Comparison of patient-reported outcomes and clinical characteristics among patients with pituitary macroadenomas and giant adenomas.

KEY POINTS: Patients with giant adenomas are more likely to have tumor extension into the paranasal sinuses. Compared to macroadenomas, giant adenomas are not associated with worse preoperative SNOT-22 scores.

Characteristics and Outcomes in Pediatric Versus Adult Craniopharyngiomas: A Systematic Review and Meta-Analysis.

BACKGROUND: Craniopharyngiomas account for 1.2% to 4.6% of all intracranial tumors. Although age at presentation is distributed bimodally, with a pediatric peak occurring between 5 and 15 years and an adult peak between 50 and 70 years, presentation, treatment, and outcome differences between these two craniopharyngioma populations have not been thoroughly characterized. OBJECTIVE: To compare treatments and outcomes between adult and pediatric craniopharyngiomas. METHODS: This is a systematic review and meta-analysis. Web of Science, MEDLINE, and Scopus databases were searched for primary studies reporting postoperative complications, functional outcomes, recurrence, and overall survival in patients with craniopharyngioma undergoing surgery. RESULTS: The search yielded 1,202 unique articles, of which 106 (n=4,202 patients) met criteria for qualitative synthesis and 23 (n=735 patients) met criteria for meta-analysis. Compared with adult, pediatric craniopharyngiomas were less likely to present with visual defects (odds ratio [OR] 0.54, 95% CI 0.36-0.80) or cognitive impairment (OR 0.29, 95% CI 0.12-0.71) and more likely with headaches (OR 2.08, 95% CI 1.16-3.73). Children presented with significantly larger tumors compared with adults (standardized mean difference 0.68, 95% CI 0.38-0.97). Comparing functional outcomes, pediatric patients sustained higher rates of permanent diabetes insipidus (OR 1.70, 95% CI 1.13-2.56), obesity (OR 3.15, 95% CI 1.19-8.31), and cranial nerve and/or neurological defects (OR 4.87, 95% CI 1.78-13.31) than adults. No significant differences were found in rates of postoperative cerebrospinal fluid leak, overall or progression-free survival, or recurrence. CONCLUSION: Adult and pediatric craniopharyngiomas seem to have fundamental differences in clinical presentation and functional outcomes. These patients frequently require multimodality treatment and are best managed with a multidisciplinary team and an individualized approach.

Intrasellar hemorrhagic chordoma masquerading as pituitary apoplexy: case report and review of the literature.

BACKGROUND AND IMPORTANCE: Chordomas are centrally located, expansile soft tissue neoplasms that arise from the remnants of the embryological notochord. Hemorrhagic presentation is exceedingly rare and can resemble pituitary apoplexy. Moreover, a purely intrasellar location of a chordoma is extremely uncommon. We report a case of a hemorrhagic intrasellar chordoma in an adult male, which presented similarly to pituitary apoplexy and was resolved with surgical resection. CLINICAL PRESENTATION: A 69-year-old male presented with a 4 week history of acute onset headache and concurrent diplopia, with significantly reduced testosterone and slightly reduced cortisol. His left eye demonstrated a sixth cranial nerve palsy. Magnetic resonance imaging of the brain showed a large hemorrhagic mass in the pituitary region with significant compression of the left cavernous sinus and superior displacement of the pituitary gland. The patient underwent an endoscopic endonasal transsphenoidal approach for the resection of the lesion. Near total resection was achieved. Final pathology revealed chordoma with evidence of intratumoral hemorrhage, further confirmed by immunopositive stain for brachyury. Post-operatively, the patient had improved diplopia and was discharged home on low dose hydrocortisone. At 3-month follow-up, his diplopia was resolved and new MRI showed stable small residual disease. CONCLUSIONS: Apoplectic chordomas are uncommon given chordoma's characteristic lack of intralesional vascularity and represent a diagnostic challenge in the sellar region. Our unique case demonstrates that despite our initial impression of pituitary apoplexy, this was ultimately a case of apoplectic chordoma that responded well to endoscopic endonasal surgery.

Tailored Surgical Access to the Cavernous Sinus and Parasellar Region: Assessment of Cavernous Sinus Entry Corridors and the Periclinoid and Pericavernous Surgical Maneuvers.

