Infants' top-down perceptual modulation is specific to own-race faces.

Recent studies have revealed the influence of higher-level cognitive systems in modulating perceptual processing (top-down perceptual modulation) in infancy. However, more research is needed to understand how top-down processes in infant perception contribute to early perceptual development. To this end, this study examined infants' top-down perception of own- and other-race faces to reveal whether top-down modulation is linked to the emergence of perceptual specialization. Infants first learned an association between a sound and faces, with the race of the faces manipulated between groups (own race vs. other race). We then tested infants' face perception across various levels of perceptual difficulty (manipulated by presentation duration) and indexed top-down perception by the change in perception when infants heard the sound previously associated with the face (predictive sound) versus an irrelevant sound. Infants exhibited top-down face perception for own-race faces (Experiment 1). However, we present new evidence that infants did not show evidence of top-down modulation for other-race faces (Experiment 2), suggesting an experience-based specificity of this capacity with more effective top-down modulation in familiar perceptual contexts. In addition, we ruled out the possibility that this face race effect was due to differences in infants' associative learning of the sound and faces between the two groups. This work has important implications for understanding the mechanisms supporting perceptual development and how they relate to top-down perception in infancy.

Postoperative Disposition Following Pediatric Sistrunk Procedures: A National Database Query.

OBJECTIVE/HYPOTHESIS: Variability exists in the postoperative disposition of children following Sistrunk procedures. Management options include discharge home versus overnight observation, with the latter allowing monitoring for immediate postoperative complications, presumably reducing the need for subsequent readmission. This study investigates the association between overnight observation and ambulatory management of children undergoing Sistrunk procedures and relevant postoperative complication and revisit rates to clarify best practice for these patients. METHODS: This was a retrospective database review using the Pediatric Health Information System database from 2007 to 2016. RESULTS: The cited dataset identified 6,434 qualifying patients, categorized into ambulatory versus overnight observation cohorts. The overall 30-day revisit rate was 4.9%; the revisit rate with overnight observation (6.1%) was higher than for ambulatory patients (3.8%, adjusted odds ratio (OR) 1.60; 95% confidence interval (CI): 1.21, 2.12). Revisit rates were significantly higher in patients 2 years of age or younger compared to older patients (6.7% vs. 4.3%). The rates of return to the operating room for the observation versus ambulatory groups were 1.8% and 0.5%, respectively. Cervical fluid collection and neck swelling were among the most common revisit indications in both groups, with a mean time to presentation of 9 days. CONCLUSIONS: This study demonstrates that ambulatory management following a Sistrunk procedure is not associated with increased rates of common postoperative complications, readmission, or need for secondary surgical intervention. A Sistrunk procedure may be safely performed on an ambulatory basis in select cases. LEVEL OF EVIDENCE: IV Laryngoscope, 131:E2352-E2355, 2021.

Role of Surgery in Rhabdomyosarcoma of the Head and Neck in Children.

OBJECTIVES: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children. The goal of this research is to analyze the role of surgery in the management of pediatric parameningeal (PM) and non-PM head and neck RMS (HNRMS). STUDY DESIGN: Retrospective review. METHODS: Retrospective chart review of patients <20 years of age treated for HNRMS between 1970 and 2015. Clinical presentation, tumor characteristics, treatment, recurrence, follow-up, and outcome data were collected. RESULTS: Of 97 patients with HNRMS, 56% were male. Overall median (IQR: interquartile range) age at diagnosis was 5.8 (3.3-9.8) years. Sixty-five patients (67%) had PM tumors. Of 75 patients with histologic subtype identified, 51 (53%) had embryonal and 20 (21%) alveolar RMS. Almost all patients received chemotherapy (99%) and radiotherapy (95%). Forty-four patients (45%) underwent surgery. Surgery was more likely to be conducted in patients with lesions of a non-PM site. Median follow-up time was 3.4 years (IQR: 1.1-10.8). In 5 years of follow-up, 20% (17 of 85) died and 29% (20 of 70) had recurrence. The estimated 5-year survival rate was 72% (95% CI, 57.8, 81.5%). Surgery was associated with a reduced risk of mortality after accounting for TNM stage 4 and tumor site (adjusted HR 0.24; 95% CI, 0.07, 0.79; P = .02). The association between surgery and risk of mortality was similar in PM and non-PM tumors. CONCLUSION: A multimodal protocol for treatment including chemotherapy, surgery, and radiotherapy is the mainstay for management of children with HNRMS. While surgery is more commonly used to treat non-PM HNRMS, patients who are able to undergo surgery have significantly higher 5-year survival. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E984-E992, 2021.

