Tracheal cartilaginous sleeves in children with syndromic craniosynostosis.

PURPOSE: Because a tracheal cartilaginous sleeve (TCS) confers a significant mortality risk that can be mitigated with appropriate intervention, we sought to describe the prevalence and associated genotypes in a large cohort of children with syndromic craniosynostosis. METHODS: Chart review of patients with syndromic craniosynostosis across two institutions. RESULTS: In a cohort of 86 patients with syndromic craniosynostosis, 31 required airway evaluation under anesthesia. TCS was found in 19, for an overall prevalence of 22%. FGFR2, TWIST1, and FGFR3 mutations were identified in children with TCS. All five children with a W290C mutation in FGFR2 had TCS, and most previously reported children with W290C had identification of TCS or early death. In contrast, TCS was not associated with other mutations at residue 290. CONCLUSION: There is an association between TCS and syndromic craniosynostosis, and it appears to be particularly high in individuals with the W290C mutation in FGFR2. Referral to a pediatric otolaryngologist and consideration of operative airway evaluation (i.e., bronchoscopy or rigid endoscopy) in all patients with syndromic craniosynostosis should be considered to evaluate for TCS. Results from genetic testing may help providers weigh the risks and benefits of early airway evaluation and intervention in children with higher-risk genotypes.Genet Med 19 1, 62-68.

Competency-Based Assessment Tool for Pediatric Esophagoscopy: International Modified Delphi Consensus.

OBJECTIVES/HYPOTHESIS: Create a competency-based assessment tool for pediatric esophagoscopy with foreign body removal. STUDY DESIGN: Blinded modified Delphi consensus process. SETTING: Tertiary care center. METHODS: A list of 25 potential items was sent via the Research Electronic Data Capture database to 66 expert surgeons who perform pediatric esophagoscopy. In the first round, items were rated as "keep" or "remove" and comments were incorporated. In the second round, experts rated the importance of each item on a seven-point Likert scale. Consensus was determined with a goal of 7 to 25 final items. RESULTS: The response rate was 38/64 (59.4%) in the first round and returned questionnaires were 100% complete. Experts wanted to "keep" all items and 172 comments were incorporated. Twenty-four task-specific and 7 previously-validated global rating items were distributed in the second round, and the response rate was 53/64 (82.8%) with questionnaires returned 97.5% complete. Of the task-specific items, 9 reached consensus, 7 were near consensus, and 8 did not achieve consensus. For global rating items that were previously validated, 6 reached consensus and 1 was near consensus. CONCLUSIONS: It is possible to reach consensus about the important steps involved in rigid esophagoscopy with foreign body removal using a modified Delphi consensus technique. These items can now be considered when evaluating trainees during this procedure. This tool may allow trainees to focus on important steps of the procedure and help training programs standardize how trainees are evaluated. LEVEL OF EVIDENCE: 5. Laryngoscope, 131:1168-1174, 2021.

Competency-Based Assessment Tool for Pediatric Tracheotomy: International Modified Delphi Consensus.

OBJECTIVES/HYPOTHESIS: Create a competency-based assessment tool for pediatric tracheotomy. STUDY DESIGN: Blinded, modified, Delphi consensus process. METHODS: Using the REDCap database, a list of 31 potential items was circulated to 65 expert surgeons who perform pediatric tracheotomy. In the first round, items were rated as "keep" or "remove," and comments were incorporated. In the second round, experts were asked to rate the importance of each item on a seven-point Likert scale. Consensus criteria were determined a priori with a goal of 7 to 25 final items. RESULTS: The first round achieved a response rate of 39/65 (60.0%), and returned questionnaires were 99.5% complete. All items were rated as "keep," and 137 comments were incorporated. In the second round, 30 task-specific and seven previously validated global rating items were distributed, and the response rate was 44/65 (67.7%), with returned questionnaires being 99.3% complete. Of the Task-Specific Items, 13 reached consensus, 10 were near consensus, and 7 did not achieve consensus. For the 7 previously validated global rating items, 5 reached consensus and two were near consensus. CONCLUSIONS: It is feasible to reach consensus on the important steps involved in pediatric tracheotomy using a modified Delphi consensus process. These items can now be considered to create a competency-based assessment tool for pediatric tracheotomy. Such a tool will hopefully allow trainees to focus on the important aspects of this procedure and help teaching programs standardize how they evaluate trainees during this procedure. LEVEL OF EVIDENCE: 5 Laryngoscope, 130:2700-2707, 2020.

