppression are still needed to support broader implementation of paediatric vascular composite allotransplantation. FUNDING: The Children's Hospital of Philadelphia.

Diagnostic features of myelomeningocele: the role of ultrafast fetal MRI.

Fetal magnetic resonance imaging (MRI) is a useful adjunct to ultrasound in the evaluation of neural tube defects. The development of ultrafast imaging techniques has contributed to the increasing clinical use of fetal MRI. This review summarizes the utility, safety and technical aspects of fetal MRI and the role it plays in the evaluation of myelomeningocele. Emphasis is placed on the benefit of fetal MRI as it pertains to presurgical planning and perinatal management.

Utility of CT-compatible EEG electrodes in critically ill children.

BACKGROUND: Electroencephalographic monitoring is being used with increasing frequency in critically ill children who may require frequent and sometimes urgent brain CT scans. Standard metallic disk EEG electrodes commonly produce substantial imaging artifact, and they must be removed and later reapplied when CT scans are indicated. OBJECTIVE: To determine whether conductive plastic electrodes caused artifact that limited CT interpretation. MATERIAL AND METHODS: We describe a retrospective cohort of 13 consecutive critically ill children who underwent 17 CT scans with conductive plastic electrodes during 1 year. CT images were evaluated by a pediatric neuroradiologist for artifact presence, type and severity. RESULTS: All CT scans had excellent quality images without artifact that impaired CT interpretation except for one scan in which improper wire placement resulted in artifact. CONCLUSION: Conductive plastic electrodes do not cause artifact limiting CT scan interpretation and may be used in critically ill children to permit concurrent electroencephalographic monitoring and CT imaging.

Atypical syncope in a child due to a colloid cyst of the third ventricle.

A 10-year-old girl presented to an emergency room with acute-onset, brief, repetitive episodes of loss of consciousness. Computed tomography indicated a 0.6 cm colloid cyst of the anterior third ventricle, adjacent to the foramen of Monro. This finding was confirmed by magnetic resonance imaging. The patient underwent transcallosal surgical resection of the cyst without major complications or neurologic sequelae, and remains symptom-free after more than 18 months. Syncope is quite common in children. In contrast, colloid cysts are relatively uncommon in children, with only 100 cases reported in the literature. Colloid cysts are a known cause of sudden death. The possibility of colloid cyst should be considered in the differential diagnosis of syncope that presents in an atypical fashion, and such cases warrant emergent evaluation via neuroimaging.

Morphometric analysis of posterior fossa after in utero myelomeningocele repair.

OBJECT: Myelomeningocele (MMC) is characterized by a defect in caudal neurulation and appears at birth with a constellation of neuroanatomical abnormalities, including Chiari malformation Type II. The authors investigated the effects of antenatal versus postnatal repair of MMC through a quantitative analysis of morphometric changes in the posterior fossa (PF). METHODS: The authors retrospectively reviewed the records of 29 patients who underwent in utero MMC repair, 24 patients who underwent postnatal repair, and 114 fetal and pediatric controls. Tonsillar displacement, cerebellum length, pons length, clivus-supraocciput (CSO) angle, and PF area were compared in antenatal and postnatal MMC repair groups as well as in controls without neural tube defects by using t-tests and correlation coefficients. RESULTS: Initially, the in utero CSO angle was significantly more acute in all patients with MMC--prenatally and postnatally repaired--as compared with controls (57.8° vs 75.4°, p < 0.001); however, the angle rapidly changed and became similar to that in controls between 30 and 31 weeks' gestation to approximately 80°, with antenatal repair having little effect. Postnatally, the CSO angle decreased in controls (R = -0.58) and in the antenatal repair group (R = -0.17). The cerebellum and pons length demonstrated no significant differences in any group. Overall, tonsil descent was corrected in the antenatal repair group as compared with postnatal repair (p < 0.001), and the PF area increased in all 3 groups in utero. Growth was less rapid in patients with MMC compared with controls, but this was corrected by antenatal repair (p = 0.015). CONCLUSIONS: Myelomeningocele was associated with tonsillar herniation and a smaller PF than in control fetuses. Antenatal surgical repair corrected both abnormalities. The CSO angle began significantly more acutely in patients with MMC, but normalized with development regardless of when surgery was performed. Determining the clinical effects of antenatal repair requires further follow-up.

Congenital tumors of the central nervous system.

