[Symmetrical hemispheric porencephalic cysts due to twin embolization syndrome: a case report].
Twin embolization syndrome (TES) is a complication of monozygotic twining following in utero demise of the co-twin. Passage of thromboplastic materials into the circulation of the surviving fetus results in ischemic structural defects of various systemic organs including the central nervous system (CNS). In our case of monozygotic twining prenatal sonography at the 18th week of gestation revealed intracranial abnormality and demise of the co-twin. Postnatal MRI demonstrates localized cerebral parenchymal defects (porencephalic cysts) in the bilateral hemispheres, which probably were the sequelae to the occlusion of the peripheral branches of the bilateral middle cerebral arteries. She developed physio-mental retardation and subsequently West syndrome. At the 6th months, right porencephalic cyst-peritoneal shunt was performed for progressive enlargement of the head. While the enlargement of the head was well controlled, no changes in her epileptic symptoms were noted. The development of generalized epilepsy in our case may indicate that the involvement of the CNS with TES in our case is not restricted to the porencephalic cysts and their surrounding areas.
D001921:Brain / Q000002:abnormalities* / Q000473:pathology; D001927:Brain Diseases / Q000209:etiology*; D003560:Cysts / Q000209:etiology*; D004200:Diseases in Twins; D004617:Embolism / Q000150:complications*; D004829:Epilepsy, Generalized / Q000209:etiology; D005260:Female; D005313:Fetal Death; D006801:Humans; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging; D009421:Nervous System Malformations / Q000150:complications*; D011247:Pregnancy; D013577:Syndrome; D014430:Twins, Monozygotic