Transpalpebral mini-orbitozygomatic approach for nonvascular skull base lesions: a single neurosurgeon's experience.

OBJECTIVE: The authors aim to describe the advantages, utility, and disadvantages of the transpalpebral mini-orbitozygomatic (MOZ) approach for tumors of the lateral and superior orbit, orbital apex, anterior clinoid, anterior cranial fossa, middle cranial fossa, and parasellar region. METHODS: The surgical approach from skin incision to closure is described while highlighting key technical and anatomical considerations, and cadaveric dissection demonstrates the surgical steps and focuses on important anatomy. Intraoperative images were included to supplement the cadaveric dissection. A retrospective review of adults who had undergone the MOZ approach for nonvascular pathology performed by a single neurosurgeon from 2017 to 2023 was included in this institutional review board-approved study. Descriptive statistics was used to summarize the data. Four representative cases were included to demonstrate the utility of the MOZ approach. RESULTS: The study included 65 patients (46 female, 19 male), average age 54.84 years, who had undergone transpalpebral MOZ surgery. Presenting symptoms included visual changes (53.8% of cases), vision loss (23.1%), diplopia (21.8%), and proptosis (13.8%). The optic nerve and optic chiasm were involved in 32.3% and 10.8% of cases, respectively. The most common pathology was meningioma (81.5% of cases), and gross-total resection was achieved in 50% of all cases. Major complications included an infection and a carotid injury. Improvement of preoperative symptoms was reported in 92.2% of cases. Visual acuity improved in 12 patients. The mean follow-up was 8.57 ± 8.45 months. CONCLUSIONS: The MOZ approach is safe and durable. The transpalpebral incision provides better cosmesis and functional outcomes than those of standard anterolateral approaches to the skull base. Careful consideration of the limits of the approach is paramount to appropriate application on a case-by-case basis. Further quantitative anatomical studies can help to define and compare the utility of the approach to open cranio-orbital and endoscopic transorbital approaches.

Comparison of Follow-Up Length-Matched Single-Center Myelomeningocele Postnatal Closure Cohort to the Management of Myelomeningocele Study (MOMS) Trial Results.

OBJECTIVE: We sought to compare our large single-institution cohort of postnatal myelomeningocele closure to the 2 arms of the Management of Myelomeningocele Study (MOMS) trial at the designated trial time points, as well as assess outcomes at long-term follow-up among our postnatal cohort. METHODS: A single-institutional retrospective review of myelomeningocele cases presenting from 1995 to 2015 at Children's Hospital of Pittsburgh was performed. We compared outcomes at 12 and 30 months to both arms of the MOMS trial and compared our cohort's outcomes at those designated time points to our long-term outcomes. Univariate statistical analysis was performed as appropriate. RESULTS: One-hundred sixty-three patients were included in this study. All patients had at least 2-year follow-up, with a mean follow-up of 10 years (range 2-20 years). There was no difference in the overall distribution of anatomic level of defect. Compared to our cohort, the prenatal cohort had a higher rate of tethering at 12 months of age, 8 versus 1.8%. Conversely, the Chiari II decompression rate was higher in our cohort (10.4 vs. 1.0%). At 30 months, the prenatal cohort had a higher rate of independent ambulation, but our cohort demonstrated the highest rate of ambulation with or without assistive devices among the 3 groups. When comparing our cohort at these early time points to our long-term follow-up data, our cohort's ambulatory function decreased from 84 to 66%, and the rate of detethering surgery increased almost 10-fold. CONCLUSIONS: This study demonstrated that overall ambulation and anatomic-functional level were significantly better among our large postnatal cohort, as well as having significantly fewer complications to both fetus and mother, when compared to the postnatal cohort of the MOMS trial. Our finding that ambulatory ability declined significantly with age in this patient population is worrisome for the long-term outcomes of the MOMS cohorts, especially given the high rates of cord tethering at early ages within the prenatal cohort. These findings suggest that the perceived benefits of prenatal closure over postnatal closure may not be as substantial as presented in the original trial, with the durability of results still remaining a concern.

Lumbar drains: Practical understanding and application for the otolaryngologist.

