✓ In the following literature review the authors consider the available evidence for the medical management of patients with ankylosing spondylitis (AS), and they critically assess current treatment guidelines. Medical therapy for axial disease in AS emphasizes improvement in patients' pain and overall function. First-line treatments include individualized physical therapy and nonsteroidal antiinflammatory drugs (NSAIDs) in conjunction with gastroprotective therapy. After an adequate trial of therapy with two NSAIDs exceeding 3 months or limited by medication toxicity, the patient may undergo tumor necrosis factor–α blockade therapy. Response should occur within 6–12 weeks, and patients must undergo tuberculosis screening. Evidence does not currently support the use of disease modifying antirheumatic drugs, corticosteroids, or radiotherapy in AS.
Alexander A. Khalessi, Bryan C. Oh and Michael Y. Wang
Alexander A. Khalessi, Gabriel Zada, Michael Y. Wang and James Forrest Calland
Nancy McLaughlin, Alexander A. Khalessi and Neil A. Martin
Jeffrey Steinberg, Vincent Cheung, Gunjan Goel, J. Scott Pannell, Javan Nation and Alexander Khalessi
Although there have been reports of carotid artery pseudoaneurysm formation after adenoidectomy and/or tonsillectomy secondary to iatrogenic injury, there are no case reports of successful endovascular reconstruction of the injured artery in the pediatric population. In most pediatric cases, the internal carotid artery (ICA) is sacrificed. The authors report on a 6-year-old girl who presented with odynophagia, left-sided Horner's syndrome, hematemesis, and severe anemia 6 months after a tonsillectomy. On examination she was found to have a pulsatile mass along the left posterior lateral oropharynx, and imaging demonstrated a dissection of the extracranial left ICA and an associated pseudoaneurysm. The lesion was managed endovascularly with stent-assisted coil embolization and ICA reconstruction. The child had a somewhat complicated postoperative course, requiring additional coil embolization for treatment of a minor recurrence of the pseudoaneurysm at 5 months after the initial treatment and then presenting with extrusion of a portion of the coil mass into the oropharyngeal cavity a year later. She underwent surgical removal of the extruded coils and repair of the defect and has since been free of symptoms or signs of recurrence.
The authors conclude that this strategy definitively protected the patient against an oral exsanguination or aspiration event secondary to aneurysm rupture and reduced her risk of stroke by preserving vessel patency and caliber. Moreover, they note that covered stent reconstruction surrenders endovascular access and cannot immediately provide these benefits.
Nancy McLaughlin, Alexander A. Khalessi and Neil A. Martin
Milli Desai, Arvin R. Wali, Harjus S. Birk, David R. Santiago-Dieppa and Alexander A. Khalessi
Women have been shown to have a higher risk of cerebral aneurysm formation, growth, and rupture than men. The authors present a review of the recently published neurosurgical literature that studies the role of pregnancy and female sex steroids, to provide a conceptual framework with which to understand the various risk factors associated with cerebral aneurysms in women at different stages in their lives.
The PubMed database was searched for “(“intracranial” OR “cerebral”) AND “aneurysm” AND (“pregnancy” OR “estrogen” OR “progesterone”)” between January 1980 and February 2019. A total of 392 articles were initially identified, and after applying inclusion and exclusion criteria, 20 papers were selected for review and analysis. These papers were then divided into two categories: 1) epidemiological studies about the formation, growth, rupture, and management of cerebral aneurysms in pregnancy; and 2) investigations on female sex steroids and cerebral aneurysms (animal studies and epidemiological studies).
The 20 articles presented in this study include 7 epidemiological articles on pregnancy and cerebral aneurysms, 3 articles reporting case series of cerebral aneurysms treated by endovascular therapies in pregnancy, 3 epidemiological articles reporting the relationship between female sex steroids and cerebral aneurysms through retrospective case-control studies, and 7 experimental studies using animal and/or cell models to understand the relationship between female sex steroids and cerebral aneurysms. The studies in this review report similar risk of aneurysm rupture in pregnant women compared to the general population. Most ruptured aneurysms in pregnancy occur during the 3rd trimester, and most pregnant women who present with cerebral aneurysm have caesarean section deliveries. Endovascular treatment of cerebral aneurysms in pregnancy is shown to provide a new and safe form of therapy for these cases. Epidemiological studies of postmenopausal women show that estrogen hormone therapy and later age at menopause are associated with a lower risk of cerebral aneurysm than in matched controls. Experimental studies in animal models corroborate this epidemiological finding; estrogen deficiency causes endothelial dysfunction and inflammation, which may predispose to the formation and rupture of cerebral aneurysms, while exogenous estrogen treatment in this population may lower this risk.
