ABSTRACT
OBJECTIVE: Evaluate implementation of an institutional protocol to reduce the time to removal of esophageal button battery (BB) and increase use of mitigation strategies. METHODS: We developed a protocol for esophageal BB management [Zakai's Protocol (ZP)]. All cases of esophageal BB impaction managed at a tertiary care center before and after implementation from 2011 to 2023 were reviewed. Time to BB removal, adherence to critical steps, and use of mitigation strategies (honey/sucralfate, acetic acid) were evaluated. RESULTS: Fifty-one patients (38 pre-ZP, 13 post-ZP) were included. Median age was 2.3 years (IQR 1.3-3.4). After implementation, the time from arrival at the institution to arrival in the operating room (OR) reduced by 4.2 h [4.6 h (IQR 3.9-6.5) to 0.4 h (IQR 0.3-0.6), p < 0.001] and there was improvement in all management steps. The number of referrals direct to otolaryngology increased from 51% to 92%, arrival notification increased from 86% to 100%, avoidance of second x-ray increased from 63% to 100%, and direct transfer to OR increased from 92% to 100%. Adherence to mitigation strategies such as preoperative administration of honey or sucralfate increased from 0% to 38%, intraoperative use of acetic acid from 3% to 77%, and nasogastric tube insertion from 53% to 92%. CONCLUSION: Implementation of ZP substantially reduced the time to BB removal and the use of mitigation strategies in our tertiary care institution. Additional strategies focused on prevention of BB ingestion, and shortening the transfer time to the tertiary care hospital are required to prevent erosive complications. LEVEL OF EVIDENCE: Level 3 Case-series Laryngoscope, 2024.
ABSTRACT
A 15-year-old male with previous open tracheoesophageal fistula (TEF) repair presented with a large, short recurrent TEF. The TEF was denuded with cautery on the tracheal side and the patient was intubated with a cuffed endotracheal tube. Suspension microesophagoscopy allowed excellent exposure of the TEF from the esophageal side, which was cauterized. Four sutures were placed endoscopically from the esophageal side, and the TEF remained closed 6 months postoperatively. Laryngoscope, 134:4114-4117, 2024.
Subject(s)
Esophagoscopy , Suture Techniques , Tracheoesophageal Fistula , Humans , Tracheoesophageal Fistula/surgery , Male , Suture Techniques/instrumentation , Adolescent , Esophagoscopy/methods , Esophagoscopy/instrumentationABSTRACT
INTRODUCTION: Button battery (BB) ingestion injuries are a devastating and preventable event within the pediatric population. Efforts to reduce the prevalence of esophageal button battery ingestion injuries include primary preventative measures. It is integral to assess the public's baseline knowledge about BB injuries to tailor future primary prevention efforts. METHODS: This is a crowdsourcing survey-based study. Participants were notified through our institution's Twitter and Instagram accounts. RESULTS: There were 930 completed survey responses from May to June 2022. The survey found that 87% (791/910) knew that swallowing a BB could cause injury and 71% knew that it could cause death (642/905). Eight-five percent of respondents did not know what signs and symptoms to look for after BB ingestion, only 30% (99/340) of healthcare professionals felt they would know. Only 10.1% (94/930) of participants knew to give children over 12 months old honey after suspected BB ingestion. Thirty-four percent (311/930) knew that complications could still occur even after BB were removed. Seventy-seven percent (719/930) knew that a dead BB could cause injury but only 17% knew the correct way to dispose of a dead button battery (158/930). Only 8% (72/930) of participants were knew that wrapping dead BB in tape could potentially prevent injury. CONCLUSION: The current study reveals gaps in the public's understanding of BB injury including: the presentation of BB injuries; the delayed harm of BB impactions; management and mitigation strategies, and BB disposal methods. This survey provided imperative insights to help guide future education and primary prevention initiatives.
