Sergio Cavalheiro, Heloisa Galvão do Amaral Campos, and Marcos Devanir Silva da Costa
Fetal brain tumors are rare. This report describes a giant posterior fossa capillary hemangioma treated with 3 mg/kg/day of propranolol for 6 months. Total regression was confirmed at 1 year, and no additional tumors were observed during the subsequent 2 years. No side effects relating to the use of this drug were detected; thus, the authors believe that propranolol may be useful for treating all intracranial capillary hemangiomas.
Marcos Devanir Silva da Costa, Bruno Fernandes de O. Santos, Felipe Bouchabki de Almeida Guardini, and Feres Chaddad-Neto
The management of arteriovenous malformations (AVMs) of the corpus callosum and choroidal fissure is challenging because they commonly receive arterial feeders from the anterior and posterior circulation, and drain to deep veins. In this video the authors present the case of a 20-year-old man who presented with acute onset of headache, loss of consciousness, and nuchal rigidity. Computed tomography, MRI, and cerebral angiography performed in tandem revealed a ruptured, large, Grade IV AVM of the corpus callosum and choroidal fissure with two groups of arterial feeders: one from the pericallosal artery and the other from the medial and lateral posterior choroidal arteries. The treatment strategy included two stages. The first stage involved preoperative embolization of the arterial feeders from the posterior circulation, which promoted reduction of the nidus flow of the AVM. The second involved a microsurgical resection, using the interhemispheric approach, with the patient in the prone position, which allowed accessing the anterior circulation feeders and the complete resection of the AVM, without associated morbidity.
The video can be found here: https://youtu.be/5wcYKhcJtls.
Sergio Cavalheiro, María Laura Canullo, Marcos Devanir Silva da Costa, Patricia Alessandra Dastoli, Jardel Mendonça Nicácio, and João Norberto Stavale
Glioependymal cysts are a rare, congenital, and benign condition. The authors report a case of a 2-year-old female patient who presented with a large exophytic glioependymal cyst located in the left cerebral peduncle, which began with a history of deteriorating function of the left third cranial nerve (CN III). A left 3-piece fronto-orbitozygomatic approach and a microsurgical complete excision of the cyst were performed without complications by accessing the midbrain through the perioculomotor entry zone. The patient’s CN III function completely recovered after 3 months. Because there are currently no widely approved and used classification systems for these cysts, their reporting, naming, and classification are quite confusing in the literature. Thus, their origin, classification, and most common locations warrant further investigation. In addition to reporting this case, the authors present their review of the literature.
Marcos Devanir Silva da Costa, Nicole Cavalari Camargo, Patricia Alessandra Dastoli, Jardel Mendonça Nicácio, Frederico Adolfo Benevides Silva, Maria Luisa Sucharski Figueiredo, Michael Jenwei Chen, Andrea Maria Cappellano, Nasjla Saba da Silva, and Sergio Cavalheiro
Tumors of the CNS are the main causes of childhood cancer and have an incidence that exceeds that of leukemia. In addition, they are the leading causes of cancer-related death in childhood. High-grade gliomas account for 11% of such neoplasms and are characterized by aggressive clinical behavior and high morbidity and mortality. There is a lack of studies focusing on the factors that can prolong survival in these patients or guide therapeutic interventions. The authors aimed to investigate the factors related to longer survival durations, with a focus on reoperation for gross-total resection (GTR).
In this retrospective cohort study, the authors analyzed 78 patients diagnosed with high-grade gliomas occurring across all CNS locations except diffuse intrinsic pontine gliomas. Patients 0 to < 19 years of age were followed up at the Pediatric Oncology Institute. Overall survival (OS) and progression-free survival (PFS) were analyzed in the context of various prognostic factors, such as age, sex, histology, extent of tumor resection, reoperation for GTR, adjuvant treatment, and treatment initiation from 2010 onward.
With a mean age at diagnosis of 8.7 years, 50% of the patients were female and approximately 39% underwent GTR at some point, which was already achieved in approximately 46% of them in the first surgery. The median OS was 17 months, and PFS was 10 months. In terms of median OS, the authors found no significant difference between those with reoperation for GTR and patients without GTR during treatment. Significant differences were observed in the OS in terms of the extent of resection in the first surgery, age, sex, Ki-67 expression, adjuvant treatment, and treatment initiation from 2010 onward. Furthermore, the PFS values significantly differed between those with GTR in the first surgery and Ki-67 expression ≥ 50%.
This study demonstrates the importance of GTR for these neoplasms, highlights the role of surgeons in its achievement in the first attempt, and questions the role of reoperation for this purpose. Finally, this study further supports the use of combined adjuvant treatment for the improvement of OS and PFS.
Feres Chaddad-Neto, Marcos Devanir Silva da Costa, Baran Bozkurt, Hugo Leonardo Doria-Netto, Daniel de Araujo Paz, Ricardo da Silva Centeno, Andrew W. Grande, Sergio Cavalheiro, Kaan Yağmurlu, Robert F. Spetzler, and Mark C. Preul
The authors report a novel surgical route from a superior anatomical aspect—the contralateral anterior interhemispheric-transcallosal-transrostral approach—to a lesion located in the subcallosal region. The neurosurgical approach to the subcallosal region is challenging due to its deep location and close relationship with important vascular structures. Anterior and inferior routes to the subcallosal region have been described but risk damaging the branches of the anterior cerebral artery.
Three formalin-fixed and silicone-injected adult cadaveric heads were studied to demonstrate the relationships between the transventricular surgical approach and the subcallosal region. The surgical, clinical, and radiological history of a 39-year-old man with a subcallosal cavernous malformation was retrospectively used to document the neurological examination and radiographic parameters of such a case.