The aim of this work is to equip the neurosurgical and obstetrical/gynecological readership with the tools to better understand, critique, and apply findings from research on sex differences in cerebral aneurysms.
Arvin R. Wali, Charlie C. Park, David R. Santiago-Dieppa, Florin Vaida, James D. Murphy and Alexander A. Khalessi
Rupture of large or giant intracranial aneurysms leads to significant morbidity, mortality, and health care costs. Both coiling and the Pipeline embolization device (PED) have been shown to be safe and clinically effective for the treatment of unruptured large and giant intracranial aneurysms; however, the relative cost-to-outcome ratio is unknown. The authors present the first cost-effectiveness analysis to compare the economic impact of the PED compared with coiling or no treatment for the endovascular management of large or giant intracranial aneurysms.
A Markov model was constructed to simulate a 60-year-old woman with a large or giant intracranial aneurysm considering a PED, endovascular coiling, or no treatment in terms of neurological outcome, angiographic outcome, retreatment rates, procedural and rehabilitation costs, and rupture rates. Transition probabilities were derived from prior literature reporting outcomes and costs of PED, coiling, and no treatment for the management of aneurysms. Cost-effectiveness was defined, with the incremental cost-effectiveness ratios (ICERs) defined as difference in costs divided by the difference in quality-adjusted life years (QALYs). The ICERs < $50,000/QALY gained were considered cost-effective. To study parameter uncertainty, 1-way, 2-way, and probabilistic sensitivity analyses were performed.
The base-case model demonstrated lifetime QALYs of 12.72 for patients in the PED cohort, 12.89 for the endovascular coiling cohort, and 9.7 for patients in the no-treatment cohort. Lifetime rehabilitation and treatment costs were $59,837.52 for PED; $79,025.42 for endovascular coiling; and $193,531.29 in the no-treatment cohort. Patients who did not undergo elective treatment were subject to increased rates of aneurysm rupture and high treatment and rehabilitation costs. One-way sensitivity analysis demonstrated that the model was most sensitive to assumptions about the costs and mortality risks for PED and coiling. Probabilistic sampling demonstrated that PED was the cost-effective strategy in 58.4% of iterations, coiling was the cost-effective strategy in 41.4% of iterations, and the no-treatment option was the cost-effective strategy in only 0.2% of iterations.
The authors’ cost-effective model demonstrated that elective endovascular techniques such as PED and endovascular coiling are cost-effective strategies for improving health outcomes and lifetime quality of life measures in patients with large or giant unruptured intracranial aneurysm.
Jeffrey A. Steinberg, Jayson Sack, Bayard Wilson, David Weingarten, Bob Carter, Alexander Khalessi, Sharona Ben-Haim and John Alksne
Trigeminal neuralgia is a debilitating pain disorder most often caused by arterial compression of the trigeminal nerve, although there are other etiologies. Microvascular decompression (MVD) remains the most definitive treatment for this disorder, with cure rates reported between 60% and 80%. Traditional MVD techniques involve a retrosigmoid craniotomy with placement of an inert foreign material, such as Teflon, between the nerve and compressive vessel. Recurrence of trigeminal neuralgia after MVD has been associated with vessel migration, adhesion formation, and arterial pulsation against the Teflon abutting the nerve. Additionally, foreign materials such as Teflon have been reported to trigger inflammatory responses, resulting in recurrence of trigeminal pain. An alternative method for decompression involves the use of a sling to transpose the compressive vessel away from the nerve. Results of various sling techniques as a decompressive strategy are limited to small series and case reports. In this study, the authors present their experience utilizing a tentorial sling for MVD in patients with trigeminal neuralgia.
Institutional review board approval was obtained in order to contact patients who underwent MVD for trigeminal neuralgia via the tentorial sling technique. Clinical outcomes were assessed utilizing the Barrow Neurological Institute (BNI) pain intensity score immediately after surgery and at the time of the study.
The tentorial sling technique was performed in 45 patients undergoing MVD for trigeminal neuralgia. In 41 of these patients, this procedure was their first decompressive surgery. Immediate postoperative relief of pain (BNI score I) was achieved in 80% of patients undergoing their first decompressive procedure. At last follow-up, 73% of these patients remained pain free. Three patients experienced recurrent trigeminal pain, with surgical exploration demonstrating an intact tentorial sling. The complication rate was 6.6%.
