Characterization of cerebral radiation necrosis following the treatment of sinonasal malignancies.

Objectives: Our study aims to determine the incidence and potential risk factors for cerebral radiation necrosis (CRN) following treatment of sinonasal malignancies. Methods: One hundred thirty-two patients diagnosed with sinonasal malignancies over an 18-year period were identified at two institutions. Forty-six patients meeting inclusion criteria and treated with radiation therapy were included for analysis. Demographic and clinical-pathologic characteristics were collected and reviewed. Post-treatment magnetic resonance imaging (MRI) at least 1 year following treatment was reviewed to determine presence or absence of CRN. Results: CRN was identified on MRI in 8 of 46 patients (17.4%) following radiation treatment. Patients with a history of reirradiation were more likely to develop CRN (50% vs. 10.5%, p < .05). The BEDs of radiation were also higher in CRN patients compared to non-CRN patients, but this difference was not significant (p > .05). CRN patients had a higher proportion of tumors with skull base involvement than non-CRN patients (100% vs. 57.9%, p = .037). Demographics, comorbidities, pathology, primary tumor subsite, chemotherapy use, and stage of disease demonstrated no significant increase in risk of CRN. Conclusions: Reirradiation and tumor skull base involvement were significant risk factors associated with CRN. Higher average total prescribed and BEDs of radiation were seen in the CRN groups, but these differences were not statistically significant. Gender, comorbidities, tumor subsite, tumor location, and treatment type were not significantly different between groups. Level of evidence: Level 3.

Novel PAX3::INO80D Fusion in Biphenotypic Sinonasal Sarcoma in an Adult.

This case report describes a novel gene fusion of PAX3::INO80D in a biphenotypic sinonasal carcinoma in a woman in her 40s.

Citations and Author Characteristics in Open-Access and Subscription-Based Otolaryngology Journals.

OBJECTIVE: To describe the academic impact and author characteristics of open-access journals in otolaryngology. METHODS: Original articles from three open-access (OTO Open, Laryngoscope Investigative Otolaryngology, and World Journal of Otorhinolaryngology) and three conventional subscription-based otolaryngology specific journals (Otolaryngology - Head & Neck Surgery, The Laryngoscope, JAMA Otolaryngology - Head & Neck Surgery) were assessed. Publication dates of articles from January 2017 to July 2020 were included. Google Scholar and Web of Science citation counts were recorded. H-indexes of first and last authors were included according to Google Scholar and Web of Science and analyzed. RESULTS: This analysis included 3284 articles. Articles published in open-access otolaryngology-specific journals had significantly fewer citations on average (6.8) than articles published in subscription-based journals (12.4, p < 0.0001). The last authors of articles published in subscription-based journals had significantly higher h-indexes (23.50) compared with the last authors of articles published in open-access journals (19.53, p < 0.0001). The first authors of articles published in open-access journals had similar h-indexes (10.26) as the first authors of articles published in subscription-based journals (10.33). CONCLUSIONS: Articles published in open-access journals in otolaryngology were cited significantly less than those published in subscription-based journals. The h-index of the last authors was significantly lower in open-access journals; however, the h-index of the first authors was similar between open-access and subscription-based journals. As measured by citations, open-access publications do not yet appear to have the impact of subscription-based publications. LEVEL OF EVIDENCE: NA Laryngoscope, 133:79-82, 2023.

Malignant Sarcomatous Degeneration of Craniofacial Fibrous Dysplasia.

BACKGROUND: Fibrous dysplasia (FD) is an uncommon bone disease characterized by the replacement of normal bone architecture with abnormal fibro-osseous connective tissue. Here, we discuss 2 cases of craniofacial FD, with malignant sarcomatous degeneration - a rare and morbid complication of the disease. CASE HISTORY: Two cases of craniofacial FD with malignant degeneration are presented. In the first, a 68-year-old male with a history of FD presented with acutely worsening left-sided facial pain and V2 and V3 hypoesthesia. Imaging findings suggested a large infratemporal fossa mass with biopsy demonstrating sarcomatous degeneration. Radical craniofacial resection achieved a gross total resection with likely microscopic disease. The patient was unable to tolerate adjuvant chemotherapy or radiation and succumbed to his disease 13 months following surgery.In the second case, a 36-year-old male with McCune-Albright Syndrome and craniofacial FD presented with acutely worsening left-sided headaches and midface hypoesthesia. Imaging revealed a heterogenous and expansile lesion with erosive changes in the left nasal cavity and infratemporal fossa. Pathology was suggestive of low grade sarcomatous degeneration. Given the extensive involvement of the skull base, the tumor was deemed unresectable, and the patient soon died following initiation of chemotherapy. CLINICAL RELEVANCE: Malignant sarcomatous transformation is a rare and challenging complication of craniofacial FD. Indolent onset, advanced spread at time of presentation, and close relationship with vital neurovascular structures are all hurdles for the treating clinician. The entity poses a diagnostic dilemma, as pathological analysis can be equivocal and may mimic nonmalignant processes, such as locally aggressive FD.