Subject(s)
Esophageal Diseases , Foreign Bodies , Social Media , Child , Humans , Infant , Cross-Sectional Studies , Foreign Bodies/epidemiology , Foreign Bodies/etiology , Foreign Bodies/prevention & control , Esophageal Diseases/complications , Electric Power Supplies , EatingABSTRACT
INTRODUCTION: Flexible nasolaryngoscopy (FNL) is a common, uncomfortable procedure performed to assess the upper airway in infants. Oral sucrose is used during various painful procedures in infants but has not been used during FNL. Our objective was to understand the impact of oral sucrose on discomfort in infants undergoing FNL. METHODS: Infants (<12-months-old) undergoing FNL in the otolaryngology clinic were randomized to treatment (0.5 mL 24% oral sucrose) or standard management (no sucrose). Sucrose was administered <2 min prior to FNL performed by a single endoscopist. Outcome measures included: EVENDOL pain scale and cry duration and visit duration. Infant discomfort was measured by a second observer who was blinded to treatment group. RESULTS: Forty-seven infants were included, 23 were treated with sucrose and 24 with standard management. The median (IQR) age was 3.0 (2-5.7) months. There were no significant differences in age, weight, or sex across groups. The median (IQR) duration of FNL was 35.2 (26.5-58.4) and 36.4 (28.9-51.8) seconds for treatment and standard management groups, respectively. Mean (SD) EVENDOL scores were significantly lower in the sucrose group [4.9 (2.0)] than standard group (6.7 [2.1]) (p = 0.003). Mean cry duration after FNL was significantly shorter in the sucrose group (29.9 [20.4] seconds) than the standard group (52.7.0 [40.6] seconds) (p = 0.02). Median (IQR) visit duration did not differ across groups (1.1 [0.9-1.3] vs. 1.1 [0.7-1.4] h [p = 0.15]). CONCLUSION: Oral sucrose given before FNL reduced EVENDOL scores and cry duration after FNL and did not prolong clinic visits in this randomized pilot study. LEVEL OF EVIDENCE: 2 Laryngoscope, 134:3826-3831, 2024.
Subject(s)
Laryngoscopy , Sucrose , Humans , Pilot Projects , Female , Infant , Sucrose/administration & dosage , Male , Laryngoscopy/methods , Laryngoscopy/adverse effects , Administration, Oral , Pain Measurement , Crying , Single-Blind MethodABSTRACT
OBJECTIVE: Pediatric esophageal foreign bodies (EFBs) are common and can result in serious complications. Little is known about the influence of socioeconomic status (SES) on EFB ingestion in children. The goal was to study SES as a risk factor for dangerous foreign body ingestion and in-hospital complications in children. METHODS: This was a retrospective cohort study of children presenting to a tertiary care pediatric hospital with an esophageal foreign body from 2010 to 2021. SES was assessed for each patient by linking their postal code to the Ontario Marginalization Index to determine a quintile score across four dimensions of deprivation: residential instability, material deprivation, dependency, and ethnic concentration. Dangerous EFBs were defined as magnets, batteries, sharp objects, or bones. In-hospital complications included: intensive care unit admission, prolonged length of stay, and postoperative sequelae. RESULTS: A total of 680 patients were included. Dangerous EFB ingestion was higher for children with increased residential instability (odds ratio [OR], 2.1; 95% confidence interval [CI], 1.2-3.6) and increased material deprivation (OR, 2.2; CI, 1.9-2.8), which was similarly true for odds of complications. Odds of dangerous EFB ingestion were higher in older children (OR, 1.1; CI, 1.0-1.1) and odds of complications were higher in children with comorbidities (OR, 1.1; CI, 1.0-1.3). CONCLUSION: Higher levels of housing instability and material deprivation are associated with dangerous EFB ingestion and complications related to EFB ingestion. These findings emphasize the role that SES plays on child health outcomes and the need for initiatives to mitigate these disparities. LEVEL OF EVIDENCE: 3 Laryngoscope, 134:2945-2953, 2024.
