An institutional experience in applying quality improvement measures to pituitary surgery: clinical and resource implications.

OBJECTIVE: The aim of this study was to report the authors' experience developing a Lean Six Sigma clinical care pathway (CCP) for endoscopic endonasal transsphenoidal operations. METHODS: Using Lean Six Sigma quality improvement principles-including the define, measure, analyze, improve, and control framework-the authors developed a CCP for endoscopic endonasal transsphenoidal operations, incorporating preoperative, intraoperative, and inpatient and outpatient postoperative phases of care. Efficacy and quality metrics were defined as postoperative length of stay (LOS), presentation to the emergency department (ED) or readmission within 30 days of discharge, and hospital charges. The study included all adult patients who underwent elective endoscopic endonasal resection for pituitary adenoma, Rathke's cleft cyst, craniopharyngioma, pituicytoma, or arachnoid cyst during the sampling period (April 1, 2018, to December 31, 2022). RESULTS: Two hundred twenty-eight patients met criteria and were included; 94 were treated before and 134 were treated after implementation of the CCP. Differences between groups in age, gender, race, BMI, American Society of Anesthesiologists classification, geographic distribution, preoperative serum sodium, tumor size, adenoma functional status, and prior surgery were not significant. The mean postoperative LOS significantly decreased from 4.5 to 1.7 days following CCP implementation (p < 0.0001); LOS variability also decreased, with the standard deviation declining from 3.1 to 1.5 days. The proportion of patients discharged on postoperative day (POD) 1 significantly increased from 0% to 61.9% (p < 0.0001). Fewer than one-quarter of the patients (23.4%) were discharged by POD 2 prior to the CCP, while 88.8% of were discharged by POD 2 after CCP implementation (p < 0.0001). Rates of 30-day ED presentations or readmissions were not significantly different (2.1% vs 6.0%, p = 0.20, and 7.5% vs 6.7%, p > 0.99, respectively). Mean per-patient hospital costs declined from $38,326 to $26,289 (p < 0.0001), with an associated change in cost variability from a standard deviation of $16,716 to $12,498. CONCLUSIONS: CCP implementation significantly improved LOS and costs of endoscopic endonasal resection, without adversely impacting postoperative ED presentations or readmissions.

Clinicopathological features and prognostic outcomes of molecularly defined entities in the new edition of the WHO classification of sinonasal carcinoma.

Introduction: We investigated the clinicopathological features and prognoses of the new molecularly defined entities in latest edition of the World Health Organization (WHO) classification of sinonasal carcinoma (SNC). Methods: Integrated data were combined into an individual patient data (IPD) meta-analysis. Results: We included 61 studies with 278 SNCs including 25 IDH2-mutant, 41 NUT carcinoma, 187 SWI/SNF loss, and 25 triple negative SNCs (without IDH2 mutation, NUTM1 rearrangement, and SWI/SNF inactivation) for analyses. Compared to other molecular groups, NUT carcinoma was associated with a younger age at presentation and an inferior disease-specific survival. Among SNCs with SWI/SNF inactivation, SMARCB1-deficient tumors presented later in life and were associated with a higher rate of radiotherapy administration. SMARCA4-deficiency was mostly found in teratocarcinosarcoma while SMARCB1-deficient tumors were associated with undifferentiated carcinoma and non-keratinizing squamous cell carcinoma. Conclusion: Our study facilitates our current understanding of this developing molecular-defined spectrum of tumors and their prognoses.

Orbital resection by intranasal technique (ORBIT): A new classification system for reporting endoscopically resectable primary benign orbital tumors.

