Category: skull base surgery

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Postoperative Hearing Preservation in Patients Undergoing Retrosigmoid Craniotomy for Resection of Vestibular Schwannomas: A Systematic Review of 2034 Patients

Neurosurgery 2019 DOI:10.1093/neuros/nyz147

Vestibular schwannomas (VS) are benign tumors derived from Schwann cells ensheathing the vestibulocochlear nerve. The retrosigmoid (RS) surgical approach is useful to resect tumors of multiple sizes while affording the possibility of preserving postoperative hearing.

OBJECTIVE: To conduct a systematic review of published literature investigating hearing preservation rates in patients who underwent the RS approach for VS treatment.

METHODS: The PubMed, Scopus, and Embase databases were surveyed for studies that reported preoperative and postoperative hearing grades on VS patients who underwent RS treatment. Hearing preservation rates were calculated, and additional patient demographic data were extracted. Tumor size data were stratified to compare hearing preservation rates after surgery for intracanalicular, small (0-20 mm), and large (>20 mm) tumors.

RESULTS: Of 383 deduplicated articles, 26 studies (6.8%) met eligibility criteria for a total of 2034 patients with serviceable preoperative hearing, for whom postoperative hearing status was evaluated. Aggregate hearing preservation was 31% and 35% under a fixed and random effects model, respectively. A mixed effects model was used to determine hearing preservation rates depending on tumor size, which were determined to be 57%, 37%, and 12% for intracanalicular, small, and large tumors, respectively. Significant crossstudy heterogeneity was found (I2 = 93%, τ 2 = .964, P < .01; Q = 287.80, P = < .001), with rates of hearing preservation ranging from 0% to 100%.

CONCLUSION: Tumor size may have an effect on hearing preservation rates, but multiple factors should be considered. Discussion of a patient’s expectations for hearing preservation is critical when deciding on VS treatment plans.

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Surgical management of Tuberculum sellae Meningiomas: Myths, facts, and controversies

Acta Neurochirurgica (2020) 162:631–640

The optimal management of tuberculum sellae (TS) meningiomas, especially the surgical strategy, continues to be debated along with several controversies that persist.

Methods A task force was created by the EANS skull base section committee along with its members and other renowned experts in the field to generate recommendations for the surgical management of these tumors on a European perspective. To achieve this, the task force also reviewed in detail the literature in this field and had formal discussions within the group.

Results The constituted task force dealt with the practice patterns that exist with respect to pre-operative radiological investigations, ophthalmological and endocrinological assessments, optimal surgical strategies, and follow-up management.

Conclusion This article represents the consensually derived opinion of the task force with respect to the surgical treatment of tuberculum sellae meningiomas. Areas of uncertainty where further clinical research is required were identified.

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Various modifications of a vascularized nasoseptal flap for repair of extensive skull base dural defects

J Neurosurg 132:371–379, 2020

Endonasal surgery of the skull base requires watertight reconstruction of the skull base that can seal the dural defect to prevent postoperative CSF rhinorrhea and consequent intracranial complications. Although the incidence of CSF leakage has decreased significantly since the introduction in 2006 of the vascularized nasoseptal flap (the Hadad- Bassagasteguy flap), reconstruction of extensive skull base dural defects remains challenging. The authors describe a new, modified vascularized nasoseptal flap for reconstruction of extensive skull base dural defects.

METHODS A retrospective review was conducted on 39 cases from 2010 to 2017 that involved reconstruction of the skull base with an endonasal vascularized flap. Extended nasoseptal flaps were generated by adding the nasal floor and inferior meatus mucosa, inferior turbinate mucosa, or entire lateral nasal wall mucosa. The authors specifically highlight the surgical techniques for flap design and harvesting of these various modifications of the vascularized nasoseptal flap.

