Evaluation and Management of Acute-Onset Hemiparesis in an Adolescent With Leukemia.

Emergency departments (EDs) are alert to the possibility of stroke and the need for early interventions to improve long-term clinical outcomes. However, new-onset hemiparesis in pediatric patients with leukemia may be due to a number of different etiologies, including most common side effects from chemotherapeutic agents. We present a case of a 15-year-old boy with pre-B acute lymphoblastic leukemia on chemotherapy, having recently received a high-dose methotrexate infusion in addition to intrathecal methotrexate therapy, who presented to our ED with acute right-sided hemiparesis. He was initially suspected as having a possible ischemic stroke. Magnetic resonance imaging (diffusion-weighted and fluid-attenuated inversion recovery sequence) demonstrated focal areas of diffusion restriction, an early sign of delayed-onset methotrexate neurotoxicity. Our patient received appropriate supportive care and leucovorin rescue with gradual clinical recovery, after a prolonged hospitalization and acute care rehabilitation over the course of several months. Our case illustrates the need for ED providers to consider methotrexate neurotoxicity in pediatric oncology patients presenting with acute neurologic changes.

Clinical and Radiological Findings in Children with Anomalous Pontine Cranial Nerves.

We retrospectively reviewed the clinical and radiologic findings in 17 children with an aberrant cisternal CN7 and found that these patients had additional anomalies involving other pontine cranial nerves. The hallmark imaging feature identified in all patients was aberrant cisternal segment of an enlarged appearing CN7. The abnormal nerve coursed anteriorly towards the Gasserian ganglion where it fanned out towards the internal auditory canal, Meckel's cave or both. This finding was accompanied by a small cisternal CN5 which often had a lateral bowed appearance. CN5 and CN7 were abnormally close to each other. Meckel's cave appeared widened posteriorly and often was close to or merged with the internal auditory canal. Other abnormalities in the pontine cranial nerves included CN8 deficiency in the majority of children and variable CN6 deficiency. This constellation of findings was most often discovered in children having MR evaluation for sensorineural hearing loss and the majority of patients had preserved facial nerve function. In patients with available genetic testing, no pathogenic variants were observed. Interestingly, in 13 children with available birth history, 9 were notable for maternal or gestational diabetes (69%), suggesting a possible early intrauterine insult to the developing nerves.ABBREVIATIONS: CN= cranial nerve; OAVS= Oculo-Auriculo-Vertebral Spectrum; IAC= Internal Auditory Canal; PTCD= Pontine Tegmental Cap Dysplasia; EMR= Electronic Medical Record; SNHL= sensorineural hearing loss.

Pediatric spinal cord diffuse midline glioma, H3 K27-altered with intracranial and spinal leptomeningeal spread: A case report.

Primary spinal cord high-grade gliomas, including those histologically identified as glioblastoma (GBM), are a rare entity in the pediatric population but should be considered in the differential diagnosis of intramedullary lesions. Pediatric spinal cord high-grade gliomas have an aggressive course with poor prognosis. The aim of this case report is to present a 15-year-old female adolescent with histopathologically confirmed spinal cord GBM with H3F3A K27 M mutation consistent with a diffuse midline glioma (DMG), H3 K27-altered, CNS WHO grade 4 with leptomeningeal seeding on initial presentation. As imaging features of H3 K27-altered DMGs are non-specific and may mimic more frequently encountered neoplastic diseases as well as demyelinating disorders, severe neurological deficits at presentation with short duration, rapid progression, and early leptomeningeal seeding should however raise the suspicion for a pediatric-type diffuse high-grade glioma like DMG, H3 K27-altered.

Neuroimaging Characteristics of Nasopharyngeal Carcinoma in Children.

BACKGROUND AND PURPOSE: Pediatric nasopharyngeal carcinoma (NPC) is a rare epithelial origin tumor associated with undifferentiated histology, Epstein-Barr virus (EBV) infection, and genetic risk factors. Childhood NPC is usually clinically silent, often presenting with advanced locoregional compromise, including skull base invasion and cervical lymphadenopathy, and has a better prognosis than adult NPC. This article describes computed tomography (CT) and magnetic resonance imaging (MRI) features in a cohort of 28 pediatric NPC patients. METHODS: A retrospective review was performed among children with histopathology proven NPC diagnoses between 1996 and 2019 for this study. The electronic medical records were reviewed to determine demographics, EBV serology, and World Health Organization (WHO) type. Nasopharyngeal CT and/or MRI at presentation for tumor spread as well as density and/or intensity, lymphadenopathy, postcontrast enhancement and diffusion characteristics before treatment were evaluated. RESULTS: Twenty-eight patients (21 males, 7 females) were included. The mean patient age at diagnosis was 13.3 (range 7 to 17) years. EBV was positive in 71.4% of patients. The majority of patients (78.6%) had a WHO type III tumor, unilateral fossa of Rosenmuller involvement (71.4%). Neuroimaging features were CT isodensity, T1-isointensity, T2-hyperintensity, and heterogeneous postcontrast enhancement for all patients (100%) and restricted diffusion (90%). CONCLUSIONS: Although uncommon in pediatric patients, NPC should be in the differential diagnosis of adolescents presenting with a nasopharyngeal mass. Recognizing key imaging characteristics is helpful in the diagnosis of NPC.

