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.

Nasal Septal Perforation Secondary to Graft-Versus-Host Disease.

Nasal septal perforation is a full-thickness defect of the nasal septum. There are many described etiologies of nasal septal perforation, including trauma, infectious, neoplastic, iatrogenic, and autoimmune. Graft-versus-host disease (GVHD) is a common and potentially life-threatening complication that can occur after an allogenic transplant. GVHD can result in the development of autoantibodies that lead to granulomatous inflammation with necrotizing vasculitis, causing perforation of the nasal septum. In this report, we describe a patient with nasal septal perforation secondary to GVHD and hope to provide novel insights into the association of GVHD and nasal septal perforation.

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.

Chronic cerebrospinal fluid rhinorrhea as an initial presentation of chordoma: illustrative case.

BACKGROUND: Skull base chordomas are typically extradural and present with cranial nerve deficits, headache, and visual disturbances. Clival chordoma involving the dura and presenting as a spontaneous cerebrospinal fluid (CSF) leak is extremely rare and can be mistaken for other skull base lesions. Here the authors present a case of chordoma with an unusual presentation. OBSERVATIONS: A 43-year-old female who presented with clear nasal drainage was diagnosed with CSF rhinorrhea secondary to a clival defect previously thought to be ecchordosis physaliphora. The patient subsequently developed bacterial meningitis and underwent endoscopic, endonasal, transclival gross-total resection of the lesion with repair of the dural defect. Pathology revealed brachyury-positive chordoma. She received adjuvant proton beam radiotherapy and has remained stable for 2 years. LESSONS: Spontaneous CSF rhinorrhea can occur as a rare primary presentation of clival chordoma, requiring careful radiological interpretation and a high index of suspicion for diagnosis. Chordoma cannot be reliably differentiated from benign notochordal lesions based on imaging alone; thus, intraoperative exploration and immunohistochemistry play key roles. Clival lesions presenting with CSF rhinorrhea should undergo prompt resection to facilitate diagnosis and prevent complications. Future studies on connections between chordoma and benign notochordal lesions may help to establish management guidelines.

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.

Decision-Making in Clival Mass Lesions: Risk Factors for Malignant Disease and an Illustrative Case Example.

Introduction Clival tumors are rare and heterogeneous. Although some benign prototypical sellar lesions may present as clival tumors, the likelihood of malignant disease is higher. Here we define a novel algorithm for the workup and management of clival masses through an illustrative case of colorectal adenocarcinoma metastasis to the clivus. Methods In this case report, the best practice guidelines for managing clival masses are described through a literature review and refined by senior author consensus. We conducted a focused systematic review to characterize the present case in the context of clival metastasis from gastrointestinal malignancy. Results An 83-year-old woman presented with 4 weeks of headaches and blurry vision. Examination revealed partial right abducens and left oculomotor palsies. Magnetic resonance imaging (MRI) identified a large, weakly enhancing sellar and clival mass with sphenoid sinus extension. An aggressive subtotal endoscopic endonasal resection was performed with removal of all sphenoid, clival, and sellar disease without cavernous sinus wall resection. Pathology confirmed colorectal adenocarcinoma; computed tomography (CT) imaging identified an ascending colon mass with metastases to the liver and mesenteric nodes. Palliative oncologic therapies were recommended, but she elected hospice, and died 3 months after initial presentation. Gastrointestinal clival metastases are exceedingly rare among sellar and clival pathologies, with eight prior cases reported, most of which presented with diplopia from abducens nerve involvement. Conclusion Clival masses are uncommon skull base lesions that are associated with more aggressive diseases. We present a consolidated framework for decision-making in these challenging patients, alongside an unusual case example that illustrates the importance of increased suspicion for malignant clinical entities in this setting.

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.

From a Slave to a Surgeon: David Kearney McDonogh, the First Black Otolaryngologist.

David McDonogh, born into chattel slavery in Louisiana in the early 1800s, accomplished the unfathomable by becoming the first Black otolaryngologist in the United States of America. With tireless determination and profound intellect, Dr McDonogh surmounted immeasurable adversity along his improbable journey to freedom and success as an eye, ear, nose, and throat doctor in New York. His doctorate in medicine was posthumously awarded to his great-great-granddaughter in 2018 by the Columbia University Vagelos College of Physicians and Surgeons. In this History of Otolaryngology piece, we share his extraordinary story.