BACKGROUND: Lesions involving the cavernous sinus (CS) represent some of the most challenging pathologies of the skull base owing to the dense traversing and surrounding neurovasculature. Extradural exposure and preparation of this region, whether as initial preparation for a combined intra-extradural approach or as the main avenue of surgical exposure, can enlarge surgical corridors and minimize the need for brain retraction in this very confined space. We provide a detailed assessment of the entry corridors to the CS that are available within each approach, the surgical exposure and freedom provided by each of these corridors, and demonstrate how extradural and intradural preparation of these corridors can be used to widen the available working space and facilitate surgery. METHODS: Pterional, frontotemporal-orbital, frontotemporal-orbitozygomatic, frontotemporal-zygomatic, perilabyrinthine transtentorial, and endoscopic transnasal transsphenoidal approaches were performed on cadaveric heads to access the perisellar and CS regions. Periclinoid maneuvers (extradural cutting of the meningo-orbital band, anterior clinoidectomy, unroofing of the optic canal, opening of the superior orbital fissure, displacement of the extra-annular structures, opening of the annulus of Zinn, and interdural dissection), pericavernous maneuvers (intradural cutting of the distal dural ring, mobilization of the supraclinoid internal carotid artery, opening of the oculomotor porus, and mobilization of cranial nerve (CN) III), peritrigeminal extensions (extradural mobilization of CN V2 [maxillary] and/or V3 [mandibular]), and other surgical maneuvers were performed and evaluated. The CS was divided into 8 anatomical compartments and 9 entry corridors were described, and exposure and freedom were assessed accordingly. RESULTS: Intradurally, the standard unextended pterional, frontotemporal-orbital, and frontotemporal orbitozygomatic transsylvian approaches provided access solely to the parasellar entry corridor into the superior wall of the CS. Expanding these approaches with extradural periclinoid maneuvers allowed for subsequent application of the intradural pericavernous maneuvers and enlargement of the parasellar corridor and exposure of the carotid cave. Extradurally, the frontotemporal-orbital approach could be expanded via application of periclinoid maneuvers, which provided access to the anterior portions of the main lateral wall entry corridors. The frontotemporal-orbitozygomatic approach could also be expanded with periclinoid maneuvers to provide extradural access to all 6 lateral wall entry corridors. The extradural frontotemporal-zygomatic approach only provided exposure following interdural dissection, which allowed for access to the inferolateral entry corridors into the lateral wall. Extradural peritrigeminal extension in the frontotemporal-orbitozygomatic and frontotemporal-zygomatic approaches allows for enlargement of the supramaxillary and pre- and postmandibular corridors. The perilabyrinthine approach to the posterior wall was enlarged with opening of Dorello's canal and the endoscopic transnasal transsphenoidal approach was enlarged with opening of the optic canal. CONCLUSIONS: Targeted extradural preparation optimizes exposure and significantly improves access to deep-seated targets by enhancing surgical maneuverability through the unlocking of neurovascular structures and widening of surgical corridors without the need for additional brain retraction.

The Influence of Facility Volume and Type on Skull Base Chordoma Treatment and Outcomes.

OBJECTIVE: To evaluate the influence of facility case volume and type on skull base chordoma treatment and overall survival (OS). METHODS: The 2004-2016 National Cancer Database was queried for skull base chordoma patients receiving definitive treatment. Facilities were categorized into 2 cohorts by calculating the mean number of patients treated per facility and using cutoff numbers that were 0.5 SD above and below the computed mean to separate the groups. As, by definition of the inclusion criteria, all included facilities treated at least 1 patient, low-volume facilities were defined as treating 1 patient, and high-volume facilities were defined as treating ≥7 patients; mid-volume facilities (facilities treating ≥2 but ≤6 patients) were excluded. Differences in treatment course, outcomes, and OS by facility type were assessed. RESULTS: The study included 658 patients (44.8% female, 79.5% White). The 187 unique facilities were categorized into 95 low-volume facilities (treating 1 patient during timeline) and 26 high-volume facilities (treating ≥7 patients during timeline). Kaplan-Meier log-rank analysis demonstrated a significant positive association between facility volume and OS (P < 0.001) and an improvement in OS in patients at academic facilities (P = 0.018). On Cox proportional hazards multivariate regression after adjusting for sex, age, Charlson-Deyo comorbidity index, and insurance type, high-volume facilities and academic facilities were associated with a lower mortality risk than low-volume facilities and nonacademic facilities (P < 0.001 and P = 0.03, respectively). CONCLUSIONS: Higher facility case volume and academic facility type appear to be associated with improved survival outcomes in treatment of skull base chordomas.

