Fred Gentili

✓ A multicenter, randomized placebo-controlled double-blind trial of nimodipine in poor-grade aneurysm patients was carried out in 17 Canadian hospitals. Of 188 patients enrolled in the trial, 32 were excluded for protocol violations and two were excluded due to statistical considerations, leaving 154 patients for valid outcome analysis. Nimodipine treatment was associated with a significantly better outcome (p < 0.001): 21 (29.2%) of 72 nimodipine-treated patients had a good outcome at 3 months after subarachnoid hemorrhage (SAH) compared to eight (9.8%) of 82 placebo-treated patients. Delayed ischemic deficits from vasospasm alone were significantly less frequent in the nimodipine group (p < 0.05) with permanent deficits occurring in five nimodipine-treated patients (6.9%) and in 22 placebo-treated patients (26.8%). Improvement in the good outcome rate and reduction in delayed ischemic deficits from vasospasm alone occurred in both Grade 3 and 4 patients, with no difference between nimodipine- and placebo-treated patients being found in Grade 5 patients. Repeat angiography after Day 4 was carried out in 124 patients. There was no significant difference in the incidence of moderate or severe diffuse spasm, which was seen in 64.3% of nimodipine-treated patients and 66.2% of placebo-treated patients. The authors conclude that nimodipine treatment in poor-grade patients with SAH results in an increase in the number of good outcomes and a reduction in the incidence of delayed neurological deterioration due to vasospasm. This effect occurs by a mechanism other than prevention of large-vessel spasm as visualized on angiography.

The aim of this study was to report the results of a consecutive series of patients undergoing pituitary surgery using a pure endoscopic endonasal approach and to evaluate the efficacy and safety of this procedure.We reviewed 200 consecutive patients with pituitary adenoma who underwent purely endoscopic transsphenoidal resection of their lesions. The patients' clinical outcomes, including remission rates, degrees of tumor removal, and complications, were evaluated and compared with a previous microscopic series.There were 111 nonfunctioning adenomas and 34 growth hormone-secreting, 27 adrenocorticotropin hormone-secreting, 25 prolactin-secreting, and 3 thyroid-stimulating hormone-secreting adenomas. The degree of gross total removal for tumors with suprasellar or parasellar extension and without cavernous sinus involvement was 96% and for intrasellar lesions was 98%. After a median follow-up period of 19 months, the remission results for patients with functioning adenomas were 71% for growth hormone-secreting, 81% for adrenocorticotropin hormone-secreting, and 88% for prolactin-secreting adenomas, with no recurrence at the time of the last follow-up. This compares with similar results reported from series using a standard microsurgical approach (growth hormone-secreting adenomas, 67%; adrenocorticotropin hormone-secreting adenomas, 78%; and prolactin-secreting adenomas, 62%). Endoscopic surgery for recurrent or residual nonfunctioning adenomas that had been previously treated using a microscopic approach revealed in the majority of cases a more limited exposure during the initial surgery, frequently with incomplete tumor removal. Complication rates have been low, and the average length of hospital stay was reduced.A purely endoscopic approach for pituitary adenoma treatment is a safe and effective alternative to the traditional microscopic procedure. Although our results reveal excellent tumor-removal rates, comparable remission rates in functioning tumors, and a very low rate of complications, additional studies with longer follow-up periods are required to confirm whether this approach should be considered the preferred procedure for pituitary surgery.

The traditional boundaries of the transsphenoidal approach can be expanded to include the region from the cribriform plate of the anterior cranial fossa to the foramen magnum in the anteroposterior plane. The introduction of endoscopy to transsphenoidal surgery, with its improved illumination and wider field of view, has added significant further potential for the resection of a variety of cranial base lesions. We review our experience with the expanded endoscopic endonasal approach in a series of 22 patients with anterior cranial base and supradiaphragmatic lesions.From June 2005 to June 2007, the expanded endoscopic endonasal approach was used in 22 patients with the following pathologies: 6 craniopharyngiomas; 4 esthesioneuroblastomas; 3 giant pituitary macroadenomas; 2 suprasellar Rathke's pouch cysts; 2 angiofibromas; and 1 each of suprasellar meningioma, germinoma, ethmoidal carcinoma, adenoid cystic carcinoma, and large suprasellar arachnoid cyst. This study specifically focused on the surgical indications and approaches to these lesions and the surgical results, complications, and limitations associated with this technique.Gross total tumor removal, as assessed by postoperative magnetic resonance imaging, was possible in the majority of patients (73%), with the exception of the craniopharyngioma group, in which only 1 lesion was completely removed. There were no permanent neurological complications except for increased visual disturbance in 1 patient. Other complications included cerebrospinal fluid fistulae in 4 patients (18%) and meningitis in 1 patient (5%). There was no operative mortality. Large lesions, significant lateral extension, encasement of neurovascular structures, and brain invasion in malignant lesions are considered some of the contraindications for this technique.The expanded endoscopic endonasal approach is a promising minimally invasive alternative to open transcranial approaches for selective lesions of the midline anterior cranial base. The avoidance of craniotomy and brain retraction and reduced neurovascular manipulation with less morbidity are potential advantages. Major complications have been few, but there are also limitations with this technique. This approach should be included in the armamentarium of cranial base surgeons and considered as an option in the management of selected patients with these complex pathologies.

Object While the use of endoscopic approaches has become increasingly accepted in the resection of pituitary adenomas, limited evidence exists regarding the success of this technique for patients with large and giant pituitary adenomas. This study reviews the outcomes of a large cohort of patients with large and giant pituitary adenomas who underwent endoscopic endonasal transsphenoidal surgery at the authors' institution and focuses on identifying factors that can predict extent of resection and hence aid in developing guidelines and indications for the use of endoscopic endonasal transsphenoidal surgery versus open craniotomy approaches to large and giant pituitary adenomas. Methods The authors reviewed 487 patients who underwent endoscopic endonasal transsphenoidal resection of sellar masses. From this group, 73 consecutive patients with large and giant pituitary adenomas (defined as maximum diameter ≥ 3 cm and tumor volume ≥ 10 cm 3 ) who underwent endoscopic endonasal transsphenoidal surgery between January 1, 2006, and June 6, 2012, were included in the study. Clinical presentation, radiological studies, laboratory investigations, tumor pathology data, clinical outcomes, extent of resection measured by volumetric analysis, and complications were analyzed. Results The mean preoperative tumor diameter in this series was 4.1 cm and the volume was 18 cm 3 . The average resection rate was 82.9%, corresponding with a mean residual volume of 3 cm 3 . Gross-total resection was achieved in 16 patients (24%), near-total in 11 (17%), subtotal in 24 (36%), and partial in 15 (23%). Seventy-three percent of patients experienced improvement in visual acuity, while 24% were unchanged. Visual fields were improved in 61.8% and unchanged in 5.5%. Overall, 27 patients (37%) experienced a total of 32 complications. The most common complications were sinusitis (14%) and CSF leak (10%). Six patients underwent subsequent radiation therapy because of aggressive tumor histopathology. No deaths occurred in this cohort of patients. Statistically significant predictors of extent of resection included highest Knosp grade (p = 0.001), preoperative tumor volume (p = 0.025), preoperative maximum tumor diameter (p = 0.002), hemorrhagic component (p = 0.027), posterior extension (p = 0.001), and sphenoid sinus invasion (p = 0.005). Conclusions Endoscopic endonasal transsphenoidal surgery is an effective treatment method for patients with large and giant pituitary adenomas, which results in high (> 80%) rates of resection and improvement in visual function. It is not associated with high rates of major complications and is safe when performed by experienced surgeons. The preoperative Knosp grade, tumor volume, tumor diameter, hemorrhagic components on MRI, posterior extension, and sphenoid sinus invasion may allow a prediction of extent of resection and in these patients a staged operation may be required to maximize extent of resection.

Endoscopic skull-base surgery (ESBS) is employed in the management of diverse skull-base pathologies. Paralleling the increased utilization of ESBS, the literature in this field has expanded rapidly. However, the rarity of these diseases, the inherent challenges of surgical studies, and the continued learning curve in ESBS have resulted in significant variability in the quality of the literature. To consolidate and critically appraise the available literature, experts in skull-base surgery have produced the International Consensus Statement on Endoscopic Skull-Base Surgery (ICAR:ESBS).Using previously described methodology, topics spanning the breadth of ESBS were identified and assigned a literature review, evidence-based review or evidence-based review with recommendations format. Subsequently, each topic was written and then reviewed by skull-base surgeons in both neurosurgery and otolaryngology. Following this iterative review process, the ICAR:ESBS document was synthesized and reviewed by all authors for consensus.The ICAR:ESBS document addresses the role of ESBS in primary cerebrospinal fluid (CSF) rhinorrhea, intradural tumors, benign skull-base and orbital pathology, sinonasal malignancies, and clival lesions. Additionally, specific challenges in ESBS including endoscopic reconstruction and complication management were evaluated.A critical review of the literature in ESBS demonstrates at least the equivalency of ESBS with alternative approaches in pathologies such as CSF rhinorrhea and pituitary adenoma as well as improved reconstructive techniques in reducing CSF leaks. Evidence-based recommendations are limited in other pathologies and these significant knowledge gaps call upon the skull-base community to embrace these opportunities and collaboratively address these shortcomings.

We have compared our local, pedicled, and free-flap reconstructions for 90 skull base defects performed over the past 10 years. The pericranial flap was found to provide a reliable dural seal. Free-flap reconstructions exhibited a significantly higher incidence of uncomplicated primary wound healing (95 versus 62.5 percent) and a much lower incidence of flap loss (0 percent), cerebrospinal fluid leak (5 percent), meningitis, and abscess (0 percent) when compared with defects reconstructed with pedicled myocutaneous flaps. We conclude that microvascular free-tissue transfer is the safest, most economical procedure when faced with moderate to large composite defects of the cranial base.

✓ Somatosensory evoked potentials have been recorded during 34 operations for intracranial aneurysm. The central conduction time (CCT), the time between the N 14 peak (recorded at C-2) and the N 20 peak (recorded at the cortex) in response to median nerve stimulation, has been found to be increased by administration of halothane, by brain retraction, and by temporary vascular occlusion in some instances. Increase of CCT to more than 10 msec, or disappearance of the response was associated in two cases with postoperative neurological deficit, neither permanent. In a further case, prolongation of CCT was used as a guide to the repositioning of an imperfectly placed clip on a middle cerebral artery aneurysm. The value of the technique as a perioperative monitoring system is discussed.

Mackinnon, Susan E. M.D., F.R.C.S.(C); Hudson, A. R. M.B., F.R.C.S.(C); Gentili, F. M.D., F.R.C.S.(C); Kline, D. G. M.D., F.A.C.S.; T., Hunter R. Author Information

We report our recent experience with an expanded purely endoscopic endonasal approach for the treatment of clival chordomas.Twelve patients underwent an expanded endoscopic approach for excision of cranial base chordomas at Toronto Western Hospital. Two patients had undergone a previous craniotomy for excision of a significant lateral intracranial extension of the tumor. All other patients had mainly centrally located lesions. Three patients had recurrent tumors. This study focused on the surgical approach, results, and complications associated with this approach.Diplopia caused by VIth nerve palsy was the most common presenting symptom and was observed in seven patients. Gross total resection of the tumor was achieved in seven patients (58%). Four patients had complete recovery of their preoperative diplopia. One patient (8%) presented with new hemiparesis postoperatively. Four patients (33%) had a cerebrospinal fluid leak postoperatively; two were treated by lumbar drainage, and two required a secondary surgical repair. All newly diagnosed patients underwent adjuvant radiotherapy. There was no mortality. The short-term outcome was excellent in all but one patient. No recurrence was observed at the median follow-up period of 16 months.The expanded endoscopic endonasal approach is a valid minimally invasive alternative for the treatment of centrally located clival chordomas or as an adjunct for the central part of chordomas with lateral extension. The early results of this technique indicate at least equivalency to more extensive open approaches, and its versatility may widen the horizon of surgical management of these aggressive lesions. The challenge with the cerebrospinal fluid leakage is being addressed with novel local flap repair techniques. This approach should be in the armamentarium of cranial base surgeons as an option in the management of clival chordomas.

Originally classified as a variant of silent corticotroph adenoma, silent subtype 3 adenomas are a distinct histologic variant of pituitary adenoma of unknown cytogenesis. We reviewed the clinical, biochemical, radiological, immunohistochemical and ultrastructural features of 31 silent subtype 3 adenomas to clarify their cellular origin. Among 25 with clinical and/or radiological data, all were macroadenomas; there was cavernous sinus invasion in 30% of cases and involvement of the clivus in 17% of cases. Almost 90% of patients were symptomatic; 67% had mass effect symptoms, 37% were hypogonadal and 8% had secondary adrenal insufficiency. Significant hormonal excess in 29% of cases included hyperthyroidism in 17%, acromegaly in 8% and hyperprolactinemia above 150 μg/l in 4%. Two individuals with hyperprolactinemia who were younger than 30 years had multiple endocrine neoplasia type 1. Immunohistochemically, all 31 tumors were diffusely positive for the pituitary lineage-specific transcription factor Pit-1. Although three only expressed Pit-1, others revealed variable positivity for one or more hormones of Pit-1 cell lineage (growth hormone, prolactin, thyroid-stimulating hormone), as well as alpha-subunit and estrogen receptor. Most tumors exhibited perinuclear reactivity for keratins with the CAM5.2 antibody; scattered fibrous bodies were noted in five (16%) tumors. The mean MIB-1 labeling index was 4% (range, 1-9%). Fourteen cases examined by electron microscopy were composed of a monomorphous population of large polygonal or elongated cells with nuclear spheridia. Sixty-five percent of patients had residual disease after surgery; after a mean follow-up of 48.4 months (median 41.5; range=2-171) disease progression was documented in 53% of those cases. These data identify silent subtype 3 adenomas as aggressive monomorphous plurihormonal adenomas of Pit-1 lineage that may be associated with hyperthyroidism, acromegaly or galactorrhea and amenorrhea. Our findings argue against the use of the nomenclature 'silent' for these tumors. To better reflect the characteristics of these tumors, we propose that they be classified as 'poorly differentiated Pit-1 lineage adenomas'.