INTRODUCTION: Lumbar drains are frequently used in patients with otolaryngologic concerns. These can be used therapeutically or prophylactically with the primary purpose being to modulate CSF pressure. Within otolaryngology, lumbar drains are most frequently used for cerebrospinal fluid leaks - either due to cerebrospinal fluid fistulas or in skull base surgery as these allow for potential healing of the defect. While not typically placed by otolaryngologists, a basic understanding of lumbar drains is beneficial in the context of patient management. MANAGEMENT: A lumbar drain is inserted into the intrathecal space in a patient's lumbar spine. Though considered to be a benign procedure, complications are relatively frequent, and adjustment or replacement of the drain may be required. Complications include infection, epidural bleeding, retained hardware, sequelae of relative immobility, or may relate to over-drainage, ranging from mild headache to cranial neuropathies, altered mental status, pneumocephalus, intracranial hemorrhage, and death. While in place, neurologic exams should be performed routinely and should include motor and sensory exams of the lower extremities. A patient should be monitored for fevers, nuchal rigidity, and other signs of infection or meningitis. The CSF fluid should be grossly examined to identify changes, but routine laboratory tests are not typically run on the fluid itself. Drainage rates will vary usually between 5 and 20 mL per hour and must be frequently reassessed and adjusted based upon signs of intracranial hypotension. Drains should be removed when appropriate and should not be left in more than 5 days due to the increased infectious risk. CONCLUSION: Lumbar drains are important tools used in patients with otolaryngologic pathologies. Otolaryngologists and otolaryngology residents should be familiar with these catheters to determine if they are working correctly and to identify adverse effects as early as possible.

The impact of mode of delivery on infant neurologic outcomes in myelomeningocele.

BACKGROUND: Controversy exists regarding the optimal route of delivery for fetuses who are diagnosed prenatally with myelomeningocele. Current recommendations are based partly on antiquated studies with questionable methods. All studies that have been published to date suffer from nonstandardized outcome measures, selection bias, and small sample size. The larger studies are >15 years old. OBJECTIVE: The purpose of this study was to provide information for evidence-based decision-making regarding the impact of route of delivery on motor outcomes for pediatric patients with prenatally were diagnosed myelomeningocele in a well-defined retrospective cohort. STUDY DESIGN: Medical records were reviewed retrospectively for all neonates who had been diagnosed with a myelomeningocele at birth from 1995-2015 within the University of Pittsburgh Medical Center system, as identified through the Children's Hospital of Pittsburgh Neurosurgery Department operative database. Records were matched with maternal records with the use of the Center for Assistance in Research that used eRecord. Data from 72 maternal-neonatal pairs were analyzed for multiple variables. The primary outcome measure was the difference between the functional and anatomic motor levels in the child at the age of 2 years, stratified by mode of delivery and presence or absence of labor. The sample size necessary to detect a difference between the groups with power of 0.8 and significance of .05 was calculated to be 52 subjects total (26 per group). RESULTS: Functional levels were slightly better than predicted by anatomic levels for all pediatric patient groups, regardless of mode of delivery or presence of labor. Anatomic levels were slightly lower (better), and defects were smaller for those infants who underwent vaginal delivery or a trial of labor, likely attributable to selection bias. Attempts to correct for this selection bias did not change the results. No other outcomes that were analyzed were associated significantly with mode of delivery or presence of labor. CONCLUSION: No benefit to motor function from delivery by cesarean section or avoidance of labor was demonstrated statistically in this mother-infant cohort.

Endoscopic transnasal skull base surgery: pushing the boundaries.

The endoscopic endonasal approach (EEA) has significantly evolved since its initial uses in pituitary and sinonasal surgery. The literature is filled with reports and case series demonstrating efficacy and advantages for the entire ventral skull base. With competence in 'minimally invasive' parasellar approaches, larger and more complex approaches were developed to utilize the endonasal corridor to create maximally invasive endoscopic skull base procedures. The challenges of these more complex endoscopic procedures include a long learning curve and navigating in a narrow corridor; reconstruction of defects presented new challenges and early experience revealed a significantly higher risk of cerebrospinal fluid leak. Despite these challenges, there are many benefits to the EEA including avoidance of brain and neurovascular retraction, improved visualization, a direct corridor onto many tumors and the two-surgeon approach. Most importantly, the EEA provides a midline corridor to directly access tumors, which displace critical neurovascular structures laterally, giving it an inherent advantage of minimizing any manipulation of these structures and thus decreasing their potential injury.

