Symptomatic epilepsy in children with poroencephalic cysts secondary to perinatal middle cerebral artery occlusion

Background Perinatal cerebral artery occlusion is responsible for ischemic cerebral infarction leading to brain cavitation and gliosis; the territory of the middle cerebral artery is most frequently involved. The resulting poroencephalic cysts are frequently associated with hemiplegia and epilepsy; that can be managed medically in most cases, only 6-7% of them being refractory to medical treatment. This particular subset of congenitally hemiplegic children will be possible candidates for electrophysiological investigation and eventually for resective surgery. Whatever the kind of surgical treatment, surgery should be performed as soon as possible to optimize functional brain reorganization. Clinical material Twelve children with poroencephalic cysts and refractory epilepsy were studied and operated on at the Divisions of Child Neurology and Pediatric Neurosurgery, the Catholic University Medical School, Rome. The hemiparesis ranged from mild to moderate; the developmental delay was of mild degree in three cases, moderate in four cases and severe in the remaining five. Behavioral disorders were observed in patients with mental retardation; two of them also manifested autistic features. All the children presented with a severe epileptic syndrome (starting almost invariably during the first year of life); six patients presented with a West syndrome followed by symptomatic partial epilepsy; the other six presented with partial epilepsy, followed in two cases by continuous spike-waves during sleep. The electroencephalograph (EEG) recordings disclosed focal unilateral interictal epileptiform abnormalities that usually corresponded to the side of the cystic lesion; however, paroxysmal activity often spread synchronously over the contralateral hemisphere. The selection of candidates for surgical treatment was based on neuroimaging and video-EEG monitoring; in particular, we did not use invasive intraoperative neurophysiologic techniques. The convergence of neuroimaging and neurophysiologic findings guided us in performing a limited cortical excision corresponding to the malacic cortex (cyst "membrane"). Results All the patients underwent excision of the cyst wall. Careful attention was paid not to enter the body of the lateral ventricle to avoid ventriculo-subarachnoid fistulas, eventually responsible for subdural hygroma or cerebrospinal fluid leak. There was one surgery-related death secondary to disseminated intravascular coagulation, following an otherwise uneventful surgical procedure. An elevated systemic blood pressure, secondary to repeated adrenocorticotropic hormone therapy, can represent a possible concurrent factor for this event. No major complications were recorded among the remaining 11 children. Seizure control was excellent in all the 11 survivors in the early postoperative period. Two children presented a relapse of seizures, after an initial improvement, respectively 3 and 4 years after the operation. These two children underwent subsequently a functional hemispherectomy. Overall, seizure outcome was excellent in all the cases. Seven patients (including the two who underwent functional hemispherectomy) are seizure-free (Engel's class Ia), and in one of them antiepileptic therapy has been weaned. In the remaining five children, seizures are sporadic and definitely improved (Engel's class II). An improvement of developmental delay, in particular of cognitive competence, was registered in 8 out of the 11 patients. Two of the four severely retarded children, who also presented behavioral abnormalities, did not show any cognitive improvement, whereas some mild improvement of their basal abilities was demonstrated in the other two. All the remaining children, even though maintaining a moderate retardation, definitely improved their abilities; in particular, one of them reached an almost borderline level. The three patients with unchanged neurodevelopmental delay presented also persistent seizures. On the other hand, two children with persistent seizures presented neurodevelopmental improvement. Conclusions Simple surgical excision of the cyst "membrane" of epileptogenic poroencephalic cysts can represent an excellent means to control epilepsy in affected children. However, postoperative seizure persistence and late recurrences, although rare, do not allow to exclude that hemispherectomy or partial resections (based on electrocorticography findings) might represent the good answer at least in some cases.