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To perform a population-based study of pituitary adenoma epidemiology, including longitudinal trends in disease incidence, treatment patterns, and outcomes.In this study of incident pituitary adenomas in Olmsted County, Minnesota, from January 1, 1989, through December 31, 2019, we identified 785 patients who underwent primary screening, 435 of whom were confirmed as harboring incident pituitary adenomas and were included. Primary outcomes of interest included demographic characteristics, presenting features, and disease outcomes (tumor control, biochemical control, and complications).Among our 435 study patients, 438 unique pituitary adenomas were diagnosed at a median patient age of 39 years (interquartile range [IQR], 27 to 58 years). Adenomas were incidentally identified in 164 of the 438 tumors (37%). Common symptomatic presentations included hyperprolactinemia (188 of 438 [43%]) and visual field deficit (47 of 438 [11%]). Laboratory tests confirmed pituitary hormone hypersecretion in 238 of the 435 patients (55%), which was symptomatic in 222. The median tumor diameter was 8 mm (IQR, 5 to 17 mm). Primary management strategies were observation (156 of 438 tumors [36%]), medication (162 of 438 tumors [37%]), and transsphenoidal resection (120 of 438 tumors [27%]). Tumor and biochemical control were achieved in 398 (95%) and 216 (91%) secreting tumors, respectively. New posttreatment pituitary or visual deficits were noted in 43 (11%) and 8 (2%); apoplexy occurred in 28 (6%). Median clinical follow-up was 98 months (IQR, 47 to 189 months). Standardized incidence rates were 3.77 to 16.87 per 100,000 population, demonstrating linear expansion over time (RPituitary adenoma is a highly incident disease, with prolactin-secreting and incidental lesions representing the majority of tumors. Incidence rates and asymptomatic detection appear to be increasing over time. Presenting symptoms and treatment pathways are variable; however, most patients achieve favorable outcomes with observation or a single treatment modality.

The vomer-rostrum mucosal flap is a useful technique utilizing vascularized mucosa of the rostrum and posterior septum to cover exposed hyperostotic bone following wide sphenoidotomy surgery. Laryngoscope, 2022.

Postoperative management strategies for endoscopic skull base surgery (ESBS) vary widely because of limited evidence-based guidance.The PubMed, EMBASE, and Cochrane databases were systematically reviewed from January 1990 through February 2022 to examine 18 postoperative considerations for ESBS. Nonhuman studies, articles written in a language other than English, and case reports were excluded. Studies were assessed for levels of evidence, and each topic's aggregate grade of evidence was evaluated.A total of 74 studies reporting on 18 postoperative practices were reviewed. Postoperative pain management, prophylactic antibiotics, and lumbar drain use had the highest grades of evidence (B). The literature currently lacks high quality evidence for a majority of the reviewed ESBS precautions. There were no relevant studies to address postoperative urinary catheter use and medical intracranial pressure reduction.The evidence for postoperative ESBS precautions is heterogeneous, scarce, and generally of low quality. Although this review identified the best evidence available in the literature, it suggests the urgent need for more robust evidence. Therefore, additional high-quality studies are needed in order to devise optimal postoperative ESBS protocols.

Esthesioneuroblastoma (ENB) is a rare malignant neoplasm arising from the olfactory epithelium of the cribriform plate. The goal of this study was to update our oncologic outcomes for this disease and explore prognostic factors associated with survival.We performed a retrospective analysis of patients with ENB treated at a single tertiary care institution from January 1, 1960, to January 1, 2020. Univariate and multivariate analysis was performed. Overall survival (OS), progression-free survival (PFS), and distant metastasis-free survival (DMFS) were reported.Among 143 included patients, the 5-year OS was 82.3% and the 5-year PFS was 51.6%; 5-year OS and PFS have improved in the modern era (2005-present). Delayed regional nodal metastasis was the most common site of recurrence in 22% of patients (median, 57 months). On univariate analysis, modified Kadish staging (mKadish) had a negative effect on OS, PFS, and DMFS (p 0.05). Higher Hyams grade had a negative effect on PFS and DMFS (p 0.05). Positive margin status had a negative effect on PFS (p 0.05). Orbital invasion demonstrated worsening OS (hazard ratio, 3.1; p 0.05). On multivariable analysis, high Hyams grade (3 or 4), high mKadish stage (C+D), and increasing age were independent negative prognostic factors for OS (p 0.05). High Hyams grade (3+4), high mKadish stage (C+D), age, and positive margin status were independent negative prognostic factors for PFS (p 0.05). High Hyams grade (3+4) was an independent negative prognostic factor for DMFS (p 0.05).Patients with low Hyams grade and mKadish stage have favorable 5-year OS, PFS, and DMFS.

