The use of imaging to identify immunocompromised children requiring biopsy for invasive fungal rhinosinusitis.

BACKGROUND AND PURPOSE: Children with severe immunocompromise due to cancer therapy or hematopoietic cell transplant are at risk both for potentially lethal invasive fungal rhinosinusitis (IFRS), and for complications associated with gold-standard biopsy diagnosis. We investigated whether early imaging could reliably identify or exclude IFRS in this population, thereby reducing unnecessary biopsy. METHODS: We reviewed clinical/laboratory data and cross-sectional imaging from 31 pediatric patients evaluated for suspicion of IFRS, 19 without (age 11.8 ± 5.4 years) and 12 with proven IFRS (age 11.9 ± 4.6 years). Imaging examinations were graded for mucosal thickening (Lund score), for fungal-specific signs (FSS) of bone destruction, extra-sinus inflammation, and nasal mucosal ulceration. Loss of contrast enhancement (LoCE) was assessed separately where possible. Clinical and imaging findings were compared with parametric or nonparametric tests as appropriate. Diagnostic accuracy was assessed by receiver operating characteristic (ROC) analysis. Positive (+LR) and negative likelihood ratios (-LR) and probabilities were calculated. RESULTS: Ten of 12 patients with IFRS and one of 19 without IFRS had at least one FSS on early imaging (83% sensitive, 95% specific, +LR = 15.83, -LR = 0.18; P < .001). Absolute neutrophil count (ANC) ≤ 200/mm3 was 100% sensitive and 58% specific for IFRS (+LR = 2.38, -LR = 0; P = .001). Facial pain was the only discriminating symptom of IFRS (P < .001). In a symptomatic child with ANC ≤ 200/m3 , the presence of at least one FSS indicated high (79%) probability of IFRS; absence of FSS suggested low (<4%) probability. CONCLUSION: In symptomatic, severely immunocompromised children, the presence or absence of fungal-specific imaging findings may effectively rule in or rule out early IFRS, potentially sparing some patients the risks associated with biopsy.

Anterior Tongue Microglossia: Impact on Face Development.

Microglossia is an extremely rare developmental condition that might impact the patient's respiratory, feeding and speech functions, in addition to other intraoral structures. Embryologically, the tongue has 2 origins, which when affected, will determine whether the patient has microglossia or aglossia. A multidisciplinary team should adopt an organized approach based on confirmation by direct laryngoscopy, determination whether associated airway anomalies, mandibular deformities are present; followed by assessment of the ventilatory and feeding status. The involvement of multiple factors, the presence of several anatomical anomalies and the growth exerted by patients, confer microglossia a rather dynamic clinical entity. Two cases of microglossia depicting these features are presented along with review of the literature and a management algorithm.

Advanced practice providers and children's hospital-based pediatric otolarynology practices.

INTRODUCTION: Advanced practice providers (APPs), including nurse practitioners and physician assistants, have been deployed in children's hospital-based academic pediatric otolaryngology practices for many years. However, this relationship in terms of prevalence, roles, financial consequences and satisfaction has not been examined. The objective of this study is to explore how APPs impact healthcare delivery in this setting. METHODS: Pediatric otolaryngology chiefs of all academic children's hospitals in the US were electronically surveyed about the ways APPs intersected clinically and financially in their respective practice. RESULTS: A total of 29 of 36 children's hospital-based pediatric otolaryngology practices completed the survey, of which 26 practices (90%) utilized APP. There were large variances within the APP practice cohort in faculty size (mean/median/range = 9.4/8.5/3-29); annual patient visits (mean/median = 18,373/17,600); number of practice site (mean/median/range = 4.3/4/2-9) and number of outpatient APP (mean/median/range = 6.3/5/1-30). No factors (faculty size, annual visits and number of practice sites) differentiated between the APP and non-APP practices. Among APP practices, significant correlation (p<.00001) was observed between size of APP cohort to faculty size and annual visits. 69% of the practices did not differentiate job functions of nurse practitioners and physician assistants. 85% of the practices utilized APPs in all practice sites and 19% utilized APPs in the operating room. 77% of APPs billed independently and 46% had on-site supervision. The most prevalent APP salary bracket based on 0-5, 6-10 and > 11 years of tenure were $76-100K (65%), $100-150K (77%) and $100-150K (86%), respectively. In 46% of the practices, APPs were able to generate enough revenue to cover more than 75% of their salary and 23% of practices generated a profit. 81% of the chiefs ranked the effectiveness of APPs as high (4 and 5) on a 5-point Likert scale. DISCUSSION: The majority of academic pediatric otolaryngology practices employed APPs. Despite the diversity seen in practice complexity, APP functionality and financial impact, most found the APP model to be beneficial in improving patient care, patient access and faculty productivity.