Histopathological examination of spine tumors after treatment with radiosurgery.

OBJECTIVE Increased survival time after diagnosis of neoplastic disease has resulted in a gradual increase in spine tumor incidence. Radiosurgery is frequently a viable alternative to operative management in a population with severe medical comorbidities. The authors sought to assess the histopathological consequences of radiosurgery in the subset of patients progressing to operative intervention. METHODS Eighteen patients who underwent radiosurgery for spine tumors between 2008 and 2014 subsequently progressed to surgical treatment. A histopathological examination of these cases was performed. Indications for surgery included symptomatic compression fractures, radiographic instability, and symptoms of cord or cauda equina compression. Biopsy samples were obtained from the tumor within the radiosurgical zone in all cases and were permanently fixated. Viable tumor samples were stained for Ki 67. RESULTS Fifteen patients had metastatic lesions and 3 patients had neurofibromas. The mean patient age was 57 years. The operative indication was symptomatic compression in 10 cases (67%). The most frequent metastatic lesions were breast cancer (4 cases), renal cell carcinoma (3), prostate cancer (2), and endometrial cancer (2). In 9 (60%) of the 15 metastatic cases, histological examination of the lesions showed minimal evidence of inflammation. Viable tumor at the margins of the radiosurgery was seen in 9 (60%) of the metastatic cases. Necrosis in the tumor bed was frequent, as was fibrotic bone marrow. Vascular ectasia was seen in 2 of 15 metastatic cases, but sclerosis with ectasia was frequent. No evidence of malignant conversion was seen in the periphery of the lesions in the 3 neurofibroma cases. In 1 case of neurofibroma, the lesion demonstrated some small areas of remnant tumor in the radiosurgical target zone. CONCLUSIONS This case series demonstrates important histopathological characteristics of spinal lesions treated by SRS. Regions with the highest exposure to radiation appear to be densely necrotic and show little evidence of tumor growth, whereas peripheral regions distant from the radiation dosage are more likely to demonstrate viable tumor in malignant and benign neoplasms. Physiological tissue appears to be similarly affected. With additional investigation, a more homogenized field of hypofractionated radiation exposure may allow for tumor obliteration with relative preservation of critical anatomical structures.

Aneurysmal subarachnoid hemorrhage with concomitant posterior communicating artery fenestration.

Fenestrations of the posterior communicating artery (PCoA) are extremely rare. Associated aneurysms have only been documented three times in the literature, and none associated with a subarachnoid hemorrhage. We describe a 52-year-old female who presented with a subarachnoid hemorrhage secondary to a ruptured saccular aneurysm at the proximal limb of a fenestrated right PCoA. The patient was also found to have bilateral middle cerebral artery (MCA) aneurysms. Surgical management included surmising the etiology of the subarachnoid hemorrhage with subsequent clipping of both the right PCoA and MCA aneurysm. The potential embryological mechanisms leading to a PCoA fenestration are discussed.

Thin-slice T2 MRI imaging predicts vascular pathology in hemifacial spasm: a case-control study.

Hemifacial spasm (HFS) is a condition that may severely reduce patients' quality of life. We sought to determine the sensitivity and specificity of thin-slice T2 magnetic resonance imaging (MRI) for detecting vascular compression in HFS patients. Prospective information was collected on 28 patients with HFS who presented to our center between March 2011 and March 2012 with thin-slice T2 MR imaging. The sensitivity and specificity for differentiating patients from controls were calculated. Sensitivities were 78.6% and 92.9% for the blinded radiologists and 75% for the partially blinded neurosurgeon. Specificities were 42.9% and 28.6% for the blinded radiologists and 75% for the partially blinded neurosurgeon. Magnetic resonance imaging of the facial nerve can guide clinicians in selecting patients who are good surgical candidates. Thin-slice T2 MRI should be viewed as supportive rather than diagnostic.

The development of Moyamoya syndrome after proton beam therapy.

The development of Moyamoya syndrome (MMS) after cranial irradiation for pediatric tumors has been well established. However, information on the development of MMS after proton beam radiotherapy is sparse. We present the case of a 2-year-old child who developed radiation-induced MMS after treatment with proton beam therapy.