Subject(s)
Esophagus , Foreign Bodies , Socioeconomic Factors , Foreign Bodies/complications , Foreign Bodies/epidemiology , Retrospective Studies , Cohort Studies , Hospitals, Pediatric/statistics & numerical data , Humans , Male , Female , Infant , Child, Preschool , Child , Adolescent , Maternal Deprivation , Housing Instability , Ontario/epidemiologyABSTRACT
OBJECTIVE: Percutaneous tracheostomy is routinely performed in adult patients but is seldomly used in the pediatric population due to concerns regarding safety and limited available evidence. This study aims to consolidate the current literature on percutaneous tracheostomy in the pediatric population. METHODS: A systematic review following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines was conducted. MEDLINE, EMBASE, CINAHL, and Web of Science were searched for studies on pediatric percutaneous tracheostomy (age ≤18). The Joanna Briggs Institute and ROBINS-I tools were used for quality appraisal. RESULTS: Twenty-one articles were included resulting in 143 patients. Patient age ranged from 2 days to 17 years, with the largest subpopulation of patients (n = 57, 40 %) being adolescents (age between 12 and 17 years old). Main indications for percutaneous tracheostomy included prolonged ventilation (n = 6), respiratory insufficiency (n = 5), and upper airway obstruction (n = 5). One-third (n = 47) of percutaneous tracheostomies were completed at the bedside in an intensive care unit. Select studies reported on surgical time and time from intubation to tracheostomy with a mean of 13.8 (SD = 7.8) minutes (n = 27) and 8.9 (SD = 2.8) days (n = 35), respectively. Major postoperative complications included tracheoesophageal fistula (n = 4, 2.8 %) and pneumothorax (n = 3, 2.1 %). There were four conversions to open tracheostomy. CONCLUSION: Percutaneous tracheostomy had a similar risk of complications to open surgical tracheostomy in children and adolescents and can be performed at the bedside in a select group of patients if necessary. However, we feel that consideration must be given to the varying anatomical considerations in children and adolescents compared with adults, and therefore suggest that this procedure be reserved for adolescent patients with a thin body habitus and clearly demarcated and palpable anatomical landmarks who require a tracheostomy. When performed, we strongly support using endoscopic guidance and a surgeon who has the ability to convert to an open tracheostomy if required.
Subject(s)
Tracheostomy , Humans , Tracheostomy/methods , Tracheostomy/adverse effects , Child , Adolescent , Child, Preschool , Infant , Postoperative Complications/epidemiology , Infant, Newborn , Female , MaleABSTRACT
OBJECTIVE: To describe the incidence of respiratory complications, postoperative hemorrhage, length of stay, and cost of care in children with mucopolysaccharidosis (MPS) undergoing adenotonsillectomy (AT). METHODS: Analysis of the 2009, 2012, and 2016 editions of the Healthcare Cost and Utilization Project Kids' Inpatient Database (HCUP KID) identified 24,700 children who underwent AT (40 children with MPS). Demographics, respiratory complications, postoperative hemorrhage, length of stay, and total cost were compared across children with and without MPS. RESULTS: Children with MPS had a higher likelihood of being male (P < 0.017). There was a higher rate of respiratory complications in children with MPS compared with children without MPS [6/40 (15%) vs. 586/24,660 (2.4%), P < 0.001], which remained significant after adjusting for sex [adjusted odds ratio 6.88 (95% CI 2.87-16.46)]. There was also a higher risk of postoperative hemorrhage [4/40 (10%) vs. 444/24,660 (1.8%), P < 0.001), with sex-adjusted odds ratio of 5.97 (95% CI 2.12-16.86). Median (IQR) length of stay was increased in children with MPS (3 days, 1-4) compared with children without MPS (1 day, 1-2, P < 0.001). There was an increase in median (IQR) charges for hospital stay in children with MPS compared with their peers [$33,016 ($23,208.50-$72,280.50 vs. $15,383 ($9937-$24,462), P < 0.001]. CONCLUSIONS: Children with MPS undergoing AT had an increased risk of respiratory complications, postoperative hemorrhage, longer length of stay, and a higher cost of treatment when compared with children without MPS. This information may help inform interventional, perioperative, and postoperative decision making.
Subject(s)
Mucopolysaccharidoses , Sleep Apnea, Obstructive , Tonsillectomy , Child , Humans , Male , Female , Sleep Apnea, Obstructive/surgery , Adenoidectomy , Postoperative Hemorrhage/epidemiology , Postoperative Hemorrhage/etiology , Mucopolysaccharidoses/surgery , Postoperative Complications/epidemiology , Postoperative Complications/surgery , Retrospective StudiesABSTRACT
OBJECTIVES: To determine outcomes following adenotonsillectomy for obstructive sleep apnea (OSA) and the impact of motor and swallowing impairment on respiratory complications in children with Cerebral Palsy (CP). METHODS: A retrospective review of children with CP and sleep disordered breathing (SDB) who underwent adenotonsillectomy (2003-2021) was performed. Children with CP were age-matched to children without CP. Motor and swallowing function was assessed using the Gross Motor Functional Classification System (GMFCS) and the Eating and Drinking Ability Classification System (EDACS). The primary outcome was postoperative obstructive apnea-hypopnea index (OAHI). Secondary outcomes were cure rate, complications, and need for additional interventions. RESULTS: Ninety-seven children with CP were assessed for SDB, and 74 underwent polysomnography. Moderate or severe OSA was found in 49% (36/74). Adenotonsillectomy was performed in 30% (29/97). All children who underwent adenotonsillectomy experienced an initial reduction in OAHI (31.7/h to 2.9/h, p < 0.0001). Children with CP were less likely to achieve an OAHI<1 compared with children without CP (62.5% vs 81.8%, p = 0.23). Children with CP had more postoperative complications (43.5% vs. 8.7%) and greater odds of respiratory complications compared with children without CP (OR 8.9 95% CI 2.1-37.9). Children with CP and a GMFCS score of 5 and EDACS score between 3 and 5 had more respiratory complications post-adenotonsillectomy compared to those with GMFCS<5 (p = 0.002) and EDACS<3 (p = 0.031). CONCLUSION: Children with CP had an improved OAHI initially following adenotonsillectomy but had higher rates of post-adenotonsillectomy complications. Respiratory complications after adenotonsillectomy were more common in children with motor and swallowing impairment. Findings may provide better preoperative planning for caregivers.
