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This is the first description of Negative Pressure Wound Therapy (NPWT) use in rescuing alloplastic ear reconstructions. Previously considered a sign of certain impending failure, severe infections with frank purulence can be salvaged. As a more recently developed technique, porous polyethylene (PPE) ear reconstruction provides benefits when compared to rib techniques. Increasing surgeon awareness of complication management may lead to further adoption of the technique and improve reconstructive results. Described herein are two cases of draining infections following PPE ear reconstruction that were salvaged with satisfactory results. Laryngoscope, 134:4122-4125, 2024.
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Terapia de Presión Negativa para Heridas , Procedimientos de Cirugía Plástica , Terapia Recuperativa , Humanos , Terapia de Presión Negativa para Heridas/métodos , Terapia Recuperativa/métodos , Procedimientos de Cirugía Plástica/métodos , Masculino , Persona de Mediana Edad , Infecciones Relacionadas con Prótesis/terapia , Infecciones Relacionadas con Prótesis/cirugía , Infecciones Relacionadas con Prótesis/etiología , Femenino , Polietileno , AdultoRESUMEN
OBJECTIVE: Families preferring to receive care in a language-other-than-English have disparities in access to care. We studied the effect of implementing an ambulatory outbound scheduling team on the timeliness of scheduling referrals to pediatric otolaryngology. We hypothesized this intervention could increase access to care. STUDY DESIGN: Retrospective cohort analysis. SETTING: Tertiary care academic center. METHODS: Data were abstracted from the hospital's enterprise database for patients referred to Otolaryngology over 3 years (October 2019-August 2022; 7675 referrals). An outbound scheduling team was created April 2021 and tasked with calling out to schedule referrals within one business day of receipt. Referral lag was compared across patient cohorts before and after the scheduling intervention. Log-transformed linear regression models were used to assess the impact of the scheduling intervention on referral lag for language cohorts. RESULTS: The median preintervention referral lag was 6 days (interquartile range [IQR] 2-18), which was reduced to 1 day postintervention (IQR 0-5; P < .001). Preintervention language-other-than-English families had a median referral lag of 8 days (IQR 2-23), which was 1.27 times higher than for patients speaking English (P < .001). With implementation of the scheduling intervention, language-other-than-English families were scheduled in a median of 1 day (IQR 0-6), and the disparity in timeliness of scheduling was eliminated (P = .131). Postintervention, referral lag was reduced by 58% in the English and 64% in the language other than English cohorts. CONCLUSION: Implementation of an outbound ambulatory scheduling process reduces referral lag for all patients and eliminated a disparity in referral lag for language-other-than-English families.
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Citas y Horarios , Otolaringología , Derivación y Consulta , Humanos , Derivación y Consulta/organización & administración , Estudios Retrospectivos , Otolaringología/organización & administración , Niño , Factores de Tiempo , Femenino , Accesibilidad a los Servicios de Salud/organización & administración , Masculino , Preescolar , LenguajeRESUMEN
OBJECTIVE: Postoperative pain is the most common morbidity associated with tonsillectomy. Opioids are frequently used in multimodal posttonsillectomy analgesia regimens; however, concerns regarding respiratory depression, drug-drug interactions, and medication misuse necessitate responsible opioid stewardship among prescribing surgeons. It is unclear if intentionally reducing opioid prescription doses negatively affects the patient experience. METHODS: A quality improvement team reviewed all posttonsillectomy opioid prescriptions at a pediatric ambulatory surgery center between January and June 2021 (preintervention, 163 patients). Following this review, we performed an opioid education session for surgeons and studied opioid prescribing habits between July and December 2021 (Plan-Do-Study-Act [PDSA] 1, 152 patients). We then implemented a standardized prescription protocol of 7 doses of oxycodone per patient and again reviewed prescriptions between January and June 2022 (PDSA 2, 178 patients). The following measures were evaluated: initial number of opioid doses prescribed, need for refills, 7-day emergency department (ED) visits, and readmissions. RESULTS: Each intervention reduced the average number of initial oxycodone doses per patient (12.2 vs 9.2 vs 6.9 doses, P < .001). There were no changes in the rate of refill requests, 7-day ED visits, and readmissions, by descriptive or Statistical Process Control analyses. DISCUSSION: In 2 PDSA cycles, we achieved a 43% reduction in the number of doses of oxycodone prescribed following tonsillectomy. We did not observe any increased rates in balancing measures, which are surrogates for unintentional effects of PDSA changes, including refills, ED presentations, and readmission rates. IMPLICATIONS FOR PRACTICE: Directed provider education and standardized posttonsillectomy prescription protocols can safely decrease postoperative opioid prescribing. Further PDSA cycles are required to consider even fewer opioid prescription doses.
