Sinonasal Chondrosarcoma Presenting With Isolated Severe Vision Loss.

ABSTRACT: A 24-year-old man presented with a 2-month history of progressive, painless vision loss in the right eye, with no history of headache, nasal congestion, rhinorrhea, or epistaxis. His visual acuity was counting fingers at 1 ft in the right eye and 20 of 20 in the left eye with a right relative afferent pupillary defect and mild temporal optic disc pallor. MRI of the brain and orbits showed a mass involving bilateral ethmoid and sphenoid sinuses and right nasal cavity. He underwent urgent extended endoscopic endonasal transsphenoidal approach for resection of the sinonasal skull base tumor and photon radiation therapy. Pathology revealed a well-differentiated cartilaginous neoplasm with focal areas of entrapped native bone, consistent with a chondrosarcoma WHO grade I/III. At 6-month follow-up after surgery, he had a visual acuity of 20/40 in the right eye and 20/20 in the left eye. Malignant tumors from the sinonasal area should be kept in the differential diagnosis for compressive optic neuropathies and may present with vision loss even in the absence of nasal or sinus symptoms.

The displacement technique: a simple method for clearing maxillary sinus stubborn mucus.

BACKGROUND: Clearing thick secretions from the hidden crevices of the maxillary sinus can be challenging and can increase operative time during endoscopic sinus surgery. METHODS: We demonstrate the Displacement Technique as a user-friendly technique to address thick secretions within the maxillary sinus barring no orbital floor dehiscence. First, a wide maxillary antrostomy is performed. Then, a surgical pledget is placed in the maxillary sinus to displace the inspissated content into the nasal cavity using a curved suction. This process is repeated as necessary. CONCLUSION: The displacement technique is a simple and inexpensive method for addressing stubborn secretions.

Disgust but not Health Anxiety Graphic Warning Labels Reduce Motivated Attention in Smokers: A Study of P300 and Late Positive Potential Responses.

Background: Graphic health warning labels (GHWLs) on tobacco products attempt to leverage avoidance-promoting emotions, such as anxiety and disgust, to encourage cessation. Prior studies have relied on self-report or attentional metrics that may not accurately illuminate GHWLs' ability to motivate change. This report evaluates the impact of disgust- and anxiety-based GHWLs on electroencephalograph (EEG) measures of motivated attention among two groups of smokers-those that report higher versus lower cigarette dependence. We hypothesized that both anxiety and disgust GHWLs would reduce appetitive attention, as indexed by lowered P300 (P3) and late positive potential (LPP) activations. Methods: Sixty-one smokers provided demographic and smoking history before completing an oddball paradigm consisting of three counterbalanced stimuli blocks. Each block (100 trials) contained a neutral, GHWL-anxiety, or GHWL-disgust frequent image and a smoking cue as the oddball image (20%). Oddball trials for each block were averaged, P3 and LPP were identified at midline electrode positions (Fz, Cz, and Pz), and mean amplitude was analyzed. Results: Separate mixed-model ANOVAs of P3 and LPP reactivity revealed disgust-focused GHWLs reduced motivated attentional processing. Conversely, the anxiety-focused GHWL appeared to increase the salience of the smoking cue (Fz only). Less-dependent smokers showed lower P3 reactivity than those with higher dependence at Fz, but greater P3 reactivity at Cz and Pz. Conclusion: These results extend prior work in demonstrating that disgust, but not anxiety-based GHWLs, may reduce EEG-assessed motivated attention to smoking cues. Disgust may thus represent a more fruitful target for public health cessation efforts. Implications: Most GHWL evaluations have focused on fear (or anxiety) elicitation rather than disgust, an emotion that may have a unique link to smoking, having evolved specifically to facilitate the avoidance of contaminants via oral incorporation. Analyses of P300 and LPP responses to GHWLs suggest that disgust-focused images interfere with the EEG-indexed attentional processing of smoking cues and do so better than health anxiety-focused messages. However, interaction effects at different electrode sites indicated that GHWLs have complex effects in more versus less-dependent smokers and that an understanding of how smoking cues and anti-smoking imagery become associated over time is needed to identify relevant targets for public health efforts.

Visual cue-specific craving is diminished in stressed smokers.

