Hemisferectomia funcional precoce na hemimegalencefalia associada à epilepsia refratária

Abstract

RESUMO -A hemimegalencefalia (HME) é malformação congênita cerebral rara de etiologia desconhecida que pode se apresentar com síndrome epiléptica de início precoce e resistente à terapia com anticonvulsivantes, associada a comprometimento significativo do desenvolvimento neuropsicomotor. A hemisferectomia funcional (HF) tem-se mostrado alternativa eficaz nos casos refratários à terapêutica medicamentosa. Em número diminuto, crianças foram operadas antes dos seis meses de idade. Esse estudo relata duas crianças com idade inferior a 6 meses com HME e síndrome epiléptica catastrófica submetidas a HF e com boa evolução clínica.PALAVRAS-CHAVE: hemimegalencefalia, hemisferectomia, epilepsia. Early functional hemispherectomy in hemimegalencephaly associated to refractory epilepsyABSTRACT -Hemimegalencephaly (HME) is a rare congenital brain malformation of unknown etiology. Patients with HME can present with an early onset epileptic syndrome which is often refractory to medical treatment and associated to impaired neurological development. Functional hemispherectomy (FH) has proven to be a valuable treatment alternative in patients with refractory epilepsy in this setting. Very few children operated under the age of 6 months and bearing HME and catastrophic epilepsy (CE) have been described in the literature. This study reports on 2 kids younger than 6 months with HME and CE submitted to FH with good clinical outcome.

Hemispherectomía en el tratamiento de las convulsiones: perspectivas actuales de las indicaciones, técnicas y complicaciones - Revisión de literatura

“…In the literature has been observed a low rate of mortality associated a anatomical and functional hemispherectomy surgery, ranging from 2% to 7% 1,4,6,8,10,18,27,33,35,38,40 and ranging from 0% to 4% 1,2,3,7,10,34,46,52,5357,58,59,60,61 , respectively. Furthermore, a seizure free rate of 81% was described in 2004; this is being related to an intraoperative technology of the modern era.…”

“…Furthermore, it is also offered at times as a purely palliative procedure for severe cases with bilateral seizure onset when one side predominates 16,38,39,40,42 . At the moment, the patients being indicated for hemispherectomy needs to comply with theses criterias: • Patients with medical intractability of seizures 3,18,19,21,27 Spread of epileptiform discharges to the normal hemisphere on EEG or even rare independent discharges on the normal side however does not imply a poor response to surgery 3,19,39 .…”

“…About the consequences of hemispherectomy surgery, many complications may be listed like a hemosiderosis, hidrocephalus, cerebralspinal fluid leaks, intracranial postoperative hematomas, osteomielitis, epiditimitis, trivial head traumas, infection, Hypothermia, "aseptic meningitis", neurological deficits, hemiparesis 1,2,3,27,35,38,40 . Cook et al 17 , in 2004, showed in a case series of comparation of anatomical hemispherectomy, functional hemispherectomy, and hemispherotomy.…”

“…Hemispherectomy is a palliative surgical approach that aims to control potentially harmful seizures, for instance, atonic or drop seizures, preventing the spread of epileptic electrical activity 1,2,3,18,27 . This technique was presented to epilepsy surgery in 1938 by Mckenzie 44 , however the first consistent description about this procedure it happened in 1950 by Krynauw 36 , whose essay described the use of this technique to remove the brain hemisphere with hemiplegic infantile in 12 children and it showed control of seizures associated to a recovery of cognitive function considered excellent by the standards of the time.…”

Hemisferotomia transilviana transopercular peri-central core: aspectos anatômicos, técnica cirúrgica e resultados clínicos.

“…As manifestações neurológicas são causadas pela presença de malformações vasculares que comprometem a perfusão cerebral (64) . O suprimento sanguíneo reduzido ao hemisfério cerebral afetado, causa degeneração neuronal progressiva consequentemente levando à atrofia do parênquima cerebral, crises convulsivas e instalação de déficits neurológicos (65) . Os sinais e sintomas mais frequentemente encontrados são cefaleia, convulsões, atraso no desenvolvimento neuropsicomotor, hemiparesia e hemianopsia (66) .…”