Predictors and Complications of Cerebrospinal Fluid Leak after Endoscopic Endonasal Surgery: A Single Institution Retrospective Review.

Background The endoscopic endonasal approach (EEA) is a commonly used technique for resection of sellar, suprasellar, and anterior fossa masses. One of the most troublesome complications of this technique is cerebrospinal fluid (CSF) leak. In this study, we evaluate the risk factors and consequences of CSF leak on surgical outcomes. Methods The current study is a retrospective single-institution cohort study evaluating patients who underwent EEA for sellar and/or suprasellar masses from July 2017 to March of 2020. Risk factors for intraoperative and postoperative CSF leak were evaluated, including sellar defect size, tumor volume and pathology, age, body mass index, prior endoscopic endonasal surgery, lumbar drain placement, nasoseptal and mucosal graft use, year of surgery, and cavernous sinus invasion. Postoperative infection, perioperative antibiotic use, and length of stay were also evaluated. Results Our study included 175 patients. Sellar defect size ( p = 0.015) and intraoperative CSF leak ( p < 0.001) were significantly associated with an increased risk of postoperative CSF leak. Patients with nasoseptal flaps were more likely to have a postoperative CSF leak than those with free mucosal grafts ( p = 0.025). Intraoperative CSF leak, Cushing's disease, and lumbar drain placement were associated with an increased length of stay. Conclusion Sellar defect size, intraoperative CSF leak, and nasoseptal flap use were associated with an increased risk of postoperative CSF leak. Intraoperative CSF leak, Cushing's disease, and lumbar drain placement are all associated with an increased length of stay.

Uninterrupted use of continuous positive airway pressure after endoscopic endonasal approach for pituitary tumors.

KEY POINTS: Resumption of continuous positive airway pressure (CPAP) in the immediate postoperative period after endoscopic endonasal approaches (EEA) for pituitary adenomas can be safe.

Multi-center study on sellar reconstruction after endoscopic transsphenoidal pituitary surgery.

INTRODUCTION: Surgical techniques for sellar reconstruction include no reconstruction, use of synthetic materials, autologous grafts, and/or vascularized flaps. The aim of this study was to conduct a multi-center study comparing the efficacy and postoperative morbidity associated with different sellar reconstruction techniques. METHODS: A retrospective chart review of patients who underwent endoscopic transsphenoidal surgery for pituitary tumors from five participating sites between January 2021 and March 2023 was performed. The variables included demographics, tumor characteristics, reconstruction technique, postoperative cerebrospinal fluid leak (CSF) leak, and 22-item Sino-Nasal Outcome Test (SNOT-22) scores. Comparisons of postoperative complications, SNOT-22 scores, and duration of surgery by type of onlay reconstruction were evaluated using Fisher's exact test, analysis of variance, and Kruskal‒Wallis test. RESULTS: Five hundred and one patients were identified. The median tumor size was 2.1 cm, and 64% were non-functioning. Intraoperative CSF leak was identified in 38% of patients. A total of 89% of patients underwent onlay reconstruction: 49% were reconstructed with mucosal grafts, 35% with nasoseptal flaps, and 5% with other onlay techniques. Nasoseptal flaps were utilized more frequently in the setting of giant pituitary adenomas (>3 cm), medial cavernous sinus wall resection, and high-flow intraoperative CSF leaks. Cases who utilized mucosal grafts had an overall shorter operating time (median: 183 min vs. 240 min; p < 0.001). Five postoperative CSF leaks were identified, and therefore, statistical analysis could not be performed for this complication. CONCLUSION: The effectiveness and morbidity of different sellar reconstruction techniques are comparable. Vascularized flaps were utilized more frequently in the setting of larger tumors and high-flow intraoperative CSF leaks.

Recurrence morbidity of olfactory neuroblastoma.

