Clinical and Imaging Findings in Children with Myelin Oligodendrocyte Glycoprotein Antibody Associated Disease (MOGAD): From Presentation to Relapse.

BACKGROUND AND PURPOSE: Myelin oligodendrocyte glycoprotein-antibody associated disease (MOGAD) is an increasingly recognized cause of demyelinating disease in children. The purpose of this study is to characterize the CNS imaging manifestations of pediatric MOGAD and identify clinical and imaging variables associated with relapse. MATERIALS AND METHODS: We retrospectively identified children with serum antibody-positive MOGAD evaluated at our institution between 1997 and 2020. Clinical and demographic data were collected. MRIs of the brain, orbit, and spine at presentation and relapse were reviewed for location and pattern of abnormality. RESULTS: Among 61 cases (34 girls), mean age at presentation was 7 years (IQR 4-11). At presentation, there was imaging involvement of the brain in 78.6% (44/56), optic pathway in 55.4% (31/56), and spine in 19.6% (11/56). Brain involvement was commonly in the frontal (70.5%, 31/44) and subcortical (75%, 33/44) white matter, with involvement of the thalamus and pons in 47.7% each (21/44). Optic neuritis (ON) was commonly bilateral (80.6%, 25/31) involving intraorbital segments (77.4%, 24/31). Spinal cord lesions were typically cervical (72.7%, 8/11) and multifocal (72.7%, 8/11).The imaging patterns were age-dependent; children ≤9 years more commonly demonstrated ADEM-like imaging pattern at presentation (39.4%, 13/33) and first relapse (8/23, 34.8%), while children >9 years more commonly had ON at presentation (34.8%, 8/23, P = .001) and FLAIR-hyperintense lesions in anti-MOG-associated encephalitis with seizures at first relapse (5/18, 27.8%, P = .008). CONCLUSIONS: We describe the CNS imaging findings in pediatric MOGAD. The imaging pattern is age-dependent at presentation and first relapse. Younger age at presentation is associated with longer time to relapse.

Why Medical Students Pursue Radiology: A Current Longitudinal Survey on Motivations and Controversial Issues in Radiology.

RATIONALE AND OBJECTIVES: Radiology is an increasingly competitive specialty. Various current factors influence medical students' decision to pursue a radiology career, including artificial intelligence (AI), remote reading, and COVID-19. This study seeks to determine the decision-making factors of all alumni from our medical school who matched into a radiology residency, and to gather opinions on emerging radiology topics. MATERIALS AND METHODS: A survey querying decision-making factors and opinions on current radiology topics was distributed to all alumni from our medical school (first graduating class in 2011) who previously matched into a diagnostic or interventional radiology residency program (n = 57). Wilcoxon Rank-Sum and Fisher's Exact tests were used to determine statistical significance. RESULTS: Forty-three of fifty-seven responses were received (75% response rate). The most influential factor that sparked respondents' interest in radiology was a radiology elective (25/43, 58%). Students who will finish radiology training in 2023 or later were more likely to be influenced by a mentor (15/23, 65%) than those who finished radiology training before 2023 (5/20, 25%) (p = 0.04). Respondents reported a 1.6/5 concern about AI negatively impacting their future career in radiology. There was 1.7/5 concern about performing radiology procedures on patients during the COVID-19 pandemic. Respondents predicted that remote reading would have a 3.2/5 positive impact on helping them achieve their preferred lifestyle. Job satisfaction among attending radiologists is rated at 4.3/5. CONCLUSION: Radiology electives had the greatest influence in piquing students' interest in radiology, while mentorship is assuming increasing influence. AI is perceived as a relatively minimal threat to negatively impact radiologists' jobs. Respondents had little concern about performing radiology procedures during the COVID-19 pandemic. Remote reading is viewed as having a moderately positive impact on lifestyle. Responding radiologists enjoy notably high job satisfaction.

Factors Predictive of Treatment Success in CT-Guided Fibrin Occlusion of CSF-Venous Fistulas: A Multicenter Retrospective Cross-Sectional Study.

