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Alexander G. Weil and Sanjiv Bhatia

Ventrally-located intramedullary cervical spinal cord cavernomas are rare entities in the pediatric population. Surgical access to these lesions is challenging. The authors present the complete resection of a symptomatic ventral cervical intramedullary cavernoma through an anterior approach in a 15-year-old boy. The lesion was accessed following left anterolateral dissection, C3–4 discectomy and C3/C4 partial corpectomy. The authors will discuss the rationale for intervening in this patient and for selecting this anterior approach over other approaches, such as the anterolateral, posterolateral or posterior approach. The steps, pitfalls and pearls of this surgical approach will be demonstrated in a detailed video.

The video can be found here: http://youtu.be/-ARTp6g13hgs.

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Anna Lo Presti, Alexander G. Weil and John Ragheb

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Alexander G. Weil, Aria Fallah, Parthasarathi Chamiraju, John Ragheb and Sanjiv Bhatia

OBJECT

Combining endoscopic third ventriculostomy (ETV) with choroid plexus cauterization (CPC) has been shown to improve the success rate compared with ETV alone in infants (less than 24 months) with hydrocephalus who were treated in developing countries. The authors sought to evaluate the safety and efficacy of this procedure, using a rigid neuroendoscope, in a single North American center, and to assess whether the ETV success score (ETVSS), the CURE Children’s Hospital of Uganda ETVSS (CCHU ETVSS), and other pre- and intraoperative variables could predict success.

METHODS

The authors performed a retrospective review of consecutive ETV/CPC procedures performed using a rigid neuroendoscope to treat infantile hydrocephalus. The infants underwent the procedure at Miami Children’s Hospital between January 2007 and 2014, with at least one postoperative follow-up. Duration of follow-up or time to failure of ETV/CPC, the primary outcome measure, was documented. A repeat CSF diversion procedure or death was considered as a failure of ETV/CPC. The time to event was measured using a Kaplan-Meier analysis. The authors analyzed ETVSS, CCHU ETVSS, and pre- and intraoperative variables to determine their suitability to predict success.

RESULTS

Eighty-five patients (45 boys) with a mean age of 4.3 months (range 1 day to 20 months) underwent ETV/CPC. Etiology included intraventricular hemorrhage of prematurity in 44 patients (51.7%), myelomeningocele (MMC) in 7 (8.2%), congenital aqueductal stenosis in 12 (14.1%), congenital communicating hydrocephalus in 6 (7.1%), Dandy-Walker complex in 6 (7.1%), postinfectious hydrocephalus in 5 (5.8%), and other cause in 5 (5.8%). Six procedure-related complications occurred in 5 (5.8%) patients, including 2 hygromas, 1 CSF leak, and 3 infections. There were 3 mortalities in this cohort. ETV/CPC was successful in 42.1%, 37.7%, and 36.8% of patients at 6, 12, and 24 months follow-up, respectively. The median (95% CI) time to ETV/CPC failure was 4.0 months (0.9–7.1 months). In univariate analyses, both the ETVSS (hazard ratio [HR] 1.03; 95% CI 1.01–1.05; p = 0.004) and CCHU ETVSS (HR 1.48; 95% CI 1.04–2.09; p = .028) were predictive of outcome following ETV/CPC. In multivariate analysis, the presence of prepontine scarring was associated with ETV/CPC failure (HR 0.34; 95% CI 0.19–0.63; p < 0.001). Other variables, such as radiological criteria (prepontine interval, prepontine space, aqueductal stenosis, Third Ventricular Morphology Index) and intraoperative findings (ventriculostomy pulsations, extent of CPC), did not predict outcome.

CONCLUSIONS

ETV/CPC is a feasible alternative to ETV and ventriculoperitoneal shunt in infants with hydrocephalus. Both the ETVSS and CCHU ETVSS predicted success following ETV/CPC in this single-center North American cohort of patients.

