Publications by authors named "Reza Rahbar"

138 Publications

Acid Suppression Does Not Improve Laryngomalacia Outcomes but Treatment for Oropharyngeal Dysphagia Might Be Protective.

J Pediatr 2021 Jun 26. Epub 2021 Jun 26.

Aerodigestive Center, Division of Gastroenterology, Hepatology and Nutrition, Boston Children's Hospital, Boston, MA. Electronic address:

Objective: To determine whether the use of acid suppression and thickened feeds impact laryngomalacia outcomes in infants, including supraglottoplasty risk, time to supraglottoplasty, and hospitalization risk.

Study Design: We performed a retrospective cohort study to compare risk and time with supraglottoplasty and frequency and duration of hospitalizations for infants diagnosed with laryngomalacia at Boston Children's Hospital between January 1 and December 31, 2017. The primary outcomes were supraglottoplasty requirement, time to supraglottoplasty, and hospitalization risk. Multivariate analyses were performed to determine predictors of supraglottoplasty and hospitalization risk after adjusting for laryngomalacia severity and comorbidities in addition to propensity score adjustment. Kaplan-Meier curves were created to determine the impact of acid suppression use on time to supraglottoplasty.

Results: In total, 236 subjects with mean age 62.6 ± 4 days were included in the analysis; 55% were treated with acid suppression. Subjects treated with acid suppression had a greater risk of supraglottoplasty (hazard ratio 3.36, 95% CI 1.36-8.29, P = .009), shorter time to supraglottoplasty (5.64 ± 0.92 vs 7.98 ± 1.92 months, P = .006), and increased respiratory hospitalization risk (relative risk 1.97, 95% CI 1.01-3.85, 0.047), even after adjustment for covariates. Subjects receiving thickening had fewer respiratory hospitalization nights and longer time to supraglottoplasty (9.3 ± 1.7 vs 4.56 ± 0.73 months, P = .004), even after adjustment.

Conclusions: Acid suppression use does not reduce the frequency of supraglottoplasty and related hospitalizations compared with untreated subjects. However, patients treated with thickening have decreased hospitalization and longer time to supraglottoplasty, suggesting that thickening of feeds may be a preferred intervention over acid suppression.
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http://dx.doi.org/10.1016/j.jpeds.2021.06.051DOI Listing
June 2021

Complex Pediatric Otolaryngology Subcertification-Now Is the Time.

JAMA Otolaryngol Head Neck Surg 2021 07;147(7):586-588

Department of Otolaryngology-Head & Neck Surgery, George Washington University, Children's National Hospital, Washington, DC.

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http://dx.doi.org/10.1001/jamaoto.2021.0533DOI Listing
July 2021

Life-Threatening Hemoptysis in a Pediatric Referral Center.

Crit Care Med 2021 03;49(3):e291-e303

Department of Cardiology, Boston Children's Hospital, Boston, MA.

Objectives: Hemoptysis is uncommon in children, even among the critically ill, with a paucity of epidemiological data to inform clinical decision-making. We describe hemoptysis-associated ICU admissions, including those who were critically ill at hemoptysis onset or who became critically ill as a result of hemoptysis, and identify predictors of mortality.

Design: Retrospective cohort study. Demographics, hemoptysis location, and management were collected. Pediatric Logistic Organ Dysfunction-2 score within 24 hours of hemoptysis described illness severity. Primary outcome was inhospital mortality.

Setting: Quaternary pediatric referral center between July 1, 2010, and June 30, 2017.

Patients: Medical/surgical (PICU), cardiac ICU, and term neonatal ICU admissions with hemoptysis during or within 24 hours of ICU admission.

Interventions: No intervention.

Measurements And Main Results: There were 326 hemoptysis-associated ICU admissions in 300 patients. Most common diagnoses were cardiac (46%), infection (15%), bronchiectasis (10%), and neoplasm (7%). Demographics, interventions, and outcomes differed by diagnostic category. Overall, 79 patients (26%) died inhospital and 109 (36%) had died during follow-up (survivor mean 2.8 ± 1.9 yr). Neoplasm, bronchiectasis, renal dysfunction, inhospital hemoptysis onset, and higher Pediatric Logistic Organ Dysfunction-2 score were independent risk factors for inhospital mortality (p < 0.02). Pharmacotherapy (32%), blood products (29%), computerized tomography angiography (26%), bronchoscopy (44%), and cardiac catheterization (36%) were common. Targeted surgical interventions were rare. Of survivors, 15% were discharged with new respiratory support. Of the deaths, 93 (85%) occurred within 12 months of admission. For patients surviving 12 months, 5-year survival was 87% (95% CI, 78-92) and mortality risk remained only for those with neoplasm (log-rank p = 0.001).

Conclusions: We observed high inhospital mortality from hemoptysis-associated ICU admissions. Mortality was independently associated with hemoptysis onset location, underlying diagnosis, and severity of critical illness at event. Additional mortality was observed in the 12-month posthospital discharge. Future directions include further characterization of this vulnerable population and management recommendations for life-threatening pediatric hemoptysis incorporating underlying disease pathophysiology.
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http://dx.doi.org/10.1097/CCM.0000000000004822DOI Listing
March 2021

International Pediatric Otolaryngology Group (IPOG) survey: Efforts to avoid complications in home tracheostomy care.