RESULTS Thirty-nine endonasal vascularized flaps were used to reconstruct skull base defects in 37 patients with nonsurgical or postoperative CSF rhinorrhea. Of the 39 procedures, extended nasoseptal flaps were used in 5 cases (13%). These included 2 extended nasoseptal flaps including the inferior turbinate mucosa and 3 extended nasoseptal flaps including the entire lateral nasal wall mucosa. These 5 extended nasoseptal flaps were used in patients who had nonsurgical CSF rhinorrhea due to extensive skull base destruction by invasive pituitary tumors. All flaps healed completely and sealed off the CSF leaks. Olfactory function slightly decreased in the 3 patients with extended nasoseptal flaps including the entire lateral nasal wall mucosa. One patient experienced nasolacrimal duct obstruction, which was treated by dacryocystorhinostomy. The authors encountered no wound complication in this series, while crusting at the donor site required daily nasal toilette and frequent debridement until the completion of mucosalization, which usually takes 8 to 12 weeks after surgery.

CONCLUSIONS Extended nasoseptal flaps are a reliable and versatile option that can be used to reconstruct extensive skull base dural defects resulting from destruction by large invasive tumors or complex endoscopic endonasal surgery. An extended nasoseptal flap that includes the entire lateral nasal wall mucosa (360° flap) is the largest endonasal vascularized flap reported to date and may be an alternative for the reconstruction of extensive skull base defects while avoiding the need for additional external approaches.

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Transcavernous sinus pituitary gland transposition

Acta Neurochirurgica (2019) 161:2123–2127

Pituitary transposition preserving gland function is possible when approaching superior clival region tumors. Clinical experience along with detailed anatomical knowledge makes this technique safe and effective.

Method We present a step by step description of our technique based on the most recent anatomical references to get a pituitary transposition through the different compartments of the cavernous sinus. By this technique, we achieve minor gland manipulation and a better surgical view of this area. We support this technique with an anatomical analysis on cadaveric specimens and clarifying dissection images.

Conclusions Transcavernous sinus pituitary gland transposition is an easily feasible technique and allows gland shifting preserving pituitary function.

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Endoscopic transorbital surgery for Meckel’s cave and middle cranial fossa tumors

J Neurosurg 131:1126–1135, 2019

Tumors involving Meckel’s cave remain extremely challenging because of the surrounding complex neurovascular structures and deep-seated location. The authors investigated a new minimal-access technique using the endoscopic transorbital approach (eTOA) through the superior eyelid crease to Meckel’s cave and middle cranial fossa lesions and reviewed the most useful surgical procedures and pitfalls of this approach.

METHODS Between September 2016 and January 2018, the authors performed eTOA in 9 patients with tumors involving Meckel’s cave and the middle cranial fossa. The lesions included trigeminal schwannoma in 4 patients, meningioma in 2 patients, metastatic brain tumor in 1 patient, chondrosarcoma in 1 patient, and dermoid cyst in 1 patient. In 7 of the 9 patients, eTOA alone was performed, while the other 2 patients underwent a combined eTOA and endoscopic endonasal approach or retrosigmoid craniotomy. Data including details of surgical techniques and clinical outcomes were recorded.

RESULTS Gross-total resection was performed in 7 of the 9 patients (77.8%). Four patients underwent extended eTOA (with lateral orbital rim osteotomy). Drilling of the trapezoid sphenoid floor, a middle fossa “peeling” technique, and full visualization of Meckel’s cave were applied to approach the lesions. Tumors were exposed and removed extradurally in 3 patients and intradurally in 6 patients. There was no postoperative CSF leak.

CONCLUSIONS The eTOA affords a direct route to access Meckel’s cave and middle cranial fossa lesions. With experience, this novel approach can be successfully applied to selected skull base lesions. To achieve successful removal of the tumor, emphasis should be placed on the importance of adequately removing the greater sphenoid wing and vertical crest. However, because of limited working space eTOA may not be an ideal approach for posterior fossa lesions.