We report our preliminary outcomes following high-dose image-guided intensity modulated radiotherapy (IG-IMRT) for skull base chordoma and chondrosarcoma.Forty-two consecutive IG-IMRT patients, with either skull base chordoma (n = 24) or chondrosarcoma (n = 18) treated between August 2001 and December 2012 were reviewed. The median follow-up was 36 months (range, 3-90 mo) in the chordoma cohort, and 67 months (range, 15-125) in the chondrosarcoma cohort. Initial surgery included biopsy (7% of patients), subtotal resection (57% of patients), and gross total resection (36% of patients). The median IG-IMRT total doses in the chondrosarcoma and chordoma cohorts were 70 Gy and 76 Gy, respectively, delivered with 2 Gy/fraction.For the chordoma and chondrosarcoma cohorts, the 5-year overall survival and local control rates were 85.6% and 65.3%, and 87.8% and 88.1%, respectively. In total, 10 patients progressed locally: 8 were chordoma patients and 2 chondrosarcoma patients. Both chondrosarcoma failures were in higher-grade tumors (grades 2 and 3). None of the 8 patients with grade 1 chondrosarcoma failed, with a median follow-up of 77 months (range, 34-125). There were 8 radiation-induced late effects-the most significant was a radiation-induced secondary malignancy occurring 6.7 years following IG-IMRT. Gross total resection and age were predictors of local control in the chordoma and chondrosarcoma patients, respectively.We report favorable survival, local control and adverse event rates following high dose IG-IMRT. Further follow-up is needed to confirm long-term efficacy.

Sinonasal carcinomas are uncommon neoplasms accounting for approximately 3% to 5% of all upper respiratory tract malignancies . Sinonasal malignancies in most cases do not cause early symptoms and present in an advanced stage of disease. Exact staging necessitates a clinical and endoscopic examination with biopsy and imaging. Tumor resection using an open or endoscopic approach is usually considered the first treatment option. In general, sinonasal carcinomas are radiosensitive, so adjuvant or neoadjuvant radiation treatment may be indicated in advanced disease. Multidisciplinary surgical and medical oncologic approaches, including ablation and reconstruction, have enhanced the survival outcome over the past few decades.

Object. The management of certain meningiomas of the skull base and those involving the dural venous sinuses remains a challenge. In recent reports it has been suggested that hydroxyurea chemotherapy can cause regression of unresectable and recurrent meningiomas. The authors report their experience in using hydroxyurea for the treatment of patients with recurrent or unresectable meningiomas. Methods. Hydroxyurea was administered at a dosage of approximately 20 mg/kg/day to 11 women and nine men (median age 59 years, range 31–75 years) with recurrent or unresectable intracranial meningiomas (12 basal, two parasagittal, and six multiple). In 16 patients the meningiomas were benign, in three they had atypical features, and in one the meningioma was malignant. All patients had measurable residual disease. Four patients with benign meningiomas had previously received radiotherapy (two were treated with conventional fractionated radiotherapy and two with stereotactic radiosurgery), three with atypical meningiomas received conventional fractionated radiotherapy, and the one with a malignant meningioma received conventional radiotherapy with additional stereotactic radiosurgery. Tumor enlargement was documented in all patients on neuroimages obtained before initiation of hydroxyurea therapy. All patients were evaluable for response to therapy. In 12 patients with benign meningiomas, the disease had stabilized on neuroimages obtained posttreatment (median duration of treatment 122 weeks, range 8–151 weeks), and two of these showed clinical improvement. One patient with a benign meningioma experienced a minor partial response that was noted after 39 weeks of treatment and was confirmed on neuroimaging and clinical evaluations. In three others with benign meningiomas, progression was confirmed on neuroimages obtained after 41, 55, and 66 weeks, respectively; the 1-year freedom from progression rate was 0.93 (standard error 0.07) in patients with benign meningiomas. In three patients with atypical meningiomas, the tumors had progressed on neuroimages obtained after 12, 19, and 45 weeks, respectively. In the patient with a malignant meningioma, progression was confirmed on neuroimages obtained at 24 weeks. Hydroxyurea has been reasonably well tolerated, although one patient discontinued therapy because of moderate myelosuppression. Conclusions. Although tumor regression appears uncommon, these results indicate that hydroxyurea may arrest progression of unresectable or recurrent benign meningiomas.

We reviewed our experience with combined approaches to lesions that transcend the bones of the skull base. Seventy-seven skull base procedures were performed on 73 patients during a 10-year period from 1982 to 1992. There were 34 patients (44%) with region lesions (anterior), seven patients (9%) with region II lesions (anterior-lateral), 25 patients (32%) with region III lesions (lateral-posterior), and 11 patients (14%) with lesions that invaded more than one anatomic site. The histopathology in this series was quite variable, with 22 patients (29%) having squamous cell carcinoma and eight patients (10%) having basal cell carcinoma. Forty-one patients had surgery by an anterior approach and 38 patients had lateral approaches, with 18 undergoing an infratemporal approach and 29 undergoing temporal bone resections. Overall, 44% of the patients had a postoperative complication. Survival of this heterogeneous group of patients is 79% at 2 years and 71% at 4 years, with those patients with region II disease having a statistically significant poorer prognosis with no survivors at 4 years.

Object The adult presentation of tethered cord syndrome (TCS) is well recognized but continues to pose significant diagnostic and management challenges. The authors performed a retrospective study of clinical outcomes after neurosurgical intervention in 60 adults with TCS. Methods All patients who underwent detethering surgery for caudal cord tethering at Toronto Western Hospital between August 1993 and 2004 were identified. Their clinical charts, operative records, and follow-up data were reviewed. Detethering procedures were performed in 62 patients (age range 17–72 years) for TCS of various origins. Long-term (mean 41.5 months) follow-up data were obtained in 60 patients. The tethering lesions were tight terminal filum in 29 patients, postrepair myelomeningocele in 15, lipomyelomeningocele/lipoma in nine, split cord malformation in four, and arachnoidal adhesions in three. Fifty-nine patients presented with progressive pain and/or neurological dysfunction. One patient underwent prophylactic sectioning of the terminal filum. Most patients (71%) had bladder dysfunction at presentation. Microsurgical release of the tethered cord was performed in each case while using multimodality intraoperative neurophysiological monitoring. The most common complication was cerebrospinal fluid leakage, which occurred in nine patients and was managed by reinforcement sutures in four patients, temporary external drainage in three, and the placement of a lumboperitoneal shunt in two. Infective complications included superficial wound infection in three patients, meningitis in one, and urinary tract infection in one. One patient who had undergone multiple previous intradural procedures experienced worsened foot weakness postoperatively. Another patient experienced temporary unilateral lower-limb numbness. At follow up, improvement was noted in the majority of patients presenting with back (78%) and leg (83%) pain. Improvement was more likely in patients with preoperative motor weakness than in those with sensory deficits. Overall, neurological status was improved or stabilized in 90% of patients. Subjective improvement in bladder function was noted in 50% of patients with bladder dysfunction at presentation. Conclusions Surgery in adult patients with TCS is safe and effective for improving pain and neurological status in the majority of patients; however, patients who have undergone previous intradural detethering procedures in general fare less well, and considerable judgment is required in their management.

Esthesioneuroblastoma is rare. The aim of the study was to review our experience and to evaluate the staging system and treatment that best correlates with the patient outcome.Thirty-nine patients were identified between 1972 and 2006.At presentation 10% had cervical metastases. None had distant metastasis. Five were treated with surgery, 2 with chemotherapy, 1 with radiotherapy, and 30 with surgery and radiation. Local disease control was 82.6% at 5 years. Recurrence was seen in 33% with local and regional disease recurrence at 15% and 18%, respectively. The 5- and 10-year overall survival rates were 87.9% and 69.2%, respectively. Dulguerov classification correlated most closely to survival and recurrence.Dulguerov classification best correlates with the patient's outcome. A combined approach is the preferred treatment. It makes no difference whether radiotherapy is given pre or postsurgical resection. Recurrence can occur even 15 years after treatment. Therefore, long-term follow-up is essential.

Copeptin is a stable surrogate marker of vasopressin release; the peptides are stoichiometrically secreted from the neurohypophysis due to elevated plasma osmolality or nonosmotic stress. We hypothesized that following stress from pituitary surgery, patients with neurohypophyseal damage and eventual diabetes insipidus (DI) would not exhibit the expected pronounced copeptin elevation.The objective was to evaluate copeptin's accuracy to predict DI following pituitary surgery.This was a prospective multicenter observational cohort study.Three Swiss or Canadian referral centers were used.Consecutive pituitary surgery patients were included.Copeptin was measured postoperatively daily until discharge. Logistic regression models and diagnostic performance measures were calculated to assess relationships of postoperative copeptin levels and DI.Of 205 patients, 50 (24.4%) developed postoperative DI. Post-surgically, median [25th-75th percentile] copeptin levels were significantly lower in patients developing DI vs those not showing this complication: 2.9 [1.9-7.9] pmol/L vs 10.8 [5.2-30.4] pmol/L; P < .001. Logistic regression analysis revealed strong association between postoperative copeptin concentrations and DI even after considering known predisposing factors for DI: adjusted odds ratio (95% confidence interval) 1.41 (1.16-1.73). DI was seen in 22/27 patients with copeptin <2.5 pmol/L (positive predictive value, 81%; specificity, 97%), but only 1/40 with copeptin >30 pmol/L (negative predictive value, 95%; sensitivity, 94%) on postoperative day 1.Lack of standardized DI diagnostic criteria; postoperative blood samples for copeptin obtained during everyday care vs at fixed time points.In patients undergoing pituitary procedures, low copeptin levels despite surgical stress reflect postoperative DI, whereas high levels virtually exclude it. Copeptin therefore may become a novel tool for early goal-directed management of postoperative DI.

OBJECTIVE A surgical series of 201 benign and malignant peripheral nerve sheath tumors (PNSTs) was assessed to characterize the anatomical and clinical presentation of tumors and identify predictors of neurological outcome, recurrence, and extent of resection. METHODS All surgically treated PNSTs from the Division of Neurosurgery at Toronto Western Hospital from 1993 to 2010 were reviewed retrospectively. Data were collected on patient demographics, clinical presentation, surgical technique, extent of resection, postoperative neurological outcomes, and recurrence. RESULTS One hundred seventy-five patients with 201 tumors had adequate follow-up for analysis. There were 182 benign and 19 malignant PNSTs. Of the benign lesions, 133 were schwannomas, 21 of which were associated with a diagnosis of schwannomatosis. There were 49 neurofibromas, and 26 were associated with neurofibromatosis Type 1 (NF1). Patients presenting with schwannomas were significantly older than those with neurofibromas. Schwannomas were more readily resected than neurofibromas, with the extent of resection of the former influenced by tumor location. Patients with benign PNSTs typically presented with a painful mass and less frequently with motor deficits. The likelihood of worsened postoperative motor function was decreased in patients with fully resected tumors or preoperative deficits. Recurrence of schwannomas and neurofibromas were seen more frequently in patients diagnosed with NF3 and NF1, respectively. Subtotal resection was associated with the increased recurrence of all benign lesions. CONCLUSIONS Outcomes following resection of benign PNSTs depend on tumor histopathology, tumor location, and genetic predisposition syndrome. Gross-total resection should be attempted for benign lesions where possible. The management of malignant PNSTs remains challenging, requiring a multimodal approach.

We compare the outcomes and postoperative MRI changes of endoscopic endonasal (EEA) and bifrontal craniotomy (BFC) approaches for olfactory groove meningiomas (OGM). All patients who underwent either BFC or EEA for OGM were eligible. Matched pairs were created by matching tumor volumes of an EEA patient with a BFC patient, and matching the timing of the postoperative scans. The tumor dimensions, peritumoral edema, resectability issues, and frontal lobe changes were recorded based on preoperative and postoperative MRI. Postoperative fluid-attenuated inversion recovery (FLAIR) hyperintensity and residual cystic cavity (porencephalic cave) volume were compared using univariable and multivariable analyses. From a total of 70 patients (46 EEA, 24 BFC), 10 matched pairs (20 patients) were created. Three patients (30%) in the EEA group and two (20%) in the BFC had postoperative cerebrospinal fluid leaks (p = 0.61). Gross total resections were achieved in seven (70%) of the EEA group and nine (90%) of the BFC group (p = 0.26), and one patient from each group developed a recurrence. On postoperative MRI, there was no significant difference in FLAIR signal volumes between EEA and BFC approaches (6.9 versus 13.3 cm3; p = 0.17) or in porencephalic cave volumes (1.7 versus 5.0 cm3; p = 0.11) in univariable analysis. However, in a multivariable analysis, EEA was associated with less postoperative FLAIR change (p = 0.02) after adjusting for the volume of preoperative edema. This study provides preliminary evidence that EEA is associated with quantifiable improvements in postoperative frontal lobe imaging.

