INI1-Intact Sinonasal Carcinoma with Rhabdoid Features.

Sinonasal malignancies are known for their associated poor prognosis and diversity of histologic features. While poor prognosis is largely due to advanced disease at presentation, histologic features also play a significant role. Therefore, accurate pathologic diagnosis is of utmost importance. Here, we describe a 63-year-old male with chronic left-sided nasal obstruction and left-sided epistaxis who was found to have a large mass occupying most of the nasal cavity extending through the nasopharynx to just below the nasopharyngeal surface of the soft palate. During surgical excision, the mass was noted to originate from the floor of the maxillary sinus with erosion of the medial wall of the maxillary sinus. Pathology revealed a diagnosis of INI1-intact poorly differentiated composite carcinoma with rhabdoid phenotype and sarcomatoid and squamous cell carcinoma foci arising within an inverted papilloma. Included in this report is a detailed description of both the patient's medical course and this pathologically novel sinonasal neoplasm. We aim to elucidate this rare tumor's complex features in order to improve future diagnosis and stimulate prospective research on sinonasal malignancies with complex histology.

A retrospective review of Paparella Type 1 tympanostomy tubes.

OBJECTIVE: This study aims to provide data on ear tube extrusion and complication rates for patients who have Paparella Type 1 tympanostomy tube (TT) placement. METHODS: Retrospective chart review of patients 6 months to 12 years old who underwent insertion of Paparella Type 1 TT by a single surgeon. RESULTS: Of 197 tubes evaluated, 3% were plugged between 1 and 3 months after surgery. Of the 144 tubes evaluated long-term, all tubes extruded within 4 years. There were no tympanic membrane perforations. CONCLUSIONS: This chart review showed expected rate of initial ear tube plugging. The rate of tympanic membrane perforation was lower than expected.

Initial findings of shortwave infrared otoscopy in a pediatric population.

OBJECTIVE: To evaluate the feasibility of Shortwave infrared (SWIR) otoscopy in a pediatric population and establish differences with visible otoscopy. METHODS: Pediatric patients 3 years of age and older seen in the otolaryngology clinic with an audiogram and tympanogram obtained within a week of the visit were recruited for video otoscopy using visible light otoscopy and SWIR otoscopy. Videos were rated by two otolaryngologists based on ability to identify the promontory, ability to identify the ossicular chain and presence or absence of middle ear fluid. RESULTS: A total of 74 video recordings of ears were obtained in 20 patients. We obtained interpretable images in 63/74 (85.1%) ears. There was no statistical significance between ability to perform SWIR otoscopy versus white light video otoscopy as indicated by a p-value of 0.376. There was high inter-rater agreement for identification of both the promontory and the ossicular chain with Kappa values of 0.81 and 0.92 respectively. There was statistical significance between SWIR otoscopy and visible otoscopy in the ability to image the promontory (p = 0.012) and the ossicular chain (p = 0.010). Increased contrast of middle ear fluid was seen in SWIR otoscopy when compared to visible otoscopy. CONCLUSION: SWIR otoscopy is feasible in a pediatric population and could offer some advantages over visible light otoscopy such as better visualization of the middle ear structures through the tympanic membrane and increased contrast for middle ear effusions.

Prevalence of Cochlear-Facial Dehiscence in a Study of 1,020 Temporal Bone Specimens.

OBJECTIVE: To determine the prevalence of cochlear-facial dehiscence (CFD) and to examine the influence of otic capsule area, age, sex, and race on CFD. STUDY DESIGN: Descriptive study of archived temporal bone specimens. MATERIALS AND METHODS: Targeted sections from 1,020 temporal bone specimens were scanned and examined for CFD. Cochlear-facial partition width (CFPW) and otic capsule area (OCA), a marker of bone thickness, were measured using image analysis software. Demographic data were analyzed using multiple linear regression analysis. RESULTS: The mean CFPW was 0.23 mm (range, 0-0.92 mm; SD, 0.15 mm). Six patients were completely dehiscent (0.59%). Fallopian canal width, age, sex, race, and OCA were found to be significant predictors of CFPW. Age was found to be negatively correlated with CFPW (ß = -0.001) (p < 0.005). Thicker CFPW was associated with males (ß = 0.024) and non-Caucasian individuals (ß = 0.031). The mean OCA for dehiscent specimens (mean, 9.48 mm; range, 6.65-11.58 mm; SD 3.21 mm) was significantly smaller than the mean OCA for nondehiscent specimens, (mean, 12.88 mm; range, 6.63-21.92 mm; SD, 2.47 mm) (p < 0.01). CONCLUSION: CFD occurred in nearly 0.6% of specimens in this temporal bone collection. Close to 35% of patients were sufficiently thin (<0.1 mm) to appear dehiscent on computed tomography scanning. Smaller OCA correlated with thinner CFPW, suggesting a developmental factor. Older, female, and Caucasian patients may have a greater risk for CFD and its associated symptoms.