Perioperative Considerations in Endoscopic Skull Base Surgery.

PURPOSE OF THE REVIEW: Present an overview of perioperative considerations specific to endoscopic skull base surgery necessary to maximize successful outcomes. RECENT FINDINGS: The majority of perioperative considerations for endoscopic skull base surgery lack strong supporting evidence and frequently have varied use or implementation amongst institutions. A notable exception comes from a recent randomized controlled trial demonstrating the benefit of lumbar drainage in high-risk cerebrospinal fluid leaks. SUMMARY: Skull base surgeons must consider a multitude of perioperative factors. While many components of perioperative management are extrapolated from related fields such as endoscopic sinus surgery or open cranial base surgery, additional high-quality studies are needed to delineate best practices specific to endoscopic skull base surgery.

Linear IgA Disease of the Gingiva Following Nivolumab Therapy.

Immunotherapy has advanced the treatment of solid organ malignancies. Although generally well tolerated, treatment with immune checkpoint inhibitors can be complicated by immune-related adverse events, some of which are relatively uncommon. We report the first case of gingival linear immunoglobulin A disease related to treatment with an antiprogrammed cell death protein 1 antibody. A 73-year-old male with advanced non-small cell lung cancer achieved a durable response to nivolumab monotherapy. After 1 year of treatment, he developed gingival swelling and pain. Biopsy revealed linear immunoglobulin A disease of the gingiva which was effectively treated with systemic steroids. Ongoing vigilance for immune-mediated toxicity is paramount during and after treatment with immune checkpoint inhibitors.

Association of Hearing Loss and Otologic Outcomes With Fibrous Dysplasia.

Importance: Fibrous dysplasia (FD) and McCune-Albright syndrome (MAS) are rare bone and endocrine disorders in which expansile fibro-osseous lesions result in deformity, pain, and functional impairment. The effect of FD on hearing and otologic function has not been established. Objectives: To characterize audiologic and otologic manifestations in a large cohort of individuals with FD/MAS and to investigate potential mechanisms of hearing loss. Design, Setting, and Participants: In this natural history study, individuals with craniofacial FD seen at a clinical research center underwent clinical, biochemical, computed tomographic, audiologic, and otolaryngologic evaluations. Main Outcomes and Measures: Clinical and radiologic features associated with hearing loss and otologic disease were evaluated. Conductive hearing loss was hypothesized to be associated with narrowing of the external auditory canal (EAC), FD involving the epitympanum, and FD crowding the ossicular chain. Sensorineural hearing loss was hypothesized to be associated with FD affecting the internal auditory canal (IAC) and otic capsule. Results: Of the 130 study participants with craniofacial FD who were evaluated, 116 (89.2%) had FD that involved the temporal bone (median age, 19.6 years; range, 4.6-80.3 years; 64 female [55.2%]), whereas 14 (10.8%) had craniofacial FD that did not involve the temporal bone. Of the 183 ears with temporal bone FD, hearing loss was identified in 41 ears (22.4%) and was conductive in 27 (65.9%), sensorineural in 12 (29.3%), and mixed in 2 (4.9%). Hearing loss was mild and nonprogressive in most participants. Whereas EACs were narrower in ears with FD (mean difference [MD], 0.33 mm; 95% CI, 0.11-0.55 mm), this finding was associated with conductive hearing loss in only 4 participants. Fibrous dysplasia crowding of the ossicles was associated with conductive hearing loss (odds ratio [OR], 5.0; 95% CI, 2.1-11.6). The IAC length was not different between ears with and without FD (MD, -0.37; 95% CI, -0.95 to 0.211); however, canals were elongated in ears with sensorineural hearing loss (MD, -1.33; 95% CI, -2.60 to -0.07). Otic capsule involvement was noted in only 4 participants, 2 of whom had sensorineural hearing loss. Both MAS-associated growth hormone excess (OR, 3.1; 95% CI, 1.3-7.5) and neonatal hypercortisolism (OR, 11; 95% CI, 2.5-55) were associated with an increased risk of hearing loss . Conclusions and Relevance: Hearing loss in craniofacial FD is common and mild to moderate in most individuals. It typically arises from FD crowding of the ossicular chain and elongation of the IAC, whereas EAC stenosis and otic capsule invasion are less common causes. Individuals with craniofacial FD should undergo otolaryngologic evaluation and monitoring, including assessment to identify those with high-risk features.