Pituitary apoplexy is associated with visual, cranial nerve, and endocrine dysfunction. In this article, the results of surgical and conservative management of pituitary apoplexy in a single center are evaluated and a review of the literature is presented. A retrospective analysis was made of patients with pituitary apoplexy who underwent surgery or conservative management at our center between January 2007 and June 2017. Surgery was typically selected for patients who presented with acute deterioration of visual status and/or level of consciousness. Patients with no visual field deficit and those who had medical contraindications to undergo a surgical procedure because of previous comorbidities typically had conservative treatment. Baseline characteristics and clinical and radiologic outcomes were reviewed. A review of the literature (1990–2018) was performed according to PRISMA guidelines. Studies comparing the results of conservative and surgical management were identified. Visual, cranial nerve, and endocrine outcomes and tumor recurrence were analyzed. Forty-nine patients (73.1%) were managed surgically and 18 (26.9%) conservatively. After careful case selection, patients underwent surgical or conservative treatment. Patients who underwent conservative treatment had fewer visual deficits. At diagnosis, visual deficit (38.8% vs. 75.5%; P = 0.008) and cranial nerve palsy (27.7% vs. 51%; P = 0.058) were less common in the conservative group. Conservative and surgical treatments had similar visual and cranial nerve improvement rates (75% vs. 58.3%, P = 0.63 and 75% vs. 69.2%, P = 1.0, respectively). In the conservative group, tumor shrinkage was observed in 76.4% of cases. The systematic review retrieved 11 studies. No significant difference between conservative and surgical treatment for clinical outcomes (visual field recovery, odds ratio [OR], 1.45; 95% confidence interval [CI], 0.72–2.92; cranial nerve recovery, OR, 2.30; 95% CI, 0.93–5.65; and hypopituitarism, OR, 1.05; 95% CI, 0.64–1.74) or tumor recurrence (OR, 0.68; 95% CI, 0.20–2.34) was observed. A tailored approach to pituitary apoplexy, one that does not include an absolute need for surgery, is appropriate. Conservative management is appropriate in selected patients presenting without visual deficits.

Clival lesions pose significant challenges with regard to their surgical management. The expanded endoscopic endonasal (EEE) approach is a promising minimally invasive technique for lesions of the central skull base. The authors' aim in the current paper was to discuss the surgical treatment of clival lesions and to present the technical details, indications, and limitations of the EEE approach. Data from a recent endoscopically treated group will be compared with findings in a previous cohort of patients treated via classic open anterior and lateral approaches.Since June 2005, 17 patients with clival lesions underwent surgery via the EEE approach. Suitable candidates were chosen according to lesion characteristics, clinical parameters, and surgical goals. Neurological outcomes, Karnofsky Performance Scale scores, the extent of lesion resection, and complications were evaluated among these patients. Eighteen percent of the patients in the endoscopic group presented with recurrent disease. Another series of 43 patients, who had undergone resection of clival lesions via an anterior (rhinotomy, maxillectomy, microscopic transsphenoidal surgery, or transoral surgery) or lateral (pterional, frontoorbitozygomatic, or combined suprainfratentorial retrosigmoid) approach, was similarly reviewed. Twenty-three of these patients (53%) presented with recurrent disease and thus had undergone prior surgery.Following the EEE approach, 11 (79%) of 14 patients who had presented with neurological symptoms experienced improvement, and gross-total resection was achieved in 59% of the patients and subtotal removal in 41%. Complications included CSF leakage (24%), tension pneumocephalus (6%), and intracranial hematoma (6%). The patient with the latter complication was the only one who experienced permanent neurological worsening. In the open resection group, neurological worsening occurred in 33% of the patients (14 of 43). Total and grosstotal removals were achieved in 84% of patients and subtotal removal in 14%.The EEE approach has been shown to be a safe and effective technique for the resection of clival lesions with limited lateral extension. The choice of surgical approach must be tailored according to both patient and tumor characteristics. Although the 2 patient series featured in this paper are not comparable-because of a selection bias-higher rates of neurological morbidity and total and gross-total resections were observed in the open resection group. Given the long survival of some patients, the EEE approach should be favored whenever reasonable.

Incompletely and even adequately clipped berry aneurysms are often reinforced with finely shredded gauze. In seven female patients this practice led to a series of events including headache, pyrexia, seizures, cranial nerve deficits, endocrinopathy, cerebrospinal fluid pleocytosis, and an enhancing mass demonstrated by computerized tomography at the aneurysm site. One patient with blindness, hydrocephalus, and panhypopituitarism died and was examined at autopsy. Three additional female patients have been identified in the literature with similar case histories. It is suggested that in these patients the gauze induced a foreign-body granuloma, accompanied by progressive occlusion of neighboring small arteries. It would seem prudent to reserve gauze reinforcement for aneurysms that cannot be securely obliterated surgically.

Abstract Background As the indications for expanded endonasal approaches continue to evolve, alternative reconstructive techniques are needed to address increasingly complex surgical skull base defects. In the absence of the nasoseptal flap, we describe our experience with the posterior pedicle inferior turbinate flap (PPITF) in skull base reconstruction. Design Case series. Setting Academic tertiary care centre. Methods Patients who underwent reconstruction of the skull base with the PPITF were identified. Medical records were reviewed for demographic, presentation, treatment, follow-up, surgical and outcomes data. Main outcome measures Flap survival, adequacy of seal, and complications. Results Two patients with residual/recurrent pituitary adenomas met the inclusion criteria. The nasoseptal flap was unavailable in each case due to a prior septectomy. Salvage of the original nasoseptal flap was not possible, as it did not provide adequate coverage of the resultant defect due to contraction from healing. All PPITFs healed uneventfully and covered the entire defect. No complications were observed in the early post-operative period. Endoscopic techniques and limitations of the PPITF are also discussed. Conclusions Our clinical experience supports the PPITF to be a viable alternative for reconstruction of the skull base in the absence of the nasoseptal flap.

OBJECTIVE The objective of this study was to identify the natural history and clinical predictors of postoperative recurrence of skull base and non-skull base meningiomas. METHODS The authors performed a retrospective hospital-based study of all patients with meningioma referred to their institution from September 1993 to January 2014. The cohort constituted both patients with a first-time presentation and those with evidence of recurrence. Kaplan-Meier curves were constructed for analysis of recurrence and differences were assessed using the log-rank test. Cox proportional hazard regression was used to identify potential predictors of recurrence. RESULTS Overall, 398 intracranial meningiomas were reviewed, including 269 (68%) non-skull base and 129 (32%) skull base meningiomas (median follow-up 30.2 months, interquartile range [IQR] 8.5-76 months). The 10-year recurrence-free survival rates for patients with gross-total resection (GTR) and subtotal resection (STR) were 90% and 43%, respectively. Skull base tumors were associated with a lower proliferation index (0.041 vs 0.062, p = 0.001), higher likelihood of WHO Grade I (85.3% vs 69.1%, p = 0.003), and younger patient age (55.2 vs 58.3 years, p = 0.01). Meningiomas in all locations demonstrated an average recurrence rate of 30% at 100 months of follow-up. Subsequently, the recurrence of skull base meningiomas plateaued whereas non-skull base lesions had an 80% recurrence rate at 230 months follow-up (p = 0.02). On univariate analysis, a prior history of recurrence (p < 0.001), initial WHO grade following resection (p < 0.001), and the inability to obtain GTR (p < 0.001) were predictors of future recurrence. On multivariate analysis a prior history of recurrence (p = 0.02) and an STR (p < 0.01) were independent predictors of a recurrence. Assessing only patients with primary presentations, STR and WHO Grades II and III were independent predictors of recurrence (p < 0.001 for both). CONCLUSIONS Patients with skull base meningiomas present at a younger age and have less aggressive lesions overall. Extent of resection is a key predictor of recurrence and long-term follow-up of meningiomas is necessary, especially for non-skull base tumors. In skull base meningiomas, recurrence risk plateaus approximately 100 months after surgery, suggesting that for this specific cohort, follow-up after 100 months can be less frequent.

The expanded endoscopic endonasal (EEE) approach for the removal of olfactory groove (OGM) and tuberculum sellae (TSM) meningiomas is currently becoming an acceptable surgical approach in neurosurgical practice, although it is still controversial with respect to its outcomes, indications and limitations. Here we provide a review of the available literature reporting results with use of the EEE approach for these lesions together with our experience with the use of the endoscope as the sole means of visualization in a series of patients with no prior surgical biopsy or resection. Surgical cases between May 2006 and January 2013 were retrospectively reviewed. Twenty-three patients (OGM n = 6; TSM n = 17) were identified. In our series gross total resection (GTR) was achieved in 4/6 OGM (66.7%) and 11/17 (64.7%) TSM patients. Vision improved in the OGM group (2/2) and 8/11 improved in the TSM group with no change in visual status in the remaining three patients. Post-operative cerebrospinal fluid (CSF) leak occurred in 2/6 (33%) OGM and 2/17 (11.8%) TSM patients. The literature review revealed a total of 19 OGM and 174 TSM cases which were reviewed. GTR rate was 73% for OGM and 56.3% for TSM. Post-operative CSF leak was 30% for OGM and 14% for TSM. With careful patient selection and a clear understanding of its limitations, the EEE technique is both feasible and safe. However, longer follow-ups are necessary to better define the appropriate indications and ideal patient population that will benefit from the use of these newer techniques.

Abstract Background Blood pressure has become one of the most important vital signs to monitor in the perioperative setting. Recently, the Italian Society of Anesthesia Analgesia Resuscitation and Intensive Care (SIAARTI) recommended, with low level of evidence, continuous monitoring of blood pressure during the intraoperative period. Continuous monitoring allows for early detection of hypotension, which may potentially lead to a timely treatment. Whether the ability to detect more hypotension events by continuous noninvasive blood pressure (C-NiBP) monitoring can improve patient outcomes is still unclear. Here, we report the rationale, study design, and statistical analysis plan of the niMON trial, which aims to evaluate the effect of intraoperative C-NiBP compared with intermittent (I-NiBP) monitoring on postoperative myocardial and renal injury. Methods The niMon trial is an investigator-initiated, multicenter, international, open-label, parallel-group, randomized clinical trial. Eligible patients will be randomized in a 1:1 ratio to receive C-NiBP or I-NiBP as an intraoperative monitoring strategy. The proportion of patients who develop myocardial injury in the first postoperative week is the primary outcome; the secondary outcomes are the proportions of patients who develop postoperative AKI, in-hospital mortality rate, and 30 and 90 postoperative days events. A sample size of 1265 patients will provide a power of 80% to detect a 4% absolute reduction in the rate of the primary outcome. Conclusions The niMON data will provide evidence to guide the choice of the most appropriate intraoperative blood pressure monitoring strategy. Clinical trial registration Clinical Trial Registration: NCT05496322, registered on the 5 th of August 2023.

The authors report a case of pituitary apoplexy resulting in bilateral internal carotid artery occlusion, with marked depression of consciousness and hemiplegia. After transsphenoidal tumor decompression, restoration of flow in both carotid arteries was documented angiographically and the patient made an excellent clinical recovery. The unique aspect of this case is that the pituitary apoplexy was apparently precipitated by neuroendocrine manipulation, performed as a preoperative test of pituitary function.

We report our experience with the treatment of tuberculum sellae meningiomas using the fronto-basal interhemispheric approach. A retrospective analysis was performed on a series of 24 patients with tuberculum sellae meningiomas who were operated between March 2000 and January 2007. Patients' presenting symptoms, radiological images, operative reports, and clinical follow-up data were reviewed with special consideration for visual outcome. Visual deterioration was the presenting symptom in all patients, followed by headache in 9 patients (37.5%). The average duration of visual symptoms was 17.6 months. The average tumor diameter was 2.63 cm; encasement of the carotid artery was identified in 7 patients (29%). Complete tumor removal was achieved in 21 patients (87.5%). Mean follow-up period was 52 months. Vision improved in 19 patients (79%), remained stable in 4 (17%) and deteriorated in 1 patient (4%). The degree of tumor removal or visual outcome were both unrelated to the tumor size (p = 0.2 and p = 0.6 respectively). While the degree of preoperative visual deficit did not affect the visual improvement rate in the whole group (p = 0.9), those patients with improvement to good functional vision (>20/40) after the surgery, had a less severe preoperative deficit (p < 0.001). The most common complication was anosmia (29.1%) and there was no mortality. The frontobasal interhemispheric approach is safe and provides a direct anatomical approach to tuberculum sellae meningiomas with relatively low incidence of complications. Patients with improved vision to good functional level had a better preoperative visual status.

SUMMARY: Injury to peripheral nerves complicating deep intramuscular injections of antibiotic and other agents is well recognized and can result in significant permanent neurological deficit. The purpose of this paper is to review the subject of nerve injection injuries, and report on a series of recent experimental studies carried out in this laboratory designed to improve our understanding of the pathophysiology of this condition and help provide a rational basis for its treatment. A wide variety of chemotherapeutic, prophylactic, and local anaesthetic agents in common use were injected into the sciatic nerve of the adult Wistar rat. Both intrafascicular and extrafascicular injections were examined. Results revealed that the site of injection was the most crucial factor in determining the degree of nerve fiber injury. Following intrafascicular injection, the degree of injury varied significantly, depending upon the specific agent injected. The most severe injuries were associated with wide-spread axonal and myelin degeneration. Pathological alterations in the nerve were evident as early as 30 minutes following injection injury. Regeneration was a constant finding in nerve damage by injection of the various agents. The mechanism of injury appeared to be a direct toxic effect of the injected compound on neural tissue, with an associated break down of the bloodnerve barrier.

Abstract In an attempt to answer questions regarding nerve injection injuries, we injected 11 agents in current use and commonly administered by intramuscular injection into the sciatic nerves of adult Wistar rats. Equal volumes of normal saline were used as control. We harvested the sciatic nerves at various times after injection and examined them by both light and electron microscopy. We performed myelinated nerve fiber counts and constructed histograms. Any impairment of motor function was also noted. We gave injections to 79 animals a total of 158 times; 116 injections were directly into the nerve fascicle (intrafascicular) and 42 were into the epineural tissue (extrafascicular). The results revealed considerable variation in the degree of nerve fiber injury according to the agent injected. Minimal damage resulted from the injection of irondextran. meperidine, and cephalothin, and maximal nerve injury followed the injection of penicillin, diazepam, and chlorpromazine. The site of injection was crucial. Intrafascicular injection was invariably associated with severe nerve injury, but, with few exceptions, extrafascicular injection resulted in minimal damage. The quantity of drug injected was also important in determining the degree of injury. Large, heavily myelinated fibers were more susceptible to injection injury than smaller, thinly myelinated nerve fibers. The effect of the injected drug seemed to be related to injury of the nerve fiber unit—both the axon and the Schwann cell with its myelin sheath. Regeneration in damaged nerves was a constant finding; even the most severely injured nerves, with total axonal degeneration, underwent subsequent regeneration.

