RESUMEN
Hypothalamic hamartomas often cause refractory epilepsy, best controlled with surgery. A transcallosal interforniceal approach provides good outcomes although it has resulted in some complications including fornix lesions with transitory and permanent memory losses.1-2 Endoscopic disconnection is less invasive, avoids interhemispheric approach, callosotomy and fornix as well as propagation of epileptic discharges.3,4 Laser disconnection does not allow objective measurement of the vaporized tissue and can cause significant hypothalamic edema. We demonstrate a simple hypothalamic disconnection endoscopy technique using the VarioGuide frameless stereotactic system (Brainlab, Germany), bipolar coagulation electrode (Karl Storz, Germany), and 3-French Fogarty catheter (Edwards Lifesciences, USA). We present a 12-yr-old right-handed girl who developed normally until 4 yr old when gelastic seizures began. Antiepileptic drugs and gamma knife radiosurgery did not control the seizures. Magnetic resonance imaging showed a nodular 13 mm left hypothalamic mass protruding into the third ventricle. The patient consented to surgery. We used a rigid 30° 6-mm neuroendoscope (Aesculap, Germany) with VarioGuide to reach the ventricle. After visualizing the foramen of Monro, the bipolar coagulation electrode perforated holes along the hamartoma/hypothalamus interface. The Fogarty balloon catheter was inserted into each hole and gently inflated to connect the holes and disconnect the mass from the hypothalamus. The patient's postsurgical recovery was excellent, presenting intact short- and long-term memory without neurological deficit or endocrine complications. She continues seizure-free 2 yr after surgery. We have successfully used this technique in 4 more cases and, if results continue to be good, will consider it a useful tool for these infrequent malformations.
Asunto(s)
Epilepsia Refractaria , Hamartoma , Niño , Epilepsia Refractaria/diagnóstico por imagen , Epilepsia Refractaria/cirugía , Endoscopía , Femenino , Hamartoma/complicaciones , Hamartoma/diagnóstico por imagen , Hamartoma/cirugía , Humanos , Enfermedades Hipotalámicas , Resultado del TratamientoRESUMEN
Multinodular and vacuolating neuronal tumor (MVNT) is a rare newly identified benign lesion, firstly included in the World Health Organization classification of tumors of the central nervous system in 2016, whose neoplastic or dysplastic nature remains unclear but with a distinctive cytoarchitectural pattern and radiological features. It is usually discovered as late-onset refractory epilepsy, headache related, or an incidental lesion of adulthood. As it is typically a stable disease, many opt for follow-up, as long as it keeps remaining asymptomatic, leaving surgery for refractory epilepsy, neurological deficits, or intracranial hypertension symptoms. A subtotal or complete resection seems to control seizures and neurological manifestations. We herein present the case of a child with refractory epilepsy related to MVNT and focal cortical dysplasia, a dual pathology case in a less frequent age group and without the typical radiological imaging. We report its radiologic features, histologic description, and management, and we present a brief literature review on MVNT focusing on the pediatric cases reported. MVNT should now be another probable low-grade epilepsy-associated lesion (LEAT) in patients of all ages, with a benign and stable course as it constitutes a curable cause of focal epilepsy. As all the refractory cases, surgery should be indicated after a comprehensive evaluation of a multidisciplinary epilepsy surgery team.
Asunto(s)
Neoplasias Encefálicas , Epilepsia Refractaria , Malformaciones del Desarrollo Cortical , Niño , Epilepsia Refractaria/diagnóstico por imagen , Epilepsia Refractaria/etiología , Epilepsia Refractaria/cirugía , Humanos , Imagen por Resonancia Magnética , Malformaciones del Desarrollo Cortical/complicaciones , Malformaciones del Desarrollo Cortical/diagnóstico por imagen , Malformaciones del Desarrollo Cortical/cirugía , ConvulsionesRESUMEN
INTRODUCTION: In 1996, Hinchey and colleagues coined the term "Posterior reversible encephalopathy syndrome" (PRES) to describe a condition seen in patients with acute neurological symptoms and reversible subcortical vasogenic edema predominantly involving parieto-occipital areas demonstrated in brain MRI. The occurrence of this phenomenon after surgical resection of CNS tumors is typically linked to pediatric cases. MATERIAL AND METHODS: Two new cases of PRES after posterior fossa surgery are reported. A thorough review of the literature is carried out with the purpose of updating and summarizing the main features regarding PRES in similar cases. Seven cases of PRES after resection of a posterior fossa tumor have been hitherto reported (4 patients were <20 years old). There is another pediatric case described after a ventriculoperitoneal shunting procedure in a patient with fourth ventricle ependymoma. Two resected tumors were ependymomas, 2 hemangiopericytomas in one patient, 1 pilocyticastrocytoma, 1 vestibular schwannoma, and 1 of the reported cases did not describe the final pathology diagnosis. CASE REPORTS: We present 2 new cases of PRES after surgical resection of a posterior fossa tumor (medulloblastoma in case 1 and ependymoma in case 2) in pediatric patients. Case 1 developed delayed seizures and altered mental status(10 days after surgical resection) after receiving treatment with bromocriptine for cerebellar mutism. Case 2 presented with generalized seizures and altered mental status within the first 48 postoperative hours followed by right hemiparesis. Both patients fully recovered and returned to neurological baseline status. A thorough review of the literature was carried out with the purpose of updating and summarizing the main features regarding PRES in similar cases. CONCLUSIONS: We report 2 new pediatric cases of posterior reversible encephalopathy syndrome (PRES) that developed after surgical resection of a posterior fossa tumor. Appropriate management includes supportive measures, antihypertensive agents, and antiepileptic drugs, if needed. Full recovery is the most likely outcome in line with previous articles.