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Analgésicos Opioides , Oxicodona , Humanos , Niño , Analgésicos Opioides/uso terapéutico , Oxicodona/uso terapéutico , Mejoramiento de la Calidad , Pautas de la Práctica en Medicina , Dolor Postoperatorio/tratamiento farmacológicoRESUMEN
OBJECTIVE: Surgical treatment of velopharyngeal insufficiency (VPI) includes a wide array of procedures. The purpose of this study was to develop a classification for VPI procedures and to describe variations in how they are performed.Design/participants/setting/outcomes: We completed an in-depth review of the literature to develop a preliminary schema that encompassed existing VPI procedures. Forty-one cleft surgeons from twelve hospitals across the USA and Canada reviewed the schema and either confirmed that it encompassed all VPI procedures they performed or requested additions. Two surgeons then observed the conduct of the procedures by surgeons at each hospital. Standardized reports were completed with each visit to further explore the literature, refine the schema, and delineate the common and unique aspects of each surgeon's technique. RESULTS: Procedures were divided into three groups: palate-based surgery; pharynx-based surgery; and augmentation. Palate-based operations included straight line mucosal incision with intravelar veloplasty, double-opposing Z-plasty, and palate lengthening with buccal myomucosal flaps. Many surgeons blended maneuvers from these three techniques, so a more descriptive schema was developed classifying the maneuvers employed on the oral mucosa, nasal mucosa, and muscle. Pharynx-based surgery included pharyngeal flap and sphincter pharyngoplasty, with variations in design for each. Augmentation procedures included palate and posterior wall augmentation. CONCLUSIONS: A comprehensive schema for VPI procedures was developed incorporating intentional adaptations in technique. There was substantial variation amongst surgeons in how each procedure was performed. The schema may enable more specific evaluations of surgical outcomes and exploration of the mechanisms through which these procedures improve speech.
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INTRODUCTION: Non-tuberculous mycobacterial (NTM) infection commonly manifests as subacute or chronic cervicofacial lymphadenitis in immunocompetent children. The optimal management of this pathology remains controversial. OBJECTIVES: This international consensus guideline aims to understand the practice patterns for NTM cervicofacial lymphadenitis and to address the primary diagnostic and management challenges. METHODS: A modified three-iterative Delphi method was used to establish expert recommendations on the diagnostic considerations, expectant or medical management, and operative considerations. The recommendations herein are derived from current expert consensus and critical review of the literature. SETTING: Multinational, multi-institutional, tertiary pediatric hospitals. RESULTS: Consensus recommendations include diagnostic work-up, goals of treatment and management options including surgery, prolonged antibiotic therapy and observation. CONCLUSION: The recommendations formulated in this International Pediatric Otolaryngology Group (IPOG) consensus statement on the diagnosis and management of patients with NTM lymphadenitis are aimed at improving patient care and promoting future hypothesis generation.
