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.

Recurrent stridor in a 9-year-old child after a choking and aspiration event.

This is a case report of a 9-year-old boy with new onset stridor 5 days after a choking event. Symptoms would last 5-45 minutes. His stridor was unresponsive to nebulized epinephrine but improved when he relaxed. Otlaryngology examination noted laryngeal irritation that was suggestive of gastroesophageal reflux (GER). Episodic stridor continued, despite treatment for GER, prompting hospitalization. On admission, barium swallow indicated hyperinflation of the left lung and bronchoscopy confirmed the aspiration of food. Within 12 hours of bronchoscopy, his stridor recurred. The recurrence of stridor after bronchoscopy resulted in further evaluation of his upper airway disorder. The true diagnosis was revealed during methacholine challenge. This case illustrates a unique presentation of a common upper respiratory disorder, the need for a high index of suspicion to make the diagnosis, and the importance of the multispecialty approach needed to treat patients with this disorder.

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.

Management of postoperative swallowing dysfunction after ependymoma surgery.

PURPOSE: This study aims to share a new postoperative care technique team approach for the management of children after they have recovered from the anesthetic and has passed through the immediate postoperative period of a day or so from their posterior fossa ependymoma surgery. METHODS: A team approach was developed consisting of a neurosurgeon, an otolaryngologist, an intensivist, and a speech pathologist/swallowing specialist. Patients were extubated 24 h after their surgery. Vocal cord function was assessed by fiberoptic exam after extubation. Tracheostomy was occasionally necessary to secure the airway. Swallowing was assessed via modified barium swallow. Aspiration with feeding was occasionally detected early and managed with a gastrostomy tube. RESULTS: Forty-five patients have undergone posterior fossa surgery at our institution and were managed by our team. Nine have had sufficient vocal cord dysfunction to require a tracheostomy. Eleven have required a gastrostomy. None developed respiratory distress and none developed aspiration pneumonia. CONCLUSIONS: A team approach, delayed airway evaluation, and modified swallowing exams have benefited our patients after posterior fossa ependymoma surgery. We have prevented any cases of respiratory distress or aspiration pneumonia.

Management of suprastomal tracheal fibroma: introduction of a new technique and comparison with other techniques.

OBJECTIVE: Suprastomal tracheal granuloma/fibroma (SSTGF) is a common cause of failure to decannulate following pediatric tracheostomy. Because larger lesions obstruct the trachea, it is necessary to remove them prior to decannulation. Various methods have been described for the management of these obstructing tracheal lesions, including the KTP laser, Nd-YAG laser, sphenoid punch, optical forceps, microsuspension laryngoscopy with an articulated arm (MSLAA), and external excision. A hollow core guide fiber for the CO(2) laser has been developed that can be advanced to better approximate targeted tissues and minimize thermal spread using a near-contact method. METHODS: A retrospective chart review was performed of 30 children under the age of 7 years (21M, 9F) with SSTGF who underwent treatment by either external excision (n=10), MSLAA (n=10), or CO(2) laser vaporization by fiberoptic laser carrier (n=10). The medical charts were reviewed for excision techniques and outcomes. RESULTS: Mean operative time for external excision was 34.9min (SD=10.2min), for MSLAA was 16.3min (SD=4.8min), and for fiberoptic CO(2) laser carrier was 19.3min (SD=7.1min). Mean hospital time postoperatively for external excision was 24h (SD=510min), for MSLAA was 3.3h (SD=37.7min), and for fiberoptic CO(2) laser carrier was 3.9h (SD=46.3min). Need for additional procedures was seen in 60% of external excision procedures, 70% of MSLAA procedures, and in 30% of fiberoptic CO(2) laser carrier procedures. Immediate postoperative decannulation was possible in 10% of the external excision group, 20% of the MSLAA group, and 40% of the fiberoptic CO(2) laser carrier group. CONCLUSIONS: The new technique of using a fiberoptic carrier for the CO(2) laser to treat children with SSTGF's is comparable to more traditional techniques of SSTGF removal when considering the need for additional procedures, postoperative hospital stay, and percentage of immediate postoperative decannulation and provides another useful tool in the armamentarium of the surgeon in treating SSTGF's.

Deep neck infection.

