The role of frailty in the clinical management of neurofibromatosis type 1: a mixed-effects modeling study using the Nationwide Readmissions Database.

OBJECTIVE: Frailty embodies a state of increased medical vulnerability that is most often secondary to age-associated decline. Recent literature has highlighted the role of frailty and its association with significantly higher rates of morbidity and mortality in patients with CNS neoplasms. There is a paucity of research regarding the effects of frailty as it relates to neurocutaneous disorders, namely, neurofibromatosis type 1 (NF1). In this study, the authors evaluated the role of frailty in patients with NF1 and compared its predictive usefulness against the Elixhauser Comorbidity Index (ECI). METHODS: Publicly available 2016-2017 data from the Nationwide Readmissions Database was used to identify patients with a diagnosis of NF1 who underwent neurosurgical resection of an intracranial tumor. Patient frailty was queried using the Johns Hopkins Adjusted Clinical Groups frailty-defining indicator. ECI scores were collected in patients for quantitative measurement of comorbidities. Propensity score matching was performed for age, sex, ECI, insurance type, and median income by zip code, which yielded 60 frail and 60 nonfrail patients. Receiver operating characteristic (ROC) curves were created for complications, including mortality, nonroutine discharge, financial costs, length of stay (LOS), and readmissions while using comorbidity indices as predictor values. The area under the curve (AUC) of each ROC served as a proxy for model performance. RESULTS: After propensity matching of the groups, frail patients had an increased mean ± SD hospital cost ($85,441.67 ± $59,201.09) compared with nonfrail patients ($49,321.77 ± $50,705.80) (p = 0.010). Similar trends were also found in LOS between frail (23.1 ± 14.2 days) and nonfrail (10.7 ± 10.5 days) patients (p = 0.0020). For each complication of interest, ROC curves revealed that frailty scores, ECI scores, and a combination of frailty+ECI were similarly accurate predictors of variables (p > 0.05). Frailty+ECI (AUC 0.929) outperformed using only ECI for the variable of increased LOS (AUC 0.833) (p = 0.013). When considering 1-year readmission, frailty (AUC 0.642) was outperformed by both models using ECI (AUC 0.725, p = 0.039) and frailty+ECI (AUC 0.734, p = 0.038). CONCLUSIONS: These findings suggest that frailty and ECI are useful in predicting key complications, including mortality, nonroutine discharge, readmission, LOS, and higher costs in NF1 patients undergoing intracranial tumor resection. Consideration of a patient's frailty status is pertinent to guide appropriate inpatient management as well as resource allocation and discharge planning.

Patient satisfaction survey experience among American otolaryngologists.

BACKGROUND: Patient Satisfaction (PS) is a commonly used metric in health care settings to assess the quality of care given by physicians. Monitoring physicians in this way may impact physician quality of life. Studies evaluating this impact are not available. This study sought to examine the physician experience of measuring PS among practicing otolaryngologists. METHODS: Using an online survey platform, a 34-item survey was given to practicing otolaryngologists through email distribution. The survey included questions about physician, practice and patient demographics, as well as inquiries regarding the way in which PS was measured and how it affected physician work and personal life. Data from these questions were reviewed and analyzed. RESULTS: 174 otolaryngologists responded to the survey. A majority of physicians' (55.3%) PS scores had been tracked with 89.9% reporting being tracked for a length of at least 1 year. PS scores for individual physicians were noted to be inconsistent and vary significantly between reports. Measuring patient satisfaction led to increased occupational stress, yet most physicians (63.8%) felt the monitoring did not lead to improvements in their practice. Some physicians (36.2%) reported that the collection of patient satisfaction scores had negatively influenced the way they practiced medicine, including the pressure to order superfluous tests or to prescribe unnecessary medications. CONCLUSION: Overall, physicians are negatively affected by the tracking of patient satisfaction scores. Occupational stress caused by the collection of patient satisfaction scores may contribute to physician burnout.

Practice Patterns Among Fellowship-Trained Rhinologists: A Survey of Past American Rhinologic Society Fellows.

BACKGROUND: The goals of this study were to understand the trends in recent and past rhinology fellows concerning their demographics, goals, and experiences. METHODS: An anonymous web-based survey was sent to graduates of the 35 rhinology fellowship programs. The survey included questions regarding the scope of practice, research contribution, societal involvement, fellow satisfaction with training, and current practice patterns. Chi-square testing and logistic regression were used to compare variables across cohorts: 0-5 years versus 6+ years from fellowship graduation, gender, and practice settings. RESULTS: Based on 171 respondents, we found no significant differences in 0-5 versus 6+ year graduates in their desire for an academic job post-fellowship. However, those who graduated 6+ years ago had significantly more success securing one (74% vs 96%, P = 0.004). Between males and females, there were no differences in goal of obtaining an academic job or success in obtaining an academic career. Females were more likely to report they attend academic society meetings regularly. Female rhinologists also reported less satisfaction with overall work-life balance and decreased satisfaction with clinical practice. Physicians in academic settings have poorer work-life balance. CONCLUSIONS: Our findings suggest that finding an academic job may be more difficult for recent rhinology fellowship graduates, although still very possible for the majority of graduates. Understanding the reason for these changes may provide insight to current rhinology fellowship directors and trainees interested in pursuing fellowship training.