BACKGROUND: With modern treatment paradigms, olfactory neuroblastoma (ONB) has favorable overall survival (OS); however, the incidence of recurrence remains high. The primary aims of this study were to delineate the prognosis of recurrence of ONB and explore how recurrence subsites are associated with OS, disease-specific survival (DSS), and further recurrence. METHODS: A retrospective chart review of ONB cases from nine academic centers between 2005 and 2021 was completed. Tumor characteristics, recurrence subsites, timelines to recurrence, additional recurrences, and survival estimates were determined using descriptive and time-to-event analyses. RESULTS: A final cohort of 233 patients was identified, with 70 (30.0%) patients recurring within 50.4 (standard deviation ±40.9) months of diagnosis on average, consisting of local (50%), neck (36%), intracranial (9%), and distant (6%) recurrence. Compared with subjects without recurrence, patients with recurrence had significantly different primary American Joint Committee on Cancer T stage (p < 0.001), overall stage (p < 0.001), and modified Kadish scores (p < 0.001). Histopathology identified that dural involvement and positive margins were significantly greater in recurrent cases. First recurrence was significantly associated with worse 5-year DSS (hazard ratio = 5.62; p = 0.003), and subjects with neck or local recurrence had a significantly better DSS compared to intracranial or distant recurrence. CONCLUSIONS: Recurrent cases of ONB have significantly different stages and preoperative imaging factors. Patients with local or neck recurrence, however, have better DSS than those with intracranial or distant recurrence, independent of initial tumor stage or Hyams grade. Identifying specific factors that confer an increased risk of recurrence and DSS is important for patient counseling in addition to surveillance planning.

Preischemic exercise reduces brain damage by ameliorating metabolic disorder in ischemia/reperfusion injury.

Physical exercise preconditioning is known to ameliorate stroke-induced injury. In addition to several other mechanisms, the beneficial effect of preischemic exercise following stroke is due to an upregulated capacity to maintain energy supplies. Adult male Sprague-Dawley rats were used in exercise and control groups. After 1-3 weeks of exercise, several enzymes were analyzed as a gauge of the direct effect of physical exercise on cerebral metabolism. As a measure of metabolic capacity, an ADP/ATP ratio was obtained. Glucose transporters (GLUT1 and GLUT3) were monitored to assess glucose influx, and phosphofructokinase (PFK) was measured to determine the rate of glycolysis. Hypoxia-induced factor-1α (HIF-1α) and 5'AMP-activated protein kinase (AMPK) levels were also determined. These same analyses were performed on preconditioned and control rats following an ischemic/reperfusion (I/R) insult. Our results show that GLUT1, GLUT3, PFK, AMPK, and HIF-1α were all increased following 3 weeks of exercise training. In addition, the ADP/ATP ratio was chronically elevated during these 3 weeks. After I/R injury, HIF-1α and AMPK were significantly higher in exercised rats. The ADP/ATP ratio was reduced in preconditioned rats in the acute phase after stroke, suggesting a lower level of metabolic disorder. GLUT1 and GLUT3 were also increased in the acute phase in exercise rats, indicating that these rats were better able to increase rates of metabolism immediately after ischemic injury. In addition, PFK expression was increased in exercise rats showing an enhanced glycolysis resulting from exercise preconditioning. Altogether, exercise preconditioning increased the rates of glucose metabolism, allowing a more rapid and more substantial increase in ATP production following stroke.

Symptomatic thoracic arachnoid cyst with coexisting tick paralysis: case report and review of the literature.

Tick paralysis is an uncommon phenomenon resulting from the release of a neurotoxin from the salivary glands of an engorged, gravid female tick about 5-7 days after attachment. The neurotoxin produces ascending weakness, mimicking other ascending paralytic processes. We present a case of a child presenting with weakness of the lower extremities and frequent falls who was found to have a compressive thoracic arachnoid cyst and a large distal syrinx. After surgical decompression, the patient made significant improvement in her leg strength, but quickly developed an ascending quadriparesis, followed by respiratory depression. Subsequent imaging and physical examination revealed an engorged tick embedded in her scalp. The tick was removed, and the patient made a rapid and complete clinical recovery. We present a unique case of concomitant tick paralysis and a symptomatic spinal intradural arachnoid cyst, and review the literature on tick paralysis.

Clival intraosseous myxoma treated with an endoscopic endonasal approach: illustrative case.

