Resection of NAFLD/NASH-related Hepatocellular Carcinoma (HCC): Clinical Features and Outcomes Compared with HCC Due to Other Etiologies.

BACKGROUND: Non-alcoholic fatty liver disease (NAFLD) and non-alcoholic steatohepatitis (NASH) are the leading causes of hepatocellular carcinoma (HCC) worldwide. Limited data exist on surgical outcomes for NAFLD/NASH-related HCC compared with other HCC etiologies. We evaluated differences in clinicopathological characteristics and outcomes of patients undergoing surgical resection for NAFLD/NASH-associated HCC compared with other HCC etiologies. METHODS: Demographic, clinicopathological features, and survival outcomes of patients with surgically resected HCC were collected. NAFLD activity score (NAS) and fibrosis score were assessed by focused pathologic review in a subset of patients. RESULTS: Among 492 patients screened, 260 met eligibility (NAFLD/NASH [n = 110], and other etiologies [n = 150]). Median age at diagnosis was higher in the NAFLD/NASH HCC cohort compared with the other etiologies cohort (66.7 vs. 63.4 years, respectively, P = .005), with an increased percentage of female patients (36% vs. 18%, P = .001). NAFLD/NASH-related tumors were more commonly >5 cm (66.0% vs. 45%, P = .001). There were no significant differences in rates of lymphovascular or perineural invasion, histologic grade, or serum AFP levels. The NAFLD/NASH cohort had lower rates of background liver fibrosis, lower AST and ALT levels, and higher platelet counts (P < .01 for all). Median overall survival (OS) was numerically shorter in NAFLD/NASH vs other etiology groups, however, not statistically significant. CONCLUSIONS: Patients with NAFLD/NASH-related HCC more commonly lacked liver fibrosis and presented with larger HCCs compared with patients with HCC from other etiologies. No differences were seen in rates of other high-risk features or survival. With the caveat of sample size and retrospective analysis, this supports a similar decision-making approach regarding surgical resection for NAFLD/NASH and other etiology-related HCCs.

Membrane-associated and secreted forms of the Rhesus macaque rhadinovirus-encoded CD200 homologue and cellular CD200 demonstrate differential effects on Rhesus Macaque CD200 Receptor signaling and regulation of myeloid cell activation.

The CD200-CD200R pathway is involved in inhibition of immune responses, and the importance of this pathway to infectious disease is highlighted by the fact that viral CD200 (vCD200) molecules have been found to be encoded by several DNA viruses, including the human gammaherpesvirus Kaposi's sarcoma-associated herpesvirus (KSHV), and the closely related rhesus macaque rhadinovirus (RRV). KSHV vCD200 is the most extensively studied vCD200 molecule, however, the only herpesvirus vCD200 molecule to be examined in vivo is that encoded by RRV. Our prior studies have demonstrated that RRV vCD200 is a functional CD200 homologue that is capable of affecting immune responses in vivo, and further, that RRV can express a secreted form of vCD200 (vCD200-Sec) during infection. Despite this information, RRV vCD200 has not been examined specifically for effects on RM CD200R signaling, and the functionality of vCD200-Sec has not been examined in any context. Thus, we developed an in vitro model system in which B cells expressing vCD200 were utilized to assess the effects of this molecule on the regulation of myeloid cells expressing RM CD200R, mimicking interactions that are predicted to occur in vivo Our findings suggest that RRV vCD200 can bind and induce functional signals through RM CD200R, while vCD200-Sec represents a non-functional protein incapable of affecting CD200R signaling. We also provide the first demonstration of the function of RM CD200, which appears to possess more robust signaling capabilities than RRV vCD200, and also show that KSHV vCD200 does not efficiently induce signaling via RM CD200R.IMPORTANCE Viral CD200 homologues are encoded by KSHV and the closely related RRV. Though RRV vCD200 has been examined, questions still exist in regard to the ability of this molecule to induce signaling via rhesus macaque CD200R, as well as the potential function of a secreted form of vCD200. Further, all previous in vitro studies of RRV vCD200 have utilized an Fc fusion protein to examine functionality, which does not replicate the structural properties of the membrane-associated form of vCD200 that is naturally produced during RRV infection. In this study, we demonstrate for the first time that membrane-expressed RRV vCD200 is capable of inducing signal transduction via RM CD200R, while the secreted form of vCD200 appears to be non-functional. Further, we also demonstrate that RM CD200 induces signaling via RM CD200R, and is more robust than RRV vCD200, while KSHV vCD200 does not appear to induce efficient signaling via RM CD200R.