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The 3F (Fat, Flap, and Flash) Technique For Skull Base Reconstruction After Endoscopic Endonasal Suprasellar Approach

World Neurosurg. (2019) 126:439-446

Endoscopic endonasal surgery is a rapidly evolving field that offers the possibility of treating a wide variety of skull base lesions. Reconstruction of the osteodural defect is of utmost importance, as an effective and watertight closure is mandatory. Over the years, various techniques and materials have been adopted in an effort of providing a resilient and reliable method of closure.

MATERIALS AND METHODS: We retrospectively analyzed data from a series of 25 patients who underwent skull base reconstruction according to a novel paradigm following endoscopic endonasal transtuberculumtransplanum approaches for tumor removal between June 2017 and June 2018. The 3F strategy involves autologous fat grafting, nasoseptal flap coverage, and flash mobilization out of the bed following the operation.

RESULTS: The 3F technique achieved successful watertight closure in 24 of 25 cases (96%). One case of postoperative cerebrospinal fluid rhinorrhea developed on postoperative day 10, which did not require reoperation. No perioperative complications related to the 3F strategy (e.g., overpacking, infections, or hematomas) occurred. No donor graft site infections or hematomas developed.

CONCLUSIONS: The 3F skull base reconstruction is a safe and effective method in achieving watertight closure after extended endoscopic endonasal approaches. Despite this study reporting a preliminary experience in a small series of patients, it seems that the 3F technique can be considered as a viable solution among the wide kaleidoscope of available skull base reconstruction methods

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Esthesioneuroblastoma: A Comprehensive Review of Diagnosis, Management, and Current Treatment Options

World Neurosurg. (2019) 126:194-211

Many controversies exist in the diagnosis and management of this aggressively malignant condition, mainly because of limited literature and lack of randomized control trials, resulting in nonstandardized treatment methods. We performed a comprehensive review of the literature to identify management approach and treatment options for esthesioneuroblastoma.

METHODS: An extensive review of the published literature was conducted in PubMed, OVID Medline, and EMBASE journals for studies of esthesioneuroblastoma. Terms for search included esthesioneuroblastoma (ENB) and olfactory neuroblastoma (ONB). No date restrictions were used.

RESULTS: The search yielded 3876 related articles. Cross-checking of articles led to exclusion of duplicate articles. The remaining 1170 articles were screened for their full text and English language availability. Of 609 full-text articles available, animal studies, irrelevant articles, and studies with mixed/ confusing data were excluded. We finalized 149 articles pertaining to the topic, including 119 original research articles, 3 book chapters, 11 reviews, 9 case reports, and 7 case series.

CONCLUSIONS: Surgical resection followed by radiotherapy is the standard for treatment for higher-grade lesions. The endoscopic endonasal approach is gaining further recognition with more favorable outcomes and better survival than for open surgery. Postoperative radiotherapy is associated with the highest overall survival and shows benefit for patients with higher-stage disease and those who receive chemotherapy. Recurrence rates after treatment vary drastically in the literature and, therefore, prolonged follow-up with repeated imaging is recommended. Lifelong surveillance is recommended because of late recurrences associated with this tumor.

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Prospective validation of a molecular prognostication panel for clival chordoma

J Neurosurg 130:1528–1537, 2019

There are currently no reliable means to predict the wide variability in behavior of clival chordoma so as to guide clinical decision-making and patient education. Furthermore, there is no method of predicting a tumor’s response to radiation therapy.

METHODS A molecular prognostication panel, consisting of fluorescence in situ hybridization (FISH) of the chromosomal loci 1p36 and 9p21, as well as immunohistochemistry for Ki-67, was prospectively evaluated in 105 clival chordoma samples from November 2007 to April 2016. The results were correlated with overall progression-free survival after surgery (PFSS), as well as progression-free survival after radiotherapy (PFSR).

