Impact of elexacaftor/tezacaftor/ivacaftor CFTR modulator therapy on rates of endoscopic sinus surgery in cystic fibrosis.

BACKGROUND: Elexacaftor/tezacaftor/ivacaftor (ETI), a combination cystic fibrosis transmembrane receptor (CFTR) modulator, has demonstrated improved pulmonary outcomes in individuals with cystic fibrosis (CF). However, ETI's impact on functional endoscopic sinus surgery (FESS) remains unclear. METHODS: The TriNetX Analytics Research Network, consisting of 120 million global de-identified electronic medical records, was queried from 2012 to 2023 for subjects with CF who underwent sinus surgery.1 Patients on ETI prior to FESS (n = 6,056) were propensity score matched to control individuals with CF not on CFTR modulators (n = 37,906) and those on other FDA-approved CFTR modulators (tezacaftor/ivacaftor, lumacaftor/ivacaftor, and ivacaftor) (n = 2437) based on relevant factors. The primary outcome was the absolute risk reduction (ARR) of undergoing FESS. Secondary outcomes included ARR of CF-related pulmonary exacerbations and hospital admission from 0 to 6, 6 to 12, and 12 to 24 months following FESS. RESULTS: ETI use demonstrated a significant ARR for FESS when compared to CF patients not on CFTR modulators (2.12%; 95% confidence interval [CI] 1.5-2.75; p-value < 0.0001) and those on other CFTR modulators (4.7%; 95% CI 3.54-5.85; p-value < 0.0001). No significant differences occurred in secondary outcomes between ETI and non-CFTR modulator groups, except for reduced CF-related pulmonary exacerbations from 0 to 6 months post-FESS. Additionally, a significant reduction in pulmonary exacerbations was observed at all time points and hospital admissions within 6 months following FESS compared to those using other CFTR modulators. CONCLUSIONS: In a large dataset, CF patients on ETI demonstrated significantly reduced risk of FESS, pulmonary exacerbations, and hospital admission compared to patients not on CFTR modulators or those on other CFTR modulators, suggesting improved sinonasal disease and overall health status in CF.

The role of histology on the outcome of sinonasal carcinomas treated with radiotherapy: a single institution experience.

PURPOSE: Sinonasal malignancies are a rare group of head and neck cancers. We aimed to report the oncological outcomes based on histological types in patients who underwent radiotherapy. MATERIALS AND METHODS: In this single-institution study, we retrospectively retrieved and analyzed data of patients with sinonasal carcinomas who underwent radiotherapy during 2011-2016 as part of their treatment. The 3-year rate of local, regional, and distant recurrences, and overall survival were evaluated according to the histological type. RESULTS: A total of 28 patients were evaluated in this study, the majority of whom were male (60%). Squamous cell carcinoma (SCC), adenoid cystic carcinoma (ACC), and adenocarcinoma (ADC) were found in 15 patients (53.5%), 8 (28.5%), and 5 (18%), respectively. The highest rates of local and regional recurrences were observed in ACC and SCC, respectively. Distant recurrences were numerically more common in ADC. The 3-year OS was 48%, 50%, and 73% in SCC, ADC, and ACC, respectively. CONCLUSION: Different histopathologies of sinonasal cancer seem to have different patterns of failure, and this may be considered in the treatment approach.

A Retrospective Study of Imaging of Invasive Rhino-Orbital-Cerebral Mucormycosis in the COVID-19 Pandemic in a Tertiary Care Center.