Congenital tumors of the central nervous system (CNS) are often arbitrarily divided into "definitely congenital" (present or producing symptoms at birth), "probably congenital" (present or producing symptoms within the first week of life), and "possibly congenital" (present or producing symptoms within the first 6 months of life). They represent less than 2% of all childhood brain tumors. The clinical features of newborns include an enlarged head circumference, associated hydrocephalus, and asymmetric skull growth. At birth, a large head or a tense fontanel is the presenting sign in up to 85% of patients. Neurological symptoms as initial symptoms are comparatively rare. The prenatal diagnosis of congenital CNS tumors, while based on ultrasonography, has significantly benefited from the introduction of prenatal magnetic resonance imaging studies. Teratomas constitute about one third to one half of these tumors and are the most common neonatal brain tumor. They are often immature because of primitive neural elements and, rarely, a component of mixed malignant germ cell tumors. Other tumors include astrocytomas, choroid plexus papilloma, primitive neuroectodermal tumors, atypical teratoid/rhabdoid tumors, and medulloblastomas. Less common histologies include craniopharyngiomas and ependymomas. There is a strong predilection for supratentorial locations, different from tumors of infants and children. Differential diagnoses include spontaneous intracranial hemorrhage that can occur in the presence of coagulation factor deficiency or underlying vascular malformations, and congenital brain malformations, especially giant heterotopia. The prognosis for patients with congenital tumors is generally poor, usually because of the massive size of the tumor. However, tumors can be resected successfully if they are small and favorably located. The most favorable outcomes are achieved with choroid plexus tumors, where aggressive surgical treatment leads to disease-free survival.

Diagnosis and treatment of vein of Galen aneurysmal malformations.

INTRODUCTION: Vein of Galen aneurysmal malformations (VGAM) are rare but clinically significant intracranial arteriovenous shunt lesions that most often present in neonates and infants. METHODS: Retrospective clinical data were collected for patients evaluated with a diagnosis of VGAM from 1994 to 2007. RESULTS: Thirteen patients with VGAM were evaluated from 1994 to 2007. Seven patients presented emergently with medically intractable cardiac failure, and six were treated in the first 2 weeks of life. Five children treated after this period (1.5-31 months of age) manifested enlarging head circumference, abnormal development, or subarachnoid hemorrhage. Eleven patients were managed endovascularly. Four disease or procedure-related complications occurred. Two complications were associated with poor outcome, both of which occurred in patients treated at less than 2 weeks of age. Two other patients experienced transient neurological deficits with no evidence of permanent sequelae. Outcome in the six patients treated emergently in the first 2 weeks of life included two patients who developed normally, one with mild to moderate neurological deficits, one with severe neurological deficits, and two deaths. Outcome in the five older patients (treated between 1.5 and 31 months) was considerably better than in the group treated early and included three with normal outcome and two with mild neurological deficits. CONCLUSIONS: Contemporary endovascular techniques remain the preferred treatment for VGAM in all age groups. Early diagnosis and multimodality treatment are essential for the best management and treatment of the complex constellation of clinical problems often arising from this disorder.

Intradural inclusion cysts following in utero closure of myelomeningocele: clinical implications and follow-up findings.

OBJECT: The goal in this study was to evaluate the incidence and clinical implications of the development of cutaneously derived intradural inclusion cysts (ICs) following fetal myelomeningocele (fMMC) closure. METHODS: Retrospective databases and responses to a parental questionnaire were reviewed to determine the incidence, clinical presentation, and outcomes of fMMCs in children in whom ICs developed at follow-up. RESULTS: Prior to the National Institutes of Health (NIH)-sponsored Management of Myelomeningocele Study (MOMS), 54 patients underwent fMMC closure at the authors' institution. Sixteen (30%) presented with symptomatic tethered cord syndrome (TCS) at a median age of 27 months (range 4-93 months). Ten (63%) of the 16 (19% of the total) developed TCS in association with an intradural IC. In 9 (90%) of 10 patients, the IC was seen on preoperative MR imaging, and in 1 it was found during surgery. Four additional children (7% of the total) with evidence of an IC on surveillance MR imaging are currently asymptomatic at 94, 84, 60, and 60 months of age, respectively. All but 1 (an L-3 level lesion) IC developed in infants with L-4 and L-5 defects. After cyst removal, 6 children are asymptomatic at a median follow-up of 36 months (range 12-63 months). Following IC removal, 4 children lost normal bladder function and now require clean intermittent catheterization, and 1 lost normal leg function and now requires a walking aid for ambulation. Histologically, 8 lesions were dermoid, 1 was an epidermoid, and 1 was a mixed dermoid-epidermoid IC. Three patients developed another IC and required its removal at 24, 39, and 51 months, respectively. One required another tethered cord release within 57 months after IC removal. CONCLUSIONS: Cutaneously derived intradural ICs can develop following fMMC surgery. Deterioration of bladder function, risk of recurrence, and loss of lower-extremity function appear to be the most important long-term complications of IC in children with fMMCs. The ongoing NIH-sponsored MOMS may help determine whether children with fMMC are at increased risk of IC development compared with children treated with postnatal MMC closure. Parents seeking fMMC closure should be informed about the possibility of IC formation and the potential clinical consequences.

Thickening and enhancement of multiple cranial nerves in conjunction with cystic white matter lesions in early infantile Krabbe disease.

We present serial MR findings in a child ultimately diagnosed with the early infantile form of Krabbe disease. MR showed typical features of Krabbe disease including cerebellar and brainstem hyperintensity, periventricular and deep white matter hyperintensity, and cerebral atrophy. In addition, the combination of both enlargement and enhancement of multiple cranial nerves in conjunction with unusual cystic lesions adjacent to the frontal horns of the lateral ventricles was previously unreported and expands the spectrum of imaging findings in early Krabbe disease.