Community Violence, PTSD, Hopelessness, Substance Use, and Perpetuation of Violence in an Urban Environment.

We investigated the relationships among chronic violence exposure, post-traumatic stress disorder (PTSD) symptom severity, hopelessness, substance use, and perpetuation of violence to facilitate the development of trauma-related interventions for residents of Newark, NJ. A convenience sample of Newark residents (N = 153) was recruited from community centers during various events in 2016-2017. Anonymous, self-report survey measures included a PTSD screen (PCL-C), Beck's Hopelessness Scale, the CAGE questionnaire, and a CDC Health Behavior Scale. Descriptive statistics, Pearson's correlations, Chi square analyses, logistic, and linear regressions were used for analysis. Thirty percent (95% CI [22.7, 37.4]) of our sample screened positive for PTSD. Drug and alcohol use, fighting, and hopelessness were related to severity of PTSD symptoms (p < 0.05). Female gender, CAGE scores, and hopelessness predicted the severity of PTSD symptoms (R2 = 0.354, p < 0.05). Our data has informed the development of a resilience support group currently in the pilot stage for community members.

Interface dermatitis as an indicator of hepatic involvement in drug reaction with eosinophilia and systemic symptoms (DRESS).

BACKGROUND: There are conflicting reports on the association between interface dermatitis and hepatic involvement in DRESS. METHODS: A cross-sectional analysis of the clinical and the histopathologic features of DRESS was performed to study the association between the histopathology of the skin rash and hepatic involvement. RESULTS: The clinical and the histopathologic findings were evaluated in 40 cases of DRESS. Thirty patients (75%) had a hepatic involvement. Thirty (75%) biopsy specimens showed a combination of different inflammatory patterns. The interface dermatitis was noted in 24 specimens (60%). Twenty-one patients with the interface dermatitis had a hepatic involvement (P = .04). CONCLUSIONS: The skin rash of DRESS often shows the coexistence of different inflammatory patterns. The interface dermatitis showed a statistically significant association with the hepatic involvement in DRESS.

Standing Tall: Feasibility of a Modified Ride-On Car That Encourages Standing.

PURPOSE: The purpose of this study was to determine the feasibility of infants with Down syndrome to use a modified ride-on car with seated and standing modes. METHODS: Participants included 4 infants with Down syndrome. Families were asked to provide at least 8 minutes of modified ride-on car driving per day, at least 5 times per week throughout the 9-month intervention. RESULTS AND CONCLUSIONS: Families demonstrated a variety of adherence rates to the intervention. Infants demonstrated independent activation of the modified ride-on car in seated and standing modes and enjoyed driving. The modified ride-on car intervention was feasible and warrants further testing to address barriers that influence adherence to the intervention.

Impact of diabetes on free flap surgery of the head and neck: A NSQIP analysis.

OBJECTIVE: Diabetes is associated with microvascular pathology and may predispose patients undergoing microvascular surgery to complications. This study assesses diabetes as a risk factor for complications following free flap surgery of the head and neck. PATIENTS AND METHODS: In this retrospective cohort study, data on free flap surgeries of the head and neck between 2005 and 2014 was collected from the National Surgical Quality Improvement Program (NSQIP) database. A propensity-matching algorithm (PSM) was used to equilibrate distribution of numerous covariates between the diabetic and nondiabetic cohorts. A sub-analysis was performed to examine the impact of insulin-dependency. RESULTS: The initial dataset contained 2187 free flaps of the head and neck. After implementing PSM, a new population was created containing 506 total cases with 253 DM patients. The majority of cases were male and white. The matched cohort did not contain any demographics or comorbidities associated with DM. Complications significantly elevated in the DM group were severe bleeding (P = .046), postoperative ventilation greater than 48 hours (P < .001), and pneumonia (P < .048). In patients with insulin-dependent diabetes, reintubation (P = .005), cardiac arrest (P = .010), severe bleeding (P = .006), overall surgical complications (P = .015), and overall complications (P = .005) were significantly increased. CONCLUSION: This study examines the impact of diabetes on postoperative complications following free flap reconstruction of the head and neck. Propensity score matching was utilized. Analysis of the PSM cohort suggests that diabetic patients have elevated rates of postoperative pulmonary complications. Additionally, patients with insulin-dependent diabetes have significantly elevated rates of medical and surgical complications.