Academic Benchmarks for Otolaryngology Leaders.

OBJECTIVE: This study aimed to characterize current benchmarks for academic otolaryngologists serving in positions of leadership and identify factors potentially associated with promotion to these positions. METHODS: Information regarding chairs (or division chiefs), vice chairs, and residency program directors was obtained from faculty listings and organized by degree(s) obtained, academic rank, fellowship training status, sex, and experience. Research productivity was characterized by (a) successful procurement of active grants from the National Institutes of Health and prior grants from the American Academy of Otolaryngology-Head and Neck Surgery Foundation Centralized Otolaryngology Research Efforts program and (b) scholarly impact, as measured by the h-index. RESULTS: Chairs had the greatest amount of experience (32.4 years) and were the least likely to have multiple degrees, with 75.8% having an MD degree only. Program directors were the most likely to be fellowship trained (84.8%). Women represented 16% of program directors, 3% of chairs, and no vice chairs. Chairs had the highest scholarly impact (as measured by the h-index) and the greatest external grant funding. CONCLUSION: This analysis characterizes the current picture of leadership in academic otolaryngology. Chairs, when compared to their vice chair and program director counterparts, had more experience and greater research impact. Women were poorly represented among all academic leadership positions.

Sinonasal squamous cell carcinoma and the prognostic implications of its histologic variants: a population-based study.

BACKGROUND: Variants of squamous cell carcinoma (SCC) make up 15% of all cases of SCC of the upper aerodigestive tract. There are 5 main histologic variants of SCC in the head and neck region: verrucous (VSCC), papillary (PSCC), spindle cell (sarcomatoid) (SCSC), basaloid (BSCC), and adenosquamous (ASC). Conventional sinonasal SCC has been studied extensively, but far less is known about its major variants. METHODS: The Surveillance, Epidemiology, and End Results (SEER) database was used to extract frequency and survival data from 1973 to 2009. A total of 4382 cases of conventional sinonasal SCC and 328 cases of its major variants were found. Statistical comparisons of data for sinonasal SCC and its variants were carried out with respect to varying demographic and disease specific parameters, such as gender, race, age at diagnosis, and specific anatomic site affected. RESULTS: Sinonasal BSCC was diagnosed at a significantly lower mean age than sinonasal SCC. Sinonasal SCSC significantly affected the maxillary sinus more commonly than SCC. In the setting of advanced stage disease, sinonasal VSCC, PSCC, and BSCC appear to be associated with a better prognosis than conventional sinonasal SCC, whereas the impact of histologic subtype on prognosis in early stage disease appears to be more limited. Survival for SCSC and ASC, both regarded as more lethal variants, was statistically similar to conventional SCC. CONCLUSION: Our study supports the practice of distinguishing between conventional sinonasal SCC and its major histologic variants, because histologic subtype appears to carry important prognostic implications.

p120 Catenin is required for normal tubulogenesis but not epithelial integrity in developing mouse pancreas.

The intracellular protein p120 catenin aids in maintenance of cell-cell adhesion by regulating E-cadherin stability in epithelial cells. In an effort to understand the biology of p120 catenin in pancreas development, we ablated p120 catenin in mouse pancreatic progenitor cells, which resulted in deletion of p120 catenin in all epithelial lineages of the developing mouse pancreas: islet, acinar, centroacinar, and ductal. Loss of p120 catenin resulted in formation of dilated epithelial tubules, expansion of ductal epithelia, loss of acinar cells, and the induction of pancreatic inflammation. Aberrant branching morphogenesis and tubulogenesis were also observed. Throughout development, the phenotype became more severe, ultimately resulting in an abnormal pancreas comprised primarily of duct-like epithelium expressing early progenitor markers. In pancreatic tissue lacking p120 catenin, overall epithelial architecture remained intact; however, actin cytoskeleton organization was disrupted, an observation associated with increased cytoplasmic PKCζ. Although we observed reduced expression of adherens junction proteins E-cadherin, ß-catenin, and α-catenin, p120 catenin family members p0071, ARVCF, and δ-catenin remained present at cell membranes in homozygous p120(f/f) pancreases, potentially providing stability for maintenance of epithelial integrity during development. Adult mice homozygous for deletion of p120 catenin displayed dilated main pancreatic ducts, chronic pancreatitis, acinar to ductal metaplasia (ADM), and mucinous metaplasia that resembles PanIN1a. Taken together, our data demonstrate an essential role for p120 catenin in pancreas development.

