Endovascular versus surgical treatment of cranial dural arteriovenous fistulas: a single‑center 8‑year experience

Acta Neurochirurgica (2022) 164:151–161

Cranial dural arteriovenous fistulas (dAVFs) are rare lesions managed mainly with endovascular treatment (EVT) and/or surgery. We hypothesize that there may be subtypes of dAVFs responding better to a specific treatment modality in terms of successful obliteration and cessation of symptoms and/or risks.

Methods All dAVFs treated during 2011–2018 at our hospital were analyzed retrospectively. Presenting symptoms, radiological variables, treatment modality, complications, and residual symptoms were related to dAVF type using the original Djindjian classification.

Results We treated 112 dAVFs in 107 patients (71, 66% males). They presented with hemorrhage (n = 23; 21%), nonhemorrhagic symptoms (n = 75; 70%), or were discovered incidentally (n = 9; 8%). There were 25 (22%) type I, 29 (26%) type II, 26 (23%) type III, and 32 (29%) type IV fistulas. EVT was the primary treatment modality in 72/112 (64%) dAVFs whereas 40/112 (36%) underwent primary surgery with angiographic obliteration rates of 60% and 90%, respectively. Using a secondary treatment modality in 23 dAVFs, we obtained a final obliteration rate of 93%, including all type III/IV and 26/27 (96%) type II dAVFs. Except for headache, residual symptoms were rare and minor. Permanent neurological complications consisted of five cranial nerve deficits.

Conclusions We recommend EVT as first treatment modality in types I, II, and in non-hemorrhagic type III/IV dAVFs. We recommend surgery as first treatment choice in acute hemorrhagic dAVFs and as secondary choice in type III/IV dAVFs not successfully occluded by EVT. Combining the two modalities provides obliteration in 9/10 dAVF cases at a low procedural risk.

Evolution and Impact of the Brain Trauma Foundation Guidelines

Neurosurgery 89:1148–1156, 2021

The Brain Trauma Foundation (BTF) Guidelines for the Management of Severe Head Injury were the first clinical practice guidelines published by any surgical specialty. These guidelines have earned a reputation for rigor and have been widely adopted around the world. Implementation of these guidelines has been associated with a 50% reduction in mortality and reduced costs of patient care.

Over their 25-yr history the traumatic brain injury (TBI) guidelines have been expanded, refined, and made increasingly more rigorous in conjunction with new clinical evidence and evolving methodologic standards.

Here, we discuss the history and accomplishments of BTF guidelines for TBI as well as their limitations. We also discuss planned changes to future TBI guidelines intended to increase their utility and positive impact in an evolving medical landscape. Perhaps the greatest limitation of TBI guidelines now is the lack of high-quality clinical research as well as novel diagnostics and treatments with which to generate substantially new recommendations.

Efficacy and safety of gamma knife radiosurgery for posterior cranial fossa meningioma

Neurosurgical Review (2020) 43:1089–1099

The management of posterior cranial fossa meningioma [PCFM] is challenging and many neurosurgeons advise gamma knife radiosurgery [GKRS] as a modality for its upfront or adjuvant treatment. Due to the varying radiosurgical response based on lesion location, tumor biology, and radiation dosage, we performed a pioneer attempt in doing a systematic review analyzing the treatment efficacy and safety profile of GKRS for PCFM based on current literature.

A systematic review was performed according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses [PRISMA] guidelines. A thorough literature search was conducted on PubMed, Web of science, and Cochrane data base; articles were selected systematically based on PRISMA protocol, reviewed completely, and relevant data was summarized and discussed.

