Faculty Bibliography

BACKGROUND AND AIMS/OBJECTIVE:Liver involvement is an increasingly recognised complication of common variable immunodeficiency (CVID). Nodular regenerative hyperplasia (NRH), a subgroup of porto-sinusoidal vascular disorder, and manifestations of portal hypertension (PH) unrelated to cirrhosis are the most common findings. Nonetheless, the evolution of liver disease over time remains unknown. METHODS:Retrospective review of patients followed at the National Institutes of Health with CVID-related liver disease and liver biopsy from 1990 to 2020. Clinical, imaging and histological follow-up were recorded as part of clinical research protocols. RESULTS:/L, spleen size 19.5 cm, hepatic venous pressure gradient 9.5 mmHg and 37.5% of patients had signs of PH. Cumulative incidence of PH was 65% at 5 years. In a subgroup of 16 patients, a follow-up liver biopsy, performed at a median time of 3 years after the index biopsy, revealed an increase in fibrosis by ≥2 stages in 31% of cases and an increase to an overall stage of 2.2 (p = 0.001). No clinical or histological factors were associated with progression of fibrosis. CONCLUSIONS:In this CVID cohort, NRH is the most common initial histological finding; however, unexpectedly fibrosis progresses over time in a subgroup of patients. A better understanding of the underlying causal process of liver disease CVID might lead to improved outcomes.

BACKGROUND:The impact of sinonasal malignancies (SNMs) on quality of life (QOL) at presentation is poorly understood. The Sinonasal Outcome Test (SNOT-22) and University of Washington Quality of Life (UWQOL) are validated QOL instruments with distinctive subdomains. This study aims to identify factors impacting pretreatment QOL in SNM patients to personalize multidisciplinary management and counseling. METHODS:Patients with previously untreated SNMs were prospectively enrolled (2015-2022) in a multicenter observational study. Baseline pretreatment QOL instruments (SNOT-22, UWQOL) were obtained along with demographics, comorbidities, histopathology/staging, tumor involvement, and symptoms. Multivariable regression models identified factors associated with reduced baseline QOL. RESULTS:Among 204 patients, presenting baseline QOL was significantly reduced. Multivariable regression showed worse total SNOT-22 QOL in patients with skull base erosion (p = 0.02). SNOT-rhinologic QOL was worse in women (p = 0.009), patients with epistaxis (p = 0.036), and industrial exposure (p = 0.005). SNOT extranasal QOL was worse in patients with industrial exposure (p = 0.016); worse SNOT ear/facial QOL if perineural invasion (PNI) (p = 0.027). Squamous cell carcinoma pathology (p = 0.037), palate involvement (p = 0.012), and pain (p = 0.017) were associated with worse SNOT sleep QOL scores. SNOT psychological subdomain scores were significantly worse in patients with palate lesions (p = 0.022), skull base erosion (p = 0.025), and T1 staging (p = 0.023). Low QOL was more likely in the presence of PNI on UW health (p = 0.019) and orbital erosion on UW overall (p = 0.03). UW social QOL was worse if palatal involvement (p = 0.023) or PNI (p = 0.005). CONCLUSIONS:Our findings demonstrate a negative impact on baseline QOL in patients with SNMs and suggest sex-specific and symptom-related lower QOL scores, with minimal histopathology association. Anatomical tumor involvement may be more reflective of QOL than T-staging, as orbital and skull base erosion, PNI, and palate lesions are significantly associated with reduced baseline QOL.

The 3D stereoscopic technique consists in providing the illusional perception of depth of a given object using two different images mimicking how the right and left eyes capture the object. Both images are slightly different and when overlapped gives a three-dimensional (3D) experience. Considering the limitations for establishing surgical laboratories and dissections courses in some educational institutions, techniques such as stereoscopy and photogrammetry seem to play an important role in neuroanatomy and neurosurgical education. The aim of this study was to describe how to combine and set up realistic models acquired with photogrammetry scans in 3D stereoscopic projections. Three donors, one dry skull, embalmed brain and head, were scanned using photogrammetry. The software used for displaying the final realistic 3D models (Blender, Amsterdam, the Netherlands) is a free software and allows stereoscopic projection without compromising the interactivity of each model. By default, the model was exported and immediately displayed as a red cyan 3D mode. The 3D projector used in the manuscript required a side-by-side 3D mode which was set up with simple commands on the software. The final stereoscopy projection offered depth perception and a visualization in 360° of each donor; this perception was noted especially when visualizing donors with different cavities and fossae. The combination of 3D techniques is of paramount importance for neuroanatomy education. Stereoscopic projections could provide a valuable tool for neuroanatomy instruction directed at clinical trainees and could be especially useful when access to laboratory-based learning is limited.

Frontal sinus surgery still represents a challenge due to its complex and highly variable anatomy. In this manuscript, we present a detailed anatomical description of an eyebrow approach that allows full exposure of the frontal sinus with a large osteoplastic bone flap and preservation of the supraorbital nerve. Laryngoscope, 134:1633-1637, 2024.

OBJECTIVES/UNASSIGNED:To report implementation and outcomes associated with a novel paid Summer Undergraduate Research Education Program (SREP) over the first 2 years in an academic otolaryngology program recruiting students underrepresented in medicine (URiM). METHODS/UNASSIGNED:A 10-week program including a research bootcamp, curriculum, mentoring, and clinical shadowing was created. Grant funding to provide salary and support for transportation, conference attendance, and graduate school preparation or applications was procured. Primary objectives included (1) development of successful mentorship relationships; (2) increasing student-reported outcomes using pre- and post-program surveys to assess confidence, career planning, and overall satisfaction; (3) increasing exposure to medicine; (4) completion of an oral presentation; and (5) submission of a manuscript. Secondary objectives included abstract submission and completion of a graduate exam course or graduate school applications. Tertiary objectives included conference attendance and graduate school matriculation. RESULTS/UNASSIGNED: < 0.0001). Eight of nine students submitted an abstract to a national conference, with five of eight students accepted for a presentation. Two students were accepted into graduate school, while five others are on track for graduate school application. CONCLUSION/UNASSIGNED:Identifying mentors, curriculum, and opportunities to meaningfully strengthen graduate school applications for URiM students through a clinically rigorous, financially supported, and research-focused summer program in an academic otolaryngology program is feasible and may be an effective means of increasing diversity in medicine and otolaryngology. SUPPLEMENTARY INFORMATION/UNASSIGNED:The online version contains supplementary material available at 10.1007/s40670-024-02021-z.

BACKGROUND/UNASSIGNED:Eagle syndrome (ES) was first described in 1937, to characterize elongation of the styloid process. It is rarely encountered by neurosurgeons but does present the potential for vascular sequelae and neurological complications. DEMONSTRATIVE CASES/UNASSIGNED:We discuss three patients with uncommon presentations of neurovascular compromise with uncommon symptomatology, secondary to ES. Their management ranged from retrospective diagnoses following self-limited events, antiplatelet therapy, and endovascular and surgical interventions. DISCUSSION/UNASSIGNED:While traumatic fractures, chiropractic manipulation, and history of prior neck surgery have been implicated as the etiologies for ES, congenital cases are common. The styloid process intimately interplays with adjacent neurovascular and nervous structures; its elongation can cause symptoms in 10% of patients. CONCLUSION/UNASSIGNED:Awareness of this potentially dangerous but rare disease - more commonly seen by our otolaryngology colleagues - may help reduce diagnostic delays when an elongated styloid process is the cause, as surgery may be required.