BACKGROUND: Craving among smokers is increased by stress and exposure to smoking-related visual cues. However, few experimental studies have tested both elicitors concurrently and considered how exposures may interact to influence craving. OBJECTIVE: The current study examined craving in response to stress and visual cue exposure, separately and in succession, in order to better understand the relationship between craving elicitation and the elicitor. METHOD: Thirty-nine smokers (21 males) who forwent smoking for 30 minutes were randomized to complete a stress task and a visual cue task in counterbalanced orders (creating the experimental groups); for the cue task, counterbalanced blocks of neutral, motivational control, and smoking images were presented. Self-reported craving was assessed after each block of visual stimuli and stress task, and after a recovery period following each task. RESULTS: As expected, the stress and smoking images generated greater craving than neutral or motivational control images (p < .001). Interactions indicated craving in those who completed the stress task first differed from those who completed the visual cues task first (p < .05), such that stress task craving was greater than all image type craving (all p's < .05) only if the visual cue task was completed first. Conversely, craving was stable across image types when the stress task was completed first. CONCLUSIONS: Findings indicate when smokers are stressed, visual cues have little additive effect on craving, and different types of visual cues elicit comparable craving. These findings may imply that once stressed, smokers will crave cigarettes comparably notwithstanding whether they are exposed to smoking image cues.

Prominent dysautonomia in a patient with POEMS syndrome.

POEMS syndrome is a rare, multisystem disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and/or skin changes. Here we present an unusual case of a patient with POEMS syndrome who exhibited a prominent autonomic neuropathy.

Farnesylation of lamin B1 is important for retention of nuclear chromatin during neuronal migration.

The role of protein farnesylation in lamin A biogenesis and the pathogenesis of progeria has been studied in considerable detail, but the importance of farnesylation for the B-type lamins, lamin B1 and lamin B2, has received little attention. Lamins B1 and B2 are expressed in nearly every cell type from the earliest stages of development, and they have been implicated in a variety of functions within the cell nucleus. To assess the importance of protein farnesylation for B-type lamins, we created knock-in mice expressing nonfarnesylated versions of lamin B1 and lamin B2. Mice expressing nonfarnesylated lamin B2 developed normally and were free of disease. In contrast, mice expressing nonfarnesylated lamin B1 died soon after birth, with severe neurodevelopmental defects and striking nuclear abnormalities in neurons. The nuclear lamina in migrating neurons was pulled away from the chromatin so that the chromatin was left "naked" (free from the nuclear lamina). Thus, farnesylation of lamin B1--but not lamin B2--is crucial for brain development and for retaining chromatin within the bounds of the nuclear lamina during neuronal migration.

Absorbable Steroid-Impregnated Spacer After Endoscopic Sinus Surgery to Reduce Synechiae Formation.

OBJECTIVE: Middle meatal synechiae formation after endoscopic sinus surgery (ESS) for chronic rhinosinusitis (CRS) leads to higher rates of revision surgery. We aimed to determine if there are different rates of postoperative synechiae following ESS for CRS between patients who received a nonabsorbable spacer (NAS) placed in the ethmoid cavity versus patients who had a steroid-impregnated absorbable spacer (AS) placed at the middle meatal entry. METHODS: We conducted a retrospective cohort study of patients who underwent ESS for CRS at a single tertiary referral center from October 2009 to October 2013. We collected data on synechiae formation between the middle turbinate and lateral nasal wall within 1 month of surgery and postoperative epistaxis. RESULTS: One hundred forty-six patients with 252 nasal cavities (52.0%) received steroid-impregnated AS, and 128 patients with 233 nasal cavities (48.0%) received NAS. Synechiae formation occurred in 2.0% of cavities with AS and 5.6% of cavities with NAS, but this difference was not statistically significant (OR = 0.34, P = .052). One patient in each cohort had significant postoperative epistaxis requiring additional nasal packing (P > .99). CONCLUSION: Steroid-impregnated absorbable spacers had statistically insignificant reduction in postoperative synechiae formation when compared to nonabsorbable spacers. Low rates of postoperative epistaxis were observed regardless of the type of spacer used.

Alzheimer's disease and chronic traumatic encephalopathy: Distinct but possibly overlapping disease entities.