ABSTRACT
OBJECTIVE/BACKGROUND: Moderate-to-severe obstructive sleep apnea (OSA) is highly prevalent in children with obesity and/or underlying medical complexity. The first line of therapy, adenotonsillectomy (AT), does not cure OSA in more than 50% of these children. Consequently, continuous positive airway pressure (CPAP) is the main therapeutic option but adherence is often poor. A potential alternative which may be associated with greater adherence is heated high-flow nasal cannula (HFNC) therapy; however, its efficacy in children with OSA has not been systematically investigated. The study aimed to compare the efficacy of HFNC with CPAP to treat moderate-to-severe OSA with the primary outcome measuring the change from baseline in the mean obstructive apnea/hypopnea index (OAHI). PARTICIPANTS/METHODS: This was a single-blinded randomized, two period crossover trial conducted from March 2019 to December 2021 at a Canadian pediatric quaternary care hospital. Children aged 2-18 years with obesity and medical complexity diagnosed with moderate-to-severe OSA via overnight polysomnography and recommended CPAP therapy were included in the study. Following diagnostic polysomnography, each participant completed two further sleep studies; a HFNC titration study and a CPAP titration study (9 received HFNC first, and 9 received CPAP first) in a random 1:1 allocation order. RESULTS: Eighteen participants with a mean ± SD age of 11.9 ± 3.8 years and OAHI 23.1 ± 21.7 events/hour completed the study. The mean [95% CI] reductions in OAHI (-19.8[-29.2, -10.5] vs. -18.8 [-28.2, -9.4] events/hour, p = 0.9), nadir oxygen saturation (7.1[2.2, 11.9] vs. 8.4[3.5, 13.2], p = 0.8), oxygen desaturation index (-11.6[-21.0, -2.3] vs. -16.0[-25.3, -6.6], p = 0.5) and sleep efficiency (3.5[-4.8, 11.8] vs. 9.2[0.9, 15.5], p = 0.2) with HFNC and CPAP therapy were comparable between conditions. CONCLUSION: HFNC and CPAP therapy yield similar reductions in polysomnography quantified measures of OSA severity among children with obesity and medical complexities. TRIAL REGISTRATION: NCT05354401 ClinicalTrials.gov.
Subject(s)
Cannula , Sleep Apnea, Obstructive , Humans , Child , Cross-Over Studies , Canada , Continuous Positive Airway Pressure , Sleep Apnea, Obstructive/therapy , ObesityABSTRACT
OBJECTIVE: To achieve consensus on critical steps and create an assessment tool for actual and simulated pediatric tracheostomy emergencies that incorporates human and systems factors along with tracheostomy-specific steps. METHODS: A modified Delphi method was used. Using REDCap software, an instrument comprising 29 potential items was circulated to 171 tracheostomy and simulation experts. Consensus criteria were determined a priori with a goal of consolidating and ordering 15 to 25 final items. In the first round, items were rated as "keep" or "remove". In the second and third rounds, experts were asked to rate the importance of each item on a 9-point Likert scale. Items were refined in subsequent iterations based on analysis of results and respondents' comments. RESULTS: The response rates were 125/171 (73.1%) for the first round, 111/125 (88.8%) for the second round, and 109/125 (87.2%) for the third round. 133 comments were incorporated. Consensus (>60% participants scoring ≥8, or mean score >7.5) was reached on 22 items distributed across three domains. There were 12, 4, and 6 items in the domains of tracheostomy-specific steps, team and personnel factors, and equipment respectively. CONCLUSIONS: The resultant assessment tool can be used to assess both tracheostomy-specific steps as well as systems factors affecting hospital team response to simulated and clinical pediatric tracheostomy emergencies. The tool can also be used to guide debriefing discussions of both simulated and clinical emergencies, and to spur quality improvement initiatives. LEVEL OF EVIDENCE: 5 Laryngoscope, 133:3588-3601, 2023.