Unique Signatures of Highly Constrained Genes Across Publicly Available Genomic Databases.

Publicly available genomic databases and genetic constraint scores are crucial in understanding human population variation and the identification of variants that are likely to have a deleterious impact causing human disease. We utilized the one of largest publicly available databases, gnomAD, to determine genes that are highly constrained for only LoF, only missense, and both LoF/missense variants, identified their unique signatures, and explored their causal relationship with human conditions. Those genes were evaluated for unique patterns including their chromosomal location, tissue level expression, gene ontology analysis, and gene family categorization using multiple publicly available databases. Those highly constrained genes associated with human disease, we identified unique patterns of inheritance, protein size, and enrichment in distinct molecular pathways. In addition, we identified a cohort of highly constrained genes that are currently not known to cause human disease, that we suggest will be candidates to pursue as novel disease-associated genes. In summary, these insights not only elucidate biological pathways of highly constrained genes that expand our understanding of critical cellular proteins but also advance research in rare diseases.

Gender-Specific Differences in Preoperative Concerns in Patients Undergoing Endoscopic Sinus Surgery for Chronic Rhinosinusitis.

OBJECTIVES: Among patients with chronic rhinosinusitis (CRS), gender differences in epidemiology as well as quality of life have been reported. However, whether gender differences in endoscopic sinus surgery (ESS) preoperative concerns exist is unclear. METHODS: CRS patients undergoing ESS at 3 tertiary care centers in Los Angeles completed the validated Western Surgical Concern Inventory - ESS assessing ESS preoperative concerns. RESULTS: Of the 75 patients included, female patients expressed greater concern than male patients in regard to nasal packing, undergoing anesthesia, impact of surgery on daily activities, and pain and discomfort following surgery. CONCLUSION: This study suggests there are gender differences in ESS preoperative concerns and otolaryngologists should be aware of these possible concerns during preoperative discussions.

An Endoscopic Endonasal Attempt at Pediatric Meningoencephalocele Repair.

Meningoencephalocele is an abnormal skull base protrusion of fluid, brain tissue, and meninges that can lead to nasal obstruction, meningitis, and Cerebrospinal Fluid (CSF) rhinorrhea. This condition can be managed operatively through an open craniotomy or a less invasive endoscopic approach. Here, we report a case of an 18-month-old female who presented with a meningoencephalocele that was part of the Sakoda complex, a rare neurosurgical phenomenon consisting of meningoencephalocele, agenesis of the corpus callosum, and cleft lip/palate. The patient was initially treated with the endoscopic transsphenoidal approach with subsequent open craniotomy.

Management of a Complex Case of a Recurrent Paraganglioma.

Paragangliomas are rare, slow-growing, hypervascular, catecholamine-secreting neuroendocrine tumors arising from the paraganglia. Paragangliomas are rarely found in the head and neck and are typically benign, presenting as a painless, slow-growing mass. Surgical extirpation in combination with long-term surveillance has been long regarded as the standard of care; however, the advances in imaging, radiation therapy, and embolization techniques have improved diagnostic and therapeutic modalities. We present a case of an 87-year-old female who had previously undergone resection of a paraganglioma in 1998, with no evidence of disease in 2002. Eighteen years later, the patient presented to the clinic with otogenic complaints. Imaging showed an expansive mass from the jugular foramen with bone destruction and opacification within the ear canal. The patient opted for observation. The patient eventually presented to the emergency room with neurologic manifestations. Imaging showed a cerebellar abscess prompting emergency drainage. Intraoperative cultures grew Prevotella loescheii and S. intermedius, and the patient was started on 6 weeks of IV antibiotic therapy. Debulking of the paraganglioma was performed followed several months by mastoid and ear canal obliteration; however, the patient experienced complications, including dehiscence of the external auditory canal and Clostridium difficile infection. The patient was eventually treated successfully, marked by a reduction in complaints, a return to baseline activities, and imaging showing no increase in tumor size.

Cogan Syndrome: A Case Study and Review of the Literature.

Cogan syndrome is an autoimmune disease characterized by vestibular symptoms, bilateral sensorineural hearing loss, and inflammatory ocular manifestations, which may be accompanied by systemic vasculitis. We herein present the case of a patient with bilateral sensorineural hearing loss who presented with pain over her cochlear implantation incision site. She was later found to have evidence of ocular disease and underlying vasculitis leading to a diagnosis of Cogan syndrome.

