Risk of Malocclusion Among Patients Undergoing Single-Stage Versus Two-Stage Cleft Palate Repair.

OBJECTIVE: Evaluate impact of single-stage versus staged palate repair on the risk of developing malocclusion among patients with cleft palate (CP). DESIGN: Retrospective cohort study 2000-2016. SETTING: Academic, tertiary children's hospital. PATIENTS: Patients undergoing CP repair between 1999-2015. INTERVENTIONS: CP repair, categorized as either single-stage or staged. MAIN OUTCOME MEASURE: Time to development of Class III malocclusion. RESULTS: 967 patients were included; 60.1% had a two-stage CP repair, and 39.9% had single-stage. Malocclusion was diagnosed in 28.2% of patients. In the model examining all patients at ≤5 years (n = 659), patients who were not white had a higher risk of malocclusion (HR 2.46, p = 0.004) and staged repair was not protective against malocclusion (HR 0.98, p = 0.91). In all patients >5 years (n = 411), higher Veau classification and more recent year of birth were significantly associated with higher hazard rates (p < 0.05). Two-staged repair was not protective against developing malocclusion (HR 0.86, p = 0.60). In the model examining patients with staged repair ≤5 years old (n = 414), higher age at hard palate closure was associated with reduced malocclusion risk (HR 0.67, p < 0.001) and patients who were not white had increased risk (HR 2.56, p = 0.01). In patients with staged repair >5 years old, more recent birth year may be associated with a higher risk of malocclusion (HR 1.06, p = 0.06) while syndrome may be associated with lower risk of malocclusion diagnosis (HR 0.46, p = 0.07). CONCLUSION: Our data suggests that staged CP repair is not protective against developing Class III malocclusion.

The Quest for a DISE Protocol.

OBJECTIVE: The objective of this quality initiative project was to modify our existing institutional drug-induced sleep endoscopy (DISE) protocol so that the surgeon could consistently determine obstructive breathing patterns while minimizing children's discomfort. METHODS: A quality initiative study utilizing the well-described plan-do-study-act (PDSA) process was conducted at a tertiary hospital for children with polysomnogram-documented obstructive sleep apnea who were undergoing DISE. A 4-point Likert measurement tool was created. Change in each Likert rating with subsequent PDSA cycle was tested with the Wilcoxon rank sum test (Mann-Whitney), and change across all PDSA cycles was tested with the Kruskal-Wallis equality-of-populations rank test. RESULTS: After a series of 4 PDSA cycles with 81 children, the DISE protocol was streamlined from 14 to 9 steps. There was significant improvement for all aspects of the DISE, with a final overall median rating of 1 (excellent) for intravenous (IV) placement, scope insertion, and anesthesiologist and surgeon satisfaction (P < .01). DISCUSSION: For sleep surgeons, DISE is quickly becoming what bronchoscopy is to the airway surgeon. Utilizing inhalational agents to obtain IV access and insert the flexible scope in the rapid "on-off" fashion optimizes DISE success regardless of the primary sedation medication and allows ample time for these agents to dissipate. IMPLICATIONS FOR PRACTICE: Adoption of a DISE protocol that includes nasal premedication and inhalational volatile gases for IV and scope insertion at the onset provides a more predictable level of sedation that is well tolerated by the patient, enabling the otolaryngologist to create an obstructive sleep apnea treatment plan.

Bone Grafting of Alveolar Clefts.

The goals of alveolar cleft repair include (1) stabilization of the maxilla, (2) permitting tooth eruption, (3) eliminating the oronasal fistula, (4) improving aesthetics, and (5) improving speech. Alveolar cleft repair should be considered one of the steps of a larger comprehensive orthodontic management plan. In conjunction with closure of the oronasal fistula, a variety of grafting materials can be used in the alveolar cleft. Autogenous grafts have been found to have greater efficacy compared with allogenic or xenogeneic bone, substitute bone, and alloplasts but with more donor site morbidity.

Radiofrequency Ablation vs Electrocautery Blinded Randomized Trial: Impact on Clinically Meaningful Outcomes.