Desmoid tumors of the head and neck in the pediatric population: Has anything changed?

INTRODUCTION: Pediatric head and neck desmoid tumors are rare neoplasms that can cause significant morbidity due to infiltration of vital anatomic structures. The goal of this study is to review presentation, evaluation, and management of these tumors. METHODS: Retrospective study of children with head and neck desmoid tumors treated from 1999 to 2018 and literature review. RESULTS: 11 patients (5 boys, 6 girls) were included. Presentation included firm neck mass (n = 8), trismus (n = 2) and tongue lesion (n = 1). All patients had preoperative imaging with CT (n = 2), MRI (n = 1) or both (n = 8). Five patients underwent needle biopsy, five had open biopsy and one was diagnosed on pathology from primary excision. Seven patients were treated by primary surgical resection, with positive surgical margins in six cases due to proximity to vital neurovascular structures. None needed chemotherapy, had disease recurrence or progression. Three patients with unresectable disease were treated with chemotherapy. One patient was monitored with imaging without any treatment and did not have disease progression. Follow-up ranged from 6 months to 6 years (median 21 months). Ten patients (7 surgical, 2 chemotherapy, 1 observation) were either disease-free or had stable disease at last follow-up. CONCLUSION: Pediatric head and neck desmoid tumors, though rare and histologically benign, are locally infiltrative and aggressive. When feasible, surgical treatment results in good disease control despite positive margins. A balance between achieving negative margins and minimizing functional deficits should be considered. Chemotherapy can be successfully utilized in patients where surgery entails a high risk of morbidity and mortality.

Familial and genetic factors in laryngeal cleft: Have we learned anything?

INTRODUCTION: Laryngeal clefts are rare congenital anomalies characterized by failed fusion of the posterior cricoid lamina or incomplete development of the tracheoesophageal septum. While most cases are sporadic, laryngeal cleft may be associated with other congenital anomalies or syndromes. Though not frequently reported, familial occurrence of laryngeal cleft has been noted in our clinical experience. The goal of this research is to describe the existing literature and our own experience surrounding familial occurrence of laryngeal cleft that may help elucidate its underlying genetic basis. METHODS: Comprehensive literature search was conducted and retrospective chart review was performed on 8 sets of siblings diagnosed at our institution. Data assessed included demographics, type of cleft, and genetic findings. RESULTS: Laryngeal cleft appears to be mostly sporadic. We evaluated data at our institution over a 10-year period and identified 19 patients from 8 families demonstrating familial occurrence of laryngeal cleft. Six (75%) families had two affected siblings, one family (12.5%) had three affected siblings, and one family (12.5%) had four affected siblings. There was no evidence of sex predilection, with half the patients being male (10/19, 52.6%). Fourteen patients (73.7%) had Type 1 clefts and five (26.3%) had Type 2 clefts. Genetic findings were available for review in five patients from three families. CONCLUSION: Beyond a few known syndromes, laryngeal cleft has largely been thought to be sporadic. However, findings from the existing literature and our own experience with familial laryngeal cleft in eight families suggest additional genetic factors are yet to be elucidated.

Pediatric Subperiosteal Abscess Secondary to Acute Sinusitis: A Systematic Review and Meta-analysis.