Prognosis and Clinicopathologic Features of Patients With Advanced Stage Isocitrate Dehydrogenase (IDH) Mutant and IDH Wild-Type Intrahepatic Cholangiocarcinoma.

BACKGROUND: Conflicting data exist regarding the prognostic impact of the isocitrate dehydrogenase (IDH) mutation in intrahepatic cholangiocarcinoma (ICC), and limited data exist in patients with advanced-stage disease. Similarly, the clinical phenotype of patients with advanced IDH mutant (IDHm) ICC has not been characterized. In this study, we report the correlation of IDH mutation status with prognosis and clinicopathologic features in patients with advanced ICC. METHODS: Patients with histologically confirmed advanced ICC who underwent tumor mutational profiling as a routine part of their care between 2009 and 2014 were evaluated. Clinical and pathological data were collected by retrospective chart review for patients with IDHm versus IDH wild-type (IDHwt) ICC. Pretreatment tumor volume was calculated on computed tomography or magnetic resonance imaging. RESULTS: Of the 104 patients with ICC who were evaluated, 30 (28.8%) had an IDH mutation (25.0% IDH1, 3.8% IDH2). The median overall survival did not differ significantly between IDHm and IDHwt patients (15.0 vs. 20.1 months, respectively; p = .17). The pretreatment serum carbohydrate antigen 19-9 (CA19-9) level in IDHm and IDHwt patients was 34.5 and 118.0 U/mL, respectively (p = .04). Age at diagnosis, sex, histologic grade, and pattern of metastasis did not differ significantly by IDH mutation status. CONCLUSION: The IDH mutation was not associated with prognosis in patients with advanced ICC. The clinical phenotypes of advanced IDHm and IDHwt ICC were similar, but patients with IDHm ICC had a lower median serum CA19-9 level at presentation. IMPLICATIONS FOR PRACTICE: Previous studies assessing the prognostic impact of the isocitrate dehydrogenase (IDH) gene mutation in intrahepatic cholangiocarcinoma (ICC) mainly focused on patients with early-stage disease who have undergone resection. These studies offer conflicting results. The target population for clinical trials of IDH inhibitors is patients with unresectable or metastatic disease, and the current study is the first to focus on the prognosis and clinical phenotype of this population and reports on the largest cohort of patients with advanced IDH mutant ICC to date. The finding that the IDH mutation lacks prognostic significance in advanced ICC is preliminary and needs to be confirmed prospectively in a larger study.

Cochlear Implant Device Failures Falling Under the 2020 FDA Voluntary Field Corrective Action: A Systematic Review and Meta-analysis.

OBJECTIVE: To compare the rate of device failure for those cochlear implants (CIs) involved in the 2020 Food and Drug Administration (FDA) voluntary field corrective action (VFCA). DATABASES REVIEWED: Medline, Embase, and Scopus. METHODS: A systematic review was performed according to the PRISMA guidelines. Publications reporting institutional experiences with implants affected by the VFCA were included. Outcomes assessed included etiology of, rate of, and time to failure and pre-/post-device failure speech perception testing. All outcomes reported in at least two independent studies were included in a meta-analysis. RESULTS: Six studies met criteria for analysis. The overall pooled failure rate was 23.7% (95% CI, 11.6-38.4%). The pooled device, inconclusive, and medical failure rates were 21.5%, 0.2%, and 0.7%, respectively. Pediatric failure rates were higher than those of adults (46.9% [95% CI, 11.2-84.5%] versus 32.6% [95% CI, 8.2-63.7%]). WRS declined with primary implant failure (55.1% [95% CI, 48.0-62.1%] to 34.1% [95% CI, 30.2-38.0%]) but improved after reimplantation (34.1% [95% CI, 30.2-38.0%] to 50.1% [95% CI, 45.2-55.1%]). CONCLUSIONS: The rate of pooled reported failure for CIs falling under the 2020 VFCA in the literature thus far is 23.7%. The overwhelming majority of these failures were device related, the rates of which were higher in children. Speech perception improved significantly after reimplantation.

