Not Your Typical Nosebleed: A Case Report and Personal Account of a Patient With Osler-Weber-Rendu Syndrome.

Introduction Osler-Weber-Rendu syndrome, or hereditary hemorrhagic telangiectasia, is a rare genetic disease that causes recurrent epistaxis and anemia. Numerous bleeding vascular malformations can be found throughout the body. Case presentation A 75-year-old woman presented to her hematologist with recurrent epistaxis, iron deficiency anemia, menorrhagia, and hypothyroidism. Her mother had similar nosebleeds, and physical examination revealed small vascular malformations on the conjunctiva, oropharynx, tongue, lip, and palate. Heavy epistaxis occurred several times per week. Multiple nasal and gastrointestinal endoscopic procedures were performed. She received over 100 iron infusions and multiple blood transfusions. Overall treatment involved integrated care with multiple medical specialties. Conclusion Hereditary hemorrhagic telangiectasia and other complex diseases are best treated with a multidisciplinary approach within an integrated health care setting.

The Adult Neck Mass: Predictors of Malignancy.

OBJECTIVE: Distinguishing benign from malignant adult neck masses can be challenging because data to guide risk assessment are lacking. We examined patients with neck masses from an integrated health system to identify patient and mass factors associated with malignancy. STUDY DESIGN: Retrospective cohort. SETTING: Kaiser Permanente Northern California. METHODS: The medical records of adults referred to otolaryngology in 2017 for a neck mass were evaluated. Bivariate and multivariable logistic regression analyses were performed. RESULTS: Malignancy was found in 205 (5.0%) of the cohort's 4103 patients. Patient factors associated with malignancy included sex, age, and race/ethnicity. Males had more than twice the odds of malignancy compared with females (adjusted odds ratio [aOR] = 2.38). Malignancy rates increased with age, ranging from 2.1% for patients younger than 40 years to 8.4% for patients 70 years or older. White non-Hispanic patients had 1.75 times the risk of malignancy compared with patients of other race/ethnicities. The percentage of patients with malignancy increased with increasing minimum mass dimension, from 3.0% in patients with mass size <1 cm to over 31% in patients with mass sizes 2 cm or larger (P < .0001). Imaging-based mass factors most highly predictive of malignancy included larger minimum mass dimension (≥1.5 cm vs <1.5 cm: aOR = 3.87), multiple masses (2 or more vs 1: aOR = 5.07), and heterogeneous/ill-defined quality (aOR = 2.57). CONCLUSION: Most neck masses referred to otolaryngology were not malignant. Increasing age, male sex, white non-Hispanic ethnicity, increasing minimum mass dimension, multiple neck masses, or heterogeneous architecture/ill-defined borders were associated with malignancy.

Transoral Excision of Parapharyngeal Tumors.

OBJECTIVES: The transoral approach for parapharyngeal space tumors has been criticized due to its restrictive exposure and increased rate of vascular and nerve injuries. However, for certain tumors-namely those that are prestyloid, benign, and well-encapsulated-the transoral approach reduces overall morbidity while providing direct access. Transoral surgeons can rely on this approach for select tumors seen clearly on intraoral examination in the bulging lateral pharynx. In this article and with a supplemental operative technique video, we demonstrate our transoral approach in these select patients. METHODS: We reviewed our 26 cases using this approach and chose a representative case of a 50-year-old man who presented with a large, prestyloid, encapsulated parapharyngeal mass affecting his voice and swallowing. A transoral approach to the parapharyngeal tumor is illustrated. RESULTS: A vertical curvilinear incision is completed from the hamulus to below the inferior extent of the tumor. Blunt dissection of the tumor capsule proceeds inferiorly. Controlled violation of the tumor capsule can aid in visualization. After tumor delivery, the wound is irrigated and closed. CONCLUSIONS: In our experience, the transoral approach is an effective method to access well-encapsulated, prestyloid parapharyngeal space tumors. Patient selection is important when considering a transoral approach.

Duplicate Omohyoid Muscle Causing Progressive Dysphagia and Dyspnea: A Case Report.