Subject(s)
Emergencies , Tracheostomy , Humans , Child , Consensus , Delphi Technique , Computer SimulationABSTRACT
Objective: Primary ciliary dyskinesia (PCD) is a rare autosomal recessive disorder whereby abnormal cilia cause a wide array of respiratory tract manifestations including chronic rhinosinusitis. The purpose of this study was to determine whether olfaction and gustation are impaired in children with PCD. Study Design: Cross-sectional study. Setting: Tertiary pediatric academic hospital. Methods: Children with confirmed PCD based on having at least 1 of 3 approved diagnostic criteria as per The American Thoracic Society guidelines were recruited from The PCD Clinic in our tertiary care pediatric hospital. Odor identification ability was tested using the Universal Sniff (U-Sniff) test and taste threshold was measured using an electrogustometer. The main outcome of this study is to determine the incidence of olfactory dysfunction in children with PCD and investigate if there is an associated gustatory dysfunction. Results: Twenty-five children participated (14 male, 11 female), The median age was 10.8 years (range: 4.1-17.9 years). Only 4/25 (16%) complained of olfactory dysfunction prior to testing. None of the patients complained of dysgeusia. However, 48% (12/25) scored less than 7 on the U-Sniff, signifying hyposmia or anosmia. In contrast, scores obtained by electrogustometry were in the normal range. There was no correlation between performance on the U-Sniff and electrogustometry testing. Conclusion: Olfactory impairment in children with PCD is common but underrecognized by patients. This is not associated with abnormal gustation. Among other, this places children with PCD at an increased risk with respect to smelling a fire or detecting spoiled or poisonous food.
ABSTRACT
BACKGROUND: Following surgical correction, many patients with esophageal atresia with or without tracheoesophageal fistula (EA/TEF) present to the emergency department (ED) with acute airway complications. We sought to determine the incidence and risk factors for severe acute life-threatening events (ALTEs) in pediatric patients with repaired congenital EA/TEF and the outcomes of operative interventions. METHODS: A retrospective cohort chart review was performed on patients with EA/TEF with surgical repair and follow-up at a single centre from 2000 to 2018. Primary outcomes included 5-year ED visits and/or hospitalizations for ALTEs. Demographic, operative, and outcome data were collected. Chi-square tests and univariate analyses were performed. RESULTS: In total, 266 EA/TEF patients met inclusion criteria. Of these, 59 (22.2%) had experienced ALTEs. Patients with low birth weight, low gestational age, documented tracheomalacia, and clinically significant esophageal strictures were more likely to experience ALTEs (p < 0.05). ALTEs occurred prior to 1 year of age in 76.3% (45/59) of patients with a median age at presentation of 8 months (range 0-51 months). Recurrence of ALTEs after esophageal dilatation was 45.5% (10/22), mostly due to stricture recurrence. Patients experiencing ALTEs received anti-reflux procedures (8/59, 13.6%), airway pexy procedures (7/59, 11.9%), or both (5/59, 8.5%) within a median age of 6 months of life. The resolution and recurrence of ALTEs after operative interventions are described. CONCLUSION: Significant respiratory morbidity is common among patients with EA/TEF. Understanding the multifactorial etiology and operative management of ALTEs have an important role in their resolution. TYPE OF STUDY: Original Research, Clinical Research. LEVEL OF EVIDENCE: Level III Retrospective Comparative Study.