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Linfadenitis , Infecciones por Mycobacterium no Tuberculosas , Otolaringología , Niño , Humanos , Micobacterias no Tuberculosas , Linfadenitis/microbiología , Antibacterianos/uso terapéutico , Escisión del Ganglio Linfático , Infecciones por Mycobacterium no Tuberculosas/diagnósticoRESUMEN
OBJECTIVE: Patients with 22q11.2 deletion syndrome (22q11DelS) often present with velopharyngeal dysfunction (VPD). VPD in patients with 22q11DelS is multifactorial beyond velopharyngeal insufficiency (VPI) alone, and differences in surgical outcomes are poorly understood. Our objective was to determine whether patients with 22q11DelS have an increased risk for persistent VPI after sphincter pharyngoplasty compared to patients without 22q11DelS. METHODS: We completed a retrospective cohort study of patients with 22q11DelS undergoing sphincter pharyngoplasty between 1995 and 2019 using a VPD clinic database. Patients with 22q11DelS were compared to a cohort of 2:1 frequency-matched (age, degree of velopharyngeal closure) patients without 22q11DelS. Variables included patient characteristics, surgical history, perceptual speech evaluation, and degree of closure on nasopharyngoscopic evaluations. Primary outcomes included postoperative VPI severity and hypernasality. Speech and nasopharyngoscopic characteristics were compared using Fisher's exact test. Postoperative VPI severity and hypernasality were compared between groups via relative risks (RR) from mixed effects Poisson regression models, with random effects of age and velopharyngeal closure. RESULTS: 134 patients (51 22q11DelS, 83 matched) were included, with mean age of 7.3 years (standard deviation 3.0) and 50% male. Cohorts had similar preoperative speech characteristics and nasopharyngoscopic findings. Patients with 22q11DelS had similar postoperative VP function as patients without 22q11DelS (RR 0.85, CI 0.46-1.57 for VPI severity, RR 0.83, CI 0.45-1.53 for hypernasality). Even after adjusting by preoperative variables, no differences were seen between both groups. CONCLUSION: Matched for age and pre-operative velopharyngeal closure, patients with and without 22q11DelS and VPI had similar benefits after sphincter pharyngoplasty. LEVEL OF EVIDENCE: Non-randomized controlled cohort study, 3 Laryngoscope, 133:2813-2820, 2023.
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Fisura del Paladar , Síndrome de DiGeorge , Insuficiencia Velofaríngea , Trastornos de la Voz , Humanos , Masculino , Niño , Femenino , Síndrome de DiGeorge/complicaciones , Síndrome de DiGeorge/cirugía , Estudios de Cohortes , Estudios Retrospectivos , Resultado del Tratamiento , Faringe/cirugía , Insuficiencia Velofaríngea/genética , Insuficiencia Velofaríngea/cirugía , Trastornos de la Voz/cirugía , Fisura del Paladar/cirugía , Esfínter Velofaríngeo/cirugíaRESUMEN
Importance: In the US, most childhood-onset bilateral sensorineural hearing loss is genetic, with more than 120 genes and thousands of different alleles known. Primary treatments are hearing aids and cochlear implants. Genetic diagnosis can inform progression of hearing loss, indicate potential syndromic features, and suggest best timing for individualized treatment. Objective: To identify the genetic causes of childhood-onset hearing loss and characterize severity, progression, and cochlear implant success associated with genotype in a single large clinical cohort. Design, Setting, and Participants: This cross-sectional analysis (genomics) and retrospective cohort analysis (audiological measures) were conducted from 2019 to 2022 at the otolaryngology and audiology clinics of Seattle Children's Hospital and the University of Washington and included 449 children from 406 families with bilateral sensorineural hearing loss with an onset younger than 18 years. Data were analyzed between January and June 2022. Main Outcomes and Measures: Genetic diagnoses based on genomic sequencing and structural variant analysis of the DNA of participants; severity and progression of hearing loss as measured by audiologic testing; and cochlear implant success as measured by pediatric and adult speech perception tests. Hearing thresholds and speech perception scores were evaluated with respect to age at implant, months since implant, and genotype using a multivariate analysis of variance and covariance. Results: Of 406 participants, 208 (51%) were female, 17 (4%) were African/African American, 32 (8%) were East Asian, 219 (54%) were European, 53 (13%) were Latino/Admixed American, and 16 (4%) were South Asian. Genomic analysis yielded genetic diagnoses for 210 of 406 families (52%), including 55 of 82 multiplex families (67%) and 155 of 324 singleton families (48%). Rates of genetic diagnosis were similar for children of all ancestries. Causal variants occurred in 43 different genes, with each child (with 1 exception) having causative variant(s) in only 1 gene. Hearing loss severity, affected frequencies, and progression varied by gene and, for some genes, by genotype within gene. For children with causative mutations in MYO6, OTOA, SLC26A4, TMPRSS3, or severe loss-of-function variants in GJB2, hearing loss was progressive, with losses of more than 10 dB per decade. For all children with cochlear implants, outcomes of adult speech perception tests were greater than preimplanted levels. Yet the degree of success varied substantially by genotype. Adjusting for age at implant and interval since implant, speech perception was highest for children with hearing loss due to MITF or TMPRSS3. Conclusions and Relevance: The results of this cross-sectional study suggest that genetic diagnosis is now sufficiently advanced to enable its integration into precision medical care for childhood-onset hearing loss.
