• Shih-Shan Lang, Ethan Goldberg, Deborah Zarnow, Mark P Johnson, Phillip B Storm, and Gregory G Heuer.
• Department of Neurosurgery, Hospital of the University of Pennsylvania, USA; Division of Neurosurgery, Center for Fetal Diagnosis and Treatment, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA. Electronic address: shihshan.lang@uphs.upenn.edu.
• World Neurosurg. 2014 Jul 1;82(1-2):241.e5-8.

BackgroundIn recent literature, there have been case reports of prenatal diagnosis of hemimegalencephaly, an extremely rare entity characterized by enlargement of all or portions of 1 cerebral hemisphere and intractable seizures. A unique case is presented of hemimegalencephaly of a fetus diagnosed in utero.MethodsA 27-year-old woman presented at 32 weeks' gestation for fetal magnetic resonance imaging after an abnormal fetal ultrasound. Fetal magnetic resonance imaging showed hemimegalencephaly of the left cerebral hemisphere with abnormal gyration.ResultsThe patient was born via cesarean section at 39 weeks' gestation. He had continuous infantile spasms and partial-onset seizures starting on day 1 of life, and electroencephalography showed burst suppression. The patient's seizures were initially managed with antiepileptics, prednisolone, and a ketogenic diet; however, he was hospitalized multiple times because of status epilepticus. At 6 months of age, he underwent a successful anatomic left hemispherectomy.ConclusionsIn utero diagnosis of complex developmental brain anomalies allows a multidisciplinary approach to provide optimal prenatal patient treatment and parental counseling.Copyright © 2014 Elsevier Inc. All rights reserved.

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