BACKGROUND: Solitary lesions of the clivus, especially nonchordomatous ones, are exceptionally rare pathologies representing only 0.1%-0.2% of intracranial tumors that may present diagnostic and therapeutic challenges. Intraosseous myxomas are benign tumors arising from mesenchymal cells with an unknown pathophysiology. OBSERVATIONS: In this article, a 15-year-old male with clival intraosseous myxoma who was treated via an endoscopic endonasal surgical approach is presented. The patient became symptomatic after a baseball struck his head, leading to throbbing headaches and visual difficulties for tracking objects. Examination noted diplopia, cranial nerve VI palsy, and ptosis. After imaging revealed a tumor extending to the left cavernous sinus with bulging of the dorsal clivus against the basilar artery/ventral pons, resection was performed. Postoperatively, the patient noted ocular motility and alignment improvement without further complications. LESSONS: Clival masses present with symptoms from compromised neurovascular structures including visual disturbances and trigeminal sensory deficits. Given the rarity of these entities, patients may postpone further treatment until workup; this patient was misdiagnosed for possible concussion until several weeks passed. A PubMed database review of cranial myxoma cases was conducted to identify solitary clival intraosseous myxoma cases. This case is one of the few in which this pathology was treated through an endoscopic endonasal operative approach without complications, demonstrating its safety and effectiveness.

Consecutive resections of double pituitary adenoma for resolution of Cushing disease: illustrative case.

BACKGROUND: Double pituitary adenomas are rare presentations of two distinct adenohypophyseal lesions seen in <1% of surgical cases. Increased rates of recurrence or persistence are reported in the resection of Cushing microadenomas and are attributed to the small tumor size and localization difficulties. The authors report a case of surgical treatment failure of Cushing disease because of the presence of a secondary pituitary adenoma. OBSERVATIONS: A 32-year-old woman with a history of prolactin excess and pituitary lesion presented with oligomenorrhea, weight gain, facial fullness, and hirsutism. Urinary and nighttime salivary cortisol elevation were elevated. Magnetic resonance imaging confirmed a 4-mm3 pituitary lesion. Inferior petrosal sinus sampling was diagnostic for Cushing disease. Primary endoscopic endonasal transsphenoidal resection was performed to remove what was determined to be a lactotroph-secreting tumor on immunohistochemistry with persistent hypercortisolism. Repeat resection yielded a corticotroph-secreting tumor and postoperative hypoadrenalism followed by long-term normalization of the hypothalamic-pituitary-adrenal axis. LESSONS: This case demonstrates the importance of multidisciplinary management and postoperative hormonal follow-up in patients with Cushing disease. Improved strategies for localization of the active tumor in double pituitary adenomas are essential for primary surgical success and resolution of endocrinopathies.

Multicenter Survival Analysis and Application of an Olfactory Neuroblastoma Staging Modification Incorporating Hyams Grade.

Importance: Current olfactory neuroblastoma (ONB) staging systems inadequately delineate locally advanced tumors, do not incorporate tumor grade, and poorly estimate survival and recurrence. Objective: The primary aims of this study were to (1) examine the clinical covariates associated with survival and recurrence of ONB in a modern-era multicenter cohort and (2) incorporate Hyams tumor grade into existing staging systems to assess its ability to estimate survival and recurrence. Design, Setting, and Participants: This retrospective, multicenter, case-control study included patients with ONB who underwent treatment between January 1, 2005, and December 31, 2021, at 9 North American academic medical centers. Intervention: Standard-of-care ONB treatment. Main Outcome and Measures: The main outcomes were overall survival (OS), disease-free survival (DFS), and disease-specific survival (DSS) as C statistics for model prediction. Results: A total of 256 patients with ONB (mean [SD] age, 52.0 [15.6] years; 115 female [44.9%]; 141 male [55.1%]) were included. The 5-year rate for OS was 83.5% (95% CI, 78.3%-89.1%); for DFS, 70.8% (95% CI, 64.3%-78.0%); and for DSS, 94.1% (95% CI, 90.5%-97.8%). On multivariable analysis, age, American Joint Committee on Cancer (AJCC) stage, involvement of bilateral maxillary sinuses, and positive margins were associated with OS. Only AJCC stage was associated with DFS. Only N stage was associated with DSS. When assessing the ability of staging systems to estimate OS, the best-performing model was the novel modification of the Dulguerov system (C statistic, 0.66; 95% CI, 0.59-0.76), and the Kadish system performed most poorly (C statistic, 0.57; 95% CI, 0.50-0.63). Regarding estimation of DFS, the modified Kadish system performed most poorly (C statistic, 0.55; 95% CI, 0.51-0.66), while the novel modification of the AJCC system performed the best (C statistic, 0.70; 95% CI, 0.66-0.80). Regarding estimation of DSS, the modified Kadish system was the best-performing model (C statistic, 0.79; 95% CI, 0.70-0.94), and the unmodified Kadish performed the worst (C statistic, 0.56; 95% CI, 0.51-0.68). The ability for novel ONB staging systems to estimate disease progression across stages was also assessed. In the novel Kadish staging system, patients with stage VI disease were approximately 7 times as likely to experience disease progression as patients with stage I disease (hazard ratio [HR], 6.84; 95% CI, 1.60-29.20). Results were similar for the novel modified Kadish system (HR, 8.99; 95% CI, 1.62-49.85) and the novel Dulguerov system (HR, 6.86; 95% CI, 2.74-17.18). Conclusions and Relevance: The study findings indicate that 5-year OS for ONB is favorable and that incorporation of Hyams grade into traditional ONB staging systems is associated with improved estimation of disease progression.

