Search

Objectives  A persistent craniopharyngeal canal (CPC) is a rare embryologic remnant that presents as a well-corticated defect of the midline sphenoid body extending from the sellar floor to the nasopharynx. Our case series aims to describe three unique presentations of this congenital anomaly and their subsequent management. Design  Retrospective review. Setting  Tertiary academic medical center. Participants  Patients who underwent endoscopic transnasal surgical repair of a CPC lesion. Main Outcome Measures  Resolution of symptoms and surgical outcomes. Results  A total of three patients were identified. The clinical presentation varied, however, all cases prompted further imaging which demonstrated a persistent CPC and associated pathologic lesion. The presentation of a persistent CPC with nasal obstruction and subsequent iatrogenic cerebrospinal fluid leak as in Case 1 demonstrates the importance of imaging in this work-up. Cases 2 and 3 in the series were representative of the larger subset of patients in the literature who present with the defect incidentally but still warrant surgical management. Nonetheless, a standard approach to diagnosis with preoperative imaging and subsequent transnasal endoscopic repair of the skull base defect was undertaken. Conclusion  The persistent CPC is a rare congenital anomaly associated with diverse pathology and careful review of preoperative radiology is critical to the management. When warranted, subsequent surgical repair and reconstruction is associated with excellent postoperative outcomes.

Objective: To report health utilization patterns and outcomes of medical and surgical management in patients with chronic suppurative otitis media (CSOM). Study Design: Retrospective cohort. Setting: Academic otology clinic. Methods: This study included 175 patients with CSOM with a first clinic visit at our institution between March 2011 and November 2016. All patients displayed a diagnosis of CSOM by International Classification of Diseases code, had at least 1 episode of active CSOM (defined as perforation with otorrhea), and had a documented history of chronic ear infections. The mean age was 49.5 ± 1.5 years, 53% were female, and mean follow-up time was 3.5 ± 0.3 years. Results: Patients had an average of 9.5 ± 0.5 otology visits, 4.7 ± 0.4 prescriptions, and 1.7 ± 0.1 surgeries, with estimated per patient cost ranging from $3927 to $20,776. Under medical management, 69% of patients displayed recurrence of disease, with a median time to recurrence of 4 months. For tympanoplasty and tympanomastoidectomy, median time to recurrence was similar at 5 and 7 years, respectively (P =.73). At the most recent visit, the prevalence of all patients with CSOM displaying moderate or worse sensorineural hearing loss (SNHL) was 41%. Conclusions: CSOM represents a major public health issue with high health care utilization and associated costs. Surgery is superior to medical therapy for achieving short- to medium-term inactive disease. Patients with CSOM display a high SNHL burden. [ABSTRACT FROM AUTHOR]

The novel coronavirus (SARS-CoV-2) pandemic has influenced the timeliness of care for patients with both common and rare conditions, particularly those affecting high-risk operative sites such as the upper aerodigestive tract. Sinonasal undifferentiated carcinoma (SNUC) represents a rare malignancy of the sinonasal tract, a unique subset of which has never been previously reported in the otolaryngology literature and is characterized by inactivation of the SMARCB (INI-1) tumor suppressor gene. This subtype exhibits a particularly poor prognosis and is characterized pathologically by its rhabdoid appearance. Here we present the case of an individual who was diagnosed with a sinonasal mass during the SARS-CoV-2 pandemic, which was ultimately found to be SMARCB (INI-1)-deficient sinonasal carcinoma. Advanced imaging was deferred in the interest of limiting the patient's exposure to the virus, and expedited operative management was performed which facilitated prompt referral for adjuvant chemoradiation. The SARS-CoV-2 pandemic presents unique challenges, but the work-up of high-risk lesions must be prioritized; this continues to be paramount as SARS-CoV-2 resurges in many cities across the USA. [ABSTRACT FROM AUTHOR]

Objectives/hypothesis: Currently, due to the rarity of pathology, there are limited data surrounding outcomes of pediatric skull base surgery. Traditionally, surgeons have proceeded with caution when electing endonasal endoscopic transsellar/transplanum approaches to the skull base in pediatric patients due to poor sphenoid pneumatization. In this study, we review outcomes of endoscopic pediatric skull base surgery based on sphenoid pneumatization patterns.Study Design: Retrospective chart review.Methods: A review of all cases of pediatric (age < 18 years) craniopharyngioma managed via an endoscopic endonasal approach at a tertiary academic medical center.Results: A total of 27 patients were included in the analysis. The median age was 8 years. Nineteen (70%) patients were male. Presellar, sellar/postsellar, and conchal sphenoid pneumatizations were found in 6, 11, and 10 patients, respectively. There was no significant association between sphenoid pneumatization pattern and extent of resection (gross vs. subtotal, P = .414), postoperative cerebrospinal fluid (CSF) leak (P = .450), intraoperative estimated blood loss (P = .098), total operative time (P = .540), and length of stay (P = .336). On multivariate analysis, after accounting for age, sex, preoperative cranial nerve involvement, and cavernous sinus invasion, there remained no significant association between sphenoid pneumatization pattern and extent of resection (P = .999) and postoperative CSF leak (P = .959).Conclusions: Sphenoid pneumatization pattern does not appear to affect outcomes in endoscopic skull base surgery in the pediatric population. Importantly, lack of sphenoid pneumatization does not impede gross total resection or increase complications. Thorough knowledge of the anatomy during the endoscopic approach is critical to optimize outcomes.Level Of Evidence: 4 Laryngoscope, 129:832-836, 2019. [ABSTRACT FROM AUTHOR]

Biochemical and genetic evidence implicate soluble oligomeric amyloid-β (Aβo) in triggering Alzheimer's disease (AD) pathophysiology. Moreover, constitutive deletion of the A/3o-binding cellular prion protein (PrPc) prevents development of memory deficits in APPswe/PSl ΔE9 mice, a model of familial AD. Here, we define the role of PrPc to rescue or halt established AD endophenotypes in a therapeutic disease-modifying time window after symptom onset. Deletion of Prnp at either 12 or 16 months of age fully reverses hippocampal synapse loss and completely rescues preexisting behavioral deficits by 17 months. In contrast, but consistent with a neuronal function for Aβo/PrPc signaling, plaque density, microgliosis, and astrocytosis are not altered. Degeneration of catecholaminergic neurons remains unchanged by PrPc reduction after disease onset. These results define the potential of targeting PrPc as a disease-modifying therapy for certain AD-related phenotypes after disease onset. [ABSTRACT FROM AUTHOR]

Alzheimer disease (AD) is characterized by amyloid-β accumulation, with soluble oligomers (Aβo) being the most synaptotoxic. However, the multivalent and unstable nature of Aβo limits molecular characterization and hinders research reproducibility. Here, we characterizedmultiple Ao forms through-out the life span of various ADmice and in post-mortemhuman brain. Aβo exists in several populations, where prion protein (PrPC)-interacting Aβo is a highmolecular weight Aβ assembly present in multiple mice and humans with AD. Levels of PrPC-interacting Ao match closely with mouse memory and are equal or superior to other Ameasures in predicting behavioral impairment. However, Aβo metrics vary considerably between mouse strains. Deleting PrPC expression in mice with relatively low PrPC-interacting Aβo (Tg2576) results in partial rescue of cognitive performance as opposed to complete recovery in animals with a high percentage of PrPC-interacting Aβo (APP/PSEN1). These findings highlight the relative contributions and interplay of Aβo forms in AD. [ABSTRACT FROM AUTHOR]