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Alexander G. Weil, Andrew L. Middleton, Toba N. Niazi, John Ragheb and Sanjiv Bhatia

OBJECT

Operations on tumors of the posteromedial temporal (PMT) region, that is, on those arising from the posterior parahippocampal, fusiform, and lingual gyri, are challenging to perform because of the deep-seated location of these tumors between critical cisternal neurovascular structures and the adjacent temporal and occipital cortexes. Traditional surgical approaches require temporal or occipital transgression, retraction, or venous sacrifice. These approaches may result in unintended complications that should be avoided. To avoid these complications, the supracerebellar-transtentorial (SCTT) approach to this region has been used as an effective alternative treatment in adult patients. The SCTT approach uses a sitting position that offers a direct route to the posterior fusiform and lingual gyri of the temporal lobe. The authors report the feasibility, safety, and efficacy of this approach, using a modified lateral park-bench position in a small cohort of pediatric patients.

METHODS

The authors carried out a retrospective case review of 5 consecutive patients undergoing a paramedian SCTT approach between 2009 and 2014 at the authors' institution.

RESULTS

The SCTT approach in the park-bench position was used in 3 boys and 2 girls with a mean age of 7.8 years (range 13 months to 16 years). All patients presented with a seizure disorder related to a tumor in a PMT region involving the parahippocampal and fusiform gyri of the left (n = 3) or right (n = 2) temporal lobe. No procedure-related complications were observed. Gross-total resection and control of seizures were achieved in all cases. Tumor classes and types included 1 Grade II astrocytoma, 1 pleomorphic xanthoastrocytoma, 1 ganglioglioma, and 2 glioneural tumors. None of the tumors had recurred by the mean follow-up of 22 months (range 1–48 months). Outcomes of epileptic seizures were excellent, with seizure symptoms in all 5 patients scoring in Engel Class IA.

CONCLUSIONS

The SCTT approach represents a viable option when resecting tumors in this region, providing a reasonable working corridor and low morbidity. The authors' experience in a cohort of pediatric patients demonstrates that complete resection of the lesions in this location is feasible and is safe when involving an approach that involves using a park-bench lateral positioning.

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Walter J. Jermakowicz, Alexander G. Weil, Artyom Vlasenko, Sanjiv Bhatia and Toba N. Niazi

Cognard Type V dural arteriovenous fistulas (dAVFs) are a unique type of cranial vascular malformation characterized by congestion of the perimedullary venous system that may lead to devastating spinal cord pathology if left untreated. The authors present the first known case of a pediatric patient diagnosed with a Type V dAVF. A 14-year-old girl presented with a 3-week history of slowly progressive unilateral leg weakness that quickly progressed to bilateral leg paralysis, sphincter dysfunction, and complete sensory loss the day of her presentation. MRI revealed an extensive T2 signal change in the cervical spine and tortuous perimedullary veins along the entire length of the cord. An emergency cranial angiogram showed a Type V dAVF fed by the posterior meningeal artery with drainage into the perimedullary veins of the cervical spine. The fistula was not amenable to embolization because vascular access was difficult; therefore, the patient underwent urgent suboccipital craniotomy and ligation of the arterialized venous drainage from the fistula. The patient’s clinical course immediately reversed; she had a complete recovery over the course of a year, and she remains asymptomatic at the 2-year follow-up. This report adds to a growing body of evidence that describes the diverse and unpredictable nature of Type V dAVFs and highlights the need to obtain a cranial angiogram in pediatric patients with unexplained myelopathy and cervical cord T2 signal change on MRI.

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Alexander G. Weil, Thomas Robert, Sultan Alsaiari, Sami Obaid and Michel W. Bojanowski

Retrochiasmatic craniopharyngiomas involving the anterior third ventricle are challenging to access. Although the pterional approach is a common route for suprasellar lesions, when the craniopharyngioma extends behind the chiasma into the third ventricle, access is even more difficult, and the lamina terminalis may offer a good working window. The translamina terminalis approach provides direct access to the retrochiasmatic portion of the tumor with minimal brain retraction and no manipulation of the visual nerves. In this video, we emphasize the utility of using the lamina terminalis corridor to resect the retrochiasmatic intraventricular portion of a craniopharyngioma.

The video can be found here: https://youtu.be/hrLNC0hDKe4.

