Dental complications in homocystinurias.

Background: Cystathionine beta synthase deficiency (causing classical homocystinuria) has been associated with high-arched palates and crowded teeth, but little has been said about other oral health complications. Other homocystinurias (e.g., the remethylation defects) also have had little reported in terms of oral health. Individuals with the homocystinurias have been described as having bone density issues which can correlate with oral health. Moreover, elevations in homocysteine have a theoretical impact on tooth health and the paucity of clinical reports of oral health issues in homocystinuria may be the consequence of lack of attention by the medical community. Significance: Oral health is essential to overall health. If inadequate attention is paid to the oral health complications which can be seen in homocystinurias, then appropriate referrals and attention in therapeutic guidelines will not reflect the importance of oral health. Specific aims/research question: What oral health complications are reported by individuals with homocystinurias? Do these differ according to diagnosis? Methods: Data were collected from patients with homocystinurias by a series of questionnaires using the RARE-X platform. All subjects were consented prior to the collection of their data. All research was performed in accordance with the Declaration of Helsinki. Demographic data were collected as the initial questionnaire and other data were collected via the oral health questionnaire. Analysis: Questionnaires were opened to the community in mid-2022 and collection of data for this study ended with data submitted up to November 2022. Descriptive statistics were done. Due to the small size of the cohort, additional statistical analyses were not attempted. Results: Patients with homocystinuria, not related to cystathionine beta synthase deficiency, are reporting some tooth structure differences. The cohort taken as a whole does not have increased risk for gingivitis, but there appears to be a risk for long-term gum disease possibly due to the rate of osteoporosis/osteopenia in this population. A large number of individuals have malalignment and malocclusion of the teeth. These data highlight oral health as an important component of care in individuals with the homocystinurias as is true of the general population at large.

Autograft-derived spinal cord mass in the cervical spine following transplantation with olfactory mucosa cells for traumatic spinal cord injury: case report.

This study describes a patient with an autograft-derived spinal cord mass following transplantation of olfactory mucosa for treatment of cervical spine injury. The authors report the case of a 35-year-old man who suffered a complete spinal cord injury (SCI) at C5-6 in 2001. The patient underwent an olfactory mucosal cell implantation at the location of injury 4 years following initial trauma. Twelve years later, the patient presented with rapidly progressive decline in upper-extremity function as well as neuropathic pain. Imaging revealed a heterogeneously enhancing intramedullary mass from C3 to C7. At surgery, the patient was found to have a posttransplant mucinous mass. Each mucinous cyst was drained and a portion of the cyst wall was removed. Histological examination demonstrated ciliated epithelium-lined fibrous tissue, submucosal glands, and mucoid material, consistent with a transplant-derived tumor. This case report both documents a rare long-term complication of olfactory mucosal cell transplantation and serves as a cautionary tale encouraging prudent use of novel treatments in a vulnerable population of patients with severe SCI.

Endovascular Treatment of Ruptured Enlarging Dissecting Anterior Spinal Artery Aneurysm.

BACKGROUND: Aneurysms of the anterior spinal artery are extremely rare. Unlike intracranial saccular aneurysms, they do not occur at branch points, are typically pseudoaneurysms, and rupture secondary to a dissection. They typically present with subarachnoid hemorrhage and demonstrate clinical and radiographic improvement over time without treatment. METHODS: We present here the first case to date of a ruptured anterior spinal artery aneurysm with clinical and radiographic progression treated with Onyx embolization. Our patient was unique in the presentation with acute onset of spinal cord injury American Spinal Injury Association B and an extensive thoracolumbar subdural hematoma. RESULTS: An emergent skip-level laminectomy and subdural decompression were performed with no improvement in examination. This was followed by progressive radiographic enlargement of the aneurysm, treated successfully with Onyx embolization. CONCLUSIONS: We analyze this case and review the literature on thoracic anterior spinal artery and artery of Adamkiewicz aneurysms.

The Effect of Stents in Cerebral Aneurysms: A Review.

The etiology of up to 95% of cerebral aneurysms may be accounted for by hemodynamically-induced factors that create vascular injury. The purpose of this review is to describe key physical properties that stents have and how they affect cerebral aneurysms. We performed a two-step screening process. First, a structured search was performed using the PubMed database. The following search terms and keywords were used: "Hemodynamics," "wall shear stress (WSS)," "velocity," "viscosity," "cerebral aneurysm," "intracranial aneurysm," "stent," "flow diverter," "stent porosity," "stent geometry," "stent configuration," and "stent design." Reports were considered if they included original data, discussed hemodynamic changes after stent-based treatment of cerebral aneurysms, examined the hemodynamic effects of stent deployment, and/or described the geometric characteristics of both stents and the aneurysms they were used to treat. The search strategy yielded a total of 122 articles, 61 were excluded after screening the titles and abstracts. Additional articles were then identified by cross-checking reference lists. The final collection of 97 articles demonstrates that the geometric characteristics and configurations of deployed stents influenced hemodynamic parameters such as aneurysmal WSS, inflow, and pressure. The geometric characteristics of the aneurysm and its position also had significant influences on intra-aneurysmal hemodynamics after treatment. In conclusion, changes in specific aneurysmal hemodynamic parameters that result from stenting relate to a number of factors including the geometric properties and configurations of deployed stents, the geometric properties of the aneurysm, and the pretreatment hemodynamics.