INTRODUCTION: Duplicate omohyoid muscles are uncommon anomalies and exceedingly rare causes of progressive clinical symptoms. The goals of this case report are to describe the clinical characteristics, cross-sectional imaging, intraoperative findings, and curative treatment of our patient, to develop a differential diagnosis for this condition, and to review the pertinent literature regarding this particular type of anomalous omohyoid muscle as one among many variations. CASE PRESENTATION: A 20 year-old man presented with progressive limited neck range of motion followed by dysphagia and then dyspnea caused by his duplicate omohyoid muscle. He underwent curative surgery in 2015 at our tertiary care center. DISCUSSION: Although rare, a duplicate omohyoid muscle should be considered in the differential diagnosis of dysphagia and dyspnea with concurrent central neck deformity. We report the first case, to our knowledge, of an anomalous omohyoid that caused significant progressive clinical symptoms. Direct excision of the restrictive anomalous tissue proved curative.

Vidian Nerve Schwannoma: A Rare Skull-Base Neoplasm Presenting with Ocular Manifestations: A Case Report and Literature Review.

INTRODUCTION: Vidian nerve schwannomas are exceedingly rare, with only 7 cases reported since 2006. Patients presenting with ocular symptoms have been reported in only 1 case. CASE PRESENTATION: A 54-year-old woman presented with a 3-month history of right periorbital pressure, third cranial nerve palsy, and visual field defect. Imaging results showed a right sphenoid skull-base mass with obliteration of the vidian canal that extended into the pterygopalatine fossa. The patient underwent an extended endoscopic resection with pterygopalatine fossa dissection. Pathologic findings demonstrated a schwannoma. DISCUSSION: A literature review showed that this is the second reported case of a vidian nerve schwannoma presenting with ocular symptoms and that endoscopic resections are becoming the standard of care. Practitioners should be aware that vidian nerve schwannomas can present as a skull-base mass with predominantly ocular symptoms, including vision loss, secondary to mass effect. Consideration should be given to this entity in the setting of typical radiographic and histopathologic characteristics. Endoscopic approaches to resection are safe and have low morbidity.

Diagnostic Pathway of Oral Cavity Cancer in an Integrated Health Care System.

CONTEXT: Survival for patients with oral cavity squamous cell carcinoma (OCSCC) has remained relatively stagnant despite advances in treatment. Few studies have examined why advanced-stage disease is diagnosed in 40% of patients with OCSCC nationally. OBJECTIVE: To characterize the diagnostic pathway of OCSCC in an integrated health care system. DESIGN: Retrospective study of patients with OCSCC (2007-2010). MAIN OUTCOME MEASURES: Referral patterns and demographic, clinical, and tumor characteristics associated with time to diagnosis (diagnostic interval). RESULTS: Of 247 patients, 167 (68%) had early-stage (I/II) disease, 86 (35%) were referred by dentists, and 70 (28%) had a history of premalignancy. The median time (interquartile range) from symptom onset to care sought from a primary care physician (patient interval), from primary care physician to otolaryngologist, and from otolaryngologist to diagnosis was 8.6 (4.0-25.8), 1.0 (0.6-3.1), 0.0 (0.0-3.0) weeks, respectively. These intervals did not differ by demographic characteristics, clinical factors, or tumor stage. Prolonged diagnostic intervals were observed among patients with premalignant lesions. CONCLUSION: The patient interval was the largest component of the total diagnostic interval. The subsequent professional workup proceeded relatively efficiently. Prolonged diagnostic interval in patients with premalignant lesions may reflect the natural history of malignant transformation rather than a delay in diagnosis. However, nearly one-fourth of these cases were diagnosed at an advanced stage; closer surveillance may represent an opportunity for diagnosis at an earlier stage. Surveillance for premalignant lesions and facilitating referrals from dentists may expedite the diagnosis and treatment of OCSCC. Further investigation is warranted.

The diagnostic pathway of oropharyngeal squamous cell carcinoma in a large U.S. healthcare system.

