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Mauricio Mandel, Igor Araújo Ferreira da Silva, Wellingson Paiva, Yiping Li, Gary K. Steinberg, and Manoel Jacobsen Teixeira

OBJECTIVE

Craniocervical junction–related syringomyelia (CCJS) is the most common form of syringomyelia. Approximately 30% of patients treated with foramen magnum decompression (FMD) will show persistence, recurrence, or progression of the syrinx. The authors present a pilot study with a new minimally invasive surgery technique targeting the pathophysiology of CCJS in adult patients.

METHODS

The authors retrospectively analyzed the clinical and radiological features of a consecutive series of patients treated for CCJS. An FMD and FM durectomy were performed through a 1.5- to 2-cm skin incision. Then arachnoid adhesions were cleared, creating a permanent communication from the fourth ventricle to the new paraspinal extradural cavity (obexostomy) and with the spinal subarachnoid space. The hypothesis was that the new CSF pouch acts like a pressure leak, interrupting the CCJS pathogenesis.

RESULTS

Twenty-four patients (13 female, 21–61 years old) were treated between 2014 and 2018. The etiology of CCJS was Chiari malformation type I (CM-I) in 20 patients (83.3%), Chiari malformation type 0 (CM-0) in 2 patients (8.3%), and CCJ arachnoiditis in 2 patients (8.3%). Two patients underwent reoperations after failed FMD for CM-I at other institutions. No major surgical complication occurred. One patient had postoperative meningitis with no CSF fistula. On postoperative MRI, shrinkage of the syrinx was seen in all patients. No patients experienced recurrence of the CCJS. No patient required a subsequent operation. The mean duration of surgery was 72 ± 11 minutes (mean ± SD), and blood loss was 35–80 ml (mean 51 ml). Follow-up ranged from 12 to 58 months. The average overall improvement in modified Japanese Orthopaedic Association scores was 10% (p < 0.001). The Odom scale showed that 19 patients (79.1%) were satisfied, 4 (16.7%) remained the same, and 1 (4.2%) reported a poor outcome. All patients experienced postoperative improvement in perception of quality of life (p < 0.001).

CONCLUSIONS

Minimally invasive FM durectomy and obexostomy is a safe and effective treatment for CCJS and for patients who have not responded to other treatment.

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Almir Ferreira de Andrade, Eberval Gadelha Figueiredo, Robson Luis Oliveira de Amorim, Wellingson S. Paiva, Guilherme Lepski, and Manoel Jacobsen Teixeira

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Sergio Brasil, Wellingson Silva Paiva, Ricardo de Carvalho Nogueira, Angela Macedo Salinet, and Manoel Jacobsen Teixeira

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Idiopathic distal lenticulostriate artery aneurysm in a child

Case report and review of the literature

Hamilton Matushita, Robison Luis Oliveira Amorim, Wellingson Silva Paiva, Daniel Dante Cardeal, and Fernando Campos Gomes Pinto

✓ The authors describe a rare case of idiopathic distal lenticulostriate artery (LSA) aneurysm in a 5-year-old boy who presented in the emergency department with a sudden onset of headache. Admission computed tomography scans revealed an intracerebral hemorrhage in the left caudate nucleus with intraventricular extension. Angiographic studies demonstrated a left medial LSA aneurysm. The patient underwent a left parasagittal frontal craniotomy, the lateral ventricle was accessed via the anterior transcallosal approach, and the aneurysm was removed after sectioning of the parent vessel. The child left the hospital after 5 days; at that time he was asymptomatic and without motor impairment. The optimum treatment of aneurysms involving small perforating arteries is controversial and depends mainly on the causative factors. The pathogenesis and treatment of these unusual aneurysms are discussed.