A total of 18 publications pertaining to GKRS for PCFM were included with a pooled sample size of 2131 patients. The median pre-GKRS tumor volume ranged from 2.28 to 10.5 cm [3]. Primary GKRS was administered in 61.1% of the pooled study cohorts, adjuvant treatment in 32.9%, and salvage therapy in 6.5% patients. Majority of the meningiomas were WHO grade 1 tumors (99.7%). The pooled mean marginal dose in the studies was 13.6 Gy (range 12–15.2 Gy) while the mean of maximum doses was 28.6 Gy (range 25–35 Gy). Most studies report an excellent radiosurgical outcome including the tumor control rate and the progression-free survival [PFS] of over 90%. The tumor control, PFS, and adverse radiation effect [ARE] rates in author’s series were 92.3%, 91%, and 9.6%, respectively. The favorable radiosurgical outcome depends on multiple factors such as small tumor volume, absence of previous radiotherapy, tumor location, elderly patients, female gender, longer time from symptom onset, and decreasing maximal dose.

GKRS as primary or adjuvant treatment modality needs to be considered as a promising management strategy for PCFM in selected patients in view of the growing evidence of high tumor control rate, improved neurological functions, and low incidence of ARE. The use of multiple isocenters, 3-D image planning, and limit GKRS treatment to tumors less than 3.5 cm help to avoid complications and achieve the best results. The treatment decisions in PCFM cases must be tailored and should consider the factors such as radiological profile, symptom severity, performance level, and patient preference for a good outcome.

Risk of First Hemorrhage of Brain Arteriovenous Malformations During Pregnancy

Neurosurgery 85:E806–E814, 2019

Recommendations on the management of brain arteriovenous malformations (bAVM) with respect to pregnancy are based upon conflicting literature.

OBJECTIVE: To systematically review the reported risk and annualized rate of first intracranial hemorrhage (ICH) from bAVM during pregnancy and puerperium.

METHODS: MEDLINE, EMBASE, and Scopus databases were searched for relevant articles in English published before April 2018. Studies providing a quantitative risk of ICH in bAVM during pregnancy were eligible.

RESULTS: From 7 initially eligible studies, 3 studies met the criteria for providing quanti- tative risk of first ICH bAVM during pregnancy. Data from 47 bAVM ICH during pregnancy across 4 cohorts were extracted for analysis. Due to differences in methodology and defini- tions of exposure period, it was not appropriate to combine the cases. The annualized risk of first ICH during pregnancy for these 4 cohorts was 3.0% (95% confidence interval [CI]: 1.7- 5.2%); 3.5% (95% CI: 2.4-4.5%); 8.6% (95% CI: 1.8-25%); and 30% (95% CI: 18-49%). Only the last result from the last cohort could be considered significantly increased in comparison with the nonpregnant period (relative rate 6.8, 95% CI: 3.6-13). The limited number of eligible studies and variability in results highlighted the need for enhanced rigor of future research.

CONCLUSION: There is no conclusive evidence of an increased risk of first hemorrhage during pregnancy from bAVM. Because advice to women with bAVM may influence the management of pregnancy or bAVM with significant consequences, we believe that a retrospective multicenter, case crossover study is urgently required.

Riskier-than-expected occlusive treatment of ruptured posterior communicating artery aneurysms

J Neurosurg 131:1269–1277, 2019

Occlusive treatment of posterior communicating artery (PCoA) aneurysms has been seen as a fairly uncomplicated procedure. The objective here was to determine the radiological and clinical outcome of patients after PCoA aneurysm rupture and treatment and to evaluate the risk factors for impaired outcome.

METHODS In a retrospective clinical follow-up study, data were collected from 620 consecutive patients who had been treated for ruptured PCoA aneurysms at a single center between 1980 and 2014. The follow-up was a minimum of 1 year after treatment or until death.