Deep neck infections present significant morbidity and mortality, particularly when associated with predisposing factors that impair a functional immunologic response. Familiarity with deep neck spaces and fascial planes is critical, because these form the basis for the emergent nature of the disease process. Common and potentially life-threatening complications include airway obstruction, jugular vein thrombosis, descending mediastinitis, sepsis, acute respiratory distress syndrome, and disseminated intravascular coagulation. The most common primary sources of deep neck infection are odontogenic, tonsillar, salivary gland, foreign body, and malignancy. Microbiology typically reveals mixed bacterial flora, including anaerobic species, that can rapidly progress to a fulminating necrotizing fasciitis. The treatment cornerstone remains securing the airway, providing efficient drainage and appropriate antibiotics, and improving immunologic status. A prolonged hospital stay should be anticipated.

Health care reform: pediatric elective surgery payer mix and utilization under Tenncare managed competition.

UNLABELLED: Managed care decreases reimbursement for surgical referral care and may decrease access for elective procedures. This study seeks to determine the impact of Medicaid managed competition on elective pediatric otolaryngology surgery. DESIGN AND SETTING: Payer mix was analyzed for tonsillectomy and adenoidectomy (T&A) and bilateral myringotomy with ventilation tube insertion (BM&VT) charges for an eight-year period surrounding TennCare implementation. The payer mix for hospital gross charges was analyzed for the same period as a control. RESULTS: After TennCare implementation, hospital gross charges shifted toward increased TennCare/Medicaid and decreased commercial insurance, whereas charges for the two elective procedures shifted toward increased commercial insurance and decreased TennCare/Medicaid. CONCLUSION: Otolaryngologists avoided impending losses under TennCare through indirect cost shifting. Numbers of T&A and BM&VT procedures performed on Tenncare/Medicaid patients remained constant, while numbers of these procedures performed on commercially-insured patients increased.

Health care reform: pediatric elective surgery payer mix and utilization under TennCare managed competition.

UNLABELLED: Managed care decreases reimbursement for surgical referral care and may decrease access for elective procedures. This study seeks to determine the impact of Medicaid managed competition on elective pediatric otolaryngology surgery. DESIGN AND SETTING: Payer mix was analyzed for tonsillectomy and adenoidectomy (T&A) and bilateral myringotomy with ventilation tube insertion (BM&VT) charges for an eight-year period surrounding TennCare implementation. The payer mix for hospital gross charges was analyzed for the same period as a control. RESULTS: After TennCare implementation, hospital gross charges shifted toward increased TennCare/Medicaid and decreased commercial insurance, whereas charges for the two elective procedures shifted toward increased commercial insurance and decreased TennCare/Medicaid. CONCLUSION: Otolaryngologists avoided impending losses under TennCare through indirect cost shifting. Numbers of T&A and BM&VT procedures performed on Tenncare/Medicaid patients remained constant, while numbers of these procedures performed on commercially-insured patients increased.

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.

Salivary gland tumors in survivors of childhood cancer.

BACKGROUND: There is an increased incidence of second malignant neoplasms in survivors of childhood cancers. The most common second malignancies are acute leukemia, bone and soft tissue tumors, and carcinoma of the skin, breast, and thyroid. Although, ionizing radiation has been demonstrated to increase the risk of developing a salivary gland neoplasm, there are few reports of salivary gland neoplasms occurring in patients treated for cancer in childhood. METHODS: A retrospective review of the patient registry of St. Jude Children's Research Hospital from 1963-2003. RESULTS: Twelve survivors of childhood cancer developed a salivary gland neoplasm after completion of treatment. These patients were initially treated for a variety of childhood cancers with a combination of radiation and chemotherapy. The pathology of the salivary gland tumors were mucoepidermoid carcinoma (10), adenoid cystic carcinoma (1) , and pleomorphic adenoma (1). All patients were treated with surgical excision of the primary tumor, and postoperative radiation was added in select patients. Eleven patients were alive with no evidence of disease at last follow-up, and 1 patient was alive with clinical evidence of pulmonary metastasis. CONCLUSION: Radiation and chemotherapy used to treat patients with childhood malignancies increases the risk of developing a second neoplasm of salivary gland origin. The majority of these neoplasms are malignant; mucoepidermoid carcinoma occurs most frequently. The treatment of these tumors includes surgical excision of the primary, with neck dissection in patients with clinical evidence of nodal metastasis, and postoperative radiation added for pathologies with adverse features. EBM RATING: C-4.