Despite the increasing interest in endoscopic techniques for pituitary surgery, little has been published on the endoscopic approach for recurrent and/or residual pituitary adenomas. We report the outcome of purely endoscopic endonasal surgery for a series of recurrent and/or residual pituitary tumors after a previous microscopic resection.We reviewed all of the patients in our institution who underwent endoscopic resection for recurrent and/or residual pituitary tumors after previous microscopic resection. All patients had clinical and magnetic resonance imaging follow-up of at least 3 months postoperatively. Careful attention was given to the operative reports documenting the degree of previous microscopic exposure. Our results were compared with published reports of surgery for recurrent and residual pituitary tumors.Thirty-nine patients met our inclusion criteria. The mean follow-up was 21 months. Tumors were comprised of 19 nonfunctional, 10 adrenocorticotropic hormone, 9 growth hormone, and 1 prolactin-secreting adenoma. The endoscopic procedure revealed limited previous exposure of the sphenoidal and sellar structures in 30 cases (76%). Sphenoidotomy and sellar opening, in terms of working area and angle of view, were significantly restricted in 64% and 61% of the cases, respectively. Gross total removal was achieved in 46% of cases. Seventeen patients had frank cavernous sinus invasion.The restricted exposure of sphenoidal and sellar structures by the microscopic approach may be a contributing factor to incomplete tumor resection. The results observed in this setting make the endoscopic technique a valid option in recurrent and residual pituitary adenomas treated initially by microscopic surgery.

The factors associated with pituitary adenoma (PA) growth rate remain unclear.The objective of the study was to establish whether the preoperative growth and extension pattern of PA can predict postoperative growth rate and recurrence in addition to whether the PA growth rate correlates with proliferation and growth factor expression.One hundred fifty-three consecutive patients who underwent surgery for pituitary adenoma from 1999 to 2011 at Toronto Western Hospital were identified.The PA growth rate was measured both pre- and postoperatively, and its association with patient demographics, magnetic resonance imaging, and histolopathological parameters was determined.The preoperative growth rate was associated with age (P = .0001), suprasellar growth (P = .003), the presence of a cyst/hemorrhage (P = .004), the mindbomb homolog-1 (P = .005), fibroblast growth factor receptor-4 positivity (P = .047), and p27 negativity (P = .007). After surgery, there were 34.6% residual volumes, which were associated with older age (P = .038) and also with growth patterns including anterior, posterior, suprasellar, and cavernous sinus extension (P = .001); 43.3% of these residuals grew and postoperative growth rate was calculated. Pre- and postoperative growth rates were correlated (r = 0.497, P = .026). Postoperative growth rate was associated with age (P = .015) and gender (P = .017).Our data suggest that the growth rate of PAs are influenced by various patient- and tumor-specific characteristics including the age and sex of the patient, the specific subtype of PA, its hormonal activity, its immunohistochemical profile including the mindbomb homolog 1 labeling index status, and its preponderance for different growth directions relative to the pituitary fossa. Furthermore, the pre- and postoperative PA growth rates were correlated, suggesting that postoperative PA growth rates can be predicted, in part, by preoperative growth rates, thus better informing postoperative outcome.

Giant adenomas represent a significant surgical challenge. Although traditionally several transcranial and transsphenoidal microscopic approaches have had a central role in their management, in the last 2 decades here have been increasing reports of the endoscopic endonasal approach for giant adenomas, citing its improved resection rates and lower complication profile. However, its role as the preferred approach has not been fully established and there is currently a paucity of evidence-based recommendations available in the literature. This article reviews the current literature and attempts to define the role and outcomes of the endoscopic endonasal surgical approach for giant pituitary adenomas.

The evolution of cavernous sinus meningiomas (CSMs) might be unpredictable and the efficacy of their treatments is challenging due to their indolent evolution, variations and fluctuations of symptoms, heterogeneity of classifications and lack of randomized controlled trials. Here, a dedicated task force provides a consensus statement on the overall management of CSMs.To determine the best overall management of CSMs, depending on their clinical presentation, size, and evolution as well as patient characteristics.Using the PRISMA 2020 guidelines, we included literature from January 2000 to December 2020. A total of 400 abstracts and 77 titles were kept for full-paper screening.The task force formulated 8 recommendations (Level C evidence). CSMs should be managed by a highly specialized multidisciplinary team. The initial evaluation of patients includes clinical, ophthalmological, endocrinological and radiological assessment. Treatment of CSM should involve experienced skull-base neurosurgeons or neuro-radiosurgeons, radiation oncologists, radiologists, ophthalmologists, and endocrinologists.Radiosurgery is preferred as first-line treatment in small, enclosed, pauci-symptomatic lesions/in elderly patients, while large CSMs not amenable to resection or WHO grade II-III are candidates for radiotherapy. Microsurgery is an option in aggressive/rapidly progressing lesions in young patients presenting with oculomotor/visual/endocrinological impairment. Whenever surgery is offered, open cranial approaches are the current standard. There is limited experience reported about endoscopic endonasal approach for CSMs and the main indication is decompression of the cavernous sinus to improve symptoms. Whenever surgery is indicated, the current trend is to offer decompression followed by radiosurgery.

Transsphenoidal pituitary surgery is the primary therapy for Cushing disease because of its potential to produce lasting remission without the need for long-term drug or hormone replacement therapy. The authors evaluated the current role of pure endoscopic endonasal pituitary surgery in the treatment of Cushing disease.Twenty-five patients underwent pure endoscopic surgery for confirmed Cushing disease. Thirteen patients had microadenomas and seven had macroadenomas; magnetic resonance images obtained in five patients were only suspicious or nondiagnostic, and thus they underwent inferior petrosal sinus sampling. Two patients had evidence of cavernous sinus involvement. Final histological results were consistent with adrenocorticotropin hormone (ACTH)-secreting adenoma in 20 patients.Twenty patients (80%) had clinical remission and laboratory confirmation of hypocortisolemia (serum cortisol < 100 nmol/L requiring substitution therapy), suppression to low-dose dexamethasone, and normal 24-hour urinary free cortisol. The median follow-up period was 17 months (range 3-32 months). There was no recurrence at the time of the last follow-up. Three patients presented with new anterior pituitary deficiency, but no one had permanent diabetes insipidus. In one patient a cerebrospinal fluid leak developed but later resolved following lumbar drainage. Treatment failure was attributable to involvement of the cavernous sinus in two patients, incomplete tumor removal in one, negative exploration in one, and nodular corticotroph hyperplasia of the pituitary gland in one.Early results indicated that endoscopic endonasal surgery is a safe and effective treatment for ACTH-producing adenomas. The rate of remission in this study is comparable to those in previous series, and the rate of major postoperative complications is extremely low. Further studies with a larger number of patients and longer follow-ups are required to determine whether this more minimally invasive pure endoscopic approach should become the standard of care for the surgical treatment of Cushing disease.

Although regional anesthesia is generally considered to have no neurotoxic properties, significant nerve injury has been reported after its use. The present study was undertaken to examine possible toxic effects on peripheral nerve tissue of local anesthetic agents. We injected the sciatic nerve of

Doctor–patient communication in the setting of a life-threatening illness poses considerable challenges. This study aimed to determine the information needs of a subset of neurosurgical patients. Qualitative case study methodology was used. Twenty-five semi-structured interviews were conducted with ambulatory adult patients who had undergone surgery for a benign brain tumor, arteriovenous malformation, or unruptured aneurysm. Interviews were digitally audio recorded and transcribed, and the data subjected to thematic analysis. Six overarching themes emerged from the data: (1) the amount of information patients want varies; (2) the type of information needed is not limited to information about treatment options and risks; (3) patients engage in independent information seeking for a variety of reasons; (4) patients consider compassion from their surgeon as important; (5) direct communication with the surgeon post-operatively is very important; and (6) patients' information needs are greatest post-operatively. Many patients felt that the amount and quality of information they received was not sufficient, particularly regarding post-operative recovery and long-term life issues, leading many to do their own research. The findings from this study emphasize the need for improved communication with patients so they can participate meaningfully in choices about their treatment, give a truly informed consent, and effectively participate in their own recovery.

The endoscopic technique is increasingly being used for the resection of pituitary adenomas. Its efficacy and safety have been generally accepted, but its impact on the quality of life of the patients treated has not been previously addressed. Most of the studies assessing the quality of life after long-term cure of pituitary adenomas suggest a significantly impaired quality of life (QoL) in all subgroups of pituitary tumours. In our study we analyse QoL data following endoscopic surgery, and attempt comparisons with normative and data from standard approaches. The validated health questionnaire SF-36 was sent to 80 patients who had undergone pure endoscopic resection of a pituitary adenoma. Fifty-four patients returned the completed questionnaire. Outcomes were compared with normative data for the Short Form-36 (SF-36) Heath survey for the general Canadian population. We also compared the results amongst different types of adenomas. We attempted a comparison to previous studies on quality of life for patients who underwent conventional trans-sphenoidal surgery. Our study suggests only minimal impairment of quality of life in patients after successful treatment of pituitary adenomas using the endoscopic approach. There were only very few differences in the perceived quality of life within the different subgroups of adenomas. There was a trend to improved scores in the endoscopic group compared with previous studies in patients treated by conventional approaches. Whilst our data suggest minimal impact on the quality of life for patients after endoscopic removal of pituitary adenomas, further studies with larger number of patients and longer follow-up are required to encourage this finding.

To describe the application of intraoperative monitoring techniques during aneurysm surgery and to discuss the advantages and limitations of these techniques in prevention of postoperative neurologic deficits.Articles found in the literature through PubMed for the time frame 1980-2011 and the authors' personal files were reviewed.Various techniques for detection of vascular insufficiency are available, including direct methods to measure cerebral blood flow and indirect methods to evaluate the integrity of neurologic pathways.The choice of monitoring modality should be governed by the vessel and by the vascular territory most at risk during the planned procedure with proper awareness of the potential limits related to each technique. Aneurysm surgery monitoring should help to address issues of continuity and provide a morphologic and functional assessment. Although the use of monitoring devices is still not routine in aneurysm surgery and no standards have been established, combining different monitoring techniques is crucial to optimize aneurysm surgery and avoid or minimize complications.

Anterior and central skull base lesions and their surgical treatment (endoscopic or open approaches) can affect quality of life. A disease-specific instrument is needed to compare quality of life for different surgical approaches.Items were generated using a composite strategy consisting of chart review, systematic review of skull base instruments, expert interviews, and qualitative analysis of patient focus groups. A cross-sectional survey study was performed to reduce items based on an item impact score.Charts of 138 patients who underwent skull base surgery were reviewed to identify physical items and domains. Five experts were interviewed for item and domain identification. Thirty-four patients were recruited into eight focus groups based on their surgical approach (open or endoscopic) and tumor location (anterior or central). Items were generated using a composite approach and then reduced into a final questionnaire using item impact scores.Chart review identified 47 physical items. Systematic review revealed nine relevant instruments with 217 relevant items. Experts identified 11 domains with 69 additional items. Qualitative analysis of focus groups generated 49 items. A total of 382 items were identified and reduced to 77 items after eliminating overlapping and irrelevant items. Further item reduction using item impact scores yielded 41 items.The Skull Base Inventory is a disease-specific quality-of-life instrument. Psychometric properties have yet to be tested. It may serve to compare quality of life for endoscopic or open procedures.

OBJECTIVE The treatment of recurrent and residual craniopharyngiomas is challenging. In this study the authors describe their experience with these tumors and make recommendations on their management. METHODS The authors performed an observational study of adult patients (≥ 18 years) with recurrent or residual craniopharyngiomas that were managed at their tertiary center. Retrospective data were collected on demographics and clinical, imaging, and treatment characteristics from patients who had a minimum 2-year follow-up. Descriptive statistics were used and the data were analyzed. RESULTS There were 42 patients (27 male, 15 female) with a mean age of 46.3 ± 14.3 years. The average tumor size was 3.1 ± 1.1 cm. The average time to first recurrence was 3.6 ± 5.5 years (range 0.2-27 years). One in 5 patients (8/42) with residual/recurrent tumors did not require any active treatment. Of the 34 patients who underwent repeat treatment, 12 (35.3%) had surgery only (transcranial, endoscopic, or both), 9 (26.5%) underwent surgery followed by adjuvant radiation therapy (RT), and 13 (38.2%) received RT alone. Eighty-six percent (18/21) had a gross-total (n = 4) or near-total (n = 14) resection of the recurrent/residual tumors and had good local control at last follow-up. One of 5 patients (7/34) who underwent repeat treatment had further treatment for a second recurrence. The total duration of follow-up was 8.6 ± 7.1 years. The average Karnofsky Performance Scale score at last follow-up was 80 (range 40-90). There was 1 death. CONCLUSIONS Based on this experience and in the absence of guidelines, the authors recommend an individualized approach for the treatment of symptomatic or growing tumors. This study has shown that 1 in 5 patients does not require repeat treatment of their recurrent/residual disease and can be managed with a "scan and watch" approach. On the other hand, 1 in 5 patients who had repeat treatment for their recurrence in the form of surgery and/or radiation will require further additional treatment. More studies are needed to best characterize these patients and predict the natural history of this disease and response to treatment.