Subject(s)
Esophageal Atresia , Tracheoesophageal Fistula , Humans , Child , Infant, Newborn , Infant , Child, Preschool , Tracheoesophageal Fistula/epidemiology , Tracheoesophageal Fistula/surgery , Tracheoesophageal Fistula/complications , Esophageal Atresia/surgery , Esophageal Atresia/complications , Retrospective Studies , Postoperative Complications/epidemiology , Postoperative Complications/etiology , Postoperative Complications/surgeryABSTRACT
BACKGROUND: Complete and accurate documentation of surgical procedures is essential for optimizing patient care, yet significant variation in operative notes persists within and across institutions. We sought to reach consensus on the most important components of an operative note for pediatric microlaryngoscopy and bronchoscopy. METHODS: A modified Delphi consensus process was used. A checklist for operative documentation, created by fellowship-trained pediatric otolaryngologists-head and neck surgeons, was sent to surgeons identified as experts in pediatric laryngoscopy and bronchoscopy. In the first round, items were rated as "keep" or "remove". In the second round, each item was rated on a 7-point Likert scale for importance. The mean score of each item was calculated to determine if consensus was reached. RESULTS: Overall, 43/74 (58.1%) surgeons responded to our survey. After two rounds of editing, 28 components reached consensus, 24 were near consensus, and 26 did not reach consensus. Items that reached final consensus had mean (SD) ratings of 6.12 (0.94) (range, 5.31-6.72). CONCLUSION: Pediatric otolaryngologists identified as bronchoscopy experts were able to create a checklist of essential components of an operative note for pediatric laryngoscopy and bronchoscopy using a Delphi method. Items reaching consensus included procedure name, description of breathing, grade of airway view, description of normal anatomic structures, grade of subglottic stenosis if present, presence and description of tracheobronchomalacia, presence of fistulae, cleft and rings, and several special cases including foreign body and tracheostomy management, as well as end of procedure disposition and complications. LEVEL OF EVIDENCE: 5 Laryngoscope, 133:1234-1238, 2023.
Subject(s)
Bronchoscopy , Laryngoscopy , Humans , Child , Delphi Technique , Consensus , ChecklistABSTRACT
Pigmented epithelioid melanocytomas (PEM) are intermediate-grade melanocytic lesions with frequent lymph node involvement and rare metastases that tend to follow an indolent course with a favorable outcome. We report two unique cases of congenital PEM with PRKCA fusion transcripts: a multifocal PEM with an aggressive incompletely resectable scalp tumor and a solitary palmar PEM with newly reported ITGB5-PRKCA fusion. Through these case reports and a summary of previously reported cases, we outline the spectrum of disease of PEM and highlight the key clinical and histopathologic features associated with PEM with PRKCA fusion transcripts. We also discuss the treatment options and suggest that surgical excision without further adjuvant systemic treatment is reasonable first-line therapy given the favorable prognosis.
Subject(s)
Nevus, Blue , Skin Neoplasms , Humans , Nevus, Blue/diagnosis , Nevus, Blue/genetics , Skin Neoplasms/diagnosis , Skin Neoplasms/genetics , Skin Neoplasms/pathology , Melanocytes/pathologyABSTRACT
BACKGROUND: Neonates and infants are obligatory nasal breathers. Nasal obstruction can therefore be life-threatening and prompt recognition of its cause is critical. Salivary gland anlage tumor (SGAT) is a rare, benign condition that can present with nasal obstruction and respiratory distress within the first weeks of life. Our objective was to outline the clinical presentation, evaluation, and management of SGAT and provide a review of existing literature. METHODS: A retrospective chart review was performed at a tertiary children's hospital. A comprehensive literature search for SGAT was conducted using Medline, Embase, PubMed, and EBSCO. We present a case from our institution to describe the clinical approach to SGAT. RESULTS: Forty-one cases of previously reported SGAT, plus our new patient, were included. Patients presented with respiratory distress (30/42, 71.4%), feeding difficulty (14/42, 33.3%) and/or upper airway obstruction (13/42, 31.0%). The majority of tumors were in the nasopharynx (25/42, 59.5%). Surgical excision (36/42, 85.7%) was most commonly performed. Surgical approaches have evolved to include transoral, transpalatal, transnasal endoscopic or a combination of the above. Our patient had a suspected residual/recurrent tumor, later found to be negative on pathology. To date, there have been no reported recurrences. CONCLUSIONS: SGAT is a rare pediatric condition that is often excluded from the differential diagnosis of nasal obstruction. Clinical evaluation includes flexible nasopharyngoscopy and neuroimaging. Management involves surgical resection, except in cases of spontaneous expulsion. Tissue pathology is required for definitive diagnosis. Tissue biopsy should be performed on follow-up if there is a suspicion for recurrence.
