Dados do Trabalho

Título

Bilateral open lip schizencephaly, a cortical malformation: a case report

RESUMO

CASE PRESENTATION A 3-year-old female patient was diagnosed with bilateral open-lip schizencephaly at 4 months of age. The pregnancy was discovered at 6 months, with placental abruption and loss of amniotic fluid. The patient was delivered by cesarean section, without complications. At 20 days, she had the first seizure and the second at 2 months, when the investigation was started. At 4 months, the patient was diagnosed with open-lip Schizencephaly through tomography, with the presence of three clefts that communicate the ventricular system to the surface of the pia mater, showing clefts in the temporal region on the right, and in the frontal and temporal lobes. on the left, with initiation of carbamazepine, but without resolution, being hospitalized in a pediatric ICU with ventilatory support due to repeated crises. After changing the medication, the condition stabilized for a period, with the use of phenytoin, valproic acid and phenorbarbital. In this year, 2022, there was a return of seizures, associated with a drop in oxygen saturation, carbamazepine was prescribed again and neuleptil was started, with the medications already prescribed. On physical examination, the patient has a delay in neuropsychomotor development, does not hold her head, does not sit without support, does not walk and does not speak, but has preserved speech understanding. Currently, the condition has improved with the medications in use and he is undergoing continuous medical follow-up.
DISCUSSION Schizencephaly is a rare malformation of the cerebral cortex (1.48/100,000 births) that can be open or closed, bilateral or not. The etiology is still not well understood, as well as the period of embryonic development that is involved in the development of the malformation. The clinical manifestations of the disease are associated with the degree to which the brain area was affected, ranging from individuals who lead a normal life without manifestations, to severe cases of neurological impairment, with epilepsy being the most common manifestation.
FINAL COMMENTS This study brings the symptomatic management of a cortical malformation, showing the importance of continued monitoring so that there is an improvement in the quality of life of the child and parents, reducing the damage caused by clinical manifestations and allowing the child to have a better life. as close to normal as possible.