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Nicole A. Silva, Belinda Shao, Michael J. Sylvester, Jean Anderson Eloy, and Chirag D. Gandhi

OBJECTIVE

Observation and neurosurgical intervention for unruptured intracranial aneurysms (UIAs) in the elderly population is rapidly increasing. Cerebral aneurysm coiling (CACo) is favored over cerebral aneurysm clipping (CAC) in elderly patients, yet some elderly individuals still undergo CAC. The cost-effectiveness of treating UIAs requires further exploration. Understanding the effect of intervention on hospital charges and length of stay (LOS) as well as perioperative mortality and complications can further shed light on its economic impact. The purpose of this study was to analyze the cost and perioperative outcomes of UIAs in elderly patients (≥ 65 years of age) after CACo or CAC intervention.

METHODS

Retrospective cohorts of CACo and CAC admissions were extracted from National (Nationwide) Inpatient Sample data obtained between 2002 and 2013, forming parallel intervention groups to compare the following outcomes between elderly and nonelderly patients: average LOS and mean hospital admission costs, in-hospital mortality, and complications. Covariates included sex, race or ethnicity, and comorbidities.

RESULTS

Elderly patients undergoing CAC experienced an average LOS of 8.0 days, whereas elderly patients undergoing CACo stayed an average of 3.2 days. The mean hospital charges incurred during admission totaled $95,960 in the elderly patients who underwent CAC versus $87,960 in the ones who underwent CACo. Elderly patients in whom CAC was performed had a 2.2% rate of in-hospital mortality, with a 2.6 greater adjusted odds of in-hospital mortality than nonelderly patients treated with CAC. In contrast, elderly patients who underwent CACo had a 1.36 greater adjusted odds of in-hospital mortality than their nonelderly counterparts. Compared to nonelderly patients receiving both interventions, elderly individuals had a significantly higher prevalence of various comorbidities and incidence of complications. Elderly patients who received CAC experienced a 10.3% incidence rate of perioperative stroke, whereas their CACo counterparts experienced this complication at a rate of 3.5%. Elderly patients treated with CAC had greater odds of perioperative acute renal failure, whereas their CACo counterparts had greater odds of perioperative deep venous thrombosis and pulmonary embolism.

CONCLUSIONS

Intervention with CAC and CACo in the elderly is resource intensive and is associated with higher risk than in the nonelderly. Those deciding between intervention and conservative management should consider these risks and costs, especially the 2.2% postoperative mortality rate associated with CAC in the elderly population. Further comparative cost-effectiveness research is needed to weigh these costs and outcomes against those of conservative management.

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James K. Liu, Nicole A. Silva, Ilesha A. Sevak, and Jean Anderson Eloy

OBJECTIVE

There has been much debate regarding the optimal surgical approach for resecting olfactory groove meningiomas (OGMs). In this paper, the authors analyzed the factors involved in approach selection and reviewed the surgical outcomes in a series of OGMs.

METHODS

A retrospective review of 28 consecutive OGMs from a prospective database was conducted. Each tumor was treated via one of 3 approaches: transbasal approach (n = 15), pure endoscopic endonasal approach (EEA; n = 5), and combined (endoscope-assisted) transbasal-EEA (n = 8).

RESULTS

The mean tumor volume was greatest in the transbasal (92.02 cm3) and combined (101.15 cm3) groups. Both groups had significant lateral dural extension over the orbits (transbasal 73.3%, p < 0.001; combined 100%), while the transbasal group had the most cerebral edema (73.3%, p < 0.001) and vascular involvement (66.7%, p < 0.001), and the least presence of a cortical cuff (33.3%, p = 0.019). All tumors in the combined group were recurrent tumors that invaded into the sinonasal cavity. The purely EEA group had the smallest mean tumor volume (33.33 cm3), all with a cortical cuff and no lateral dural extension. Gross-total resection was achieved in 80% of transbasal, 100% of EEA, and 62.5% of combined cases. Near-total resection (> 95%) was achieved in 20% of transbasal and 37.5% of combined cases, all due to tumor adherence to the critical neurovascular structures. The rate of CSF leakage was 0% in the transbasal and combined groups, and there was 1 leak in the EEA group (20%), resulting in an overall CSF leakage rate of 3.6%. Olfaction was preserved in 66.7% in the transbasal group. There was no significant difference in length of stay or 30-day readmission rate between the 3 groups. The mean modified Rankin Scale score was 0.79 after the transbasal approach, 2.0 after EEA, and 2.4 after the combined approach (p = 0.0604). The mean follow-up was 14.5 months (range 1–76 months).