Intraventricular Choroid Plexus Cavernoma Resection Using Tubular Retractor System and Exoscope Visualization: A Technical Case Report.

BACKGROUND AND IMPORTANCE: Cavernous malformations (CMs) account for approximately 5% to 10% of all CNS vascular malformations, and intraventricular CMs (IVCMs) are a rare subtype, accounting for 2.5% to 10.8% of all intracranial CMs. IVCMs can expand rapidly, leading to compression of adjacent structures, intraventricular hemorrhage, and obstructive hydrocephalus. Diagnosis is challenging because it can mimic a variety of other lesions. CLINICAL PRESENTATION: A 71-year-old man presented after a fall because of imbalance. MRI of the head showed a homogenously enhancing 2-cm mass in the posterior aspect of the right lateral ventricle, with blood layering in the right occipital horn and adjacent parietal edema and leptomeningeal enhancement, as well as a pituitary lesion. DISCUSSION: The patient underwent a right parietal craniotomy for resection of the mass. The ventricle was accessed through a transsulcal approach through the intraparietal sulcus using a tubular retractor system. The mass was arising from the choroid plexus and dissected free in a piecemeal fashion. Postoperative imaging confirmed gross total resection, and the patient had an uneventful recovery. CONCLUSION: Here, we present the first case of a choroid plexus IVCM removed using a tubular retractor system. We demonstrate that this is a safe and effective approach for this rare lesion given the minimal traction on brain parenchyma and enhanced visualization of a deep-seated cavernoma in the lateral ventricle.

Pigmented ependymoma, a tumor with predilection for the middle-aged adult: case report with methylation classification and review of 16 literature cases.

Ependymomas have rarely been described to contain pigment other than melanin, neuromelanin, lipofuscin or a combination. In this case report, we present a pigmented ependymoma in the fourth ventricle of an adult patient and review 16 additional cases of pigmented ependymoma from the literature. A 46-year-old female showed up with hearing loss, headaches, and nausea. Magnetic resonance imaging revealed a 2.5 cm contrast-enhancing cystic mass in the fourth ventricle, which was resected. Intraoperatively, the tumor appeared grey-brown, cystic, and was adherent to the brainstem. Routine histology revealed a tumor with true rosettes, perivascular pseudorosettes and ependymal canals consistent with ependymoma, but also showed chronic inflammation and abundant distended pigmented tumor cells that mimicked macrophages in frozen and permanent sections. The pigmented cells were positive for GFAP and negative for CD163 consonant with glial tumor cells. The pigment was negative for Fontana-Masson, positive for Periodic-acid Schiff and autofluorescent, which coincide with characteristics of lipofuscin. Proliferation indices were low and H3K27me3 showed partial loss. H3K27me 3 is an epigenetic modification to the DNA packaging protein Histone H3 that indicates the tri-methylation of lysine 27 on histone H3 protein. This methylation classification was compatible with a posterior fossa group B ependymoma (EPN_PFB). The patient was clinically well without recurrence at three-month post-operative follow-up appointment. Our analysis of all 17 cases, including the one presented, shows that pigmented ependymomas are most common in the middle-aged with a median age of 42 years and most have a favorable outcome. However, one patient that also developed secondary leptomeningeal melanin accumulations died. Most (58.8%) arise in the 4th ventricle, while spinal cord (17.6%) and supratentorial locations (17.6%) were less common. The age of presentation and generally good prognosis raise the question of whether most other posterior fossa pigmented ependymomas may also fall into the EPN_PFB group, but additional study is required to address that question.