BACKGROUND: Alzheimer's disease (AD) and chronic traumatic encephalopathy (CTE) have long been recognized as sharing some similar neuropathological features, mainly the presence of neurofibrilary tangles and hyperphosphorylated tau, but have generally been described as distinct entities. Evidence indicates that neurotrauma increases the risk of developing dementia and accelerates the progression of disease. Findings are emerging that CTE and AD may be present in the same patients. CLINICAL PRESENTATION: This study presents a series of previously unpublished cases, with one case demonstrating possible neurotrauma-related AD, one pure CTE, and an example of a case exhibiting features of both AD and CTE. The future significance of this work lies not only in the confirmation of AD-CTE co-existence, but, more importantly, ways of generating a hypothesis about the possibility that CTE may accelerate AD development. Understanding the relationship between neurotrauma and neurodegenerative disease will help elucidate how distinct disease entities can co-exist in the same patient. It will ultimately require the use of pre-clinical animal models and repeat injury paradigms to investigate clinically relevant injury mechanisms. These models should produce a CTE-like phenotype that must be both neuropathologically and behaviourally similar to human disease. CONCLUSION: This case series and review of the literature presents a discussion of AD and CTE in the context of neurotrauma. It highlights recent work from repetitive neurotrauma models with an emphasis on those exhibiting a CTE-like phenotype. Potential mechanisms of interest shared amongst AD and CTE are briefly addressed and future experiments are advocated for to enhance understanding of CTE pathophysiology and the relationship between CTE and AD.

Skull base embryology: a multidisciplinary review.

INTRODUCTION: The skull base represents a central and complex bone structure of the skull and forms the floor of the cranial cavity on which the brain lies. Anatomical knowledge of this particular region is important for understanding several pathologic conditions as well as for planning surgical procedures. Embryology of the cranial base is of great interest due to its pronounced impact on the development of adjacent regions including the brain, neck, and craniofacial skeleton. MATERIALS AND METHODS: Information from human and comparative anatomy, anthropology, embryology, surgery, and computed modelling was integrated to provide a perspective to interpret skull base formation and variability within the cranial functional and structural system. RESULTS AND CONCLUSIONS: The skull base undergoes an elaborate sequence of development stages and represents a key player in skull, face and brain development. Furthering our holistic understanding of the embryology of the skull base promises to expand our knowledge and enhance our ability to treat associated anomalies.

Myoepithelioma of the Orbital Apex and Middle Cranial Fossa: Case Report and Review of the Literature.

Myoepitheliomas are rare tumours that originate from glandular tissues such as the parotid or salivary glands, and less commonly from soft tissues of the head, neck, and other parts of the body. Intraorbital myoepitheliomas generally arise from the lacrimal gland. Intracranial myoepitheliomas are rare. We report a myoepithelioma of the orbital apex that did not originate from the lacrimal gland. It extended to the middle cranial fossa from the orbital apex and involved the dura and adjacent bone. A diagnostic biopsy via a lateral orbitotomy preceded resection. We review the natural course and histopathology of myoepithelial neoplasms, the surgical nuances of approaching an orbital apex tumour with maximal functional preservation, and the optimal management practices of these rare lesions.

Levels of nasal nitric oxide and nitric oxide synthase expression in chronic rhinosinusitis with nasal polyposis.

KEY POINTS: CRSwNP patients had decreased nNO and increased SNOT-22, endoscopy, and CT scores. CRSwNP patients exhibited decreased nNO despite elevated iNOS and eNOS mRNA expression. The mechanism behind lowered nNO in CRSwNP may not be related to NOS expression.

Assessment of a large language model's utility in helping pathology professionals answer general knowledge pathology questions.

OBJECTIVES: To assess the utility and performance of the large language model ChatGPT 4.0 regarding accuracy, completeness, and its potential as a time-saving tool for pathologists and laboratory directors. METHODS: A deidentified database of questions previously sent to pathology residents from health care providers was used as a source of general knowledge-type pathology questions. These questions were submitted to the large language model and the responses graded by subject matter experts in different pathology subspecialties. The grading criteria assessed accuracy, completeness, and the potential time savings for helping the pathologist craft the response. RESULTS: Overall, respondents thought that most of the answers would take less than 5 minutes of additional work to be used (85%). Accuracy and completeness for the 61 questions was high, with 98% of responses being at least "completely or mostly accurate" and 82% of responses "containing all relevant information." Of the respondents, 97% stated that the response would have "zero or near-zero potential for medical harm," and all thought the tool had potential to save time in constructing answers to health care providers' queries. Performance was similar in both Anatomic Pathology (AP) and Clinical Pathology (CP), with the only exception being some relevant information was excluded in 46% of AP answers vs only 10% in CP (P < .01). CONCLUSIONS: ChatGPT version 4.0 gave responses that were predominantly accurate and complete for general knowledge-type pathology questions. With further research and when reviewed by a pathologist or laboratorian, this could facilitate its use as a pathologist's aid in answering questions from health care providers.