OBJECTIVES/HYPOTHESIS: The surgical versus medical management of subperiosteal abscess can be controversial. The purpose of this study was to summarize current literature and provide pooled analyses to help direct management decisions. STUDY DESIGN: Systematic review and meta-analysis. METHODS: Patients <18 years old with subperiosteal abscess secondary to acute sinusitis were reviewed, and a meta-analysis was conducted. Studies including five or more patients written in English were the primary search focus. RESULTS: Thirty-eight studies met inclusion criteria for the systematic review, and seven studies contained sufficient data for the meta-analysis. A total of 1,167 patients between the ages of 10 days and 18 years were included. Eleven sign/symptom categories were identified, with restricted ocular motility (n = 289, 45.9%), proptosis (n = 277, 44%), and fever (n = 223, 35.4%) being most frequent. Staphylococcus aureus was the most common pathogen isolated from cultures. Patients with abscess volume greater than the threshold specified in each individual study were over three times more likely to require surgical intervention compared to those with smaller abscess volume (pooled risk ratio [RR] = 3.61, 95% confidence interval [CI]: 2.40-5.44). Proptosis and gaze restriction also significantly predicted surgical intervention (pooled RR = 1.65: 95% CI: 1.29-2.12 for proptosis/pooled RR = 1.90; 95% CI: 1.20-3.00 for gaze restriction). CONCLUSIONS: Approximately half of patients with a subperiosteal abscess undergo surgical drainage. Abscess volume appears to be the most significant predictive risk factor. Detailed data from future studies regarding radiologic and ophthalmologic parameters are needed to provide more definitive values predictive of which patients are likely to fail medical therapy. LEVEL OF EVIDENCE: 2a Laryngoscope, 2020.

Preoperative augmentative and alternative communication enhancement in pediatric tracheostomy.

OBJECTIVES/HYPOTHESIS: Describe augmentative communication tools and strategies used by pediatric patients referred to inpatient speech-language pathologists prior to tracheostomy placement. STUDY DESIGN: Retrospective review. METHODS: A review of patients who underwent initial tracheostomy placement from 2013-2016 was conducted at a tertiary pediatric center. Eligible patients were those who were referred to a specialized speech-language pathologist prior to the date of the tracheostomy placement to support communication abilities. Patients were identified by surgical procedural and billing codes. Data collected included patient demographics, speech and language disorders, and interventions performed. Chart review and cross analysis of billing data for types of assessment and intervention procedures were conducted by two speech-language pathologists for consensus agreement. RESULTS: Forty-six patients (aged 1 month-27 years, mean = 12.9 years) were included in the study. Average time between the bedside communication assessment and tracheostomy procedure date was 17 days. Baseline speech-language disorders were identified in 11 patients (24%). Thirty-eight (83%) patients were nonspeaking at the time of consultation. Thirty-two (70%) patients utilized an electronic communication tool, and 36 (78%) utilized low-technology communication strategies during the preoperative period. A total of 32 (70%) patients were documented as using no-technology or speech-enhancement strategies during the acute hospitalization. CONCLUSIONS: Multidisciplinary tracheostomy teams should consider consultation to speech-language pathologists for patients prior to tracheostomy placement to assess for utility of high-technology, low-technology, and no-technology augmentative and alternative communication strategies. LEVEL OF EVIDENCE: 4 Laryngoscope, 130:1817-1822, 2020.

Current evaluation and management of plastic bronchitis in the pediatric population.

OBJECTIVE: To describe a multidisciplinary approach for the treatment of plastic bronchitis (PB) in children. METHODS: Retrospective chart review of children with PB between 1997 and 2017. Data regarding clinical presentation, diagnosis, management, and outcomes were analyzed. RESULTS: Of 34 patients presenting with PB, 24 had single ventricle (SV) heart disease, 9 had pulmonary disease, and one had no underlying disease. Median (IQR: interquartile range) age at the time of PB diagnosis was 5.5 years (IQR: 9.0). Presenting symptoms included cough productive of casts (n = 27, 79%), wheezing (n = 5, 15%), dyspnea (n = 18, 53%), hypoxia (n = 31, 91%), and respiratory failure (n = 9, 26%). Diagnosis was made based on clinical evaluation, bronchoscopy findings, and/or pathology of casts. Treatment methods included bronchoscopy for cast removal (25% of SV patients, 91% of non-SV patients), chest physiotherapy (SV: 92%, non-SV: 45%), albuterol (SV: 79%, non-SV: 73%), inhaled steroids (SV: 75%, non-SV: 18%), nebulized hypertonic saline (SV: 29%, non-SV: 9%), nebulized heparin (SV: 8%, non-SV: 55%), nebulized tissue plasminogen activator (tPA; SV: 33%, non-SV: 9%), inhaled Dornase Alfa (SV: 54%, non-SV: 9%), antibiotics (SV: 46%, non-SV: 45%), systemic steroids (SV: 13%, non-SV: 45%), and lymphatic embolization (SV: 8%, non-SV: 45%). Of SV patients, 11 had no recurrence, 5 underwent heart transplantation, one awaits transplant, and 3 died due to cardiac disease. Three patients with respiratory disease had recurrent PB and one died from MRSA pneumonia. CONCLUSION: PB is a highly morbid disease with limited treatment options. Bronchoscopy and chest physiotherapy for airway clearance are among the most-utilized therapies.

