Case report:
A 4-year-old male child, who had no pathologic background, presented due
to the progressive onset since 1 month of headaches, vomiting, visual
blur and diplopia. On physical examination, the patient was fully
conscious, presented a 6th cranial nerve palsy without
any motor or sensitive deficit. Fundus examination found a grade 2
papilledema. Brain CT scan (Figure n°1) showed a sellar and supra sellar
cystic tumor, surrounded by calcifications disposed on its wall. The
lesion was responsible for an important mass effect on the third
ventricle thus an upward hydrocephalus. A brain MRI (Figure n°2) was
also performed showing that this lesion was hypointense on T1-weighted
imaging (WI), hyperintense on T2-WI, and slightly enhancing on its
fleshy component after injection of Gadolinium. The tumor filled the
optico chiasmatic cistern, pushed forward the optic chiasm, and
compressed the roof of the 3rd ventricle. Biological
and hormonal assessment showed no signs for any endocrinologic
dysfunction.
Due to the major hydrocephalus, the decision was first to perform a CSF
shunting in order to decrease the intracranial pressure and facilitate
an ulterior resection of the tumor. A VP shunt was performed without any
peroperative or postoperative inadvertence. Following the surgery, the
patient presented a complete resolution of the symptoms, and he was
discharged 3 days after surgery.
Four days after discharge, the patient represented for the onset of a
low grade fever (38°), headaches, and abdominal distension, with an
unchanged neurological status. Physical examination revealed a stiff
neck and an abdominal shifting dullness.
Brain CT scan (Figure n°3) showed a significant regression of the volume
cystic portion as well as a complete resolution of the hydrocephalus.
Abdominal CT scan (Figure n°4) showed an abundant intra-abdominal fluid
collection without any evidence septa or partitions.
A lumbar puncture was performed, showing a hyperproteinorrachia (2,5
g/l), a hypercytosis (40 elements/mm3) and a normoglucorrachia. A
puncture of the ascites was also done. This liquid contained contained 6
g of protein per 100ml. Cytologic examination was negative for tumor
cells. Both cultures were sterile after 72 hours.
The decision received corticosteroids with observation of the clinical
evolution. The boy presented a total regression of both fever and
abdominal symptoms. The ascites was completely resorbed as shown by
ultrasound.
The patient was discharged after 10 days. Viewing the actual clinical
and radiological status of the patient, related to a quasi totally
calcified non voluminous supra sellar mass without any major visual or
endocrinologic impairment, a regular follow up is planned. Surgery will
be discussed in case of clinical or radiological evidence of tumoral
progression.