Tympanic membrane perforation with squamous epithelial ingrowth.

The squamous epithelium of the tympanic membrane and external auditory canal exhibits an inherent migration pattern to facilitate the exfoliation of keratinizing squamous cells as part of a natural cleansing mechanism.

Transillumination-guided endoscopic endonasal dacryocystorhinostomy: approach to revision cases and challenging anatomy.

Dacryocystorhinostomy (DCR) is a surgical procedure in which a connection is established between the lacrimal sac and the nasal cavity in an effort to bypass an obstruction of the distal lacrimal apparatus. Endoscopic endonasal DCR (EEDCR) is a minimally invasive technique used to achieve this goal. In patients with altered anatomy, EEDCR can be challenging. Here, we describe the use of canalicular transillumination with EEDCR in three cases, and discuss the benefits of this technique.

Endoscopic endonasal resection of sinonasal and anterior skull base schwannomas.

Sinonasal and anterior skull base (ASB) schwannomas are rare entities. The majority of these lesions are found within the sinonasal tract, although some have intracranial extension via invasion of the ASB. Often, these tumors can be confused for other entities, especially olfactory groove meningiomas and esthesioneuroblastomas in the olfactory groove region, and juvenile nasopharyngeal angiofibromas in the infratemporal fossa. We present a single institutional series of four patients with sinonasal and ASB schwannomas that were resected purely via an endoscopic endonasal approach. A retrospective chart review was performed to identify individuals with sinonasal and/or ASB schwannomas. Demographic data, presenting symptoms, imaging, treatment, and follow-up were recorded. Two male and two female patients were included in this study. The average age was 53.5 years (range, 21 to 71). The most common presenting symptoms were facial pain and hyposmia. All tumors were treated with endoscopic endonasal resection. One patient had intradural intracranial extension and required an extended endoscopic endonasal transcribriform approach with ASB resection, while another case involving the infratemporal fossa was treated with an extended endoscopic endonasal transpterygoid approach to this region. There were no major complications at an average follow-up of 9 months (range, 0 to 16). Sinonasal and ASB schwannomas are rare entities, with often nondescript symptoms and can often mimic other types of skull base tumors. Total resection via a purely endoscopic endonasal approach can be considered for these rare neoplasms.

Transporting children with toxicological emergencies.

OBJECTIVE: Each year, the Newborn and Paediatric Emergency Transport Service (NETS) receives over 3600 calls from health professionals regarding the management and transportation of critically ill children across New South Wales, with toxicological emergencies making up 1.5% of these calls. The aim of the present study is to describe the characteristics of patients transported for toxicological emergencies and their retrieval management. METHODS: A retrospective review of patients referred for management of a toxicological emergency between 2007 and 2011. Extracted data included patient demographics, substances involved, consultation with toxicological expertise, interventions performed and major adverse outcomes. RESULTS: Two hundred and thirty patients, with 307 toxicological exposures, were referred to NETS, of whom 169 (73.5%) were subsequently transported. Pharmaceutical poisonings (223, 72.6%) were the most common, followed by non-pharmaceutical poisonings (61, 19.9%) and envenomation (23, 7.5%). Psychotropics, analgesics and chemicals were the most frequently ingested substances. The most common source of accidentally ingested pharmaceuticals was a family member. The most frequently given therapies were specific antidotes, in particular naloxone and N-acetylcysteine. Nearly half (43.2%) of transported children required only non-invasive monitoring. There was one death during the retrieval process. CONCLUSIONS: Many children with toxicological emergencies require only non-invasive monitoring, which could be provided by trained ambulance crews in select scenarios. Involvement of a toxicologist in the initial consultation to identify these patients might reduce retrieval numbers and costs. Children on regular medication and those living with family members on psychotropic or cardiac drugs were identified as high-risk groups that should be targeted for medication safety education.

