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A previously healthy 10-year-old female reported a 1-month history of wheezing and hemoptysis. Initial evaluation and treatment were focused on refractory reactive airway disease and infectious etiologies prompted by her recent travels in Africa. Worsening respiratory distress prompted emergent evaluation with imaging and endoscopy. Bronchoscopy diagnosed a distal tracheal tumor; pathology of this tumor was benign fibrous histiocytoma. Successful management of this condition included imaging, rigid bronchoscopy with biopsy, and tracheal resection to surgically excise the lesion. Although rare, tracheal tumors should be considered when presentation of asthma is atypical and nonresponsive to medical interventions. Laryngoscope, 125:1004–1007, 2015

Rates of patient transfers, cancellations, and patient visits to the emergency department after discharge are quality metrics for ambulatory surgery centers. To improve these metrics, it is imperative to establish best practices for conducting preoperative assessments, including identifying key patient conditions (ie, obstructive sleep apnea, cardiovascular disease, reactive airway disease, obesity). To guide appropriate patient selection, practitioners should review the patient's allergies and sensitivities, alcohol use, medications, and medical history. To help ensure good patient outcomes, it is imperative to provide complete preoperative instructions (eg, NPO guidelines, medications, what to bring, cancellation instructions) and discharge instructions (eg, postoperative medications, appropriate activity restrictions, diet, surgical and anesthetic side effects, special circumstances [eg, regional blocks], symptoms of possible complications, treatment and tests, access to postdischarge follow-up care). Generally, the routine outpatient surgical patient is discharged home; however, there are circumstances that occasionally necessitate transfer or admission to a higher level of care. For transfers, ambulatory surgery centers should adhere to applicable federal and state guidelines and should have a clear policy in place to guide transfers.

SummaryIntroduction Laryngeal cleft is a rare congenital malformation that is being reported with increasing frequency. Diagnosis requires suspension microlaryngoscopy under general anesthesia during spontaneous respiration. Repair may be attempted by a minimally invasive endoscopic approach or open surgical repair. The authors report on their experience with total intravenous anesthesia (TIVA) and spontaneous ventilation without an endotracheal tube during suspension laryngoscopy and CO2 laser application for this specific surgical procedure. Of particular interest were the rate at which this technique failed and rescue techniques were employed and the ability to predict patients in whom this might occur. Methods Between July 2004 and September 2012, 110 endoscopic laryngeal cleft repairs were completed under TIVA with spontaneous ventilation without an endotracheal tube. Anesthetic induction was achieved by inhalation of sevoflurane and oxygen by mask or infusion of propofol at 300 mcg kg−1 min−1 and remifentanil at 0.05–1.0 mcg kg−1 min. The vocal cords and surgical site were sprayed with up to 2 mg kg−1 of 4% lidocaine. If the oxygen saturation decreased during the procedure or the patient became apneic, a rescue process utilizing jet ventilation or intermittent intubation was instituted. Results Ten (9.1%) of the 110 cases required rescue (95% confidence interval [CI]: 5.0–15.8%). The most prevalent comorbidities included reactive airway disease, chronic lung disease, failure to thrive, developmental delay, and an unrelated syndrome. Thirty-nine patients (36%) had reactive airway disease and twelve (11%) had chronic lung disease. Intraoperative complications included six cases requiring a brief, temporary period of intubation (5.5%) and four cases requiring a brief period of jet ventilation (3.6%). Conclusion The technique of TIVA with spontaneous respirations without an endotracheal tube is a safe and effective technique for laryngeal cleft repair. Although the potential for intraoperative adverse events may be high, the actual rate was very low. The need to convert to other techniques is not significant although the children who did require brief periods of jet ventilation or intubation tended to have reactive airway disease or chronic lung disease.