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Barbara A. Morais, Daniel D. Cardeal, Fernanda G. Andrade, Wellingson S. Paiva, Hamilton Matushita, and Manoel J. Teixeira

Constipation can cause transient malfunction of the ventriculoperitoneal shunt (VPS). Patients with myelomeningocele or cerebral palsy are often diagnosed with hydrocephalus and constipation due to neurogenic bowel. These patients are more prone to VPS dysfunction, often requiring surgical revision. The authors report the case of a 6-year-old girl with a VPS that had been implanted due to hydrocephalus secondary to myelomeningocele. The patient was brought to the emergency department with intermittent headache, vomiting, constipation, and abdominal distension and pain. A CT scan revealed ventricular dilatation and radiography of the abdomen showed bowel loop distension. After a Fleet enema and digital maneuvers, her abdominal distension and symptoms improved. A CT scan obtained 24 hours later showed a reduction in ventricular size. The mechanism by which constipation can lead to VPS malfunction can be traced to indirect increases of intraabdominal pressure and direct obstruction of the catheter by distended intestinal loops. Treating constipation can restore the free circulation of the CSF and avoid surgical intervention. Careful neurological monitoring of these patients is essential, because some measures used to treat constipation can increase intracranial pressure. The objective of this report was to highlight constipation as a possible cause of transient VPS malfunction, thereby avoiding unnecessary surgical revisions, to which children with hydrocephalus are frequently submitted.

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Davi J. Fontoura Solla, Manoel Jacobsen Teixeira, and Wellingson Silva Paiva

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Weston Northam, Kristi Hildebrand, Scott Elton, and Carolyn Quinsey

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Mauricio Mandel, Carlo Emanuel Petito, Rafael Tutihashi, Wellingson Paiva, Suzana Abramovicz Mandel, Fernando Campos Gomes Pinto, Almir Ferreira de Andrade, Manoel Jacobsen Teixeira, and Eberval Gadelha Figueiredo

OBJECTIVE

Advances in video and fiber optics since the 1990s have led to the development of several commercially available high-definition neuroendoscopes. This technological improvement, however, has been surpassed by the smartphone revolution. With the increasing integration of smartphone technology into medical care, the introduction of these high-quality computerized communication devices with built-in digital cameras offers new possibilities in neuroendoscopy. The aim of this study was to investigate the usefulness of smartphone-endoscope integration in performing different types of minimally invasive neurosurgery.

METHODS

The authors present a new surgical tool that integrates a smartphone with an endoscope by use of a specially designed adapter, thus eliminating the need for the video system customarily used for endoscopy. The authors used this novel combined system to perform minimally invasive surgery on patients with various neuropathological disorders, including cavernomas, cerebral aneurysms, hydrocephalus, subdural hematomas, contusional hematomas, and spontaneous intracerebral hematomas.

RESULTS

The new endoscopic system featuring smartphone-endoscope integration was used by the authors in the minimally invasive surgical treatment of 42 patients. All procedures were successfully performed, and no complications related to the use of the new method were observed. The quality of the images obtained with the smartphone was high enough to provide adequate information to the neurosurgeons, as smartphone cameras can record images in high definition or 4K resolution. Moreover, because the smartphone screen moves along with the endoscope, surgical mobility was enhanced with the use of this method, facilitating more intuitive use. In fact, this increased mobility was identified as the greatest benefit of the use of the smartphone-endoscope system compared with the use of the neuroendoscope with the standard video set.

CONCLUSIONS

Minimally invasive approaches are the new frontier in neurosurgery, and technological innovation and integration are crucial to ongoing progress in the application of these techniques. The use of smartphones with endoscopes is a safe and efficient new method of performing endoscope-assisted neurosurgery that may increase surgeon mobility and reduce equipment costs.