Aim The study aims to analyze the imaging findings of invasive rhino-orbital-cerebral mucormycosis (ROCM) in patients who had COVID-19. Materials and methods This retrospective descriptive study was done on confirmed (culture and histopathology) patients who had a COVID-19 infection. The data was collected from the record section from May 2021 to June 2021. Imaging data were analyzed, and findings were tabulated according to statistical methods. Results Radiological evaluation, including CT and MRI, was done in 48 cases. The ethmoid sinus was the most common sinus involved in 60.41% of cases, followed by the maxillary sinus (52.09%). Unilateral pansinusitis was observed in 21 cases (43.75%). Among periantral extensions, retroantral fat involvement was the most common finding, seen in 24 cases (50%). Lamina papyracea and the walls of the maxillary sinus were involved in eight cases (16.67%). A total of 38 cases (79.17%) exhibited involvement of the extraconal compartment, while 32 cases (66.67%) showed involvement of the intraconal compartment. In intracranial involvement, infarct was noted in 13 cases (27%), and cavernous sinus involvement in nine cases (18.75%). Conclusions ROCM is a life-threatening fungal infection in immunocompromised patients, especially diabetics. Imaging of ROCM plays a pivotal role in early diagnosis, the extent of disease, surgical planning, prognosis, and the response to treatment. Radiologists must know the imaging features and patterns of extension of ROCM.

Multi-institutional validation of an AI-based sinus CT analytic platform with olfactory assessments.

KEY POINTS: AI-based CT sinus analysis may have advantages over visual based systems, for example, Lund-Mackay score. Here, we show multi-institutional validation of an AI algorithm using novel OMC classification. Significant, robust correlations are seen between algorithm outputs and clinical outcomes.

Changes in paranasal sinus volumes, temporal bone pneumatization, internal acoustic canal and olfactory cleft dimensions over the centuries: a comparison of skulls from different epochs in Anatolia.

OBJECTIVES: Investigating changes in temporal bone pneumatization (TBP) and paranasal sinus volumes (PSV) across different eras may help understanding not only changes in skull anatomy but also pathophysiology of chronic otitis media and sinusitis, respectively, which are common health problems. METHODS: Eight skulls from the second century AD, 20 skulls were from the 10th-11th centuries AD, 20 skulls from the 16th-19th centuries AD, and 60 contemporary skulls were included in this cross-sectional observational study. Using computerized tomography (CT) scans, the PSV were calculated by multiplying the height, width, and antero-posterior distance of the sinuses. TBP was divided into three types. Internal acoustic canal (IAC) length and width, and olfactory cleft (OC) width were measured. RESULTS: No statistically significant differences were found between the paranasal sinus (frontal, maxillary, and sphenoid) volumes between the groups. However, TBP decreased statistically significantly over time on both sides of the skulls (p = 0.001). The contemporary IAC and OC measures were found to be significantly lower on both sides compared to the skulls from the other three eras (p < 0.001 for both). CONCLUSIONS: Although no significant change was observed in PSV, decreases were evident in TBP, OC width and IAC length and width over time. It appears a fair inference that changes in size of OC and IAC might be another indication of the fact that olfaction and hearing were more vital for survival in old eras. Since we do not know incidence of chronic ear problems in old eras, we cannot speculate outcome of increased TBP in terms of developing chronic ear diseases. On the contrary, increased TBP was likely to play a protective role in traumas in old ears. Additionally, the environmental influences may be crucial role in the development of paranasal sinuses.

[Analysis of imaging features of rare tumors in nasal cavity and paranasal sinus].