CONCLUSIONS

The transbasal approach provided the best clinical outcomes with the lowest rate of complications for large tumors (> 40 mm) and for smaller tumors (< 40 mm) with intact olfaction. The role of EEA appears to be limited to smaller, appropriately selected tumors in which olfaction is already absent. EEA also plays an important adjunctive role when combined with the transbasal approach for recurrent OGMs invading the sinonasal cavity. Careful patient selection using an individualized, tailored strategy is important to optimize surgical outcomes.

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James K. Liu, Ilesha A. Sevak, Peter W. Carmel, and Jean Anderson Eloy

Resection remains the mainstay of treatment for craniopharyngiomas with the goal of radical resection, if safely possible, to minimize the rate of recurrence. Endoscopic endonasal and microscopic transcranial surgical approaches have both become standard methods for the treatment for craniopharyngiomas. However, the approach selection paradigm for craniopharyngiomas is still a point of discussion. Choosing the optimal surgical approach can play a significant role in maximizing the extent of resection and surgical outcome while minimizing the risks of potential complications. Craniopharyngiomas can present with a variety of different sizes, locations, and tumor consistencies, and each individual tumor has distinct features that favor one specific approach over another. The authors review standard cranial base techniques applied to craniopharyngioma surgery, using both the endoscopic endonasal approach and traditional open microsurgical approaches, and analyze factors involved in approach selection. They discuss their philosophy of approach selection based on the location and extent of the tumor on preoperative imaging as well as the advantages and limitations of each surgical corridor, and they describe the operative nuances of each technique, using a personalized, tailored approach to the individual patient with illustrative cases and videos.

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James K. Liu, Jimmy Patel, Ira M. Goldstein, and Jean Anderson Eloy

The transoral approach is considered the gold-standard surgical route for performing anterior odontoidectomy and ventral decompression of the craniovertebral junction for pathological conditions that result in symptomatic cervicomedullary compression, including basilar invagination, rheumatoid pannus, platybasia with retroflexed odontoid processes, and neoplasms. Extended modifications to increase the operative corridor and exposure include the transmaxillary, extended “open-door” maxillotomy, transpalatal, and transmandibular approaches. With the advent of extended endoscopic endonasal skull base techniques, there has been increased interest in the last decade in the endoscopic endonasal transclival transodontoid approach to the craniovertebral junction. The endonasal route represents an attractive minimally invasive surgical alternative, especially in cases of irreducible basilar invagination in which the pathology is situated well above the palatine line. Angled endoscopes and instrumentation can also be used for lower-lying pathology. By avoiding the oral cavity and subsequently using a transoral retractor, the endonasal route has the advantages of avoiding complications related to tongue swelling, tracheal swelling, prolonged intubation, velopharyngeal insufficiency, dysphagia, and dysphonia. Postoperative recovery is quicker, and hospital stays are shorter. In this report, the authors describe and illustrate their method of purely endoscopic endonasal transclival odonotoidectomy for anterior decompression of the craniovertebral junction and describe various operative pearls and nuances of the technique for avoiding complications.

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Jimmy Patel, Jean Anderson Eloy, and James K. Liu

Nelson's syndrome is a rare clinical manifestation that occurs in 8%–47% of patients as a complication of bilateral adrenalectomy, a procedure that is used to control hypercortisolism in patients with Cushing's disease. First described in 1958 by Dr. Don Nelson, the disease has since become associated with a clinical triad of hyperpigmentation, excessive adrenocorticotropin secretion, and a corticotroph adenoma. Even so, for the past several years the diagnostic criteria and management of Nelson's syndrome have been inadequately studied. The primary treatment for Nelson's syndrome is transsphenoidal surgery. Other stand-alone therapies, which in many cases have been used as adjuvant treatments with surgery, include radiotherapy, radiosurgery, and pharmacotherapy. Prophylactic radiotherapy at the time of bilateral adrenalectomy can prevent Nelson's syndrome (protective effect). The most promising pharmacological agents are temozolomide, octreotide, and pasireotide, but these agents are often administered after transsphenoidal surgery. In murine models, rosiglitazone has shown some efficacy, but these results have not yet been found in human studies. In this article, the authors review the clinical manifestations, pathophysiology, diagnostic criteria, and efficacy of multimodal treatment strategies for Nelson's syndrome.