Imaging of Paranasal Sinus Infections in Children: A Review.

Paranasal sinuses (PNS) infections are common in children. They may cause common and well-known complications, but also, unusual and potentially devastating complications. Diagnosing PNS infections and complications in children requires knowledge of the unique anatomy of the nasal cavity and the PNS. In fetal life, nasal mucosa evaginations into the lateral nasal walls initiate the development of the PNS. The PNS continue to develop after birth and complete their maturation and pneumatization at different ages during childhood which makes the pattern of PNS infections determined by patient age. Complications are caused by direct spread of the infection to the orbit, face, intracranial or osseous structures or hematogenous spread of the infection to the intracranial structures. Emergent imaging studies are often necessary in the evaluation of the complications in pediatric patients when the symptoms persist for 10 days and/or if there is evidence of intracranial or orbital complications. In addition, immunocompromised children are especially vulnerable to developing unusual complications. Computed tomography (CT) is excellent for determining whether there is intraorbital extension of PNS disease. However, when the infection approaches the orbital apex, a magnetic resonance imaging (MRI) study with contrast is necessary to assess spread into the cavernous sinus and the intracranial compartment. The goal of this manuscript is to review and characterize imaging findings of PNS infections using CT and MRI allowing determination of the extent of PNS infections and their common and unusual complications in children. In addition, a summary of the development of the normal PNS is provided.

Imaging of rhinosinusitis and its complications: plain film, CT, and MRI.

Conventional plain-film radiography may be used as a screening method for various pathological conditions of the sinonasal cavities. However, CT scanning remains the study of choice for the imaging evaluation of acute and chronic inflammatory diseases of sinonasal cavities. MRI is superior to CT in differentiating inflammatory conditions from neoplastic processes. The most common complications of rhinosinusitis in children occur in the orbit. The information obtained from the CT scan and MRI, together with clinical findings, may be the best guidelines for clinical management and the mode of treatment. Although intracranial complications of sinusitis are relatively rare, prompt recognition of these disease states is important to prevent permanent neurological deficit or fatality. It is prudent to obtain MRI of the sinuses, orbits, and brain whenever extensive or multiple complications of sinusitis are suspected, in addition to CT scanning. Chronic rhinosinusitis is a clinical diagnosis, confirmed and staged with the CT scan of sinonasal cavities. Chronic inflammatory disease is often associated with mucosal thickening and sclerosis of the bone, particularly within the sinuses. Chronic extramucosal fungal sinusitis develops as a saprophytic growth in retained secretions in a sinus cavity. The imaging manifestations of chronic mycotic rhinosinusitis may be nonspecific or highly suggestive of the presence of fungal infection. The presence of diffuse increased attenuation within the paranasal sinuses and nasal cavity should be considered as chronic allergic hypersensitivity aspergillosis (chronic noninvasive aspergillosis) or chronic hyperplastic sinusitis and polyposis associated with desiccated, retained mucosal secretions. The MRI characteristics of fungal sinusitis depend on the stage of the disease.

The efficacy of routine use of recombinant human bone morphogenetic protein-2 in occipitocervical and atlantoaxial fusions of the pediatric spine: a minimum of 12 months' follow-up with computed tomography.

OBJECT The purpose of this study focusing on fusion rate was to determine the efficacy of recombinant human bone morphogenetic protein-2 (rhBMP-2) use in posterior instrumented fusions of the craniocervical junction in the pediatric population. The authors previously reported the short-term (mean follow-up 11 months) safety and efficacy of rhBMP-2 use in the pediatric age group. The present study reports on their long-term results (minimum of 12 months' follow-up) and focuses on efficacy. METHODS The authors performed a retrospective review of 83 consecutive pediatric patients who had undergone posterior occipitocervical or atlantoaxial spine fusion at Texas Children's Hospital or Riley Children's Hospital during the period from October 2007 to October 2012. Forty-nine patients were excluded from further analysis because of death, loss to follow-up, or lack of CT evaluation of fusion at 12 or more months after surgery. Fusion was determined by postoperative CT scan at a minimum of 12 months after surgery. The fusion was graded and classified by a board-certified fellowship-trained pediatric neuroradiologist. Other factors, such as patient age, diagnosis, number of vertebral levels fused, use of allograft or autograft, dosage of bone morphogenetic protein (BMP), and use of postoperative orthosis, were recorded. RESULTS Thirty-four patients had a CT scan at least 12 months after surgery. The average age of the patients at surgery was 8 years, 1 month (range 10 months-17 years). The mean follow-up was 27.7 months (range 12-81 months). There were 37 fusion procedures in 34 patients. Solid fusion (CT Grade 4 or 4-) was achieved in 89.2% of attempts (33 of 37), while incomplete fusion or failure of fusion was seen in 10.8%. Based on logistic regression analysis, there was no significant association between solid fusion and age, sex, BMP dose, type of graft material, use of postoperative orthosis, or number of levels fused. Three of 34 patients (8.8%) required revision surgery. CONCLUSIONS Despite the large number of adult studies reporting positive effects of BMP on bone fusion, our long-term outcomes using rhBMP-2 in the pediatric population suggest that rates of fusion failure are higher than observed in contemporary adult and pediatric reports of occipitocervical and atlantoaxial spine fusions.