Objective:To explore the imaging features of rare tumors of nasal cavity and sinuses, and to improve the understanding of these diseases, thereby aiding clinical diagnosis and treatment. Methods:The CT and MRI findings of 79 cases of rare neoplasm of nasal cavity and sinuses confirmed by pathology were retrospectively analyzed, and the imaging features were summarized. Results:Among the 79 cases, there were 16 cases of neuroendocrine carcinoma, most showing expansive and infiltrative bone destruction without hyperosteogeny and sclerosis. The sphenoid sinus exhibited a "pigeon" shape. In 28 cases of malignant melanoma, MRI signals were diverse, typical signals were rare, but mixed signals were more common. In 12 cases of rhabdomyosarcoma, MRI enhancement mostly showed "grape-like" enhancement and partial ring enhancement; There were 10 cases of olfactory neuroblastoma, the lesions were consistent with the distribution area of olfactory mucosa, most of them were lobulated, marginal nodules, and "flower ring" enhancement, and 2 cases grew across intracranial and external, with multiple cystic lesions and surrounding flaky edema bands. In 5 cases of solitary fibrous tumor, Benign tumors had regular shape and uniform density, while malignant tumors had irregular shape and uneven density, The enhancement was obviously uneven and showed a "pattern" change. There were 2 cases of sarcomatoid carcinoma, both with lobed appearance, uneven density, lamellar low-density shadow, and osteolytic bone destruction. In 4 cases of schwannoma, the enhancement showed obvious inhomogeneous enhancement. One case showed cystic necrosis, one case showed calcification, and the surrounding structure was compressed without damage. There was 1 case of neurofibroma, with many cystic components, low signal separation and compartmentalized enhancement. One case of paraganglioma showed moderate enhancement in the arterial phase and progressive enhancement in the venous phase, accompanied by significant swelling bone destruction. Conclusion:Rare tumors of nasal cavity and paranasal sinuses have distinctive imaging features. CT and MRI can effectively show the extent of the lesions and the degree of infiltration into adjacent tissues and organs, which is helpful for early clinical diagnosis and staging. However, definitive diagnosis still depends on pathology and immunohistochemistry.

University of Washington Quality of Life subdomain outcomes after treatment of sinonasal malignancy: A prospective, multicenter study.

PURPOSE: Sinonasal malignancies (SNMs) adversely impact patients' quality of life (QOL) and are frequently identified at an advanced stage. Because these tumors are rare, there are few studies that examine the specific QOL areas that are impacted. This knowledge would help improve the care of these patients. METHODS: In this prospective, multi-institutional study, 273 patients with SNMs who underwent definitive treatment with curative intent were evaluated. We used the University of Washington Quality of Life (UWQOL) instrument over 5 years from diagnosis to identify demographic, treatment, and disease-related factors that influence each of the 12 UWQOL subdomains from baseline to 5 -years post-treatment. RESULTS: Multivariate models found endoscopic resection predicted improved pain (vs. nonsurgical treatment CI 2.4, 19.4, p = 0.01) and appearance versus open (CI 27.0, 35.0, p < 0.001) or combined (CI 10.4, 17.1, p < 0.001). Pterygopalatine fossa involvement predicted worse swallow (CI -10.8, -2.4, p = 0.01) and pain (CI -17.0, -4.0, p < 0.001). Neck dissection predicted worse swallow (CI -14.8, -2.8, p < 0.001), taste (CI -31.7, -1.5, p = 0.02), and salivary symptoms (CI -28.4, -8.6, p < 0.001). Maxillary involvement predicted worse chewing (CI 9.8, 33.2; p < 0.001) and speech (CI -21.8, -5.4, p < 0.001) relative to other sites. Advanced T stage predicted worse anxiety (CI -13.0, -2.0, p = 0.03). CONCLUSIONS: Surgical approach, management of cervical disease, tumor extent, and site of involvement impacted variable UWQOL symptom areas. Endoscopic resection predicted better pain, appearance, and chewing compared with open. These results may aid in counseling patients regarding potential QOL expectations in their SNM treatment and recovery course.

Rosai Dorfman Disease of Nose and Paranasal Sinuses: An Incidental Discovery.

Rosai Dorfman disease is a benign lymphoproliferative disorder. Isolated extranodal involvement is rarely encountered. The causation has not been attributed to any particular factor and the clinical progress of the disease is varied. Treatment options range from observation to medical therapy to surgical excision. Long term follow up is advocated. We report an elderly female with complaints of nasal obstruction who underwent ESS and was diagnosed with this disease on account of histopathology. Follow up period has been uneventful. The case report illustrated here is aimed at highlighting high index of suspicion as well as creating awareness regarding diagnosis and management of a rarely seen pathology.

The Relationship Between Destruction Sites and Clinical Findings in Diffuse Paranasal Sinus Mucoceles.