While pedicled intranasal flaps, such as the nasoseptal flap (NSF), successfully reduce postoperative CSF leaks following endoscopic endonasal approaches (EEA) to the skull base, morbidity remains prevalent. This study describes the first cadaveric description and radiographic analysis of the posterior septal nasal floor mucosal flap (PSNF) designed to reduce pedicled nasal flap morbidity. A case series is also detailed.Cadaveric dissection, radiographic analysis, and case series.Seven cadaver specimens underwent harvest of the PSNF. PSNF flap dimensions were measured on the thirty deidentified sinus computed tomography (CT) scans. A retrospective case series was performed on the first set of patients who received the PSNF for reconstruction of a sellar base defect. Information concerning complications, symptoms, and the appearance of the flap was recorded from records of the patient's postoperative inpatient stay, 1-week postop visit, and 1-month postop visit.Cadaver dissection of the PSNF flap demonstrated good arc rotation along its pedicle with ability to cover both sellar and planum defects. Cadaveric flap measurements and CT-derived measurements estimated a mean surface area of 16.7 and 18.3 cmThe PSNF flap provides a sizeable pedicled region for reconstruction comparable to other pedicled nasal flaps. Our case series demonstrated good postoperative outcomes without reduced donor site morbidity.4 Laryngoscope, 132:1561-1568, 2022.

Esthesioneuroblastoma is a rare malignancy originating from the olfactory epithelium. Treatment consists of surgical resection with strong consideration for adjuvant treatment in advanced Kadish stage and high Hyams grade. In the modern era, overall outcomes for esthesioneuroblastoma are favorable compared with many other sinonasal malignancies with 5-year overall survival estimated to be 80%. When selecting the optimal surgical approach, the surgeon must consider the approach that will allow for a negative margin resection and adequate reconstruction. In appropriately selected patients, endoscopic outcomes appear at least equivalent to open approaches and unilateral endoscopic approach may be used in select olfactory preservation cases.

PURPOSE In Prolactinomas, surgery or radiation are reserved for DA failure due to tumor resistance, intolerance to medication-induced side-effects, or patient preference. This systematic review and meta-analysis summarizes the currently available literature regarding the effectiveness of surgery to treat prolactinomas in patients who have failed DA therapy. METHOD A literature search was conducted according to PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines for studies that reported outcomes of medically resistant and intolerant prolactinoma treated surgically. RESULTS 10 articles (Total N = 816, Surgery N = 657) met the inclusion criteria. 38% of patients who underwent surgery following DA failure achieved remission without need for further treatment (p

Objective Primary resection of adrenocorticotropic hormone (ACTH)-secreting pituitary adenoma has become a front-line standard-of-care treatment for Cushing disease. However, surgical intervention can be challenging because of elevated blood pressure, as well as direct cortisol impacts on endothelial cells, vascular permeability, and tissue friability—potentially resulting in increased intraoperative bleeding. Tranexamic acid (TXA) is a well-studied, widely used intravenous hemostatic; however, the potential benefit during resection of ACTH-secreting pituitary adenoma is unstudied. The purpose of this study was to define an institutional protocol for perioperative administration of TXA in patients undergoing endoscopic endonasal approach for resection of ACTH-secreting pituitary adenoma, and to study the implementation of our novel protocol in a prospective fashion. Methods Criteria for preoperative TXA were defined by age, medical history, and risk factors. Descriptive statistics were reported for all patients receiving perioperative TXA. Results Thirty patients met inclusion criteria and underwent perioperative administration of TXA, using a standardized dosing protocol of a 10 mg/kg bolus in 30 minutes prior to incision, followed by maintenance infusion of 2 mg/kg/hour for the duration of the procedure. No incidence of myocardial infarction or postoperative thromboembolic events were noted. Subjective assessments indicated satisfaction with the patient selection protocol, and meaningful reduction in the extent of intraoperative bleeding. Conclusions Perioperative TXA represents a potentially efficacious approach for control of intraoperative bleeding during endonasal resection of ACTH-secreting tumors. Careful preoperative patient selection is emphasized, given the potential for thromboembolic complications; however, initial experience with our institutional protocol suggests a favorable risk/benefit profile when this treatment is applied judiciously.

