Nasopharyngoscopic Evaluation of Velopharyngeal Closure During Speech.

Definitive oronasal separation through closure of the velopharyngeal (VP) sphincter is necessary for the development of normal speech and feeding. Individuals with velopharyngeal incompetence or insufficiency often exhibit hypernasal speech, poor speech intelligibility, and nasal regurgitation. Assessment of VP sphincter function using nasopharyngoscopy is a key element in identifying VP dysfunction. A foundational understanding of normal anatomy and physiology of the velopharyngeal mechanism is paramount to successful diagnosis. This includes recognition of 4 distinct VP sphincter closure patterns: coronal, sagittal, circular, and circular with Passavant's ridge. In this study, the authors showcase 2 patients with velopharyngeal competence who presented to an ear, nose, and throat clinic for nasopharyngoscopic evaluation. This study sought to demonstrate the use of nasopharyngoscopy to recognize velopharyngeal closure patterns and discuss how they may influence the surgical management of VP dysfunction.

The Effect of JAK1/2 Inhibitors on HIV Reservoir Using Primary Lymphoid Cell Model of HIV Latency.

HIV eradication is hindered by the existence of latent HIV reservoirs in CD4+ T cells. Therapeutic strategies targeting latent cells are required to achieve a functional cure, however the study of latently infected cells from HIV infected persons is extremely challenging due to the lack of biomarkers that uniquely characterize them. In this study, the dual reporter virus HIVGKO was used to investigate latency establishment and maintenance in lymphoid-derived CD4+ T cells. Single cell technologies to evaluate protein expression, host gene expression, and HIV transcript expression were integrated to identify and analyze latently infected cells. FDA-approved, JAK1/2 inhibitors were tested in this system as a potential therapeutic strategy to target the latent reservoir. Latent and productively infected tonsillar CD4+ T cells displayed similar activation profiles as measured by expression of CD69, CD25, and HLADR, however latent cells showed higher CXCR5 expression 3 days post-infection. Single cell analysis revealed a small set of genes, including HIST1-related genes and the inflammatory cytokine, IL32, that were upregulated in latent compared to uninfected and productively infected cells suggesting a role for these molecular pathways in persistent HIV infection. In vitro treatment of HIV-infected CD4+ T cells with physiological concentrations of JAK1/2 inhibitors, ruxolitinib and baricitinib, used in clinical settings to target inflammation, reduced latent and productive infection events when added 24 hr after infection and blocked HIV reactivation from latent cells. Our methods using an established model of HIV latency and lymphoid-derived cells shed light on the biology of latency in a crucial anatomical site for HIV persistence and provides key insights about repurposing baricitinib or ruxolitinib to target the HIV reservoir.

Neurofibromatosis type 1 presenting with retinal detachment and laryngeal plexiform neurofibroma in a toddler.

PURPOSE: To present a 22-month-old girl with a complete retinal detachment who was found to have systemic exam findings consistent with neurofibromatosis type 1 during the course of multi-specialty exam under anesthesia. OBSERVATIONS: During examination under anesthesia, ophthalmic exam findings demonstrated retinal detachment with cyst formation, as well as peripheral non-perfusion of the retina in the left eye. Non-ophthalmic findings discovered on difficulty with intubation included a laryngeal plexiform neurofibroma and café-au-lait spots. CONCLUSIONS: Pediatric retinal detachments are uncommon compared to those in adults. Pediatric patients with neurofibromatosis type 1 can present with vision loss as the presenting symptom. Systemic signs and symptoms should be carefully screen and monitored.

First Locally Acquired Congenital Zika Syndrome Case in the United States: Neonatal Clinical Manifestations.

In the spring of 2017, a full-term infant with microcephaly was delivered in South Florida. During first trimester, the mother presented with fever, nausea, and vomiting. She reported no foreign travel for herself or her partner. The infant's neurologic, ophthalmologic, neuroradiologic, and audiologic findings were highly suggestive of congenital Zika syndrome (CZS), confirmed by IgM antibodies and plaque reduction neutralization test. New observations, including peripheral temporal retinal avascularity and peripapillary retinal nerve fiber layer thinning, are presented from this first known case of non-travel-associated CZS in the United States. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:e93-e98.].