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Implantación Coclear , Implantes Cocleares , Sordera , Pérdida Auditiva Sensorineural , Pérdida Auditiva , Percepción del Habla , Adulto , Femenino , Niño , Humanos , Masculino , Estudios Transversales , Estudios Retrospectivos , Sordera/cirugía , Pérdida Auditiva Sensorineural/diagnóstico , Pérdida Auditiva Sensorineural/genética , Pérdida Auditiva Sensorineural/cirugía , Pérdida Auditiva Bilateral/diagnóstico , Pérdida Auditiva Bilateral/genética , Proteínas de la Membrana , Proteínas de Neoplasias , Serina EndopeptidasasRESUMEN
OBJECTIVE: The Deaf community is an ethnolinguistic minority group. Low sensitivity to Deaf culture contributes to health disparities among Deaf patients. This study determines the level of sensitivity to Deaf culture among otolaryngology-head and neck surgery (OHNS) and audiology trainees. METHODS: Cross-sectional survey study of OHNS and audiology trainees from 10 large US institutions. Trainees were queried on their exposure to and comfort with Deaf patients and their education on, attitude toward, and awareness and knowledge of Deaf culture. Sensitivity to Deaf culture was operationalized as awareness and knowledge of Deaf culture. These were assessed using a 35-item instrument that was previously developed using a d/Deaf community-based participatory approach to research. We used T-tests to compare the sample to previous samples of medical students with training in Deaf culture (MS-TDCs) and general practitioners (GPs). RESULTS: There were 91 completed surveys (response rate 44.5%). Almost all were aware of Deaf culture (97.8%). The mean knowledge score was 55.0% (standard deviation (SD) 13.4%), which was significantly higher than that for GPs at 43.0% (SD 15.0%) (95% confidence interval 8.1%, 15.8%, P < .0001) but significantly lower than that for MS-TDCs at 69.0% (SD 13.0%)(CI -20.3%, -7.6%, P < .0001). Knowledge scores were comparable for OHNS and audiology trainees (P = .09). CONCLUSION: This sample of OHNS and audiology trainees was more sensitive to Deaf culture than GPs but less sensitive than MS-TDCs. Developing specialty-specific education may be warranted. LEVEL OF EVIDENCE: 4.
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Audiología , Otolaringología , Humanos , Estudios Transversales , Otolaringología/educaciónRESUMEN
OBJECTIVES: To describe the hearing thresholds of children under 3 years of age with cleft palate with or without cleft lip. METHODS: This retrospective study from a tertiary care children's hospital included children with cleft palate ± cleft lip born between January 01, 2008, and December 31, 2015. All patients who had the initial palate repair at our institution and at least one behavioral audiogram were included. Behavioral audiograms collected either in the sound field or under insert earphones showed results from the better hearing ear. We collected demographic information, cleft type, parent report of newborn hearing screen, syndrome diagnosis, comorbid conditions, and dates of tympanostomy tube placement and palatoplasty. The main outcome measure was hearing status as measured by behavioral audiograms, and, for a subset of patients, brainstem auditory evoked responses (BAER). Results were grouped by test type (behavioral vs BAER) and characteristics at time of testing (pre- or post-palatoplasty, with or without tympanostomy tube placement) to evaluate the range of severity of hearing loss. RESULTS: Three hundred and sixty patients were included in the cohort. The cohort had two groups: Group 1 included 37 patients who had BAER results prior to surgery and behavioral audiogram results post-surgery; Group 2 included 322 patients who had behavioral audiograms pre-surgery (n = 206) and/or post-surgery (n = 215). The median age of patients at time of BAER in Group 1 was 3.00 months [IQR: 1.00,3.75]; pre-surgery BAER results showed mild (45.9%, n = 17) or moderate hearing loss (29.7%, n = 11) by this age. Patients in Group 2 with pre-surgery behavioral audiograms had a median age of 12.00 months [IQR: 11.00, 14.00] at time of audiogram. Most patients had a mild (33.5%, n = 69) or moderate hearing loss (31.1%, n = 64). Post-surgery, patients had a median age of 13.00 months [IQR: 11.00, 15.00] at time of hearing assessment and typically had normal hearing (86.0%, n = 185). In most patients, hearing improved after palate repair with concurrent tympanostomy tube placement. CONCLUSIONS: Most patients with cleft palate ± cleft lip had evidence of mild or moderate hearing loss prior to cleft palate repair, and hearing loss was evident from early infancy. Hearing improved after concurrent tympanostomy tube placement and palatoplasty. Further research is needed to understand the clinical significance of untreated transient hearing loss during the first year of life in this patient population.