Laryngeal Web in the Pediatric Population: Evaluation and Management.

OBJECTIVE: To discuss the presentation, evaluation, and management of pediatric laryngeal web. STUDY DESIGN: Retrospective case series. SETTING: Single tertiary care center. SUBJECTS: All patients with laryngeal web at Boston Children's Hospital in the past 22 years. METHODS: No exclusion criteria. Charts mined for age at presentation, presenting symptoms, degree/location of web, associated syndromes, number/type of surgical procedures, and postoperative outcomes. RESULTS: Thirty-seven patients were included (13 male, 24 female). Average age at diagnosis was 3.7 years (0-19.5 years). Mean follow-up was 4.4 years (range, 0-16.4 years). There were 26 congenital webs (70.2%) and 11 acquired webs (29.8%). Presenting symptoms were vocal (29 patients, 78.4%) and respiratory (22 patients, 60%). Underlying syndromes or synchronous airway lesions included the following: premature (n = 5), congenital heart disease (n = 18), subglottic stenosis (n = 5), 22q11.2 deletion syndrome (n = 10), and recurrent respiratory papillomatosis (n = 4). There were 20 type 1 webs, 6 type 2 webs, 8 type 3 webs, and 3 type 4 webs; 10 had subglottic extension of the laryngeal web. Twelve patients were managed conservatively with observation. Eighty-four interventions were performed: 18 open and 66 endoscopic (sharp division, 32; dilation, 33; mitomycin C, 14; laser, 5; keel, 6; triamcinolone injection, 8; stent, 15; removal of granulation tissue, 5). Tracheotomy was required in 11 patients, and 5 patients were decannulated. Voice improved in 12 patients, with respiratory symptoms in 12 patients. Web recurred in 17 patients. One patient died due to airway complications. CONCLUSIONS: Pediatric laryngeal web is an uncommon but challenging lesion. Patients need to be evaluated for comorbid syndromes and synchronous airway lesions. Management includes open and endoscopic procedures. Procedures should be tailored to the child's presentation.

Balloon Sinuplasty Utilization in the Pediatric Population: A National Database Perspective.

OBJECTIVE: Balloon sinuplasty (BS) is a surgical management option in the treatment of chronic rhinosinusitis. The purpose of this study was to examine BS utilization among children with a national database. STUDY DESIGN: Retrospective review. SETTING: National pediatric database. SUBJECTS AND METHODS: All cases of children aged ≤18 years who underwent BS or traditional endoscopic sinus surgery (ESS) 5 years before and after the introduction of BS billing codes were studied with the Pediatric Health Information System database. We evaluated overall trends, demographics, performing physicians, readmissions, and cost data. RESULTS: A total of 14,079 patients met inclusion criteria: 13,555 underwent traditional ESS and 524 had a BS procedure. There was no significant increase in BS rates between 2011 and 2016. BS was more commonly performed among younger children than ESS (median age [interquartile range], 6 years [4-10] vs 9 years [6-13]; P < .001). There were 23 (4.4%) readmissions within 30 days in the balloon cohort versus 474 (3.5%) in the ESS cohort. The median cost of balloon maxillary antrostomy (US $6560 [$5420-$8250]) was higher than that of traditional maxillary antrostomy (US $5630 [$4130-$7700], P < .001). Physicians who performed BS had a larger volume of ESS procedures when compared with those who did not perform BS. CONCLUSION: Rates of BS performance in the pediatric population have not increased over time. Results showed no difference in readmission rates between BS and ESS. BS was associated with higher costs as compared with ESS. The role of BS in the pediatric chronic rhinosinusitis population remains unclear.