Comparison of cidofovir and the measles, mumps, and rubella vaccine in the treatment of recurrent respiratory papillomatosis.

We conducted a retrospective study of the use of cidofovir and the measles, mumps, and rubella (MMR) vaccineas adjunctive treatments to lesion debridement in patients with recurrent respiratory papillomatosis (RRP). Our study population was made up of 15 children-7 boys and 8 girls, aged 1 to 16 years at diagnosis (mean: 6.2)-with pathologically confirmed RRP who had been followed for at least 1 year. In addition to demographic data, we compiled information on disease severity, the type of adjunctive treatment administered to each patient, the frequency of debridements, the length of observation, and remission rates. Of the 15 patients, 5 had been treated with cidofovirafter debridement (cidofovir-only group), 6 were treated with MMR vaccine after debridement (MMR-only group), 3 were treated with one and later switched to the other based on parental preference, and 1 received neither treatment, only debridement. The initial mean Derkay disease severity scores were 12.6 for the cidofovir-only group and 11.0 for the MMR-only group (p = 0.61). The cidofovir-only patients underwent an average of 11.8 adjunctive treatments and the MMR-only patients an average of 17.7 (p = 0.33). The average duration of observation was 44.0 months in the cidofovir-only group and 64.7 months in the MMR-only group (p = 0.29). Remission rates were 20% in the cidofovir-only group and 50% in the MMR-only group (p = 0.54). Our study found insufficient evidence of any significant differences between cidofovir and the MMR vaccinein terms of the number and frequency of adjunctive treatments and the rates of remission.

Retrospective Review of Management and Outcomes of Pediatric Descending Mediastinitis.

OBJECTIVES: To review the management and outcomes of pediatric patients treated for descending mediastinitis at a single institution and contribute to an updated mortality rate. STUDY DESIGN: Case series with chart review. SETTING: Tertiary care pediatric hospital. SUBJECTS AND METHODS: This study is a 19-patient case series of all patients treated for descending mediastinitis at a tertiary pediatric hospital from 1997 to 2015, and it serves as an update to the case series published from this institution in 2008. Review of management included time to diagnosis, time to surgery, surgical procedures performed, and antibiotics administered. The primary outcomes measured were length of hospitalization and mortality. RESULTS: In addition to 8 previously reported patients, we identified 11 pediatric patients treated for descending mediastinitis in the period of review. All 19 patients were <18 months old, and all survived their hospitalization. Fourteen patients underwent surgical drainage at least twice. The median length of hospital stay was 15 days. Retropharyngeal abscess was the source of infection in 16 of 19 patients, and methicillin-resistant Staphylococcus aureus (MRSA) was the isolated organism in 14 of 15 positive cultures. CONCLUSION: This review represents the largest reported series of pediatric patients with descending mediastinitis. With 100% survival, our results suggest that pediatric descending mediastinitis can be safely managed by prompt surgical drainage. Broad-spectrum antibiotics covering MRSA and a low threshold for repeat surgical intervention have been an important part of our successful approach and may decrease length of stay.

Palatopharyngoplasty with bilateral buccal mucosal graft repair to alleviate oropharyngeal stenosis.

Oropharyngeal stenosis is rare, but known complication from tonsillectomy procedure. A 15-year-old female presented with refractory dyspnea, mild obstructive sleep apnea, and dysphagia. She underwent tonsillectomy 3 years prior. Severe cicatricial oropharyngeal scar involving soft palate, anterior tonsillar pillars, and base of tongue, resulted in 1-cm(2) airway. Case report describing lysis of severe palatopharyngeal scar bands from tonsillectomy misadventure with immediate buccal mucosal grafts to repair resultant oropharyngeal defects. Patient no longer complains of difficulty breathing or dysphagia.