OBJECTIVE: To examine the current diagnostic pathway of oropharyngeal squamous cell carcinoma (OPSCC) and identify factors associated with time to diagnosis. METHODS: Retrospective cohort study of patients with OPSCC in an integrated healthcare system from January 1, 2013, to December 31, 2013. Patient demographics, tobacco and alcohol use, chief complaint, tumor stage, human papilloma virus (HPV) status, physician factors (diagnosis, antibiotic prescription, performance of endoscopic exam, biopsy), and time intervals were examined. Time variations by patient characteristics and physician practice were assessed. RESULTS: We identified 152 patients with OPSCC. Of those, 90% had stage III to IV disease. The cohort was largely male (85%), white (79%), with HPV-positive tumors (84%). Most common chief complaints were neck mass (52%) and sore throat (20%). Among those with neck a mass, 94% had HPV-positive tumors. Prescription of antibiotics was associated with longer time to first otolaryngology evaluation. Median time from symptom onset to first primary care physician (PCP) contact was 3.0 weeks; from PCP to otolaryngologist was 1.1 weeks, and from otolaryngologist to tissue diagnosis was 0.4 weeks. At the first otolaryngology visit, 82% underwent in-office flexible endoscopy and 58% had same-day biopsy, resulting in rapid time to tissue diagnosis. Diagnostic time intervals did not differ by HPV status. CONCLUSION: The overall diagnostic process was efficient, although initial antibiotic treatment resulted in longer time to first otolaryngology visit. Tumor HPV status was associated with presenting findings but not time to diagnosis. The variation in diagnostic delay time and impact on survival outcomes is unknown and merits further investigation. LEVEL OF EVIDENCE: 4. Laryngoscope, 1867-1873, 2018.

Deadly Sphenoid Fungus-Isolated Sphenoid Invasive Fungal Rhinosinusitis: A Case Report.

INTRODUCTION: Acute invasive fungal rhinosinusitis (AIFRS) is a potentially fatal infection, usually affecting immunocompromised patients. Isolated sphenoid sinus involvement is rare and has been reported in only a few cases. We discuss the clinical characteristics, histopathologic features, and differential diagnosis of AIFRS of the sphenoid sinus. CASE PRESENTATION: A 57-year-old man with a history of refractory non-Hodgkin lymphoma and neutropenia presented with a 1-week duration of left-sided headache and ipsilateral cheek paresthesia. Nasal endoscopy showed mucoid drainage from the sphenoethmoidal recess. Magnetic resonance imaging demonstrated left sphenoid mucosal thickening and enhancement along the adjacent skull base. The patient underwent endoscopic sinus surgery with extended sphenoidotomy and débridement. The lateral wall and recess of the left sphenoid sinus demonstrated pale mucosa and fungal debris. Pathologic analysis demonstrated necrotic tissue and fungal hyphae with angioinvasion. Microbiology studies isolated Aspergillus fumigatus. The right maxillary sinus contained a synchronous fungal ball, which was removed during surgery; there was no evidence of tissue necrosis or invasive fungus in the maxillary sinus. He was treated with long-term voriconazole therapy, and 6-month follow-up showed disease resolution. DISCUSSION: AIFRS should be considered in the differential diagnosis of immunocompromised patients with nonspecific sinonasal symptoms. Usually, AIFRS is diffuse with multiple sinus involvement; however, isolated sphenoid AIFRS can occur. This is one of the few cases of AIFRS demonstrating isolated sphenoid involvement and is thought to be the first case showing a synchronous noninvasive fungal ball of another sinus cavity. Prompt recognition and surgical treatment may be curative and lifesaving.

Relationship of eosinophils and plasma cells to biofilm in chronic rhinosinusitis.

BACKGROUND: This study investigates the relationship of eosinophils and plasma cells to biofilm in chronic rhinosinusitis (CRS). A prospective observational study was performed at the Keck Hospital, University of Southern California, Department of Otolaryngology, Los Angeles, CA. METHODS: A total of 29 patients, 20 undergoing endoscopic sinus surgery for CRS and 9 control patients undergoing septoplasty for nasal obstruction without history or evidence of CRS, were included in this study. Contiguous sinonasal mucosa sample sections were examined by hematoxylin and eosin (H&E), fluorescence in situ hybridization (FISH), and immunohistochemistry (IHC) for biofilm, microbes, eosinophil major basic protein (EMBP), and cluster designation 27 (CD27). EMBP and CD27 were used as eosinophil and plasma cell markers, respectively. RESULTS: Biofilm was visualized in 15 of 20 patients with CRS on H&E sections, confirmed by microbial presence using FISH. Biofilm was not identified in tissue samples of the nine control patients. On IHC analysis, CD27 and EMBP expression were significantly higher in patients with CRS compared with control (p < 0.05) and had greater expression in biofilm-positive patients compared with biofilm-negative patients. Nasal polyps correlated with higher expression of CD27 and EMBP, but in CRS patients without polyps CD27 and EMBP was also significantly greater in biofilm-positive specimens compared with biofilm-negative specimens. CONCLUSION: Biofilm presence in CRS appears to correlate to host inflammatory response involving plasma cell and eosinophil recruitment.