In the eyes of the law: malpractice litigation in oculoplastic surgery.

PURPOSE: To assess characteristics associated with various outcomes of malpractice litigation, resulting from injuries sustained during oculoplastic procedures. METHODS: The Westlaw legal database (Thomson Reuters, New York, NY, U.S.A.) was used to obtain jury verdicts and settlements. Pertinent data were extracted from 69 malpractice cases litigated from 1988 to 2012 involving oculoplastic procedures, including alleged cause of malpractice, outcome, and defendant specialty. RESULTS: The most commonly litigated surgical procedures were blepharoplasty (63.8% of total) and brow lift surgery (11.6%). The most commonly alleged complications included excessive scarring (24.6%), lagophthalmos (24.6%), visual defects (23.2%), and exposure keratitis (21.7%). Plastic surgeons were the most commonly named defendants (46.4%), followed by both comprehensive ophthalmologists and fellowship-trained ophthalmic plastic surgeons (17.3% each). A defense verdict was held in 60.9% of cases, a plaintiff verdict in 31.9% of cases, and a settlement was reached in 7.2% of cases. Blindness, cranial nerve injury, and the allegation of a permanent deficit increased the likelihood of a case being resolved with payment to the plaintiff (Fisher exact tests, p < 0.05). CONCLUSIONS: Most litigated oculoplastic malpractice cases were resolved in favor of the defendant, while settlements and plaintiff decisions averaged $455,703. Blepharoplasty constituted two-thirds of cases, with the most frequently cited associated complications being unsightly scarring, lagophthalmos, and visual deficits. An alleged lack of informed consent (30.4%) or the need for additional surgery (39.1%) was present in a considerable proportion of cases, emphasizing the importance of a detailed informed consent and clear communication preoperatively regarding patient expectations.

Temporal bone fracture.

Transverse fractures account for approximately 20% of temporal bone fractures. They occur secondary to frontal or occipital head trauma, and they run perpendicular to the petrous pyramid.

Cochlear-facial dehiscence--a newly described entity.

Dehiscence of the cochlear otic capsule has recently been described as a pathologic entity. We describe two cases of cochlear-facial dehiscence, which are the first reported: a 69-year-old male who complained of hearing loss, autophony, and pulsatile tinnitus and a 41-year-old female who complained of left-sided hearing loss, pulsatile tinnitus, and vertigo. In both, computed tomography (CT) showed bony dehiscence between the facial nerve and cochlea. Cochlear-facial dehiscence is another example of otic capsule dehiscence that produces symptoms of third-window lesions. When patients present with symptoms of third-window lesions and CT does not show superior canal dehiscence, cochlear-facial dehiscence should be considered.

Epistaxis: the factors involved in determining medicolegal liability.

BACKGROUND: The purpose of this study was to examine litigation involving epistaxis and analyze factors that determine liability. METHODS: Jury verdicts and settlements regarding cases involving epistaxis were gathered utilizing the Westlaw database. Factors involved in litigation gathered included demographics, defendant specialty, procedure, alleged cause of malpractice, outcome, monetary award, and other variables. RESULTS: A total of 26 cases were analyzed. The majority of cases (57.7%) were decided in favor of the plaintiff or settled out of court. Total awards amounted to $24,501,252. Average awards for cases decided in favor of the plaintiff were $2,260,893 and ranged from $499,845 to $9,022,643. Settlements averaged $1,084,375 and ranged from $300,000 to $3,800,000. Common causes of malpractice encountered included delay in diagnosis, complications from medical procedures, and failure to recognize complications in a timely manner. CONCLUSION: Contrary to previous reports analyzing malpractice for varying medical procedures and complications, litigation in epistaxis is more commonly resolved in favor of the plaintiff or resolved through out-of-court settlements. Substantial financial awards and therapeutic complications from blindness to death make epistaxis a candidate for litigation. Of importance from a medicolegal stand is the fact that 30.8% (8) of the patients involved in epistaxis litigation died, either from complications of therapy or from experiencing epistaxis as a complication of another procedure/pathology. Using necessary diagnostic imaging, ensuring proper management techniques, and recognizing complications in a timely manner can serve to limit legal liability and enhance patient safety.