Skull base chordoma is a rare and locally destructive malignancy which presents unique therapeutic challenges. While achieving gross total resection (GTR) confers the greatest survival advantage, the role of adjuvant radiotherapy (RT) for patients who receive GTR remains unclear in the absence of prospective trials. Here, we aim to assess the effect of RT on survival outcomes in skull base chordoma patients who receive GTR by utilizing the Surveillance, Epidemiology, and End Results (SEER) database.Patients with diagnostic, primary site, and resection codes specific for chordoma, skull base, and GTR, respectively, were queried in the SEER database (2000-2018). Kaplan-Meier curves (log-rank test) were constructed and Cox proportional hazards models were used to assess survival outcomes.A total of 115 skull base chordomas undergoing GTR were identified, of which 37 (32%) received no RT and 78 (68%) received RT. Median follow up was 55.00 months (range: 0.00 - 227.00). OS of patients with GTR was 85% and 70% at 5 and 10 years, respectively. Multivariate Cox proportional hazard analysis among chordoma patients undergoing GTR found age65 (p0.01) was associated with poorer OS outcomes. RT appeared to trend towards offering benefit in terms of OS in patients after GTR, however this did not achieve statistical significance in the adjusted model (HR=0.51, CI=0.23-1.16, p=0.09). When comparing disease-specific survival, DSS was also not improved in patients undergoing RT (HR=0.58, CI=0.23-1.46, p=0.25).It remains unclear whether RT after GTR of chordoma improved survival outcomes among SEER database patients.

Aspirin-exacerbated respiratory disease (AERD) is characterized by abnormal arachidonic acid metabolism leading to chronic rhinosinusitis with nasal polyposis (CRSwNP), asthma, and upper and/or lower respiratory symptoms after ingestion of cyclooxygenase-1 inhibiting nonsteroidal antiinflammatory drugs. Diagnosis is clinical and may involve an aspirin challenge. Inflammatory biomarkers may be useful for diagnosis and treatment monitoring. Conventional medical management for asthma and CRSwNP is often inadequate. Endoscopic sinus surgery followed by continued medical management with or without aspirin desensitization frequently improves symptoms and objective disease measures. Biological agents targeting eosinophilic inflammation are promising alternatives to conventional management.

To assess the early outcomes of two types of proton therapy: passive scattering proton therapy (PSPT) and pencil beam proton therapy (PBPT).The consecutive patients who had surgery in our facility were retrospectively reviewed.Thirty-two patients were identified (PBPT 22 patients [69%]). The mean (±standard deviation [SD]) tumor size was 3.8±1.8 cm, and the most common location was the upper clivus (41%). Four cases (13%) were revision surgeries referred from elsewhere, and two cases underwent additional surgery elsewhere to achieve near-total resection before radiation. The cerebrospinal fluid leak occurred in three patients (9%). The mean (±SD) prescribed dose of PSPT and PBPT was 74±3 Gy and 72±3 Gy, respectively (P=0.07). The mean (±SD) fractionation of PSPT and PBPT was 39±2 and 36±2, respectively (P=0.001). Radiation toxicities were recorded in endocrine (11 patients [34%]), ophthalmic (3 patients [9%]), otologic (7 patients [22%]), and radiation necrosis (4 patients [13%]). PSPT was associated with endocrinopathy (odds ratio [OR], 10.5; 95% confidence interval [CI], 1.86 to 59.4, P=0.008), and radiation dose was associated with otologic toxicity (OR 1.57; 95% CI, 1.02 to 2.44; P=0.04). The gross-near total resection group had better progression-free survival (PFS) than the subtotal resection group regardless of radiation therapy (P=0.01). Overall, 3-year PFS was 73%, and 5-year overall survival was 93%.The PBPT group showed comparable outcome to the PSPT group. The degree of resection was more important than the modality of proton therapy. Further follow-up and cases are necessary to evaluate the benefit of PBPT.