BACKGROUND AND PURPOSE: CSF-to-venous fistulas contribute to spontaneous intracranial hypotension. CT-guided fibrin occlusion has been described as a minimally invasive treatment strategy; however, its reproducibility across different institutions remains unclear. This multi-institution study evaluated the clinical and radiologic outcomes of CT-guided fibrin occlusion, hypothesizing a correlation among cure rates, fibrin injectate spread, and drainage patterns. MATERIALS AND METHODS: A retrospective evaluation was conducted on CT-guided fibrin glue treatment in patients with CSF-to-venous fistulas from 6 US and UK institutions from 2020 to 2023. Patient information, procedural characteristics, and injectate spread and drainage patterns were examined. Clinical improvement assessed through medical records served as the primary outcome. RESULTS: Of 119 patients at a mean follow-up of 5.0 months, fibrin occlusion resulted in complete clinical improvement in 59.7%, partial improvement in 34.5%, and no improvement in 5.9% of patients. Complications were reported in 4% of cases. Significant associations were observed between clinical improvement and concordant injectate spread with the fistula drainage pattern (P = .0089) and pretreatment symptom duration (P < .001). No associations were found between clinical improvement and cyst puncture, intravascular extension, rebound headache, body mass index, age, or number of treatment attempts. CONCLUSIONS: Fibrin occlusion performed across various institutions shows cure when associated with injectate spread matching the CVF drainage pattern and shorter pretreatment symptom duration, emphasizing the importance of accurate injectate placement and early intervention.

Solid and Vascular Neck Masses in Children.

Neck masses are frequent in the pediatric population and are usually divided into congenital, inflammatory, and neoplastic. Many of these lesions are cystic and are often benign. Solid masses and vascular lesions are relatively less common, and the imaging appearances can be similar. This article reviews the clinical presentation and imaging patterns of pediatric solid and vascular neck masses.

Neuroimaging features of genetic syndromes associated with CNS overgrowth.

Overgrowth syndromes can manifest with enlargement of the brain and other body parts and are associated with malignancy. Much of the current literature focuses on the imaging findings of the somatic overgrowth, while there is relatively little describing the overgrowth of the central nervous system. In this pictorial essay, we discuss common syndromes with central nervous system overgrowth, highlight key imaging features, and review the underlying genetics, including the PI3K-AKT-mTOR pathway as well as other syndromes from various genes.

Headache response after CT-guided fibrin glue occlusion of CSF-venous fistulas.

OBJECTIVE: To assess headache response and patient perception of improvement after computed tomography (CT)-guided fibrin glue occlusion of cerebrospinal fluid-venous fistulas (CVFs) in a large sample size and with a long clinical follow-up. BACKGROUND: CVFs are an increasingly identified type of spinal leak in patients with spontaneous intracranial hypotension (SIH), and CT-guided fibrin glue occlusion has been introduced as a treatment option in a prior small series. METHODS: Retrospective case series review of medical records from a single institution was performed for all patients with CVFs that were treated with CT-guided fibrin glue occlusion between August 2018 and April 2022 in an outpatient or inpatient setting. Pre- and posttreatment Headache Impact Tests (HIT-6) were administered to patients, and a change in scores was evaluated. In some patients, pretreatment HIT-6 tests were not obtained prior to the fibrin glue procedure, and the patient was asked to fill out the pretreatment test based on personal recall of their symptoms prior to treatment. Patients completed a Patient Global Impression of Change (PGIC) scale after treatment. Pre- and posttreatment brain imaging was compared using Bern SIH scores. RESULTS: Thirty-five patients (19 females, 16 males; mean age 60 years) with CVFs treated with CT-guided fibrin glue occlusion met the inclusion criteria. Mean pretreatment and posttreatment HIT-6 scores were 64.7 ± 10.2 and 43.4 ± 9.9 (p < 0.001), respectively. The posttreatment HIT-6 questionnaires were completed on average 10.3 months after treatment, and 20 patients filled out the pretreatment HIT-6 form after their treatment. The mean PGIC score was 6.1 ± 1.3. Mean pretreatment and posttreatment Bern SIH scores were 5.9 ± 2.5 and 1.5 ± 1.5 (p < 0.001), respectively. CONCLUSIONS: We report a large series of patients who underwent CT-guided fibrin glue occlusion of CVFs. We showed that headache scores decreased after treatment, and the majority of patients had high PGIC scores. Posttreatment brain MRIs also showed improved Bern SIH scores.

Spontaneous Intracranial Hypotension in the Critical Patient.