Abstract BACKGROUND The role of expanded endonasal endoscopic surgery for primary and recurrent craniopharyngioma is not yet fully established. OBJECTIVE To report and evaluate our experience with the endoscopic endonasal approach (EEA) for the resection of primary and recurrent craniopharyngiomas. METHODS This is a retrospective cohort analysis of 43 consecutive EEA procedures in 40 patients operated from September 2006 to February 2012 for suprasellar craniopharyngiomas. In 21 patients (48.8%) the disease was recurrent. We have assessed the surgical results, visual, endocrinological, and functional outcomes and resection rates in this patient cohort. RESULTS At presentation, 31 (72.1%) patients had visual deficits, 15 patients (34.9%) complained of headaches, 25 patients (58.1%) had anterior pituitary insufficiency, and 14 (32.5%) had diabetes insipidus. Total resection was achieved in 44.2% surgeries, of which 77.3% were in primary lesions and 9.5% in recurrent lesions ( P &lt; .001). Vision improved in 92.6% patients and worsened in 2.3%. Complications other than vision were encountered in 25.6% including 9/43 cerebrospinal fluid leak, 2/43 meningitis. A total of 51.9% of patients with preoperative residual anterior pituitary function had new anterior pituitary deficiencies and 42.8% had new diabetes insipidus. There was no mortality. Six patients (14%) had recurrence of disease during the follow-up period (mean 56.8 mo), 5 of which required repeat surgery. CONCLUSION The EEA can be integrated in the overall management of both primary and recurrent craniopharyngiomas with good results; however, in our series recurrent surgery was associated with significantly lower rates of gross total resection.

Tumors of hypothalamic neurons that produce vasopressin are rare. We retrieved all cases of vasopressin-positive tumors in the sellar region from the database of the Department of Pathology. Five cases fulfilled the selection criteria, representing the first series of such tumors. Clinical, radiologic, and pathologic features were reviewed. Four tumors classified as neurocytomas were identified in 3 females and 1 male patient; the ages at onset of symptoms ranged from 17 to 40 years. All were large sellar masses with suprasellar extension and/or invasion of the parasellar sinuses. Three patients had the syndrome of inappropriate antidiuresis; in one of these, a 6-year history was initially considered to be idiopathic. One patient died of progressive disease; 3 had incomplete resections and are being followed. In contrast to these patients with neurocytoma, a 65-year-old woman had Cushing disease and a 0.8 cm mass that was completely resected at transsphenoidal surgery; this tumor was a gangliocytoma producing vasopressin associated with corticotroph hyperplasia. We postulate that the small amount of vasopressin secreted by this mature gangliocytic tumor was locally bound to corticotrophs, resulting in hyperplasia and Cushing disease, without sufficient overproduction to cause systemic effects of vasopressin excess. Hypothalamic neurocytoma is a tumor that can mimic pituitary neuroendocrine tumors and olfactory neuroblastoma but is distinguished by positivity for neurofilaments, NeuN, and TTF-1 and negative staining for adenohypophysial biomarkers. Our cases illustrate that neurocytoma and gangliocytoma are 2 variants of tumors of hypothalamic neurons that can produce vasopressin. The morphologic and proliferative features of these 2 tumor types represent 2 ends of a spectrum; their function also can result in divergent clinical manifestations, one characterized by reduced urine output and the other by the more insidious features of glucocorticoid excess.

Surgery is the main treatment option for the management of craniopharyngiomas. Transcranial microsurgical approaches, such as pterional and subfrontal approaches, have constituted the classic operative strategy for resection of these tumors. However, the development of endoscopic endonasal approaches has revolutionized the treatment of craniopharyngiomas in the last 15 years, and endoscopic resection is favored for most craniopharyngiomas. In this article, we discuss our experience with the management of craniopharyngiomas and review the current results of the surgical treatment of those tumors, including discussion of goals of surgery, complications, recurrences, and the role of adjuvant treatment.

OBJECTIVE Transsphenoidal surgery is advocated as the first-line management of growth hormone (GH)–secreting adenomas. Although disease control is defined by strict criteria for biochemical remission, the length of follow-up needed is not well defined in literature. In this report, the authors present their long-term remission rate and identify various predictive factors that might influence the clinical outcome. METHODS The authors conducted a single-institute retrospective analysis of all transsphenoidal procedures for GH-secreting adenomas performed from January 2000 to June 2016. The primary outcome was defined as biochemical remission according to the 2010 consensus criteria and measured at the 1-year postoperative mark as well as on the last recorded follow-up appointment. Secondary variables included recurrence rate, patterns of clinical presentation, and outcome of adjuvant therapy (including repeat surgery). Subgroup analysis was performed for patients who had biochemical or radiological “discordance”—patients who achieved biochemical remission but with incongruent insulin-like growth factor 1 (IGF-1)/GH or residual tumor on MRI. Recurrence-free survival analysis was conducted for patients who achieved remission at 1 year after surgery. RESULTS Eighty-one patients (45 female and 36 male) with confirmed acromegaly treated with transsphenoidal surgery were included. In 62 cases the patients were treated with a pure endoscopic approach and in 19 cases an endoscopically assisted microscopic approach was used. Primary biochemical remission after surgery was achieved in 59 cases (73%) at 1 year after surgery. However, only 41 patients (51%) remained in primary surgical remission (without any adjuvant treatment) at their last follow-up appointment, indicating a recurrence rate of 31% (18 of 59 patients) over the duration of follow-up (mean 100 ± 61 months). Long-term remission rates for pure endoscopic and endoscopically assisted cases were not significantly different (48% vs 52%, p = 0.6). Similarly, no significant difference in long-term remission was detected between primary surgery and repeat surgery (54% vs 33%, p = 0.22). Long-term remission was significantly influenced by extent of resection, cavernous sinus invasion (radiologically as well as surgically reported), and preoperative and early postoperative GH and IGF-1 levels (within 24–48 hours after surgery) as well as by clinical grade, with lower remission rates in patients with dysmorphic features and/or medical comorbidities (grade 2–3) compared to minimally symptomatic or silent cases (grade 1). CONCLUSIONS The long-term surgical remission rate appears to be significantly less than “early” remission rates and is highly dependent on the extent of tumor resection. The authors advocate a long-term follow-up regimen and propose a clinical grading system that may aid in predicting long-term outcome in addition to the previously reported anatomical factors. The role of repeat surgery is highlighted.

The consequences of congenital brain disorders for adult cognitive function are poorly understood. We studied different forms of memory in 29 young adults with spina bifida meningomyelocele (SBM), a common and severely disabling neural tube defect. Nondeclarative and semantic memory functions were intact. Working memory was intact with low maintenance and manipulation requirements, but impaired on tasks demanding high information maintenance or manipulation load. Prospective memory for intentions to be executed in the future was impaired. Immediate and delayed episodic memory were poor. Memory deficits were exacerbated by an increased number of lifetime shunt revisions, a marker for unstable hydrocephalus. Memory status was positively correlated with functional independence, an important component of quality of life.

Purpose To define clinical and dosimetric predictors of nonauditory adverse radiation effects after radiosurgery for vestibular schwannoma treated with a 12 Gy prescription dose. Methods We retrospectively reviewed our experience of vestibular schwannoma patients treated between September 2005 and December 2009. Two hundred patients were treated at a 12 Gy prescription dose; 80 had complete clinical and radiological follow-up for at least 24 months (median, 28.5 months). All treatment plans were reviewed for target volume and dosimetry characteristics; gradient index; homogeneity index, defined as the maximum dose in the treatment volume divided by the prescription dose; conformity index; brainstem; and trigeminal nerve dose. All adverse radiation effects (ARE) were recorded. Because the intent of our study was to focus on the nonauditory adverse effects, hearing outcome was not evaluated in this study. Results Twenty-seven (33.8%) patients developed ARE, 5 (6%) developed hydrocephalus, 10 (12.5%) reported new ataxia, 17 (21%) developed trigeminal dysfunction, 3 (3.75%) had facial weakness, and 1 patient developed hemifacial spasm. The development of edema within the pons was significantly associated with ARE (p = 0.001). On multivariate analysis, only target volume is a significant predictor of ARE (p = 0.001). There is a target volume threshold of 5 cm3, above which ARE are more likely. The treatment plan dosimetric characteristics are not associated with ARE, although the maximum dose to the 5th nerve is a significant predictor of trigeminal dysfunction, with a threshold of 9 Gy. The overall 2-year tumor control rate was 96%. Conclusions Target volume is the most important predictor of adverse radiation effects, and we identified the significant treatment volume threshold to be 5 cm3. We also established through our series that the maximum tolerable dose to the 5th nerve is 9 Gy. To define clinical and dosimetric predictors of nonauditory adverse radiation effects after radiosurgery for vestibular schwannoma treated with a 12 Gy prescription dose. We retrospectively reviewed our experience of vestibular schwannoma patients treated between September 2005 and December 2009. Two hundred patients were treated at a 12 Gy prescription dose; 80 had complete clinical and radiological follow-up for at least 24 months (median, 28.5 months). All treatment plans were reviewed for target volume and dosimetry characteristics; gradient index; homogeneity index, defined as the maximum dose in the treatment volume divided by the prescription dose; conformity index; brainstem; and trigeminal nerve dose. All adverse radiation effects (ARE) were recorded. Because the intent of our study was to focus on the nonauditory adverse effects, hearing outcome was not evaluated in this study. Twenty-seven (33.8%) patients developed ARE, 5 (6%) developed hydrocephalus, 10 (12.5%) reported new ataxia, 17 (21%) developed trigeminal dysfunction, 3 (3.75%) had facial weakness, and 1 patient developed hemifacial spasm. The development of edema within the pons was significantly associated with ARE (p = 0.001). On multivariate analysis, only target volume is a significant predictor of ARE (p = 0.001). There is a target volume threshold of 5 cm3, above which ARE are more likely. The treatment plan dosimetric characteristics are not associated with ARE, although the maximum dose to the 5th nerve is a significant predictor of trigeminal dysfunction, with a threshold of 9 Gy. The overall 2-year tumor control rate was 96%. Target volume is the most important predictor of adverse radiation effects, and we identified the significant treatment volume threshold to be 5 cm3. We also established through our series that the maximum tolerable dose to the 5th nerve is 9 Gy.

Objective To compare financial and perioperative outcomes between endoscopic and open surgical approaches in the surgical management of sinonasal malignancies. Design Retrospective chart review. Setting Tertiary care hospital. Participants Patients undergoing surgical resection of a sinonasal malignancy from January 2000 to December 2014. Main Outcome Measures In-hospital costs, complications, and length of stay (LOS). Results Of 106 patients, 91 received open surgery (19 free flap and 72 non-free flap) and 15 were treated with purely endoscopic approaches. Free flaps had a significantly higher average cost, operative time, and LOS compared to both non-free flap (p &lt; 0.001, &lt; 0.001, and &lt; 0.01) and endoscopic (p = 0.01, 0.04, and &lt; 0.01) groups. There were no significant differences in average costs between endoscopic and non-free flap groups ($19,157 vs. $14,806, p = 0.20) or LOS (5.7 vs. 6.4 days, p = 0.72). Compared with the non-free flap group, the endoscopic group had a longer average operative time (8.3 vs. 5.5 hours, p &lt; 0.01) and higher rates of cerebrospinal fluid (CSF) leak (13 vs. 0%, p = 0.01) and intensive care unit (ICU) admission (80 vs. 36%, p &lt; 0.01). Surgical approach (open vs. endoscopic) was not a significant predictor of any financial or perioperative outcome on multivariable analysis. Conclusion Hospital costs are comparable between endoscopic and open approaches when no free tissue reconstruction is required. Longer operative times, higher CSF leak rates, and our institutional protocol necessitating ICU admission for endoscopic cases may account for the failure to demonstrate cost savings with endoscopic surgery.

To review the current literature and provide our institutional approach and opinion on the indications and limitations of traditional open craniofacial resection (CFR) and a minimally invasive pure endonasal endoscopic approach for anterior skull base tumors concentrating primarily on malignant lesions.Based on 3 decades of experience with both open and more recently endoscopic techniques, we examined our current practice in treating tumors and other lesions involving the skull base and our current indications and limitations in the use of these techniques. We conducted a retrospective chart analysis to see which operative techniques were used for malignant tumors of the anterior skull base in the last 10 years at our institution.There were 30 cases identified. Traditional CFR was performed in 16, a pure endoscopic resection was performed in 9, and an endoscopic procedure combined with a frontal craniotomy was performed in 5. Gross total resection was achieved in 83.3% in the CFR group and 75% in the pure endoscopic resection group. Near-total resection was 10% in the CFR group and 33.3% in pure endoscopic resection group. Of the 5 patients who underwent a combined approach, 80% had gross total resection, and 20% had near-total resection.Both traditional CFR and the endonasal endoscopic approach offer advantages and disadvantages. Both approaches can achieve good results with appropriate patient selection. Numerous important factors, including location and the extent of tumor, should be taken into consideration when considering either approach. The most important determinant of outcome is the ability to achieve gross total resection with microscopic negative margins rather than the type of approach used. In the future, skull base surgeons will need to be familiar with and capable of offering both techniques to the patient.