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Anni Wong, Jean Anderson Eloy, and James K. Liu

Cushing's syndrome (CS) results from sustained exposure to excessive levels of free glucocorticoids. One of the main causes of CS is excessive adrenocorticotropic hormone (ACTH) secretion by tumors in the pituitary gland (Cushing's disease [CD]). Cushing's disease and its associated hypercortisolism have a breadth of debilitating symptoms associated with an increased mortality rate, warranting urgent treatment. Currently, the first line of treatment for CD is transsphenoidal surgery (TSS), with excellent long-term results. Transsphenoidal resections performed by experienced surgeons have shown remission rates ranging from 70% to 90%. However, some patients do not achieve normalization of their hypercortisolemic state after TSS and continue to have persistent or recurrent CD. For these patients, various therapeutic options after failed TSS include repeat TSS, radiotherapy, medical therapy, and bilateral adrenalectomy (BLA). Bilateral adrenalectomy has been shown to be a safe and effective treatment modality for persistent or recurrent CD with an immediate and definitive cure of the hypercortisolemic state. BLA was traditionally performed through an open approach, but since the advent of laparoscopic adrenalectomy, the laparoscopic approach has become the surgical method of choice. Advances in technology, refinement in surgical skills, competency in adrenopathology, and emphasis on multidisciplinary collaborations have greatly reduced morbidity and mortality associated with adrenalectomy surgery in a high-risk patient population. In this article, the authors review the role of BLA in the treatment of refractory CD. The clinical indications, current surgical and endocrinological results reported in the literature, surgical technique (open vs laparoscopic), drawbacks, and complications of BLA are discussed.

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Richard F. Schmidt, Frederick Yick, Zain Boghani, Jean Anderson Eloy, and James K. Liu

Object

Malignant peripheral nerve sheath tumors (MPNSTs) are a rare form of malignancy arising from the Schwann cells of peripheral nerves. MPNSTs of the trigeminal nerve are exceptionally rare, with only a handful of reports in the literature. These tumors are typically very aggressive, resulting in significant patient morbidity and a generally grim prognosis. Most current reports suggest that radical resection with radiation therapy offers the best benefit. In this study, the authors systematically reviewed the world English-language literature on MPNSTs of the trigeminal nerve to analyze the presentations, treatment options, and outcomes for patients with this disease.

Methods

A literature search for MPNSTs of the trigeminal nerve confined to nonanimal, English-language articles was conducted utilizing the PubMed database, with additional cases chosen from the references of selected articles. Only cases of confirmed MPNSTs of the trigeminal nerve or its peripheral branches, based upon surgical, pathological, or radiological analysis, were included.

Results

From the literature search, 29 articles discussing 35 cases of MPNSTs of the trigeminal nerve were chosen. With the addition of 1 case from their own institution, the authors analyzed 36 cases of trigeminal MPNSTs. The average age of onset was 44.6 years. These tumors were more commonly seen in male patients (77.1%). The gasserian ganglion was involved in 36.1% of the cases. Of the cases in which the nerve distribution was specified (n = 25), the mandibular branch was most commonly involved (72.0%), followed by the maxillary branch (60.0%) and the ophthalmic branch (32.0%), with 44.0% of patients exhibiting involvement of 2 or more branches. Altered facial sensation and facial pain were the 2 most commonly reported symptoms, found in 63.9% and 52.8% of patients, respectively. Mastication difficulty and diplopia were seen in 22.2% of patients, facial weakness was seen in 19.4%, and hearing loss was present in 16.7%. With regard to the primary treatment strategy, 80.6% underwent resection, 16.7% underwent radiation therapy, and 2.9% received chemotherapy alone. Patients treated with complete resection followed by postoperative radiation therapy had the most favorable outcomes, with no patients showing evidence of disease recurrence with a mean follow-up of 34.6 months. Patients treated with incomplete resection followed by postoperative radiation therapy had more favorable outcomes than patients treated with incomplete resection without radiation therapy or radiation therapy alone.

Conclusions

Trigeminal MPNSTs most commonly present as altered facial sensation or facial pain, although they exhibit a number of other clinical manifestations, including the involvement of other cranial nerves. While a variety of treatment options exist, due to their highly infiltrative nature, aggressive resection followed by radiation therapy appears to offer the greatest chance of recurrence-free survival.