Transnasal Transsphenoidal Elevation of Optic Chiasm in Secondary Empty Sella Syndrome Following Prolactinoma Treatment.

BACKGROUND: Prolactinomas are typically treated nonsurgically with a dopamine agonist. Once the tumor shrinks, adjacent eloquent structures, such as the optic apparatus, can become skeletonized and herniate into the dilated parasellar space. CASE DESCRIPTION: A 48-year-old man with a prolactin-secreting macroadenoma treated with cabergoline presented with progressive bitemporal hemianopsia. Magnetic resonance imaging showed no recurrence of disease and a stretched optic chiasm herniating into an empty sella. Elevation of the optic chiasm via a transnasal transsphenoidal approach with ALLODERM graft and septal cartilage strut was performed. The patient was discharged home the next day with significant improvement in vision; magnetic resonance imaging showed interval elevation of the optic chiasm. CONCLUSIONS: We review secondary empty sella syndrome and discuss surgical strategies for optic chiasmapexy.

Recurrent post-tonsillectomy bleeding due to an iatrogenic facial artery pseudoaneurysm.

This is a report of an illustrative case of recurrent post-tonsillectomy bleeding that was caused by an iatrogenic facial artery pseudoaneurysm and controlled by endovascular embolization. A 37 year-old female who underwent bilateral tonsillectomy for chronic tonsillitis had recurrent secondary hemorrhage despite multiple operative interventions to control the bleeding. Because of the recurrent nature of the bleeding, an angiography of the external carotid artery was performed demonstrating a pseudoaneurysm of the left facial artery with active extravasation. This was successfully embolized with ethylene vinyl alcohol copolymer and the bleeding did not recur. Most post-operative bleeds can be managed with bedside or intraoperative interventions. However, pseudoaneurysms should be considered in the differential diagnosis of recurrent bleeds refractory to surgical control.

Carotid Endarterectomy to Remove Retained Solitaire Stent Retriever inside Carotid Stent after Mechanical Thrombectomy.

BACKGROUND: Tandem occlusions of the internal carotid artery (ICA) and middle cerebral artery (MCA) occur in up to a third of patients with acute ischemic strokes undergoing endovascular mechanical thrombectomy. Understanding open neurosurgical management of associated complications with this procedure is important. CASE REPORT: A 67-year-old man with acute onset of left hemiparesis and a tandem right ICA and MCA occlusion. He underwent carotid stent angioplasty of a stenotic ICA, followed by attempted Solitaire stent retrieval of an MCA clot. On withdrawal, the tines of the Solitaire stent lodged inside the Precise carotid stent. The patient was started on aspirin, Plavix, and heparin infusion, and underwent a carotid endarterectomy (CEA) with safe removal of the stents and primary vessel repair. CONCLUSION: This is the first case reported to date of a Solitaire stent becoming lodged inside a Precise carotid stent, salvaged by CEA with safe removal of the stents and primary vessel repair. We discuss the timing, indication, alternatives, and technical nuances of a CEA in this setting.

Symbiotic relationship of IV-tPA and mechanical thrombectomy in a case of acute tandem ICA-MCA occlusion.

INTRO: In light of the recent successful mechanical thrombectomy trials, the need for IV-tPA prior is under investigation. Few cases demonstrate angiographically the role of both mechanical and chemical strategies at achieving reperfusion. CASE REPORT: A 63year-old male presented with an NIHSS 20. CTA demonstrated an acute occlusion of the left cervical and intracranial ICA and MCA. IV-tPA was administered, followed by immediate reperfusion of the cervical ICA with carotid stenting and mechanical thrombectomy. Within the next 10min, the entire intracranial clot burden dissolved under angiographic control. TICI 3 reperfusion was achieved without any further intervention. Post-procedure, the patient recovered fully to an NIHSS of 0. CONCLUSION: This case underscores the importance of IV-tPA administration in conjunction to mechanical thrombectomy. The interventionalist should take advantage of the symbiotic effect of the IV-tPA administration, which remains the standard of care so far.

Rare Lesions of the Internal Auditory Canal.