Long-term outcomes following pediatric endoscopic titanium ossiculoplasty: A single-institution experience.

INTRODUCTION: Endoscopic ossicular chain reconstruction (OCR) in adults has demonstrated equivalent outcomes to the traditional microscopic approach. Less data exist on endoscopic OCR outcomes in children, who have unique considerations including a smaller transcanal corridor and variable pathology. The purpose of this study was to investigate surgical and audiometric outcomes in children undergoing fully endoscopic and endoscopic-assisted OCR in both the short and long-term. METHODS: Retrospective review of all children (<17 years) who underwent endoscopic OCR at one tertiary care center between 2017 and 2021. Children undergoing primary and revision endoscopic OCR with either partial (PORP) and total ossicular reconstruction prostheses (TORP) were included. Children undergoing surgery for juvenile otosclerosis or congenital stapes fixation, or any child receiving a stapes prosthesis were excluded. Primary outcome measures were post-operative change in 4 frequency (500 Hz, 1, 2, 4 KHz) air conduction pure tone average (AC PTA) and change in air-bone gap (ABG). Secondary measures included need for readmission and/or revision surgery, complication rate, and surgery duration. RESULTS: Seventeen patients met inclusion criteria. Average age was 11.3 years (range, 5-17 years); 14 were male. A variety of fixed length, titanium total and partial prostheses were used. The most common prosthesis length was 2 mm (range 2-5 mm), and there were no intra- or perioperative complications. Mean long-term follow-up was 2.6 years. Most common pathology was congenital cholesteatoma (11/17, 64%), followed by chronic otitis media with tympanic membrane perforation (5/17, 29.4%), and extruded prosthesis (1/17, 5.9%). Intraoperatively, the most common finding was incus erosion (10/17, 58.8%), followed by malleus erosion (6/17, 35.3%), stapes erosion (4/17, 23.5%), and stapes absence (4/17, 23.5%). Eight children (47%) were reconstructed with PORPs, and 9 children (52.9%) were reconstructed with TORPs. Average ABG improved from 36.8 dB preoperatively to 19.9 dB postoperatively in the short-term and remained stable at 19.5 dB in the long-term. Average short-term ABG improvement was 4.2 dB for PORPs and 18 dB for TORPs. In the long-term, average ABG improved by 2.3 dB in PORPs and 13.4 dB in TORPs. PORPs had higher rates of ABG closure and lower AC PTAs than TORPs in the long-term. DISCUSSION: Endoscopic ossiculoplasty is a viable option in children presenting with ossicular erosion from various causes. Audiometric improvement following endoscopic partial and total ossicular reconstruction remains stable over time, with a preference towards partial in the long-term, and mirrors published outcomes for microscopic surgery.

Use of Soft Cervical Collar Improves Surgeon Ergonomics During Simulated Otologic Surgery.

