Open and Endoscopic Endonasal Optic Nerve Decompression for Craniofacial Fibrous Dysplasia in an Adolescent: 2-Dimensional Operative Video.

Craniofacial fibrous dysplasia (CFFD) is a benign, bony disease that may affect the skull base.1,2 Most cases are asymptomatic and observed; however, advanced disease can present with cranial neuropathy or craniofacial deformity requiring intervention.3-5 A 16-year-old adolescent girl with known CFFD involving the sphenoid and frontal bones with severe bilateral optic canal narrowing developed progressive right eye visual decline and frontal cosmetic deformity. Visual acuity worsened oculus dextrus (OD) to 20/30 with a new superior nasal scotoma and 20% loss in the retinal nerve fiber layer and remained oculus sinister (OS) 20/20. The patient was recommended a staged subfrontal craniotomy for right optic decompression and simultaneous correction cosmetic deformity followed by endonasal right optic decompression. On postoperative day one, visual acuity OD improved to 20/20; however, she developed OS visual decline to 20/800. Curiously, there were no episodes of intraoperative hypotension or additional iatrogenic compression. Use of methylprednisolone led to improvement OS 20/400. Given persistent visual decline, urgent second stage endonasal bilateral optic nerve decompression, rather than unilateral, was performed. Postoperatively, vision improved to OS 20/200. At one month, her vision improved to OD 20/15 and OS returned to 20/20 with a paracentral scotoma and 29% decline in left retinal nerve fiber layer with further improvement anticipated. This video describes a multidisciplinary, multistaged approach in treatment of optic nerve compression due to CFFD in addition to the management of unanticipated contralateral visual decline. The patient consented to the procedure and publication of her image. No Institutional Review Board/ethics committee approval was necessary for this case report.

Endoscopic endonasal resection of Rathke cleft cysts: a single-institution analysis of 148 consecutive patients.

OBJECTIVE: The traditional treatment of sellar Rathke cleft cysts (RCCs) generally involves transsellar drainage; however, suprasellar RCCs present unique challenges to appropriate management and technical complexity. Reports on overall outcomes for the endoscopic endonasal approach (EEA) for this pathology are limited. The EEA for RCCs allows three surgical techniques: marsupialization, fenestration, and fenestration with cyst wall resection. METHODS: The authors performed a retrospective review of consecutive patients with RCCs that had been treated via an EEA at a single institution between January 2004 and May 2021. Marsupialization entailed the removal of cyst contents while maintaining a drainage pathway into the sphenoid sinus. Fenestration involved the removal of cyst contents, followed by separation from the sphenoid sinus, often with a free mucosal graft or vascularized nasoseptal flap. Cyst wall resection, either partial or complete, was added to select cases. RESULTS: A total of 148 patients underwent an EEA for RCC. Marsupialization or fenestration was performed in 88 cases (59.5%) and cyst wall resection in 60 (40.5%). Cysts were classified as having a purely sellar origin (43.2%), sellar origin with suprasellar extension (37.8%), and purely suprasellar origin (18.9%). Radiological recurrence was demonstrated in 22 cases (14.9%) at an average 39.7 months' follow-up (median 45 months, range 0.5-99 months), including 13 symptomatic cases (8.8%). Cases with cyst wall resection had no significantly different rate of recurrence (11.7% vs 15.9%, p = 0.48) or postoperative permanent anterior pituitary dysfunction (21.6% vs 12.5%, p = 0.29) compared to those of fenestrated and marsupialized cases. There was no significant difference in postoperative permanent posterior pituitary dysfunction based on technique, although such dysfunction tended to worsen with cyst wall resection (13.6% vs 4.0%, p = 0.09). Based on cyst location, purely suprasellar cysts were more likely to have a radiological recurrence (28.6%) than sellar cysts with suprasellar extension (12.5%) and purely sellar cysts (9.4%; p = 0.008). Most notably, of the 28 purely suprasellar cysts, selective cyst wall resection significantly improved the long-term (10-year) recurrence risk compared to fenestration alone (17.4% vs 80.0%, p = 0.0005) without any significant added risk of endocrinopathy. CONCLUSIONS: Endoscopic endonasal marsupialization or fenestration of sellar RCCs may be the ideal treatment strategy, whereas purely suprasellar cysts benefit from partial cyst wall resection to prevent recurrence. Selective cyst wall resection reduced long-term recurrence rates without significantly increasing rates of hypopituitarism.