RESULTS Although Ki-67 and the percentages of tumor cells with 1q25 hyperploidy, 1p36 deletions, and homozygous 9p21 deletions were all found to be predictive of PFSS and PFSR in univariate analyses, only 1p36 deletions and homozygous 9p21 deletions were shown to be independently predictive in a multivariate analysis. Using a prognostication calculator formulated by a separate multivariate Cox model, two 1p36 deletion strata (0%–15% and > 15% deleted tumor cells) and three 9p21 homozygous deletion strata (0%–3%, 4%–24%, and ≥ 25% deleted tumor cells) accounted for a range of cumulative hazard ratios of 1 to 56.1 for PFSS and 1 to 75.6 for PFSR.

CONCLUSIONS Homozygous 9p21 deletions and 1p36 deletions are independent prognostic factors in clival chordoma and can account for a wide spectrum of overall PFSS and PFSR. This panel can be used to guide management after resection of clival chordomas.

 

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Endoscopic endonasal transoculomotor triangle approach for adenomas invading the parapeduncular space

J Neurosurg 130:1304–1314, 2019

Pituitary adenomas may extend into the parapeduncular space by invading through the roof of the cavernous sinus. Currently, a transcranial approach is the preferred choice, with or without the combination of an endonasal approach. In this paper the authors present a novel surgical approach that takes advantage of the natural corridor provided by the tumor to further open the oculomotor triangle and resect tumor extension into the parapeduncular space.

METHODS Six injected specimens were used to demonstrate in detail the surgical anatomy related to the approach. Four cases in which the proposed approach was used were retrospectively reviewed.

RESULTS From a technical perspective, the first step involves accessing the superior compartment of the cavernous sinus. The interclinoid ligament should be identified and the dura forming the oculomotor triangle exposed. The oculomotor dural opening may be then extended posteriorly toward the posterior petroclinoidal ligament and inferolaterally toward the anterior petroclinoidal ligament. The oculomotor nerve should then be identified; in this series it was displaced superomedially in all 4 cases. The posterior communicating artery should also be identified to avoid its injury. In all 4 cases, the tumor invading the parapeduncular space was completely removed. There were no vascular injuries and only 1 patient had a partial oculomotor nerve palsy that completely resolved in 2 weeks.

CONCLUSIONS The endoscopic endonasal transoculomotor approach is an original alternative for removal of tumor extension into the parapeduncular space in a single procedure. The surgical corridor is increased by opening the dura of the oculomotor triangle and by working below and lateral to the cisternal segment of the oculomotor nerve.

 

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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.

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Endoscopic endonasal approach to primitive Meckel’s cave tumors

Acta Neurochirurgica (2018) 160:2349–2361

Recently, an alternative endoscopic endonasal approach to Meckel’s cave (MC) tumors has been proposed. To date, few studies have evaluated the results of this route. The aim of our study was to evaluate long-term surgical and clinical outcome associated with this technique in a cohort of patients with intrinsic MC tumors.

Methods All patients with MC tumors treated at out institution by endoscopic endonasal approach (EEA) between 2002 and 2016 were included. Patients underwent brain MRI, CT-angiography, and neurological evaluation before surgery. Complications were considered based on the surgical records. All examinations were repeated after 3 and 12 months, then annually. The median follow-up was of 44.1 months (range 16–210).

Results The series included 8 patients (4 F): 5 neuromas, 1 meningioma, 1 chondrosarcoma, and 1 epidermoid cyst. The median age at treatment was 54.5 years (range 21–70). Three tumors presented with a posterior fossa extension. Radical removal of the MC portion of the tumor was achieved in 7 out of 8 cases. Two patients developed a permanent and transitory deficit of the sixth cranial nerve, respectively. No tumor recurrence was observed at follow-up.

Conclusion In this preliminary series, the EEA appeared an effective and safe approach to MC tumors. The technique could be advantageous to treat tumors located in the antero-medial aspects of MC displacing the trigeminal structures posteriorly and laterally. A favorable index of an adequate working space for this approach is represented by the ICA medialization, while tumor extension to the posterior fossa represents the main limitation to radical removal of this route.