Ectopic parathyroid adenoma in the pyriform sinus.

BACKGROUND: Ectopic parathyroid glands may complicate the surgical approach to primary hyperparathyroidism. We describe a rare ectopic parathyroid location, the pyriform sinus. METHODS: Three patients, 2 after prior unsuccessful parathyroid exploration, were found to have ectopic parathyroid adenomas in the pyriform sinus. Two cases were identified by 4D-CT of the neck. The other was localized by a technetium 99m sestamibi single photon emission CT (SPECT). In each case, office endoscopy confirmed the lesions. The patients were treated by either transoral laser or robotic resection of the parathyroid adenoma. RESULTS: After surgery, the patients had resolution of their hyperparathyroidism. CONCLUSION: Ectopic parathyroid adenomas in the pyriform sinus are rare, but should be considered in those patients who have had a failed neck exploration and in those who undergo 4D-CT scanning or SPECT scanning.

Endoscopic Endonasal Optic Nerve Decompression for Fibrous Dysplasia.

Objective To evaluate visual outcomes and potential complications for optic nerve decompression using an endoscopic endonasal approach (EEA) for fibrous dysplasia. Design Retrospective chart review of patients with fibrous dysplasia causing extrinsic compression of the canalicular segment of the optic nerve that underwent an endoscopic endonasal optic nerve decompression at the University of Pittsburgh Medical Center from 2010 to 2013. Main Outcome Measures The primary outcome measure assessed was best-corrected visual acuity (BCVA) with secondary outcomes, including visual field testing, color vision, and complications associated with the intervention. Results A total of four patients and five optic nerves were decompressed via an EEA. All patients were symptomatic preoperatively and had objective findings compatible with compressive optic neuropathy: decreased visual acuity was noted preoperatively in three patients while the remaining patient demonstrated an afferent pupillary defect. BCVA improved in all patients postoperatively. No major complications were identified. Conclusion EEA for optic nerve decompression appears to be a safe and effective treatment for patients with compressive optic neuropathy secondary to fibrous dysplasia. Further studies are required to identify selection criteria for an open versus an endoscopic approach.

Endoscopic Endonasal Approach for Transclival Resection of a Petroclival Meningioma: A Technical Note.

The endoscopic endonasal transclival approach has been widely described for its use to resect clivus chordomas, but there have only been isolated reports of its use for petroclival meningiomas. These tumors are most often resected utilizing open transpetrosal approaches, but these operations, difficult even in the hands of dedicated skull base surgeons, are particularly challenging if the meningiomas are medially-situated and positioned mainly behind the clivus. For this subset of petroclival meningiomas, a transclival approach may be preferable. We report a meningioma resected via an endoscopic endonasal transclival technique. The patient was a 63-year-old man who presented originally for medical attention because of diplopia related to an abducens palsy on the left. A workup at that time revealed a meningioma contained entirely in the left cavernous sinus, and this was treated with stereotactic radiosurgery. His symptoms resolved and his meningioma was stable on MRI for several years after treatment. The patient was then lost to follow-up until 13 years after radiosurgery when he experienced intermittent diplopia again. At this point, workup revealed a large petroclival meningioma compressing the brainstem. He underwent a successful endoscopic endonasal transclival resection of this tumor. A demonstration of the step-by-step surgical technique, discussion of the nuances of the operation, and a comparison with the open transpetrosal approaches are included in our report.

Sinonasal disease in polyostotic fibrous dysplasia and McCune-Albright Syndrome.