Objective: This study aimed to investigate that complicated mucoceles of paranasal sinuses and their clinical presentation, complications of relations with size, destructions areas, extension limits in affected patients. Materials and methods: A retrospective review was performed on patients who were diagnosed and treated as paranasal sinus mucoceles at ENT department from 2002 to 2013 years. Patients' demographic data, mucoceles location, symptoms, surgical approach and complications were evaluated. Results: The study group included 9 male and 10 female patients with a mean age of 45,0 years (range, 12-76 years). This case series include fronto-ethmoidal complex (52,6%), followed by frontal (26,3%), maxillary (15,7%) and sphenoid sinus mucoceles (5,2%). Endoscopic sinus surgery (ESS) was performed on 11 patients (57.8%); endoscopic procedures included marsupialization through an infundibulotomy, associated with an anterior ethmoidectomy and maxillary sinus antrostomy. Four patients (21%) underwent an external surgical procedure. 8 patients (42%) were treated by a combination of both endoscopic and external approaches such as trephination of the anterior wall of the frontal sinus, osteoplastic flap approach, Caldwell-Luc operation, anterior craniotomy and Lynch procedure. Complications occurred only in 2 cases (10.5%). Conclusion: The majority of patients with extensive paranasal sinus mucoceles exhibited ophthalmologic and intracranial symptoms. A satisfactory outcome can be achieved after surgical treatment of both endoscopic and external approaches.

Sinonasal Schwannoma: A Case Report.

Schwannomas are benign tumours of the peripheral nerve sheath that can develop anywhere in the body. In the nasal cavity, they hardly ever happen. Lesions known as paranasal schwannomas make up fewer than 4% of all head and neck schwannomas. Here, we present a case of sinonasal schwannoma in a 24-year-old male who had been experiencing nasal obstruction and loss of sense of smell for the previous 2 years. The lump was successfully removed without any postoperative complications.

THE PRESENCE OF FLUIDS IN THE PARANASAL SINUSES MIGHT COMPLEMENT OTHER POST-MORTEM DIAGNOSTIC METHODS TO CONFIRM OR REFUTE DROWNING AS THE CAUSE OF DEATH.

ARTICLE TITLE AND BIBLIOGRAPHIC INFORMATION: da Trindade RQ, Vieira MG, Vieira RB, Vicentin-Junior CA, Damascena NP, Santiago BM, Martins-Filho PR, Machado CE. The role of paranasal sinus fluid in determining drowning as the cause of death: a systematic review and meta-analysis. J forensic Leg Med. 2023;12:102591. SOURCE OF FUNDING: No funding was received for this review. TYPE OF STUDY/DESIGN: Systematic review (SR) with meta-analysis (MA) of data.

Sinonasal mucosal melanoma in The Netherlands between 2001 and 2021: a clinical and epidemiological overview of 320 cases.

PURPOSE: Sinonasal mucosal melanoma (SNMM) is a rare malignancy, characterised by high (local) recurrence rates and poor survival. Comprehensive understanding of tumour etiology is currently lacking, which complicates adequate tumour treatment. Besides examining trends in incidence, this study aims to assess the association between clinical characteristics, treatment practices and patient outcomes, with the objective of establishing a baseline from which SNMM management can be enhanced. METHODS: All newly diagnosed SNMM cases in The Netherlands between 2001 and 2021 were included using data from The Netherlands Cancer Registry (NCR). RESULTS: A total of 320 patients were included. The annual incidence rate for the overall population was stable over the inclusion period with an annual percentage change (APC) of only - 0.01%. The 5-year overall survival (OS) and relative survival (RS) were 24.5 and 32.4%, respectively. Relative survival did not increase over time. The addition of adjuvant radiotherapy to surgery was not associated with a higher OS and RS compared to surgery alone. CONCLUSION: Sinonasal mucosal melanoma is a rare disease with stable incidence rates in the Netherlands between 2001 and 2021. There has been no improvement in survival over the course of the inclusion period. The study reaffirms that adjuvant radiotherapy does not seem to improve patient outcomes. Given the generally poor outcomes for SNMM patients, novel therapeutic options ought to be considered in order to improve care.