BACKGROUND: The Cavernous Hemangioma Exclusively Endonasal Resection (CHEER) staging system has become the gold standard for outcomes reporting in endoscopic orbital surgery for orbital cavernous hemangiomas (OCHs). A recent systematic review demonstrated similar outcomes between OCHs and other primary benign orbital tumors (PBOTs). Therefore, we hypothesized that a simplified and more comprehensive classification system could be developed to predict surgical outcomes of other PBOTs. METHODS: Patient and tumor characteristics as well as surgical outcomes from 11 international centers were recorded. All tumors were retrospectively assigned an Orbital Resection by Intranasal Technique (ORBIT) class and stratified based on surgical approach as either exclusively endoscopic or combined (endoscopic and open). Outcomes based on approach were compared using chi-squared or Fisher's exact tests. The Cochrane-Armitage test for trend was used to analyze outcomes by class. RESULTS: Findings from 110 PBOTs from 110 patients (age 49.0 ± 15.0 years, 51.9% female) were included in the analysis. Higher ORBIT class was associated with a lower likelihood of gross total resection (GTR). GTR was more likely to be achieved when an exclusively endoscopic approach was utilized (p < 0.05). Tumors resected using a combined approach tended to be larger, to present with diplopia, and to have an immediate postoperative cranial nerve palsy (p < 0.05). CONCLUSION: Endoscopic treatment of PBOTs is an effective approach, with favorable short-term and long-term postoperative outcomes as well as low rate of adverse events. The ORBIT classification system is an anatomic-based framework that effectively facilitates high-quality outcomes reporting for all PBOTs.

Prolactinoma extension as a contributing factor in dopamine agonist-induced CSF rhinorrhea: a systematic review of the literature.

BACKGROUND: Dopamine agonist-induced cerebrospinal fluid (CSF) rhinorrhea is an uncommon treatment-related complication arising in 6.1% of prolactinoma patients treated with dopamine agonists. Locally invasive prolactinomas may create CSF fistulae through formation of dural and osseous skull base defects. Tumor shrinkage secondary to dopamine agonist therapy unmasks skull base defects, thus inducing CSF rhinorrhea. In these cases, repair of the leak may be achieved through collaborative surgical intervention by rhinologists and neurosurgeons. Multiple variables have been investigated as potential contributors to the risk of CSF rhinorrhea development in medically treated prolactinoma patients, with little consensus. OBJECTIVE: The primary aim of our study was the characterization of risk factors for CSF rhinorrhea development following dopamine agonist treatment. METHODS: A systematic review of the literature was conducted to identify cases of CSF rhinorrhea following dopamine agonist treatment of prolactinoma. The clinical history, radiographic findings and treatment outcomes are discussed. RESULTS: Fifty-four patients with dopamine agonist-induced CSF rhinorrhea were identified across 23 articles published from 1979 to 2019. Description of diagnostic imaging [computed tomography (CT)/magnetic resonance imaging (MRI)] was not provided for 18/54 subjects. For the 36 cases that described prolactinoma appearance on CT or MRI, invasion of the cavernous sinuses was reported in 13 (36.1%) and invasion of the sphenoid sinus was reported in 18 (50%). CONCLUSION: Based on our systematic review, we propose that CT findings of osseous erosion of the sella or the anterior skull base may predict dopamine agonist-induced CSF rhinorrhea. We recommend obtaining a thin-slice CT of the sinuses in cases with MRI evidence of sphenoid involvement.

High-Grade Biphenotypic Sinonasal Sarcoma: A Case Report.

