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Brain Dev. 2009 May;31(5):347-51. doi: 10.1016/j.braindev.2008.03.013. Epub 2008 May 15.

Early surgery of hamartoma of the floor of the fourth ventricle: a case report.

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Department of Neurosurgery, Severance Children's Hospital, Brain Korea 21 Project for Medical Science, Brain Research Institute, Yonsei University College of Medicine, Seoul, Republic of Korea.


Epilepsy from hamartoma of the floor of fourth ventricle (HFFV) is very rare, starting in neonate or infantile period, good response to surgery. We report a 3-month-old boy with episodes of medically intractable abnormal eyelid blinking and hemifacial seizure. The episodes began from the first day of life and were unresponsive to medication. A magnetic resonance imaging scan revealed mass lesion on the floor of fourth ventricle, with extended cerebellar peduncle and cerebellar hemisphere. Surgery had been performed two times previously for treatment; only a subtotal resection was performed due to severe bradycardia during the first operation. The patient underwent second operation for gross total removal of tumor. Complete resection of the mass after second surgery resulted in remission of seizures and histopathology revealed hamartoma with hemangiomatous vessel proliferation. Identification of characteristic semiology and associated HFFV can help prediction of intractability even in infant and favoring surgical treatment in early age. To our knowledge, this is the youngest patient who underwent early surgery just after progressing into status for HFFV, showing complete lesion resection resulting in successful seizure outcome.

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