Int J Pediatr Otorhinolaryngol 2021 Feb 18;141:110563. Epub 2020 Dec 18.

Department of Otolaryngology - Head and Neck Surgery, Massachusetts Eye and Ear Infirmary, Boston, MA, USA. Electronic address:

Objective: To provide guidance for home care tracheostomy management in the pediatric population. The mission of the IPOG is to develop expertise-based recommendations for the management of pediatric otolaryngologic disorders with the goal of improving patient care.

Methods: Survey of expert opinion by the members of the International Pediatric Otolaryngology Group (IPOG).

Results: Survey results provide guidance for caregiver teaching, the reuse of tracheostomies and suction catheters while inpatient and following discharge, acceptable sterilization practices for tracheostomies, tracheitis workup and management, and outpatient follow-up practices.

Conclusion: This presentation of common home tracheostomy care practices are aimed at improving patient-centered care in the pediatric population.
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http://dx.doi.org/10.1016/j.ijporl.2020.110563DOI Listing
February 2021

International Pediatric Otolaryngology Group (IPOG) management recommendations: Pediatric tracheostomy decannulation.

Int J Pediatr Otorhinolaryngol 2021 Feb 15;141:110565. Epub 2020 Dec 15.

Division of Otolaryngology, Children's National Medical Center, George Washington University, Washington DC, USA.

Objectives: To provide recommendations to otolaryngologists, pulmonologists, and allied clinicians for tracheostomy decannulation in pediatric patients.

Methods: An iterative questionnaire was used to establish expert recommendations by the members of the International Pediatric Otolaryngology Group.

Results: Twenty-six members completed the survey. Recommendations address patient criteria for decannulation readiness, airway evaluation prior to decannulation, decannulation protocol, and follow-up after both successful and failed decannulation.

Conclusion: Tracheostomy decannulation recommendations are aimed at improving patient-centered care, quality and safety in children with tracheostomies.
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http://dx.doi.org/10.1016/j.ijporl.2020.110565DOI Listing
February 2021

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

Int J Pediatr Otorhinolaryngol 2021 Jan 18;140:110511. Epub 2020 Nov 18.

Department of Otolaryngology & Communication Enhancement, Boston Children's Hospital, Boston, MA, United States; Department of Otolaryngology, Harvard Medical School, Boston, MA, United States. Electronic address:

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.
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http://dx.doi.org/10.1016/j.ijporl.2020.110511DOI Listing
January 2021

Increased Colonic Expression of ACE2 Associates with Poor Prognosis in Crohn's disease.

bioRxiv 2020 Nov 24. Epub 2020 Nov 24.

Background And Aims: The host receptor for SARS-CoV-2, angiotensin-converting enzyme 2 (ACE2), is highly expressed in small intestine. Our aim was to study colonic ACE2 expression in Crohn's disease (CD) and non-inflammatory bowel disease (non-IBD) controls. We hypothesized that the colonic expression levels of ACE2 impacts CD course.

Methods: We examined the expression of colon using RNA-seq and quantitative (q) RT-PCR from 69 adult CD and 14 NIBD control patients. In a subset of this cohort we validated ACE2 protein expression and localization in formalin-fixed, paraffin-embedded matched colon and ileal tissues using immunohistochemistry. The impact of increased expression in CD for the risk of surgery was evaluated by a multivariate regression analysis and a Kaplan-Meier estimator. To provide critical support for the generality of our findings, we analyzed previously published RNA-seq data from two large independent cohorts of CD patients.

Results: Colonic expression was significantly higher in a subset of adult CD patients (ACE2-high CD). IHC in a sampling of ACE2-high CD patients confirmed high ACE2 protein expression in the colon and ileum compared to ACE2-low CD and NIBD patients. Notably, we found that ACE2-high CD patients are significantly more likely to undergo surgery within 5 years of diagnosis, with a Cox regression analysis finding that high levels is an independent risk factor (OR 2.18; 95%CI, 1.05-4.55; p=0.037).

Conclusion: Increased intestinal expression of ACE2 is associated with deteriorated clinical outcomes in CD patients. These data point to the need for molecular stratification that may impact CD disease-related outcomes.
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http://dx.doi.org/10.1101/2020.11.24.396382DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7709167PMC
November 2020

International Pediatric Otolaryngology Group (IPOG) consensus recommendations: Management of suprastomal collapse in the pediatric population.

Int J Pediatr Otorhinolaryngol 2020 Dec 13;139:110427. Epub 2020 Oct 13.

Section of Pediatric ORL, Department of ORL, University Hospital Center S. Joao, University of Porto Faculty of Medicine and CINTESIS, Porto, Portugal.

Introduction: Suprastomal Collapse (SuStCo) is a common complication of prolonged tracheostomy in children. There is a paucity of literature on this subject, especially regarding how to manage significant suprastomal collapse that prevents safe decannulation.