To provide a comprehensive state-of-the-art review of the emerging role of urine leukotriene E4 (uLTE4) as a biomarker in the diagnosis of chronic rhinosinusitis (CRS), aspirin-exacerbated respiratory disease (AERD), and asthma.Ovid MEDLINE(R), Ovid EMBASE, Ovid Cochrane Central Register of Controlled Trials, Ovid Cochrane Database of Systematic Reviews, and Scopus.A state-of-the-art review was performed investigating the role of uLTE4 as a diagnostic biomarker, predictor of disease severity, and potential marker of selected therapeutic efficacy.uLTE4 has been shown to be a reliable and clinically relevant biomarker for CRS, AERD, and asthma. uLTE4 is helpful in ongoing efforts to better endotype patients with CRS and to predict disease severity.Aside from being a diagnostic biomarker, uLTE4 is also able to differentiate aspirin-tolerant patients from patients with AERD and has been associated with objective disease severity in patients with CRS with nasal polyposis. uLTE4 levels have also been shown to predict response to medical therapy, particularly leukotriene-modifying agents.

Objectives Juvenile nasopharyngeal angiofibromas (JNAs) are uncommon tumors with an evolving treatment paradigm. The objective of this study was to compare our prior experience reported in 2005 with our most contemporary series to compare practice improvements and the impact of expanded endonasal procedures. Design Retrospective review comparing a contemporary 22 patients with JNA who underwent surgical management between 2005 and 2019, compared with a historical cohort of 65 patients from the same center. Results The most common presenting symptom was epistaxis (68%). The median maximum tumor diameter was 4.4 cm. All patients underwent preoperative embolization. An endoscopic endonasal approach (EEA) was used in 18 patients (82%), compared with 9% in the series prior to 2005. Gross total resection was achieved in all patients. The median estimated blood loss was 175 and 350 mL for EEA and open (transfacial) cases, respectively. Only two patients (9%) required a blood transfusion compared with 52% on the previous series. The median follow-up was 19 months. The overall recurrence rate was 9% in this series and 24% in the previous series. No patient required radiation therapy in follow-up compared with 3% in our historical cohort. Conclusion There have been significant changes regarding the management of patients with JNA compared with the previous Mayo Clinic experience. The EEA has become the preferred route over the transfacial approaches to treat JNA in selected patients who do not have intracranial extension. Preoperative embolization has aided in reducing the postoperative transfusion rates.

The anterior cranial fossa (ACF) is a complex anatomical region that can be affected by a broad spectrum of pathology. For the surgical treatment of these lesions, many approaches have been described, each of them with different scope and potential surgical complications, often associated with significant morbidity. Traditionally, tumors involving the ACF have been operated by transcranial approaches; however, in the last two decades, endoscopic endonasal approaches (EEAs) have been gaining popularity. In this work, the authors review and describe the anatomical aspects of the ACF and the technical nuances of transcranial and endoscopic approaches for tumors located in this region. Four approaches were performed in embalmed cadaveric specimens and the key steps were documented. Four illustrative cases of ACF tumors were selected to demonstrate the clinical application of anatomical and technical knowledge, which are essential in the preoperative decision-making process.

Trigeminal schwannomas (TSs) with solitary extracranial location are rare, and surgical excision is challenging. In recent years, the endoscopic endonasal transmaxillary transpterygoid approach (EETPA) has been advocated as an effective strategy for TSs in the infratemporal fossa (ITF).We describe the steps of the EETPA combined with the sublabial transmaxillary approach for the surgical excision of a giant mandibular schwannoma of the ITF. Indications, advantages, and approach-specific complications are also discussed. The main surgical steps are shown in an operative video.A combined EETPA and sublabial transmaxillary approach represents a safe and effective option for the surgical excision of extracranial TSs.