Background Respiratory infections are associated with clinically significant illness in patients with sickle cell disease (SCD). The 2009 H1N1 pandemic was perceived as a significant threat to this population. Methods We undertook a chart review of all patients with SCD followed at our institution to identify those with confirmed H1N1 infection. Further chart and laboratory data was collected on affected patients to analyze clinical courses and the factors that correlated with disease severity. Results Approximately half of the patients with confirmed H1N1 infection were managed successfully on an outpatient basis with oseltamivir therapy. Among the patients admitted, the most common diagnosis was acute chest syndrome (ACS). Most admitted patients had uncomplicated clinical courses, with a median length of admission of 3 days and no mortality or requirement for mechanical ventilation. A past history of ACS or reactive airway disease correlated with a higher rate of admission and of ACS incidence during the acute illness. Chronic transfusion therapy or hydroxyurea therapy with high hemoglobin F levels had a strong inverse correlation with incidence of ACS. Conclusions Our results indicate that that in general the impact of the H1N1 influenza pandemic on patients with SCD was mild but that past clinical history correlated with the severity of illness. Additionally, effective hydroxyurea therapy and chronic transfusion therapy appeared to be protective against the incidence of ACS. Our results suggest guidelines for the management of patients with SCD during future influenza pandemics as well as during seasonal influenza epidemics. Pediatr Blood Cancer 2011; 57: 648–653. © 2011 Wiley-Liss, Inc.

Aim: To evaluate the long-term effect of montelukast on symptoms of cough and wheeze following RSV bronchiolitis. Methods: Fifty eight patients (aged ≤ 24 months) hospitalized with a first episode of RSV bronchiolitis were enrolled in this double blind prospective randomized trial comparing montelukast (n = 31) vs placebo (n = 27). Results: During the 3-month treatment period, there were no statistical significant differences between the two groups for symptom-free days and nights (48.5 [interquartile range 33.0.0–66.0] for montelukast vs 57.0 [29.0–71.0] for placebo p = 0.415) nor disease-free days and nights (44.5 days [26.0–54.0] vs 53.0 [22.3–71.0]; p = 0.266). During the 1 year follow-up, there were 41 exacerbations in the montelukast group vs 54 exacerbations in the placebo group (p = 0.57). Time to first exacerbation was not different. Number of unscheduled visits and need to start inhaled steroids were comparable in the two groups. Conclusion: Treatment with montelukast after hospital admission for RSV bronchiolitis in children younger than 2 years of age did not reduce symptoms of cough and wheeze. We cannot exclude that a subgroup of children may, however, benefit from this treatment.

There is mounting evidence suggesting that infection with respiratory syncytial virus (RSV) in early life increases the risk of developing reactive airway disease (RAD) later in childhood. A recent prospective study demonstrated that children hospitalized with RSV bronchiolitis in infancy face a significantly increased risk of recurrent wheezing and allergy at least until the age of 7 y that is independent of hereditary factors. Proposed mechanisms for this link include immune dysregulation, in which RSV-specific IgE or an imbalance between T-lymphocyte-dependent immune pathways may be involved, and abnormal neural control, in which the non-adrenergic, non-cholinergic pathways are altered by RSV infection. More recent studies suggest that immune and neural mechanisms may be linked and that post-RSV airway inflammation may be explained, at least in part, on the basis of these neuroimmune interactions. Conclusion: Passive immunoprophylaxis may protect against persistent viral-induced inflammation of the respiratory tract, long-term changes in pulmonary function and increased frequency of RAD episodes.