The Influence of Modifiable Risk Factors on Postoperative Outcomes in Patients Receiving Surgery for Resection for Acoustic Neuroma.

Acoustic neuromas are the most common tumor of the cerebellopontine angle that are associated with a number of symptoms that negatively impact a patient's quality of life. While the mainstay of treatment for these benign tumors remains microsurgical resection, there is limited research exploring how certain modifiable risk factors (MRFs) may affect the perioperative course. The purpose of this study was to investigate how MRFs including malnutrition, obesity, dyslipidemia, uncontrolled hypertension, and smoking may affect postoperative rates of readmission and nonroutine discharges. We utilized the 2016 and 2017 Healthcare Cost and Utilization Project Nationwide Readmissions Database. MRFs were queried using appropriate International Classification of Diseases, Tenth Revision (ICD-10) coding for categories including malnutrition, obesity, dyslipidemia, smoking, alcohol, and hypertension. The statistical analysis was done using RStudio (Version 1.3.959). Chi-squared tests were done to evaluate differences between categorical variables. The Mann-Whitney U-testing was utilized to evaluate for statistically significant differences in continuous data. The "Epitools" package was used to develop logistic regression models for postoperative complications and post hoc receiver operating characteristic curves were developed. Pertaining to nonroutine discharge, predictive models using malnutrition outperformed all other MRFs as well as those with no MRFs (P < .05). In the case of readmission, models using malnutrition outperformed those of obesity and smoking (P < .05). Again, an increase in predictive power is seen in models using dyslipidemia when compared to obesity, smoking, or uncontrolled hypertension. Lastly, models using no MRFs outperformed those of obesity, smoking, and uncontrolled hypertension (P < .05). This is the first study of its kind to evaluate the role of MRFs in those undergoing surgical resection of their acoustic neuroma. We concluded that certain MRFs may play a role in complicating a patient's perioperative surgical course.

Tracheal Rupture After Tracheostomy Tube Exchange in a Patient With Recurrent Oral Cavity Spindle Cell Carcinoma.

Oral Cavity Spindle Cell Carcinoma (OCSCC) is a rare variant of squamous cell carcinoma involving the nasopharynx and oral mucosa. This tumor has a high propensity to invade local structures of the head and neck region, making surgical removal challenging and potentially morbid for the patient. Here, we report a case of OCSCC and the complications that were confronted during its surgical resection. Additionally, a review of the literature regarding OCSCC complications and their treatments was performed.

Rhinoplasty Complication Requiring Multiple Revisions.

Cosmetic rhinoplasties are complex surgical procedures that can present with serious complications requiring revision surgeries. Septal graft difficulties are common complications in rhinoplasties, requiring surgeon awareness of the potential future need for corrective surgery. Here, we report a case of multiple revision cosmetic rhinoplasties due to recurrent complications. Additionally, a review of the literature regarding cosmetic rhinoplasty complications and risk factors was performed.

A Case of Mediastinal Emphysema Following Functional Endoscopic Sinus Surgery.

Mediastinal emphysema is an extremely rare and potentially fatal complication of endoscopic sinus surgery. The case presented involves a 73-year-old man who developed mediastinal emphysema following endoscopic sinus surgery for severe sinonasal polyposis. We describe the multiple etiologies that may have contributed to his condition including possible microtrauma at the time of intubation or during intubation, microfracture of the orbital wall/lamina papyracea, and severe hiccups.

Acute Variceal Hemorrhage and Airway Obstruction in a Patient With Reinke's Edema.

Reinke's edema and variceal hemorrhage are complex structural pathologies that affect the vocal cord mucosa. The vocal cords are highly susceptible to environmental stressors, such as smoking and vocal cord usage, thus, treatment involves their corresponding cessation.1 Here, we report a case of a patient with severe Reinke's edema and bilateral chronic vocal cord varices. The patient had a 30-pack-year history of cigarette smoking and was obstructed with intubation due to acute variceal hemorrhage and severe swelling of Reinke's edema. Moreover, a review of the literature regarding Reinke's edema and variceal hemorrhage treatment and their complications was performed.

Penetration of the Vestibule Following a History of Stapedectomy.

This case study describes a 72-year-old female with a history of stapedectomy 40 years prior. She presented experiencing vertigo, fogginess, and imbalance for 9 months. Computed tomography (CT) imaging revealed that the prosthesis was displaced into the vestibule by approximately 2.1 to 2.4 mm. The patient was presented with treatment options, including observation, removal, replacement of the prosthesis, and an oval window patch. The patient opted for observation as the symptoms she was experiencing did not significantly impact her quality of life. Although a stapedectomy may fail for a multitude of reasons, some of the most common causes are prosthesis displacement, especially out of the oval window into the middle ear or away from the incus. Incus necrosis may also play a substantial role in failure.