Objective: Provide a definition, classification system, and recommend management options for significant suprastomal collapse in children with tracheostomy.

Methods: Members of the International Pediatric Otolaryngology Group (IPOG) who are experts in pediatric airway conditions were surveyed and results were refined using a modified Delphi method.

Results: Consensus was defined as > 70% agreement on a subject. The experts achieved consensus: CONCLUSION: This consensus statement provides recommendations for medical specialists who manage infants and children with tracheostomies with significant Suprastomal Collapse. It provides a classification system to facilitate diagnosis and treatment options for this condition.
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http://dx.doi.org/10.1016/j.ijporl.2020.110427DOI Listing
December 2020

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

Laryngoscope 2021 03 20;131(3):E984-E992. Epub 2020 Jul 20.

Department of Otolaryngology & Communication Enhancement, Boston Children's Hospital, Boston, Massachusetts, U.S.A.

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.
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http://dx.doi.org/10.1002/lary.28785DOI Listing
March 2021

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

Laryngoscope 2021 05 9;131(5):1168-1174. Epub 2020 Oct 9.

Department of Otolaryngology, Head and Neck Surgery, Stanford University, Lucile Salter Packard Children's Hospital, Palo Alto, California, U.S.A.

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.
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http://dx.doi.org/10.1002/lary.29126DOI Listing
May 2021

Calcifying synovial sarcoma of the tongue with SS18 rearrangement: a rare variant in a rare location.

Oral Surg Oral Med Oral Pathol Oral Radiol 2021 Nov 20;132(5):e186-e189. Epub 2020 Aug 20.

Department of Oral Medicine Infection and Immunity, Harvard School of Dental Medicine, Boston, MA, USA; Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA.

Synovial sarcoma is a soft tissue malignancy harboring t(X;18) resulting in fusion of two genes SS8 (at 18q11) and SSX (1, 2 or 4 at Xp11) forming the gene fusion product SS18-SSX. It affects adults in their 3rd-4th decades, most frequently in the para-articular regions of the extremities. Less than 10% of the cases occur within the head and neck region and of these, 60% occur in the neck and only 10% occur in the oral cavity. We report a synovial sarcoma of the tongue in a 14-year-old female patient with unusual histology. The patient presented with a mass occupying most of the tongue with extension into the floor of mouth and the lingual gingiva of the anterior mandibular teeth. The tumor was composed of a highly cellular proliferation of spindle cells in a herringbone pattern with many small vessels but without glandular structures, and with extensive calcifications throughout the tumor. Tumor cells were positive for epithelial membrane antigen and transducin-like enhancer of split-1, and fluorescence in situ hybridization studies identified SS18 gene rearrangement. The patient was managed with two debulking procedures followed by chemoradiation and is currently alive with disease.
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http://dx.doi.org/10.1016/j.oooo.2020.08.016DOI Listing
November 2021

Patient and caregiver experiences at a Multidisciplinary Tracheostomy Clinic.

Int J Pediatr Otorhinolaryngol 2020 Oct 11;137:110250. Epub 2020 Jul 11.

Department of Otolaryngology and Communication Enhancement, Boston Children's Hospital, 300 Longwood Avenue, Boston, MA, 02130, USA; Department of Otolaryngology and Head and Neck Surgery, Harvard Medical School, Longwood Avenue, Boston, MA, 02130, USA. Electronic address:

Introduction: Children with tracheostomy are a heterogeneous population requiring care from multiple specialties. Multidisciplinary approaches to treating such patients helps to improve the quality of care they receive. Our institution established a Multidisciplinary Tracheostomy Clinics (MDTC) to address outpatient care coordination for tracheostomy patients by providing care from multiple disciplines at a single visit. We report patient/caregivers' experiences of our MDTC.

Methods: Patients with tracheostomy or their caregivers were prospectively recruited between Dec 2017-Oct 2019 to complete surveys assessing their experience at the MDTC. Demographic and satisfaction questionnaires were sent electronically by a REDCap survey distribution tool. Demographic data were collected, such as patient's residence and education level. Medical care variables assessed included history of MDTC attendance, commute time, medical specialties seen, tracheostomy "Go-Bag" use, home-care nursing, and MDTC satisfaction ratings.

Results: Twenty-nine patients/caregivers completed the satisfaction survey and 22 completed both the satisfaction survey and demographics questionnaire. Patient ages ranged from 11 months to 36 years. Twenty-three (79%) participants commuted for up to 2 h to the MDTC, and 6 (21%) commuted for more than 2 h. The median number of medical specialties seen at the MDTC was 3. All participants were satisfied that they saw all requested specialties. Tracheostomy supplies were checked for 25 of 28 patients. Twenty-three of 28 subjects rated staff teamwork as "excellent." Twenty-four of 28 patients were "highly likely" to recommend the MDTC. Twenty-three of 28 participants were "highly likely" to return, and 4 were "somewhat likely" to return.