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Endoscopic transorbital superior eyelid approach: anatomical study from a neurosurgical perspective

J Neurosurg 129:1203–1216, 2017

Recent studies have proposed the superior eyelid endoscopic transorbital approach as a new minimally invasive route to access orbital lesions, mostly in otolaryngology and maxillofacial surgeries. The authors undertook this anatomical study in order to contribute a neurosurgical perspective, exploring the anterior and middle cranial fossa areas through this purely endoscopic transorbital trajectory.

METHODS Anatomical dissections were performed in 10 human cadaveric heads (20 sides) using 0° and 30° endoscopes. A step-by-step description of the superior eyelid transorbital endoscopic route and surgically oriented classification are provided.

RESULTS The authors’ cadaveric prosection of this approach defined 3 modular routes that could be combined. Two corridors using bone removal lateral to the superior and inferior orbital fissures exposed the middle and anterior cranial fossa (lateral orbital corridors to the anterior and middle cranial base) to unveil the temporal pole region, lateral wall of the cavernous sinus, middle cranial fossa floor, and frontobasal area (i.e., orbital and recti gyri of the frontal lobe). Combined, these 2 corridors exposed the lateral aspect of the lesser sphenoid wing with the Sylvian region (combined lateral orbital corridor to the anterior and middle cranial fossa, with lesser sphenoid wing removal). The medial corridor, with extension of bone removal medially to the superior and inferior orbital fissure, afforded exposure of the opticocarotid area (medial orbital corridor to the opticocarotid area).

CONCLUSIONS Along with its minimally invasive nature, the superior eyelid transorbital approach allows good visualization and manipulation of anatomical structures mainly located in the anterior and middle cranial fossae (i.e., lateral to the superior and inferior orbital fissures). The visualization and management of the opticocarotid region medial to the superior orbital fissure are more complex. Further studies are needed to prove clinical applications of this relatively novel surgical pathway.

 

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Surgical outcome in smaller symptomatic vestibular schwannomas. Is there a role for surgery?

Acta Neurochirurgica (2018) 160:2263–2275

Currently, there is no consensus in the initial management of small vestibular schwannomas (VSs). They are routinely watched and/or referred for radiosurgical treatment, although surgical removal is also an option. We hereby evaluate clinical outcomes of patients who have undergone surgical removal of smaller symptomatic VSs.

Methods Patients with vestibular schwannomas (grade T1–T3b according to Hannover classification) were reviewed. Patients with symptomatic tumors who underwent surgery were evaluated. Their preoperative hearing status was based on the guideline of the committee on hearing and equilibriumof the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) foundation. Their postoperative facial nerve function, hearing status, vestibular symptoms, and degree of tumor resection were assessed.

Results Thirty patients were selected for surgery via a retrosigmoid approach based on their age, symptoms, and their own decision-making after discussion of management options. Most patients presented with hearing loss. Seventeen patients had useful hearing preoperatively. Among them, 10 patients (59%) preserved useful hearing (class A or B) postoperatively. MRI at 1-year follow-up confirmed complete resection in 26/29 patients. Also, 29 patients (97%) had HB grade I–II, and 1 patient had HB III at 1-year follow-up. Except for 1 patient with CSF leak, 1 patient with delayed facial nerve palsy, and 2 patients with asymptomatic sigmoid sinus occlusion, there were no other new morbidities.

Conclusion Although both observation and radiosurgery are valid options in the management of smaller size vestibular schwannomas, surgical treatment seems to offer a high rate of facial nerve preservation, a reasonable rate of hearing sparing, and a high total resection rate. Clinicians should consider surgical treatment as a valid option in the initial management of symptomatic small vestibular schwannomas in younger patients.