Neuroimaging with Rotterdam Scoring System and long-term outcomes in severe traumatic brain injury patients.

PURPOSE: The Rotterdam Scoring System (RSS) attempts to prognosticate early mortality and early functional outcome in patients with traumatic brain injury (TBI) based on non-contrast head computed tomography (CT) imaging findings. The purpose of this study was to identify the relationship between RSS scores and long-term outcomes in patients with severe TBI. METHODS: Consecutively treated patients with severe TBI enrolled between 2008 and 2011, in the prospective, observational, Brain Trauma Research Center database were included. The Glasgow Outcome Scale (GOS) was used to measure long-term functional outcomes at three, six, 12, and 24 months. GOS scores were categorized into favorable (GOS = 4-5) and unfavorable (GOS = 1-3) outcomes. RSS scores were calculated at the time of image acquisition. RESULTS: Of the 89 patients included, 74 (83.4%) were male, 81 (91.0%) were Caucasian, and the mean age of the cohort was 41.9 ± 18.5 years old. Patients with an RSS score of 3 and lower were more likely to have a favorable outcome with increased survival rates than patients with RSS scores greater than 3. CONCLUSIONS: The RSS score determined on the head CT scan acquired at admission in a cohort of patients with severe TBI correlated with long-term survival and functional outcomes up to two years following injury.

A novel classification and management scheme for craniocervical junction disorders with ventral neural element compression.

OBJECTIVE: Craniocervical junction (CCJ) pathologies with ventral neural element compression are poorly understood, and appropriate management requires accurate understanding, description, and a more uniform nomenclature. The aim of this study was to evaluate patients to identify anatomical clusters and better classify CCJ disorders with ventral compression and guide treatment. METHODS: A retrospective review of adult and pediatric patients with ventral CCJ compression from 2008 to 2022 at a single center was performed. The incidence of anatomical abnormalities and compressive etiologies was assessed. Surgical approach, radiographic data, and outcomes were recorded. Association rules analysis (ARA) was used to assess variable clustering. RESULTS: Among 51 patients, the main causes of compression were either purely bony (retroflexed dens [n = 18]; basilar invagination [BI; n = 13]) or soft tissue (degenerative pannus [n = 16]; inflammatory pannus [n = 2]). The primary cluster in ARA was a retroflexed dens, platybasia, and Chiari malformation (CM), and the secondary cluster was BI, C1-2 subluxation, and reducibility. These, along with degenerative pannus, formed the three major classes. In assessing the optimal treatment strategy, reducibility was evaluated. Of the BI cases, 12 of the 13 patients had anterolisthesis of C1 that was potentially reducible, compared with 2 of the 18 patients with a retroflexed dens (both with concomitant BI), and no pannus cases. The mean C1-2 facet angle was significantly higher in BI at 32.4°, compared with -2.3° in retroflexed dens and 8.1° in degenerative pannus (p < 0.05). Endonasal decompression with posterior fixation was performed in 48 (94.0%) of the 51 patients, whereas posterior reduction/fixation alone was performed in 3 patients (6.0%). Of 16 reducible cases, open posterior reduction alone was successful in 3 (60.0%) of 5 cases, with all successes containing isolated BI. Reduction was not attempted if vertebral anatomy was unfavorable (n = 9) or the C1 lateral mass was absent (n = 5). The mean follow-up was 28 months. Symptoms improved in 88.9% of patients and were stable in the remaining 11.1%. Tracheostomy and percutaneous G-tube placement occurred in 7.8% and 11.8% of patients, respectively. Reoperation for an endonasal CSF leak repair or posterior cervical wound revision both occurred in 3.9% of patients. CONCLUSIONS: In classifying, one cluster caused decreased posterior fossa volume due to an anatomical triad of retroflexed dens, platybasia, and CM. The second cluster caused pannus formation due to degenerative hypertrophy. For both, endonasal decompression with posterior fixation was ideal. The third group contained C1 anterolisthesis characterized by a steep C1-2 facet angle causing reducible BI. Posterior reduction/fixation is the first-line treatment when anatomically feasible or endonasal decompression with in situ posterior fixation when anatomical constraints exist.

Bow Hunter Syndrome: An Illustrative Case and Operative Management.

