Objective: Multicystic encephalomalacia (EMQ) is a pediatric entity where the brain tissue is substituted by cavities of variably sizes.
Patients and methods: Nineteen children diagnosed of EMQ were studied retrospectively. We analyzed the etiology, diagnosis and the clinical and radiological evolution.
Results: There were three different etiologies: twin pregnancy, 12 cases; perinatal hypoxia, 4 cases; perinatal infection, 1 case; cardiac arrest, 1 case; and 1 patient with multiple embolism of unknown cause. The diagnosis of EMQ was made with cerebral ultrasonography (14 patients), computed tomography (19 patients) and magnetic resonance imaging (5 patients). We stress the utility of cerebral ultrasonography in early and reliable diagnosis of EMQ. The clinical development was unfavorable. We studied the outcome of 15 patients. Two children died. Eleven patients (73%) show severe consequences (mental retardation, microcephaly, spastic tetraplegia, and in 7 children epilepsy). In 2 cases, the radiological development was hydranencephaly. Examination of the 4 remaining children (26.66%) revealed a spastic hemiplegia.
Conclusions: EMQ is a rare disease in childhood and has a very poor outcome, for this reason early diagnosis is very important. To this effect, cerebral ultrasonography is the imaging modality of choice.