The objective of this study is to determine the association of proliferation indices and pathologic biomarkers on overall and recurrence/metastasis-free survival (OS and RMFS) in patients with sinonasal mucosal melanoma (SNMM) and to assess the genetic mutational landscape of SNMM.This is a retrospective cohort study of 45 SNMM patients without neoadjuvant therapy who underwent surgical therapy with curative intent and had tumor tissue available for histopathologic review, molecular analysis, and genetic mutational assessment. The OS and RMFS were assessed for associations with numerous tumor and patient-related factors.Among proliferative indices, higher Ki67 and mitotic rates were associated with worsened OS and RMFS (Ki67: p = 0.0007 and p 0.0001; mitotic rate: p = 0.005 and p = 0.0009, respectively). The presence of brisk tumor-infiltrating lymphocytes (TILs) was associated with improved RMFS (p = 0.007) and the presence of lymphovascular invasion was associated with worsened OS and RMFS (p = 0.02 and p = 0.04, respectively). Patients with amelanotic tumors were more likely to have higher T-stage (p = 0.046), less likely to have brisk TILs (p = 0.02) and had worsened RMFS (p = 0.03). Patients on immunotherapy with tumor Ki67 40% had better 3-year OS compared to those with higher Ki67 index (p = 0.004). Actionable genetic mutations such as BRAF V600E are rare and present in only 1 of 20 patients tested.In SNMM patients, pathologic and proliferation markers such as Ki67, mitotic rate and brisk TILs are associated with survival and may be considered in future staging systems. Clinical response to immunotherapy appears to correlate with the Ki67 index. Given the distinct genetic profile of SNMM, targeted therapies against the MAPK kinase pathway have limited utility.3 Laryngoscope, 132:2350-2358, 2022.

Oronasal fistula (ONF) is a common complication encountered after palatoplasty. Repair is indicated when symptoms impact speech and swallowing. In spite of the variety of surgical approaches described to repair these defects, recurrence rates remain high. Traditionally, successful closure is said to be achieved in using a double-layered approach due to the three-dimensional aspect of the defect. The extent of the fistula into the nasal cavity has incited an increased curiosity in using local endonasal flaps. In recent years, endonasal reconstructive procedures have seen increased interest and application, from cranial base defect repairs to orbital reconstruction and beyond. The nasoseptal (NSF) and inferior turbinate flaps (ITF) possess a robust arterial supply and an exceptional reach with excellent results demonstrated in large defect repair. However, the use of these flaps in ONF repair is scarcely discussed in the literature, and their effectiveness is relatively undetermined. In this manuscript, we present a series of three patients who underwent a triple layer ONF closure, with the oral portion incorporating a turn-in mucosal flap plus a local palate rotation flap or greater palatine artery pedicled-rotation flap, and a NSF or an ITF for the nasal portion of the defect.

Objective To present our center’s experience with a maintenance treatment algorithm for intravenous bevacizumab that allows for personalized therapy decisions. Patients and Methods We reviewed all patients treated with intravenous bevacizumab for hereditary hemorrhagic telangiectasia–related bleeding and/or high-output cardiac failure (HOCF) from January 1, 2013, to July 1, 2019, at the Mayo Clinic, Rochester, Minnesota. Data regarding subsequent bevacizumab dosing were abstracted. Results A total of 57 patients (n=40, 70.2% females) were identified with a median age of 65 (55 to 74; range, 37 to 89) years. High-cardiac output state was present in 21 patients (36.8%) and 10 (17.5%) were treated with intravenous bevacizumab primarily for HOCF. The median duration of follow-up after completion of the initial intravenous bevacizumab treatment was 25 (12.3 to 40.8; range, 0.1 to 65.4) months. A total of 20 (35.1%) patients with a median follow-up of 13.5 (range, 0 to 48.4) months required no maintenance dosing throughout the duration of follow-up. Among those who required subsequent maintenance doses, only a small fraction (8 patients; 14.0%) required regular maintenance doses every 4 to 8 weeks during follow-up whereas the majority of patients required intermittent “as-needed” doses at varying intervals. Conclusion There is significant inter-individual variability in the need for maintenance intravenous bevacizumab when patients are followed using a predefined bevacizumab maintenance dosing treatment algorithm. The use of “as-needed” maintenance bevacizumab appears to be an effective strategy for management of hereditary hemorrhagic telangiectasia–related bleeding and HOCF.

