Comparative Study of Pharyngeal Flap Outcomes between Children With 22q11.2 Deletion Syndrome and Non-Syndromic Cleft Lip and Palate.

BACKGROUND: Management of velopharyngeal insufficiency (VPI) in 22q11.2 deletion syndrome (22q) is challenging. This study compares pharyngeal flap outcomes in children with 22q to those with non-syndromic cleft lip and palate (CLP) to assess risk of poor speech outcomes and negative sequelae. METHODS: Children with 22q or CLP treated with pharyngeal flap through a multidisciplinary VPI clinic between 2009 and 2020 were retrospectively reviewed. Pre- and postoperative speech assessments, perioperative characteristics, and complications were identified. RESULTS: 36 children with ​22q and 40 with CLP were included. Age at surgery (p=0.121), pre-operative velopharyngeal competence score (VPC) (p=0.702), and pre-operative resonance (p=0.999) were similar between groups. Pharyngeal flaps were wider (p=0.038*) and length of stay longer in the 22q group (p=0.031*). On short term follow 4 months after surgery, similar speech outcomes were seen between groups. At long term follow up >12 months after surgery, 86.7% 22q v. 100% CLP (p=0.122) had improvement in velopharyngeal function, however fewer children with 22q (60.0%) achieved a completely "competent" VPC score compared to those with CLP (92.6%) (p=0.016*). Nasal regurgitation improved for both groups, with a greater improvement in those with 22q (p=0.026*). Revision rate (p=0.609) and new onset OSA (0.999) were similar between groups. CONCLUSION: Children with 22q have improved speech after pharyngeal flap, but may be less likely to reach normal velopharyngeal function over the long term than those with CLP; however, negative sequelae do not differ. Improvement in nasal regurgitation is a uniquely positive outcome in this population.

Barriers to Voice Therapy in Dysphonic Children.

BACKGROUND: The most common etiologies of dysphonia in the pediatric population are vocal fold nodules and muscle tension dysphonia. Vocal therapy is the first line treatment for these disorders in children. Despite this, not all children undergo therapy. The goal of this study is to examine how factors such as patient demographics and parental perceptions differ between children that choose to undergo or not to undergo voice therapy. METHODS: A retrospective review was conducted of all pediatric patients seen at a tertiary voice clinic between January 2014 and December 2017. Patients were included if diagnosed with vocal fold nodules and/or muscle tension dysphonia. Patients were divided into groups of children that received voice therapy at our institution and those that did not. Data include demographics, Consensus Auditory-Perceptual Evaluation of Voice (CAPE-V) scores and pediatric Voice Handicap Index (pVHI) scores. Distance to therapy site was approximated using patient zip codes. RESULTS: Three hundred and forty-six children were included, 224 (65%) boys and 122 (35%) girls. In the 2 years following initial diagnosis, 74 (21%) children participated in voice therapy at our institution. Patients who underwent voice therapy were older than those who did not (mean age: 9.1 [SD 3.5] vs 7.6 [SD 3.8] years; P = 0.004). Patients who received voice therapy were more likely to live closer to the therapy site (mean distance: 15.5 [SD 13.0] vs 24.3 [SD 23.9] miles; P< 0.001). Likelihood of receiving voice therapy did not differ by gender or health insurance status (private vs public). Patients who underwent voice therapy had significantly greater CAPE-V Overall Severity scores than those who did not (mean score: 44.6 [SD 19.4] vs 37.4 [SD 18.0]; P = 0.003). Higher CAPE-V Strain scores were associated with increased likelihood of voice therapy. pVHI scores did not differ between the two groups. CONCLUSION: Older age, shorter distance to therapy site, and increased CAPE-V Overall Severity and Strain scores were associated with higher likelihood of receiving voice therapy. Gender, insurance status, and pVHI scores did not affect likelihood of receiving voice therapy. Patients may primarily consider ease of access and necessity of treatment when considering voice therapy.

Laryngeal pathologies in dysphonic children with Down Syndrome.

INTRODUCTION: Down syndrome is the most common chromosomal abnormality and is associated with a higher incidence of congenital heart defects, which often require surgery within the first year of life. Previous studies have found that children with Down syndrome are at higher risk for subglottic stenosis, vocal fold paralysis, and laryngomalacia. The goal of this study is to review children with Down syndrome presenting with dysphonia and to characterize their laryngeal pathologies. METHODS: A retrospective review was performed of patients with Down syndrome seen at a tertiary pediatric hospital's department of otolaryngology from Jan. 2007-Jul. 2021 for voice-related concerns. Inclusion criteria included age less than 18 years, diagnosis of Trisomy 21, and complaint of dysphonia. The data extracted included history of dysphonia, co-morbidities, demographic information, age at presentation, perceptual voice assessments, voice quality of life scores, acoustic data, laryngoscopic and/or videostroboscopic exams, and surgical procedures. RESULTS: Twenty-three total patients met the study criteria. Of these children, 13 (57%) were male and 10 (43%) were female. The mean age at first presentation was 4.08 years (range 12 days-16.3 years). Eleven of the 23 patients presented within the first 12 months of life. Sixteen patients were diagnosed with vocal fold immobility, 13 of which were left-sided unilateral immobility and the remaining 3 were bilateral immobility. 5 patients were diagnosed with vocal fold nodules. 12 children in the immobility group had a history of cardiothoracic surgery at our institution. Only 3 patients had Consensus Auditory-Perceptual Evaluation of Voice (CAPE-V) assessments, though all three showed overall dysphonia ratings of severely deviant, with roughness and strain scores being the most severe. DISCUSSION: The most common etiology of dysphonia in our Down syndrome patient population was vocal fold immobility and hypomobility, as opposed to vocal fold nodules (which is the most common in the general pediatric population). The higher likelihood of cardiac surgery in patients with Trisomy 21 may result in the increased incidence of vocal fold immobility. There should be a low threshold to refer dysphonic patients with Down syndrome for laryngoscopic evaluation, as treatment options may be available.

Subglottic stenosis: a ten-year review of treatment outcomes.

OBJECTIVES/HYPOTHESIS: To evaluate the endoscopic surgical management of adult subglottic stenosis and describe treatment outcomes. STUDY DESIGN: Retrospective review. METHODS: Ten-year review of adult patients with subglottic stenosis. RESULTS: Ninety-two adults (23 male, 69 female) with subglottic stenosis underwent 247 endoscopic dilations between 2001 and 2010. The mean age was 48 years at time of first surgery. Etiology was GPA (granulomatosis with polyangiitis, formerly Wegener's granulomatosis) (45%), intubation (25%), or idiopathic (33%). Forty-one patients (45%) underwent a single procedure; 51 patients (55%) required multiple surgeries. The average interval for patients requiring a subsequent procedure was 13.7 months. Variations in surgical technique did not show differences in time to next procedure, and the use of mitomycin-C did not result in longer intervals between procedures. No significant complications were encountered after dilation. CONCLUSIONS: Subglottic stenosis remains a treatment challenge. Although patients are often symptomatically improved after endoscopic dilation, recurrence rates remain high.