OBJECTIVE: To determine whether surgeon use of a soft cervical collar during endoscopic and microscopic otologic surgery is feasible and impacts surgeon ergonomics as measured by inertial sensors. STUDY DESIGN: Prospective crossover trial. SETTING: US-based otolaryngology training program. PATIENTS: Otolaryngology residents and fellows. INTERVENTIONS: Therapeutic-use of a soft cervical collar during simulated otologic surgery. MAIN OUTCOME MEASURES: Time spent in high-risk angles of neck and back flexion and extension; average angle of neck flexion, extension, rotation, and lateral bending; validated assessment of neck pain; average daily phone use. RESULTS: Fifteen subjects met criteria for inclusion. Ten of 15 (67%) were male. Seven of 15 (47%) were postgraduate year 1-2. Seven of 15 (47%) reported a history of neck pain. None reported prior spinal steroid injections or surgery. Across all subjects, use of the soft cervical collar significantly reduced time spent in high-risk angles of neck flexion/extension during both endoscopic (56% vs. 35%, p < 0.05) and microscopic (60% vs. 32%, p < 0.05) otologic surgery. There was no effect on back flexion or extension. There was no difference in time spent in high-risk neck or back angles between endoscopic and microscopic surgery. Average angles of neck or back flexion, extension, lateral bending, and rotation were not significantly different for subgroups with more operative experience, increased phone use, perception of good posture, or history of neck pain. CONCLUSIONS: Use of a soft cervical collar during simulated otologic surgery significantly reduced time spent in high-risk neck positions. These data support feasibility of soft collar use during otologic surgery and hold promise for reduction in the high rates of neck pain reported by neurotologists. PROFESSIONAL PRACTICE GAP AND EDUCATIONAL NEED: Improving surgeon ergonomics for otologic surgery. LEARNING OBJECTIVE: To identify a therapeutic intervention to mitigate neck pain in surgeons caused by assumption of high-risk cervical neck flexion and extension. DESIRED RESULT: To demonstrate that use of a readily available soft cervical collar reduces risk of neck pain in otologic surgeons. LEVEL OF EVIDENCE: II. INDICATE IRB OR IACUC: Exempt.

Multivalent MVA-vectored vaccine elicits EBV neutralizing antibodies in rhesus macaques that reduce EBV infection in humanized mice.

Introduction: Epstein-Barr virus (EBV) is an oncogenic human herpesvirus associated with ~350,000 cases of lymphoid and epithelial malignancies every year, and is etiologically linked to infectious mononucleosis and multiple sclerosis. Despite four decades of research, no EBV vaccine candidate has yet reached licensure. Most previous vaccine attempts focused on a single viral entry glycoprotein, gp350, but recent data from clinical and pre-clinical studies, and the elucidation of viral entry mechanisms, support the inclusion of multiple entry glycoproteins in EBV vaccine design. Methods: Here we generated a modified vaccinia Ankara (MVA)-vectored EBV vaccine, MVA-EBV5-2, that targets five EBV entry glycoproteins, gp350, gB, and the gp42gHgL complex. We characterized the genetic and translational stability of the vaccine, followed by immunogenicity assessment in BALB/c mice and rhesus lymphocryptovirus-negative rhesus macaques as compared to a gp350-based MVA vaccine. Finally, we assessed the efficacy of MVA-EBV5-2-immune rhesus serum at preventing EBV infection in human CD34+ hematopoietic stem cell-reconstituted NSG mice, under two EBV challenge doses. Results: The MVA-EBV5-2 vaccine was genetically and translationally stable over 10 viral passages as shown by genetic and protein expression analysis, and when administered to female and male BALB/c mice, elicited serum EBV-specific IgG of both IgG1 and IgG2a subtypes with neutralizing activity in vitro. In Raji B cells, this neutralizing activity outperformed that of serum from mice immunized with a monovalent MVA-vectored gp350 vaccine. Similarly, MVA-EBV5-2 elicited EBV-specific IgG in rhesus macaques that were detected in both serum and saliva of immunized animals, with serum antibodies demonstrating neutralizing activity in vitro that outperformed serum from MVA-gp350-immunized macaques. Finally, pre-treatment with serum from MVA-EBV5-2-immunized macaques resulted in fewer EBV-infected mice in the two challenge experiments than pretreatment with serum from pre-immune macaques or macaques immunized with the monovalent gp350-based vaccine. Discussion: These results support the inclusion of multiple entry glycoproteins in EBV vaccine design and position our vaccine as a strong candidate for clinical translation.

Hypopigmented Skin Lesions with Doubtful/Minimal Sensory Impairment: A Histopathology-Based Analysis.