The differential for vertebrobasilar insufficiency is wide and can be caused by posterior circulation infarcts, steal-type phenomena, or other systemic causes. In the absence of imaging findings explaining symptomology, the utility of appropriate history gathering and dynamic angiography cannot be understated in identifying Bow Hunter's syndrome, a rare cause of dynamic vertebrobasilar insufficiency. We present a case of a 69-year-old man who complained of presyncope and severe dizziness when turning his head towards the right. On examination he had no radiculopathy but did have objective evidence of myelopathy. Computed tomography imaging and dynamic angiography demonstrated C3-C4 right uncovertebral joint hypertrophy and near complete stenosis of the right vertebral artery with dynamic head position towards the right. Given vertebrobasilar insufficiency and myelopathy, he was taken to the operating room for C3-C4 anterior cervical discectomy and fusion with vertebral artery decompression (Video 1). The patient provided consent for the procedure. Standard anterior cervical neck dissection was undertaken with additional platysmal undermining to facilitate exposure of the right uncovertebral joint and transverse processes. The vertebral artery was first decompressed above and below the area of most significant stenosis at the respective transverse foramina before the hypertrophied uncovertebral joint was removed. Next, discectomy and posterior osteophyte removal were completed in typical fashion followed by graft, plate, and screw placement. Postoperatively the patient had immediate resolution of symptoms and continued so at eight month follow-up. Imaging demonstrated return to normal caliber of the right vertebral artery and successful decompression.

Endoscopic Endonasal Far-Medial Approach: 2-Dimensional Operative Video.

INDICATIONS CORRIDOR AND LIMITS OF EXPOSURE: Endoscopic endonasal far-medial approach provides an effective and safe corridor to access the parasagittal structures of the lower clivus such as the medial jugular tubercle (JT) and occipital condyle (OC) for lesions that displace neurovascular structures laterally. ANATOMIC ESSENTIALS NEED FOR PREOPERATIVE PLANNING AND ASSESSMENT: Parapharyngeal internal carotid arteries (ICAs) run posterolateral to the eustachian tubes and lateral to the OC. The supracondylar groove is a superficial landmark for the hypoglossal canal, which divides the lateral extension of clivus into the JT and OC. ESSENTIAL STEPS OF THE PROCEDURE: Typically, approach starts with opening of the sphenoid sinus to localize the paraclival ICA. An "inverted U" rhinopharyngeal (RP) flap exposing the supracondylar groove and lower clivus. Doppler and navigation can confirm the course of the ICA. Drilling is started in the midline in the lower clivus and extended laterally to expose the hypoglossal canal, JT, and OC. PITFALLS/AVOIDANCE OF COMPLICATIONS: Neurovascular injuries can be avoided by using intraoperative Doppler and nerve stimulator. Multilayer reconstruction with vascularized nasoseptal (NSF) and RP flaps minimize postoperative cerebrospinal fluid leak. VARIANTS AND INDICATIONS FOR THEIR USE: The contralateral transmaxillary approach provides an increased angle of access behind foramen lacerum and the petrous ICA.The endoscopic endonasal far-medial approach can be used for a variety of pathologies, including petroclival or JT meningiomas, chordomas and chondrosarcomas, and hypoglossal schwannomas, inferiorly extending cholesterol granulomas and even rare, ventral posterior inferior cerebellar artery aneurysms.The patients consented to the procedure.

Long-term Benefits for Younger Patients with Aggressive Immediate Intervention following Severe Traumatic Brain Injury: A Longitudinal Cohort Analysis of 175 Patients from a Prospective Registry.

BACKGROUND: The prevalence of traumatic brain injury (TBI) continues to rise, in part as a reflection of a growing elderly population. Concomitantly, nihilism may exist following substantial neurotrauma from a myriad of commonplace mechanisms, such as traffic incidents, assaults, or falls. OBJECTIVE: This study assesses long-term outcomes following aggressive surgical intervention with invasive neuromonitoring to guard against nihilism, especially for patients with advantageous characteristics such as younger age. METHODS: A consecutive series of patients with severe TBI treated between 2008 and 2018 and enrolled into the Brain Trauma Research Center (BTRC) database, an Institutional Review Board (IRB 19030228) approved prospective, longitudinal cohort study, were extracted. Demographic and clinical data were analyzed. Long-term functional outcome was recorded with the eight-point Glasgow Outcome Scale-Extended (GOS-E) score at 3-, 6-, 12-, and 24-months by trained, qualified neuropsychology technicians. Chi-squared and analysis of variance tests were used to evaluate the relationship of age groups between different variables. RESULTS: For this analysis, 175 patients with severe TBI who were enrolled in the BTRC database and required decompressive hemicraniectomy during the study period were included. Over one-third of the patients with a severe TBI, who were aged 35 years and younger, had a favorable outcome. CONCLUSIONS: Despite enduring a severe TBI, a substantial percentage of younger patients achieved favorable outcomes following aggressive treatment. As such, establishing a prognosis should be deferred to allow for recovery via individualized rehabilitation, multidisciplinary support, and community reintegration programs to cope with various long-term psychological, cognitive, and functional disabilities.