BACKGROUND: Approximately 95% of tumors occurring within the internal auditory canal (IAC) are vestibular schwannomas. Many undergo stereotactic radiation without definitive tissue diagnosis. Rare IAC tumors are not all radiosensitive and are poorly described. METHODS: Between 1992 and 2015, 289 consecutive patients with IAC lesions operated on were reviewed retrospectively. RESULTS: Fifteen patients (5.2%) (16 operations) had unusual histologic findings, including nonvestibular schwannomas (2 facial schwannomas, 2 cochlear schwannomas, 2 intermedius schwannomas), 3 meningiomas, 3 cavernous hemangiomas, a mucosa-associated lymphoid tissue lymphoma, an arachnoid cyst, and a lipochoristoma. None of these rare tumors could be identified before surgery. Three operative approaches were used: the retrosigmoid approach, middle fossa subtemporal approach, or translabyrinthine approach. Few complications occurred, including facial nerve palsy, loss of hearing, and vestibular function. Five-year average follow-up revealed one patient with recurrence. CONCLUSIONS: Clinical examination and imaging alone were insufficient to correctly identify these tumors. Definitive pathologicdiagnosis should be strongly considered to help tailor treatment.

Optimal pediatric dosing of anti-platelet agents for pipeline stent embolization -a case report and review of the literature.

BACKGROUND: Various strategies are emerging for dosing antiplatelet therapies in preparation for pipeline stent embolization in adults. Hyper-response is associated with hemorrhagic complications. Hypo-response is associated with thromboembolic events. Dosing of antiplatelet agents is highly variable, with little consensus among experts for adults-and even more so for children. To date, pipeline stents have been deployed in 11 pediatric patients, ages 4-15. A variety of clopidogrel and aspirin dosing regimens have been used, with response tested in only three patients, who were all therapeutic. Thrombotic events occurred in two patients, neither of whom were tested. CASE: We describe here the first case of a hemorrhagic complication in a hyper-responsive pediatric patient undergoing placement of a pipeline stent. DISCUSSION: As the use of endovascular therapies requiring dual anti-platelet agents becomes more established, there is an increasing need to develop titration protocols that minimizes the risk of thrombotic and hemorrhagic events.

Inferior Vena Cava Filter Thrombosis in the Postoperative Neurosurgical Setting: Case Report and Review of the Literature.

There are no definitive treatment guidelines for caval-filter thrombosis in the postoperative setting. Clinical management for partial or complete postoperative inferior vena cava (IVC) occlusion relies solely on expert opinion, anecdotal evidence, and small clinical trials. As such, the primary objective of the present report is to offer a complex case of extensive IVC filter occlusion in a neurosurgical patient with past medical history significant for protein C deficiency. The presentation, unique radiological findings, management, and outcome will be discussed. No similar cases of massive IVC-occlusive disease in a thrombophilic patient early in the postoperative course following neurosurgical intervention are documented in the medical literature.

Superficial temporal artery: the "C" shape half-buttonhole configuration as it courses over the zygomatic arch.

PURPOSE: To describe the course and configuration of the superficial temporal artery (STA) around the zygomatic arch. METHODS: Volume rendered 3D reconstructions of computed tomography angiography of 25 healthy patients were performed and analyzed at Duke University Hospitals. RESULTS: The STA coursed over the zygomatic arch or over the condylar process of the mandible in all cases (25/25 pts, 100 %). The STA courses over the posterior zygomatic arch in 23/25 pts (92 %), creating a characteristic "C" shape half-buttonhole configuration as it embraces the arch. When the STA travels posterior to the zygomatic arch, there is no C shape configuration (2/25 pts, 8 %). The STA bifurcates distal to the zygomatic arch in 24/25 pts (96 %). CONCLUSIONS: The "C" shape half-buttonhole configuration is a useful identifying characteristic of the most common course of the STA-over the posterior zygomatic arch before it bifurcates.

Simulation in Neurosurgery-A Brief Review and Commentary.

Neurosurgery is one of the most technically demanding and liable of all medical professionals. More than 75% of neurosurgical errors are deemed as preventable and technical in nature. Yet in a specialty that requires such high level of technical expertise, with large consequences for error, there are even fewer opportunities for residents in training to practice on the most complicated cases. Although there is no replacement for actual experiences in the operating room, interpersonal mentorship, coaching, and training, there is room to supplement residency education through simulation. Here we review the evidence to support surgical simulation, describe the strengths and weaknesses of existing technologies in direct neurosurgery specific and indirect simulation applications, and advocate for the development of more neurosurgery-specific applications using emerging kinetic technologies.

Direct Repair of Iatrogenic Internal Carotid Artery Injury During Endoscopic Endonasal Approach Surgery With Temporary Endovascular Balloon-Assisted Occlusion: Technical Case Report.

BACKGROUND AND IMPORTANCE: Iatrogenic internal carotid artery (ICA) injuries during endoscopic endonasal approach (EEA) surgeries are associated with a high morbidity and mortality, with few acceptable methods described for repair. CLINICAL PRESENTATION: A 13-year-old girl with a large anterior and central skull base osteoblastoma incurred an iatrogenic cavernous ICA injury during a staged EEA approach. Intraoperative angiogram was performed with balloon-assisted EEA primary microsurgical repair of the lacerated ICA. CONCLUSION: By integrating current techniques commonly used in open aneurysm surgeries and in endovascular procedures, we developed a rapid, safe technique to repair an EEA-associated iatrogenic ICA injury.