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Labio Leporino , Fisura del Paladar , Pérdida Auditiva , Niño , Preescolar , Labio Leporino/cirugía , Fisura del Paladar/complicaciones , Fisura del Paladar/diagnóstico , Fisura del Paladar/cirugía , Audición , Pérdida Auditiva/diagnóstico , Pérdida Auditiva/etiología , Pérdida Auditiva/cirugía , Humanos , Lactante , Recién Nacido , Ventilación del Oído Medio , Estudios RetrospectivosRESUMEN
PURPOSE: The purpose of this study is to provide updated national estimates on the annual number, educational environments, and secondary school outcomes of students who are D/deaf and hard of hearing (D/HH) receiving special education (SpEd) and related services in the United States. METHOD: We performed a retrospective cross-sectional descriptive analysis of Individuals with Disabilities Education Act, Part B, Section 618 data from 2012 to 2018. Participants included students 6-21 years old in SpEd with "hearing impairment" reported as their primary disability. The general population of students in secondary school served as a comparator, via Current Population Survey data. We described the annual number of students (a) overall, (b) by educational environment, and (c) by reason for exiting SpEd, including the proportion graduating from and dropping out of secondary school. We described variation over time. RESULTS: The median annual number of students was 67,655, with minimal variation by year. The proportion in general education (GenEd) for ≥ 80% of the day increased by 4.2% over 6 years from 57.8% to 62.0%, whereas the proportions in GenEd for < 40% and 40%-79% of the day decreased by 1.6% and 1.3%, respectively. Proportions in the remainder of the environments changed < 1.0% each. Of exiters, 86.8% of students graduated, whereas 3.9% dropped out, compared to a dropout rate of 5.0% in the general population. CONCLUSION: From 2012 to 2018, students who are D/HH receiving SpEd in the United States have spent increasingly more time in GenEd, most graduated from high school, and few dropped out, with dropout patterns appearing similar to the general population.
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Sordera , Pérdida Auditiva , Personas con Deficiencia Auditiva , Adolescente , Adulto , Niño , Estudios Transversales , Educación Especial , Humanos , Estudios Retrospectivos , Instituciones Académicas , Estudiantes , Estados Unidos/epidemiología , Adulto JovenRESUMEN
Microtia techniques have evolved to improve esthetic outcomes, reduce donor site morbidities, and reduce complications. Patients with microtia commonly have aural atresia associated with conductive hearing loss. We present the evolution of our technique for microtia reconstruction and considerations for hearing management in these patients.
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Microtia Congénita , Humanos , Microtia Congénita/cirugía , Estética Dental , Oído/cirugía , Oído/anomalíasRESUMEN
Hearing loss is one of the most common indications for temporal bone imaging in children. Hearing loss may be congenital or acquired, and it may be conductive, sensorineural, or mixed audiologically. Temporal bone imaging plays an important role in the assessment and management of this condition. An understanding of the embryology of ear structures better enables the radiologist to interpret abnormalities on imaging of the temporal bone. Here, we provide a general review of ear development and a description of known genetic defects that contribute to congenital ear anomalies associated with hearing loss. We provide appropriate imaging techniques for the temporal bone depending on the clinical presentation and a systematic approach to imaging for children with hearing loss. Diagnostic imaging for developmental anomalies of the ear and cholesteatoma will be discussed.