Endoscopic Techniques in Vascular Neurosurgery.

This section reviews the selection criteria and best practices for endoscopic cerebrovascular surgery, focusing on purely endoscopic endonasal approaches. Although these approaches still play a limited role in open vascular neurosurgery, they offer a robust and potentially safer technique for establishing visualization and vascular control of particular, well-selected pathologies, such as aneurysms of the proximal circulation; this requires strong, multidisciplinary experience with endoscopic anatomy and surgical technique, advanced reconstruction techniques, and instruments designed to be maneuvered within this relatively novel corridor and application.

Low Preoperative Prealbumin Levels Are a Strong Independent Predictor of Postoperative Cerebrospinal Fluid Leak Following Endoscopic Endonasal Skull Base Surgery.

OBJECTIVE: Prealbumin levels correlate with overall nutritional status, and low values are associated with poor wound healing. We investigated whether low preoperative prealbumin levels predict risk of endoscopic endonasal skull base surgery (EESBS) reconstruction failure, as demonstrated by postoperative cerebrospinal fluid (CSF) leak and/or infection. METHODS: Between October 2018 and February 2020, 98 patients with documented preoperative prealbumin levels were prospectively followed. The incidence of CSF leak and infection in patients with low prealbumin levels (≤20 mg/dL) was compared with those with normal prealbumin levels (>20 mg/dL). Numerous factors previously shown to influence CSF leak rates were assessed. Both univariate and multivariable analyses were performed to identify independent predictive factors. RESULTS: Within this prospectively gathered patient cohort composed of >95% "high-risk" expanded EESBS, 14 of 98 patients (14.3%) experienced a postoperative CSF leak. Factors univariately associated with postoperative complications at the 0.2 level of significance were used in a multivariable model. Low prealbumin levels (≤20 mg/dL) proved to be a strong independent predictive factor associated with a 5-fold increased risk of postoperative CSF leak (odds ratio 5.01, P = 0.01), and postoperative surgical-site infection (P = 0.0009). These associations remained after controlling for multiple other factors, including body mass index, surgical pathology, previous EESBS, risk assessment index, and high- versus low-flow intraoperative CSF leaks. CONCLUSIONS: Preoperative prealbumin levels are an independent predictor of EESBS associated CSF leak and infection. Future studies are needed to investigate the utility of screening and correcting prealbumin levels to limit postoperative complications.

Ventriculoperitoneal Shunt Failure Due to Distal Peritoneal Catheter Kinking.

BACKGROUND Hydrocephalus is a common condition associated with high morbidity and mortality rates. Despite advancements in shunt systems and valve designs, complications associated with ventriculoperitoneal (VP) shunts are steadily recognized and reported in the literature. Here, we present an unusual case of VP shunt failure due to catheter kinking at the site of the slits in the distal peritoneal catheter. CASE REPORT A 30-year-old woman with type I Chiari malformation, prior suboccipital craniectomy, and shunted hydrocephalus with prior revisions presented with 2 months of progressive, low-pressure headaches. Shunt series X-rays demonstrated kinking of the distal peritoneal catheter. A computed tomography (CT) scan showed interval enlargement of her ventricles concerning for shunt failure, which prompted return to the operating room. During shunt revision, her valve was nonfunctioning with loss of resistance and her distal catheter was kinked at the most proximal peritoneal slit. Postoperative shunt series X-rays demonstrated an intact shunt system without kinking or discontinuity and a CT of her head showed interval decease in the caliber of her ventricles. CONCLUSIONS Distal peritoneal catheter kinking at the site of slits is an unusual complication of VP shunts and should be considered. Surgeons should add this possibility to the differential diagnosis of shunt malfunction when an imaging irregularity is identified in the peritoneal catheter.

Neuroophthalmological manifestations of congenital aqueductal stenosis.

