Clinical and radiological evaluation of caudal regression syndrome.

Caudal regression syndrome is a form of segmental spinal dysgenesis involving the caudal spinal column, ranging from segmental coccygeal agenesis to extensive thoracolumbar agenesis with varying degrees of spinal cord dysgenesis. A majority of caudal regression cases are sporadic but maternal pre-gestational diabetes mellitus is an important risk factor. Imaging is an integral part of management of caudal regression syndrome. Antenatal diagnosis on obstetric ultrasound and evaluation with fetal MRI is ideal. Early postnatal diagnosis and/or detailed evaluation with MRI is essential for early management to improve outcomes. Pang classification categorizes caudal regression syndrome into two categories based on the position of the conus while Renshaw classification is based on the degree of vertebral column agenesis. Caudal regression syndrome may be associated with several additional anomalies, both spinal and extraspinal. A number of genitourinary and gastrointestinal anomalies have been described in association with caudal regression syndrome. The field of view of MRI of the lumbosacral spine in caudal regression syndrome needs to be extended to visualize the retroperitoneal structures without the use of a saturation band. Syndromic associations may be suspected, and additional imaging performed, based on findings of extended field of view MRI of the spine. Associated sacral masses and filar abnormalities need to be identified and may also require surgical treatment. The multisystem nature of this disease necessitates a multimodality approach to the evaluation and management of caudal regression syndrome with close cooperation between pediatric neuroradiologists and body radiologists as well as multiple clinical teams. Appropriate early management with surgical correction as necessary can significantly improve prognosis and survival in caudal regression syndrome.

Neonatal osteoblastic tumor with a novel PTBP1::FOSB fusion.

Congenital/neonatal bone neoplasms are extremely rare. We present the case of a patient with a neonatal bone tumor of the fibula that had osteoblastic differentiation and a novel PTBP1::FOSB fusion. FOSB fusions are described in several different tumor types, including osteoid osteoma and osteoblastoma; however, these tumors typically present in the second or third decade of life, with case reports as young as 4 months of age. Our case expands the spectrum of congenital/neonatal bone lesions. The initial radiologic, histologic, and molecular findings supported the decision for close clinical follow-up rather than more aggressive intervention. Since the time of diagnosis, this tumor has undergone radiologic regression without treatment.

Imaging of pediatric calvarial and skull base tumors: A COG Diagnostic Imaging Committee/SPR Oncology Committee/ASPNR White Paper.

A standardized imaging protocol for pediatric oncology patients is essential for accurate and efficient imaging, while simultaneously promoting collaborative understanding of pathologies and radiologic assessment of treatment response. The objective of this article is to provide standardized pediatric imaging guidelines and parameters for evaluation of tumors of the pediatric orbit, calvarium, skull base, and temporal bone. This article was drafted based on current scientific literature as well as consensus opinions of imaging experts in collaboration with the Children's Oncology Group Diagnostic Imaging Committee, Society of Pediatric Radiology Oncology Committee, and American Society of Pediatric Neuroradiology.

Utility of balanced steady-state field precession sequence in the evaluation of retinal and subdural hemorrhages in patients with abusive head trauma.

Abusive head trauma is the leading cause of physical child abuse deaths in children under 5 years of age in the United States. To evaluate suspected child abuse, radiologic studies are typically the first to identify hallmark findings of abusive head trauma including intracranial hemorrhage, cerebral edema, and ischemic injury. Prompt evaluation and diagnosis are necessary as findings may change rapidly. Current imaging recommendations include brain magnetic resonance imaging with the addition of a susceptibility weighted imaging (SWI) sequence which can detect additional findings that suggest abusive head trauma including cortical venous injury and retinal hemorrhages. However, SWI is limited due to blooming artifacts and artifacts from the adjacent skull vault or retroorbital fat, which can affect the evaluation of retinal, subdural, and subarachnoid hemorrhages. This work explores the utility of the high-resolution, heavily T2 weighted balanced steady-state field precession (bSSFP) sequence to identify and characterize retinal hemorrhage and cerebral cortical venous injury in children with abusive head trauma. The bSSFP sequence provides distinct anatomical images to improve the identification of retinal hemorrhage and cortical venous injury.

Utility of three-dimensional and reformatted head computed tomography images in the evaluation of pediatric abusive head trauma.