Mucoepidermoid carcinoma of the head and neck in children.

INTRODUCTION: Mucoepidermoid carcinoma is a rare malignant salivary gland neoplasm in the pediatric population. Few studies have discussed best practice with respect to diagnosis and treatment in children. OBJECTIVE: To present our institution's experience with the evaluation and management of pediatric mucoepidermoid carcinoma of the head and neck. METHODS: Retrospective chart review of patients under 20 years of age diagnosed with mucoepidermoid carcinoma of the head and neck between 1998 and 2017. Data assessed includes demographics, clinical presentation, imaging examinations, histopathology, treatment, complications, local recurrence, distant metastasis, and follow-up. RESULTS: Sixteen patients (10 female, 6 male) were identified with a median age of 12.9 (IQR: 10.9-15.0) years. Tumors were located within the parotid gland (n = 11, 68.8%), accessory lobe of the parotid gland (n = 2, 12.5%), palate (n = 2, 12.5%), and submandibular region (n = 1, 6.3%). In 9 patients (56.3%) a neoplastic etiology was suspected based on the clinical and/or radiographic findings and confirmed pathologically on biopsy or excision. All patients were treated surgically and five patients required adjuvant radiotherapy. One patient had recurrence requiring re-excision. Seven patients (43.8%) had transient facial paresis post-operatively and one had Frey syndrome. Median follow-up time was 59.7 months (IQR: 18.9-99.3). CONCLUSION: The malignant nature of mucoepidermoid carcinoma requires comprehensive, multidisciplinary management. Imaging and tissue sampling by fine needle aspiration give clinicians the best insight into location and nature of the mass. Complete surgical excision with attention to preservation of facial nerve and achieving negative margins is desired.

Evaluation of Aspiration in Infants With Laryngomalacia and Recurrent Respiratory and Feeding Difficulties.

Importance: Laryngomalacia is the most common laryngeal anomaly and is commonly associated with stridor in children, but the recurrent respiratory and/or feeding difficulties associated with this condition may pose a threat to the well-being of the affected child. Objective: To describe the prevalence of aspiration in pediatric patients with laryngomalacia who present with recurrent respiratory issues and/or feeding difficulty. Design, Setting, and Participants: This retrospective review of medical records involved 142 pediatric patients. These patients received a laryngomalacia diagnosis, presented with recurrent respiratory and/or feeding difficulties, and underwent a modified barium swallow (MBS) study at a tertiary referral children's hospital from January 1, 2015, to December 31, 2015. Each patient was assessed for aspiration and swallowing discoordination. Data collection and analysis were performed from December 1, 2016, to September 30, 2017. Main Outcomes and Measures: Demographic data, presenting symptoms, medical comorbidities, and feeding and dysphagia details were collected and analyzed. Speech-language pathologists reviewed all MBS reports to identify the presence and type of aspiration (ie, silent vs nonsilent) with different textures and consistencies of food and liquid. Results: A total of 395 patients were diagnosed with laryngomalacia in 2015. One hundred forty-two of these patients (35.9%) presented with recurrent respiratory issues and/or feeding difficulties and were referred for MBS study for further evaluation. Ninety-two (64.8%) were male, with a median (interquartile range) age at the time of MBS study of 7.9 (3.2-20.5) months. Among these patients, 128 (90.1%) had swallowing dysfunction documented during the MBS study. Aspiration was identified in 60 patients (42.3%), and silent aspiration was documented in 59 (98.3%) of these 60 patients. Epilepsy or seizures (risk difference [RD], 11%; 95% CI, 5%-17%), laryngeal cleft (RD, 8%; 95% CI, 3%-13%), and premature birth (RD, 15%; 95% CI, 5%-25%) were statistically significantly associated with abnormal MBS findings. Conclusions and Relevance: Swallowing dysfunction and aspiration were commonly found in pediatric patients with laryngomalacia and recurrent feeding and/or respiratory issues; these children should undergo an MBS study for dysphagia and silent aspiration.