Traumatic tracheal injury in children: a case series supporting conservative management.

OBJECTIVES: Injuries to the tracheobronchial region are rare, but have the potential for rapid progression and can become life-threatening. Etiologies of non-penetrating tracheobronchial injuries include blunt cervical trauma, endotracheal intubation, and other iatrogenic causes. Several options for treatment ranging from conservative to surgical exist, but no single treatment has been implemented with consensus. While early surgical repair was once considered the cornerstone of therapy, evidence supporting conservative treatment continues to gain strength. METHODS: All pediatric patients who suffered from non-penetrating injuries to the tracheobronchial tree who were treated by the Otolaryngology Service at a tertiary children's hospital from May 2012 through March 2014 were recorded. A total of 8 patients were identified. The cases were collected from the patients treated by the Otolaryngology Department based on retrospective review. The available electronic medical records were reviewed for each patient. Data including type of injury, endoscopic assessment of injury, treatment received, and follow-up were collected. RESULTS: The ages ranged from 2 to 15 years old, with a mean of 9.25 years old. Six of the eight patients had injuries related to endotracheal intubation. Each patient was taken to the operating suite for diagnostic direct laryngoscopy and bronchoscopy, and treated with initial conservative management. All but one of the patients was treated with endotracheal intubation, and the average length of intubation was 11.71 days. All of the injuries healed spontaneously without requiring initial open surgery. Five patients (62.5%) developed some degree of tracheal stenosis. Three patients (37.5%) required further surgery; one received a tracheostomy and two patients required balloon dilation. CONCLUSIONS: This case series is the largest to date documenting the outcomes of conservative treatment of non-penetrating traumatic tracheal injuries in children. By using initial conservative therapy, we were able to avoid open surgical procedures in many of our patients. We believe that this case series provides further support for conservative management for children with tracheobronchial injuries.

Non-otogenic lateral sinus thrombosis: a complication of acute sphenoid sinusitis.

The consequences of intracranial spread of sinus infection can be dismal. The subtle presentation of sphenoid sinusitis often leads to a delay in diagnosis. The disease may go unrecognized until complications are severe enough to cause more localizing symptoms. Often infections in the head and neck spread into the cranial cavity, leading to a localized effect. For example, otogenic infections can spread to the mastoid or lateral venous sinus. We report a case of sphenoid sinusitis complicated by lateral and sigmoid venous sinus thrombosis.

Methicillin-resistant Staphylococcus aureus as a cause of neonatal suppurative parotitis: a report of two cases and review of the literature.

Suppurative parotitis is an uncommon entity identified in newborns. While Staphylococcus aureus has been frequently identified as the causative pathogen among the few patients diagnosed with neonatal suppurative parotitis (NSP), there has only been one prior case described in the literature that was due to methicillin-resistant Staphylococcus aureus (MRSA). Because of its virulence, MRSA presents new and substantial challenges for the surgeon; we describe two cases of NSP caused by MRSA and the subsequent surgical intervention necessitated for cure. We also include a review of all cases of NSP described in the English-language literature.

Histopathological and postoperative behavioral comparison of rodent oral tongue resection: fiber-enabled CO2 laser versus electrocautery.