Subject(s)
Nasal Obstruction , Respiratory Distress Syndrome , Salivary Gland Neoplasms , Infant , Infant, Newborn , Humans , Child , Salivary Gland Neoplasms/diagnosis , Salivary Gland Neoplasms/surgery , Nasal Obstruction/diagnosis , Nasal Obstruction/etiology , Nasal Obstruction/surgery , Retrospective Studies , Neoplasm Recurrence, Local , Salivary Glands/pathology , Respiratory Distress Syndrome/complicationsABSTRACT
Mammalian target of rapamycin (mTOR) inhibitors are clinically effective at treating some complex lymphatic malformations (LM). The mTOR inhibitor rapamycin blocks the phosphoinositide 3-kinase (PI3K) pathway, which is commonly mutated in this condition. Although rapamycin is effective at controlling symptoms of LM, treatment courses are long, not all LMs respond to treatment, and many patients relapse after treatment has stopped. Concurrent rat sarcoma virus (RAS) pathway abnormalities have been identified in LM, which may limit the effectiveness of rapamycin. Protein tyrosine phosphatase-2 (SHP2) controls the RAS pathway upstream, and SHP2 inhibitors are being investigated for treatment of various tumors. The objective of this study was to determine the impact of SHP2 inhibition in combination with rapamycin on LM growth in vitro. Using primary patient cells isolated from a surgically resected LM, we found that combination treatment with rapamycin and the SHP2 inhibitor SHP099 caused a synergistic reduction in cell growth, migration and lymphangiogenesis. These results suggest that combination treatment targeting the PI3K and RAS signaling pathways may result in effective treatment of LMs of the head and neck.
Subject(s)
Endothelial Cells , Phosphatidylinositol 3-Kinases , Phosphatidylinositol 3-Kinases/pharmacology , Signal Transduction , Sirolimus/pharmacology , TOR Serine-Threonine KinasesABSTRACT
Importance: Button batteries (BBs) are commonly found in many household items and present a risk of severe injury to children if ingested. The direct apposition of the trachea and recurrent laryngeal nerves with the esophagus puts children at risk of airway injury secondary to the liquefactive necrotic effects of BB impactions. Objective: To review airway injuries, including long-term sequelae, after BB ingestion in children. Evidence Review: For this systematic review, a comprehensive strategy was designed to search MEDLINE, Embase, Cochrane Database of Systematic Reviews, Web of Science, and CINAHL (Cumulative Index of Nursing and Allied Health Literature) from inception to July 31, 2021, in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) reporting guideline. Additional cases were identified from the National Capital Poison Center BB registry. Individual authors were contacted for additional information. Studies with pediatric patients (<18 years) who developed airway injuries after BB ingestion were included. A total of 195 patients were included in the analysis; 95 were male. The mean (SD) age at BB ingestion was 17.8 (10.2) months. The mean (SD) time from BB ingestion to removal was 5.8 (9.0) days. The 2 most common airway sequelae observed in our series were 155 tracheoesophageal fistulae and 16 unilateral vocal cord paralyses. Twenty-three children had bilateral vocal cord paralysis. The mean (SD) duration of ingestion leading to vocal cord paralysis was shorter than that of the general cohort (17.8 [22.5] hours vs 138.7 [216.7] hours, respectively). Children presenting with airway symptoms were likely to have a subsequent tracheoesophageal fistula or vocal cord paralysis. Conclusions and Relevance: Airway injuries are a severe consequence of BB ingestion, occurring more often in younger children. This systematic review found that tracheoesophageal fistulae and vocal cord paralyses were the 2 most common airway injuries, often requiring tracheostomy. Vocal cord injury occurred after a shorter BB exposure time than other airway injuries. Continued efforts should be directed toward prevention strategies to avoid the devastating sequelae of BB-associated airway injury.