Immature Teratoma of Ethmoid Sinus Masquerading as Antrochoanal Polyp: A Rare Case Report with Literature Review.

Teratomas are rare neoplasms that arise from totipotent stem cells. Teratomas of the head and neck are extremely rare, constituting about 10% of all cases and usually present in the neonatal period. Extensive literature search has shown that there are only two cases reportedof teratoma of the ethmoid sinus; one as a mature teratoma in a neonate and another was histologically immature teratoma in an adult male (Mwang'ombe et al. in East Afr Med J 79(2):106-107, 2002; Aggarwal et al. in J Postgrad Med 59(2):138-141, 2013). We hereby report the second case of immature teratoma of ethmoid sinus origin in an adult male.

Endoscopic Retrieval of a Penetrating Ethmoid Sinus Foreign Body with a Successful Minimally Invasive Approach: A Case Report.

A 14-year-old girl suffered a rare injury when a pen pierced her left eye, leading to a foreign object lodged in her ethmoid sinus. Prompt diagnosis and endoscopic sinus surgery (ESS) effectively removed the object, highlighting ESS as a valuable approach for managing uncommon sinus foreign bodies while minimizing complications.

Surgical management of advanced sinonasal cancer: a 10-year mono-institutional experience.

Objective: Endoscopic endonasal surgery is effective in the treatment of sinonasal cancers. However, in cases of well-differentiated locally advanced neoplasms as well as recurrences, the most appropriate treatment is debated. The purpose of this study is to report a mono-institutional experience on craniofacial surgery performed in a tertiary-care referral centre. Methods: This was a retrospective analysis of 90 patients treated with transcranial and/or transfacial resection for sinonasal cancer between 2010 and 2020. Outcome measures included overall survival (OS), disease-specific survival (DSS), disease-free survival (DFS) and recurrence-free survival (RFS). Results: The 5-year OS, DSS and DFS were 48.2%, 60.6% and 28.7%, respectively. Factors correlated with prognosis were pT-classification (p = 0.002), histotype (p = 0.012) and dural involvement (p = 0.004). Independent prognostic factors were orbital apex infiltration (p = 0.03), age (p = 0.002) and adjuvant therapy (p = 0.03). Conclusions: When endoscopic endonasal surgery is contraindicated and chemoradiotherapy is not appropriate, craniofacial and transfacial approaches still represent an option to consider, despite the non-negligible morbidity.

Ethmoid Osteoblastoma: A Case Report and Pooled Analysis of Cases.

Osteoblastoma is a typically noncancerous bone tumor commonly found in the spine and long bones of the arms and legs. It is exceedingly rare for this tumor to occur in the paranasal sinuses. We present a case of osteoblastoma in a 13-year-old boy affecting the ethmoid sinus, which manifested as exophthalmos. A computed tomography scan revealed an expansive lesion in the right ethmoid sinus, causing compression and displacement of the orbital contents to the right. The lesion exhibited a mix of ground glass opacity and dense bone. On magnetic resonance imaging, the less dense areas of the tumor showed strong enhancement, while the densely sclerotic regions appeared as signal voids on all imaging sequences. A combined transorbital and transnasal approach was performed to remove the tumor. Histologically, the tumor consisted expansile growth surrounded by a sclerotic rim of inter-anastomosing trabeculae of woven bone set within loose edematous fibrovascular stroma. This case highlights the unusual occurrence of osteoblastoma in the ethmoid sinus, a location seldom associated with this type of tumor, and adds to the existing literature on this topic and offers a new surgical approach to managing this entity.

Systematic review of non-invasive ventilation and craniofacial fractures: a multidisciplinary perspective and recommendations for management.

