Hydrocephalus As the Initial Presentation of a Spinal Cord Astrocytoma Associated with Leptomeningeal Spread

A 3-year-old boy presented with headaches, vomiting, lethargy and papilledema. Communicating hydrocephalus along with transependymal fluid absorption and meningeal contrast enhancement was identified on CT. The enhancement was initially thought to be the result of a partially treated meningitis (child was previously on oral antibiotics for a presumed mycoplasma pneumonia). A right ventricular-peritoneal shunt was placed. CSF studies procured during the procedure were all normal. In contrast, CSF from a lumbar puncture contained a high protein, and cytology was highly suspicious for malignancy. Spine MRI showed diffuse leptomeningeal enhancement and a 1.5-cm intramedullary lesion at T12-L1 associated with minimal edema. The lesion was subtotally resected (70%) and diagnosed as an astrocytoma (mostly Kernohan grade 2 but with areas of grade 3). Chemotherapy was administered and follow-up spine MRI at 2 months did not reveal any residual tumor, however, the leptomeningeal enhancement persisted. Sixteen months later, at the completion of the chemotherapy and radiation therapy, the spine MRI remained unchanged. Neurological examination has always been normal. This case illustrates how a spinal cord astrocytoma can metastasize via spinocranial dispersion and present early with hydrocephalus rather than myelopathy.