Introduction Biphenotypic sinonasal sarcoma (BSNS) is a recently found entity that first described by Lewis et al. It was then added to the 4th edition of the World Health Organization (WHO) of head and neck tumors in 2012. BSNS has been described as a rare low-grade sarcoma arising in the upper sinonasal tract. It is believed that in the past, BSNS was, likely, previously diagnosed as other low-grade or benign malignancies. Fibrosarcoma, leiomyosarcoma, and peripheral nerve sheath tumors, all fall within the differential diagnosis of BSNS. However, BSNS is unlike other mesenchymal sinonasal tumors, as it displays both neural and myogenic differentiation. BSNS has thus far been recognized in only a hand full of case reports, all of which have reported similar morphologic features of a low-grade soft tissue tumor with neural involvement arising from the nasal cavity or ethmoid air cells in middle aged individuals. In fact, being low-grade sarcoma became such a hallmark characteristic of this tumor that it even received the name low-grade sinonasal sarcoma with neural and myogenic features or LGSSNMF. Case Presentation We present, however, for the first time, a high-grade differentiation of BSNS in an otherwise healthy 72-year-old female. The patient was referred from an outside ENT (ear, nose, and throat) after pathology from a presumed polypectomy returned positive for a BSNS. Initial imaging revealed erosion through the bilateral lamina papyracea, anterior cranial fossa floor, and posterior table of the frontal sinus. She then underwent a combined endoscopic and bicoronal open approach for resection of the skull base lesion that was found to encompass the entirety of the sinonasal cavities bilaterally. Postoperatively, the patient underwent significant complications including infection of the pericranial flap, pneumocephalus, and eventually death. Discussion As BSNS is a fairly new entity, currently there has only been four case series conducted, each identifying features of a low-grade sarcoma with both myogenic and neural differentiation. Histologically, BSNS has monophasic spindle cells with uniform, elongated nuclei with scant cytoplasm between benign proliferations of surface-type respiratory epithelium, with a low mitotic rate. Our case, however, revealed pleomorphic hyperchromatic cells with high mitotic activity and necrosis with invasion of bone, staging it as high grade. Immunohistochemistry also differed from the previously reported standards. This case describes a new category for BSNS which may change the differential diagnosis, management, and surgical recommendations that are currently utilized for this skull base neoplasm.

Primary Repair of Posteriorly Located Anterior Skull Base Dural Defects Using Nonpenetrating Titanium Clips in Cranial Trauma.

Objective Primary repair of posteriorly located anterior skull base (ASB) dural defects following cranial trauma is made difficult by narrow operative corridors and adherent dura mater. Inadequate closure may result in continued cerebrospinal fluid (CSF) leak and infectious sequelae. Here, we report surgical outcomes following the use of nonpenetrating titanium microclips as an adjunctive repair technique in traumatic anterior skull base dural defects extending from the olfactory groove to the tuberculum sellae. Methods All trauma patients who underwent a bifrontal craniotomy from January 2013 to October 2019 were retrospectively reviewed. Patients with ASB defects located at posterior to the olfactory groove were analyzed. Patients with isolated frontal sinus fractures were excluded. All patients presented with CSF leak or radiographic signs of dural compromise. Patients were divided according to posterior extent of injury. Patient characteristics, imaging, surgical technique, and outcomes are reported. Results A total of 19 patients who underwent a bifrontal craniotomy for repair of posteriorly located ASB dural defects using nonpenetrating titanium microclips were included. Defects were divided by location: olfactory groove (10/19), planum sphenoidale (6/19), and tuberculum sellae (3/19). No patients demonstrated a postoperative CSF leak. No complications related to the microclip technique was observed. Clip artifact did not compromise postoperative imaging interpretation. Conclusion Primary repair of posteriorly located ASB dural defects is challenging due to narrow working angles and thin dura mater. Use of nonpenetrating titanium microclips for primary repair of posteriorly located dural defects is a reasonable adjunctive repair technique and was associated with no postoperative CSF leaks in this cohort.

Absence of Survival Improvement for Patients with Esthesioneuroblastoma Over the Past 2 Decades: A Population-Based Study.