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Sergio Brasil, Edson Bor-Seng-Shu, Marcelo de-Lima-Oliveira, Fabio Silvio Taccone, Gabriel Gattás, Douglas Mendes Nunes, Raphael A. Gomes de Oliveira, Bruno Martins Tomazini, Paulo Fernando Tierno, Rafael Akira Becker, Estevão Bassi, Luiz Marcelo Sá Malbouisson, Wellingson da Silva Paiva, Manoel Jacobsen Teixeira, and Ricardo de Carvalho Nogueira

OBJECTIVE

The present study was designed to answer several concerns disclosed by systematic reviews indicating no evidence to support the use of computed tomography angiography (CTA) in the diagnosis of brain death (BD). Therefore, the aim of this study was to assess the effectiveness of CTA for the diagnosis of BD and to define the optimal tomographic criteria of intracranial circulatory arrest.

METHODS

A unicenter, prospective, observational case-control study was undertaken. Comatose patients (Glasgow Coma Scale score ≤ 5), even those presenting with the first signs of BD, were included. CTA scanning of arterial and venous vasculature and transcranial Doppler (TCD) were performed. A neurological determination of BD and consequently determination of case (BD group) or control (no-BD group) was conducted. All personnel involved with assessing patients were blinded to further tests results. Accuracy of BD diagnosis determined by using CTA was calculated based on the criteria of bilateral absence of visualization of the internal cerebral veins and the distal middle cerebral arteries, the 4-point score (4PS), and an exclusive criterion of absence of deep brain venous drainage as indicated by the absence of deep venous opacification on CTA, the venous score (VS), which considers only the internal cerebral veins bilaterally.

RESULTS

A total of 106 patients were enrolled in this study; 52 patients did not have BD, and none of these patients had circulatory arrest observed by CTA or TCD (100% specificity). Of the 54 patients with a clinical diagnosis of BD, 33 met the 4PS (61.1% sensitivity), whereas 47 met the VS (87% sensitivity). The accuracy of CTA was time related, with greater accuracy when scanning was performed less than 12 hours prior to the neurological assessment, reaching 95.5% sensitivity with the VS.

CONCLUSIONS

CTA can reliably support a diagnosis of BD. The criterion of the absence of deep venous opacification, which can be assessed by use of the VS criteria investigated in this study, can confirm the occurrence of cerebral circulatory arrest.

Clinical trial registration no.: 12500913400000068 (clinicaltrials.gov)

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Benedito Jamilson Araújo Pereira, Antonio Marcondes Lerario, Paula Rodrigues Sola, Talita de Sousa Laurentino, Dipika R. Mohan, Antonio Nogueira de Almeida, Paulo Henrique Pires de Aguiar, Wellingson da Silva Paiva, Alda Wakamatsu, Manoel Jacobsen Teixeira, Sueli Mieko Oba-Shinjo, and Suely Kazue Nagahashi Marie

OBJECTIVE

The authors searched for genetic and transcriptional signatures associated with tumor progression and recurrence in their cohort of patients with meningiomas, combining the analysis of targeted exome, NF2-LOH, transcriptome, and protein expressions.

METHODS

The authors included 91 patients who underwent resection of intracranial meningioma at their institution between June 2000 and November 2007. The search of somatic mutations was performed by Next Generation Sequencing through a customized panel and multiplex ligation-dependent probe amplification for NF2 loss of heterozygosity. The transcriptomic profile was analyzed by QuantSeq 3′ mRNA-Seq. The differentially expressed genes of interest were validated at the protein level analysis by immunohistochemistry.

RESULTS

The transcriptomic analysis identified an upregulated set of genes related to metabolism and cell cycle and downregulated genes related to immune response and extracellular matrix remodeling in grade 2 (atypical) meningiomas, with a significant difference in recurrent compared with nonrecurrent cases. EZH2 nuclear positivity associated with grade 2, particularly with recurrent tumors and EZH2 gene expression level, correlated positively with the expression of genes related to cell cycle and negatively to genes related to immune response and regulation of cell motility.

CONCLUSIONS

The authors identified modules of dysregulated genes in grade 2 meningiomas related to the activation of oxidative metabolism, cell division, cell motility due to extracellular remodeling, and immune evasion that were predictive of survival and exhibited significant correlations with EZH2 expression.