Acute invasive fungal sinusitis (AIFS) is a potentially life-threatening diagnosis in immunocompromised patients. Identifying patients who could benefit from evaluation and intervention can be challenging for referring providers and otolaryngologists alike. We aimed to develop and validate an accessible diagnostic tool to estimate the probability of AIFS.Retrospective chart review from 1999 to 2017 identified all patients evaluated for possible AIFS at a tertiary care center. AIFS was diagnosed by pathologic confirmation of fungal tissue angioinvasion. Stepwise selection and univariate logistic regression were used to screen risk factors for a multivariable predictive model. Model performance was assessed using Tukey's goodness-of-fit test and the area under the receiver operator characteristic curve (AUC). Model coefficients were internally validated using bootstrapping with 1000 iterations.A total of 283 patients (244 negative controls, 39 with AIFS) were included. Risk factors in our final diagnostic model included: fever ≥38°C (log-odds ratio [LOR] 1.72; 95% CI, 0.53 to 2.90), unilateral facial swelling, pain, or erythema (LOR 2.84; 95% CI, 1.46 to 4.23), involvement of the orbit or pterygopalatine fossa on imaging (LOR 3.02; 95% CI, 1.78 to 4.26), and mucosal necrosis seen on endoscopy (LOR 5.52; 95% CI, 3.81 to 7.24), with p0.01 for all factors. The model had adequate goodness of fit (p0.05) and discrimination (AUC = 0.96).We present an internally validated diagnostic tool to stratify the risk for AIFS. The estimated risk may help determine which patients can be observed with serial nasal endoscopy, which ones could be biopsied, and which ones would benefit from immediate surgical intervention.

To address concerns related to the safety profile of both Food and Drug Administration (FDA)-approved and non-FDA-approved intranasal corticosteroid (INCS) use in the pediatric population.Systematic review of MEDLINE, PubMed, and EMBASE databases using comprehensive search strategy, including all INCS formulations and adverse events. The study design was developed using Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Additional sources were identified from study references of relevant articles.A structured literature search was conducted. Extracted data included age, population size, study design, drug (dosage, route, and frequency), presence of hypothalamic-pituitary-adrenal (HPA) axis suppression, ocular symptoms, and growth velocity adverse events.A total of 33 studies met inclusion criteria. The studies included use of INCS as nasal sprays and drops. There were no persistent abnormalities noted in cortisol level or intraocular pressure change. Growth velocity reduction was reported in 3 of 10 randomized trials. Meta-analysis of epistaxis and headache showed no significant difference in the incidence of headache or epistaxis when FDA-approved INCSs were compared to placebo, with a relative risk of 1.12 (95% CI, 0.77-1.63;INCSs in FDA-approved routes of administration are generally safe to use in the pediatric population. Use of non-FDA-approved INCS drops may increase risk of iatrogenic Cushing's syndrome. Growth velocity reduction, HPA axis suppression, and visual changes due to INCS are uncommon.

To address concerns related to the safety profile of both Food and Drug Administration (FDA)-approved and non-FDA-approved intranasal corticosteroid (INCS) use in the adult population.Systematic review of MEDLINE, PubMed, and EMBASE databases using a comprehensive search strategy including all INCS formulations and adverse events. The study design was developed using Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Additional sources were identified from study references of relevant articles.A structured literature search was conducted. Each study was graded for level of evidence using the Oxford Centre for Evidence-Based Medicine. Extracted data included population size, study design, drug (dosage, route, and frequency), presence of hypothalamus pituitary axis suppression, ocular symptoms, and treatment-related adverse events.A total of 60 studies met inclusion criteria. The studies included use of INCS as metered nasal sprays, drops, injections, aerosols, and irrigations. There were no persistent abnormalities in cortisol level or intraocular pressure change. Meta-analysis of epistaxis showed a significantly increased risk in the FDA-approved treatment group in comparison with control (risk ratio 1.56; 95% confidence interval, 1.13-2.14;Overall, it appears that the use of both FDA and published non-FDA application of INCS are safe in the adult population. Meta-analysis demonstrated an increased risk of epistaxis in patients using INCS compared with placebo. Otherwise, there was no significant difference between in adults in the treatment group and placebo group. As an important caveat, the interpretation of safety of nonstandard INCS is restricted to delivery methods and dosages published in the literature.