Subject(s)
Foreign Bodies , Tracheoesophageal Fistula , Vocal Cord Paralysis , Child , Electric Power Supplies/adverse effects , Female , Foreign Bodies/complications , Humans , Male , Tracheoesophageal Fistula/etiology , Vocal Cord Paralysis/etiologyABSTRACT
AIM: Most button battery (BB) ingestions in children are unwitnessed leading to prolonged exposures and severe complications. One third of ingestions occur from free BB, that are stored or awaiting disposal. Recommendations have been made to cover the terminals of discarded BB with adhesive tape; however, it is unclear if this practice prevents injury. Our aim was to determine if tape could prevent oesophageal injury in a cadaveric porcine model. METHODS: Electrical, masking, packing and duct tape were compared. One BB was left untaped. Taped BBs were placed in a cadaveric porcine oesophagus controlled for temperature and humidification. Specimens were assessed at 0, 0.5, and hourly for 6 h by visual inspection, temperature and pH. BB voltage was measured before and after testing. All tests were repeated in triplicate. RESULTS: Oesophageal specimens demonstrated burn prevention in the packing and duct tape trials. Burns were seen in 2/3 trials with electrical tape and 3/3 trials with masking tape. pH remained neutral throughout the study for all packing and duct tape specimens. pH remained neutral initially for masking tape but increased rapidly to 12 by 2 h. There was no change in battery voltage for the packing tape and duct tape trials. There was a 16.3% reduction in voltage for masking tape which was similar to controls. CONCLUSIONS: Taping BB with packing tape and duct tape prevented oesophageal burns. This may provide a novel method of burn prevention for loose BB intended for disposal.
Subject(s)
Foreign Bodies , Animals , Cadaver , Eating , Electric Power Supplies/adverse effects , Esophagus/injuries , Foreign Bodies/complications , Foreign Bodies/prevention & control , Humans , SwineABSTRACT
OBJECTIVE: Traditionally, data regarding thyroidectomy were extracted from billing databases, but information may be missed. In this study, a multi-institutional pediatric thyroidectomy database was used to evaluate recurrent laryngeal nerve (RLN) injury and hypoparathyroidism. STUDY DESIGN: Retrospective multi-institutional cohort study. SETTING: Tertiary care pediatric hospital systems throughout North America. METHODS: Data were individually collected for thyroidectomies, then entered into a centralized database and analyzed using univariate and multivariable regression models. RESULTS: In total, 1025 thyroidectomies from 10 institutions were included. Average age was 13.9 years, and 77.8% were female. Average hospital stay was 1.9 nights and 13.5% of patients spent at least 1 night in the pediatric intensive care unit. The most frequent pathology was papillary thyroid carcinoma (42%), followed by Graves' disease (20.1%) and follicular adenoma (18.2%). Overall, 1.1% of patients experienced RLN injury (0.8% permanent), and 7.2% experienced hypoparathyroidism (3.3% permanent). Lower institutional volume (odds ratio [OR], 3.57; 95% CI, 1.72-7.14) and concurrent hypoparathyroidism (OR, 3.51; 95% CI, 1.64-7.53) correlated with RLN injury on multivariable analysis. Graves' disease (OR, 2.27; 95% CI, 1.35-3.80), Hashimoto's thyroiditis (OR, 4.67; 95% CI, 2.39-9.09), central neck dissection (OR, 3.60; 95% CI, 2.36-5.49), and total vs partial thyroidectomy (OR, 7.14; 95% CI, 4.55-11.11) correlated with hypoparathyroidism. CONCLUSION: These data present thyroidectomy information and complications pertinent to surgeons, along with preoperative risk factor assessment. Multivariable analysis showed institutional volume and hypoparathyroidism associated with RLN injury, while hypoparathyroidism associated with surgical indication, central neck dissection, and extent of surgery. Low complication rates support the safety of thyroidectomy in pediatric tertiary care centers.
Subject(s)
Graves Disease , Hypoparathyroidism , Recurrent Laryngeal Nerve Injuries , Thyroid Neoplasms , Humans , Female , Child , Adolescent , Male , Thyroidectomy/methods , Retrospective Studies , Cohort Studies , Recurrent Laryngeal Nerve Injuries/surgery , Thyroid Neoplasms/surgery , Graves Disease/complications , Graves Disease/surgery , Postoperative Complications/surgeryABSTRACT
Auriculocondylar syndrome (ACS) is a rare craniofacial disorder characterized by mandibular hypoplasia and an auricular defect at the junction between the lobe and helix, known as a "Question Mark Ear" (QME). Several additional features, originating from the first and second branchial arches and other tissues, have also been reported. ACS is genetically heterogeneous with autosomal dominant and recessive modes of inheritance. The mutations identified to date are presumed to dysregulate the endothelin 1 signaling pathway. Here we describe 14 novel cases and reassess 25 published cases of ACS through a questionnaire for systematic data collection. All patients harbor mutation(s) in PLCB4, GNAI3, or EDN1. This series of patients contributes to the characterization of additional features occasionally associated with ACS such as respiratory, costal, neurodevelopmental, and genital anomalies, and provides management and monitoring recommendations.