High prevalence of fibromyalgia in patients with HFE-related hereditary hemochromatosis.

OBJECTIVES: Subjects with HFE-related hereditary hemochromatosis (HH) may present with arthralgias, fatigue, and stiffness, yet little is known on the presence of fibromyalgia syndrome (FMS) in these subjects. We determined the prevalence of FMS in a cohort of subjects with HH and evaluated its relationship to subject demographics, disease status, and quality of life. METHODS: In a cross-sectional study we collected data on 395 consecutive subjects diagnosed with HH who were attending a tertiary referral Hepatology outpatient clinic at Galway University Hospital, Ireland (between October 2009 and June 2010). Subjects underwent a standard assessment including history, clinical examination, and functional assessments for pain and disability. Univariate logistic regression was applied to determine risk factors independently associated with prevalent FMS in these subjects. RESULTS: Three hundred ninety-five subjects met the inclusion criteria. Mean age was 43 years (range, 21 to 59 y) and 260 (66%) were males. One hundred seventy (43%) of the subjects were diagnosed with FMS. Among those with fibromyalgia fatigue and ≥ 11 tender points were present in all of the subjects, widespread pain in 150 (88%), depression in 70 (41%), and arthralgia/joint stiffness in 70 (41%). In subjects with FMS 33% reported some functional impairment (HAQ-DI>0), with 10% reporting moderate-severe functional impairment (HAQ-DI ≥ 1.5). CONCLUSIONS: This study reveals a high prevalence of FMS (43%) among subjects with HFE-related hemochromatosis. Prospective studies are needed to better understand the risk factors for FMS in such patients.

Primary Osteosarcoma of the Skull Base in a Pregnant Patient.

An 18-year-old female who was 14 weeks pregnant first noted vision loss in her left eye six days prior to admission. Ophthalmologic examination revealed 20/20 vision in the right eye and count fingers vision in the left eye. A marked relative afferent pupillary defect was present in the left eye. Ophthalmoscopic examination revealed a trace optic nerve pallor temporally in the left eye without associated disc oedema or haemorrhage. Magnetic resonance imaging of the brain demonstrated a heterogeneous mass of the left sphenoid sinus extending superiorly causing compression of the intracranial portion of the left optic nerve, and laterally into the left cavernous sinus. The patient underwent transphenoidal resection of the tumour whose histologic morphology revealed a grade 2 osteosarcoma. Following resection, vision returned to 20/20 in the left eye. The patient has been treated with chemotherapy with close monitoring of her pregnancy.

Endoscopic sinus surgery outcomes in patients with chronic rhinosinusitis and immunoglobulin deficiencies.

BACKGROUND: Patients with chronic rhinosinusitis (CRS) and immunoglobulin deficiencies (ID) have more recalcitrant sinonasal disease and a subset of these patients undergo surgical management for their CRS. However, there is a paucity of literature on the surgical outcomes in this patient population and appropriate treatment algorithms for CRS in patients with ID. The objective of this study was to better elucidate the outcomes of endoscopic sinus surgery (ESS) in patients with ID in terms of disease-specific quality-of-life scores and the need for revision surgery. METHODS: A case-control study was performed comparing adult patients with ID and healthy controls that had undergone ESS for CRS. Patients were matched based on age, sex, CRS phenotype, and preoperative Lund-Mackay score. The revision surgery rates, time to revision surgery, and changes in sinonasal outcome tests (SNOT-22) were evaluated. RESULTS: Thirteen patients with CRS and ID were matched to 26 control patients with CRS. The revision surgery rate for cases and controls was 31% and 12%, respectively, but there was no statistical difference (p > 0.05). There was a clinically meaningful reduction in SNOT-22 scores in both groups from the preoperative to postoperative period [mean of 12 points in patients with ID (p = 0.323) and 25 points in controls (p < 0.001)], however, there was again no significant difference between cases and controls (p > 0.05). CONCLUSION: Our data suggests that patients with ID have clinically meaningful improvement in SNOT-22 scores after ESS but may have higher revision rates than immunocompetent patients with CRS. ID are rare disease entities, thus most attempts at studying this cohort would be limited by sample size. Further homogenous data on immunoglobulin deficient patients is required for future meta-analysis to better understand the impact of ESS in patients with ID.

Cryoablation for the treatment of chronic rhinitis: a systematic review.