OBJECTIVE: Esthesioneuroblastoma (ENB) is a rare malignancy of the sinonasal tract and its infrequency has confounded efforts at clearly describing the survival trends associated with this neoplasm over the years. In this study, we reviewed survival trends in ENB and investigated the impact of treatment extent and modality on patient outcomes. METHODS: We accessed the Surveillance, Epidemiology, and End Result (SEER) program to identify ENB cases from 1998 to 2016. A χ2 test was used to compare the categorical covariates and a t test or Mann-Whitney U test was utilized for continuous variables. The impact of prognostic factors on survival was computed using a Kaplan-Meier analysis and multivariate Cox proportional hazards model. We divided ENB patients into 4 periods including 1998-2002, 2003-2007, 2008-2012, and 2013-2016, and investigated survival trends using the Kaplan-Meier curve and log-rank test. RESULTS: ENB patients who underwent biopsy alone were associated with older age, larger tumor diameter, increased rates of tumor extension, nodal/distant metastases, and advanced stages as compared with patients undergoing tumor resection. Our results also demonstrated that surgical resection and adjuvant radiotherapy could confer survival advantages, whereas chemotherapy was associated with reduced survival in patients with ENB. Over the past 2 decades, surprisingly, there has been no change in survival rates for patient with ENB (P = 0.793). CONCLUSIONS: Despite advanced diagnostic studies and modernized treatment approaches, ENB survival has remained unchanged over the years, calling for improved efforts to develop appropriate individualized interventions for this rare tumor entity. Our results also confirmed that surgery and adjuvant radiotherapy is associated with improved patient survival whereas the use of chemotherapy should be considered carefully.

Sinonasal immunoglobin G4-related disease: case report and review.

Immunoglobin G4-related disease (IgG4-RD) is a chronic fibro-inflammatory condition that presents as a single or multiple tumefactive lesions affecting virtually any organ system. Here we report a case of recurrent sinonasal IgG4-RD and review the literature of this evolving entity.

A combined approach for stabilization and endoscopic/ endonasal odontoid and clivus resection for treatment of basilar invagination.

BACKGROUND: Basilar invagination (BI) is a complex condition characterized by prolapse of the odontoid into the brain stem/upper cervical cord. This lesion is often associated with Chiari malformations, and rheumatoid arthritis (RA). Treatment options for BI typically include cervical traction, an isolated anterior transoral odontoidectomy, anterior endonasal odontoidectomy, an isolated posterior fusion, or combined anterior/ posterior surgical approach. CASE DESCRIPTION: A 45-year-old female with a Chiari I malformation and RA underwent a combined posterior C0-C5 posterior decompression/fusion, followed by an anterior odontoidectomy (i.e. endoscopic/endonasal under neuronavigation). Postoperatively, the patient's symptoms and neurological signs resolved. CONCLUSION: BI in was successfully managed with a combined posterior C0-C5 decompression/fusion followed by an anterior endoscopic/endonasal odontoidectomy performed under neuronavigation.

Periostin and Inflammatory Disease: Implications for Chronic Rhinosinusitis.

OBJECTIVE: To provide a comprehensive overview of the emerging role of periostin, an extracellular matrix protein, as a key component in the development, diagnosis, and treatment of patients with chronic rhinosinusitis. DATA SOURCES: Medline database. REVIEW METHODS: A state of the art review was performed targeting English-language studies investigating the role of periostin in cardiopulmonary, neoplastic, and inflammatory diseases, with emphasis on recent advances in the study of periostin in chronic rhinosinusitis. CONCLUSIONS: Periostin has emerged as a novel biomarker and therapeutic target for numerous human pathologies, including cardiac, pulmonary, and neoplastic disease. The upregulation of periostin in chronic rhinosinusitis suggests the potential for similar roles among patients with sinonasal disease. IMPLICATIONS FOR PRACTICE: Chronic rhinosinusitis is a widespread disease with major clinical and societal impact. A critical limitation in the current treatment of patients with chronic rhinosinusitis is the absence of clinically relevant biomarkers to guide diagnosis and treatment selection. A review of the literature supports a likely role of periostin as a biomarker of chronic rhinosinusitis, as well as a novel therapeutic target in the future treatment of patients with sinonasal disease.

Development of the international orbital Cavernous Hemangioma Exclusively Endonasal Resection (CHEER) staging system.