The full expression of Churg-Strauss syndrome (CSS) is characterized by eosinophilia, a necrotizing vasculitis of small to medium blood vessels, and extravascular granuloma formation. The development of CSS typically occurs on a background of asthma, allergic rhinitis, or nasal polyps (1). A number of allergic, infectious, neoplastic, and idiopathic diseases may present with blood as well as tissue eosinophilia, sharing patterns of organ involvement and clinical features that are similar to those of CSS (2). Prompt distinction of CSS from these other entities is essential to the timely institution of appropriate treatment and the prevention of the potentially devastating complications of this disorder: congestive heart failure, severe gastrointestinal ischemia, central nervous system involvement, crippling vasculitic neuropathy (i.e., mononeuritis multiplex), glomerulonephritis, and others (3). In CSS, allergic manifestations and upper respiratory tract symptoms may occur anywhere frommonths to many years before the onset of systemic vasculitis. Empiric glucocorticoid treatment of the atopic features that characterize the disease’s early phase may suppress the emergence of vasculitis, at least temporarily. Conversely, the introduction of more effective inhaled agents for the management of asthma (e.g., the leukotriene inhibitors) is believed to unmask latent CSS in many patients by permitting reductions in the systemic glucocorticoids used to treat reactive airway disease (4). In the absence of pathologic evidence of destructive inflammation within blood vessel walls, distinguishing early CSS from other forms of eosinophilia is challenging. The pathologic diagnosis of CSS has traditionally been linked to the detection of necrotizing vasculitis accompanied by presence of eosinophilic granulomas (1). All of the patients described in 1951 by Churg and Strauss died from their conditions. Consequently, the clinical and pathologic features of the cases described in the original report of the disease were advanced. Moreover, complete postmortem examinations were available on all patients. In the current era, when awareness of CSS is higher, diagnostic testing is more facile, and the availability of effective therapy is widespread, the diagnosis of CSS is more likely to be suspected in the clinic than at postmortem. The authors describe a case of CSS in a patient with long-standing asthma and rhinitis who developed enlarged salivary glands, lymphadenopathy, pulmonary infiltrates, and respiratory distress. Identification of nonvasculitic eosinophilic tissue infiltrates within the salivary glands and skin in the context of the patient’s overall clinical picture led to the diagnosis of CSS at an early stage, the prompt institution of therapy, and a good outcome.

Previously healthy infants less than 6 months of age with severe respiratory syncytial virus bronchiolitis who required hospitalization were identified from hospital records. Infants had been treated either conservatively (control group, n = 19) or with ribavirin added to conservative management (study group, n = 22). All infants underwent a 1-year follow-up after the initial illness. There was a significant reduction in the prevalence of reactive airway disease in the group treated with ribavirin (P < 0.05) compared with the control group, both in terms of the proportion of patients developing airway reactivity (59% vs. 89%) and the number of episodes of reactive airway disease (31 vs. 70). Our data suggest that ribavirin reduces the prevalence of airway reactivity.

Typically patients with recurrent respiratory papillomatosis (RRP) initially manifest laryngeal disease with symptoms of hoarseness, weak cry, and stridor. Extralaryngeal spread is not uncommon, and distal progression may be facilitated by airway epithelial injury, inducing squamous metaplasia. Kashima et al1 found laryngeal papilloma in 97.9% of 417 patients with RRP, but tracheal papilloma only when there was a positive history of tracheostomy. Indeed the diagnosis of RRP may be delayed, as its clinical features often resemble those of more common respiratory disorders.2 We present an 11-year-old boy with no history or evidence of laryngeal papillomata who nevertheless was discovered to have extensive tracheal papillomas.

Endoscopic sinus surgery has been reported to be successful, but lack of a standardized classification system hampers comparison of results between studies, and long-term results of surgery have not been reported in a series of consecutive patients. The results of our first 100 endoscopic sinus surgery procedures, reported previously after an average 9-month follow-up, were reviewed with the application of a new classification scheme and in light of a longer (4-year) follow-up. Surgery was successful in all patients whose sinus symptoms resulted from anatomical variations or suppurative infection, but failed in some patients with hyperplastic disease or polyps. In addition, the presence of reactive airway disease or the acetylsalicylic acid (ASA) triad was a bad prognostic sign. The overall success of the procedure in relieving sinus symptoms decreased from 98% at early follow-up to 91% at 4-year follow-up. Sixty-six percent were successful after one procedure and 25% required more than one procedure to achieve success. The decline in success since our first report in 1990 was mostly attributable to late failure in patients with recurrent symptomatic polyposis. Because symptoms may not recur in these patients for up to 3 years, long-term results of surgery for this disorder are necessary. Symptoms of recurrent polyposis can be controlled medically or by revision surgery.