OBJECTIVE: To compare operative time and hemostasis of fiber-enabled CO(2) laser (FECL) energy to that of the electrocautery (EC) technique for oral tongue resection, to compare return to oral intake and preoperative weight after FECL and EC resection, and to compare histologic changes in adjacent tissue after FECL and EC resection. STUDY DESIGN: Prospective animal study. SETTING: Research laboratory. SUBJECTS AND METHODS: The CO(2) laser fiber and the Bovie cautery were each used to resect the anterior tongue in 15 adult rats. Fixative perfusion and killing were performed on postoperative day 0 (n = 10), 3 (n = 10), or 7 (n = 10). Body weight, food intake, and water intake were recorded daily for 3- and 7-day survival rats. After preparation for histologic analysis, the tongue tissue was graded with a mucosal wound-healing scale (MWHS). RESULTS: A higher incidence of intraoperative bleeding and shorter operative times were noted in the EC group. No statistically significant difference in postoperative food or water intake between the EC and FECL groups was noted. The FECL group returned to baseline weight by postoperative day 6. MWHS scores were lower in the EC group by postoperative day 3 and lower in the FECL group by postoperative day 7. CONCLUSIONS: Both EC and FECL are effective for resection of the tongue in rats. EC has the advantage of shorter operative time and lower MWHS scores by postoperative day 3; FECL has the advantages of less intraoperative bleeding, faster return to baseline body weight, and lower MWHS score by postoperative day 7.

A fatal presentation of dermatomyositis with facial swelling.

Juvenile dermatomyositis (JDM) is the most common inflammatory autoimmune myopathy in children. Most common presentations consist of heliotrophic rash and/or gottron's papules in addition to proximal muscle weakness. A typical presentations have been reported. We present a 13-year-old African American male who presented with a two-week history of bilateral periorbital edema that was unresponsive to glucocorticoids. He had elevated transaminases but no detectable muscle weakness. A muscle biopsy was consistent with juvenile dermatomyositis. This case highlights the need to consider dermatomyositis in cases of facial swelling and the use of aggressive immunosuppressive therapies due to its associated vasculopathies.

Olaryngologic manifestations in children with chronic neutropenia.

OBJECTIVE: Chronic neutropenia of childhood (CNC) is a rare disorder in which the absolute neutrophil count is below 1500/µL over an extended period of time. The objective of this study is to describe the otolaryngolgic manifestations associated with CNC to facilitate diagnosis and treatment of this condition. METHODS AND MATERIALS: We performed a retrospective chart review of patients with the diagnosis of CNC between 1970 and 2005 at a tertiary pediatric hematology center. After Institutional Review Board approval, 43 patients were evaluated. The average age at hematologic diagnosis was 49 months (range: 1 month - 15 years with 35% <1 year, 44% 1-10 years, 21% >10 years). A total of 2049 encounters were analyzed from the hospital charts. RESULTS: Twenty four subjects (56%) presented with recurrent otitis media (ROM), sinusitis or pharyngo-tonsillitis, while 11% presented with oral mucosal lesions. After diagnosis, otolaryngologic problems persisted, including ROM (81%), viral upper respiratory tract infection (67%), oral ulcers or gingivitis (53%), tonsillitis (39%) and sinusitis (37%) and were more common than other systemic infections. Myringotomy tube placement, endoscopic sinus debridement, adeno-tonsillectomy or tracheostomy were required in 42% of patients. After G-CSF (granulocyte colony-stimulating factor) became available in the early 1990s, the infection rate markedly decreased. Five deaths occurred (12% mortality) including one due to sepsis from otolaryngologic infection. CONCLUSION: The majority of children with CNC had otolaryngologic problems at presentation and these continued after diagnosis. While managing common otolaryngologic infections in children, a high index of suspicion for chronic neutropenia is necessary. An otolaryngologist is frequently one of the first physicians to encounter children with this condition. Awareness of CNC and its management will enhance earlier diagnosis and more effective treatment for these children.

Dilated esophagus and tracheal compression secondary to a slipped Nissen fundoplication: a case report.

We describe the case of a 20-month-old girl with a gastrostomy tube who presented with stridor, daily emesis, stertor, and mild neck retractions. Endoscopic and radiologic investigations revealed a dilated esophagus, an associated tracheal compression, and a paraesophageal hernia secondary to a slipped Nissen fundoplication. The patient underwent a revision fundoplication, and her stridor, stertor, and neck retractions subsided significantly. She tolerated tube feeding without emesis and was discharged home. We recommend a careful evaluation of fundoplication in patients who have undergone the procedure who present with stridor and frequent emesis. Esophageal dilation and associated tracheal compression should be considered in the differential diagnosis, and in such a case, revision of the gastric wrap should alleviate the problem.