BACKGROUND: Orbital cavernous hemangiomas (OCH) are the most common adult orbital tumor and represent an ideal index lesion for endonasal orbital tumor surgery. In order to standardize outcomes reporting, an anatomic-based staging system was developed. METHODS: An international, multidisciplinary panel of 23 experts in orbital tumor surgery was formed. A modified Delphi method was used to develop the cavernous hemangioma exclusively endonasal resection (CHEER) staging system with a total of 2 rounds being completed. RESULTS: Tumors medial to a plane along the long axis of the optic nerve may be considered amenable for an exclusively endonasal resection. In select cases, tumors may extend inferolaterally if the tumor remains below a plane from the contralateral naris through the long axis of the optic nerve (ie, plane of resectability [POR]). This definition reached consensus with 91.3% of panelists in agreement. Five stages were designed based on increasing technical resection difficulty and potential for morbidity. Stages were based on the relationship of the tumor to the extraocular muscles, the inferomedial muscular trunk of the ophthalmic artery (IMT), and orbital foramina. Staging by anatomic location also reached consensus with 87.0% of panelists in agreement. Size was not included in the staging system due to the lack of agreement on the contribution of size to resection difficulty. CONCLUSION: Endoscopic orbital tumor surgery is a nascent field with a growing, yet heterogeneous, body of literature. The CHEER staging system is designed to facilitate international, high-quality, standardized studies establishing the safety, efficacy, and outcomes of endonasal resection of OCH.

Impact of Age on Sinus Surgery Outcomes.

OBJECTIVES/HYPOTHESIS: To evaluate the impact of age on patient-reported quality of life (QOL) following endoscopic sinus surgery (ESS) for chronic rhinosinusitis (CRS). STUDY DESIGN: Prospective cohort study. METHODS: Six hundred thirty-six patients with CRS were recruited from 11 otolaryngologic practices and completed the sinonasal-specific, 22-item Sino-Nasal Outcome Test-22 (SNOT-22) and general health-related EuroQol 5-Dimension (EQ-5D) questionnaires at baseline and 12 and 24 months after ESS. Patients were grouped chronologically to determine whether age at time of ESS was associated with clinical outcomes. RESULTS: Ages ranged from 18 to 80 years (mean ± standard deviation = 48.5 ± 14.4). Improvement was observed in postoperative SNOT-22 scores at 12 and 24 months for all decades of life. Similar improvements were observed for EQ-5D-based health utility value (HUV) scores in all decades of life, except for the eldest cohort (ages 70-80, N = 33), who did not exceed the minimal clinically important difference at either 12 or 24 months following ESS. In regression analysis, age was not associated with sinonasal-specific outcomes (change in SNOT-22 scores) at 12 (P = .507) or 24 months (P = .955). In general health-related outcomes, however, age was significantly associated with change in EQ-5D-based HUV scores from baseline to 12 months following ESS after adjusting for patient demographics, comorbidities, and surgical history (P = .049). CONCLUSIONS: This study demonstrates that ESS for adult CRS sufferers offers improved QOL outcomes through the eighth decade of life. The impact of comorbidities on QOL needs to be carefully considered when assessing older patients for sinus surgery. LEVEL OF EVIDENCE: 2b Laryngoscope, 128:2681-2687, 2018.

Technical Refinements and Outcomes of the Modified Anterior Septal Transplant.

