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Cranial MR Imaging in Hypomelanosis of Ito
J Comput Assist Tomogr 14:981-983, Williams III,D.W.&Elster,A.D., 1990
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Article Abstract
Hypomelanosis of Ito(HI)is a rare neurocutaneous syndrome frequently associated with neurologic abnormalities.Approximately 95 cases have been reported in the literature.Intracranial findings demonstrated by magnetic resonance imaging in a patient with HI include hemimegalencephaly,abnormal white matter signal,and small discrete bilateral periventricular cysts.No gray matter heterotopias or other migrational abnormalities were identified.Periventricular cystic lesions have not been described previously in this condition.newabs GN183D We have observed many focal dilatations or very small aneurysms in terminal arterioles and capillaries of 4 of 5 patients and 6 dogs who had recently undergone cardiopulmonary bypass.A smaller number of sausagelike dilatations distended medium-sized arterioles.Two other patients had a small number of the same microvascular changes following proximal aortography.Thirty-four patients and 6 dogs not undergoing cardiopulmonary bypass had none.(A 35th patient who had not undergone cardiopulmonary bypass or aortography showed a small number of dilatations;mediastinal air was a suggested source.)Some of the dilatations exhibited various forms of birefringence.Because most of the dilatations appear empty,we speculate that they are the sites of gas bubbles or fat emboli that have been removed by the solvents used in processing.These microvascular events,occurring only in conjunction with major arterial interventions,may be the anatomical correlate of the neurological deficits or moderate to severe intellectual dysfunction seen in at least 24%of patients after cardiac surgical procedures assisted by cardiopulmonary bypass.
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congenital malformation
developmental retardation
hypomelanosis of Ito
hypopigmentation of skin
MRI
MRI,abnormal
skin,lesions in neurologic disorders
white matter disease
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