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Decision Making in theWait-and-Scan Approach for Vestibular Schwannomas: Is There a Price to Pay in Terms of Hearing, Facial Nerve, and Overall Outcomes?

Neurosurgery 83:858–870, 2018

The wait-and-scan modality has emerged as an important strategy in the management of vestibular schwannoma (VS) as it has been demonstrated that many tumors grow slowly or do not show any growth over long periods.

OBJECTIVE: To analyze long-term outcomes of wait-and-scan in the treatment of patients with VS, discuss the factors contributing to the decision making, determine the inherent risks of the policy, and compare our results with literature.

METHODS: In total, 576 patients with sporadic unilateral VS who were managed with wait-and-scan were reviewed retrospectively. Of these, a subset of 154 patients with 5-yr follow-up was separately analyzed. The tumor characteristics including patterns of growth, rate of growth, hearing outcomes, and likely factors affecting the above parameters were analyzed.

RESULTS: The mean period of follow-up was 36.9 ± 30.2 mo. The mean age was 59.2 ± 11.6 yr. Thirteen different patterns of tumor growth were observed. Eighty-four (54.5%) of 154 tumors with 5-yr follow-up showed no growth throughout 5 yr. Fifty-six (36.4%) tumors showedmixed growth rates. Only 57 (37%) patients had serviceable hearing at the start of follow-up, but 32 (56.1%) maintained it at the end of follow-up. One hundred fifty (26%) of the 576 patients who failed wait-and-scan had to be taken up for surgery.

CONCLUSION: While there may be no price to pay in wait-and-scan as far as hearing is concerned, this may not be the case for facial nerve outcomes, wherein the results may be better if the patients are taken earlier for surgery.

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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.

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Pediatric Clival Chordoma: A Curable Disease that Conforms to Collins’ Law

Neurosurgery 82:652–660, 2018

Skull base chordomas in children are extremely rare. Their course, management, and outcome have not been defined.

OBJECTIVE: To describe the preeminent clinical and radiological features in a series of pediatric patients with skull base chordomas and analyze the outcome of a cohort who underwent uniform treatment. We emphasize predictors of overall survival and progression-free survival, which aligns with Collins’ law for embryonal tumors.

METHODS: Thirty-one patients with a mean age of 10.7 yr (range 0.8-22) harboring skull base chordomas were evaluated. We retrospectively analyzed the outcomes and prognostic factors for 18 patients treated by the senior author, with uniform management of surgery with the aim of gross total resection and adjuvant proton-beam radiotherapy. Mean follow-up was 119.2 mo (range 8-263).

RESULTS: Abducens nerve palsy was the most common presenting symptom. Imaging disclosed large tumors that often involve multiple anatomical compartments. Patients undergoing gross total resection had significantly increased progression-free survival (P = .02) and overall survival (P = .05) compared with those having subtotal resection. Those who lived through the period of risk for recurrence without disease progression had a higher probability of living entirely free of progression (P = .03; odds ratio = 16.0). Age, sex, and histopathological variant did not yield statistical significance in survival.

CONCLUSION: Long-term overall and progression-free survival in children harboring skull base chordomas can be achieved with gross surgical resection and proton-beam radiotherapy, despite an advanced stage at presentation. Collins’ law does apply to pediatric skull base chordomas, and children with this disease have a high hope for cure.

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Clival chordomas: considerations after 16 years of endoscopic endonasal surgery

J Neurosurg 128:329–338, 2018

In the past decade, the role of the endoscopic endonasal approach (EEA) has relevantly evolved for skull base tumors. In this study, the authors review their surgical experience with using an EEA in the treatment of clival chordomas, which are deep and infiltrative skull base lesions, and they highlight the advantages and limitations of this ventral approach.