IMPORTANCE: Severe anterior septal deviation and resultant nasal obstruction represent a difficult surgical task to correct. The goal of surgery is to straighten the anterior dorsal and caudal struts, while maintaining nasal tip and midvault support. This study presents a novel extracorporeal septoplasty technique to straighten the crooked anterior septum. OBJECTIVE: To describe the novel anterior septal transplant technique, which consists of complete resection of the caudal septum and reconstruction with extended spreader grafts and a columellar strut, without a separate caudal septal replacement graft. DESIGN, SETTING, AND PARTICIPANTS: This study was a retrospective case series at a tertiary academic referral center. Participants were sequential adult patients undergoing anterior septal transplant from January 1, 2008, to December 31, 2015. MAIN OUTCOMES AND MEASURES: Patient-reported nasal obstruction using Nasal Obstruction Symptom Evaluation (NOSE) scores and objective photographic analysis. Nasal tip deviation, projection, and rotation were measured. Preoperative and postoperative outcomes were compared. Complications are reported. RESULTS: Seventy-one patients (mean age, 46 years [age range, 16-72 years]; 48 [67.6%] female and 23 [32.4%] male) were included in the case series. Postoperative NOSE scores (mean [SD], 24.00 [24.58]) were significantly better than preoperative NOSE scores (mean [SD], 72.25 [14.55]) (P < .001). A separate cohort of 32 patients (mean age, 42 years [age range, 13-72 years]; 23 [71.9%] female and 9 [28.1%] male) had photographs available for analysis. In the frontal view, nasal deviation improved from a mean (SD) of 2.9 (2.0) degrees before surgery to a mean (SD) of 1.4 (1.7) degrees after surgery (P = .004). In the base view, the deviation was corrected from a mean (SD) of 4.9 (2.8) degrees to a mean (SD) of 1.7 (1.2) degrees (P < .001). Tip rotation and projection were unchanged after surgery. Four patients had mild dorsal irregularities after surgery. CONCLUSIONS AND RELEVANCE: Anterior septal transplant by the described technique is a safe and effective treatment option for severe anterior septal deviation. LEVEL OF EVIDENCE: 4.

Practice patterns in the management of post-tonsillectomy hemorrhage: An American Society of Pediatric Otolaryngology survey.

OBJECTIVES: To evaluate practice patterns amongst pediatric otolaryngologists in the management of post-tonsillectomy hemorrhage. METHODS: A cross-sectional survey of the American Society of Pediatric Otolaryngology membership was administered electronically. The survey contained questions related to practice type, availability of resident and fellow call coverage, and management of different scenarios of post-tonsillectomy hemorrhage. Anonymous responses were collected and tabulated. RESULTS: The response rate was 157/443 (35%). For patients presenting with a convincing history of post-tonsillectomy hemorrhage but no clot or bleeding on exam, the most common management was overnight observation (55%) or discharge home with close follow-up (29%). In patients presenting with tonsillar clot but no active bleeding, the most common management was operating room for control (50%), followed by observation (25%) and bedside topical treatment (13%). In the same scenario with a cooperative teenager, bedside topical treatment was most common (45%), followed by operating room for control (27%) and observation (16%). In patients presenting with active tonsillar bleeding, operating room for control was most common (83%) while few (6%) attempted bedside treatment. If the patient was a cooperative teenager, 38% attempted bedside treatment while 52% would still go to the operating room. CONCLUSIONS: There is substantial variation in the management of post-tonsillectomy hemorrhage amongst the pediatric otolaryngologists. Further studies to determine outcomes associated with differing treatment strategies would be useful in establishing practice recommendations.

Cell Adhesion Molecule CD166/ALCAM Functions Within the Crypt to Orchestrate Murine Intestinal Stem Cell Homeostasis.

BACKGROUND & AIMS: Intestinal epithelial homeostasis is maintained by active-cycling and slow-cycling stem cells confined within an instructive crypt-based niche. Exquisite regulating of these stem cell populations along the proliferation-to-differentiation axis maintains a homeostatic balance to prevent hyperproliferation and cancer. Although recent studies focus on how secreted ligands from mesenchymal and epithelial populations regulate intestinal stem cells (ISCs), it remains unclear what role cell adhesion plays in shaping the regulatory niche. Previously we have shown that the cell adhesion molecule and cancer stem cell marker, CD166/ALCAM (activated leukocyte cell adhesion molecule), is highly expressed by both active-cycling Lgr5+ ISCs and adjacent Paneth cells within the crypt base, supporting the hypothesis that CD166 functions to mediate ISC maintenance and signal coordination. METHODS: Here we tested this hypothesis by analyzing a CD166-/- mouse combined with immunohistochemical, flow cytometry, gene expression, and enteroid culture. RESULTS: We found that animals lacking CD166 expression harbored fewer active-cycling Lgr5+ ISCs. Homeostasis was maintained by expansion of the transit-amplifying compartment and not by slow-cycling Bmi1+ ISC stimulation. Loss of active-cycling ISCs was coupled with deregulated Paneth cell homeostasis, manifested as increased numbers of immature Paneth progenitors due to decreased terminal differentiation, linked to defective Wnt signaling. CD166-/- Paneth cells expressed reduced Wnt3 ligand expression and depleted nuclear ß-catenin. CONCLUSIONS: These data support a function for CD166 as an important cell adhesion molecule that shapes the signaling microenvironment by mediating ISC-niche cell interactions. Furthermore, loss of CD166 expression results in decreased ISC and Paneth cell homeostasis and an altered Wnt microenvironment.