RESULTS Of the 620 patients, 83% were treated with microsurgical clipping, 8% with endovascular coiling, 2% with the two procedures combined, 1% with indirect surgical methods, and 6% with conservative methods. The most common procedural complications were treatment-related brain infarctions (15%). The occurrence of artery occlusions (10% microsurgical, 8% endovascular) was higher than expected. Most patients made a good recovery at 1 year after aneurysmal subarachnoid hemorrhage (modified Rankin Scale [mRS] score 0–2: 386 patients [62%]). A fairly small proportion of patients were left severely disabled (mRS score 4–5: 27 patients [4%]). Among all patients, 20% died during the 1st year. Independent risk factors for an unfavorable outcome, according to the multivariable analysis, were poor preoperative clinical condition, intracerebral or subdural hematoma due to aneurysm rupture, age over 65 years, artery occlusion on postoperative angiography, occlusive treatment–related ischemia, delayed cerebral vasospasm, and hydrocephalus requiring a shunt.

CONCLUSIONS Even though most patients made a good recovery after PCoA aneurysm rupture and treatment during the 1st year, the occlusive treatment–related complications were higher than expected and caused morbidity even among initially good-grade patients. Occlusive treatment of ruptured PCoA aneurysms seems to be a high-risk procedure, even in a high-volume neurovascular center.

Natural history of cavernous sinus meningiomas

J Neurosurg 130:435–442, 2019

Meningiomas confined to the cavernous sinus (MCSs) are benign tumors. Due to the high risk of severe complications, the intracavernous surgical procedure was abandoned in favor of radiotherapy. However, the choice of treatment remains complicated due to the fact that the natural history of this lesion has not yet been described.

METHODS The authors studied the natural history of this lesion using a prospective series of 53 consecutive patients suffering from MCSs. The median follow-up duration was 10.2 years (range 2–25 years), from 1990 to 2016.

RESULTS Patients ranged in age from 30 to 72 years (mean 53 years). The meningiomas were diagnosed by major symptoms (mainly oculomotor palsy and neuralgia experienced in 28 patients), minor symptoms (headache, intermittent diplopia in 15 patients), or incidental findings (10 patients). Simple symptomatic treatment (short courses of cortico- steroids and carbamazepine) allowed patients to become asymptomatic in 19 (67.9%) of 28 cases experiencing major symptoms, and for 12 (80%) of 15 patients with initial minor symptoms (p < 0.0001). All patients with incidental findings remained asymptomatic. Forty four (83%) of 53 MCSs did not show any significant growth and 42 (80%) of 53 patients were not symptomatic at the end of follow-up (p < 0.001). The radiographic progression-free survival rates (± SD) at 5, 10, and 20 years were 90% ± 4.2%, 82% ± 5.7%, and 70% ± 10.2%, respectively. Five patients (9.4%) with no evidence of any effect of the initial medical treatment desired additional conventional radiation therapy.

CONCLUSIONS Because of the capricious, unpredictable, and slow growth of MCSs, together with high growth variability from one patient to the next, the symptomatic medical treatment of these tumors is a highly effective method. This series shows that these lesions are naturally, clinically, and radiologically indolent.

Cranial Chordoma: A New Preoperative Grading System

Neurosurgery 83:403–415, 2018

Chordomas are rare but challenging neoplasms involving the skull base. A preoperative grading system will be useful to identify both areas for treatment and risk factors, and correlate to the degree of resection, complications, and recurrence.

OBJECTIVE: To propose a new grading system for cranial chordomas designed by the senior author. Its purpose is to enable comparison of different tumors with a similar pathology to clivus chordoma, and statistically correlate with postoperative outcomes.

METHODS: The numerical grading system included tumor size, site of the tumor, vascular encasement, intradural extension, brainstem invasion, and recurrence of the tumor either after surgery or radiotherapy with a range of 2 to 25 points; it was used in 42 patients with cranial chordoma. The grading system was correlated with number of operations for resection, degree of resection, number and type of complications, recurrence, and survival.

RESULTS: We found 3 groups: low-risk 0 to 7 points, intermediate-risk 8 to 12 points, and high-risk≥13 points in the grading system. The 3 groupswere correlated with the following: extent of resection (partial, subtotal, or complete; P < .002); number of operative stages to achieve removal (P < .014); tumor recurrence (P = .03); postoperative Karnofsky Performance Status (P < .001); and with successful outcome (P = .005). The grading system itself correlated with the outcome (P = .005).