The contralateral anterior interhemispheric-transcallosal-transrostral approach provides access to the subcallosal area that also includes the inferior portion of the pericallosal cistern, lamina terminalis cistern, the paraterminal and paraolfactory gyri, and the anterior surface of the optic chiasm. The approach avoids the neurocritical perforating branches of the anterior communicating artery.
The contralateral anterior interhemispheric-transcallosal-transrostral approach may be an alternative route to subcallosal area lesions, with less risk to the branches of the anterior cerebral artery, particularly the anterior communicating artery perforators.
Daniela de Souza Coelho, Bruno Fernandes de Oliveira Santos, Marcos Devanir Silva da Costa, Gisele Sampaio Silva, Sergio Cavalheiro, Flávia H. Santos, and Feres Chaddad-Neto
A cerebral arteriovenous malformation (cAVM) can change over time and cause symptoms, but clinical studies tend to define only the patients with ruptured cAVMs as symptomatic and do not consider neurocognitive aspects prior to neurosurgical intervention. The objective of this study was to describe the neurocognitive function of patients with ruptured and unruptured cAVMs according to the Spetzler-Martin (SM) grade, flow status, and anatomical topography.
In this blinded cross-sectional study, 70 patients of both sexes and ages 18–60 years were evaluated using the Brazilian Brief Neuropsychological Assessment Battery Neupsilin.
Of the 70 patients with cAVMs, 50 (71.4%) demonstrated deficits in at least one of the eight neurocognitive domains surveyed, although they did not exhibit neurological deficits. cAVMs in the temporal lobe were associated with memory deficits compared with the general population. The SM grade was not significantly associated with the results of patients with unruptured cAVMs. However, among patients with ruptured cAVMs, there were deficits in working memory in those with high-grade (SM grade) cAVMs and deficits in executive function (verbal fluency) in those with low-grade cAVMs (p < 0.001).
This study indicates that patients with untreated cAVMs, either ruptured or unruptured, already exhibit neurocognitive deficits, even the patients without other neurological symptoms. However, the scales used to evaluate disability in the main clinical studies, such as A Randomized Trial of Unruptured Brain Arteriovenous Malformations (ARUBA), do not assess neurocognitive alterations and therefore disregard any deficits that may affect quality of life. The authors’ finding raises an important question about the effects of interventional treatment because it reinforces the hypothesis that cognitive alterations may be preexisting and not determined by interventions.
Sergio Cavalheiro, Marcos Devanir Silva da Costa, Jardel Mendonça Nicácio, Patricia Alessandra Dastoli, Italo Capraro Suriano, Mauricio Mendes Barbosa, Hérbene Jose Milani, Stéphanno Gomes Pereira Sarmento, Tereza Cristina Carbonari de Faria, and Antonio Fernandes Moron
In this study, the authors retrospectively reviewed two cohorts of patients with occipital encephalocele (OE) and demonstrate the technical aspects of an innovative and unprecedented method for fetal OE correction.
This was a cross-sectional retrospective study of 22 patients who underwent surgery from July 2012 to July 2018. The inclusion criteria for participants were a gestational age between 19 weeks and 27 weeks, a maternal age ≥ 18 years, a normal fetal karyotype, the presence of microcephaly, and a cystic hernial sac with the cystic component accounting for more than 80% of the total volume, which was larger than 10 ml. The fetuses with OE and microcephaly underwent intrauterine repair. The exclusion criteria were the presence of a fetal anomaly not associated with OE, chromosomopathy, presence of the brainstem inside the hernial sac or venous sinuses inside the herniated content, the risk of premature birth, placenta previa, and maternal conditions that would constitute an additional risk for fetal and maternal health, as well as refusal for fetal surgery. The authors reviewed the potential for microcephaly reversal and the long-term neurocognitive development.
The authors report the cases of 9 fetuses with OE and microcephaly who underwent intrauterine OE repair at gestational ages of less than 27 weeks (fetal group). One case selected for fetal surgery also presented with placental abruption. All patients who underwent the fetal operation had microcephaly reversal, and 3 patients required ventriculoperitoneal shunting in the 1st year of life. The authors reviewed the cases of 12 patients who underwent postnatal repair. In 10 cases, fetal surgery was refused (postnatal group), and in 2 cases, the inclusion criteria for fetal surgery were not met. The authors evaluated the neurocognitive development of the patients with the Bayley Scales of Infant Development II. The median score for the fetal group was 98.7, and that for the postnatal group was 27.8.
The intrauterine repair of OE may stop the progression of encephalocele sac herniation and result in microcephaly reversal. The fetal group had a better cognitive outcome than the postnatal group. The technique required to correct this defect is feasible for those with previous experience in the correction of fetal myelomeningocele. However, more studies are needed to ensure the efficacy of this procedure.
Marcos Devanir Silva da Costa, Daniel de Araujo Paz, Thiago Pereira Rodrigues, Ana Camila de Castro Gandolfi, Fabricio Correa Lamis, João Norberto Stavale, Italo Capraro Suriano, Luiz Daniel Marques Neves Cetl, and Sergio Cavalheiro
Spinal angiolipomas are rare benign tumors that generally induce slow progressive cord compression. Here, the authors describe a case of sudden-onset palsy of the lower extremities caused by hemorrhagic spinal angiolipoma. An emergent laminectomy was performed to achieve total lesion removal. Follow-up examinations indicated neurological improvement and the absence of recurrence.