The endonasal endoscopic approach (EEA) for the resection of tuberculum sellae meningiomas (TSMs) has, more recently, been advocated as an alternative approach to deal with this challenging tumor. The aim of this study was to conduct a systematic review of publications of TSMs excised through the transsphenoidal route in the past 10 years and review data on the extent of excision, visual outcomes and complication rates.We performed a thorough systematic review of the medical literature following the PRISMA guidelines. A medical librarian retrieved a list of 3443 articles published from 2006-2015 from the MEDLINE, EMBASE and Cochrane Central databases. Two of the authors independently screened for titles and abstracts and excluded 3340 of them. We reviewed the full text of the remaining 103 articles and included in our analysis 12 that met the following inclusion criteria: 1) 5 or more cases reported; 2) the extent of resection, visual outcomes and complication rates that were specifically documented for TSMs excised through the transsphenoidal route.Twelve studies that included 150 patients were analyzed. The mean age was 55 years. The mean tumor volume, reported in 2 studies, was 6.6 cc and mean maximum diameter, reported in 11 studies, was 25 mm. The gross total resection rate was 77.2%. Vision improved in 79.5% of cases and deteriorated in 7.3%. CSF leak postoperatively occurred in 15.3% of patients. In the 11 studies that reported hormonal outcomes, there was a 9.4% transient hyponatremia or diabetes insipidus and 2.2% of patients developed a new permanent endocrine dysfunction. A symptomatic vascular injury was reported in 2.6% of patients. There was one mortality (0.6%).The endonasal endoscopic transsphenoidal excision of TSMs is a feasible, safe and effective surgical option with a low morbidity and mortality. The use of this approach has evolved in the last 10 years and in some centers has replaced the transcranial route for selected cases. Given the limited availability and heterogeneity of comparative observational studies, a direct comparison with transcranial approaches was not performed for the purpose of this review analysis. Likewise, from an epidemiological and statistical perspective a meta-analysis was deemed inappropriate.

Despite advances in instrumentation and the use of microsurgical techniques, neurosurgical procedures involving extensive areas of skull base or other critical areas of brain still carry significant risk for neurological injury. The use of intraoperative recording of sensory evoked potentials (SEP) has been advocated to monitor neurologic function during these major neurosurgical procedures to reduce the risk of injury to neural structures. This report summarizes our experience with intraoperative monitoring of SEP in over 200 patients, and details our findings in a group of 12 patients with skull base and posterior fossa tumours. Somatosensory evoked potentials (SSEP) were monitored in all patients, and brain stem auditory evoked potentials (BAEP) in five. While minor changes in BAEP and SSEP parameters were noted in most patients, significant changes occurred in five. Irreversible loss of BAEP in one patient was associated with complete hearing loss postoperatively. Marked, persistent alteration of both BAEP and SSEP was associated with postoperative brainstem dysfunction. No patient with stable BAEP and SSEP at the end of the procedure suffered additional neurological deficit. We conclude that intraoperative SEP monitoring may be valuable in minimizing neural injury during major neurosurgical procedures.

A 32-year-old man had biopsy-proven progressive multifocal leukoencephalopathy (PML) and long-standing sarcoidosis. Treatment with several courses of cytarabine failed, and the patient died of the neurologic disease. Reports of therapeutic experiences in PML are summarized.

The objective of the study was to measure upper limb motor function in young adults with spina bifida meningomyelocele (SBM) and typically developing age peers. Participants were 26 young adults with SBM, with a Verbal or Performance IQ score of at least 70 on the Wechsler scales, and 27 age- and gender-matched controls. Four upper limb motor function tasks were performed under four different visual and cognitive challenge conditions. Motor independence was assessed by questionnaire. Fewer SBM than control participants obtained perfect posture and rebound scores. The SBM group performed less accurately and was more disrupted by cognitive challenge than controls on limb dysmetria tasks. The SBM group was slower than controls on the diadochokinesis task. Adaptive motor independence was related to one upper limb motor task, arm posture, and upper rather than lower spinal lesions were associated with less motor independence. Young adults with SBM have significant limitations in upper limb function and are more disrupted by some challenges while performing upper limb motor tasks. Within the group of young adults with SBM, upper spinal lesions compromise motor independence more than lower spinal lesions.

Skull base tumors are associated with physical symptoms that vary depending on location and surgical approach.Skull base surgery patients (n = 138) were retrospectively reviewed and physical symptoms were quantified. Patients were divided into 4 groups by surgical approach (open, endoscopic) and tumor location (anterior, central). Multivariate analyses determined odds for symptom development.Patients with anterior lesions presented with more nasal symptoms compared to those with central lesions (63% vs 6.8%; p < .001). Those with central lesions presented with more neurologic (41.1% vs 12.3%; p < .001) and endocrine symptoms (19.2% vs 0%; p < .001). Three of 4 groups experienced a reduction in neurologic and visual symptoms after surgery. One group (endoscopic/central) experienced a reduction in endocrine and an increase in nasal symptoms. Anterior tumors (p = .02) and endoscopic approaches (p = .002) predicted increased nasal morbidity.Physical morbidity from skull base tumors may vary based on tumor location and surgical approach.

Bone invasive skull base meningiomas are a subset of meningiomas that present a unique clinical challenge due to brain and neural structure involvement and limitations in complete surgical resection, resulting in higher recurrence and need for repeat surgery. To date, the pathogenesis of meningioma bone invasion has not been investigated. We investigated immunoexpression of proteins implicated in bone invasion in other tumor types to establish their involvement in meningioma bone invasion.Retrospective review of our database identified bone invasive meningiomas operated on at our institution over the past 20 years. Using high-throughput tissue microarray (TMA), we established the expression profile of osteopontin (OPN), matrix metalloproteinase-2 (MMP2), and integrin beta-1 (ITGB1). Differential expression in tumor cell and vasculature was evaluated and comparisons were made between meningioma anatomical locations.MMP2, OPN, and ITGB1 immunoreactivity was cytoplasmic in tumor and/or endothelial cells. Noninvasive transbasal meningiomas exhibited higher vascular endothelial cell MMP2 immunoexpression compared to invasive meningiomas. We found higher expression levels of OPN and ITGB1 in bone invasive transbasal compared to noninvasive meningiomas. Strong vascular ITGB1 expression extending from the endothelium through the media and into the adventitia was found in a subset of meningiomas.We have demonstrated that key proteins are differentially expressed in bone invasive meningiomas and that the anatomical location of bone invasion is a key determinant of expression pattern of MMP1, OPN, and ITGB1. This data provides initial insights into the pathophysiology of bone invasion in meningiomas and identifies factors that can be pursued as potential therapeutic targets.

OBJECT In this paper, the authors’ aim was to determine short-term volumetric and diametric tumor growth and identify clinical, radiological, and dosimetric predictors of adverse radiation events (AREs) following stereotactic radiosurgery (SRS) for intracranial WHO Grade I meningiomas. METHODS This is a retrospective review of all WHO Grade I meningiomas that were treated with SRS (primary or adjuvant) between December 2005 and June 2012 at the University Health Network. Seventy-five patients had at least 24 months of both clinical and radiological follow-up and were, therefore, included in this study. Tumor growth was defined as any volumetric or diametric change greater than 10% per year. Any variation less than +10% was considered growth stability. Volumetric measurements were made using T1-weighted gadolinium-enhanced 3-T MRI scans and ITK-SNAP software. Tumor growth rates were calculated using the specific growth rate (SGR). Univariate statistics were used to identify predictors of post-SRS AREs. All statistical analyses were performed using IBM SPSS. RESULTS Women accounted for 69.3% of patients, and the mean treatment age was 58.6 years. Median follow-up was 36.2 months. Twenty-one (28%) patients had undergone prior resection. Two (3%) patients required salvage surgical intervention following SRS. The majority of the lesions (56%) were skull base tumors. Median tumor volume and diameter were 5.2 cm 3 and 27.5 mm, respectively. The absence of tumor growth was observed in 39 cases (52%) based on the volumetric measurements, while the absence of tumor growth was observed in 69 cases (92%) based on the diametric measurements. Twenty-six patients (34.6%) experienced new-onset AREs, including headache (17.3%), cranial neuropathy (10.6%), speech impairment (2.7%), tremors (2.7%), and ataxia (1.3%). Fourteen patients (18.7%) experienced new-onset edema, and 4 of these patients were symptomatic. A lower conformity index (1.24 vs 1.4) was significantly associated with the development of edema (p &lt; 0.001 power &gt; 0.8). Patients with meningiomas that had growth rates of more than 10% per year were more likely to experience long-term headaches after SRS (p = 0.022). CONCLUSIONS Volume-based reporting of SRS outcomes for meningiomas may be a more accurate method given the complex morphology of some lesions. The conformity index was identified as a predictor of edema following radiosurgery.

To quantitatively compare different microsurgical and endoscopic approaches to the middle cranial fossa in a preclinical setting with a novel, computer-based research method.Different approaches were performed bilaterally in 5 head and neck specimens that underwent high-resolution computed tomography scans: 5 transcranial anterolateral (supraorbital, mini-pterional, pterional, pterional-transzygomatic, fronto-temporal-orbito-zygomatic) without and with anterior clinoidectomy; 2 transcranial lateral (subtemporal and subtemporal-transzygomatic); 2 endoscopic transnasal (transpterygoid, transpterygoid to infratemporal fossa); 2 endoscopic transorbital (superior eyelid and inferolateral), and endoscopic transmaxillary. A dedicated navigation system was used to quantify surgical working volumes and exposure of different areas of the middle cranial fossa (ApproachViewer, part of GTx-Eyes II, University Health Network, Toronto, Canada). Statistical analysis was performed using a mixed linear model with bootstrap resampling.Endoscopic transnasal and fronto-temporal-orbito-zygomatic approaches with anterior clinoidectomy showed the largest surgical volumes. Endoscopic approaches allowed a wider exposure of medial anatomical surfaces (e.g., the petrous apex) compared with transcranial ones. Transcranial approaches with larger craniotomies allowed the widest exposure of superomedial anatomical structures (e.g., roof of cavernous sinus). The resection of the zygomatic arch allowed exposure of more medial surfaces with an inferior to superior trajectory.This study implemented a novel neuronavigation-based research method to quantitatively compare different approaches to the middle cranial fossa; its results might guide, after consideration of clinical implications, the choice of the neurosurgical approach to different areas of this complex skull base region.

Resective surgery remains the main treatment option for most patients with craniopharyngiomas. Understanding of the microsurgical anatomy of the sella and suprasellar region and its relationship with these tumors is necessary to achieve effective surgical treatment and minimize complications. In this article, we review the surgical anatomy related to craniopharyngiomas and divide it in 5 compartments according to tumor extension. Endoscopic and microsurgical dissection were performed in 3 freshly injected cadaver heads at the Weill Cornell Surgical Innovations Laboratory (New York, New York, USA) and at the Surgical Skills Center at Mount Sinai Hospital (Toronto, Ontario, Canada). Tumor extension was classified as 1) inferomedial or sellar, 2) superomedial or suprasellar, 3) lateral or sylvian, 4) posterior or interpeduncular/prepontine, and 5) intraventricular. The selection of surgical approaches is discussed based on the anatomic nuances of each these regions. In addition, we reviewed the literature regarding previous anatomic classifications for resection of craniopharyngiomas. Different approaches should be considered according to tumor extension into different compartments. Purely sellar tumors are amenable to endoscopic transsellar approaches, whereas those with a suprasellar extension require an extended transtuberculum approach. In some of those patients, a narrow chiasm-pituitary window may block access to the tumor and a transcranial translamina terminalis approach may be favored. Tumors occupying the interpeduncular fossa may pose a significant challenge for an endoscopic endonasal approach and transcranial approaches. Transcavernous approaches and anterior and posterior clinoidectomies may be required for adequate exposure in such patients. Translamina terminalis and/or transcallosal approaches are recommended for resection of purely intraventricular tumors. Tumors extending into the lateral compartment should be considered for transcranial frontotemporal approaches. The understanding of such anatomic nuances aids in the selection of the most appropriate surgical approach and in the prevention of potential complications. Because most craniopharyngiomas are midline lesions, the endoscopic endonasal approach represents an excellent approach for most of those tumors. However, transcranial approaches should be considered for tumors with extension into the lateral compartment and for selected tumors involving the ventricular compartment (purely intraventricular tumors and those with extension to the foramen of Monro and/or lateral ventricles).

The aim of this study was to evaluate the effectiveness of combined radical surgical resection and radiotherapy for the treatment of esthesioneuroblastoma, a rare malignant neoplasm accounting for 1 to 5% of malignant neoplasms of the nasal cavity.A 10-year review of 12 patients treated at our institution suggests that primary treatment consisting of a combined otolaryngologic/neurosurgical craniofacial approach results in good local control with relatively low morbidity and complication. Patients tend to present with advanced stage disease because of the nonspecific presenting signs and symptoms. Eighty-three percent of the patients were treated with combined surgery and radiation.Seventy-five percent of the patients are free of disease with an average follow-up time of 54 months. Based on our experience in anterior skull base surgery (zone I), we strongly advocate the pericranial flap for dural reconstruction. Postoperative complications occurred in 25% of the cases with no postoperative deaths.Failure analysis suggests that radical surgical resection combined with radiotherapy provides the best chance of disease control.

Rupture of cerebral aneurysms is an important cause of morbidity and mortality in patients with adult polycystic kidney disease (PKD). In the present paper, we review the literature regarding the association of cerebral aneurysms and PKD and emphasize, by means of a case report, the importance of familial clustering of aneurysms in patients with PKD. We conclude that the prevalence of intracranial aneurysms in patients with PKD approaches 40% and is likely significantly higher in patients with PKD and a positive family history for cerebral aneurysm. We recommend that screening cerebral angiography be strongly considered in all patients with PKD and a family history of cerebral aneurysm or subarachnoid hemorrhage.

✓ A prospective randomized double-blind trial was conducted to study the effect of platelet-inhibiting drugs on mural thrombus formation after carotid endarterectomy. Twenty-two patients undergoing carotid endarterectomy were randomly assigned to perioperative administration of an aspirin/dipyridamole combination or a placebo, and the postoperative results were compared. Autologous indium-111-labeled platelets were injected postoperatively, and platelet deposition was measured at the endarterectomy site. It was found that the treated group had a significant reduction in platelet accumulation compared with the placebo group. The results suggest that the perioperative use of aspirin/dipyridamole may reduce the risk of operative stroke and the long-term risk of repeat carotid stenosis.