CONCLUSION: The proposed chordoma grading system can help surgeons to predict the difficulty of the case and know which areas of the skull base will need attention to plan further therapy.

Cushing’s disease: pathobiology, diagnosis, and management

J Neurosurg 126:404–417, 2017

Cushing’s disease (CD) is the result of excess secretion of adrenocorticotropic hormone (ACTH) by a benign monoclonal pituitary adenoma. The excessive secretion of ACTH stimulates secretion of cortisol by the adrenal glands, resulting in supraphysiological levels of circulating cortisol.
The pathophysiological levels of cortisol are associated with hypertension, diabetes, obesity, and early death. Successful resection of the CD-associated ACTH-secreting pituitary adenoma is the treatment of choice and results in immediate biochemical remission with preservation of pituitary function.
Accurate and early identification of CD is critical for effective surgical management and optimal prognosis. The authors review the current pathophysiological principles, diagnostic methods, and management of CD.

Hydrocephalus: cerebral aquaporin-4 and computational modeling

Aquaporin-4 channels

Neurosurg Focus 41 (3):E8, 2016

Aquaporin-4 (AQP4) channels play an important role in brain water homeostasis. Water transport across plasma membranes has a critical role in brain water exchange of the normal and the diseased brain. AQP4 channels are implicated in the pathophysiology of hydrocephalus, a disease of water imbalance that leads to CSF accumulation in the ventricular system. Many molecular aspects of fluid exchange during hydrocephalus have yet to be firmly elucidated, but review of the literature suggests that modulation of AQP4 channel activity is a potentially attractive future pharmaceutical therapy. Drug therapy targeting AQP channels may enable control over water exchange to remove excess CSF through a molecular intervention instead of by mechanical shunting.

This article is a review of a vast body of literature on the current understanding of AQP4 channels in relation to hydrocephalus, details regarding molecular aspects of AQP4 channels, possible drug development strategies, and limitations. Advances in medical imaging and computational modeling of CSF dynamics in the setting of hydrocephalus are summarized.

Algorithmic developments in computational modeling continue to deepen the understanding of the hydrocephalus disease process and display promising potential benefit as a tool for physicians to evaluate patients with hydrocephalus.

Outcome of conservative and surgical treatment of pyogenic spondylodiscitis

pyogenic spondylodiscitis

Eur Spine J (2016) 25:983–999

Spondylodiscitis is a spinal infection affecting primarily the intervertebral disk and the adjacent vertebral bodies. Currently many aspects of the treatment of pyogenic spondylodiscitis are still a matter of debate.

Purpose The aim of this study was to review the currently available literature systematically to determine the outcome of patients with pyogenic spondylodiscitis for conservative and surgical treatment strategies.

Methods A systematic electronic search of MEDLINE, EMBASE, Cochrane Collaboration, and Web of Science regarding the treatment of pyogenic spondylodiscitis was performed. Included articles were assessed on risk of bias according the Cochrane Handbook for Systematic Reviews of Interventions, and the quality of evidence and strength of recommendation was evaluated according the GRADE approach.

Results: 25 studies were included. Five studies had a high or moderate quality of evidence. One RCT suggest that 6 weeks of antibiotic treatment of pyogenic spondylodiscitis results in a similar outcome when compared to longer treatment duration. However, microorganism-specific studies suggest that at least 8 weeks of treatment is required for S. aureus and 8 weeks of Daptomycin for MRSA. The articles that described the outcome of surgical treatment strategies show that a large variety of surgical techniques can successfully treat spondylodiscitis. No additional long-term beneficial effect of surgical treatment could be shown in the studies comparing surgical versus antibiotic only treatment.