A pediatric carotid body tumor.

Carotid body tumors are rare childhood extra-adrenal paragangliomas. We present an 8-year old female with a neck mass mistaken as reactive lymphadenopathy. Computed tomography and magnetic resonance angiography, as well as preoperative embolization and balloon test occlusion, were utilized for planning and management. Surgical excision of the tumor was successful and pathological examination revealed a benign paraganglioma. Surgical treatment is curative for these benign lesions, however rare cases have presented years later with metastatic disease. Therefore, a child, like our patient, will require lifelong surveillance for the development of potential metastasis.

Zika Virus: An Emerging Global Health Threat.

Zika virus (ZIKV) is an emerging healthcare threat. The presence of the mosquito Aedes species across South and Central America in combination with complementary climates have incited an epidemic of locally transmitted cases of ZIKV infection in Brazil. As one of the most significant current public health concerns in the Americas, ZIKV epidemic has been a cause of alarm due to its known and unknown complications. At this point, there has been a clear association between ZIKV infection and severe clinical manifestations in both adults and neonates, including but not limited to neurological deficits such as Guillain-Barré syndrome (GBS) and microcephaly, respectively. The gravity of the fetal anomalies linked to ZIKV vertical transmission from the mother has prompted a discussion on whether to include ZIKV as a formal member of the TORCH [Toxoplasma gondii, other, rubella virus, cytomegalovirus (CMV), and herpes] family of pathogens known to breach placental barriers and cause congenital disease in the fetus. The mechanisms of these complex phenotypes have yet to be fully described. As such, diagnostic tools are limited and no effective modalities are available to treat ZIKV. This article will review the recent advancements in understanding the pathogenesis of ZIKV infection as well as diagnostic tests available to detect the infection. Due to the increase in incidence of ZIKV infections, there is an immediate need to develop new diagnostic tools and novel preventive as well as therapeutic modalities based on understanding the molecular mechanisms underlying the disease.

Predicting revision sinus surgery in allergic fungal and eosinophilic mucin chronic rhinosinusitis.

BACKGROUND: Chronic rhinosinusitis consists of several disease processes. Eosinophilic mucin is found in the subtypes of allergic fungal sinusitis (AFS) and eosinophilic mucin chronic rhinosinusitis (EMCRS). These entities frequently require surgical intervention and have high recurrence rates. OBJECTIVE: We aimed to determine factors in patients with AFS and EMCRS that may be associated with a higher rate of revision surgeries. Our hypothesis is that patients who have polyps, high Lund-Mackay score (LMS), and fungus may have higher revision rates. STUDY DESIGN: Retrospective cohort study. METHODS: This is a retrospective analysis of 117 patients identified over a 5-year period (2005-2009) with the diagnosis of AFS or EMCRS. Contingency tables were created to obtain the odds ratios estimates, and 95% confidence intervals were used to access the association between the outcome (having revision surgery or not) and other clinical binary predictors. RESULTS: Twenty-six of 117 (22%) of the study patients underwent revision surgery. Within the 2-year follow-up period, an additional five of 26 (19%) required another revision surgery. Average LMS was slightly higher in those who underwent revision surgery (16 vs. 13) on a scale of 0 to 24, with an overall mean score of 18 and standard deviation of 6.82 for the whole sample (117). Other factors evaluated were the presence of fungus, polyps, eosinophilic mucin, and the eosinophilic count and medical therapy received. CONCLUSION: The presence of eosinophilic mucin was significantly associated a higher rate of revision surgery. LEVEL OF EVIDENCE: 4. Laryngoscope, 127:59-63, 2017.

Congenital airway anomaly of double aortic arch in a 2-day-old infant.