Objectives The purpose of this article is to provide a state‐of‐the‐art review of the literature and summarize the latest publications on medical and surgical treatment of cystic fibrosis (CF) chronic rhinosinusitis (CRS), with an emphasis on describing recent advancements in cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies. Methods A comprehensive literature review was conducted utilizing the PubMed database with search phrases detailed within the body of the article. Abstracts were reviewed to include publications detailing medical, surgical, and CFTR modulating therapies for CF CRS. Findings from studies not previously reviewed and publications regarding CFTR modulators were emphasized. Results No clear guidelines for treatment of CF CRS are available. Nasal saline rinses, topical steroids, topical antibiotics, and topical dornase alfa are reported medical therapies. Ivacaftor has some sinonasal symptomatic benefit. A new triple therapy CFTR modulator, elexacaftor/tezacaftor/ivacaftor, is capable of treating 90% of patients with CF and has shown the greatest FEV1 improvement of any CFTR modulator yet. There is no clear consensus on surgical indications or technique, though aggressive surgery in recalcitrant disease has shown some symptomatic benefit. Endoscopic sinus surgery after lung transplantation may benefit some patients in whom the sinuses serve as a reservoir for recurrent pulmonary infections by decreasing rates of bronchiolitis obliterans syndrome and improving 5 year survival. Conclusion As lifespan increases for patients with CF, further investigation into medical therapy, CFTR modulator sinonasal outcomes, and surgical technique and outcomes for CF CRS is needed. Level of Evidence 5.

The aim of this review is to summarize characteristic imaging findings of common skull base lesions. In addition, this study aims to review the literature regarding important and interesting new advancements in imaging of the anterior and central skull base. Imaging techniques including magnetic resonance elastography, indocyanine green fluorescence, Gallium-DOTATATE PET/CT, peptide receptor radionucleotide therapy, and 3D printing are being used to advance the care of patients with skull base pathology. Characteristic CT and MRI findings can be used to narrow the differential diagnosis and plan treatment for anterior and central skull base lesions. New imaging techniques show promise in improving the care of patients with skull base lesions.

Objective The objective of this study is to describe the clinical presentation, tumor characteristics, natural history, and treatment patterns of sinonasal osteosarcoma. Methods Fourteen patients who had been treated for osteosarcoma of the nasal cavity and paranasal sinuses at a tertiary care center were reviewed. In addition, a systematic review of the literature for osteosarcoma of the sinonasal cavity was performed. Results In a systematic review, including 14 patients from the authors' institution, 53 total studies including 88 patients were assessed. Median follow-up was 18 months (interquartile range: 8–39 months). The most common presenting symptoms were facial mass or swelling (34%), and nasal obstruction (30%). The most common paranasal sinus involved by tumor was the maxillary sinus (64%), followed by the ethmoid sinuses (52%). The orbit (33%), dura (13%) and infratemporal fossa (10%) were the most common sites of local invasion. The majority of patients underwent surgery followed by adjuvant therapy (52.4%). Increasing age was associated with decreased overall survival rate (unit risk ratio [95% confidence interval (CI)] = 1.02 [1.003–1.043]; p = 0.0216) and T4 disease was associated with decreased disease-specific survival rate (hazard ratio [HR] = 2.87; p = 0.0495). The 2- and 5-year overall survival rates were 68 and 40%, respectively, while 2- and 5-year disease-specific survival rates were 71% and 44%, respectively. Conclusion Sinonasal osteosarcomas are uncommon tumors and can pose a significant therapeutic challenge. Increasing age and T4 disease are associated with worse prognosis. This disease usually warrants consultation by a multidisciplinary team and consideration of multimodality therapy.