Conclusion There is a strong level of recommendation for 6 weeks of antibiotic treatment in pyogenic spondylodiscitis although this has only been shown by one recent RCT. If surgical treatment is indicated, it has been suggested by two prospective studies with strong level of recommendation that an isolated anterior approach could result in a better clinical outcome.

International differences in the management of intracranial aneurysms: implications for the education of the next generation of neurosurgeons

MCA aneurysm

Acta Neurochir 2015, 157,(9):1467-1475

The publication of the International Subarachnoid Aneurysm Trial rapidly changed the management of patients with subarachnoid hemorrhage. The present and perceived future trends of aneurysm management have significant implications for patients and how we educate future cerebrovascular specialists.

Objective

To determine present perceived competencies of final-year neurosurgical residents who have just finished their residencies and to relate those to what practitioners from a variety of continents expect of these persons. The goal is to provide a basis for further discussion regarding the design of further educational programs in neurosurgery.

Methods

A 55-item questionnaire with 33 questions related to competencies and expectations of competency from final-year residents who have just finished residency was completed by 229 neurosurgeons and neuro-radiologists (81 % response rate) of mixed seniority from 45 countries. We used bivariate and descriptive analyses to determine future trends and geographic differences in cerebral aneurysm management as well as the educational implications on the future.

Results

More North Americans than those from the rest of the world are of the opinion that graduating residents are presently competent to perform basic cerebrovascular procedures like evacuation of a hematoma and clipping a simple 7-mm middle cerebral artery aneurysm. Extremely few graduating neurosurgical residents anywhere are presently capable of performing endovascular techniques for even the most basic of aneurysms. Most of those surveyed also believe that endovascular and open surgical management of aneurysms should be a part of residency training for all residents (70.4 and 88.7 %, respectively).

Conclusions

Our findings have implications for the design of neurosurgical curricula for residents as well as for certification examinations and procedures. Specialty and educational organizations and those responsible for the education of future clinicians who will care for patients with cerebrovascular problems should adjust educational objectives and implement curricula and learning experiences that will ensure that cerebrovascular specialists are capable of providing the best care possible to the patient with an aneurysm, whether that be open surgery or endovascular management. These findings mean that organizations around the world will need to make these adjustments to the education of future specialists.

The role of cancer stem cells in glioblastoma

The role of cancer stem cells in glioblastoma

Neurosurg Focus 37 (6):E6, 2014

Recurrence in glioblastoma is nearly universal, and its prognosis remains dismal despite significant advances in treatment over the past decade. Glioblastoma demonstrates considerable intratumoral phenotypic and molecular heterogeneity and contains a population of cancer stem cells that contributes to tumor propagation, maintenance, and treatment resistance. Cancer stem cells are functionally defined by their ability to self-renew and to differentiate, and they constitute the diverse hierarchy of cells composing a tumor. When xenografted into an appropriate host, they are capable of tumorigenesis.

Given the critical role of cancer stem cells in the pathogenesis of glioblastoma, research into their molecular and phenotypic characteristics is a therapeutic priority.

In this review, the authors discuss the evolution of the cancer stem cell model of tumorigenesis and describe the specific role of cancer stem cells in the pathogenesis of glioblastoma and their molecular and microenvironmental characteristics. They also discuss recent clinical investigations into targeted therapies against cancer stem cells in the treatment of glioblastoma.

Perianeurysmal edema as a predictive sign of aneurysmal rupture

Brain edema in aneurysm

J Neurosurg 121:1112–1114, 2014

Subarachnoid hemorrhage following intracranial aneurysmal rupture is a major cause of morbidity and mortality. Several factors may affect the probability of rupture, such as tobacco and alcohol use; size, shape, and location of the aneurysm; presence of intraluminal thrombus; and even the sex of the patient. However, few data correlate such findings with the timing of aneurysmal rupture.

The authors report 2 cases of middle-age women with headache and MRI findings of incidental aneurysms. Magnetic resonance imaging showed evidence of surrounding parenchymal edema, and in one case there was a clear increase in edema during follow-up, suggesting a progressive inflammatory process that culminated with rupture.