Conclusion: This study demonstrates that patients with tracheostomy and caregivers were satisfied with the improved coordination and facilitation of care through a Multidisciplinary Tracheostomy Clinic.
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http://dx.doi.org/10.1016/j.ijporl.2020.110250DOI Listing
October 2020

International Pediatric Otolaryngology Group (IPOG): Consensus recommendations on the prenatal and perinatal management of anticipated airway obstruction.

Int J Pediatr Otorhinolaryngol 2020 Nov 8;138:110281. Epub 2020 Aug 8.

Department of Otolaryngology, University of Iowa Hospitals and Clinics, Iowa City, IA, USA.

Objective: To make recommendations on the identification, routine evaluation, and management of fetuses at risk for airway compromise at delivery.

Methods: Recommendations are based on expert opinion by members of the International Pediatric Otolaryngology Group (IPOG). A two-iterative Delphi method questionnaire was distributed to all members of the IPOG and responses recorded. The respondents were given the opportunity to comment on the content and format of the survey, which was modified for the second round. "Consensus" was defined by >80% respondent affirmative responses, "agreement" by 51-80% affirmative responses, and "no agreement" by 50% or less affirmative responses.

Results: Recommendations are provided regarding etiologies of perinatal airway obstruction, imaging evaluation, adjunct evaluation, multidisciplinary team and decision factors, micrognathia management, congenital high airway obstruction syndrome management, head and neck mass management, attended delivery procedure, and delivery on placental support procedure.

Conclusions: Thorough evaluation and thoughtful decision making are required to optimally balance fetal and maternal risks/benefits.
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http://dx.doi.org/10.1016/j.ijporl.2020.110281DOI Listing
November 2020

International Pediatric Otolaryngology group (IPOG) consensus on the diagnosis and management of pediatric obstructive sleep apnea (OSA).

Int J Pediatr Otorhinolaryngol 2020 Nov 5;138:110276. Epub 2020 Aug 5.

Antwerp University of Antwerp, Department of Otolaryngology Head and Neck Surgery, University of Antwerp, Antwerp, Belgium. Electronic address:

Objective: To develop an expert-based consensus of recommendations for the diagnosis and management of pediatric obstructive sleep apnea.

Methods: A two-iterative Delphi method questionnaire was used to formulate expert recommendations by the members of the International Pediatric Otolaryngology Group (IPOG).

Results: Twenty-six members completed the survey. Consensus recommendations (>90% agreement) are formulated for 15 different items related to the clinical evaluation, diagnosis, treatment, postoperative management and follow-up of children with OSA.

Conclusion: The recommendations formulated in this IPOG consensus statement may be used along with existing clinical practice guidelines to improve the quality of care and to reduce variation in care for children with OSA.
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http://dx.doi.org/10.1016/j.ijporl.2020.110276DOI Listing
November 2020

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

Int J Pediatr Otorhinolaryngol 2020 Nov 29;138:110283. Epub 2020 Jul 29.

Department of Otolaryngology & Communication Enhancement, Boston Children's Hospital, Boston, MA, 02115, USA; Department of Otolaryngology, Harvard Medical School, Boston, MA, 02115, USA. Electronic address:

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.
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http://dx.doi.org/10.1016/j.ijporl.2020.110283DOI Listing
November 2020

Decreased Colonic Activin Receptor-Like Kinase 1 Disrupts Epithelial Barrier Integrity in Patients With Crohn's Disease.

Cell Mol Gastroenterol Hepatol 2020 16;10(4):779-796. Epub 2020 Jun 16.

Center for Gastrointestinal Biology and Disease, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina. Electronic address:

Background & Aims: Intestinal epithelial cell (IEC) barrier dysfunction is critical to the development of Crohn's disease (CD). However, the mechanism is understudied. We recently reported increased microRNA-31-5p (miR-31-5p) expression in colonic IECs of CD patients, but downstream targets and functional consequences are unknown.

Methods: microRNA-31-5p target genes were identified by integrative analysis of RNA- and small RNA-sequencing data from colonic mucosa and confirmed by quantitative polymerase chain reaction in colonic IECs. Functional characterization of activin receptor-like kinase 1 (ACVRL1 or ALK1) in IECs was performed ex vivo using 2-dimensional cultured human primary colonic IECs. The impact of altered colonic ALK1 signaling in CD for the risk of surgery and endoscopic relapse was evaluated by a multivariate regression analysis and a Kaplan-Meier estimator.

Results: ALK1 was identified as a target of miR-31-5p in colonic IECs of CD patients and confirmed using a 3'-untranslated region reporter assay. Activation of ALK1 restricted the proliferation of colonic IECs in a 5-ethynyl-2-deoxyuridine proliferation assay and down-regulated the expression of stemness-related genes. Activated ALK1 signaling increased colonic IEC differentiation toward colonocytes. Down-regulated ALK1 signaling was associated with increased stemness and decreased colonocyte-specific marker expression in colonic IECs of CD patients compared with healthy controls. Activation of ALK1 enhanced epithelial barrier integrity in a transepithelial electrical resistance permeability assay. Lower colonic ALK1 expression was identified as an independent risk factor for surgery and was associated with a higher risk of endoscopic relapse in CD patients.

