Presentation
Long history of neurological impairment since birth.
Patient Data
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MRI of the brain demonstrates a small posterior fossa with low lying tentorial attachment posteriorly. The tectum is beaked and hydrocephalus is present.
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The low lying tonsils in the foramen magnum are noted and the cervical spine shows a local syrinx between C6 - T2. Additionally, the C3 and C4 vertebral bodies are congenitally fused.
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The lumbar spine shows the tethered cord down to the meningomyelocele at the level of S1 - S3. No abnormality is seen to the overlying subcutaneous fat.
Case Discussion
This case illustrates many of the common features of Chiari II malformations, particularly the presence of a lumbosacral myelomeningocoele, small posterior fossa with tectal beaking and hydrocephalus. In this case the corpus callosum is intact.