OBJECTIVES/HYPOTHESIS: To characterize the spectrum, symptoms, progression, and effects of endocrine dysfunction on sinonasal disease in polyostotic fibrous dysplasia (PFD) and McCune-Albright Syndrome (MAS). STUDY DESIGN: Retrospective review. METHODS: A prospectively followed cohort of subjects with PFD/MAS underwent a comprehensive evaluation that included otolaryngologic and endocrine evaluation, and imaging studies. Head and facial computed tomography scans were analyzed, and the degree of fibrous dysplasia (FD) was graded using a modified Lund-MacKay scale. Those followed for >4 years were analyzed for progression. RESULTS: A total of 106 patients meeting inclusion criteria were identified with craniofacial FD. A majority (92%) demonstrated sinonasal involvement. There were significant positive correlations between the sinonasal FD scale score and chronic congestion, hyposmia, growth hormone excess, and hyperthyroidism (P < .05 for all). Significant correlations were not found for headache/facial pain or recurrent/chronic sinusitis. Thirty-one subjects met the criteria for longitudinal analysis (follow-up mean, 6.3 years; range, 4.4-9 years). Those who demonstrated disease progression were significantly younger than those who did not (mean age, 11 vs. 25 years). Progression after age of 13 years was uncommon (n = 3) and minimal. Concomitant endocrinopathy or bisphosphonate use did not have any significant effect on progression of disease. CONCLUSIONS: Sinonasal involvement of fibrous dysplasia in PFD/MAS is common. Symptoms are usually few and mild, and disease progression occurs primarily in young subjects. Concomitant endocrinopathy is associated with disease severity, but not progression.

Meta-analysis of endoscopic versus sublabial pituitary surgery.

OBJECTIVES/HYPOTHESIS: To determine whether the endoscopic or sublabial transseptal transsphenoidal approach for pituitary surgery has superior outcomes or decreased complication rates. STUDY DESIGN: Retrospective meta-analysis of literature. METHODS: A MEDLINE search was conducted identifying all relevant literature in the English language from 1998 through 2010. Studies explicitly identifying pure sublabial or completely endoscopic approaches were included. Meta-analysis comparing the two techniques was performed for multiple outcome measures utilizing a random effects approach. RESULTS: A total of 21 endoscopic studies (n = 2,335) and 17 sublabial studies (n = 2,565) met inclusion criteria. Analysis revealed significantly superior rates of gross tumor resection (79% vs. 65%, P < .0001), as well as lower rates of cerebral spinal fluid leak (5% vs. 7%, P < .01), septal perforation (0% vs. 5%), and postoperative epistaxis (1% vs. 4%, P < .0001) for the endoscopic approach compared to the sublabial approach. There was no difference between the two techniques in the incidence of meningitis, diabetes insipidus, or resolution of hormonal abnormality. Hospital stay (P = .01) was shorter for endoscopic surgery compared to sublabial surgery, and there was no significant difference in terms of length of operation. CONCLUSIONS: The sublabial transsphenoidal approach has been the gold standard for pituitary surgery for many years. However, meta-analysis of the recent literature demonstrates superior outcomes and decreased postoperative complications with the endoscopic approach, potentially justifying a shift toward endoscopic pituitary surgery.

Differential and history-dependent modulation of a stretch receptor in the stomatogastric system of the crab, Cancer borealis.

Neuromodulators can modify the magnitude and kinetics of the response of a sensory neuron to a stimulus. Six neuroactive substances modified the activity of the gastropyloric receptor 2 (GPR2) neuron of the stomatogastric nervous system (STNS) of the crab Cancer borealis during muscle stretch. Stretches were applied to the gastric mill 9 (gm9) and the cardio-pyloric valve 3a (cpv3a) muscles. SDRNFLRFamide and dopamine had excitatory effects on GPR2. Serotonin, GABA, and the peptide allatostatin-3 (AST) decreased GPR2 firing during stretch. Moreover, SDRNFLRFamide and TNRNFLRFamide increased the unstimulated spontaneous firing rate, whereas AST and GABA decreased it. The actions of AST and GABA were amplitude- and history-dependent. In fully recovered preparations, AST and GABA decreased the response to small-amplitude stretches proportionally more than to those evoked by large-amplitude stretches. For large-amplitude stretches, the effects of AST and GABA were more pronounced as the number of recent stretches increased. The modulators that affected the stretch-induced GPR2 firing rate were also tested when the neuron was operating in a bursting mode of activity. Application of SDRNFLRFamide increased the bursting frequency transiently, whereas high concentrations of serotonin, AST, and GABA abolished bursting altogether. Together these data demonstrate that the effects of neuromodulators depend on the previous activity and current state of the sensory neuron.