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Pierre-Olivier Champagne, Florence Cayouette, Anne Lortie, Jean-Claude Decarie and Alexander G. Weil

The occipital and marginal sinuses, when present, must be sacrificed in order to open the dura in most posterior fossa surgeries in the pediatric population, including posterior fossa decompression for Type I Chiari malformation (CM-I) with duraplasty. Apart from the immediate risk of hemorrhage, the voluntary occlusion of this structure is almost universally well tolerated. The authors report a case of intracranial hypertension following the sacrifice of occipital and marginal sinuses following posterior fossa decompression with duraplasty for CM-I. The specific draining pattern variant of the occipital and marginal sinuses leading to this complication as well as avoidance and management strategies of this condition are discussed.

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Adil Harroud, Alexander G. Weil, Jean Turgeon, Claude Mercier and Louis Crevier

OBJECT

A major challenge in sagittal craniosynostosis surgery is the high transfusion rate (50%–100%) related to blood loss in small pediatric patients. Several approaches have been proposed to prevent packed red blood cell (PRBC) transfusion, including endoscopic surgery, erythropoietin ortranexamic acid administration, and preoperative hemodilution. The authors hypothesized that a significant proportion of postoperative anemia observed in pediatric patients is actually dilutional. Consequently, since 2005, at CHU Sainte-Justine, furosemide has been administered to correct the volemic status and prevent PRBC transfusion. The purpose of this study was to evaluate the impact of postoperative furosemide administration on PRBC transfusion rates.

METHODS

This was a retrospective study of 96 consecutive patients with sagittal synostosis who underwent surgery at CHU Sainte-Justine between January 2000 and May 2012. The mean age at surgery was 4.9 ± 1.5 months (range 2.8–8.7 months). Patients who had surgery before 2005 constituted the control group. Those who had surgery in 2005 or 2006 were considered part of an implementation phase because furosemide administration was not routine. Patients who had surgery after 2006 were part of the experimental (or furosemide) group. Transfusion rates among the 3 groups were compared. The impact of furosemide administration on transfusion requirement was also measured while accounting for other variables of interest in a multiple logistic regression model.

RESULTS

The total transfusion rate was significantly reduced in the furosemide group compared with the control group (31.3% vs 62.5%, respectively; p = 0.009), mirroring the decrease in the postoperative transfusion rate between the groups (18.3% vs 50.0%, respectively; p = 0.003). The postoperative transfusion threshold remained similar throughout the study (mean hemoglobin 56.0 g/dl vs 60.9 g/dl for control and furosemide groups, respectively; p = 0.085). The proportion of nontransfused patients with recorded hemoglobin below 70 g/dl did not differ between the control and furosemide groups (41.7% vs 28.6%, respectively; p = 0.489). Surgical procedure, preoperative hemoglobin level, estimated blood loss, and furosemide administration significantly affected the risk of receiving a postoperative PRBC transfusion. When these variables were analyzed in a multiple logistic regression model, furosemide administration remained strongly associated with a reduced risk of being exposed to a blood transfusion (OR 0.196, p = 0.005). There were no complications related to furosemide administration.

CONCLUSIONS

A significant part of the postoperative anemia observed in patients who underwent sagittal craniosynostosis surgery was due to hypervolemic hemodilution. Correction of the volemic status with furosemide administration significantly reduces postoperative PRBC transfusion requirements in these patients.

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Andre Nzokou, Alexander G. Weil and Daniel Shedid

Object

Resection of spinal tumors traditionally requires bilateral subperiosteal muscle stripping, extensive laminectomy, and, in cases of foraminal extension, partial or radical facetectomy. Fusion is often warranted in cases of facetectomy to prevent deformity, pain, and neurological deterioration. Recent reports have demonstrated safety and efficacy of mini-open removal of these tumors using expandable tubular retractors. The authors report their experience with the minimally invasive removal of extradural foraminal and intradural-extramedullary tumors using the nonexpandable tubular retractor.

Methods

A retrospective chart review of consecutive patients who underwent minimally invasive resection of spinal tumors at Notre Dame Hospital was performed.