METHODS All consecutive cases of chordoma treated with an EEA between 1998 and 2015 at a single institution are included in this study. Preoperative assessment consisted of neuroimaging (MRI and CT with angiography sequences) and endocrinological, neurological, and ophthalmological evaluations, which were repeated 3 months after surgery and annually thereafter. Postoperative adjuvant therapies were considered.

RESULTS Sixty-five patients (male/female ratio 1:0.9) were included in this study. The median age was 48 years (range 9–80 years). Gross-total resection (GTR) was achieved in 47 cases (58.7%). On univariate analysis, primary procedures (p = 0.001), location in the superior or middle third of the clivus (p = 0.043), extradural location (p = 0.035), and histology of conventional chordomas (p = 0.013) were associated with a higher rate of GTR. The complication rate was 15.1%, and there were no perioperative deaths. Most complications did not result in permanent sequelae and included 2 CSF leaks (2.5%), 5 transient cranial nerve VI palsies (6.2%), and 2 internal carotid artery injuries (2.5%), which were treated with coil occlusion of the internal carotid artery without neurological deficits. Three patients (3.8%) presented with complications resulting in permanent neurological deficits due to a postoperative hematoma (1.2%) causing a hemiparesis, and 2 permanent ophthalmoplegias (2.5%). Seventeen patients (26.2%) have died of tumor progression over the course of follow-up (median 52 months, range 7–159 months). Based on Kaplan-Meier analysis, the survival rate was 77% at 5 years and 57% at 10 years. On multivariate analysis, the extent of tumor removal (p = 0.001) and the absence of previous treatments (p = 0.001) proved to be correlated with a longer survival rate.

CONCLUSIONS The EEA was associated with a high rate of tumor removal and symptom control, with low morbidity and preservation of a good quality of life. These results allow for a satisfactory overall survival rate, particularly after GTR and for primary surgery. Considering these results, the authors believe that an EEA can be a helpful tool in chordoma surgery, achieving a good balance between as much tumor removal as possible and the preservation of an acceptable patient quality of life.

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Transcondylar approach for resection of lateral medullary cavernous malformation

Acta Neurochir (2018) 160:291–294

Resection of a medullary cavernous malformation requires aggressive exposure, but there is controversy on how much occipital condyle can be safely removed during the transcondylar approach.

Method We describe and demonstrate the use of the transcondylar approach to a medullary cavernous malformation, with emphasis on adequate surgical exposure while preserving the atlanto-occipital joint.

Conclusions Despite conservative handling of the occipital condyle, craniocervical stability may vary in patients after transcondylar surgery. A “dynamic” computer tomography, with views of the atlanto-occipital joint at each end-rotational extreme, may be the best postoperative assessment tool to evaluate the stability of the craniocervical junction.

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Endoscopic endonasal versus transcranial approach to tuberculum sellae and planum sphenoidale meningiomas

J Neurosurg 128:40–48, 2018

Planum sphenoidale (PS) and tuberculum sellae (TS) meningiomas cause visual symptoms due to compression of the optic chiasm. The treatment of choice is surgical removal with the goal of improving vision and achieving complete tumor removal. Two options exist to remove these tumors: the transcranial approach (TCA) and the endonasal endoscopic approach (EEA). Significant controversy exists regarding which approach provides the best results and whether there is a subset of patients for whom an EEA may be more suitable. Comparisons using a similar cohort of patients, namely, those suitable for gross-total resection with EEA, are lacking from the literature.

METHODS The authors reviewed all cases of PS and TS meningiomas that were surgically removed at Weill Cornell Medical College between 2000 and 2015 (TCA) and 2008 and 2015 (EEA). All cases were shown to a panel of 3 neurosurgeons to find only those tumors that could be removed equally well either through an EEA or TCA to standardize both groups. Volumetric measurements of preoperative and postoperative tumor size, FLAIR images, and apparent diffusion coefficient maps were assessed by 2 independent reviewers and compared to assess extent of resection and trauma to the surrounding brain. Visual outcome and complications were also compared.