OBJECTIVE: Congenital aqueductal stenosis (CAS) is a common etiology of hydrocephalus that occurs in a subset of infants and may be linked to an increased incidence of ophthalmological abnormalities and delayed developmental milestones. Although hydrocephalus is common and widely studied, sparse literature exists on patients with isolated (no identifiable genetic link) CAS along with analysis of ophthalmological manifestations. In this study, the authors sought to describe the ophthalmological abnormalities and delayed developmental milestones of patients with isolated CAS. METHODS: Data of patients with CAS were prospectively entered and monitored in a surgical database maintained by the Department of Neurological Surgery at Children's Hospital of Pittsburgh from January 2005 to October 2016. Patients with a family history of congenital hydrocephalus, positive testing for genetic forms of aqueductal stenosis, other congenital abnormalities suggesting an underlying genetic syndrome, and stenosis/obstruction due to secondary causes were excluded from this study. Prenatal and perinatal history, CSF diversion history, and a variety of outcomes, including ophthalmological deficits and developmental milestones, were collected and analyzed. RESULTS: A total of 41 patients with isolated CAS were identified, with a mean follow-up duration of 6 years. Among that cohort, 26 patients (63.4%) developed neuroophthalmological complications, which were further stratified. Fourteen patients (34.1%) developed strabismus and 11 (26.8%) developed astigmatism, and 1 patient (2.4%) with papilledema was recorded. Among patients with ophthalmological abnormalities, 76.9% had delayed developmental milestones (p = 0.045). CONCLUSIONS: Patients with CAS were found to have increased risk of ophthalmological abnormalities requiring correction, along with an increased risk of delayed developmental milestones. Importantly, there was a significant correlation between the development of ophthalmological abnormalities and delayed developmental milestones that was independent of CSF diversion history. Larger patient cohort studies are required to explore whether earlier development of hydrocephalus, as is the case in CAS, causes elevated rates of neurological and ophthalmological complications, and if earlier CSF diversion correlates with improved outcomes.

Staged Repositioning in Endoscopic Endonasal Odontoidectomy Maximizes Decompression While Allowing Preservation of the C1 Anterior Arch: A Technical Note.

BACKGROUND: Preservation of the anterior arch of C1 in endoscopic endonasal odontoidectomy has been proposed as an alternative to complete C1 arch resections, potentially affording less destabilization of the craniocervical junction. Nonetheless, this approach may limit the decompression achieved. In this case, intraoperative repositioning allowed maximal decompression while preserving the anterior arch of C1. METHODS: A 79-year-old woman presented with suboccipital pain caused by an expansile and compressive mass centered on the dens. Notably, the mass occluded both vertebral arteries resulting in small cerebellar strokes. An endoscopic endonasal approach for diagnosis and decompression was performed followed by posterior fixation. RESULTS: Given the significant compression, the patient was initially positioned in slight cervical extension. After rhinopharyngeal flap harvest, the top half of the anterior arch of C1 was resected, maintaining its structural integrity. The odontoidectomy was completed flush to the superior border of the reduced C1 arch. After an intraoperative computed tomography (CT) scan, performed in a neutral position, the patient was then repositioned with cervical flexion. This maneuver presented the residual odontoid above the C1 arch, but, given the partial removal of the dens, it did not result in any change in neuromonitoring. Further odontoid resection was then completed and follow-up CT scan revealed maximal dens removal, extending below the C1 anterior arch in neutral position. CONCLUSIONS: In cases of odontoid/atlantoaxial pathology causing significant neural compression, staged intraoperative repositioning can safely maximize the odontoidectomy, while affording preservation of the structural integrity of the anterior arch of C1.

Impact of Coronavirus Disease 2019 Shutdown on Neurotrauma Volume in Pennsylvania.