Conclusions: Decreased colonic ALK1 disrupted colonic IEC barrier integrity and was associated with poor clinical outcomes in CD patients.
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http://dx.doi.org/10.1016/j.jcmgh.2020.06.005DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7502566PMC
June 2020

Crohn's Disease Differentially Affects Region-Specific Composition and Aerotolerance Profiles of Mucosally Adherent Bacteria.

Inflamm Bowel Dis 2020 11;26(12):1843-1855

Department of Genetics, UNC at Chapel Hill, Chapel Hill, North Carolina, USA.

Background: The intestinal microbiota play a key role in the onset, progression, and recurrence of Crohn disease (CD). Most microbiome studies assay fecal material, which does not provide region-specific information on mucosally adherent bacteria that directly interact with host systems. Changes in luminal oxygen have been proposed as a contributor to CD dybiosis.

Methods: The authors generated 16S rRNA data using colonic and ileal mucosal bacteria from patients with CD and without inflammatory bowel disease. We developed profiles reflecting bacterial abundance within defined aerotolerance categories. Bacterial diversity, composition, and aerotolerance profiles were compared across intestinal regions and disease phenotypes.

Results: Bacterial diversity decreased in CD in both the ileum and the colon. Aerotolerance profiles significantly differed between intestinal segments in patients without inflammatory bowel disease, although both were dominated by obligate anaerobes, as expected. In CD, high relative levels of obligate anaerobes were maintained in the colon and increased in the ileum. Relative abundances of similar and distinct taxa were altered in colon and ileum. Notably, several obligate anaerobes, such as Bacteroides fragilis, dramatically increased in CD in one or both intestinal segments, although specific increasing taxa varied across patients. Increased abundance of taxa from the Proteobacteria phylum was found only in the ileum. Bacterial diversity was significantly reduced in resected tissues of patients who developed postoperative disease recurrence across 2 independent cohorts, with common lower abundance of bacteria from the Bacteroides, Streptococcus, and Blautia genera.

Conclusions: Mucosally adherent bacteria in the colon and ileum show distinct alterations in CD that provide additional insights not revealed in fecal material.
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http://dx.doi.org/10.1093/ibd/izaa103DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7676424PMC
November 2020

International Pediatric ORL Group (IPOG) Robin Sequence consensus recommendations.

Int J Pediatr Otorhinolaryngol 2020 Mar 28;130:109855. Epub 2019 Dec 28.

Department of Plastic and Oral Surgery, Boston Children's Hospital, Boston, MA, USA.

Objective: To provide recommendations for the comprehensive management of airway obstruction in patients with Robin Sequence.

Methods: Expert opinion by the members of the International Pediatric Otolaryngology Group (IPOG).

Results: The consensus statement provides recommendations for medical specialists who manage infants with Robin Sequence including: evaluation and treatment considerations for commonly debated issues in post-natal airway obstruction, assessment of antenatal obstruction and perinatal airway management.

Conclusion: Consensus recommendations are aimed at improving management of airway obstruction in patients with Robin Sequence.
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http://dx.doi.org/10.1016/j.ijporl.2019.109855DOI Listing
March 2020

Carbon dioxide laser versus cold-steel supraglottoplasty: A comparison of post-operative outcomes.

Int J Pediatr Otorhinolaryngol 2020 Mar 24;130:109843. Epub 2019 Dec 24.

Department of Otolaryngology and Communication Enhancement, Boston Children's Hospital, USA; Department of Otology and Laryngology, Harvard Medical School, USA.

Objective: Supraglottoplasty is the mainstay of surgical treatment for laryngomalacia, and is commonly performed via two methods: cold steel or carbon dioxide (CO) laser. The degree of post-operative monitoring following supraglottoplasty varies, both within and between institutions. The aim of this study was to compare the post-operative monitoring and interventions required by patients undergoing cold-steel versus CO laser supraglottoplasty.

Design: Retrospective cohort of pediatric patients (age < 18 years) undergoing supraglottoplasty at a tertiary care pediatric hospital. The primary exposure was the surgical instrument(s) used during supraglottoplasty. The primary outcome was prolonged intensive care unit (ICU)-stay (defined as >24 h).

Results: 155 cases were eligible for inclusion. Fifty-eight (37.4%) patients had a comorbid condition. Common indications for surgery included feeding difficulty (56.1%), severe respiratory distress (33.5%), and obstructive sleep apnea (25.2%). CO laser was employed in 49 cases and cold-steel in 106 cases. Prolonged ICU-stay (>24 h) was observed in 14 CO laser cases (28.6%) and 11 cold-steel cases (10.4%) (adjusted OR 3.42; 95% CI 1.43, 8.33). CO laser cases were more likely to require post-operative intubation, non-invasive positive pressure ventilation, and nebulized racemic epinephrine. Concomitant neurological condition was associated with an increased risk of prolonged ICU-stay, while extent of surgery and age were not.