RESULTS Thirty-two patients were identified who underwent either EEA (n = 17) or TCA (n = 15). The preoperative tumor size was comparable (mean 5.58 ± 3.42 vs 5.04 ± 3.38 cm3 [± SD], p = 0.661). The average extent of resection achieved was not significantly different between the 2 groups (98.80% ± 3.32% vs 95.13% ± 11.69%, p = 0.206). Postoperatively, the TCA group demonstrated a significant increase in the FLAIR/edema signal compared with EEA patients (4.15 ± 7.10 vs -0.69 ± 2.73 cm3, p = 0.014). In addition, the postoperative diffusion-weighted imaging signal of cytotoxic ischemic damage was significantly higher in the TCA group than in the EEA group (1.88 ± 1.96 vs 0.40 ± 0.55 cm3, p = 0.008). Overall, significantly more EEA patients experienced improved or stable visual outcomes compared with TCA patients (93% vs 56%, p = 0.049). Visual deterioration was greater after TCA than EEA (44% vs 0%, p = 0.012). While more patients experienced postoperative seizures after TCA than after EEA (27% vs 0%, p = 0.038), there was a trend toward more CSF leakage and anosmia after EEA than after TCA (11.8% vs 0%, p = 0.486 and 11.8% vs 0%, p = 0.118, respectively).

CONCLUSIONS In this small single-institution study of similarly sized and located PS and TS meningiomas, EEA provided equivalent rates of resection with better visual results, less trauma to the brain, and fewer seizures. These preliminary results merit further investigation in a larger multiinstitutional study and may support EEA resection by experienced surgeons in a subset of carefully selected PS and TS meningiomas.

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Clinical features and surgical outcomes of patients with skull base chordoma

J Neurosurg 127:1257–1267, 2017

Skull base chordoma is relatively rare, and a limited number of reports have been published regarding its clinical features. Moreover, the factors associated with extent of resection, as well as the value of marginal resection for long-term survival, are still in question for this disease. The objective of this study was to investigate these factors by evaluating their clinical features and surgical outcomes.

METHODS A retrospective analysis of 238 patients with skull base chordomas, who met the inclusion criteria, was performed. This study summarized the clinical features, selection of approaches, degree of resection, and postoperative complications by statistical description analyses; proposed modified classifications of tumor location and bone invasion; studied the contributions of the clinical and radiological factors to the extent of resection by Pearson c2, ANOVA, rank test, and binary logistic regression analysis; and estimated the differences in overall survival and progression-free survival rates with respect to therapeutic history, classification of tumor location, extent of bone invasion, and extent of tumor resection by the Kaplan-Meier method. A p value < 0.05 was considered statistically significant.

RESULTS The study included 140 male and 98 female patients with a mean age of 38.1 years. Headache and neck pain (33.2%) and diplopia (29%) were the most common initial symptoms. Sphenoclival type accounted for the largest proportion of tumor location (59.2%); endophytic chordoma was the more common type of bone invasion (81.5%). Lateral open approaches were performed in two-thirds of the study population (78.6%). The rate of marginal resection was 66%, composed of gross-total resection (11.8%) and near-total resection (54.2%). Meningitis (8%) and CSF leakage (3.8%) were the most frequent complications. The mean follow-up period was 43.7 months. The overall survival and progression-free survival rates at 5 years were 76% and 45%, respectively. Recurrent tumor and larger tumor volume (≥ 40 cm3) were identified as risk factors of marginal resection. Patients who presented with recurrent tumor and underwent intralesional resection had a worse long-term outcome.

CONCLUSIONS The classifications of both tumor location and bone invasion demonstrated clinical value. Marginal resection was more likely to be achieved for primary lesions with smaller volumes (< 40 cm3). The rate of CSF leakage declined due to improved dura mater repair with free fat grafts. Marginal resection, or gross-total resection when possible, should be performed in patients with primary chordomas to achieve better long-term survival.

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