Object Gamma Knife surgery (GKS) is used to treat benign and malignant brain tumors, arteriovenous malformations, trigeminal neuralgia, and other conditions. Patients experience reduced neurological morbidity from GKS compared with open microneurosurgery, but risks of radiation injury and technical limitations persist. The authors report treatment complications from the early experience of 2 Canadian GKS programs in Toronto and Sherbrooke. Methods In Toronto, a prospective administrative database was searched for adverse events and incomplete treatment administrations. In Sherbrooke, data were acquired by chart review. Patients were accrued until August 1, 2007, and a total of 973 patients were included in this report. Results During the radiosurgical procedure, 19 patients (2%) suffered anxiety or syncopal episodes, and 2 patients suffered acute coronary events. Treatments were incompletely administered in 12 patients (1.2%). Severe pain was a delayed complication: 8 patients suffered unexpected headaches, and 9 patients developed severe facial pain. New motor deficits developed in 11 patients, including edema-induced ataxia in 4 and one case of facial weakness after treatment of a vestibular schwannoma. Four patients required shunt placement for symptomatic hydrocephalus, and 16 patients suffered delayed seizures. Conclusions Gamma Knife surgery is a minimally invasive treatment modality for many intracranial diseases. Treatment is not risk free, and some patients will develop complications; these are likely to decrease as institutional experience matures. Expanding availability and indications necessitate discussion of these risks with patients considering treatment.

Several quality of life (QOL) instruments exist for skull base pathology, however, there have been no attempts to appraise and systematically review these instruments.We systematically reviewed MEDLINE, EMBASE, Central, AMED, Health and Psychosocial Instruments, and PsychoInfo for anterior or central skull base QOL instruments to January 2010. We queried experts, bibliographies, and meeting proceedings from the North American Skull Base Society from 2005 to 2009. Included instruments were evaluated for instrument characteristics, item generation and reduction, field testing, and measurement properties using predefined criteria.We identified 9 QOL instruments: 7 measuring QOL for pituitary pathology, 1 for midface pathology, and 1 for anterior skull base pathology. Eight of the 9 instruments have had some psychometric testing. None demonstrated all of the predefined psychometric properties.There are several QOL instruments for patients with skull base pathology. None of these instruments met all predefined requirements, and further instrument development is needed.

The Skull Base Inventory (SBI) was developed to assess the quality of life of patients undergoing endoscopic or open approaches for anterior and central skull base pathologies. In this study, we sought to establish the discriminative and evaluative properties for this instrument.The SBI was administered in a cross-sectional fashion to patients who previously had skull base surgery after treatment and then again 2 weeks after completing the instrument. Internal consistency, test-retest reliability, and construct validity were determined. Four constructs were evaluated with the following a priori hypotheses: lower scores will be seen in patients with 1.malignant versus benign histology, 2.a history of radiation versus none, and those with 3.recurrences versus no recurrence, and 4.items deemed relevant versus irrelevant by respondents.Fifty-two patients completed the questionnaire; 32 had endoscopic and 20 open surgeries. Internal consistency was good (>0.7 and <0.95) for all domains except one. Test-retest reliability was good (>0.70) for 38 of 41 items. Four constructs were evaluated and three were consistent with a priori hypotheses (p < 0.05). The instrument failed to confirm the hypothesis that malignant tumours are associated with poorer scores than benign.The SBI demonstrated preliminary reliability and validity for discriminative use.

Quantitative comparison of minimally invasive and standard cranial approaches remains a methodologic challenge. The aim of this study was to apply a new digital, navigation-based method to quantify multiple parameters of the pterional, supraorbital, lateral supraorbital and mini-pterional approaches and to describe a target-specific maneuverability score. Supraorbital, lateral supraorbital, mini-pterional, and standard pterional craniotomies were performed on 8 sides in 4 cadaver heads. The limits of superficial and deep exposure and surgical target points were registered with image-guidance (Medtronic Stealth). A custom software (Guided Therapeutics Eyes II Software [GTxEyesII]) calculated superficial and deep surface areas, working volumes, and target distances. Volumes were reconstructed digitally and visualized with GTxEyesII. Finally, we defined for each approach a target-specific maneuverability score based on surface exposure, target distance, target position, and anatomical obstacles. Surface exposure and volumes were significantly smaller for keyhole approaches compared with standard pterional craniotomy (P < 0.01). Maneuverability index scores showed the greatest values for standard pterional craniotomies, but nearly equally high scores could be achieved by one or several of the less-invasive approaches for most of the targets. The combination of a navigation-based measurement method with custom software (GTxEyesII) provides a powerful tool for visualization and quantification of surgical approaches. Compared with standard pterional craniotomy, alternative keyhole craniotomies offer comparable deep exposure with smaller working volumes and relatively high maneuverability for specific targets.

Endoscopic endonasal approaches (EEAs) provide improved access and operative visualization for resection of pituitary adenomas. Although the technique has gained wide acceptance, there is a paucity of data regarding late recurrence. We aim to assess long-term outcomes of patients with nonfunctioning pituitary adenomas (NFPAs) who underwent EEA. We reviewed 269 patients operated on for an NFPA between 2005 and 2015. Clinical and radiologic factors including those potentially related to higher chances of recurrence were analyzed. Progression-free survival was analyzed using the Kaplan-Meier method, and univariate and multivariate survival were analyzed using a Cox regression model. The study included 269 patients. The gross total resection rate was 46.0% (n = 124) but cavernous sinus involvement was present in almost half the patients (n = 115). The probability of recurrence at 5 years and 10 years was 22.0% and 47.2%, respectively. The median time to recurrence was 10 years for patients without cavernous sinus involvement and 6 years for those with cavernous sinus involvement. Univariate and multivariate analysis showed that tumor size, cavernous sinus invasion, anterior skull base extensions, and residual tumor were significantly associated with recurrence. Recurrence rate of NFPA remains high despite the better visualization offered by EEA, especially in those tumors involving the cavernous sinus and/or previously operated on. Repeat surgery is adequate for tumor debulking and decompression of the optic apparatus but is unlikely to achieve gross total resection if a successful previous EEA has been performed. Radiation therapy is an effective option for management of recurrent tumors.

The incidence of idiopathic intracranial hypertension (IIH) has increased in the past decades, and therefore its impacts on patients and health care have increased as well. Although medical therapy, lifestyle changes, and weight loss remain the primary treatment modality, surgical treatment plays a major role in patients with refractory IIH and those with progressive and rapid visual decline. This chapter discusses the roles of surgical approaches for the management of refractory IIH. Current results demonstrate that classic surgical interventions, such as ventriculoperitoneal shunt and lumboperitoneal shunts and optic nerve sheath fenestration remain useful options, but there is growing evidence that other modalities, such as venous stenting and bariatric surgery, may lead to good outcomes, with relatively low complication rates, in select cases. Further studies are needed to clarify the ideal candidates for each specific procedure and also for evaluation of long-term outcome of modern surgical options.

As more women with cerebrospinal fluid shunts reach child-bearing age, neurosurgeons, obstetricians and other health care providers will increasingly be called upon to care for them once they become pregnant. A review of the literature reveals that these patients may develop symptoms of shunt malfunction as uterine size increases. In most cases, symptoms can be managed conservatively during pregnancy and usually resolve following delivery. The presence of a CSF shunt per se, is not a contraindication to pregnancy and eventual fetal and maternal outcome has been excellent in the majority of cases. Labor and delivery should be allowed to progress naturally and interventions limited to those indicated for obstetrical reasons alone. Peripartum prophylactic antibiotics may be indicated and special care ought to be exercised if epidural analgesia or cesarian section is deemed necessary. Genetic investigations and counseling may be indicated in selected patients.

Cerebellar EndometriosisDipanka Sarma1, Pratibha Iyengar2, Thomas R. Marotta1 3, Karel G. terBrugge1, Fred Gentili4 and William Halliday5Audio Available | Share

This article presents an overview of endoscopic endonasal repair of cerebrospinal fluid (CSF) rhinorrhea. In recent years, endoscopic repair has become the standard of care for managing this condition, because it gradually replaces the traditional open transcranial approach. Discussion includes the etiologic classification of CSF rhinorrhea, management paradigm for each category, diagnosis algorithm, comprehensive description of the surgical technique, and an updated review of the literature regarding the safety and efficacy of this procedure. In addition, the authors present their experience, including 2 surgical videos demonstrating endoscopic repair of CSF rhinorrhea in 2 distinct clinical scenarios.

To describe the development and validation of a novel neuronavigation-based method, which allows the quantification of the anatomical features that define an approach, as well as real-time visualization of the surgical pyramid.The method was initially developed with commercially-available hardware for coordinate collection (a digitizer and a frameless navigation system) and software for volume rendering; dedicated neuronavigation software (ApproachViewer, part of GTx-UHN) was then developed. The accuracy of measurements and the possibility of volumetric rendering of surgical approaches simulated in a phantom were compared among three different methods and commercially-available radiological software. In the anatomy laboratory, ApproachViewer was applied to the comparative quantitative analysis of multiple neurosurgical approaches and was used by many surgeons who were untrained for the research method.The accuracy of ApproachViewer is comparable to commercially-available radiological software. In the anatomy laboratory, the method appears versatile. The system can be easily used after brief training. ApproachViewer allows for real-time evaluation and comparison of surgical approaches, as well as post-dissection analyses of collected data. The accuracy of the method depends on the navigation registration: with a 1-2 mm registration error, it is adequate for evaluation and comparison of most neurosurgical approaches.This new research method and software allows semi-automated visualization, quantification, and comparison of neurosurgical approaches in the anatomy laboratory.

With the advent of skull base endoscopy, tuberculum sellae meningiomas (TSMs) are currently operated both from the traditional transcranial (TC) route as well as the extended endonasal endoscopic trasns-sphenoidal approach (EETS). The aim of this study was to conduct a systematic review of TSMs excised via the TC route in the modern era when the EETS excision is gaining popularity.We performed a systematic review in the medical literature following the PRISMA guidelines. A medical librarian retrieved a list of 3443 articles published from 2006-2016 from the Medline, Embase and Cochrane Central databases. Two of the authors independently screened for titles and abstracts and excluded 3340 of them. We reviewed the full text of the remaining 103 articles and included in our analysis 31 that met the following inclusion criteria: 1) 5 or more cases reported; and 2) report of the extent of resection, visual outcomes and complications specifically for TSMs were documented.Thirty-one articles were selected for this systematic review with a total number of 983 patients with TSM. The mean age was 54.1±4.6 years, 75% of them being female. The follow-up was 43.9±20.7 months. The mean tumor diameter was 27.8±4.9 mm. Gross total resection was achieved in 84% while subtotal or near total resection was 14%. Vision improved, worsened and remained unchanged in 65.5%, 10.4%, and 24.7% respectively. The CSF leak rate was 3.4%. Transient or permanent pituitary dysfunction was reported in 6.9% of patients. There was a vascular injury in 5.1% of the patients with the majority (4.3%) being symptomatic. The recurrence rate was 3.8% and mortality 1.1%.In the past decade, the ETTS excision of TSMs has gained popularity and in some centers has become the approach of choice. However the TC route still remains the most common approach for most TSMs meningiomas and for the majority of neurosurgeons. The evolution of transcranial surgery including the use of minimally invasive techniques, such as endoscope-assisted transcranial resection are associated with relatively high resection rates and improved visual outcomes with low morbidity and mortality. A direct comparison with TS approaches was not done for the purpose of this review analysis. Given the limited availability and heterogeneity of comparative observational studies, a meta-analysis was deemed inappropriate.

Abstract BACKGROUND The use of high-definition endoscopes in extended transsphenoidal approaches to the suprasellar area has significantly improved visualization of its vascularization. OBJECTIVE To systematically examine the superior hypophyseal arteries (SHAs) anatomy from an endonasal endoscopic perspective. METHODS The endoscopic endonasal transsphenoidal trans-tuberculum approach was performed in 19 adult, fresh and latex injected specimens. Dissections recordings were reviewed to analyze SHAs type, number, and branches, as well as internal carotid arteries (ICA) branches that vascularized optic nerves and chiasm. RESULTS Identification of SHAs was possible in all specimens (37/38 sides). The number of SHAs varied from 1 to 3 per side (mean: 1.7). The anterior superior hypophyseal artery was visible in almost all cases (35/37 sides) and originated at the level of the carotid cave in 18/35 specimens; number of branches ranged from 1 to 6 (mean: 3.5), directed to the optic nerve (86%), chiasm (57%), infundibulum (86%), and/or parallel to the pituitary stalk (74%). The 4 main branches and patterns, originally described by McConnell in 1953, were confirmed. The posterior superior hypophyseal artery was evident in 28/37 sides with number of branches ranging from 0 to 4 (mean: 2.1), directed to the optic chiasm (50%), optic tract (32%), infundibulum (79%), and/or pituitary stalk (36%). The surgical implications of this study, together with anatomical and clinical videos, are also briefly discussed. CONCLUSION SHAs constitute a complex of anterior and posterior branches that stem from the medial ICA with different patterns, vascularizing the optic apparatus and pituitary stalk.

Radiosurgery dose rate and biologically effective dose (BED) are associated with outcomes after stereotactic radiosurgery (SRS) for functional neurosurgical conditions and some benign tumors. It is not known if these factors affect the efficacy of SRS for meningioma.To determine the association between cobalt-60 dose rate and BED on outcomes in patients with meningioma treated with SRS.A single-institution cohort of 336 patients treated between 2005 and 2018 with cobalt-based SRS for 414 separate meningioma lesions was assembled. BED was calculated using an SRS-specific monoexponential model accounting for treatment time per lesion, assuming α/β = 2.47 Gy. Cumulative incidences of local failure (LF) were reported after considering the competing risk of death, on a per-lesion basis. Multivariable analysis of LF was performed using a proportional hazards model.The most common SRS dose was 12 Gy (n = 227); 140 lesions received 14 Gy. Five-year LF was 15.6% (95% confidence interval 10.4-21.9) and 4.3% (1.4-9.8) in patients who had a dose rate of <2.95 and ≥2.95 Gy/min, respectively (P = .0375). Among 354 grade I or unresected lesions treated with SRS, BED >50 Gy2.47 was associated with a lower incidence of LF (P = .0030). Each 1 Gy/min increase in dose rate was associated with an adjusted hazard ratio of 0.53 (95% confidence interval, 0.29-0.97, P = .041) for LF. Prescription dose >12 Gy was not associated with a lower incidence of LF.Patients with meningiomas treated with lower dose rates experienced a higher incidence of LF than those treated with higher dose rates.