These findings raise the possibility that bleb formation and an enlargement of a cerebral aneurysm might be associated with an inflammatory reaction of the aneurysm wall resulting in perianeurysmal edema and subsequent aneurysmal rupture. There may be a temporal link between higher degree of edema and higher risk for rupture, including risk for immediate rupture.

Robot-Assisted Stereotactic Laser Ablation in Medically Intractable Epilepsy

Robot_Assisted_Stereotactic_Laser_Ablation_in

Operative Neurosurgery 10:167–173, 2014

Stereotactic laser ablation offers an advantage over open surgical procedures for treatment of epileptic foci, tumors, and other brain pathology. Robotassisted stereotactic laser ablation could offer an accurate, efficient, minimally invasive, and safe method for placement of an ablation catheter into the target.

OBJECTIVE: To determine the feasibility of placement of a stereotactic laser ablation catheter into a brain lesion with the use of robotic assistance, via a safe, accurate, efficient, and minimally invasive manner.

METHODS: A laser ablation catheter (Visualase, Inc) was placed by using robotic guidance (ROSA, Medtech Surgical, Inc) under general anesthesia into a localized epileptogenic periventricular heterotopic lesion in a 19-year-old woman with 10-year refractory focal seizure history. The laser applicator (1.65 mm diameter) position was confirmed by using magnetic resonance imaging (MRI). Ablation using the Visualase system was performed under multiplanar imaging with real-time thermal imaging and treatment estimates in each plane. A postablation MRI sequence (T1 postgadolinium contrast injection) was used to immediately confirm the ablation. RESULTS: MRI showed accurate skin entry point and trajectory, with the applicator advanced to the lesion’s distal boundary. Ablation was accomplished in less than 3 minutes of heating. The overall procedure, from time of skin incision to end of last ablation, was approximately 90 minutes. After confirmation of proper lesioning by using a T1 contrast-enhanced MRI, the applicator was removed, and the incision was closed using a single stitch. No hemorrhage or other untoward complication was visualized. The patient awoke without any complication, was observed overnight after admitting to a regular floor bed, and was discharged to home the following day.

CONCLUSION: This technique, using a combination of Visualase laser ablation, ROSA robot, and intraoperative MRI, facilitated a safe, efficacious, efficient, and minimally invasive approach that could be used for placement of 1 or multiple electrodes in the future.

Low-pressure hydrocephalus: indication for custom-made catheters?

Low-pressure hydrocephalus- indication for custom-made catheters?

Acta Neurochir (2013) 155:1981–1985

Low-pressure hydrocephalus (LPH) is characterized by ventriculomegaly with persistent low intracranial pressure (ICP). Sub-zero drainage is needed for its management and multiple solutions have been described. Our aim is to report our experience with custom-made peritoneal catheters with larger inner diameter as an alternative treatment option.

Methods We made a retrospective review of all patients diagnosed with LPH and treated with custom-made peritoneal catheters at the Virgen del Rocío Pediatric Neurosurgical Unit. Catheters were coated with antibiotic or silver. The inner diameter of ventricular catheters was 1.4 mm; peritoneal catheters were larger than usual (1.9 mm inner diameter).

Results We identified four patients in whom five custommade peritoneal catheters were used over a 3-year period. There were two males and the mean age was 10 years (6 months–17 years). In all patients, placement of an EVD was necessary for sub-zero drainage, with maximum negative pressure of −8 cm H20. The mean time of maintenance of EVD was 102 days (10 days–1 year). Finally, three ventriculoperitoneal (VP) valveless systems, one with antigravitation device, and one Pro-GAV VP shunt were placed, all of them with larger custom-made peritoneal catheters. After a mean follow-up period of 2.3 years (6 months– 3 years), two patients are completely recovered, one patient is partially dependent for daily activities with good cognitive status, and the last one is a child who died due to his brain tumor.