OBJECTIVE: The 2020 coronavirus disease 2019 (COVID-19) pandemic resulted in state-specific quarantine protocols and introduced the concept of social distancing into modern parlance. We assess the impact of the COVID-19 pandemic on neurotrauma presentations in the first 3 months after shutdown throughout Pennsylvania. METHODS: The Pennsylvania Trauma Systems Foundation was queried for registry data from the Pennsylvania Trauma Outcomes Study between March 12 and June 5 in each year from 2017 to 2020. RESULTS: After the COVID-19 shutdown, there was a 27% reduction in neurotrauma volume, from 2680 cases in 2017 to 2018 cases in 2020, and a 28.8% reduction in traumatic brain injury volume. There was no significant difference in neurotrauma phenotype incurred relative to total cases. Injury mechanism was less likely to be motor vehicle collision and more likely caused by falls, gunshot wound, and recreational vehicle accidents (P < 0.05). Location of injury was less likely on roads and public locations and more likely at indoor private locations (P < 0.05). The proportion of patients with neurotrauma with blood alcohol concentration >0.08 g/dL was reduced in 2020 (11.4% vs. 9.0%; P < 0.05). Mortality was higher during 2020 compared with pre-COVID years (7.7% vs. 6.4%; P < 0.05). CONCLUSIONS: During statewide shutdown, neurotrauma volume and alcohol-related trauma decreased and low-impact traumas and gunshot wounds increased, with a shift toward injuries occurring in private, indoor locations. These changes increased mortality. However, there was not a change in the types of injuries sustained.

COVID-19 Impact in Neurosurgery Residency: Grit During Pandemic.

BACKGROUND: The coronavirus disease-19 (COVID) pandemic has presented a significant challenge to health care providers. Neurosurgical patients are often critically ill and at particularly high risk for COVID, and the pandemic has produced ever-shifting circumstances to allow their continued care. This study explores the psychologic impact of the pandemic on neurosurgical residents at a single institution. METHODS: Residents completed the Perceived Stress Scale (PSS) and the Inventory of Depressive Symptomology 30 (IDS-30), two validated surveys, before and during the pandemic. PSS scores range from 0 to 40 with higher scores indicative of increased stress. IDS-30 ranges from 0 to 84 with higher scores indicating more severe levels of depression. Survey results were compared collectively among residents and statistically analyzed. RESULTS: The surveys had 19 out of 28 total responders (68%). Mean score on the PSS was 16.1 prepandemic and 14.7 during the pandemic (P > 0.05). The mean score on the IDS-30 was 14.7 prepandemic and 12.0 during the pandemic (P > 0.05). Matching the survey questionnaire results by each resident revealed an average decrease of 2.3 and 2.4 for the PSS and IDS-30, respectively. CONCLUSIONS: There were no significant changes in perceived stress or depressive symptoms among neurosurgical residents during the pandemic.

Endoscopic endonasal surgery for anterior cranial fossa meningiomas.

Meningiomas along the anterior skull base arise from the midline but have historically been resected via open cranial approaches with lateral to medial trajectories. The endoscopic endonasal approach (EEA) offers a direct, inferomedial approach which has demonstrated several superior qualities for their resection. These meningiomas include tuberculum sellae, planum sphenoidale, and olfactory groove meningiomas. While early gross total resection (GTR) was lower than open approaches, EEA has currently achieved comparable rates of GTR and significantly improved postoperative visual outcomes. Rate of cerebrospinal fluid (CSF) leak was one of the early complicating features preventing widespread use of EEA. However, CSF leak rates have dramatically fallen into a tolerable range with introduction of the vascularized nasoseptal flap. Olfactory groove meningiomas often present with anosmia which is persistent after endonasal approach. Rates of other complications have proven similar between EEA and open approaches and include: vascular injury, infection, morbidity, and mortality. With the appropriate team and experience, EEA for anterior skull base meningiomas is increasingly becoming the standard for resection of these lesions. However, there are certain anatomic considerations, patient features, and other aspects which may favor the open approach over EEA, and vice versa; these must be carefully and judiciously evaluated preoperatively. Overall, resection and recurrence rates are comparable, complication rates fall within a very acceptable range, and patients experience superior cosmesis and improved visual outcome with this approach.

Seizure Risk following Open and Expanded Endoscopic Endonasal Approaches for Intradural Skull Base Tumors.

Objectives The incidence of seizures following a craniotomy for tumor removal varies between 15 and 20%. There has been increased use of endoscopic endonasal approaches (EEAs) for a variety of intracranial lesions due to its more direct approach to these pathologies. However, the incidence of postoperative seizures in this population is not well described. Methods This is a single-center, retrospective review of consecutive patients undergoing EEA or open craniotomy for resection of a cranial base tumor between July 2007 and June 2014. Patients were included if they underwent an EEA for an intradural skull base lesion. Positive cases were defined by electroencephalograms and clinical findings. Patients who underwent a craniotomy to remove extra-axial skull base tumors were analyzed in the same fashion. Results Of the 577 patients treated with an EEA for intradural tumors, 4 experienced a postoperative seizure (incidence 0.7%, 95% confidence interval [CI]: 0.002-0.02). Over the same period, 481 patients underwent a craniotomy for a skull base lesion of which 27 (5.3%, 95% CI: 0.03-0.08) experienced a seizure after surgery. The odds ratio for EEA was 0.13 (95% CI: 0.05-0.35). Both populations were different in terms of age, gender, tumor histology, and location. Conclusion This study is the largest series looking at seizure incidence after EEA for intracranial lesions. Seizures are a rare occurrence following uncomplicated endonasal approaches. This must be tempered by selection bias, as there are inherent differences in which patients are treated with either approach that influence the likelihood of seizures.