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Richard F. Schmidt, Zain Boghani, Osamah J. Choudhry, Jean Anderson Eloy, Robert W. Jyung, and James K. Liu

With the relatively recent increase in the use of MRI techniques, there has been a concurrent rise in the number of vestibular schwannomas (VSs) detected as incidental findings. These incidental VSs may be prevalent in up to 0.02%–0.07% of individuals undergoing MRI and represent a significant portion of all diagnosed VSs. The management of these lesions poses a significant challenge for practitioners. Most incidental VSs tend to be small and associated with minimal symptoms, permitting them to be managed conservatively at the time of diagnosis. However, relatively few indicators consistently predict tumor growth and patient outcomes. Furthermore, growth rates have been shown to vary significantly over time with a large variety of long-term growth patterns. Thus, early MRI screening for continued tumor growth followed by repeated MRI studies and clinical assessments throughout the patient's life is an essential component in a conservative management strategy. Note that tumor growth is typically associated with a worsening of symptoms in patients who undergo conservative management, and many of these symptoms have been shown to significantly impact the patient's quality of life. Specific indications for the termination of conservative management vary across studies, but secondary intervention has been shown to be a relatively safe option in most patients with progressive disease. Patients with incidental VSs will probably qualify for a course of conservative management at diagnosis, and regular imaging combined with the expectation that the tumor and symptoms may change at any interval is crucial to ensuring positive long-term outcomes in these patients. In this report, the authors discuss the current literature pertaining to the prevalence of incidental VSs and various considerations in the management of these lesions. It is hoped that by incorporating an understanding of tumor growth, patient outcomes, and management strategies, practitioners will be able to effectively address this challenging disease entity.

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Richard F. Schmidt, Osamah J. Choudhry, Ramya Takkellapati, Jean Anderson Eloy, William T. Couldwell, and James K. Liu

A little over a century ago, in 1907, at the University of Innsbruck, Hermann Schloffer performed the first transsphenoidal surgery on a living patient harboring a pituitary adenoma. Schloffer used a superior nasal route via a transfacial lateral rhinotomy incision. This was perhaps his greatest academic contribution to neurosurgery. Despite the technological limitations of that time, Schloffer's operation was groundbreaking in that it laid the foundation for future development and refinement of transsphenoidal pituitary surgery, influencing prominent surgeons such as Oskar Hirsch and Harvey Cushing. Even after undergoing multiple modifications and a brief fall into obscurity, the transsphenoidal approach has endured through generations of surgeons and remains the preferred approach for lesions of the sella turcica to this day. Although Schloffer performed primarily abdominal surgery in his practice, his contributions to the transsphenoidal approach have had a lasting impact in the field of pituitary and skull base surgery. The authors review the life and career of Hermann Schloffer, the surgical details of his transsphenoidal operation, and the legacy that it has left on the field of pituitary surgery.

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Smruti K. Patel, Qasim Husain, Jean Anderson Eloy, William T. Couldwell, and James K. Liu

Developed over a century ago, the transsphenoidal approach to access lesions of the pituitary gland and sella turcica has transformed the field of neurosurgery, largely due to the work of Oskar Hirsch and Harvey Cushing. Furthermore, its use and modification in the early 1900s was perhaps one of Cushing's greatest legacies to skull base surgery. However, Cushing, who had worked relentlessly to improve the transsphenoidal route to the pituitary region, abandoned the approach by 1929 in his pursuit to master transcranial approaches to the suprasellar region. Hirsch and a few other surgeons continued to perform transsphenoidal operations, but they were unable to maintain the popularity of the approach among their peers.

During a time when transsphenoidal surgery was on the brink of extinction, a critical lineage of 3 key surgeons—Norman Dott, Gerard Guiot, and Jules Hardy—would resurrect the art, each working to further improve the procedure. Dott, Cushing's apprentice from 1923 to 1924, brought his experiences with transsphenoidal surgery to Edinburgh, Scotland, and along the way, developed the lighted nasal speculum to provide better illumination in the narrow working area. Guiot, inspired by Dott, adopted his technique and used intraoperative radiofluoroscopic technique for image guidance. Hardy, a fellow of Guiot, from Montreal, Canada, revolutionized transsphenoidal microsurgery with the introduction of the binocular microscope and selective adenomectomy.

The teachings of these pioneers have endured over time and are now widely used by neurosurgeons worldwide. In this paper, we review the lineage and contributions of Dott, Guiot, and Hardy who served as crucial players in the preservation of transsphenoidal surgery.