Skull fractures are common in the pediatric population following head trauma and are estimated to occur post head trauma in 11% of children younger than 2 years. A skull fracture indicates potential underlying intracranial injury and might also help explain the mechanism of injury. Multiple primary and accessory sutures complicate the identification of non-depressed fractures in children younger than 2 years. Detection of linear skull fractures can be difficult on two-dimensional (2-D) CT and can be missed, particularly when the fracture is along the plane of image reconstruction. Knowledge of primary and accessory sutures as well as normal anatomical variants is of paramount importance in identifying pediatric skull fractures with a greater degree of confidence. Acute fractures appear as lucent cortical defects that do not have sclerotic borders, in contrast to sutures, which might demonstrate sclerotic margins. Three-dimensional (3-D) CT has increased sensitivity and specificity for detecting skull fractures and is essential in the evaluation of pediatric head CTs for distinguishing subtle fractures from sutural variants, especially in the setting of trauma. In this review, we present our experience of the use of 3-D reformats in head CT and its implications on the interpretation, especially in the setting of accidental or abusive head trauma.

Spinal ligamentous injury in abusive head trauma: a pictorial review.

There is growing evidence of spine injury in abusive head trauma (AHT). Historically, spine injury was considered rare in AHT because of a lack of attributable clinical symptoms or signs and a lack of advanced imaging. Increased use of MRI in AHT has been instrumental in helping identify evidence of ligamentous injuries of the spine. These findings can be difficult to identify on autopsy because of the size and location of the ligaments. Because spinal injury in AHT mostly involves ligamentous and soft tissues and only rarely involves bony fractures, more than 90% of the injury findings are missed on CT or radiography of the spine. Investigation of these findings and the injury patterns should lead to a better understanding of the mechanism of spinal injury. In this pictorial review, we describe the various manifestations of spinal ligamentous injury in AHT, as seen on MRI, in children younger than 48 months.

Spinal subdural hemorrhage in abusive head trauma: a pictorial review.

A growing body of evidence links abusive head trauma (AHT) to patterns of direct and indirect spinal injuries, such as spinal subdural hemorrhage (SDH). Identification of evidence of spinal injury such as spinal SDH plays a crucial role in the diagnosis and subsequent management of the index child with AHT and his or her siblings. In a value-based practice of medicine, it can be argued that adding spine imaging to identify spinal SDH in the workup of AHT adds value to both the short- and long-term management of the patient. This pictorial review describes the normal appearance of spinal SDH and challenges of identifying spinal SDH, and it explores the mechanism of spinal SDH development in AHT.

Venous injury in pediatric abusive head trauma: a pictorial review.

Abusive head trauma (AHT) is the leading cause of fatal head injuries in children younger than 2 years. An intracranial pathology can exist even in the setting of a normal physical exam. A delay in the diagnosis of AHT can have serious life-threatening consequences for the child and increases the potential the child will be abused again. In this article, we review the traumatic subdural hematoma as well as various morpho-structural patterns of shearing injuries and thrombosis of intracranial bridging veins. This work serves as a summary of patterns of imaging features of intracranial venous injury in AHT, as described in the literature, to facilitate familiarity and early detection of abusive head trauma in the pediatric population. Essentially, in AHT there is a traumatic injury to the bridging vein with either partial or complete tear. This can secondarily result in thrombosis at the terminal end of the bridging vein with blood clots adjacent to the bridging vein.

Ultra-Low-Dose Computed Tomography Protocol for Preoperative Evaluation in Children With Craniofacial Anomalies.