Bilateral vocal cord paralysis in children.

Bilateral vocal cord paralysis in children with its many causes presents a challenging problem to the pediatric otolaryngologist. Traditionally, management of bilateral vocal cord paralysis includes securing the airway with a tracheotomy and waiting for spontaneous recovery. Surgeons have tried a variety of surgical procedures in lieu of or in addition to tracheotomy, but none are perfect solutions to the problem. This article reviews the current surgical procedures for bilateral vocal cord paralysis in the pediatric population with a particular focus on the senior author's experience with the endoscopic posterior costal cartilage grafting procedure.

Endoscopic posterior cricoid split with costal cartilage graft: A fifteen-year experience.

OBJECTIVE: To evaluate outcomes of the endoscopic posterior cricoid split with rib graft (EPCS/RG) procedure in the treatment of subglottic stenosis (SGS), posterior glottic stenosis (PGS), and bilateral vocal fold immobility (BVFI). STUDY DESIGN: Retrospective chart review. METHODS: Chart review of all patients who underwent EPCS/RG at a single tertiary-care facility between 1999 and 2014. Patients were grouped based on the primary indication for the procedure. Decannulation was the primary endpoint. Secondary endpoints were the number of subsequent airway procedures and length of hospitalization. RESULTS: Thirty-three patients were identified; 32 had tracheotomy. Overall decannulation rate was 65.6%. Subgroup analysis demonstrated the following decannulation rates: 53.8% for SGS, 100% for PGS, and 28.6% for BVFI. Fisher exact test found a significant difference in overall decannulation rates between groups (P = 0.002). Operation-specific decannulation rates for patients who never required an open procedure were 23% for SGS, 91.6% for PGS, and 28.6% for BVFI. This difference was also statistically significant (P = 0.001). Multivariate logistic regression analysis found prematurity had a positive correlation with decannulation that approached statistical significance (P < 0.051; odds ratio 6.1; 95% confidence interval 0.99, 37.6). The percentage of patients who underwent repeat airway procedures for the groups was 61.5% for SGS, 16.6 % for PGS, and 14.3% for BVFI. The median length of hospitalization after EPCS/RG was 3 days. CONCLUSION: This represents the largest series of patients who have undergone EPCS/RG and demonstrates that the majority of patients can be decannulated after this procedure. Patients with PGS had the highest operation-specific decannulation rates. LEVEL OF EVIDENCE: 4. Laryngoscope, 127:252-257, 2017.

International Pediatric Otolaryngology Group: Consensus guidelines on the diagnosis and management of type I laryngeal clefts.

INTRODUCTION: The diagnosis and management of type I laryngeal clefts can be controversial and varies across centers and surgeons. Using existing peer-reviewed literature to develop an expert-based consensus will help guide physicians in the treatment of these patients as well as develop research hypotheses to further study this condition. OBJECTIVE: To provide recommendations for the diagnosis and management of type I laryngeal clefts. METHODS: Determination of current expert- and literature-based recommendations, via a survey of the International Pediatric Otolaryngology Group, using a modified Delphi method. SETTING: Multinational, multi-institutional, tertiary pediatric hospitals. RESULTS: Consensus recommendations include diagnostic workup, medical management, pre-operative, intra-operative and post-operative considerations for type I laryngeal clefts. CONCLUSIONS: This guide on the diagnosis and management of patients with type I laryngeal clefts is aimed at improving patient care and promoting future hypothesis generation and research to validate the recommendations made here.

Recurrent thyroglossal duct cysts: a 23-year experience and a new method for management.

