Vigabatrin-associated Reversible MRI Abnormalities in an Infant with Tuberous Sclerosis.

Vigabatrin therapy is commonly used in infants diagnosed with tuberous sclerosis complex, particularly in the setting of epilepsy. Utilization of vigabatrin can result in bilateral and symmetric abnormal sequence changes within the deep brain matter and brainstem on magnetic resonance imaging. These abnormalities occur predominantly in infancy, are reversible, and can be asymptomatic or result in symptomatic clinical manifestations. We present a case with classic neuroimaging findings. Familiarity with these findings can prevent unnecessary follow up tests or studies and the cost of continuing or discontinuing vigabatrin therapy should be weighed heavily against the potential manifestation of extrapyramidal symptoms.

Flat sticker as a mobile airway foreign body: A case report and review of the literature.

Diagnosis of an airway foreign body in the setting of an unwitnessed aspiration event remains a challenge for physicians in the emergency setting. We describe a case of a 2-year-old male who presented to the emergency department with atypical symptoms resulting from ingestion and aspiration of a large, flat sticker. The airway foreign body remained in place for over 24 hours despite obtaining appropriate airway imaging, and the object was later removed without complication via rigid bronchoscopy in the operating room. Further review of this case and the current literature highlighted multiple lessons. Initial evaluations should combine a rigorous history and physical with strict guidelines on usage of multiple imaging modalities (eg, plain radiographs and CT). Imaging should be obtained with the patient devoid of all obstructive materials in multiple positions (eg, upright, supine, lateral) in order to maximize the physician's ability to positively diagnose airway foreign bodies prior to definitive treatment with rigid bronchoscopy.

Pleuropulmonary blastoma in an adolescent.

Primary pulmonary malignancies are rare in childhood. The most common, pleuropulmonary blastoma (PPB), has an incidence of 25-50 cases per year in the United States (Knight and et al., 2019) [1]. The majority of children are diagnosed with PPB before the age of four years. PPB is divided into subtypes I, Ir (type I-regressed), II, and III, which correlates to the age of diagnosis and patient prognosis [2,3]. Here we report an unusual presentation of PPB in a teen-aged female who presented with a one month history of a non-productive cough.

Fetal Neuroimaging Update.

OBJECTIVES: To review the current imaging techniques available for the evaluation of the fetal brain. FINDINGS: Ultrasound remains the initial screening modality with routine scanning typically performed at 18-20 weeks gestation. When a central nervous system (CNS) abnormality is noted by ultrasound, MRI is increasingly being used to further clarify findings. Fetal MRI has the unique ability to provide high detailed anatomical information of the entire human fetus with high contrast resolution. This technique has grown due to the development of rapid single shot image acquisition sequences, improvement of motion correction strategies and optimizing shimming techniques. CONCLUSIONS: The assessment of fetal CNS anomalies continues to improve. Advanced MRI techniques have allowed for further delineation of CNS anomalies and have become a cornerstone in the assessment of fetal brain well-being. Those interpreting fetal studies need to be familiar with the strengths and limitations of each exam and be sensitive to the impact discussing findings can have regarding perinatal care and delivery planning. Collaboration with neurologists, neurosurgeons, geneticists, counselors, and maternal fetal specialists are key in providing the best care to the families we treat.

ACR Appropriateness Criteria® Headache.

Headache is one of the most common human afflictions. In most cases, headaches are benign and idiopathic, and resolve spontaneously or with minor therapeutic measures. Imaging is not required for many types of headaches. However, patients presenting with headaches in the setting of "red flags" such as head trauma, cancer, immunocompromised state, pregnancy, patients 50 years or older, related to activity or position, or with a corresponding neurological deficit, may benefit from CT, MRI, or noninvasive vascular imaging to identify a treatable cause. This publication addresses the initial imaging strategies for headaches associated with the following features: severe and sudden onset, optic disc edema, "red flags," migraine or tension-type, trigeminal autonomic origin, and chronic headaches with and without new or progressive features. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

Neuroimaging findings in Emanuel Syndrome.

Emanuel syndrome is a rare inherited chromosomal abnormality caused by an unbalanced translocation of chromosomes 11 and 22. Clinically, Emanuel syndrome is characterized by a wide spectrum of congenital anomalies, dysmorphisms, and developmental disability often confused with other similar syndromes. Outside of genetic testing, diagnosis remains challenging and current literature on typical radiologic findings is limited. We present classic neuroimaging findings of Emanuel syndrome consistent with prior literature including microcephaly, microretrognathia, external auditory canal stenosis, and cleft palate; and also introduce the additional maxillofacial anomaly of dysplastic middle ear ossicles, to our knowledge not previously described in the literature. Recognition of findings leading to earlier diagnosis of Emanuel syndrome may improve outcomes and quality of life for patients and their families.

Pathology of callosal damage in ALS: An ex-vivo, 7 T diffusion tensor MRI study.

OBJECTIVES: The goal of this study was to better understand the changes in tissue microstructure that underlie white matter diffusion changes in ALS patients. METHODS: Diffusion tensor imaging was carried out in postmortem brains of 4 ALS patients and two subjects without neurological disease on a 7 T MRI scanner using steady-state free precession sequences. Fractional anisotropy (FA) was measured in the genu, body, and splenium of the corpus callosum in formalin-fixed hemispheres. FA of the body and genu was expressed as ratio to FA of the splenium, a region unaffected in ALS. After imaging, tissue sections of the same segments of the callosum were stained for markers of different tissue components. Coded image fields were rated for pathological changes by blinded raters. RESULTS: The FA body/FA splenium ratio was reduced in ALS patients compared to controls. Patchy areas of myelin pallor and cells immunostained for CD68, a microglial-macrophage marker, were only observed in the body of the callosum of ALS patients. Blinded ratings showed increased CD68 + microglial cells in the body of the corpus callosum in ALS patients, especially those with C9orf72 mutations, and increased reactive astrocytes throughout the callosum. CONCLUSION: Reduced FA of the corpus callosum in ALS results from complex changes in tissue microstructure. Callosal segments with reduced FA had large numbers of microglia-macrophages in addition to loss of myelinated axons and astrogliosis. Microglial inflammation contributed to reduced FA in ALS, and may contribute to a pro-inflammatory state, but further work is needed to determine their role.

Pre- and postoperative imaging of the Rex shunt in children: what radiologists should know.

OBJECTIVE: The objective of this article is to illustrate the imaging features of patients with extrahepatic portal venous obstruction who are evaluated before or after a Rex shunt surgery. CONCLUSION: The Rex shunt is a potentially curative surgical procedure that reestablishes physiologic hepatopetal portal flow. It is typically accomplished by interposing a vascular conduit between the superior mesenteric vein to the still patent intrahepatic portal venous system. This procedure results in resolution of portal hypertension.