Prenatal diagnosis of hemimegalencephaly.
BACKGROUND
In 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.


METHODS
A 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.


RESULTS
The 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.


CONCLUSIONS
In utero diagnosis of complex developmental brain anomalies allows a multidisciplinary approach to provide optimal prenatal patient treatment and parental counseling.
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.
Shih-Shan
SS
Lang
Division of Neurology, Center for Fetal Diagnosis and Treatment, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
Ethan
E
Goldberg
Department of Radiology, Center for Fetal Diagnosis and Treatment, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
Deborah
D
Zarnow
Department of Surgery, Center for Fetal Diagnosis and Treatment, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
Mark P
MP
Johnson
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.
Phillip B
PB
Storm
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.
Gregory G
GG
Heuer
D000328:Adult; D038481:Anterior Temporal Lobectomy; D001921:Brain / Q000473:pathology; D002585:Cesarean Section; D004569:Electroencephalography; D005260:Female; D005333:Fetus / Q000473:pathology; D005500:Follow-Up Studies; D038421:Hemispherectomy / Q000379:methods; D006801:Humans; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging; D008297:Male; D054220:Malformations of Cortical Development / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D019635:Neurosurgical Procedures / Q000379:methods; D011247:Pregnancy; D011296:Prenatal Diagnosis; D012640:Seizures / Q000209:etiology; D013226:Status Epilepticus / Q000209:etiology / Q000601:surgery
Cortical dysplasia; Fetal imaging; Hemimegalencephaly; Hemispherectomy; MRI; Neuronal migration