Squamous cell carcinoma of the head and neck in children.

OBJECTIVE: To discuss the presentation, evaluation, and management of squamous cell carcinoma of the head and neck in the pediatric population. METHODS: Medical records of pediatric (≤20 years) patients treated for squamous cell carcinoma of the head and neck between 1996 and 2016 were reviewed. Data pertaining to clinical presentation, diagnostic methods, treatment plan, complications, recurrence, follow-up, or outcome were collected. RESULTS: Eleven patients were diagnosed with squamous cell carcinoma. Seven of these patients had medical histories significant for prior malignancies, immunosuppressant therapy, and/or genetic syndromes. Lesions were identified in the oral cavity (n = 5, 45.5%), lip/upper lip (n = 3, 27.3%), larynx (n = 2, 18.2%), and nasal cavity (n = 1, 9.1%). Tumors were most commonly treated with surgical excision alone. Three patients underwent irradiation (2 adjuvant and 1 without surgery) and chemotherapy (1 adjuvant, 1 neoadjuvant, and 1 without surgery). Of these patients, one reported complications of hearing loss, loss of dentition, and laryngeal stenosis. Two patients developed local recurrence at 1 month and 5 years post-operatively, respectively. One patient developed an orocutaneous fistula and subsequently died. No other complications were reported. Median follow-up time was 4.6 years (interquartile range: 2.4-8.4 years). Complications of radiation included: laryngeal stenosis, wound breakdown, and orocutaneous fistula. CONCLUSION: Squamous cell carcinoma is rare in the pediatric population. Most frequently, it is associated with previous malignancies, immunosuppressant therapy, and/or genetic conditions. Complete surgical excision is recommended to obviate the need for radiation whenever possible.

Aspiration in children with unilateral vocal fold paralysis.

OBJECTIVES/HYPOTHESIS: To describe the prevalence of aspiration in children with unilateral vocal fold paralysis who underwent objective assessment of swallow function. STUDY DESIGN: Retrospective chart review. METHODS: A study of patients presenting to our institution with unilateral vocal fold paralysis in 2015 was conducted. All patients were diagnosed using flexible laryngoscopy. Patients were included if they underwent at least one modified barium swallow (MBS) study for evaluation of their swallowing function due to recurrent respiratory issues and/or feeding difficulty. RESULTS: Twenty-eight patients diagnosed with unilateral vocal fold paralysis underwent an MBS study at our institution in 2015. Median age at the time of MBS study was 1.7 years (interquartile range: 0.4-4.3). Twenty-six patients (92.9%) had dysphagia. Sixteen patients were found to aspirate on MBS study. All patients who aspirated did so without overt signs (silent aspiration). Eighteen patients had congenital heart disease (64.3%) and nine had a history of prematurity (32.1%). Eight patients (28.6%) presented with developmental delays. CONCLUSIONS: Patients who present with unilateral vocal fold paralysis and recurrent respiratory and/or feeding issues may be affected by prominent issues such as swallowing dysfunction and silent aspiration. Clinicians should be aware of this risk and evaluate patients for any signs of feeding or swallowing difficulties. LEVEL OF EVIDENCE: 4 Laryngoscope, 129:569-573, 2019.

Pleomorphic adenoma of the head and neck in children: presentation and management.

