- Hindbrain Herniation in Chiari II Malformation on Fetal and Postnatal MRI
The authors examined the neuroimaging findings with a focus on hindbrain herniation and ventricular size in fetuses with open spinal dysraphism and compared them with postnatal imaging features in groups undergoing prenatal-versus-postnatal repair. Thirty-two of 102 (31.3%) fetuses underwent in utero repair of open spinal dysraphism; 68.6% (70/102) underwent postnatal repair. Of those who underwent prenatal repair 81.3% (26/32) had resolved cerebellar ectopia postnatally. Of those who had severe cerebellar ectopia (grade 3) that underwent postnatal repair, 65.5% (36/55) remained grade 3, while 34.5% (19/55) improved to grade 2. They conclude that most fetuses who undergo in utero repair have resolved cerebellar ectopia postnatally.
- Imaging Characteristics of Pediatric Diffuse Midline Gliomas with Histone H3 K27M Mutation
The 2016 WHO Classification of Tumors of the Central Nervous System includes “diffuse midline glioma with histone H3 K27M mutation” as a new diagnostic entity. This study of 33 patients with diffuse midline gliomas found histone H3 K27M mutation was present in 24 patients (72.7%) and absent in 9 (27.3%). The location was the thalamus in 27.3%; the pons in 42.4%; within the vermis/fourth ventricle in 15%; and the spinal cord in 6%. The radiographic features of diffuse midline gliomas with histone H3 K27M mutation were highly variable, ranging from expansile masses without enhancement or necrosis with large areas of surrounding infiltrative growth to peripherally enhancing masses with central necrosis with significant mass effect.
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