Spontaneous intraventricular rupture of craniopharyngioma cyst

Rupture of a cystic craniopharyngioma is a rare phenomenon. The rupture of the cyst causes decompression of the adjacent neural structures resulting in spontaneous improvement of the visual symptoms or level of sensorium. The leakage of its contents into the subarachnoid space gives rise to meningismus. We report an extremely rare phenomenon of an intraventricular rupture of a cystic craniopharyngioma, which resulted in acute neurological deterioration and chemical ventriculitis.

A 38-year-old lady presented with a 1-year history of frontal lobe dysfunction and bilateral primary optic atrophy. The CT scan showed a multi-loculated, hyperdense lesion in the region of the third ventricle and suprasellar cistern. She suffered acute deterioration of neurological status; computed tomography (CT) scan showed a hypodense lesion in the suprasellar cistern with persistent hydrocephalus. She was treated with ventricular drainage, steroids and anticonvulsants. Ventricular fluid showed high cholesterol and LDH levels. The diagnosis of craniopharyngioma was subsequently verified histologically. CONCLUSIONS The intraventricular rupture of a cystic craniopharyngioma can result in acute clinical deterioration and morbidity because of chemical ventriculitis. This is unlike the rupture in the subarachnoid space or sphenoid sinus which usually results in symptomatic improvement, although chemical meningitis may occur. This rare phenomenon should be recognized, and prompt ventricular drainage is advised. The literature is reviewed, and management of this condition is discussed.