Background: Cardinal criteria proposed by the World Health Organisation (WHO) lack sensitivity to diagnose indeterminate leprosy. Aims: To estimate the frequency of hypopigmented skin lesions with doubtful/minimal sensory impairment showing histopathology features of indeterminate leprosy. To compare between the histopathology findings noted in specimens showing features suggestive of indeterminate leprosy and those showing a non-specific dermatitis pattern. Materials and Methods: Data on patients who attended our department with hypopigmented patches with doubtful/minimal sensory impairment from January 2018 to December 2019 and who underwent a skin biopsy were collected. A pathologist blinded to the clinical findings reviewed the histopathology specimens using a pre-set questionnaire. Results: We studied sixteen biopsy specimens from 14 patients. Eight specimens (50%) showed histopathology suggestive of indeterminate leprosy and the remaining eight showed a non-specific dermatitis pattern. A higher percentage of patients with indeterminate pattern showed mast cells (87.5% vs 25%) and fibrosis around nerve twig or sweat duct (75% vs 12.5%) when compared to those who showed a non-specific dermatitis pattern. Limitations: Small sample size and retrospective study design were the limitations. Conclusions: We found histopathology features of indeterminate leprosy in 50% of the skin biopsy specimens from hypopigmented lesions with doubtful/minimal sensory impairment. The present study highlights the need to improve the diagnostic definition of indeterminate leprosy.

Supratentorial and cerebellar liponeurocytomas: report of four cases with review of literature.

Liponeurocytoma is not exclusive to the cerebellar or fourth ventricular location. Since its inclusion in the central nervous system tumor classification in 2000, six cases with similar radiological, histomorphological and immunohistochemical features have also been described in the lateral ventricles. In the present study, we report clinical, radiological and pathological findings of three supratentorial and one cerebellar liponeurocytoma from our records, evaluated with an extensive panel of immunohistochemistry, and review published cases in the literature. The immunohistochemical pattern of supratentorial and infratentorial liponeurocytomas are almost identical, which indicates that these tumors are homologous.

Post-Tonsillectomy Bleeding: A National Perspective.

OBJECTIVES: Tonsillectomy is one of the most common otolaryngologic procedures. Hemorrhage after tonsillectomy is one of the most feared complications by surgeons and patients alike. The objective of this study was to analyze the risk factors associated with an increase in post-tonsillectomy hemorrhage in the pediatric population using a large national database. METHODS: The Kids' Inpatient Database (KID) from 2000 to 2012 was queried for patients between the ages of 1 to 17 years with post-tonsillectomy hemorrhage. Univariate and multivariate analyses were performed to obtain trends in patient demographics, comorbidities, and hospital information. RESULTS: Overall 45 940 pediatric cases of tonsillectomy were identified of which 5470 (11.9%) resulted in postoperative hemorrhage. Patients with postoperative hemorrhage were significantly older with mean age of 8.62 (±4.75) compared to all patients. Females were found to have higher rates of hemorrhage when compared to males 14.6% versus 11.5% (P < .001). The rate of white patients who had hemorrhage was 16.3% (P < .001). The most common comorbidities significantly associated with hemorrhage were fluid and electrolyte disorders (6.6% P < .001), deficiency anemia (3.8%; P < .001), and coagulopathy (2.7%; P < .001). Urban nonteaching hospitals had higher rates of hemorrhage (19.6%; P < .001) and the Northeast was the most common region (15.2%; P < .001). On multivariate analysis, older patients and white ethnicity showed a significantly increased risk of hemorrhage. Patients with pre-existing comorbidities such as coagulopathy, deficiency anemia, and fluid and electrolyte disorders circulation were also found to have higher odds ratios of bleeding. CONCLUSION: Postoperative hemorrhage is a common complication following tonsillectomy with a rate of 11.9% in this study. A higher rate of hemorrhage was seen with increasing age, white race, and males overall, as well as patients with fluid and electrolyte disorders. Urban non-teaching hospitals and the Northeast region also saw increased rates of hemorrhage.

Displacement of a Total Ossicular Replacement Prosthesis Following Ossicular Chain Reconstruction.

OBJECTIVE: Herein we describe the diagnosis and management of total ossicular replacement prosthesis (TORP) displacement following tympanoplasty with ossicular chain reconstruction (OCR). METHODS: Case report with literature review. RESULTS/CASE: A 40-year-old male with otorrhea and tympanic membrane perforation underwent a right revision tympanoplasty with OCR using a TORP with a tragal chondroperichondrial graft. On postoperative day (POD) 4, he developed vertigo and profound right-sided hearing loss. Temporal bone computed tomography showed the prosthesis in the vestibule. After a steroid taper with mild improvement in symptoms, the TORP was removed two weeks later and the patient continued to improve. CONCLUSION: TORP displacement into the vestibule is a very rare complication following OCR. Conservative management with high dose steroids may improve symptoms, however further middle ear exploration and surgical management may be warranted depending on the depth of displacement into the inner ear as well as symptom severity.

