Spontaneous Intraventricular Rupture of Craniopharyngioma Cyst Vaijayantee Kulkarni, M.Ch., Roy Thomas Daniel, M.Ch., and Ramachandra Pranatartiharan, M.Ch. Department of Neurological Sciences, Christian Medical College and Hospital, Vellore, India Kulkarni V, Daniel RT, Pranatartiharan R. Spontaneous intraven- tricular rupture of craniopharyngioma cyst. Surg Neurol 2000;54:249 –253. BACKGROUND Rupture of a cystic craniopharyngioma is a rare phenom- enon. The rupture of the cyst causes decompression of the adjacent neural structures resulting in spontaneous improvement of the visual symptoms or level of senso- rium. 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 cys- tic craniopharyngioma, which resulted in acute neurolog- ical deterioration and chemical ventriculitis. CASE DESCRIPTION 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; com- puted tomography (CT) scan showed a hypodense lesion in the suprasellar cistern with persistent hydrocephalus. She was treated with ventricular drainage, steroids and anticon- vulsants. Ventricular fluid showed high cholesterol and LDH levels. The diagnosis of craniopharyngioma was subse- quently verified histologically. CONCLUSIONS The intraventricular rupture of a cystic craniopharyngi- oma can result in acute clinical deterioration and mor- bidity because of chemical ventriculitis. This is unlike the rupture in the subarachnoid space or sphenoid sinus which usually results in symptomatic improvement, al- though chemical meningitis may occur. This rare phe- nomenon should be recognized, and prompt ventricular drainage is advised. The literature is reviewed, and man- agement of this condition is discussed. © 2000 by Elsevier Science Inc. KEY WORDS Craniopharyngioma cyst, spontaneous rupture, intra- ventricular. S pontaneous rupture of a craniopharyngioma cyst is a very rare but well-recognized phe- nomenon. Around 15 cases have been reported so far [1– 8,10]. The features of meningismus are often accompanied by improvement in the neurological symptoms, as the cyst decompresses by discharg- ing its contents into the subarachnoid space [3,5, 8 –10]. The recognition of this phenomenon is usu- ally based on the clinical picture, CSF abnormality and radiological evidence of shrinkage of the cyst. However, intraventricular rupture of a craniophar- yngioma cyst is extremely rare and only one case has been reported so far where the ventricular com- munication was demonstrated by ventriculography and then post-mortem [6]. We report the first com- puted tomography (CT)-documented case of intra- ventricular rupture of a multi-compartmental cra- niopharyngioma cyst, which was associated with acute clinical deterioration. The radiological find- ings and the management are discussed. Case Report A 38-year-old lady presented with a 3-month history of personality change, behavioral disturbances, uri- nary incontinence, and gait ataxia. Examination re- vealed a placid, apathetic lady with reduced visual acuity and bilateral optic disc pallor. A non contrast-enhanced CT scan of the brain revealed a large, lobulated hyperdense lesion in the region of the suprasellar cistern and the third ventricle with retrosellar extension. (Figure 1) A day after admis- sion, she had acute deterioration in sensorium. She became unresponsive, with dilated non-reactive pu- pils. She did not have fever, neck pain, nuchal ri- gidity, or seizures during this period. An emergency Address reprint requests to: Dr Roy Thomas Daniel, Lecturer in Neuro- surgery, CMC Hospital, Vellore 632004, India. Received July 12, 2000; accepted August 9, 2000. © 2000 by Elsevier Science Inc. 0090-3019/00/$–see front matter 655 Avenue of the Americas, New York, NY 10010 PII S0090-3019(00)00288-3