CincyKidsRad

Cincinnati Children's Radiology

Coronal (image 1) and axial (image 2) T2-W images show a mass (arrow) arising from the optic chiasm. Optic pathway gliomas are more common in children and are associated with neurofibromatosis type 1. The tumor can affect any portion of the optic pathway. Approximately a third of optic pathway gliomas primarily involve the optic chiasm.

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This patient has Neurofibromatosis type 1 (NF 1) with extensive lumbar and sacral neurofibromas extending through the neural foramina and into the adjacent paraspinal soft tissues. Optic pathway gliomas are an associated orbital feature, as are plexiform neurofibromas involving the orbit, Lisch nodules (iris hamartomas), buphthalmos or large globes, and tortuosity and ectasia of the optic nerve sheaths. Skin findings in NF 1 may include café au lait spots (hyperpigmented lesions) and inguinal and axillary freckling.

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