Transposition techniques for MVD have been described previously in small series and case reports. This study represents the largest experience in which the utilization of a tentorial sling for MVD in patients with trigeminal neuralgia is described. The technique represents a novel method for decompression of the trigeminal nerve by transposition of the offending vessel without the use of foreign material. Although the authors’ preliminary results parallel the historical cure rate, further outcome data are required to assess long-term durability of this method.
Corey T. Walker, Chiazo S. Amene, Jeffrey S. Pannell, David R. Santiago-Dieppa, Robert C. Rennert, Lawrence A. Hansen and Alexander A. Khalessi
The differential diagnosis of spinal tumors is guided by anatomical location and imaging characteristics. Diagnosis of rare tumors is made challenging by abnormal features. The authors present the case of a 47-year-old woman who presented with progressive subacute right lower-extremity weakness and numbness of the right thigh. Physical examination further revealed an extensor response to plantar reflex on the right and hyporeflexia of the right Achilles and patellar reflexes. Magnetic resonance imaging of the lumbar spine demonstrated an 8-mm intramedullary exophytic nodule protruding into a hematoma within the conus medullaris. Spinal angiography was performed to rule out an arteriovenous malformation, and resection with hematoma evacuation was completed. Pathological examination of the resected mass demonstrated a spindle cell neoplasm with dense bundles of collagen. Special immunostaining was performed and a diagnosis of solitary fibrous tumor (SFT) was made. SFTs are mesenchymally derived pleural neoplasms, which rarely present at other locations of the body, but have been increasingly described to occur as primary neoplasms of the spine and CNS. The authors believe that this case is unique in its rare location at the level of the conus, and also that this is the first report of a hemorrhagic SFT in the spine. Therefore, with this report the authors add to the literature the fact that this variant of an increasingly understood but heterogeneous tumor can occur, and therefore should be considered in the differential of clinically similar tumors.
Michael G. Brandel, Robert C. Rennert, Arvin R. Wali, David R. Santiago-Dieppa, Jeffrey A. Steinberg, Christian Lopez Ramos, Peter Abraham, J. Scott Pannell and Alexander A. Khalessi
Preoperative embolization of meningiomas can facilitate their resection when they are difficult to remove. The optimal use and timing of such a procedure remains controversial given the risk of embolization-linked morbidity in select clinical settings. In this work, the authors used a large national database to study the impact of immediate preoperative embolization on the immediate outcomes of meningioma resection.
Meningioma patients who had undergone elective resection were identified in the National (Nationwide) Inpatient Sample (NIS) for the period 2002–2014. Patients who had undergone preoperative embolization were propensity score matched to those who had not, adjusting for patient and hospital characteristics. Associations between preoperative embolization and morbidity, mortality, and nonroutine discharge were investigated.
Overall, 27,008 admissions met the inclusion criteria, and 633 patients (2.34%) had undergone preoperative embolization and 26,375 (97.66%) had not. The embolization group was younger (55.17 vs 57.69 years, p < 0.001) with a lower proportion of females (63.5% vs 69.1%, p = 0.003), higher Charlson Comorbidity Index (p = 0.002), and higher disease severity (p < 0.001). Propensity score matching retained 413 embolization and 413 nonembolization patients. In the matched cohort, preoperative embolization was associated with increased rates of cerebral edema (25.2% vs 17.7%, p = 0.009), posthemorrhagic anemia or transfusion (21.8% vs 13.8%, p = 0.003), and nonroutine discharge (42.8% vs 35.7%, p = 0.039). There was no difference in mortality (≤ 2.4% vs ≤ 2.4%, p = 0.82). Among the embolization patients, the mean interval from embolization to resection was 1.49 days. On multivariate analysis, a longer interval was significantly associated with nonroutine discharge (OR 1.33, p = 0.004) but not with complications or mortality.
Relative to meningioma patients who do not undergo preoperative embolization in the same admission, those who do have higher rates of cerebral edema and nonroutine discharge but not higher rates of stroke or death. Thus, meningiomas requiring preoperative embolization represent a distinct clinical entity that requires prolonged, more complex care. Further, among embolization patients, the timing of resection did not affect the risk of in-hospital complications, suggesting that the timing of surgery can be determined according to surgeon discretion.