Results

Between December 2005 and March 2012, 13 patients underwent minimally invasive removal of spinal tumors at Notre Dame Hospital, Montreal. There were 6 men and 7 women with a mean age of 55 years (range 20–80 years). There were 2 lumbar and 2 thoracic intradural-extramedullary tumors and 7 thoracic and 2 lumbar extradural foraminal tumors. Gross-total resection was achieved in 12 patients. Subtotal resection (90%) was attained in 1 patient because the tumor capsule was adherent to the diaphragm. The average duration of surgery was 189 minutes (range 75–540 minutes), and the average blood loss was 219 ml (range 25–500 ml). There were no major procedure-related complications. Pathological analysis revealed benign schwannoma in 8 patients and meningioma, metastasis, plasmacytoma, osteoid osteoma, and hemangiopericytoma in 1 patient each. The average equivalent dose of postoperative narcotics after surgery was 66.3 mg of morphine. The average length of hospitalization was 66 hours (range 24–144 hours). All working patients returned to normal activities within 4 weeks. The average MRI and clinical follow-up were 13 and 21 months, respectively (range 2–68 months). At last follow-up, 92% of patients had improvement or resolution of pain with a visual analog scale score that improved from 7.8 to 1.2. All patients with neurological impairment improved. The American Spinal Injury Association grade improved in all but 1 patient.

Conclusions

Intradural-extramedullary and extradural tumors can be completely and safely resected through a minimally invasive approach using the nonexpandable tubular retractor. This approach may be associated with even less tissue destruction than mini-open techniques, translating into a quicker functional recovery. In cases of foraminal tumors, by eliminating the need for facetectomy, this minimally invasive approach may decrease the incidence of postoperative deformity and eliminate the need for adjunctive fusion surgery.

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Alain Bouthillier, Alexander G. Weil, Laurence Martineau, Laurent Létourneau-Guillon and Dang Khoa Nguyen

OBJECTIVE

Operculoinsular cortectomy (also termed operculoinsulectomy) is increasingly recognized as a therapeutic option for perisylvian refractory epilepsy. However, most neurosurgeons are reluctant to perform the technique because of previously experienced or feared neurological complications. The goal of this study was to quantify the incidence of basic neurological complications (loss of primary nonneuropsychological functions) associated with operculoinsular cortectomies for refractory epilepsy, and to identify factors predicting these complications.

METHODS

Clinical, imaging, and surgical data of all patients investigated and surgically treated by our team for refractory epilepsy requiring an operculoinsular cortectomy were retrospectively reviewed. Patients with tumors and encephalitis were excluded. Logistic regression analysis was used for uni- and multivariate statistical analyses.

RESULTS

Forty-four operculoinsular cortectomies were performed in 43 patients. Although postoperative neurological deficits were frequent (54.5% of procedures), only 3 procedures were associated with a permanent significant neurological deficit. Out of the 3 permanent deficits, only 1 (2.3%; a sensorimotor hemisyndrome) was related to the technique of operculoinsular cortectomy (injury to a middle cerebral artery branch), while the other 2 (arm hypoesthesia and hemianopia) were attributed to cortical resection beyond the operculoinsular area. With multivariate analysis, a postoperative neurological deficit was associated with preoperative insular hypometabolism on PET scan. Postoperative motor deficit (29.6% of procedures) was correlated with fewer years of neurosurgical experience and frontal operculectomies, but not with corona radiata ischemic lesions. Ischemic lesions in the posterior two-thirds of the corona radiata (40.9% of procedures) were associated with parietal operculectomies, but not with posterior insulectomies.

CONCLUSIONS

Operculoinsular cortectomy for refractory epilepsy is a relatively safe therapeutic option but temporary neurological deficits after surgery are frequent. This study highlights the role of frontal/parietal opercula resections in postoperative complications. Corona radiata ischemic lesions are not clearly related to motor deficits. There were no obvious permanent neurological consequences of losing a part of an epileptic insula, including on the dominant side for language. A low complication rate can be achieved if the following conditions are met: 1) microsurgical technique is applied to spare cortical branches of the middle cerebral artery; 2) the resection of an opercula is done only if the opercula is part of the epileptic focus; and 3) the neurosurgeon involved has proper training and experience.