Nonfunctioning Pituitary Lesions.

Nonfunctioning pituitary lesions represent a subset of pituitary adenomas that do not manifest with clinical features of hormone hypersecretion. Because of their indolent nature, their diagnosis is elusive, often resulting in presentation after the tumors have grown large enough to cause compressive symptoms. Although they are clinically silent, the various subtypes correspond to the predominant cell line of origin and therefore are biochemically distinct from one another. This article reviews the biochemical, clinical, and histopathologic features of each of these subtypes. A rubric is provided for diagnostic work-up of these lesions and the management options available to the treating clinician.

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.

Oral resveratrol therapy inhibits cancer-induced skeletal muscle and cardiac atrophy in vivo.

The mechanism by which cancer mediates muscle atrophy has been delineated in the past 3 decades and includes a prominent role of tumor-derived cytokines, such as IL-6, TNFα, and IL-1. These cytokines interact with their cognate receptors on muscle to activate the downstream transcription factor NF-κB and induce sarcomere proteolysis. Experimentally, inhibiting NF-κB signaling largely prevents cancer-induced muscle wasting, indicating its prominent role in muscle atrophy. Resveratrol, a natural phytoalexin found in the skin of grapes, has recently been shown to inhibit NF-κB in cancer cells, which led us to hypothesize that it might have a protective role in cancer cachexia. Therefore, we investigated whether daily oral resveratrol could protect against skeletal muscle loss and cardiac atrophy in an established mouse model. We demonstrate resveratrol inhibits skeletal muscle and cardiac atrophy induced by C26 adenocarcinoma tumors through its inhibition of NF-κB (p65) activity in skeletal muscle and heart. These studies demonstrate for the first time the utility of oral resveratrol therapy to provide clinical benefit in cancer-induced atrophy through the inhibition of NF-κB in muscle. These findings may have application in the treatment of diseases with parallel pathophysiologies such as muscular dystrophy and heart failure.

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.

When, how and why to treat the neck in patients with esthesioneuroblastoma: a review.

Esthesioneuroblastoma is an uncommon tumor that presents in the sinonasal cavity and anterior skull base. Cervical metastases are not frequently found on initial presentation but eventually occur in 20-25% of these patients. This presents the treating physician with the difficult decision as to how and when to treat the neck in this disease. The aims of this study were to provide a comprehensive review of the incidence of N+ disease at presentation, make recommendations about the optimal treatment strategy of patients with N+ disease, explain the role of elective neck treatment in patients with N0 disease, and comment on treatment of patients with late cervical metastases that require salvage therapy, using the literature review of the incidence and treatment of neck disease in patients with esthesioneuroblastoma. This review revealed an approximately 5-8% incidence of cervical nodal metastasis at the time of presentation. Combined modality therapy with surgery and radiotherapy is recommended to treat the N+ neck at the time of diagnosis and later. Chemotherapy may have a role combined with radiation treatment, but there are little data to support this. There is limited evidence to substantiate the use of elective neck dissection or elective radiotherapy in the clinically and radiologically N0 neck. Patients who have late cervical metastases have a clear survival advantage (59 vs. 14%) when treated with combined surgery and radiotherapy relative to single modality methods alone. The results indicate that the management of the neck in esthesioneuroblastoma continues to be a significant challenge in the treatment algorithm of these complex patients.

Endoscopic trans-pterygoid resection of a low-grade cribriform cystadenocarcinoma of the infratemporal fossa.

This article presents a case of low-grade cribriform cystadenocarcinomas (LGCCC), a rare salivary gland tumor manifesting in the infratemporal fossa (ITF). The lesion in this case is unique in its location, histopathology, and management in that the tumor resection was performed using an exclusively endoscopic, endonasal approach. This case highlights the expanding application of endoscopic skull base techniques to address an indolent, slow-growing malignancy of the ITF.

Asthma and Chronic Rhinosinusitis: Diagnosis and Medical Management.