Double aortic vascular ring is a complete vascular ring that is formed when the distal portion of the right dorsal aorta fails to regress and the ascending aorta bifurcates to surround and compress both trachea and esophagus and rejoins to form the descending aorta.

Rhinosinusitis in the pediatric patient with cystic fibrosis.

Cystic fibrosis (CF) is a common autosomal recessive genetic disorder where a deletion mutation and subsequent downstream alteration in transmembrane regulator proteins results in increased mucus viscosity. CF manifests clinically with chronic multisystem inflammation and recurrent infections. Nearly all children with CF have chronic sinusitis, and a large majority will have concurrent sinonasal polyposis. Chronic sinusitis and sinonasal polyposis in pediatric patients with CF can be managed conservatively initially, though most will fail medical management and require surgical intervention. Unfortunately, symptom resolution is marginal and polyp recurrence rates are high. Currently, no cure exists for CF and the mainstay of treatment is to provide symptomatic relief, and minimize disease morbidity.

Malignant tumors of the parotid gland in children: incidence and outcomes.

BACKGROUND: Population-based analysis of the incidence, demographics, and management outcomes in children with malignant tumors of the parotid gland. METHODS: Surveillance, Epidemiology, and End Results database (1973-2009) was researched for all patients younger than 20 years. RESULTS: Overall, 284 patients were identified. Annual incidence of these tumors was 1.43 cases per million. The highest incidence occurred in girls (0.86/1,000,000), black children (0.849/1,000,000), and adolescents (1.56/1,000,000). Median age at diagnosis was 13.5 years. Most patients were 10 years or older (n = 256, 90%). Most patients presented with local disease (n = 207, 76%). Only 3% had metastasis at time of diagnosis. Most tumors were mucoepidermoid carcinomas (n = 139, 49%) or acinar cell carcinomas (n = 113, 40%). There were no differences in survival between mucoepidermoid and acinar cell carcinomas (96% vs 98% respectively, P = 0.317). Overall mortality was 4.6% over the study period. Overall survival was 96% at 5 years, 95% at 10 years, and 83% at 20 years. Adolescents had significantly higher mortality rates (7.1% vs 1.6% for children <15 years of age, P = 0.23). Multivariate analysis identified the use of adjuvant radiation therapy (hazard ratio, 6.01; 95% confidence interval, 1.15-31.45; P = 0.034) as the only independent predictor of poor outcome. CONCLUSIONS: Malignant parotid gland tumors are most common in adolescents, and this subgroup has worse outcomes. The role of radiotherapy remains controversial.

Endoscopic embolization with onyx prior to resection of JNA: a new approach.

OBJECTIVE: To report a novel pioneering approach of endoscopic embolization (EE) and resection of juvenile nasopharyngeal angiofibroma (JNA) and describe all outcomes and results. METHODS: Four patients presented to the University of Miami with repeated episodes of unilateral epistaxis diagnosed by fiberoptic and radiographic examination as nasal JNA. Subsequently, in conjunction with neurosurgery, endoscopic visualization was provided to perform intratumor needle insertion, through which the liquid embolic agent Onyx was infused to embolize the JNA's under fluoroscopic and endoscopic guidance. The day after EE, endoscopic resection was performed. Operating room time, estimated blood loss (EBL), and other intraoperative and post-operative results are reported and compared to published literature. RESULTS: A total of 4 patients (all males), had EE of JNA and subsequent endoscopic resection between September 2008 and January 2009. Average EBL during surgery was 412.5 ml (range 150-800) with an average operating room time of 228 min (range 95-485). We experienced no bleeding from the tumor or its attachments, only from the approach. Two patients experienced mild numbness in the V2 distribution, which began to resolve one week post-operatively. No other complications were encountered. CONCLUSIONS: This is the first published report of direct endoscopic embolization of JNA with Onyx. Although further studies are needed, it seems to provide a safe, less invasive alternative to traditional embolization and endoscopic resection, but must be done in cooperation with interventional neurosurgery to maximize its safety profile.