OBJECTIVES: We present an experience in the management of primary and recurrent thyroglossal duct cysts (TGDCs) and describe a novel method for recurrent TGDC removal. METHODS: We performed a retrospective review of TGDC surgery at Children's Hospital in Seattle from 1980 to 2003. The surgical techniques for primary and recurrent TGDCs and the factors associated with TGDC recurrence were evaluated and analyzed. RESULTS: During the study period, 231 patients underwent 296 TGDC surgeries. Thirty-four of the 231 patients (15%) underwent a total of 88 procedures for recurrent TGDCs. Successful procedures used for secondary TGDC management included central neck dissection with directed base of tongue (BOT) excision in 6 of 9 patients (67%), secondary Sistrunk operation with limited BOT resection in 12 of 27 patients (44%), revision Sistrunk operation with BOT dissection in 7 of 11 patients (64%), and suture-guided transhyoid pharyngotomy in 8 of 8 patients (100%). Ten of the 231 patients (4%) had initial TGDC incision and drainage and then underwent a total of 21 procedures, excluding the incision and drainage. The factors associated with TGDC recurrence were inaccurate initial diagnosis (17 of 34 or 50%), infection (5 of 34 or 15%), unusual TGDC presentation (5 of 34 or 15%), and lack of BOT musculature removal (7 of 34 or 20%). The level of surgeon training affected the surgical outcome. CONCLUSIONS: Successful TGDC treatment requires consideration of factors associated with recurrence. Recurrent TGDCs can be treated by several methods, including suture-guided transhyoid pharyngotomy.

Laryngeal anatomic differences in pediatric patients with severe laryngomalacia.

OBJECTIVES: To compare the aryepiglottic (AE) length in pediatric patients who have severe laryngomalacia (SL) and are undergoing aryepiglottoplasty with the AE length of a convenience sample of control patients without laryngomalacia. DESIGN: Prospective case-control study. SETTING: A tertiary-care pediatric hospital. RESULTS: The mean AE fold length-glottic length ratio for patients with SL (0.380) was significantly lower than the mean ratio for controls (0.535) (P = .004 in 2-sample t test with unequal variance). For patients with SL, the aryepiglottoplasy procedure resulted in an average AE length increase-glottic length ratio of 0.330. Seven of the patients with SL were also diagnosed as having an underlying neurologic condition, and 18 had a diagnosis of gastroesophageal reflux disease. Two patients with SL required a tracheotomy for treatment of persistent airway obstruction. CONCLUSIONS: In this series, patients with SL had lower AE fold length-glottic length ratios and more frequent occurrence of neuromuscular tone abnormalities (especially gastroesophageal reflux) than controls. These 2 findings may be related in that low intrauterine tone might contribute to anatomic underdevelopment.

Plastic laryngeal foreign bodies in children: a diagnostic challenge.

OBJECTIVE: To review Children's Hospital and Regional Medical Center experience with pediatric airway foreign bodies, and examine the incidence and treatment of laryngeal foreign bodies. To determine if plastic laryngeal foreign bodies present differently than other laryngeal foreign bodies. METHODS: A retrospective review of all cases of children (1874 patients) undergoing direct laryngoscopy and/or bronchoscopy from 1st January 1997 to 9th September 2003 at a tertiary care children's hospital. Patients with endoscopically documented laryngeal foreign bodies were identified and the medical record reviewed in more detail. Patient age, gender, foreign body location, foreign body type, duration of foreign body presence, radiographic findings, endoscopic findings and treatment complications were recorded. RESULTS: One hundred and five aspirated foreign bodies were identified. The nine laryngeal foreign bodies included five clear plastic radiolucent items, two radiolucent food items, and two sharp radioopaque pins. Time to diagnosis and treatment was on average 11.6 days with 17.6 days for thin/plastic foreign bodies and 1.6 days for metal/food foreign bodies. CONCLUSION: Laryngeal foreign bodies represent a small portion of all pediatric airway foreign bodies. Difficulty in identifying laryngeal foreign bodies, especially thin, plastic radiolucent foreign bodies can delay treatment. Thin plastic foreign bodies can present without radiographic findings, can be difficult to image during endoscopy and can be particularly difficult to diagnose. A history of choking and vocal changes is associated with laryngeal foreign bodies. Laryngeal foreign bodies should be in the differential diagnosis of all children presenting with atypical upper respiratory complaints especially if a history suggestive of witnessed aspiration and dysphonia can be obtained.

Endoscopic posterior cricoid split and rib grafting in 10 children.

