Two-Stage Removal of Giant Craniopharyngioma with a Favorable Outcome

Usanov E.I., Korshunov N.B., Kirichenko K.N., Ivanov O.B.

(Polenov Research Neurosurgical Institute, Raukhfus Municipal Hospital N 19)

Male patient B., aged 4, was admitted to the Neurosurgical Department of Rakhfus Municipal Hospital N 19 on March 29, 2000 with complaints of constant headache. nausea, vomiting in the morning, failing sight, mental retardation, hyperexcitability and aggressive behavior. He had been sick since birth and was followed up by a neurologist for perinatal encephalopathy. Frequent attacks of headache appeared at the age of 2; besides, there were undue fatigability, failing sight, retarded growth. The patient's height, weight and head circumference were equal to 98 cm, 16 kg and 53.5 cm respectively. There was right-sided reflex hemiparesis. Vision acuity was 0.7 (both eyes); visual fields were normal; there was marked engorgement of disks. CT examination of March 28, 2000 revealed craniopharyngioma in an endo-suprasellar area and a paratumor cyst (7.8 x 4.7 cm), localized in the left frontal and temporal lobes and going beyond the median line.

The first stage of the operation was performed on April 20, 2000. It consisted in removal of the craniopharyngeal cyst together with its capsule, using the transcortical approach. The cyst contained 200 ml of amber-colored fluid. The operation resulted in a better general state, absence of headache and improved visual functions.

The second stage took place in six months (October 18, 2000). Removal of craniopharyngioma with extra-intraventricular localization was performed with the help of the right-sided subfrontal approach (Prof. Usanov E.I.). The tumor enlarged considerably during this period of time. Marked diabetes insipidus developed during a postoperative period. It was arrested by taking adiuretin-SD. On discharge urine specific gravity was 1011-1018. Improvement of visual functions was observed (Vis OD=0.9, OS=0.8). There were signs of primary atrophy of the optic nerve disks. The patient was discharged on the 12 day after the operation.

The demonstration goal:

To demonstrate a rare atypical form of craniopharyngioma, possibility of its two-stage removal and to attract attention of children's neurologists to delayed diagnosis of craniopharyngiomas in their patients.