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Fucosidosis is an autosomal recessive lysosomal storage disorder caused by the deficiency of alpha-L-fucosidase. We present the case of an affected female in the second decade of life with chronic rhinosinusitis (CRS) including recalcitrant polypoid inflammation, which has not been previously reported in the literature. With the advancement of life-prolonging measures, children with lysosomal storage disorders may suffer increasingly from CRS due to the lymphohistiocytic and macrophage infiltrate of the paranasal sinus mucosa that resembles severe polypoid inflammation., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Background: The effect of comorbid conditions such as asthma and atopy on the severity of chronic rhinosinusitis (CRS) and the presence of nasal polyps (NPs) remains an area of investigation. We sought to elucidate the relationship among these entities., Methods: The study population included 106 consecutive patients who were referred to a multidisciplinary, university-based allergy and sinus clinic that underwent computed tomography (CT) scan, skin-prick testing, and had CRS. Data were analyzed to determine Lund-MacKay score (LMS), presence of NPs, asthma status, and sensitivity to seven classes of aeroallergens., Results: Skin tests were positive in 52 cases and negative in 54 cases. Although, there was no statistical relationship between LMS and atopic status in the entire group, among the asthmatic subgroup, mean LMS was greater in nonatopic asthmatic patients than in atopic asthmatic patients. Asthmatic patients had a higher LMS than nonasthmatic patients (p < 0.0001). Asthmatic patients were more likely than nonasthmatic patients to have NPs (57.6% versus 25%; p = 0.0015), regardless of atopic status. Mean LMS was higher in NP patients compared with nonpolyp patients (p < 0.0001), independent of atopic status. Mean LMS was not affected by sensitivity to any particular allergen, with the exception of cockroach-allergic patients who were more likely to have an LMS of >10 (p = 0.0236) and had more severe maxillary sinus involvement (p = 0.0391)., Conclusion: These data indicate a strong relationship between CRS severity, as measured by LMS, and chronic airway inflammatory diseases, asthma, and NPs. The association between LMS and atopic status appears weak. The present study suggests that CRS is an inflammatory disease that occurs independently of systemic IgE-mediated pathways.

Purpose of Review: Rhinosinusitis is one of the most common medical complaints, affecting nearly 31 million US citizens annually. Multiple medical professionals including emergency medicine, internal medicine, allergy, and otolaryngology treat rhinosinusitis, resulting in high-practice variability. This review will discuss recommendations of the 2007 American Academy of Otolaryngology - Head and Neck Surgery's multispecialty panel on evidence based clinical practice guidelines for diagnosis and treatment of rhinosinusitis., Recent Findings: The task force for recommendations defines rhinosinusitis as follows: acute bacterial, viral, chronic, or recurrent acute rhinosinusitis. For acute rhinosinusitis three symptoms are required: purulent nasal discharge with nasal obstruction and/or facial pain-pressure-fullness lasting between 10 days and 4 weeks. For viral rhinosinusitis, imaging is not recommended and treatment is symptomatic. For acute bacterial rhinosinusitis in an otherwise healthy patient, symptomatic relief is recommended including pain control. Amoxicillin is the first-line antibiotic of choice if needed. Radiographic imaging should be considered if an infectious complication is suspected. Chronic rhinosinusitis is likely when symptoms persist for greater than 12 weeks, with computed tomography being the gold standard for diagnostic testing. Nasal endoscopy and allergy/immune testing are also considered., Summary: Accurate diagnosis of rhinosinusitis with recommended treatments should standardize management. However, these recommendations are guidelines and the clinician's judgment is highly important.

Background/Objective: Complicated acute rhinosinusitis in the pediatric population is an uncommon problem that may affect the orbit or brain and is life‐threatening. This condition requires surgical intervention with endoscopic sinus surgery for source control, and prior studies have demonstrated the safety of balloon sinuplasty in chronic frontal sinusitis. Methods/Results: We present our approach with a balloon sinus dilation hybrid procedure to resolve four distinct types of complicated acute frontal sinusitis in pediatric patients, including intracranial manifestations, intraorbital complications, and recurrent disease. All four patients were able to be managed operatively with frontal balloon sinuplasty. Conclusions: Prior efficacy has been demonstrated for chronic frontal sinusitis in the pediatric population. We demonstrate that frontal balloon sinuplasty is also feasible in the proper clinical setting for acute frontal sinusitis, even in the presence of regional complications or recurrent disease. Level of Evidence: 4 [ABSTRACT FROM AUTHOR]