OBJECTIVE: To report our experience with endoscopic posterior cricoid split and rib graft insertion (EPCS/RG) in children with posterior glottic (PGS) and subglottic stenosis (SGS). DESIGN: Retrospective analysis of case series, with 1 to 2 year follow-up. SETTING: Tertiary-care pediatric referral center. PATIENTS: Ten consecutive patients undergoing EPCS/RG. INTERVENTION: EPCS/RG is a new procedure that expands the posterior glottic opening by dividing the posterior cricoid lamina endoscopically with a laser and inserting a rib cartilage graft through the laryngoscope. MAIN OUTCOME MEASURES: Laryngeal function and hospital stay. RESULTS: Successful decannulation in two of two patients with PGS and one of five patients with PGS and SGS without further surgery. Of the four not initially decannulated, two were decannulated with adjunctive procedures, and the other two can now tolerate tracheostomy capping for extended periods of time. We achieved improvement in exercise tolerance in three nontracheotomy-dependent patients. For those with established tracheotomies, median hospital stay was 3 days and intensive care unit care was unnecessary. There were no major complications or deterioration of voice or feeding. CONCLUSIONS: EPCS/RG appears to be safe and effective in the management of PGS in selected pediatric patients. This minimally invasive procedure has advantages over traditional open approaches and destructive endoscopic techniques (cordotomy and arytenoidectomy). The role of EPCS/RG alone in the face of severe grades of SGS appears to be limited.

Impact of adenotonsillectomy on quality of life in children with obstructive sleep disorders.

OBJECTIVES: To determine the impact of adenotonsillectomy on quality of life (QOL) in children with obstructive sleep disorders (OSDs) before and after surgery. DESIGN: Prospective, observational, before-and-after trial. SETTING: Seven tertiary pediatric otolaryngology practices. PATIENTS: Convenience sample of 101 children (mean age, 6.2 years) with adenotonsillar hypertrophy and OSD scheduled for adenotonsillectomy. INTERVENTION: Adenotonsillectomy was performed in children for OSDs. Quality of life was assessed using the Obstructive Sleep Disorders-6 survey, a validated instrument for detecting QOL change in children with OSDs. Surveys were completed at the initial office visit (visit 1), the day of surgery (visit 2), and at the postoperative office visit (visit 3). Physical characteristics were assessed using tonsillar and orocraniofacial scales (visit 1). Satisfaction with health care decisions was assessed using the Satisfaction With Decision and Satisfaction With Office Visit scales (visit 1). MAIN OUTCOME MEASURES: Short-term changes in QOL before (visits 1 and 2) and after (visits 2 and 3) surgery. RESULTS: Changes in QOL before surgery were trivial or small, and smaller than changes after surgery (mean change score, 0.18 vs 2.3; P<.001). Large, moderate, and small improvements in QOL were seen in 74.5%, 6.1%, and 7.1% of children, respectively. Sleep disturbance, caregiver concern, and physical suffering were the most improved domains, although significant changes also occurred for speech and swallowing problems, emotional disturbance, and activity limitations. Five percent of children had poorer QOL after surgery, but no predictive factors were identified. CONCLUSION: Adenotonsillectomy produces large improvements in at least short-term QOL in most children with OSDs.

Asthma, allergy, and sinopulmonary disease in pediatric condition falsification.

Reports of pediatric condition falsification (PCF) have noted, but not emphasized, exaggerated complaints of real and common illnesses. Among the most frequent chronic childhood illnesses are asthma, allergy, drug sensitivity, and ear and sinopulmonary infections. The most common pediatric surgery is the insertion of myringotomy tubes. A computer database of 104 PCF victims from 68 families spanning from 1974 to 1998 was searched for the frequency of these conditions. Outright falsification or extreme exaggeration of severity of asthma or allergies was noted in 52 children (50%), sinopulmonary infections in 50 (48%), and drug reactions or sensitivities in 30 (29%). Forty-five children (43%) had otolaryngologic surgery, including ear tubes. In all, 71 children (68%) had at least one of these conditions. Associated victim and perpetrator characteristics are described. Children with PCF are not only subjected to induced illnesses and excessive medical diagnostic and therapeutic efforts but also victimized b) the consequences of false and exaggerated complaints of common pediatric diseases.

Improved airway visualization during direct laryngoscopy using self-retaining laryngeal retractors: a quantitative study.