Spontaneous intracranial hypotension typically manifests with orthostatic headaches and is caused by spinal dural tears, ruptured meningeal diverticula, or CSF-venous fistulas. While most patients are diagnosed and treated in the outpatient setting, some patients will occasionally present in the emergent ICU setting due to subdural hematomas, coma, or downward brain herniation. In this review paper, we will discuss the diagnostic and treatment steps that intensivists can undertake to coordinate a team approach to successfully manage these patients. A brief general overview of spontaneous intracranial hypotension will also be discussed.

Cerebrospinal fluid-lymphatic fistula causing spontaneous intracranial hypotension in a child with kaposiform lymphangiomatosis.

Spontaneous intracranial hypotension is an uncommon etiology of secondary headaches in children. We report a unique case of a girl with kaposiform lymphangiomatosis who developed postural headaches and imaging features of spontaneous intracranial hypotension without a spinal extradural collection. The girl underwent dynamic computed tomography myelography which revealed a cerebrospinal fluid (CSF)-lymphatic fistula related to a lymphatic malformation associated with the right T10 nerve. She underwent surgical ligation of the CSF-lymphatic fistula, resulting in resolution of the headaches. Spinal CSF-lymphatic fistulas are rare and have previously been reported in two patients with Gorham-Stout disease. The current report suggests that patients with systemic lymphatic anomalies who develop postural headaches should undergo evaluation for spontaneous intracranial hypotension and a CSF-lymphatic fistula. If discovered, surgical ligation is a potential treatment.

CT-guided Fibrin Glue Occlusion of Cerebrospinal Fluid-Venous Fistulas.

Background Cerebrospinal fluid-venous fistulas (CVFs) are one of the less common etiologic causes of spontaneous intracranial hypotension. CVFs are most commonly treated with open surgical ligation and have reportedly not responded well to percutaneous treatments. Purpose To study treatment outcomes of CT-guided fibrin glue occlusion for CVFs. Materials and Methods Retrospective review of medical records from two institutions was performed for all patients with CVFs who underwent CT-guided percutaneous fibrin glue occlusion from March to October 2020. CVFs were assessed for resolution or persistence at posttreatment decubitus CT myelography (CTM). Pre- and posttreatment brain MRI scans were reviewed for principal signs of spontaneous intracranial hypotension. Clinical symptoms were documented before and immediately after therapy, and the current symptoms to date after fibrin glue occlusion were documented. Results CT-guided fibrin glue occlusion was performed in 13 patients (mean age, 62 years ± 14 [standard deviation]; eight women) with CVFs. Ten of 10 patients who underwent final posttreatment decubitus CTM examinations showed CVF resolution. All 13 patients showed improvement on posttreatment brain MRI scans. All 13 patients are currently asymptomatic, although three patients were asymptomatic before fibrin glue occlusion. Conclusion CT-guided fibrin glue occlusion is an effective treatment for patients with cerebrospinal fluid-venous fistulas (CVFs). Direct fibrin glue administration within the CVF may be one of the key factors for success. Further studies are needed to determine the long-term efficacy of this treatment. © RSNA, 2021.

MRI in verrucous venous malformation: role in diagnosis and treatment.

Verrucous venous malformation (VVM), recently reclassified from verrucous hemangioma, is a rare congenital vascular anomaly that is traditionally diagnosed on histopathologic analysis of deep tissue biopsy. This case report documents the utility of magnetic resonance imaging in confirming VVM diagnosis, characterizing lesion extent and guiding therapy.

Developing a Spinal CSF Leak Program in a Multihospital Network.

OBJECTIVE: Spontaneous spinal cerebrospinal fluid (CSF) leaks are rare and challenging to diagnose and treat. Patients may present to a variety of physicians, and many patients are often referred to a specialized center with a dedicated spinal CSF leak program and expertise in this condition. To our knowledge, there are no reported publications on how to create such a program. CONCLUSION: In this article, we describe the specific steps we took to develop a spinal CSF leak program, which we have implemented over a multihospital network.

Vascular anomaly imaging mimics and differential diagnoses.

Vascular anomalies can be correctly diagnosed in the majority of instances using the combination of clinical history, physical examination and imaging. In certain cases, the clinical work-up may be inconclusive or unavailable to the radiologist, and the imaging findings can be nonspecific, yielding more than one possible diagnosis. In this pictorial essay, we discuss diagnoses that can mimic vascular anomalies and highlight key differentiating imaging features.

Hearing loss in PHACE syndrome: clinical and radiologic findings.