PURPOSE: Preoperative three-dimensional computed tomography is currently the gold standard imaging modality in patients with craniofacial anomalies. In these patients, bone structural evaluation is paramount for surgical planning and evaluation of brain parenchyma is often secondary. With the significant complexity of these patients, a majority of patients undergo multiple Computed Tomography (CT) studies from infancy into adulthood. This study presents an ultra-low-dose CT protocol that limits the radiation exposure per CT scan in patients with craniofacial anomalies. MATERIAL AND METHODS: A total of 200 consecutive patients who underwent head CT for preoperative evaluation of craniofacial anomalies at Tertiary Children's Hospital were included in the study. The kVp, (KiloVoltage Peak) mA (milliAnperage), CT dose index (CTDI), and dose-length product (DLP) were documented from the dose page. Patients were stratified based on age for determining age specific effective dose and for age matched comparison. The age specific effective dose was derived by using the established conversion factor as described in the paper. (1) Standard t test was performed to determine the statistical significance of radiation dose reduction. The Institutional Review Board approved the study and data was collected from 2012-2014. FINDINGS: Of the 200 patients assessed in our study, 90 patients had low-dose CT scans and 110 patients had ultra-low-dose CT scans of the head. All patients had diagnostic quality CT studies. The low-dose CT was performed at 120 kVp and 100 mA. The ultra-low-dose CT was performed at 80 kVp and fixed 80 mA. The minimum, maximum and mean effective dose before the introduction of the ultra-low-dose protocol was 0.8 mSv, 6.9 mSV and 2.82 mSv. The minimum, maximum and mean effective dose after the introduction of the ultra-low-dose protocol was 0.6 mSv, 3.8 mSV, and 1.37 mSv. The reduction in the effective radiation dose was statistically significant (standard t test; P = 0.0001). CONCLUSION: Compared to the regular low-dose protocol, the ultra-low-dose CT protocol provided appropriate diagnostic images with a significantly decreased radiation dose.

Diffusion Tensor MRI of White Matter of Healthy Full-term Newborns: Relationship to Neurodevelopmental Outcomes.

Background It is well known that white matter injuries observed at birth are associated with adverse neurodevelopmental outcomes later in life. Whether white matter developmental variations in healthy newborns are also associated with changes in later neurodevelopment remains to be established. Purpose To evaluate whether developmental variations of white matter microstructures identified by MRI correlate with neurodevelopmental outcomes in healthy full-term infants. Materials and Methods In this prospective study, pregnant women were recruited and their healthy full-term newborns underwent a brain MRI including diffusion tensor imaging at approximately 2 weeks of age. These infants were tested at approximately 2 years of age with the Bayley Scales of Infant Development (BSID). Voxel-wise correlation analyses of fractional anisotropy (FA), measured with diffusion tensor MRI, and neurodevelopmental test scores, measured by using BSID, were performed by using tract-based spatial statistics (TBSS), followed by region-of-interest (ROI) analyses of correlations between mean FA in selected white matter ROIs and each BSID subscale score. Results Thirty-eight full-term infants (20 boys, 18 girls) underwent MRI examination at 2 weeks of age (14.3 days ± 1.6) and BSID measurement at 2 years of age (732 days ± 6). TBSS analyses showed widespread clusters in major white matter tracts, with positive correlations (P ≤ .05, corrected for the voxel-wise multiple comparisons) between FA values and multiple BSID subscale scores. These correlations were largely independent of several demographic parameters as well as family environment. Gestational age at birth appeared to be a confounding factor as TBSS-observed correlations weakened when it was included as a covariate; however, after controlling for gestational age at birth, ROI analyses still showed positive correlations (P ≤ .05, R = 0.35 to 0.48) between mean FA in many white matter ROIs and BSID cognitive, language, and motor scores. Conclusion There were significant associations between white matter microstructure developmental variations in healthy full-term newborns and their neurodevelopmental outcomes. © RSNA, 2019 Online supplemental material is available for this article. See also the editorial by Hu and McAllister in this issue.

Idiopathic Intracranial Hypertension Progressing to Venous Sinus Thrombosis, Subarachnoid Hemorrhage, and Stroke.

Idiopathic intracranial hypertension (IIH) is a syndrome characterized by increased intracranial pressure (ICP), the absence of structural lesions on neuroimaging, and normal cerebrospinal fluid composition. Cerebral venous sinus thrombosis (CVST) is a common cause of increased ICP and can be differentiated from IIH with magnetic resonance venography. We describe a young woman with typical IIH who underwent lumbar puncture and was treated with a short course of high-dose corticosteroids followed by acetazolamide. She subsequently developed CVST, subarachnoid hemorrhage, and stroke. Risk factors that may have resulted in CVST are discussed.

Subarachnoid haemorrhage from undiagnosed mycotic aortic aneurysm in a child.

We report a case of subarachnoid haemorrhage resulting from a mycotic aortic aneurysm in a child with CHD. The patient previously underwent operations for CHD and had a subarachnoid haemorrhage of unknown cause before the scheduled re-operation. During the re-operation, a sealed rupture of an undiagnosed mycotic ascending aortic aneurysm was identified, and the causative organism was later identified as Streptococcus. A postoperative MRI indicated a partially thrombosed cerebral aneurysm. This case demonstrates that a mycotic aortic aneurysm can be a cause of intracranial haemorrhage in children.