PURPOSE To compare rotational angiography with conventional digital subtraction angiography in the assessment of cerebral aneurysms. METHODS Conventional digital subtraction angiography and rotational angiography were compared in 57 patients investigated for the preoperative diagnosis of subarachnoid hemorrhage and in 13 patients after surgery. Images were compared for location, visibility of the aneurysmal neck, vascular branch anatomy, projection, size, presence of spasm, and shape of the aneurysm. RESULTS Rotational angiography was superior to the digital angiogram in assessing aneurysms and vascular anatomy in the following percentage of cases: 12% for location, 46% for the presence of a neck, 32% in the assessment of branch anatomy, 19% for projection, 12% for size, 3.5% for spasm, and 19% for shape. After surgery, rotational angiography more clearly demonstrated the presence or absence of a neck in 69% of the cases. CONCLUSIONS Rotational angiography often allows better visualization of vascular anatomy and therefore improves the angiographic assessment of aneurysms when compared with conventional digital subtraction angiography, making it an excellent adjunct in the investigation of subarachnoid hemorrhage. The lack of subtraction artifacts from the surgical clips and multiple angles of view also allow better assessment of the presence or absence of a residual neck in postoperative cases.

Previous preclinical studies of localized intraoperative virtual endoscopy-image-guided surgery (LIVE-IGS) for skull base surgery suggest a potential clinical benefit.The first aim was to evaluate the registration accuracy of virtual endoscopy based on high-resolution magnetic resonance imaging under clinical conditions. The second aim was to implement and assess real-time proximity alerts for critical structures during skull base drilling.Patients consecutively referred for sinus and skull base surgery were enrolled in this prospective case series.Five patients were used to check registration accuracy and feasibility with the subsequent 11 patients being treated under LIVE-IGS conditions with presentation to the operating surgeon (phase 2).Sixteen skull base patients were endoscopically operated on by using image-based navigation while LIVE-IGS was tested in a clinical setting.Workload was quantitatively assessed using the validated National Aeronautics and Space Administration Task Load Index (NASA-TLX) questionnaire.Real-time localization of the surgical drill was accurate to ~1 to 2 mm in all cases. The use of 3-mm proximity alert zones around the carotid arteries and optic nerve found regular clinical use, as the median minimum distance between the tracked drill and these structures was 1 mm (0.2-3.1 mm) and 0.6 mm (0.2-2.5 mm), respectively. No statistical differences were found in the NASA-TLX indicators for this experienced surgical cohort.Real-time proximity alerts with virtual endoscopic guidance was sufficiently accurate under clinical conditions. Further clinical evaluation is required to evaluate the potential surgical benefits, particularly for less experienced surgeons or for teaching purposes.

Sinonasal tumors can invade into the critical structures of the anterior and central skull base. Although the determination of precise tumor histology is difficult with imaging, radiology is important in helping differentiate malignant from benign disease. Imaging helps to map the anatomical extent of intracranial and intraorbital tumor, which has important implications for staging, treatment and prognosis. Imaging also helps to facilitate and plan for craniofacial or endoscopic surgical approaches and radiation planning. This paper will review the locoregional invasion patterns for sinonasal tumors, with emphasis on their imaging features. The authors will discuss the implications for staging, resection potential, choice and details of radiotherapy with or without chemotherapy and prognosis. The imaging assessment of structures and compartments that are critical to the skull base team are highlighted: orbit, cavernous sinus, anterior cranial fossa dura/intracranial tumor, lateral frontal sinus, vascular tumor encasement, perineural tumor spread and tumor effect on the surrounding bony structures.

Adult sellar atypical teratoid/rhabdoid tumor (ATRT) is a rare diagnosis that has recently been shown to be a clinicopathologically and genetically distinct variant of ATRT occurring almost exclusively in middle-aged women. Although up to one third of pediatric ATRT is caused by a familial syndrome, no previous cases of a familial adult sellar ATRT have been reported. We present the first case report of a familial germline mutation causing adult sellar ATRT and a literature review of 29 previously reported cases of sporadic adult sellar ATRT.A 51-year-old woman with a family history of brain tumors spanning 3 generations presented with visual decline and was diagnosed with an adult sellar ATRT. Genetic studies showed a heterozygous splice-site loss-of-function mutation of the INI1 gene in exon 7. Treatment included endoscopic endonasal biopsy, craniospinal irradiation, and focal tumor boost, followed by adjuvant chemotherapy.This is the first case report of a familial germline mutation causing adult sellar ATRT. This article highlights the importance of a thorough family history and genetic testing in these individuals and reviews the current genetics, histopathology, and multidisciplinary treatment approach in this rare condition.

Endoscopic endonasal approaches (EEA) to the skull base have significantly impacted the management of lesions located in the cranial base. Specifically, lesions arising from the anterior cranial fossa, such as pituitary macroadenomas, craniopharyngiomas meningiomas and craniofacial malignancies have benefited from the development of such approaches. Understanding of the anatomy of the anterior fossa is of utmost importance for the successful selection of the approach and application of surgical techniques in EEA. In the current manuscript, we review the most relevant points of surgical anatomy and nuances of the surgical technique of EEA to the anterior fossa. Anatomical landmarks for the transtuberculum transplanum and transcribriform approaches are discussed and a step-by-step description for those approaches is presented. We reinforce that safe and effective application of such techniques follow the same principles of other skull base surgery techniques: mastering of surgical anatomy, adequate case selection, correct instrumentation and surgical experience.

To evaluate the relationships between calibration dose rate, calculated biologically effective dose (BED), and clinical factors and tumor control after stereotactic radiosurgery (SRS) for acoustic neuroma.We performed a retrospective study of all patients with acoustic neuromas treated with frame-based cobalt-60 SRS at a single institution between 2005 and 2019. The calibration dose rate and cobalt-60 half-life were used to calculate the nominal dose rate during treatment. An SRS-specific monoexponential model accounting for treatment time per lesion was used to estimate BED.The study included 607 patients with 612 acoustic neuromas. Median follow-up was 5.0 years. There was no association between dose rate or BED with local failure (LF), radiologic edema, or symptomatic edema. Cystic tumors (adjusted hazard ratio 0.26, P = 0.028) were associated with lower LF, while use of SRS as salvage treatment for growing tumors (adjusted hazard ratio 4.9, P < 0.0001) was associated with higher LF. LF occurred more frequently in larger-diameter tumors, while radiologic or symptomatic edema occurred more frequently in larger-volume tumors.Radiosurgery dose rate and BED were not associated with tumor control or radiologic or symptomatic edema. Salvage SRS and larger tumors were associated with a higher LF rate, while cystic tumors were associated with a lower LF rate. Patients with larger tumors should be counseled appropriately about potential side effects and when to seek follow-up care.

Destructive spondyloarthropathy is a recently recognized disease that has not been reported in the neurosurgical literature. It is associated with spinal amyloid deposition in long-term renal failure and dialysis, and it occurs increasingly as the number of dialysis patients and their survival times increase. Clinically, there is a multisegmental and often rapidly progressive radiculomyelopathy that may require emergency stabilization. The radiological features are disc space narrowing with erosion of vertebral end plates and subarticular cysts. The pathological features include deposition of amyloid, which stains with Congo Red and antibodies to beta-2-microglobulin. We present two cases with clinical, radiological, and pathological features and a review of the literature.

Abstract Many women with cerebrospinal fluid shunts are now reaching reproductive age. Shunt malfunction may occur during pregnancy, and management requires a well-planned, combined neurosurgical and obstetrical approach. We present a case of ventriculoperitoneal shunt obstruction manifesting during the third trimester managed successfully in a conservative fashion. The literature on ventriculoperitoneal shunt malfunction during pregnancy is reviewed.

Central conduction time following median nerve stimulation has been recorded in 28 patients with infratentorial space occupying lesions.Fourteen of these (50%) showed a pro- longed central conduction time, which invariably involved that segment of the conduction time between the dorsal column nuclei generators and the generation site for P15, which we believe to be in the upper brain stem or thalamus.Three other cases showed prolongation of the N14-P15 segments though total central conduction time was within normal limits.One case with marked hydrocephalus showed prolongation both before and after the P15 wave.The possible pathophysiology of prolongation of central conduction time in these cases and the possible neural generation site of P15 are discussed.

Using light and electron microscopy and a fluorescent tracer technique, the authors have studied various aspects of injection injuries to nerves in an attempt to elucidate the pathophysiology of this condition and to help establish a rational basis for treatment. In 72 animals 144 nerve injections were carried out, using five drugs in current use and commonly administered by intramuscular injection. The nerves were examined at varying times (10 minutes to 10 weeks) following injection. Pathological alterations in the nerve were evident as early as 30 minutes after injection. The mechanism of injury appears to be a direct neurotoxic effect on the nerve fibre-both axon and Schwann cell-with a breakdown in the blood-nerve barrier.

Although the indication of endoscopic approaches for anterior skull base meningiomas (ASBM) has been progressively refined, there remains a paucity of data on recurrence rates after resection. To analyze and compare recurrence rates of ASBM resected through endoscopic endonasal (EEE) versus transcranial (TCA) approaches. We performed a retrospective analysis of patients submitted to the EEE or TCA approach for ASBM resection from May 2006 to January 2016 in our center. Clinical, radiological, and pathology data were retrieved for analysis. Tumor size, location, surgical technique, extent of resection, and tumor grade were assessed. The 2 groups were compared to identify predictors and differences regarding tumor recurrence. Fifty-two patients (17 olfactory groove meningioma [OGM] and 35 tuberculum sellae meningioma [TSM]) were included; 26 (6 OGM and 20 TSM) underwent EEE and 26 (13 OGM and 13 TSM) TCA, with a mean follow-up of 41 months. Gross total resection was achieved in 38 (73%) patients (18 [69%] in EEE and 20 [77%] in TCA). Eight (15%) patients presented with recurrence: 5 (19%) in the EEE group and 3 (11.5%) in the TCA group without a statistical difference (P = 0.69). Among the recurrences, gross total resection had been achieved in 1 case of each group. In the EEE group, 1 patient underwent TCA for a recurrent tumor and another patient was referred for radiosurgery. This study has shown an overall similar recurrence rate of ASBM regardless of the technique used. However, the analysis of larger series with longer follow-up is necessary to clearly define the indications and to fully validate the efficacy of EEE.

Endoscopic endonasal approach (EEA) has become the preferred surgical approach for resection of pituitary adenomas in most centers. This technique has a number of advantages such as improved visualization and maneuverability, when compared to microscopic transsphenoidal approach. However, the long-term results of this approach are still scarce. Ten years ago, we published our initial series of patients having undergone an endoscopic removal of their pituitary adenomas reporting favorable short-term results. This project aims to revisit the results of that series, addressing the long-term results regarding recurrence of pituitary adenomas.A retrospective analysis of consecutive, endoscopically managed pituitary adenomas in a single center from 2004-2007. Only patients with >5 years of follow up (FU) and complete follow up data were included in this study. Recurrences were defined as evidence of any new tumor growth or enlargement of previously noted residual adenoma and/or biochemical recurrence of disease activity, in cases of functioning adenomas.A total of 98 patients matched the inclusion criteria for this study. The median follow-up period was 144 months. Nonfunctioning adenoma was the most common subtype (n = 66, 67.3%), followed by GH-secreting tumors (n = 19, 19.4%), ACTH-secreting tumors (n = 7, 7.1%), prolactinomas (n = 4, 4.1%) and TSH-secreting adenomas (n = 2, 2%). Age ranges from 23 to 82 years, with median age of 53 years. Preoperative visual deficits were observed in 46 patients (46.9%) and hormonal deficits were identified in 31% of cases. 22.4% of patients had undergone a previous pituitary adenomas resection prior to treatment in our center. Surgery achieved gross total resection (GTR) and near total resection (NTR) in 89 cases (90.8%) (56.1% and 34.7%, respectively). A total of 37 cases had recurrences during FU (mean recurrence free survival: 80 months). Recurrences were observed in 34% of patients who had had GTR while recurrences were observed in 39.5% of cases that underwent subtotal resection. Most recurrences occurred after 5 years of FU and univariate analysis demonstrated previous surgery (P = 0.005), cavernous sinus invasion (P = 0.05) and Ki-67 >5% (P = 0.01) to be factors associated with higher chance of recurrence. Multivariate Cox-regression analysis demonstrate that previous surgery and Ki-67 >5% are factors associated with recurrences. Surgery and/or radiation were utilized for management of recurrences in 29/37 cases.Long-term FU analysis demonstrates that progression/recurrence of previously resected adenomas is observed in a significant number of patients, especially in those with previous/multiple surgical resections, elevated ki-67 and cavernous sinus invasion. Short-term FU may shadow real tumor control rates achieved after EEA and underscores the importance of long-term FU in these patients. Therefore, long-term FU should be pursued in all cases.

Despite its benign histopathology, the treatment of craniopharyngioma remains one of the most formidable challenges faced by skull base surgeons. The technical challenges of tackling these complex central skull base lesions are paralleled by clinical challenges related to their unique tumor biology and the often-complex decision making required. In this article, we critically appraise the most recent literature to explore the challenges and controversies surrounding the management of these lesions. The role of curative resections and the shift in the surgical paradigm toward the multidisciplinary goal-directed management approach are discussed.