Facial Paresis After Mandibular Reconstruction.

Condylar neck fractures are common injuries that occur in the facial and mandibular regions. The proximity of this area to the vital neurovasculature creates the increased importance of proper surgical intervention to limit damage to the underlying structures. Here, we report a case of a condylar neck fracture that resulted in temporary paresis of the facial nerve. In addition, a review of the literature regarding condylar fracture treatment and its complications was conducted.

Severe Airway Edema Following Calcium Hydroxylapatite (CaHA) Injection Augmentation.

This case study presents an abnormal complication after routine injection augmentation using calcium hydroxylapatite (CaHA) vocal fold filler in-office on a 73-year-old female. The patient presented initially with severe dysphonia, hypophonia, and a past surgical history of total thyroidectomy, bilateral neck dissection, and a right lateral neck dissection for history of metastatic papillary thyroid carcinoma. She also had a past medical history of hypothyroidism. Post-injection of CaHA, the patient developed severe laryngeal edema, limited vocal cord mobility, obliteration of the pyriform, and a significantly reduced airway aperture requiring intensive care monitoring. Although uncommon, injectable fillers can result in complications which can be severe. Careful technique, the volume of injectate, and hypersensitivity should be considered in reducing complications following injection augmentation. There are multiple injection techniques to consider. The most direct approach is with direct laryngoscopy to allow for visualization of glottic incompetence. A smaller slotted laryngoscope can be considered for vocal fold injection as an alternative and without endotracheal intubation. Although these techniques allow for injection, real-time assessment of vocal fold closure is done in an awake patient. Therefore, laryngeal injection can be considered via percutaneous, per-oral, and trans-nasal approaches.

Laryngotracheal Recurrent Papillomatosis: A Case Study and Survey of Surgical and Systemic Management.

This case report presents a 72-year-old man with longstanding recurrent respiratory papillomatosis (RRP). He has undergone multiple procedures for the condition, including an apparent urgent surgical airway followed by tracheoplasty repair. Modern management of complicated RRP should include both local debulking and systemic approaches. Systemic bevacizumab (Avastin) has shown some initial success as an effective treatment option, in addition to other medications such as pembrolizumab. Other future care strategies may include an HPV vaccination and other adjuvants; vaccination has been reported to have the possibility of drastically reducing the incidence of RRP.

Retained Dental Implant in the Maxillary Sinus.

Implant dentistry has become a popularized means of replacing damaged or missing teeth. Although it has become common practice, there are accounts of implants displacing into surrounding structures, commonly the maxillary sinus. We present the case of a 54-year-old man who presented with chronic left sided pain and pressure found to be secondary to a displaced implant obstructing the left maxillary outflow sinus tract. A systematic review was conducted to assess the current management and treatment options for dental implants displaced into the maxillary sinus. Functional endoscopic sinus surgery (FESS) and the lateral window approach were both found to be safe techniques with minimal postoperative complications.

Endoscopic Management of a Post-Traumatic Internal Maxillary Artery Pseudoaneurysm: Case Report and Review of the Literature.

Internal maxillary artery (IMA) pseudoaneurysms are rare, and typically occur following trauma or orthognathic surgery. Pseudoaneurysms are life-threatening conditions, and expeditious workup and treatment is critical. Endoscopic treatment of an IMA pseudoaneurysm is a feasible option and should be considered when IR embolization is not available. The objective of this study is to review the literature on IMA pseudoaneurysms and present the first reported case of an IMA pseudoaneurysm successfully treated endoscopically.

Invasive Fungal Sinusitis During Active COVID-19 Infection.

This case study demonstrates a 58-year-old female who contracted COVID-19 post-vaccination presenting with severe left-sided facial pain, headaches, and dyspnea. A computed tomography was ordered and showed acute sinusitis, and upon bedside endoscopy, the patient was shown to have necrosis of the left-sided middle turbinate with no discoloration, palate necrosis, or facial changes. All samples of the necrotic tissue were reported to be invasive fungal sinusitis. The entire turbinate was resected in the operating room and ethmoid, frontal, and maxillary sinuses were healthy. Chest x-rays post-operatively showed pulmonary effusions and edema although the patient was not stable enough for a lung examination to rule out a pulmonary fungal infection. A bedside endoscopy showed no further necrosis post-operatively although a repeat endoscopy showed duskiness at the lateral attachment of the basal lamella right at the most posterior resection of the middle turbinate. The patient was placed on multiple antifungal agents. The patient remained in hypoxemic respiratory failure and septic shock while on pressors and 2 weeks following this, expired. Post-COVID-19 patients have been shown in the literature to have an increased risk of developing invasive fungal sinusitis (IFS) and all IFS cases during active COVID-19 infection have had a 100% mortality rate.