CASE PRESENTATION
A three years old boy, previously healthy, presented to the emergency department with severe abdominal pain one month before referral to the pediatric neurology clinic for evaluation of his neck stiffness. The abdominal pain was as periumbilical, occurring in episodes with each episode lasting from few minutes to several hours. It was worse while the child was lying on his back, and It occasionally awakened him from sleep. As the patient was also having hard and infrequent bowel movements, therefore, he was given daily laxative therapy as his symptoms were attributed to constipation. Initial neurologic and abdominal examinations revealed no abnormalities. Magnetic resonance imaging (MRI) of the pelvis and abdomen was performed and reported as normal. Laboratory investigations, including comprehensive metabolic panel, complete blood count, and C-reactive protein (CRP), were also normal. The abdominal pain improved with a high dose of laxative. Both parents reported that the patient repeatedly bent his trunk while walking, with limitation of neck movements. They also noted unexplained night awakening with bouts of hallucination. He was then evaluated in the pediatric neurology clinic at our institution for his new complaints. Clinical examination showed marked limitation of his neck movements in all directions, more so on neck extension. His cranial nerve examination, motor power, tone, and deep tendon reflexes were normal. MRI of the head and neck was done and showed a relatively well-defined thoracic intramedullary cord lesion showing irregular peripheral enhancement and central areas of necrosis (Figure 1). The tumor showed significant perilesional cord edema extending to the cervico-medullary junction superiorly and down to T10 vertebral level. The differential diagnosis included low-grade astrocytoma, ependymoma, and, less likely, ganglioglioma.
The patient underwent C5-T5 laminotomy, and gross total resection of the intradural intramedullary spinal tumor was achieved. Intraoperative neuromonitoring was used.
Histopathology showed a neoplasm of low to moderate cellularity, characterized by a biphasic texture of dense fibrillar areas with bipolar cells and Rosenthal fibers and areas with low fibril density containing cells with multipolar processes and variably mucoid matrix. The nuclei of cells in both areas showed bland features. There was also focal microvascular proliferation; there was no necrosis. Mitotic figures were very sparse. The neoplastic cells were diffusely GFAP positive. Hence, the histological diagnosis was pilocytic astrocytoma (Figure 2).
Post-operative MRI revealed near-complete resection of the spinal tumor with small nodular residual tumor at the cranial extent a well as significant cord decompression with reduced cord edema cranial and caudal to the tumor site (Figure 3).
Clinically the patient was doing well during his follow-up with no neurological deficits.