Unusual location of a second branchial cleft cyst presenting in the suprasternal notch.

BACKGROUND: Second branchial cleft cysts (SBCCs) are congenital benign tumors that comprise up to 90% of all branchial cleft anomalies. SBCCs typically present in the lateral neck along the anterior border of the upper third of the sternocleidomastoid muscle. We describe a case of a SBCC presenting in an unusual location in the lower neck close to midline. METHODS: An 18-year-old male presented with a 2-year history of a neck mass in the suprasternal notch. Imaging findings were reviewed with a head and neck radiologist who felt that the findings were highly suggestive of a fourth branchial cleft cyst. RESULTS: The patient underwent surgical excision of the mass. Final pathologic evaluation confirmed the diagnosis of a second branchial cleft cyst. CONCLUSIONS: Though extremely uncommon, second branchial cleft cysts can extend to the suprasternal notch and should not be excluded from the differential diagnoses of lower neck masses.

Intraoperative navigation during atresiaplasty for congenital aural atresia.

OBJECTIVE: The purpose of this study was to evaluate the feasibility of intraoperative navigation (ION) using adhesive fiducials and high-resolution computed tomography (HRCT) of the temporal bone in pediatric patients undergoing atresiaplasty for congenital aural atresia (CAA). METHODS: From June 2018 to August 2019, a retrospective review was performed on pediatric patients with unilateral or bilateral CAA who underwent atresiaplasty with or without concurrent bone anchored implant (BAI) placement. Single stage atresiaplasty was performed at a tertiary referral center with ION linked to image-guidance compatible HRCT non-contrast temporal bone images. Up to six adhesive fiducials were placed for navigation. Patient demographics were collected including Schuknecht classification type and Jahrsdoerfer score. Main outcome measures included the number of useable adhesive fiducials for navigation, navigation system registration accuracy, operative time, intraoperative complications, and postoperative outcomes including facial nerve function, surgical site infection rate, restenosis rate, and audiometric data. RESULTS: Five patients (3 male, 1 AU atresia, 2 AS atresia, all Schuknecht type C) with an average age of 9.2 years (range 6.8-11.8 years) underwent single sided atresiaplasty with ION. Two patients underwent concurrent BAI placement. Average Jahrsdoerfer score was 8.6 (range 7-10). Preoperative audiogram demonstrated a mean air-bone gap (ABG) of 45 dB (range 35-54 dB). The navigation system registration accuracy ranged from 0.08 to 1.80 mm (mean 1.00 mm). Mean operative time was 268 min (range 217-307 min). There were no intraoperative complications. At an average follow up of 7.2 months (range 2-15 months), postoperative facial nerve function was normal in all patients and no patients developed postoperative stenosis or surgical site infections. Four patients underwent postoperative audiogram at an average of 4 months after surgery (range 2-5 months) and all demonstrated an ABG of less than 30 dB (mean 20 dB, range 15-26 dB) with an average improvement in ABG of 23 dB (range 11-39 dB). The one patient who did not undergo postoperative pure tone audiometry underwent AzBio speech perception testing and demonstrated an improvement from 81% to 89%. CONCLUSIONS: The use of ION in otologic surgery is uncommon. Inherent aberrant temporal bone anatomy in CAA makes this a unique population to study the value of this technology. The use of adhesive fiducials is feasible, with navigation registration accuracy and surgical outcomes comparable to those in the literature. More data is necessary regarding the impact of ION on long-term surgical and audiometric outcomes.

Rare case of metastatic liposarcoma to the thyroid gland and a review of the literature of metastatic sarcomas to the thyroid.