Radiographic predictors of occult intracranial involvement in olfactory neuroblastoma patients.

BACKGROUND: Traditional management of olfactory neuroblastoma (ONB) includes margin-negative resection with removal of cribriform plate, dura, and olfactory bulb, regardless of intracranial disease. This approach may be overtreating certain patients. Our investigation examines risk factors associated with occult intracranial disease to optimize therapeutic outcomes. METHODS: This retrospective, multi-institutional cohort study examined clinical covariates associated with occult intracranial involvement. Patient demographics, staging, Hyam's grade, and pathologic involvement of dura, olfactory bulb/tract, and brain were collected. Diagnostic imaging was reviewed. Positive and negative predictive value (NPV) were estimated along with effect size estimates. Cox hazard regression examined associations with overall survival (OS) and disease-free survival (DFS). RESULTS: A total of 224 subjects with new diagnoses of ONB (2005-2021) were identified. Skull base bone involvement on computed tomography (CT) had the highest NPV for pathologic dura (88.0%), olfactory bulb (88%), and brain involvement (97.3%). Hyam's grade category was significantly associated with dural involvement (φC  = 0.26; 95% confidence interval [CI]: 0.16, 0.42). Subjects without radiologic skull base involvement (n = 66) had pathologic positivity of 12.1%. Within this subgroup, Hyam's grade was clinically significant for dural positivity (φ = 0.34; 95% CI: -0.12, 0.71) with 28.6% involvement in high grade tumors. Neither clinical nor pathologic positivity of intracranial structures were associated with significantly different OS or DFS. CONCLUSIONS: Both CT and magnetic resonance imaging (MRI) had reasonably good NPV for involvement of dura and olfactory bulb. Higher Hyam's grade was associated with dural involvement. Patients with low-grade tumors not involving the skull base may be suitable for avoiding skull base resection; however, further investigation is warranted.

Intraosseous Meningioma Along the Left Petrous Bone: A Rare Cause of Trigeminal Neuralgia.

Trigeminal neuralgia (TN) presents with symptoms of intense recurrent shock-like brief pain localized to specific areas of the face innervated by the fifth cranial nerve. The pathology of trigeminal neuralgia is attributed to the fifth cranial nerve compression or demyelination. Most cases of this diagnosis are not due to bony structures, making this case an uncommon presentation of trigeminal neuralgia. Herein, we present a case of trigeminal neuralgia due to an intraosseous meningioma that formed along the left petrous bone, resulting in trigeminal nerve compression. On head computed tomography (CT), osteomatous growths along the left petrous bone were noticed compressing the trigeminal nerve. After trigeminal nerve decompression and drilling out the protruding part of the petrous bone through middle cranial fossa craniotomy, the patient's symptoms were completely improved postoperatively and at the two-month follow-up. To our knowledge, there are only four reported cases of trigeminal neuralgia caused by petrous bone compression in the literature. We emphasize the significance of considering petrous bone lesions as a cause of trigeminal neuralgia.