Intracranial complications of pediatric sinusitis.

OBJECTIVE: To study intracranial extension of pediatric sinusitis, an infrequent but potentially fatal complication. STUDY DESIGN AND SETTING: Ten-year retrospective review at a tertiary children's hospital identified 21 cases of intracranial complications of sinusitis. RESULTS: Thirteen males and eight females with mean age of 13.3 years were identified. Overall 18 of 21 (81%) exhibited abscess formation, most commonly epidural. Only 3 of 21 (14%) had meningitis alone. All but 4 patients were managed surgically, requiring craniotomy in 13 of 21 (61.9%) and endoscopic sinus surgery (ESS) in 10 of 21 (48%). Seven patients (33%) required multiple operations during admission. Nineteen patients (90%) had a total of 30 organisms cultured. Oral flora was observed in 12 of 21 (57%). Polymicrobial infections, seen in 9 of 21 (43%), were significantly associated with the need for craniotomy (P=0.02). Mean hospital stay was 15 days, and mean length of IV antibiotic was 5 weeks. CONCLUSIONS: Intracranial complications of pediatric sinusitis often require craniotomy. Oral flora and polymicrobial infections were prominent in this series. EBM RATING: C-4.

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.

Frontal sinus fractures in children.

OBJECTIVE: To review the epidemiologic characteristics, clinical course, and management of pediatric patients with frontal sinus fractures. METHODS: Retrospective review of 120 patients with maxillofacial fractures who presented to a tertiary children's hospital from 1998 to 2003 revealed 11 patients with frontal sinus fractures. RESULTS: The study group included 9 males and 2 females with a mean age of 9.7 (range 4-14) years. The most common mechanisms of injury were unrestrained motor vehicle accident and all-terrain vehicle accident. All patients suffered concomitant orbital fractures. Other maxillofacial fractures included sphenoid (4), naso-orbitoethmoid (3), midface (2), and mandible (1). Seven (63.6%) patients sustained significant intracranial injuries including intraparenchymal hemorrhage, expanding pneumocephalus, and subdural hematoma. The average age of patients with intracranial injury was younger than those without intracranial injury (8.1 vs. 12.8 years, P = .025). Four patients had a total of six sites of cerebrospinal fluid (CSF) leak. The most common sites of dural injury were the cribriform area (4) and frontal region (2). All patients with CSF leaks had significant intracranial injuries and required bifrontal craniotomy. CONCLUSIONS: Pediatric frontal sinus fractures are likely to involve other maxillofacial injuries, particularly involving the orbit. Frontal sinus fractures in children are associated with increased risk of serious intracranial injury and CSF leak when compared with adults. The most common site of dural injury was the cribriform area. A multidisciplinary approach is necessary to manage concomitant injuries, obtain separation of the sinonasal tract from intracranial contents, and to restore cosmesis to the brow.

Hearing loss as a late complication of radiotherapy in children with brain tumors.

Late postirradiation hearing loss has been well described in the adult population. Few reports exist on the pediatric population. We conducted a retrospective review of 157 consecutive children with brain tumors treated exclusively with irradiation at St Jude Children's Research Hospital. Twenty-six patients developed a hearing loss, 74 did not, and 57 were excluded because of incomplete records. We report a statistically significant 27.41% cumulative risk of a stringent 20-dB hearing loss in the voice frequency range by the fifth year after radiotherapy. The right side demonstrated a significant frequency effect, with a higher incidence of loss in the higher-frequency region. We found no difference in cumulative incidence of hearing shift between the low-, middle-, and high-frequency ranges for either ear. This risk should be anticipated and managed as part of the treatment plan for radiotherapy for the treatment of malignancies. Radiation-induced hearing loss is important to acknowledge so that techniques of hyperfractionation, total dose, ports, preservative infusion medical therapy, or prolonged medical intervention (such as anticoagulants) can be developed that might reduce this disabling problem of postirradiation sensorineural hearing loss in future patients.