Porencephalic cyst after endoscopic third ventriculostomy and Ommaya reservoir placement: case report and review of the literature.

BACKGROUND: A 13-year-old female patient was diagnosed with a tectal glioma (TG), a subgroup of astrocytoma that can result in obstructive hydrocephalus secondary to aqueductal stenosis. Endoscopic third ventriculostomy (ETV) is used to treat this type of hydrocephalus with a good success rate. Our institution performs ETV and Ommaya reservoir (OR) placement in these cases. The OR allows measurement of intracranial pressure (ICP) and cerebrospinal fluid (CSF) access and a method for performing ventricular dye studies to evaluate third ventricular stoma (TVS) patency. In this case, a porencephalic cyst (PC) developed around the OR's ventricular catheter (OVC) two and a half months after surgery. CONCLUSION: The PC is thought to have developed in association with TVS stoma closure and resolved after ETV revision.

Frailty and spontaneous intracerebral hemorrhage: Does the modified frailty index predict mortality?

OBJECTIVE: This study aims to elucidate the impact of frailty on spontaneous intracranial hemorrhage (SICH) patient outcomes in the United States. PATIENTS AND METHODS: This is a single center retrospective chart review of all adult patients (≥18 years old) admitted with a primary diagnosis of SICH due to hypertension, amyloid angiopathy, and coagulopathy from 2014-2017. The studied variables included length of stay, age, sex, ICH score variables, medications, and frailty as measured by the modified Frailty Index (mFI). RESULTS: A total of 240 patients with 248 SICH were included in the analysis. In this study, mFI was not predictive of overall mortality (p = 0.12). To further investigate this issue, patients with ICH scores of 2 or 3 were separately analyzed to assess the impact of mFI on moderate ICH cases. However, mFI was also not associated with increased hospital mortality in moderate ICH cases (p = 0.812). In bivariate Spearman analysis, mFI significantly correlated with several outcome measures including modified Rankin Scale (MRS) at discharge (p = 0.01), but ICH score also correlated with these outcomes (p < 0.001). Although ICH & mFI scores were both predictive of MRS with linear regression, multivariate demonstrated that the ICH score was the only independent risk factor for MRS (p = 0.698, p < 0.001 respectively). CONCLUSION: Frailty, as measured by the mFI, was not an independent risk factor for increased mortality or worse outcomes in SICH patients. This study does not support incorporating the mFI score for SICH patients for prognostication.

Decompressive craniectomy with scalp expansion graft using a temporary synthetic skin substitute in the pediatric population: case series and review of the literature.

INTRODUCTION: The use of decompressive craniectomy in children is controversial and often reserved for patients with refractory intracranial hypertension. Following decompression, skin closure in select cases can be challenging due to brain herniation and swelling through the craniectomy defect. In these cases, partial cortical debridement is sometimes performed. METHODS: We describe two cases in which a synthetic skin substitute was used to facilitate a tension-free closure, rather than performing a partial lobectomy. RESULTS: At 6-month follow-up, both patients are at preoperative cognitive baseline, with some residual hemiparesis. DISCUSSION: We believe that use of a synthetic skin substitute for skin closure after decompression is a suitable option for closure of traumatic scalp wounds and may contribute to improved functional outcome in patients with severe intraoperative brain swelling.

Temporal evolution of a patient with a spinal dural arteriovenous fistula on serial MRI.

INTRODUCTION: A spinal dural arteriovenous fistula is a rare type of vascular malformation. If left untreated, these fistulas can result in significant neurological deficits secondary to spinal cord infarct or hemorrhage. CASE PRESENTATION: A 70-year-old female with a longstanding history of episodic progressive bilateral lower extremity weakness and sensory disturbances was previously misdiagnosed with multiple sclerosis. Imaging revealed a T2 signal change from T7 to the conus with associated signal change and she subsequently underwent a T10-L1 laminectomy for clip ligation of a spinal dural arteriovenous fistula. Here we present the clinical and radiographic progression of one patient with a spinal dural arteriovenous fistula. DISCUSSION: Spinal dural arteriovenous fistulas are a rare but treatable cause of myelopathy, so it is important to understand its natural progression and radiologic findings as it is frequently misdiagnosed.