OBJECTIVE: Pleomorphic adenoma is the most common benign salivary gland neoplasm in children. Recurrence and malignant transformation are concerns necessitating proper treatment. The goal of this research was to discuss the presentation, evaluation, and management of pediatric pleomorphic adenoma of the head and neck. METHODS: Retrospective chart review of patients under 20 years of age treated for pleomorphic adenoma of the head and neck between 1998 and 2017. Data assessed included demographics, clinical presentation, imaging, treatment, complications, recurrence, and follow-up. RESULTS: Forty-one patients with pleomorphic adenoma were identified. Major salivary gland lesions were most common (n = 32, 78.0%); 78.1% (25 of 32) arising in the parotid and 21.9% (7 of 32) in the submandibular glands. Minor salivary gland lesions were removed from the palate (5 of 9, 55.6%), buccal mucosa (2 of 9, 22.2%), parapharyngeal space (1 of 9, 11.1%), and upper lip (1 of 9, 11.1%). Preoperative imaging was reviewed in 32 patients and consisted of nine ultrasound exams, 15 computerized tomography (CT) exams, and 23 magnetic resonance imaging (MRI) exams. Multimodality imaging was performed in 14 of 32 patients. Surgical excision was performed in all patients. Postoperative complications included transient facial paresis (n = 9), Frey syndrome (n = 1), and bacterial pharyngitis (n = 1). Average length of follow-up was 25.5 months; confirmed recurrence occurred in two patients (4.9%). CONCLUSION: Evaluation of pleomorphic adenoma in children should include preoperative imaging. The goal of surgery should be complete surgical excision with negative margins. Rate of recurrence is low; however, prolonged monitoring is recommended. LEVEL OF EVIDENCE: 4. Laryngoscope, 129:2603-2609, 2019.

Vallecular cyst in the pediatric population: Evaluation and management.

OBJECTIVE: To review the presentation of pediatric vallecular cysts and outline an approach for evaluation and management. METHODS: Medical records of patients diagnosed with vallecular cyst between 2005 and 2017 were reviewed. Data on demographics, clinical characteristics, diagnostic methods, surgical procedures, and outcomes were collected and analyzed. A comprehensive literature search for pediatric cases of vallecular cyst was conducted for comparative analysis. RESULTS: Twenty patients underwent surgery for congenital vallecular cysts during the study period. Age at diagnosis ranged from birth to 8 years (median age = 1.1 years). The most common preoperative symptom was inspiratory stridor (45%) followed by feeding difficulties (40%). Eight patients (40%) initially presented with laryngomalacia and 7 (35%) with feeding difficulties. Imaging was obtained in 16 patients and consisted of plain films, ultrasound, computed tomography (CT), or magnetic resonance imaging (MRI). Marsupialization of the cyst was performed in all 20 patients. Three patients (15%) presented with recurrence. CONCLUSION: Vallecular cysts can cause feeding difficulties due to upper airway obstruction and pressure at the laryngeal inlet. Diagnostic work-up for vallecular cysts should include a detailed medical history, complete head and neck examination including endoscopic examination, and appropriate imaging, as each of these components complements the histopathologic findings. Our findings indicate that performing marsupialization is associated with low rate of recurrence or complication.

Development and validation of a quality of life instrument for patients with laryngeal cleft.

OBJECTIVE: To develop and validate a novel pediatric health-related quality of life (HR-QoL) instrument for patients with laryngeal cleft and their families. METHODS: We surveyed primary caregivers of patients that underwent endoscopic repair of Type I or II laryngeal cleft. The proposed HR-QoL instrument consists of 40 items representing four domains, assessing the patient's physical symptoms, the patient's and family's social functioning, and the family's emotions regarding the patient's illness pre- and post-operatively. Confirmatory factor analysis was employed to assess construct validity, dimensionality, and optimal simple structure. RESULTS: Of 78 eligible participants reached by phone, 40 (51%) of them completed the questionnaire. Confirmatory factor analysis suggested that all four measured constructs were well supported by the measured items in comparison to a unidimensional model. All factor loadings and factor correlations were significant and factor correlations ranged between 0.723 and 0.879. Indices of test-retest reliability and internal consistency reliability were well above recommended standards. There was a significant correlation between current instrument and PedsQL™ score. The overall QoL score significantly improved from 112.3 (±28.1) before surgery to 158.0 (±28.5) after surgery (mean difference 45.7; 95% CI: 37.3, 54.1; p < 0.001). CONCLUSION: Our proposed pediatric HR-QoL instrument is a valid tool for measuring quality of life in patients with laryngeal cleft and their families. This instrument can provide insight into the effects of medical and surgical therapy and guide pre- and post-operative management of laryngeal cleft.

Silent aspiration: Who is at risk?