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Pérdida Auditiva Conductiva , Pérdida Auditiva , Niño , Pérdida Auditiva/diagnóstico por imagen , Humanos , Radiólogos , Hueso Temporal/diagnóstico por imagen , Tomografía Computarizada por Rayos XRESUMEN
PURPOSE: To assess how pharyngeal wall and soft palate motion are affected after two common interventions for velopharyngeal insufficiency. MATERIALS AND METHODS: A retrospective observational study was performed. A database of patients who had undergone Furlow palatoplasty or pharyngeal flap surgery between 2011 and 2019 and had video-archived preoperative and postoperative nasopharyngoscopy recordings was created. Recordings were deidentified and randomized, with 5 randomly-selected videos duplicated to determine intra-rater reliability. The videos were scored by 3 experienced raters using a modified Golding-Kushner scale. Pre- and postoperative scores were compared using paired t-test. Inter- and intra-rater reliability were estimated using intra-class correlation (ICC). RESULTS: There were 17 patients who met inclusion criteria. The mean age was 6.9 years (range 3-22 years, 59% male). In the Furlow palatoplasty group (n = 9), an increase in left soft palate motion was noted postoperatively (t(8) = 2.71, p = 0.02). In the pharyngeal flap group (n = 8), increases in lateral pharyngeal wall motion (left: t(7) = 3.58, p = 0.008, right: t(7) = 3.84, p = 0.006) and right soft palate motion (t(6) = 2.49, p = 0.04) were identified. Intra-rater reliability and inter-rater agreement were lower than prior studies utilizing the Golding Kushner scale. CONCLUSIONS: Our results provide objective evidence that Furlow palatoplasty and pharyngeal flap surgeries achieve velopharyngeal closure by increasing movement at different anatomical sites. Palatal and pharyngeal wall motion observed during preoperative nasopharyngoscopy may influence a surgeon's choice of intervention.
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Habla , Insuficiencia Velofaríngea , Adolescente , Adulto , Niño , Preescolar , Femenino , Humanos , Masculino , Paladar Blando/cirugía , Faringe/cirugía , Reproducibilidad de los Resultados , Estudios Retrospectivos , Resultado del Tratamiento , Insuficiencia Velofaríngea/cirugía , Adulto JovenRESUMEN
OBJECTIVE: To date, the recording of outcomes of interventions for velopharyngeal dysfunction (VPD) has not been standardized. This makes a comparison of results between studies challenging. The aim of this study was to develop a core outcome set (COS) for reporting outcomes in studies examining the management of VPD. DESIGN: A two-round Delphi consensus process was used to develop the COS. PATIENTS, PARTICIPANTS: The expert Delphi panel comprised patients and caregivers of patients with VPD, surgeons and speech and language therapists specializing in cleft palate, and researchers with expertise in VPD. INTERVENTIONS: A long list of outcomes was derived from the published literature. In each round of a Delphi survey, participants were asked to score outcomes using the Grading of Recommendations, Assessment, Development, and Evaluations scale of 1 to 9, with 1 to 3 labeled "not important," 4 to 6 labeled "important but not critical," and 7 to 9 labeled "critical." MAIN OUTCOME MEASURE: Consensus criteria were specified a priori. Outcomes with a rating of 75% or more of the panel rating 7 to 9 and 25% or fewer rating 1 to 3 were included in the COS. RESULTS: A total of 31 core outcomes were identified from the Delphi process. This list was condensed to combine topic areas to produce a final COS of 10 outcomes, including both processes of care and patient-reported outcomes that should be considered for reporting in future studies of VPD. CONCLUSIONS: Implementation of the COS-VPD will facilitate consistency of outcomes data collection and comparison of results across studies.