Endoscopic Endonasal Approach for Craniopharyngiomas with Intraventricular Extension: Case Series, Long-Term Outcomes, and Review.

BACKGROUND: Traditionally, craniopharyngiomas with intraventricular extension were approached transcranially; however, endoscopic approaches are now increasingly used. We sought to study the endoscopic endonasal approach (EEA) in the setting of complex craniopharyngiomas with intraventricular extension and to compare it with existing literature. METHODS: Patients undergoing EEA for resection of craniopharyngioma with ventricular involvement from 2002 to 2015 were retrospectively reviewed. Outcomes were compared with previously published EEA and transcranial approach (TCA) studies for all craniopharyngioma locations. RESULTS: Sixty-two patients were included. Average tumor and intraventricular volume were 13.93 cm3 and 2.61 cm3, respectively. Patients presented with visual impairment, endocrinopathy, and, headache. Gross total resection (GTR) was achieved in 47% of all cases and increased to 77% after 2012 Approximately 98% experienced improvement or stability of vision. Postoperative cerebrospinal fluid (CSF) leak and meningitis rates were 19% and 8.1%, respectively. However, nasoseptal flap (NSF) use reduced CSF leak rate to 10%. Six (9.6%) patients required shunting before resection and 25% were shunted postoperatively. Seven of 10 patients (70%) treated before NSF use required shunting, whereas only 7 of 46 (15%) required shunting with NSF reconstruction. Review demonstrated similar outcomes between the present cohort and EEA or TCA for all craniopharyngioma locations. TCA had a greater GTR, however, with large study variation. EEA showed improved visual outcomes but also increased CSF leaks. CONCLUSIONS: EEA for craniopharyngiomas with intraventricular extension shows similar outcomes to TCA and EEA for all craniopharyngiomas, expanding this anatomic limit. Given ventricular involvement, CSF leak rates are expectedly high. GTR increased and CSF leak rates dramatically decreased with time, suggestive of the steep learning curve to complex resection.

Familial Cerebral Cavernous Malformation Syndrome with Concomitant Fourth Ventricular Ependymoma: True Association or Mere Coincidence?

Familial cerebral cavernous malformation syndromes are most commonly caused by mutations in one of three genes. The overlap of these genetic malformations with other acquired neoplastic lesions and congenital malformations is still under investigation. To the best of our knowledge, the concurrent occurrence of familial cavernous malformations and ependymoma has not been previously reported in the literature. Herein, we describe a patient with familial cerebral cavernous malformation syndrome and posterior fossa ependymoma. A 17-year-old asymptomatic male was referred to our outpatient neurosurgery clinic after genetic testing identified a familial KRIT1 (CCM1) mutation. The patient's sister had presented with a seizure disorder previously; multiple cavernous malformations were discovered, and a symptomatic large cavernous malformation required a craniotomy for resection. Two years later, she was diagnosed with follicular thyroid cancer due to HRAS (c.182A>G) mutation. The patient and his sister were found to have a novel germline KRIT1 disease-causing variant (c.1739deletion, p.ASN580Ilefs*2) and a variant of uncertain significance, potentially pathogenic (c.1988 A>G, p.Asn663Ser) in cis in CCM1 (KRIT1), of paternal inheritance. Due to the presence of genetic abnormalities, the patient underwent screening imaging of his neuraxis. Multiple cavernous malformations were identified, as was an incidental fourth ventricular mass. Resection of the fourth ventricular lesion was performed, and histopathological examination was consistent with ependymoma. We report a unique case of posterior fossa ependymoma in an individual with a familial cerebral cavernous malformation syndrome and a novel genetic abnormality in KRIT1. The association of these two findings may be valuable in determining a potential genetic association between the two pathologies and elucidating the pathogenesis of both cavernous malformations and ependymomas.