OBJECTIVE: To measure the degree to which the Lindholm laryngeal distending forceps improve visualization during direct laryngoscopy in selected pediatric patients. STUDY DESIGN: Case series with chart review. SETTING: Pediatric hospital. SUBJECTS AND METHODS: Subjects included children undergoing direct laryngoscopy using the Lindholm laryngeal distending forceps. Intraoperative endoscopic photos with and without false cord retraction via the Lindholm laryngeal distending forceps were obtained from the Seattle Children's Hospital airway endoscopy photo library. Analysis was performed using imaging software. Comparisons of visible vocal cord and glottic opening areas as well as anterior commissure angles with and without the Lindholm laryngeal distending forceps were performed with a paired and unpaired Student t test. RESULTS: The use of the Lindholm laryngeal distending forceps increased the glottic opening by a mean of 359% (95% confidence interval [CI], 255%-463%) and increased visualized true vocal cord area by 337% (197%-477%). Angle at the anterior commissure increased from a mean of 24.9° to a mean of 71.5°, resulting in a net mean angle increase of 46.6° (95% CI, 40.2°-52.9°). All measured changes were statistically significant with P values <.01. CONCLUSIONS: When placed at the level of the false vocal folds, Lindholm laryngeal distending forceps will, at least in certain cases, greatly increase the visible area of the superior surface of the vocal folds, the anterior commissure, and, by increasing the glottic opening, the subglottic region. This improved visualization may enhance the surgeon's ability to diagnose and treat pathologies in these anatomic regions during direct laryngoscopy.

How airway venous malformations differ from airway infantile hemangiomas.

OBJECTIVE: To compare airway infantile hemangiomas (IHs) and venous malformations (VMs) clinically, radiographically, endoscopically, and histologically. DESIGN: Retrospective cohort study. SETTING: Tertiary care pediatric hospital. PATIENTS: The study included patients seen in the Vascular Anomaly Clinic, Seattle Children's Hospital, Seattle, Washington, between 2001 and 2008. METHODS: All patients with airway vascular anomalies were identified by searching the Vascular Anomaly Quality Improvement Database and hospital discharge data. The data, which were analyzed with descriptive statistics and the Fisher exact test, included presenting age, sex, presenting signs, lesion site, and radiographic, endoscopic, and histologic findings.. RESULTS: Seventeen patients with airway lesions were identified, 6 with VMs and 11 with IHs. Patients with VMs presented at a mean (SD) age of 11.3 (13.7) months (age range, 3-39 months), while those with IHs presented at 3 (1.8) months of age (age range, 1-6 months) (P = .03). The patients with IHs were predominantly female (9 of 11 [81%]), while no sex difference was noted among the patients with VMs (3 of 6 [50%]). All patients with IHs presented with stridor and cutaneous lesions, whereas patients with VMs more often presented with hemoptysis or dysphagia (P = .001). Computed tomographic angiograms demonstrated enhancing endolaryngeal lesions in all IHs, while VMs enhanced poorly. Endoscopically, IHs were transglottic, while VMs were postcricoid or epiglottic (P < .001). Histologically, immunostained lesions showed submucosal lobules of capillaries lined by GLUT-1 (glucose transporter isoform 1)-positive endothelium in IHs, whereas VMs consisted of loosely organized venous channels that lacked GLUT-1 staining. CONCLUSION: Patients with airway IHs and VMs differ in presenting age and signs, sex, airway lesion location, enhancement on computed tomographic angiograms, and histologic appearance.

Transoral approach for direct and complete excision of vallecular cysts in children.

OBJECTIVE: To review the presentation, evaluation, and treatment of children with vallecular cysts and introduce a new technique of transoral excision for this entity. METHODS: Retrospective case series of children diagnosed with vallecular cyst between 2001 and 2008 at a single tertiary care children's hospital. Data collected, including age at diagnosis, presenting symptoms, additional diagnoses, diagnostic modality, prior and subsequent surgical therapy, length of hospital stay, length of follow-up, and recurrence were analyzed with descriptive statistics. RESULTS: Seven children (mean age 198 days, range 2 days to 2.9 years) were included in this series. Five children presented with respiratory distress and/or swallowing difficulties. Vallecular cyst was diagnosed by initial flexible fiberoptic laryngoscopy (5/7), MRI (1/7), and intubating laryngoscopy (1/7). All children underwent complete cyst excision via transoral surgical approach. Two children underwent additional supraglottoplasty for concomitant laryngomalacia, one of whom underwent tracheotomy for persistent respiratory distress and vocal cord immobility. The average length of hospital stay postoperatively was 9.5 days, and four patients stayed less than 2 days. No patients experienced recurrence of the vallecular cyst at last follow-up (range 4-755 days, mean 233 days). CONCLUSIONS: Vallecular cysts are rare but should be considered in children with respiratory distress and dysphagia. Awake, flexible fiberoptic laryngoscopy with particular attention to the vallecular region should be performed on any child presenting with these symptoms. Direct, transoral approach for excision of the vallecular cyst is our preferred method of treatment with no recurrences to date.