BACKGROUND: ClariFix is a novel intranasal cryotherapy device developed for clinic-based cryosurgical ablation of the posterior nasal nerves region. As a relatively new technology, there is a paucity of studies within the literature assessing the efficacy and safety profile of ClariFix for chronic rhinitis. METHODS: A systematic review was completed in accordance with PRISMA guidelines. Databases searched included: Ovid Medline, Ovid EMBASE, Pubmed, Cochrane and Web of Science. Inclusion criteria consisted of studies investigating the use of ClariFix in chronic rhinitis (i.e., allergic and non-allergic rhinitis) in patients of all ages. RESULTS: The initial search identified 1110 studies. Final analysis consisted of 8 articles, evaluating a total of 472 patients. The data showed a significant reduction in scores post-treatment across all studies based on validated outcome measures. In all studies, at all time intervals, there was a significant improvement in outcome scores from baseline. Minor adverse effects included post-procedural pain and discomfort, headache and palate numbness. No major adverse events were identified. CONCLUSION: ClariFix is a novel intranasal cryotherapy device that was introduced in Canada in 2021. This is the first systematic review evaluating its efficacy and safety profile. Across all studies, there was a significant reduction in validated outcome scores at multiple time intervals. Further, the treatment is safe with only minor adverse effects reported by patients. Overall, the consensus from this study highlights an apparent benefit in using this intervention for chronic rhinitis that is refractory to medical management.

Clinical outcomes of dupilumab therapy in chronic rhinosinusitis with nasal polyps in a Canadian tertiary care rhinology practice.

BACKGROUND: In 2020, dupilumab became the first monoclonal antibody therapy to be approved by Health Canada for the treatment of chronic rhinosinusitis with nasal polyps (CRSwNP). The primary aim of this study was to characterize the outcomes in an initial cohort of patients with CRSwNP who have undergone dupilumab therapy. METHODS: A retrospective study was conducted of patients with CRSwNP who were treated with dupilumab. Demographic information, comorbidities, number of previous surgeries, and insurance information were collected. The primary outcome were changes in the sinonasal outcome test (SNOT-22) scores from baseline to timepoints after receiving dupilumab. RESULTS: Forty-eight patients were considered for dupilumab therapy, and 27 (56%) received coverage or were able to fund the medication independently. Patients waited an average of 3.6 months to obtain access to the medication. The mean age of the patients was 43. Forty-one percent (11/27) of patients had aspirin exacerbated respiratory disease, and 96% (26/27) had a diagnosis of asthma. The mean length of time on dupilumab was 12.1 months. The baseline SNOT-22 score was 60.6. The mean decrease at 1 month, 3 months, 6 months, and 12 months after starting dupilumab was 8.8, 26.5, 42.8, and 33.8, respectively. There were no serious adverse events. CONCLUSION: Patients treated with dupilumab in a Canadian tertiary care rhinology clinic demonstrated substantial clinical improvement as measured by disease-specific sinonasal outcomes. Further studies are needed to determine the longer-term effectiveness and adverse event profile of this novel therapy.

Quantifying surgical completeness in patients with aspirin exacerbated respiratory disease.

BACKGROUND: Aspirin exacerbated respiratory disease (AERD) in patients who have had sinus surgery remains a management challenge. Aspirin desensitization and biologics are additional treatment options. It remains unclear if patients require a more comprehensive surgery prior to implementing such additional therapies. The purpose of this study was to quantify prior surgery completeness in AERD patients at a tertiary rhinology practice. METHODS: Paranasal sinus CT scans were reviewed by four academic rhinologists to assess surgery completeness. Using a published CT grading system, each sinus was graded on the completeness of surgery and middle turbinate reduction. A score out of 14 was calculated for each patient (7 per side). RESULTS: Sixty-one patients with AERD out of 141 available were included. Mean inter-rater agreement across all sinuses was moderate (k = 0.42). The mean completeness score was 6.7/14. The following procedures were rated as complete (means): uncinectomy (L: 84%, R: 82%, k = 0.44), maxillary (L: 83%, R: 77%, k = 0.32), middle turbinate reduction (L: 45%, R: 46%, k = 0.31), anterior ethmoid (L: 35%, R: 39%, k = 0.51), sphenoid (L: 36%, R: 35%, k = 0.4), posterior ethmoid (L: 30%, R: 30%, k = 0.48), frontal (L: 22%, R: 21%, k = 0.46). CONCLUSION: Prior surgery in AERD patients were mostly deemed incomplete. Uncinectomy and maxillary antrostomy are the most common procedures previously performed. It remains toe seen whether this would be considered 'adequate' surgery or more 'complete' surgery is required to achieve greater disease control.