Multiple Cavernous Malformations with Hemorrhage of Brain

Abstract

To the Editor: The cavernous malformations of brain are benign and rarely manifest in children [1]. We report a nonfamilial multiple cavernomatous malformation of brain with intra parenchymal hemorrhage in a developmentally delayed child, presented in emergency with refractory status epilepticus and hemiparesis. A 16-mo-old boy presented with fever for 2 mo, multiple episodes of abnormal movement right side and unconsciousness for 2 d. There was no history of vomiting, ear discharge and skin rashes. On examination, child was drowsy and had right sided hemiparesis, grade-3 power with ipsilateral upper motor neuron type of facial nerve palsy. Other system examinations were normal. The hemogram and cerebrospinal fluid examinations were normal. Arterial blood gases (ABG) at admission revealed metabolic acidosis. Cranial tomograph showed multiple hyperdense foci with few ring enhancing lesions in left cortex along vascular channels (Fig. 1). MRI of brain (T1-weighted) showed multiple hemorrhagic cystic lesions in parietotemporal-occipital region, internal capsule and cerebellum with perilesional gliosis, calcifications and hemorrhages suggestive of multiple cavernoma with parenchymal dysplasia (Fig. 2). Patient was treated with lorazepam, followed by phenytoin, valproic acid and midazolam infusion to control seizure and discharged on oral antiepileptics. Patient was seizure free and hemiparesis improved after 6 mo. The prevalence of cavernous malformation is 0.5– 0.7 % and usually present in 3rd–6th decade [2, 3]. It may be sporadic or familial but familial type usually have multiple cavernous malformations and lead to recurrent hemorrhages in neural tissues and manifests as seizure, headache and neurological deficits. The deficiency of collagen IV fraction [4] and high plasma vascular endothelial growth factor (VEGF) [5] were correlated with recurrent microhemorrhages, reactive gliosis and progressive cavernous malformations respectively. MRI is the most sensitive tool for diagnosis. Stereotactic surgery of hemorrhagic lesions is recommended in children