Quantitative Multiplex Immunohistochemistry Reveals Myeloid-Inflamed Tumor-Immune Complexity Associated with Poor Prognosis.

Here, we describe a multiplexed immunohistochemical platform with computational image processing workflows, including image cytometry, enabling simultaneous evaluation of 12 biomarkers in one formalin-fixed paraffin-embedded tissue section. To validate this platform, we used tissue microarrays containing 38 archival head and neck squamous cell carcinomas and revealed differential immune profiles based on lymphoid and myeloid cell densities, correlating with human papilloma virus status and prognosis. Based on these results, we investigated 24 pancreatic ductal adenocarcinomas from patients who received neoadjuvant GVAX vaccination and revealed that response to therapy correlated with degree of mono-myelocytic cell density and percentages of CD8+ T cells expressing T cell exhaustion markers. These data highlight the utility of in situ immune monitoring for patient stratification and provide digital image processing pipelines to the community for examining immune complexity in precious tissue sections, where phenotype and tissue architecture are preserved to improve biomarker discovery and assessment.

Lymphoma of the nasal cavity and paranasal sinuses: A case series.

BACKGROUND: Lymphomas of the sinonasal tract are a rare and heterogeneous subset of solid sinonasal neoplasms. OBJECTIVE: To characterize, in this case series, presenting symptoms, treatment modalities, and outcomes for patients with sinonasal lymphoma within a single institution. METHODS: Retrospective patient data were collected from an academic, oncologic center and entered into a repository designed to capture outcomes for sinonasal malignancies. Patient demographics, presenting symptoms, imaging findings, treatment modalities, and health status were retrospectively extrapolated and evaluated by using Kaplan-Meier estimations for survival probability. RESULTS: Patients with sinonasal lymphoma with a mean follow-up of 50 months were identified (n = 18). Histologic diagnosis included the following: diffuse large B-cell lymphoma (n = 9), natural killer/T-cell lymphoma (n = 5), follicular lymphoma (n = 1), T-cell lymphoma (n = 1), and lymphoma-not otherwise specified (n = 2). The most frequent presenting symptoms were nasal obstruction (78%), facial pain (72%), facial swelling (50%), and nasal discharge (44%). Treatment before lymphoma diagnosis included antibiotics (83%), oral steroids (22%), decongestants (22%), and topical steroids (11%). Treatment regimens after diagnosis included both chemotherapy (94%) and chemoradiotherapy (56%). Survival rates by lymphoma subtype were 56% for B-cell lymphoma and 40% for natural killer/T-cell lymphoma. Overall, 2- and 5-year survival rates were 67% and 50%, respectively. The combination of chemotherapy and radiation resulted in significantly higher survival rates (p ≤ 0.001) than chemotherapy alone. CONCLUSION: Sinonasal lymphomas are characterized by meager survival rates, which differ by histopathologic subtype. The diagnosis of sinonasal lymphoma is challenging because symptoms frequently parallel those of chronic rhinosinusitis. Increased awareness of these rare malignancies may improve detection and more timely treatment. Clinical trial registration NCT01332136.

