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J Neurosurg Pediatr. 2013 Aug;12(2):155-9. doi: 10.3171/2013.5.PEDS1328. Epub 2013 Jun 21.

Repeated cyst formation in a patient with leukoencephalopathy, cerebral calcifications, and cysts: effectiveness of stereotactic aspiration with Ommaya reservoir placement.

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  • 1Department of Neurosurgery, Oita University Faculty of Medicine, Oita, Japan.


The combination of leukoencephalopathy, cerebral calcifications, and cysts (LCC) constitutes a rare cerebral disorder characterized by distinctive neuroradiological and clinical findings. Twenty-eight cases of LCC have been reported. Surgery is usually required to treat progressively expanding cysts, but surgical strategies have been varied. The authors present the case of a patient who underwent 4 surgical procedures for repeated cyst formation. The first operation was performed for the removal of a cyst and to make a histopathological diagnosis. The second and third operations were performed to treat de novo cysts. The fourth operation was performed to treat a recurrence. This is the first reported case of LCC in which it was necessary to perform surgery 4 times, in large part due to de novo cyst formation. It provides evidence that multiple cysts may develop in these patients and that several surgical operations may be needed to treat LCC. Stereotactic aspiration with Ommaya reservoir placement is an appropriate procedure for this condition because it is less invasive and more repeatable than open surgery.

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