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Cuidadores , Proyectos de Investigación , Consenso , Técnica Delphi , Humanos , Evaluación de Resultado en la Atención de Salud , Resultado del TratamientoRESUMEN
OBJECTIVES: To evaluate the performance of 4-dimensional computed tomography (4D-CT) in assessing upper airway obstruction (UAO) in patients with Robin sequence (RS) and compare the accuracy and reliability of 4D-CT and flexible fiber-optic laryngoscopy (FFL). STUDY DESIGN: Prospective survey of retrospective clinical data. SETTING: Single, tertiary care pediatric hospital. METHODS: At initial and 30-day time points, a multidisciplinary group of 11 clinicians who treat RS rated UAO severity in 32 sets of 4D-CT visualizations and FFL videos (dynamic modalities) and static CT images. Raters assessed UAO at the velopharynx and oropharynx (1 = none to 5 = complete) and noted confidence levels of each rating. Intraclass correlation and Krippendorff alpha were used to assess intra- and interrater reliability, respectively. Accuracy was assessed by comparing clinician ratings with quantitative percentage constriction (QPC) ratings, calculated based on 4D-CT airway cross-sectional area. Results were compared using Wilcoxon rank-sum and signed-rank tests. RESULTS: There was similar intrarater agreement (moderate to substantial) with 4D-CT and FFL, and both demonstrated fair interrater agreement. Both modalities underestimated UAO severity, although 4D-CT ratings were significantly more accurate, as determined by QPC similarity, than FFL (-1.06 and -1.46 vs QPC ratings, P = .004). Overall confidence levels were similar for 4D-CT and FFL, but other specialists were significantly less confident in FFL ratings than were otolaryngologists (2.25 and 3.92, P < .0001). CONCLUSION: Although 4D-CT may be more accurate in assessing the degree of UAO in patients with RS, 4D-CT and FFL assessments demonstrate similar reliability. Additionally, 4D-CT may be interpreted with greater confidence by nonotolaryngologists who care for these patients.
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Laringoscopía , Síndrome de Pierre Robin , Niño , Tomografía Computarizada Cuatridimensional , Humanos , Laringoscopía/métodos , Síndrome de Pierre Robin/diagnóstico por imagen , Estudios Prospectivos , Reproducibilidad de los Resultados , Estudios RetrospectivosRESUMEN
Objectives: To present recommendations for the coordinated evaluation and management of the hearing and reconstructive needs of patients with microtia and aural atresia. Methods: A national working group of 9 experts on microtia and atresia evaluated a working document on the evaluation and treatment of patients. Treatment options for auricular reconstruction and hearing habilitation were reviewed and integrated into a coordinated care timeline. Results: Recommendations were created for children with microtia and atresia, including diagnostic considerations, surgical and non-surgical options for hearing management and auricular reconstruction, and the treatment timeline for each option. These recommendations are based on the collective opinion of the group and are intended for otolaryngologists, audiologists, plastic surgeons, anaplastologists, and any provider caring for a patient with microtia and ear canal atresia. Close communication between atresia/hearing reconstruction surgeon and microtia repair surgeon is strongly recommended.
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OBJECTIVES/HYPOTHESIS: A tracheal cartilaginous sleeve (TCS) is a rare anomaly characterized by anterior fusion of tracheal cartilages. TCS is associated with syndromic craniosynostoses including Apert, Crouzon and Pfeiffer syndromes and FGFR2, FGFR3, and TWIST1 variants. This study presents a 30-year review of patients with syndromic craniosynostosis and TCS and describes diagnostic methods, genetic variants, surgical interventions, and long-term outcomes. STUDY DESIGN: Retrospective, single-institution review. METHODS: This review included patients with syndromic craniosynostosis and TCS treated at Seattle Children's Hospital from 1990 to 2020. Tracheostomy, genetic variants, and additional surgery were primary measures. Fisher's exact test compared need for tracheostomy in patients with proposed high-risk (FGFR2 p.W290 or FGFR2 p.C342) versus low-risk genetic variants. RESULTS: Thirty patients with TCS were identified. Average age at diagnosis was 12 months (range 2-weeks to 7.9-years; standard deviation 19.8 months). Syndromes included Pfeiffer (37%), Apert (37%), and Crouzon (26%). Severe obstructive sleep apnea was present in 76% of patients. Tracheostomy was performed in 17 patients (57%); five were successfully decannulated. Additional interventions included adenotonsillectomy (57%), nasal (20%), laryngeal (17%), and craniofacial skeletal surgery (87%). All patients with Pfeiffer syndrome and FGFR2 p.W290C variants and 83% of patients with FGFR2 p.C342 variants required tracheostomy, differing from other variants (P = .02, odds ratio 33, 95% confidence interval 1.56-697.96). One patient (3%) died. CONCLUSION: TCS contributes to multilevel airway obstruction in patients with syndromic craniosynostosis. Genetic testing in patients with FGFR2-related syndromic craniosynostoses may identify those at risk of TCS and facilitate early intervention. A better understanding of this patient population may foster individualized airway management strategies and improve outcomes. LEVEL OF EVIDENCE: 4 Laryngoscope, 132:215-221, 2022.