Conclusions: CO laser supraglottoplasty is associated with an increased risk of prolonged ICU-stay and need for ICU-level airway intervention, compared to the cold-steel technique. While this association should not be misconstrued as a causal relationship, the current study demonstrates that specific surgical factors may influence the patient monitoring requirements following supraglottoplasty, particularly the choice of instrument and the extent of surgery.
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http://dx.doi.org/10.1016/j.ijporl.2019.109843DOI Listing
March 2020

Laryngeal Web in the Pediatric Population: Evaluation and Management.

Otolaryngol Head Neck Surg 2020 Feb 17;162(2):234-240. Epub 2019 Dec 17.

Department of Otolaryngology, Children's National Medical Center, Washington, DC, USA.

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.
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http://dx.doi.org/10.1177/0194599819893985DOI Listing
February 2020

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

Int J Pediatr Otorhinolaryngol 2020 Mar 29;130:109799. Epub 2019 Nov 29.

Department of Cardiology, Boston Children's Hospital, Boston, MA, 02115, USA; Department of Cardiac Surgery, Boston Children's Hospital, Boston, MA, 02115, USA; Department of Surgery, Harvard Medical School, Boston, MA, 02115, USA. Electronic address:

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.
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http://dx.doi.org/10.1016/j.ijporl.2019.109799DOI Listing
March 2020

International Pediatric Otolaryngology Group (IPOG): Juvenile-onset recurrent respiratory papillomatosis consensus recommendations.

Int J Pediatr Otorhinolaryngol 2020 Jan 28;128:109697. Epub 2019 Sep 28.

Department Pediatric Surgery, Bambino Gesù Pediatric Hospital, Rome, Italy.

Objectives: To develop consensus recommendations for the evaluation and management of juvenile-onset recurrent respiratory papillomatosis (JORRP) in pediatric patients.

Methods: Expert opinion by the members of the International Pediatric Otolaryngology Group (IPOG). The mission of the IPOG is to develop expertise-based consensus recommendations for the management of pediatric otolaryngologic disorders with the goal of improving patient care. The consensus recommendations herein represent the first publication by the group.

Results: Consensus recommendations including diagnostic considerations, surgical management, systemic adjuvant therapies, postoperative management, surveillance, and voice evaluation. These recommendations are based on the collective opinion of the IPOG members and are targeted for otolaryngologists, primary care providers, pulmonologists, infectious disease specialists, and any other health care providers that manage patients with JORRP.

Conclusions: Pediatric JORRP consensus recommendations are aimed at improving care and outcomes in this patient population.
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http://dx.doi.org/10.1016/j.ijporl.2019.109697DOI Listing
January 2020

Association of a Multidisciplinary Care Approach With the Quality of Care After Pediatric Tracheostomy.

JAMA Otolaryngol Head Neck Surg 2019 Nov;145(11):1035-1042

Department of Otolaryngology and Communication Enhancement, Boston Children's Hospital, Boston, Massachusetts.

Importance: Incidence of tracheostomy placement in children is increasing, and these children continue to have high incidences of morbidity and mortality. A multidisciplinary tracheostomy program may help improve the quality of care received by these patients.

Objective: To determine whether implementation of a multidisciplinary tracheostomy program can improve the care of children who received a tracheostomy through reduction in tracheostomy-related adverse events (TRAEs), improved tracheostomy education, and caregiver preparedness.

Design, Setting, And Participants: A prospective cohort study was conducted from January 2015 to June 2018 at a pediatric tertiary referral center in Boston, Massachusetts. The participants included 700 children who had received a tracheostomy, most of whom were aged birth to 18 years, but some patients with congenital disorders were much older.

Exposures: Institution of a multidisciplinary tracheostomy team (MDT) whose activities included conducting staff meetings, organizing outpatient clinics, conducting inpatient tracheostomy ward rounds, and conducting inpatient tracheostomy rounds at a local rehabilitation hospital. Quality improvement initiatives included monitoring standardized TRAEs and distributing standardized tracheostomy "go-bags."

Main Outcomes And Measures: Reduction of TRAEs and improved caregiver preparedness through distribution of tracheostomy go-bags were assessed following the establishment of a multidisciplinary tracheostomy program.

Results: In total, 700 children who had received a tracheostomy during the study period were actively followed up by the MDT. Of these children, 378 (54.0%) were males and 322 (46.0%) were females; mean (SD) age was 4.1 (6.1) years. More than 60 new pediatric tracheostomies were performed annually at the referral center. Reported TRAEs were reduced by 43.0% from the first to the third year after the implementation of a standardized, closed-loop monitoring system (from a mean [SD] of 6.1 [5.2] TRAEs per 1000 inpatient tracheostomy-days in 2015 to a mean [SD] of 4.0 [2.5] in 2018). The most common TRAE was unplanned decannulation, which occurred 64 times during the study period. On average, 10 patients were seen in each monthly multidisciplinary tracheostomy clinic. Clinic interventions included continuing care (146 [52.5%]), communication enhancement (67 [23.6%]), plans for decannulation (52 [18.6%]), and referrals for comorbidities (13 [4.6%]). Approximately 19 inpatients were seen during biweekly rounds and 8 during monthly rounds at a local rehabilitation hospital. A total of 297 patients received standardized tracheostomy go-bags, and more than 70 positive bag checks were performed in the monthly MDT clinics. A positive bag check refers to the incidence when a family is given a go-bag and also uses it. In contrast, a negative bag check refers to when a family is given a go-bag but neither brings it to the clinic nor acknowledges that they use it.