OBJECTIVE: To determine the prevalence of silent aspiration in pediatric patients and identify which diagnoses may be associated with this finding. METHODS: An institutional review board-approved retrospective review was conducted for all patients under the age of 18 who underwent modified barium swallow (MBS) studies at a tertiary children's hospital in 2015. Speech-language pathologists reviewed MBS studies to identify aspiration/silent aspiration on each fluid consistency tested. Charts were reviewed to collect demographic information and the otolaryngologic, neurologic, genetic, and syndromic diagnoses of each patient. RESULTS: Among 1,286 patients who underwent MBS, 440 (34%) demonstrated aspiration. Within the aspiration group, 393 (89%) specifically demonstrated silent aspiration. Thin fluids were silently aspirated in 81% of these patients. Of children aged <6 months, 41% were found to aspirate and, of those, 95% silently aspirated. Median age at which patients demonstrated silent aspiration was 1.1 years. Silent aspiration was documented in 41% of patients with laryngeal cleft, 41% of patients with laryngomalacia, and 54% of patients with unilateral vocal fold paralysis. Laryngeal cleft, laryngomalacia, unilateral vocal fold paralysis, developmental delay, epilepsy/seizures, syndrome, and congenital heart disease were all associated with silent aspiration. CONCLUSION: Silent aspiration may be associated with a number of underlying conditions and is more common than previously described. Caregivers and clinicians should be aware that the absence of cough does not eliminate the possibility of aspiration. Modified barium swallow studies can reveal silent aspiration, which is difficult to detect on clinical feeding evaluation. Modified barium swallow findings can guide feeding therapy and the overall management of aspiration. LEVEL OF EVIDENCE: 4. Laryngoscope, 1952-1957, 2018.

Prevalence of Laryngeal Cleft in Pediatric Patients With Esophageal Atresia.

Importance: Esophageal atresia (EA), with or without tracheoesophageal fistula (TEF), and laryngeal cleft are rare congenital anomalies that often occur together. Previous reports have established a link between EA/TEF and laryngeal cleft, but there have been no large case series to further characterize this relationship. Objectives: To assess the prevalence of laryngeal cleft among patients with EA/TEF, identify associations between types of laryngeal cleft and EA/TEF, and identify factors associated with the timing of diagnosis for both conditions. Design, Setting, and Participants: Retrospective analysis of 270 patients. The Esophageal Atresia database was used to identify patients seen at the Esophageal and Airway Treatment (EAT) Center at Boston Children's Hospital, Boston, Massachusetts, a tertiary referral hospital, from August 1, 2009, to August 1, 2016. Patients were included if they were younger than 18 years at the time they were diagnosed with EA, TEF, or both and had undergone a procedure or examination by a surgeon from the EAT Center. Patients with acquired airway or esophageal problems were excluded. Main Outcomes and Measures: Data regarding patient demographics, primary diagnoses, dates of diagnosis, comorbidities, and airway/esophageal surgical interventions were collected and analyzed. Association between type of EA/TEF and laryngeal cleft type was evaluated. Secondary outcomes were age at diagnosis between patients who resided in Massachusetts and those who resided outside the state. Results: Of 270 patients diagnosed with EA/TEF during the 7-year period, 138 (51.1%) were male, and the median age at diagnosis of EA/TEF was 1.0 years (IQR, 0.4-2.0) and at diagnosis of laryngeal cleft was 1.1 years (interquartile range [IQR], 0.6-2.8). Laryngeal cleft was diagnosed in 53 of the 270 patients (19.6%) (95% CI, 14.9%-24.4%). No apparent difference was found in the distribution of types of laryngeal cleft by type of EA/TEF. Among patients with EA/TEF type A or B, 9 patients (56%) had a type I cleft, 6 (38%) had a type II cleft, and 1 (6%) had a type III cleft. Among those with EA/TEF type C or D, 20 (57%) had a type I cleft, 11 (31%) had a type II cleft, and 3 (9%) had a type III cleft. Out-of-state patients were more likely to be diagnosed with EA/TEF at an older age than in-state patients (mean difference, 1.5 years; 95% CI, 0-2.9 years). Conclusions and Relevance: Pediatric patients with EA/TEF have a much greater prevalence of laryngeal cleft than the general population. Multidisciplinary esophageal and airway programs serve as an ideal clinical setting for management of EA/TEF.