Sensitivity analysis and diagnostic accuracy of the Brief Smell Identification Test in patients with chronic rhinosinusitis.

BACKGROUND: The Brief Smell Identification Test (BSIT) is an abbreviated version of the Smell Identification Test (SIT) used to assess olfactory function. Although the BSIT can be efficiently administered in under 5 minutes, the accuracy of the BSIT in relation to the SIT in patients with chronic rhinosinusitis (CRS) is unknown. METHODS: Patients with CRS were recruited as part of an ongoing multi-institutional observational cohort study. A total of 183 participants provided both BSIT and SIT olfactory function scores during initial enrollment. Linear associations between BSIT and SIT scores were evaluated using Pearson's correlation coefficients (rp ). The sensitivity, specificity, and accuracy of BSIT scores were determined using SIT scores as the "gold standard." RESULTS: A strong bivariate linear association was found between BSIT and SIT scores (rp = 0.893; p < 0.001) for all participants. A significantly lower proportion of patients were identified as having abnormal olfaction using the BSIT compared to the SIT (47% vs 68%, respectively; p < 0.001). Using the currently defined score of ≤8 as a cut-point for abnormal olfactory function, the BSIT demonstrated a sensitivity of 63% and specificity of 88% with an overall accuracy of 71%. Increasing the cut-point to ≤9 resulted in an increased sensitivity of 86%, a specificity of 76%, and an improved overall accuracy of 83%. CONCLUSION: In patients with CRS, BSIT scores strongly correlate with SIT scores; however, the BSIT underestimates olfactory dysfunction as defined by the suggested cut-point of ≤8. Increasing the cut-point to ≤9 increased the sensitivity and accuracy of the BSIT.

Improvements in sleep-related symptoms after endoscopic sinus surgery in patients with chronic rhinosinusitis.

BACKGROUND: Sleep impairment is highly prevalent in patients with chronic rhinosinusitis (CRS). Although endoscopic sinus surgery (ESS) has been shown to improve overall patient-reported sleep quality, the postoperative impact on individual sleep symptoms remains unclear. METHODS: Patients with medically-recalcitrant CRS who elected to undergo ESS were prospectively enrolled into a multi-institutional, observational cohort study. Sleep-related symptom severity and treatment outcomes were assessed using the sleep domain questions within the 22-item Sino-Nasal Outcome Test (SNOT-22). RESULTS: A total of 334 participants met criteria and were followed postoperatively for an average of 14.5 ± 4.9 months (mean ± standard deviation [SD]). Mean SNOT-22 sleep domain scores improved from 13.7 ± 6.8 to 7.7 ± 6.6 (p < 0.001). Significant mean relative improvements were reported for "difficulty falling asleep" (45%; p < 0.001), "waking up at night" (40%; p < 0.001), "lack of a good night's sleep" (43%; p < 0.001), "waking up tired" (40%; p < 0.001), and "fatigue" (42%; p < 0.001) scores. A total of 66% of study participants reported postoperative improvement in "lack of a good night's sleep," "waking up tired," and "fatigue"; 62% reported improvement in "waking up at night"; and 58% reported improvement in "difficulty falling asleep." CONCLUSION: Patients with CRS report significant and sustained improvements following ESS in common sleep-related symptoms as assessed by the SNOT-22 sleep domain. Despite these significant improvements, some degree of persistent postoperative sleep impairment was reported. Further study is necessary to determine what factors are associated with continued sleep dysfunction after sinus surgery.

Propranolol-responsive cranial nerve palsies in a patient with PHACES syndrome.

PHACES syndrome is a neurocutaneous disorder characterized by the presence of segmental hemangiomas with associated anomalies of the posterior fossa, cerebral vasculature, cardiovascular system, eyes, and ventral or midline structures. We present the first case of propranolol-responsive congenital trigeminal and facial nerve palsies secondary to an intracranial hemangioma in a patient with PHACES syndrome.