PURPOSE: To characterize the types of hearing loss, auditory-related imaging findings, and hemangioma characteristics in patients with Posterior fossa malformations, Hemangiomas, Arterial anomalies, Cardiac defects, and abnormalities of the Eye (PHACE) syndrome. METHODS: Retrospective medical records, audiologic data, and imaging review of all patients presenting to a tertiary care children's hospital with a proven diagnosis of PHACE syndrome from 2005 to 2016. RESULTS: Twelve patients were identified with hearing and imaging data. 5/12 had hearing loss, 1 had unilateral severe sensorineural loss with minor conductive component, 1 had unilateral moderate sensorineural loss with minor conductive component, 1 had mild bilateral conductive loss, 1 had bilateral hearing loss (left severe mixed and right severe sensorineural), and 1 had moderate bilateral conductive loss. All patients passed their newborn hearing screening. Of the 5 patients with hearing loss, 3 had IAC hemangiomas (1 bilateral), 3 had enlarged IACs with prominent posterior petrous bones (1 bilateral), 2 had dysgenesis of the cerebellar vermis and hemispheres, there was 1 patient each with a deformed pinna and middle ear and mastoid effusions, and 1 patient had no abnormal auditory-related imaging findings. Patients with hearing loss were more likely to have more areas of cutaneous hemangioma involvement (mean 6.4 vs 3.1, p = .05). Laterality of hearing impairment correlated with the side of cutaneous hemangioma in all patients with hearing loss. Treatment with systemic propranolol did not improve hearing. CONCLUSIONS: Patients with PHACE are at risk for hearing loss and may demonstrate radiologic abnormalities within the ear structures, although the type of hearing loss, imaging findings, and their respective correlation vary. While our results are limited by our small sample size, comprehensive audiology evaluations (as opposed to newborn screening testing only) should be considered for PHACE patients who have extensive cutaneous hemangioma or auditory-related imaging abnormalities, such as internal auditory canal hemangiomas.

Developing a Multidisciplinary Vascular Anomalies Clinic and Reviewing the Radiologist's Clinic Role.

Cutaneous vascular anomalies can be challenging to diagnose and treat given their rare presentation and that they do not always fall under the purview of a specific medical specialty. Patients with cutaneous vascular anomalies are best managed in vascular anomalies clinics, but to our knowledge, no reported publications exist on how to develop such a clinic. In this article, we describe the specifics steps our institution took to create a vascular anomalies clinic and discuss how it benefitted our patients and referring providers. We also highlight the roles of the diagnostic and interventional radiologist within a vascular anomalies clinic.

Case 245: Erdheim-Chester Disease.

History A 53-year-old man experienced headache and double vision that progressed over 1 year. After a traumatic fall, he was hospitalized, and proptosis was identified at physical examination. Laboratory tests were remarkable for leukocytosis. Hematocrit level, thyroid stimulating hormone level, autoimmune antibody level, erythrocyte sedimentation rate, and C-reactive protein level were normal. Computed tomography (CT) of the head revealed bilateral intraconal masses, for which magnetic resonance (MR) imaging of the orbits was subsequently performed ( Fig 1 ). CT imaging of the chest and abdomen ( Fig 2 ) revealed periaortic and retroperitoneal stranding. Perinephric biopsy was performed, and a diagnosis of immunoglobulin G4 (IgG4)-related disease was made based on identification of a few plasma cells per high-power field that were positive for IgG4. Orbital biopsy was then performed, but the results were inconclusive for IgG4-related disease. The patient was discharged and given steroid therapy for presumed IgG4-related disease. [Figure: see text][Figure: see text][Figure: see text][Figure: see text][Figure: see text][Figure: see text] Several months later, the patient returned to our institution with progressive symptoms despite ongoing steroid treatment. His case was reviewed by several specialists to develop alternative treatments for IgG4-related disease. After review of the available images, a neuroradiology fellow (M.D.M.) performed history taking and a physical examination and subsequently recommended radiography of the lower extremities ( Fig 3 ). [Figure: see text][Figure: see text].

Tips and tricks to optimize MRI protocols for cutaneous vascular anomalies.

Magnetic resonance imaging (MRI) serves as the principal imaging modality to diagnose and plan treatment for children with cutaneous vascular anomalies. While routine MRI protocols can lead to the correct diagnosis in the majority of cases, the imaging appearances can sometimes be nonspecific or confusing, yielding more than one possible diagnosis. This review highlights specific MRI sequence recommendations and scanning tips that can optimize the imaging protocol to increase diagnostic confidence.