Conclusions And Relevance: This study's findings suggest that a multidisciplinary tracheostomy program may be a powerful tool for enhancing patient safety and quality improvement. Ongoing studies will develop measurable pediatric tracheostomy outcome metrics and assess long-term outcomes.
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http://dx.doi.org/10.1001/jamaoto.2019.2500DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6753653PMC
November 2019

Preoperative augmentative and alternative communication enhancement in pediatric tracheostomy.

Laryngoscope 2020 07 5;130(7):1817-1822. Epub 2019 Sep 5.

Department of Otolaryngology and Communication Enhancement, Boston Children's Hospital, Boston, Massachusetts.

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.
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http://dx.doi.org/10.1002/lary.28288DOI Listing
July 2020

Rectal-prolapse repair in men is safe, but outcomes are not well understood.

Gastroenterol Rep (Oxf) 2019 Aug 9;7(4):279-282. Epub 2019 May 9.

University of Iowa, Iowa City, IA, USA.

Introduction: Rectal prolapse is a condition that occurs infrequently in men and there is little literature guiding treatment in this population. The purpose of this study was to evaluate the surgical approach and outcomes of rectal-prolapse repair in men.

Methods: A retrospective multicenter review was conducted of consecutive men who underwent rectal-prolapse repair between 2004 and 2014. Surgical approaches and outcomes, including erectile function and fecal continence, were evaluated.

Results: During the study period, 58 men underwent rectal-prolapse repair and the mean age of repair was 52.7 ± 24.1 years. The mean follow-up was 13.2 months (range, 0.5-117 months). The majority of patients underwent endoscopic evaluation (78%), but few patients underwent anal manometry (16%), defecography (9%) or ultrasound (3%). Ten patients (17%) underwent biofeedback/pelvic-floor physical therapy prior to repair. Nineteen patients (33%) underwent a perineal approach (most were perineal proctosigmoidectomy). Thirty-nine patients (67%) underwent repair using an abdominal approach (all were suture rectopexy) and, of these, 77% were completed using a minimally invasive technique. The overall complication rate was 26% including urinary retention (16%), which was more common in patients undergoing the perineal approach (32% vs. 8%,  = 0.028), urinary-tract infection (7%) and wound infection (3%). The overall recurrence rate was 9%, with no difference between abdominal and perineal approaches. Information on sexual function was missing in the majority of patients  both before and after surgery (76% and 78%, respectively).

Conclusion:  Rectal-prolapse repair in men is safe and has a low recurrence rate; however, sexual function was poorly recorded across all institutions. Further studies are needed to evaluate to best approach to and functional outcomes of rectal-prolapse repair in men.
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http://dx.doi.org/10.1093/gastro/goz016DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6688730PMC
August 2019

International Pediatric Otolaryngology Group (IPOG) consensus recommendations: Diagnosis, pre-operative, operative and post-operative pediatric choanal atresia care.

Int J Pediatr Otorhinolaryngol 2019 Aug 13;123:151-155. Epub 2019 May 13.

La Timone Children's Hospital, Aix-Marseille Université, Marseille, France.

Objective: To provide recommendations to otolaryngologists and allied physicians for the comprehensive management of young infants who present with signs or symptoms of choanal atresia.

Methods: A two-iterative delphi method questionnaire was used to establish expert recommendations by the members of the International Otolaryngology Group (IPOG), on the diagnostic, intra-operative, post-operative and revision surgery considerations.

Results: Twenty-eight members completed the survey, in 22 tertiary-care center departments representing 8 countries. The main consensual recommendations were: nasal endoscopy or fiberscopy and CT imaging are recommended for diagnosis; unilateral choanal atresia repair should be delayed after at least age 6 months whenever possible; transnasal endoscopic repair is the preferred technique; long term follow-up is recommended (minimum one year) using nasal nasofiberscopy or rigid endoscopy, without systematic imaging.

Conclusion: Choanal atresia care consensus recommendations are aimed at improving patient-centered care in neonates, infants and children with choanal atresia.
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http://dx.doi.org/10.1016/j.ijporl.2019.05.010DOI Listing
August 2019

Innovative management of severe tracheobronchomalacia using anterior and posterior tracheobronchopexy.

Laryngoscope 2020 02 25;130(2):E65-E74. Epub 2019 Mar 25.

Department of Otolaryngology and Communication Enhancement, Boston Children's Hospital, Boston, Massachusetts, U.S.A.

Objectives/hypothesis: Combined anterior and posterior tracheobronchopexy is a novel surgical approach for the management of severe tracheobronchomalacia (TBM). We present our institutional experience with this procedure. Our objective was to determine the utility and safety of anterior and posterior tracheopexy in the treatment of severe TBM.

Study Design: Retrospective chart review.