Conclusion The custom-made peritoneal catheters with larger inner diameter could be a good option for the management of this complex pathology.

Surgical treatment of tentorial dural arteriovenous fistulae located around the tentorial incisura

Surgical treatment of tentorial dural arteriovenous fistulae

Neurosurg Rev (2013) 36:429–435

Tentorial dural arteriovenous fistulae (DAVF) are relatively uncommon and are the most dangerous type of DAVF. Because of a high incidence of hemorrhage and subsequent neurological deficits, treatment is mandatory.

A consecutive series of nine surgically treated patients with symptomatic tentorial DAVF were analyzed in this study. All lesions were located around the tentorial incisura and were treated microsurgically using a subtemporal approach in eight cases and a supracerebellar approach in one case. The dural bases of the lesions were located adjacent to the tentorial edge in six patients and the tentorial apex in three patients.

Complete obliteration was achieved in all treated tentorial DAVF. In one patient, the torcular fistula remained untreated without cortical venous reflux. Postoperative asymptomatic temporal lobe hemorrhage was diagnosed in one patient with a tentorial apex DAVF; however, no new neurological symptoms were present after surgical treatment.

The subtemporal approach for unilateral tentorial DAVF is a favorable and direct approach for the highly skilled surgeon. Perimesencephalic venous dilatation or varix is an important finding onMRI to help localize tentorial DAVF in the tentorial edge or apex.

Growing skull fracture stages and treatment strategy

J Neurosurg Pediatrics 9:670–675, 2012. (http://thejns.org/doi/abs/10.3171/2012.2.PEDS11538) 

A growing skull fracture (GSF) is a rare but significant late complication of skull fractures, usually occurring during infancy and early childhood. Delayed diagnosis and improper treatment could exacerbate this disease. The aim of this study was to introduce a new hypothesis about, describe the stages of, and discuss the treatment strategy for GSF.

Methods. The authors performed a retrospective review of 27 patients with GSF, who were grouped according to 3 different GSF stages.

Results. Over a period of 20 years, 27 patients with GSF (16 males and 11 females) were treated in the authors’ department. The mean follow-up period was 26.5 months. Six patients were in the prephase of GSF (Stage 1), 10 patients in the early phase (Stage 2), and 11 in the late phase (Stage 3). All patients underwent duraplasty. All 6 patients at Stage 1 and 5 patients at Stage 2 underwent craniotomy without cranioplasty. Five patients at Stage 2 and all of the patients at Stage 3 underwent cranioplasty with autologous bone and alloplastic materials, respectively. Among all patients, 5 underwent ventriculoperitoneal shunt placement. Symptoms in all patients at Stages 1 and 2 were alleviated or disappeared, and the cranial bones developed without deformity during follow-up. Among patients with Stage 3 GSF, no obvious improvement in neurological deficits was observed. Three patients underwent additional operations because of cranial deformation or infection.

Conclusions. The authors identify the stages of GSF according to a new hypothesis. They conclude that accurately diagnosing and treating GSF during Stages 1 and 2 leads to a better prognosis.

The molecular biology and novel treatments of vestibular schwannomas

J Neurosurg 115:906–914, 2011. DOI: 10.3171/2011.6.JNS11131

Vestibular schwannomas are histopathologically benign tumors arising from the Schwann cell sheath surrounding the vestibular branch of cranial nerve VIII and are related to the NF2 gene and its product merlin.

Merlin acts as a tumor suppressor and as a mediator of contact inhibition. Thus, deficiencies in both NF2 genes lead to vestibular schwannoma development.

Recently, there have been major advances in our knowledge of the molecular biology of vestibular schwannomas as well as the development of novel therapies for its treatment.