Endoscopic electrocauterization of pyriform fossa sinus tracts as definitive treatment.

OBJECTIVE: To (1) update the technique of endoscopic electrocauterization of patients with pyriform fossa sinus tracts and (2) evaluate its effectiveness as a definitive treatment. METHODS: Retrospective case series with nine patients (age range, 3.3-16.1 years) who were diagnosed with pyriform fossa sinus tracts between 2000 and 2007 at a single tertiary care children's hospital and underwent endoscopic electrocauterization of the sinus tract. Data collected including age of diagnosis, presenting symptoms, time from presentation to diagnosis, diagnostic studies, prior and subsequent treatments, length of hospital stay, and recurrence, were reviewed and analyzed with descriptive statistics. RESULTS: All nine patients presented with recurrent left or midline neck masses or abscesses. Seven patients (78%) had at least one previous incision and drainage procedures for abscess treatment. All patients had a computed tomography scan with findings suspicious for left-sided pyriform fossa sinus tract. The diagnosis was confirmed with laryngoscopy. For seven patients (78%), endoscopic electrocauterization was definitive treatment with no recurrences to date. Two patients (22%) had recurrent left neck abscesses after endoscopic treatment; these patients ultimately underwent excision of sinus tract with left thyroid lobectomy without complications or further recurrences. CONCLUSIONS: Endoscopic electrocauterization of pyriform fossa sinus tracts is a safe and definitive treatment for most patients. We advocate this minimally invasive procedure as first line of treatment for pyriform fossa sinus tracts, reserving open excision with or without thyroid lobectomy for failures.

Polymerase chain reaction for pathogen identification in persistent pediatric cervical lymphadenitis.

OBJECTIVE: To study routine culture-negative persistent cervical lymphadenitis in children treated surgically during a 10-year period (December 26, 1997, to October 1, 2007) at a single institution. DESIGN: Retrospective case series. SETTING: Tertiary university-based pediatric referral center. PATIENTS: Patients 18 years or younger with cervical lymphadenitis managed surgically (incision and drainage, curettage, and/or excisional lymphadenectomy) and medically (antibiotic therapy), culture-negative after 48 hours, and subsequently diagnosed using the polymerase chain reaction, extended culture incubation, and/or histopathologic evaluation. MAIN OUTCOME MEASURES: Number of surgical interventions, causative organisms, histopathologic features, and resolution of lymphadenitis. RESULTS: Ninety surgical procedures were performed in 60 patients. The cure rate was 23% (approximately 14 patients) with incision and drainage, 58% (approximately 35 patients) with curettage, and 95% (57 patients) with excisional lymphadenectomy. Nontuberculous mycobacteria were the most prevalent causative organisms, followed by Bartonella and Legionella organisms. Four of 6 patients with Bartonella infection had a history of cat exposure, and 4 of 6 patients with Legionella infection had a history of hot tub exposure. CONCLUSIONS: Excisional lymphadenectomy is the preferred treatment of mycobacterial persistent cervical lymphadenitis in children. Sufficient data are lacking for similar recommendations in patients with disease caused by Bartonella organisms, whereas for neck disease caused by Legionella organisms, excisional lymphadenectomy may be superior to incision and drainage. The polymerase chain reaction is useful for pathogen identification in pediatric cervical lymphadenitis, although it is less sensitive in identification of mycobacteria. To our knowledge, our study is the first to report multiple cases of legionellosis in otherwise healthy children. Legionella seems to be a previously unrecognized but relatively common pathogen in culture-negative persistent cervical lymphadenitis in children.