Methods: All patients who underwent anterior and posterior tracheopexy from January 2013 to July 2017 were retrospectively reviewed. Charts were reviewed for indications, preoperative work-up, tracheobronchomalacia classification and severity, procedure, associated syndromes, synchronous upper aerodigestive tract lesions, and aberrant thoracic vessels. Main outcomes measured included improvement in respiratory symptoms, successful extubation and/or decannulation, vocal fold immobility, and new tracheotomy placement.

Results: Twenty-five patients underwent anterior and posterior tracheopexy at a mean age of 15.8 months (range, 2-209 months; mean, 31 months if 2 outliers of 206 and 209 months included). Mean length of follow-up was 26.8 months (range, 13-52 months). Indications for surgery included apneic events, ventilator dependence, need for positive pressure ventilation, tracheotomy dependence secondary to TBM, recurrent pneumonia, and exercise intolerance. Many patients had other underlying syndromes and synchronous upper aerodigestive tract lesions (8 VACTERL, 2 CHARGE, 1 trisomy 21, 1 Feingold syndrome, 17 esophageal atresia/tracheoesophageal fistula, 20 cardiac/great vessel anomalies, 1 subglottic stenosis, 1 laryngomalacia, 7 laryngeal cleft). At preoperative bronchoscopy, 21 of 25 patients had >90% collapse of at least one segment of their trachea, and the remaining four had 70% to 90% collapse. Following anterior and posterior tracheopexy, one patient developed new bilateral vocal-fold immobility; one patient with a preoperative left cord paralysis had a new right vocal-fold immobility. Postoperatively, most patients had significant improvement in their respiratory symptoms (21 of 25, 84%) at most recent follow-up. Three patients with preexisting tracheotomy were decannulated; two patients still had a tracheotomy at last follow-up. Two patients required new tracheotomy for bilateral vocal-fold immobility.

Conclusions: Combined anterior and posterior tracheopexy is a promising new technique for the surgical management of severe TBM. Further experience and longer follow-up are needed to validate this contemporary approach and to minimize the risk of recurrent laryngeal nerve injury.

Level Of Evidence: 4 Laryngoscope, 130:E65-E74, 2020.
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http://dx.doi.org/10.1002/lary.27938DOI Listing
February 2020

Mucoepidermoid carcinoma of the head and neck in children.

Int J Pediatr Otorhinolaryngol 2019 May 10;120:93-99. Epub 2019 Feb 10.

Department of Otolaryngology & Communication Enhancement, Boston Children's Hospital, Boston, MA, 02115, United States; Department of Otolaryngology, Harvard Medical School, Boston, MA, 02115, United States. Electronic address:

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.
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http://dx.doi.org/10.1016/j.ijporl.2019.02.020DOI Listing
May 2019

Diagnosis, Classification, and Management of Pediatric Tracheobronchomalacia: A Review.

JAMA Otolaryngol Head Neck Surg 2019 Mar;145(3):265-275

Department of Surgery, Boston Children's Hospital, Boston, Massachusetts.

Importance: Tracheobronchomalacia (TBM) describes an increased collapsibility of the trachea and bronchi that is greatest on forced expiration. A broad term, TBM encompasses intrinsic tracheal weakness, some forms of tracheal deformation, and extrinsic compression. Tracheobronchomalacia is the most common congenital tracheal anomaly, affecting 1 in 2100 children. Tracheobronchomalacia is often associated with recurrent and prolonged respiratory tract infections, can lead to chronic lung disease, and can be fatal in its most severe form. Tracheobronchomalacia is often associated with other congenital anomalies and syndromes.

Observations: There is a paucity of information on TBM treatment in the modern otolaryngology literature. The primary treatment modalities described include tracheotomy, tracheal stents, and anterior aortopexy. In this review, a new TBM classification scheme and new treatment strategies are introduced to the otolaryngology literature. Diagnosis is made through history and physical examination, dynamic airway computed tomography, and dynamic 3-phase tracheobronchoscopy. Medical management includes nebulizer treatments, minimal use of inhaled corticosteroids, gastroesophageal reflux disease therapy, and continuous positive airway pressure. Surgical techniques, including anterior and posterior tracheobronchopexy and anterior and posterior aortopexy, are described.

Conclusions And Relevance: Tracheobronchomalacia is an entity of relevance to pediatric otolaryngologists and should be considered as being associated with respiratory distress, stridor, cough, recurrent pneumonia, or feeding difficulties, especially in children with syndromes or other congenital anomalies. A multidisciplinary approach to these patients is essential. A classification scheme facilitates discussion of individual patients among health care professionals and guides appropriate management. Novel surgical approaches for the treatment of TBM, including anterior and posterior tracheopexy and aortopexy, may be considered in management of the treatment of children with symptomatic TBM.
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http://dx.doi.org/10.1001/jamaoto.2018.3276DOI Listing
March 2019

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

JAMA Otolaryngol Head Neck Surg 2019 02;145(2):146-151

Department of Otolaryngology and Communication Enhancement, Boston Children's Hospital, Boston, Massachusetts.

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.
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http://dx.doi.org/10.1001/jamaoto.2018.3642DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6439583PMC
February 2019
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