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.