Erythropoietin and Brain Magnetic Resonance Imaging Findings in Hypoxic-Ischemic Encephalopathy: Volume of Acute Brain Injury and 1-Year Neurodevelopmental Outcome.

In the Neonatal Erythropoietin and Therapeutic Hypothermia Outcomes study, 9/20 erythropoietin-treated vs 12/24 placebo-treated infants with hypoxic-ischemic encephalopathy had acute brain injury. Among infants with acute brain injury, the injury volume was lower in the erythropoietin than the placebo group (P = .004). Higher injury volume correlated with lower 12-month neurodevelopmental scores. TRIAL REGISTRATION: ClinicalTrials.gov: NCT01913340.

Predictors of poor outcome in patients with posterior reversible encephalopathy syndrome.

PURPOSE: Posterior reversible encephalopathy syndrome (PRES) is an acute neurotoxic syndrome that, although characteristically reversible, can be fatal or result in long-term disability in a subset of patients. Our aim was to identify factors associated with poor discharge outcome in PRES patients. MATERIALS AND METHODS: We retrospectively reviewed the clinical and radiological records of all patients with PRES admitted at our tertiary care medical center from 2007 to 2014. They were divided based their modified Rankin Score at discharge and compared for their baseline variables, clinical, laboratory and imaging features. Poor outcome was defined by a modified Rankin scale 2-6 and was subdivided based on the primary mechanism that led to poor outcome. RESULTS: Out of 100 PRES subjects, 36% had poor discharge outcomes. Factors associated with poor outcomes on univariate analysis were history of diabetes mellitus, coma, high Charlson comorbidity index, post-transplantation, autoimmune condition, lack of systolic or diastolic hypertension, elevated blood urea nitrogen and involvement of the corpus callosum. On multivariate analysis, only prior diabetes mellitus odd ratio (OR) = 6.8 (95% CI 1.1-42.1, p = 0.04), corpus callosum involvement (OR = 11.7; 95% CI 2.4-57.4, p = 0.00) were associated with poor outcome. Poor outcome also correlated with increased length of hospital stay (OR = 7.9; 95% CI 1.3-49.7, p = 0.03). CONCLUSION: Large prospective studies incorporating serial blood glucose values and advanced imaging studies are required to validate these findings.

Compression of the posterior fossa venous sinuses by epidural hemorrhage simulating venous sinus thrombosis: CT and MR findings.

BACKGROUND: Posterior fossa dural venous sinus thrombus is a well-described complication of head trauma, especially when fracture crosses the dural sinus grooves or in association with epidural hemorrhage. We have found that post-traumatic posterior fossa epidural hematoma compressing a dural venous sinus can mimic dural venous thrombus. OBJECTIVE: To discuss the CT and MRI findings of posterior fossa epidural hemorrhages simulating sinus thrombosis, to make radiologists aware of this important imaging pitfall. MATERIALS AND METHODS: We describe radiologic findings in four children in whom a posterior fossa epidural hemorrhage mimicked dural venous sinus thrombus. Routine CT head and CT venography were obtained on Toshiba volume and helical CT scanners. MRI and MR venography were performed on a Philips scanner. RESULTS: In all cases there was medial displacement and compression of the posterior fossa dural venous sinuses without intraluminal thrombosis. The epidural hemorrhage was seen tracking along sinus grooves in the occipital bone, peeling the dura containing the sinuses from the calvarium and compressing the sinus, simulating thrombosis on axial CT views. CONCLUSION: Both venous sinus thrombosis and posterior fossa epidural hemorrhages in children are well-described complications of head trauma. Posterior fossa epidural hemorrhage can mimic a sinus thrombus by compressing and displacing the sinuses. It is important to recognize this pitfall because treatment of a suspected thrombus with anticoagulation can worsen epidural hemorrhage.

Neuromyelitis Optica Spectrum Disorder in a Pediatric Patient.

Neuromyelitis optica in the pediatric population is rare. Patients present with acute neurological deficits predominantly involving the optic nerves and spinal cord, usually with a relapsing course. Clinical overlap between multiple sclerosis and other autoimmune disorders can occur and must be ruled out as NMO requires a more aggressive treatment plan. We present a pediatric patent with NMO spectrum disorder in order to draw awareness to this potentially neurologi- cally devastating disease.