BACKGROUND: Liposarcomas are the second most common type of soft tissue sarcomas. Typically, myxoid liposarcomas have a metastatic rate of 10%, usually involving the retroperitoneal space, abdomen, and spine. Metastasis to the thyroid is extremely rare. DESIGN/METHOD: A 62-year-old male with a history of metastatic myxoid liposarcoma in his right thigh presented to our clinic for evaluation of a thyroid nodule incidentally identified on a CT scan. A subsequent FNA biopsy was suggestive of a metastatic liposarcoma. RESULTS: The patient underwent a left thyroid lobectomy and final pathology confirmed a grade II/III metastatic myxoid liposarcoma that measured 3.3 cm. The patient tolerated the procedure well. CONCLUSIONS: Our case highlights the role of a patient's medical history when evaluating thyroid nodules to optimize accurate diagnosis, as liposarcomas do not typically metastasize to the thyroid. We also provide an updated review of the literature on all cases of metastatic sarcomas to the thyroid.

Myelin-specific T cells in animals with Japanese macaque encephalomyelitis.

OBJECTIVE: To determine whether animals with Japanese macaque encephalomyelitis (JME), a spontaneous demyelinating disease similar to multiple sclerosis (MS), harbor myelin-specific T cells in their central nervous system (CNS) and periphery. METHODS: Mononuclear cells (MNCs) from CNS lesions, cervical lymph nodes (LNs) and peripheral blood of Japanese macaques (JMs) with JME, and cervical LN and blood MNCs from healthy controls or animals with non-JME conditions were analyzed for the presence of myelin-specific T cells and changes in interleukin 17 (IL-17) and interferon gamma (IFNγ) expression. RESULTS: Demyelinating JME lesions contained CD4+ T cells and CD8+ T cells specific to myelin oligodendrocyte glycoprotein (MOG), myelin basic protein (MBP), and/or proteolipid protein (PLP). CD8+ T-cell responses were absent in JME peripheral blood, and in age- and sex-matched controls. However, CD4+ Th1 and Th17 responses were detected in JME peripheral blood versus controls. Cervical LN MNCs from eight of nine JME animals had CD3+ T cells specific for MOG, MBP, and PLP that were not detected in controls. Mapping myelin epitopes revealed a heterogeneity in responses among JME animals. Comparison of myelin antigen sequences with those of JM rhadinovirus (JMRV), which is found in JME lesions, identified six viral open reading frames (ORFs) with similarities to myelin antigen sequences. Overlapping peptides to these JMRV ORFs did not induce IFNγ responses. INTERPRETATIONS: JME possesses an immune-mediated component that involves both CD4+ and CD8+ T cells specific for myelin antigens. JME may shed new light on inflammatory demyelinating disease pathogenesis linked to gamma-herpesvirus infection.

Role of Histopathology in Predicting type 1 Lepra Reaction in Borderline Tuberculoid Leprosy.

CONTEXT: Lepra reactions if not managed promptly are an important cause of sudden onset nerve palsy and disability due to leprosy. AIM: To evaluate the usefulness of histology in predicting type 1 lepra reaction. SETTING AND DESIGN: After obtaining clearance from institutional research and ethics committees, all histologically proven borderline tuberculoid patients diagnosed at our center from 1.8.2016 to 31.7.2018 were included in this retrospective cross-sectional study. METHOD: Clinical details were collected from patient records. The pathologist who was blinded to clinical evidence of type 1 lepra reaction at the time of biopsy re-evaluated the histopathology slides for evidence of type 1 reaction. The data of individual patient was analyzed to identify those who had a type 1 reaction at the time of the biopsy or who developed a lepra reaction during follow up. STATISTICAL ANALYSIS USED: Association between histological evidence of type 1 reaction and clinical manifestation of the same subsequently, was assessed using Pearson's Chi square test. RESULTS: Study group comprised of 22 females and 18 males. Clinicohistological concordance was noted in 27 patients (67.5%). Subclinical type 1 reaction was documented in 11 patients (27.5%) based on histopathology evaluation. Five (45.5%) of these 11 patients subsequently developed clinical features of type 1 reaction. This was found to be statistically significant (P value 0.02). LIMITATIONS: Main limitation was the small sample size. CONCLUSIONS: Histology could serve as a useful tool in predicting future type 1 lepra reaction.