In this article the authors comprehensively review the recent advances in the molecular biology and characterization of vestibular schwannomas as well as the development of modern treatments for vestibular schwannoma. For instance, merlin is involved with a number of receptors including the CD44 receptor, EGFR, and signaling pathways, such as the Ras/raf pathway and the canonical Wnt pathway. Recently, merlin was also shown to interact in the nucleus with E3 ubiquitin ligase CRL4DCAF1.

A greater understanding of the molecular mechanisms behind vestibular schwannoma tumorigenesis has begun to yield novel therapies. Some authors have shown that Avastin induces regression of progressive schwannomas by over 40% and improves hearing. An inhibitor of VEGF synthesis, PTC299, is currently in Phase II trials as a potential agent to treat vestibular schwannoma.

Furthermore, in vitro studies have shown that trastuzumab (an ERBB2 inhibitor) reduces vestibular schwannoma cell proliferation.

With further research it may be possible to significantly reduce morbidity and mortality rates by decreasing tumor burden, tumor volume, hearing loss, and cranial nerve deficits seen in vestibular schwannomas.

Treatment policy for petroclival meningioma based on tumor size: aiming radical removal in small tumors for obtaining cure without morbidity

Neurosurg Rev (2011) 34:327–335. DOI 10.1007/s10143-011-0308-7

Aggressive tumor removal is not always the best treatment for petroclival meningioma (PCM). However, radical removal actually provides the cure with minimal morbidity.

We evaluated the relation of surgical results and tumor size in the PCM removal to clarify the treatment policy for PCM. This study comprised 32 consecutive patients with newly-diagnosed PCM who underwent tumor removal; tumor size was small (< 3 cm) in 12 patients and large (≥3 cm) in 20. Tumor removal was classified into radical (Simpson’s grade I/II) and non-radical (Simpson’s grade III/IV). Removal of small PCM was 11 radical and one non-radical; no surgical morbidity/mortality occurred and postoperative regular follow-up using magnetic resonance imaging showed no recurrence in the period of 66± 45 months. Removal of large PCM was eight radical and 12 non-radical; despite no mortality, the incidence of permanent cranial nerve deficits and major neurological deficits newly developed postoperatively was 35% and 25%, respectively. Radical removal was significantly more frequent in small PCMs than in large PCMs. Permanent cranial nerve deficits newly developed postoperatively and poor outcome (Karnofsky score ≤80) were significantly more frequent in large PCMs than in small PCMs. Radical removal of small PCM is achieved with minimal morbidity and results in the cure. Notwithstanding high morbidity, aggressive removal of large PCM does not achieve a high rate of radical removal.

To find and remove PCM radically while it is small is the only way to cure the disease with minimal morbidity.

Guidelines for the Management of Spontaneous Intracerebral Hemorrhage

Stroke. 2010;41:2108-2129. DOI: 10.1161/STR.0b013e3181ec611b

Purpose—The aim of this guideline is to present current and comprehensive recommendations for the diagnosis and treatment of acute spontaneous intracerebral hemorrhage.

Methods—A formal literature search of MEDLINE was performed. Data were synthesized with the use of evidence tables. Writing committee members met by teleconference to discuss data-derived recommendations. The American Heart Association Stroke Council’s Levels of Evidence grading algorithm was used to grade each recommendation. Prerelease review of the draft guideline was performed by 6 expert peer reviewers and by the members of the Stroke Council Scientific Statements Oversight Committee and Stroke Council Leadership Committee. It is intended that this guideline be fully updated in 3 years’ time.

Results—Evidence-based guidelines are presented for the care of patients presenting with intracerebral hemorrhage. The focus was subdivided into diagnosis, hemostasis, blood pressure management, inpatient and nursing management, preventing medical comorbidities, surgical treatment, outcome prediction, rehabilitation, prevention of recurrence, and future considerations.

Conclusions—Intracerebral hemorrhage is a serious medical condition for which outcome can be impacted by early, aggressive care. The guidelines offer a framework for goal-directed treatment of the patient with intracerebral hemorrhage.

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