Asthma is a prevalent inflammatory condition of the lower airways characterized by variable and recurring symptoms, reversible airflow obstruction, and bronchial hyperresponsiveness (BHR). Symptomatically, these patients may demonstrate wheezing, breathlessness, chest tightness, and coughing. This disease is a substantial burden to a growing population worldwide that currently exceeds 300 million individuals. This is a condition that is frequently encountered, but often overlooked in the field of otolaryngology. In asthma, comorbid conditions are routinely present and contribute to respiratory symptoms, decreased quality of life, and poorer asthma control. It is associated with otolaryngic diseases of the upper airways including allergic rhinitis (AR) and chronic rhinosinusitis (CRS). These conditions have been linked epidemiologically and pathophysiologically. Presently, they are considered in the context of the unified airway theory, which describes the upper and lower airways as a single functional unit. Thus, it is important for otolaryngologists to understand asthma and its complex relationships to comorbid diseases, in order to provide comprehensive care to these patients. In this article, we review key elements necessary for understanding the evaluation and management of asthma and its interrelatedness to CRS.

The Endoscopic Endonasal Approach to the Hypoglossal Canal: The Role of the Eustachian Tube as a Landmark for Dissection.

IMPORTANCE: Improvements in endoscopic technology and reconstructive techniques have made the endoscopic endonasal approach (EEA) a viable option to approach ventromedial lesions in the region of the hypoglossal canal. Prior to contemplating this surgical corridor, a thorough understanding of anatomic relationships and landmarks is essential to safely approach this region of the posterior skull base through an EEA. OBJECTIVE: To describe the surgical technique and anatomic landmarks in the EEA to the hypoglossal canal through referencing nasopharyngeal and posterior skull base anatomy. DESIGN, SETTING, AND PARTICIPANTS: Study of latex-injected cadaveric heads at the North Carolina Eye Bank Multidisciplinary Surgical Skills Laboratory at the University of North Carolina. INTERVENTIONS: An EEA to the hypoglossal canal was carried out bilaterally in 5 embalmed, latex-injected cadaver heads. MAIN OUTCOMES AND MEASURES: Cadaveric measurements of anatomic landmarks and relationships in the approach were obtained using a 10-cm surgical ruler and were reported as mean distances. Additionally, high-quality endoscopic images demonstrating the operative technique and anatomic relationships were obtained. RESULTS: The distance between the lacerum segment of the internal carotid arteries, the superolateral boundary, was 23.6 mm (SD, 11.8 mm). The distance between the anterolateral edge of the occipital condyles, the inferolateral boundary, was 19 mm (SD, 0.80 mm). The supracondylar groove was identified in the same anteroposterior plane as the nasopharyngeal orifice of the eustachian tube, and the anterior-most edge of the occipital condyle was 14 mm (SD, 0.82 mm) from the posterosuperior edge of the salpingopharyngeal fold. Additionally, the transtubercular corridor was on the same plane as the superior edge of the torus tubarius in the anteroposterior axis. The distance to the hypoglossal canal from midline was 10 mm, which was found after completing drilling in the transcondylar and transtubercular corridors. Last, the hypoglossal nerve rootlets were identified entering the canal 6 mm inferiorly and 8 mm laterally from the vertebrobasilar junction. CONCLUSIONS AND RELEVANCE: The eustachian tube and other elements of nasopharyngeal anatomy are fixed landmarks that provide important points of reference when approaching the hypoglossal canal through an EEA. A thorough understanding of these anatomic relationships is vital in safely navigating this direct, surgical corridor to the posterior fossa.

Image-guided robotic skull base surgery.

Objectives To describe the potential uses of computed tomography image guidance in concert with the surgical robot for skull base surgery. Design An anatomical study was conducted. Setting Tertiary academic center. Participants Cadaveric skull. Main Outcome Measures The primary outcome measure was to measure the accuracy of robotic arm positioning to anatomical landmarks on a skull using image guidance and the surgical robot synchronously. Instruments with different angles of rotations were used. Estimated systematic error was calculated and compared with achieved errors. Clinical applications of metachronous image guidance and robotic system were discussed. Results The skull model approximated < 1 mm accuracy using standard image guidance instruments and the 0-degree robotic arm positioning. Increased angles of instruments from 20 to 60 degrees on the robotic system revealed more significant increases in error than estimated. Conclusions Image guidance may be useful for transoral robotic approaches. Precise movements are improved by limiting the angle of deviation. Future studies will help optimize the combined technologies before validating the study in clinical settings.