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.