The anatomically complex craniofacial skeleton demands special consideration when caring for cases of polytrauma or medically compromised patients with craniofacial fractures. This paper utilises a systematic review and multidisciplinary opinions to create an algorithm for the hospital-based care of patients with craniofacial fractures (base of skull, orbit, paranasal sinus, and mandible) who require non-invasive ventilation (NIV). Each fracture location has a unique predisposition to a different type of emphysema and associated morbidity. The risk of developing emphysema, combined with its potential severity, is stratified against the harm of not providing NIV for the holistic care of the patient. The aim of this paper is to synthesise evidence from a systematic review of existing literature with multidisciplinary opinions to develop a concise algorithm that outlines the optimal treatment of patients with craniofacial fractures who require NIV.

Evaluation of Some Prognostic Biomarkers in Human Papillomavirus-Related Multiphenotypic Sinonasal Carcinoma.

Background: Human papillomavirus (HPV)-related multi phenotypic sinonasal carcinoma (HMSC) is a recently described tumor subtype with an unknown prognosis, often misdiagnosed with other sinonasal carcinomas, and associated with high-risk HPV (HR-HPV). The present study aimed to evaluate the expression of vascular endothelial growth factor (VEGF), Bcl-2-associated X protein (BAX), epidermal growth factor receptors (EGFR), ProExTMC, and human telomerase reverse transcriptase (hTERT) and assess their association with survival and clinicopathological characteristics. Methods: Between 2017 and 2022, 40 HMSC patients underwent surgical resection at the School of Medicine, Zagazig University Hospitals (Zagazig, Egypt). Tissue samples were examined for the presence of HR-HPV; absence of myeloblastosis (MYB), MYB proto-oncogene like 1 (MYBL1), and nuclear factor I/B (NFIB) fusions and the presence of myoepithelial proteins (calponin, S100, SMA), squamous differentiation markers (p63, p40, calponin), VEGF, BAX, ProExTMC, and hTERT by immunohistochemistry. All patients were followed up for about 54 months until death or the last known survival data. Data were analyzed using the Chi square test and Kaplan-Meier method. Results: The expression of VEGF, hTERT, and ProExTMC was significantly associated with age, advanced tumor stages, lymph node metastasis, tumor size, mortality, relapse, poor disease-free survival (DFS), and overall survival (OS) (P<0.001). BAX expression was significantly associated with tumor size, age, poor DFS, and relapse (P=0.01, P<0.001, P=0.035, and P=0.002, respectively). Conclusion: HMSC is strongly associated with HR-HPV. The expression of VEGF, EGFR, BAX, hTERT, and ProExTMC is associated with aggressive malignant behavior, poor survival, and poor prognosis, making them novel prognostic biomarkers for targeted therapeutics in HMSC.

Anosmia in COVID-19: Investigating the Role of Paranasal Sinus Mucosal Thickening.

Background Anosmia has been identified as a distinctive symptom of COVID-19, leading to hypotheses about its pathophysiological underpinnings, including the potential role of paranasal sinus mucosal thickening. Objective To investigate the association between paranasal sinus mucosal thickening and anosmia in COVID-19 patients, providing insights into the complex clinical manifestations of the disease. Methods This retrospective cohort study analyzed CT paranasal sinus from 270 confirmed COVID-19 patients, divided into those with anosmia (n = 23, 8.52%) and those without anosmia (n = 247, 91.48%). Statistical analysis, including independent t-tests, was employed to compare mucosal thickening between the groups. Results The study found an average mucosal thickening of 0.03 in patients with anosmia and 0.02 in those without, with no statistically significant difference between the groups (p = 0.480, which is greater than 0.05). The findings suggest that mucosal thickening in the paranasal sinuses does not serve as a definitive correlate of anosmia among COVID-19 patients. Conclusion The absence of a significant correlation between paranasal sinus mucosal thickening and anosmia in COVID-19 patients indicates that the pathophysiology of anosmia may involve factors beyond anatomical changes, including direct viral effects and systemic inflammatory responses.