Single-stage total endoscopic resection of a plexiform neurofibroma of the maxillary sinus in a child with type 1 neurofibromatosis.

Plexiform neurofibromas are peripheral nerve sheath tumors associated with neurofibromatosis type 1. The maxillary sinus is an extremely rare location of the plexiform neurofibroma and only two adult cases have been previously reported. We report the first case of plexiform neurofibroma of the maxillary sinus occurring in a child with neurofibromatosis type 1. This unusual location presents a management challenge considering the infiltrative nature and the potential malignant degeneration of this type of tumor. MRI is highly valuable to diagnose and plan the surgical approach of the plexiform neurofibroma of the maxillary sinus. Due to the location of the tumor and the patient's age, conservative surgery is highly recommended. We performed an endoscopic total en-bloc resection of the tumor with no recurrence after nine months of follow-up.

Improving the penetration of ototopicals through tympanostomy tubes: role of surfactants.

OBJECTIVE: To evaluate the ability of surfactants to increase the penetration of ototopicals through tympanostomy tubes (TT). METHODS: An in vitro model was used to test the penetration of ototopicals with and without two surfactants (docusate sodium and beractant) through fluoroplastic and titanium TTs. The model was created by placing a TT through a perforation (myringotomy) in a model of the tympanic membrane (silastic sheet) fixed between the ends of two 1 mL syringes. Measurements were recorded of the maximum height various solutions (distilled water, soapy water, ofloxacin otic, ciprofloxacin/dexamethasone otic) achieved before penetrating the TTs (1.27 mm Reuter Bobbin titanium, 1.27 mm fluoroplastic). These same measurements were then performed with the addition of docusate sodium or beractant to the distilled water and each of the ototopical solutions. RESULTS: The addition of docusate sodium significantly increased the penetration of water and ofloxacin otic through both types of TTs. It significantly increased penetration of ciprofloxacin/dexamethasone otic through fluoroplastic tubes with a trend towards increased penetration through titanium tubes. Adding beractant showed a modest trend toward increasing the penetration of both ototopicals in fluoroplastic tubes which did not reach statistical significance. CONCLUSIONS: These results demonstrate that the penetration of ototopicals through TTs can be improved by the addition of surfactants. Increasing the penetration of ototopicals may make them more effective in treating TT otorrhea. However, we strongly discourage clinicians from using these compounds clinically until their safety has been established.

Tonsillectomy and adenoidectomy techniques: past, present and future.

Although the number of procedures has significantly decreased over the past century, tonsillectomy and adenoidectomy are commonly performed surgeries in the pediatric group, with obstructive sleep apnea being the most common indication. Despite the availability of newer technologies, tonsillectomy is still associated with a relatively high risk of postoperative morbidity. Pain and postoperative bleeding can have a significant impact on the child's health and quality of life. This article reviews the currently available techniques for tonsillectomy and adenoidectomy, with their characteristics and potential benefits. No gold standard exists at this point, and further prospective clinical trials are needed in this perspective.

Pediatric obstructive sleep apnea: current management.

Obstructive sleep apnea syndrome (OSAS) is a condition frequently encountered in children, and belongs to a spectrum of diseases called sleep-disordered breathing. Its pathophysiology is related to decreased ventilation despite respiratory effort, leading to episodes of hypopnea and apnea. Left untreated, this condition could have a significant impact on both physical and mental health. Primary snoring is often the presenting symptom reported by parents, and should warrant careful screening for OSAS. Although overnight polysomnography remains the gold standard diagnostic test for OSAS, its feasibility in clinical practice is less obvious, and furthermore, interpretation of its results is not unanimously agreed on. This is why in clinical practice history and physical examination remain among the most useful diagnostic tools. Management of OSAS traditionally includes tonsillectomy and/or adenoidectomy to which most of the cases respond. However, other risk factors and coexisting conditions should be carefully investigated and treated prior to considering surgical treatment.