OBJECTIVE: To analyze patients' return to normal activity, pain scores, narcotic use, and adverse events after undergoing tonsillectomy or adenotonsillectomy with monopolar electrocautery or radiofrequency ablation. STUDY DESIGN: Randomized double-blinded clinical trial based on prospective parallel design. SETTING: Academic medical center and tertiary children's hospital between March 2018 and July 2019. METHODS: Inclusion criteria included patients aged ≥3 years with surgical indication of recurrent tonsillitis or airway obstruction/sleep-disordered breathing. Patients were randomly assigned to monopolar electrocautery or radiofrequency ablation. Patients were blinded to treatment assignment. Survey questions answered via text or email were collected daily until postoperative day 15. The primary outcome was the patient's return to normal activity. Secondary outcomes included daily pain score, total amount of postoperative narcotic use, and adverse events. RESULTS: Of the 236 patients who met inclusion criteria and were randomly assigned to radiofrequency ablation or monopolar electrocautery, 230 completed the study (radiofrequency ablation, n = 112; monopolar electrocautery, n = 118). There was no statistically significant difference between the groups in the number of days for return to normal activity (P = .89), daily pain scores over 15 postoperative days (P = .46), postoperative narcotic use (P = .61), or return to hospital for any reason (P = .60), including bleeding as an adverse event (P = .13). CONCLUSIONS: As one of the largest randomized controlled trials examining instrumentation in tonsillectomy, our data do not show a difference between monopolar electrocautery and radiofrequency ablation with regard to return to normal activity, daily pain scores, total postoperative narcotic use, or adverse events.

Management of Velopharyngeal Insufficiency in Cleft Patients With and Without Multidisciplinary Team Care.

OBJECTIVE: To evaluate the effect of an American Cleft Palate-Craniofacial Association (ACPA)-approved multidisciplinary team on velopharyngeal insufficiency (VPI) diagnosis and treatment. DESIGN: Retrospective cohort setting; tertiary children's hospital patients; children with cleft palate repair identified through procedure codes. MAIN OUTCOME MEASURES: Velopharyngeal insufficiency diagnosis was assigned based on surgeon or team assessment. Age at diagnosis and surgery was recorded. Difference in age and rate of VPI diagnosis and surgery was analyzed with t test. Multivariate linear and logistic regression adjusted for confounding variables. RESULTS: Nine hundred forty patients were included with 71.5% cared for by an ACPA-approved multidisciplinary team. More (38.8% ) team care patients were found to have a diagnosis of VPI in comparison to 10% in independent care (P < .001). Team care was associated with an almost 6-fold increase in VPI diagnosis (P < .001). Team care was associated with a higher proportion of speech surgery (21% vs 10%, P < .001). Among children receiving team care, each visit was associated with 25% increased odds of being diagnosed with VPI (P < .001) and 20% increased odds of receiving speech surgery (P < .001). Age at VPI diagnosis and speech surgery were similar between groups (P = .55 and .29). DISCUSSION: Team care was associated with more accurate detection of VPI, resulting in more VPI speech therapy visits and surgical management. A higher number of team visits were similarly associated. CONCLUSION: Further studies of the clinical implication of timely and accurate VPI diagnosis, including quality of life assessments, are recommended to provide stronger guidance on team visit and evaluation planning.

Oronasal Fistula Risk After Palate Repair.

OBJECTIVE: To assess risk factors for oronasal fistula, including 2-stage palate repair. DESIGN: Retrospective analysis. SETTING: Tertiary children's hospital. PATIENTS: Patients with non-submucosal cleft palate whose entire cleft repair was completed at the study hospital between 2005 and 2013 with postsurgical follow-up. INTERVENTIONS: Hierarchical binary logistic regression assessed predictive value of variables for fistula. Variables tested for inclusion were 2 stage repair, Veau classification, sex, age at surgery 1, age at surgery 2, surgeon volume, surgeon, insurance status, socioeconomic status, and syndrome. Variables were added to the model in order of significance and retained if significant at a .05 level. MAIN OUTCOME MEASURE: Postoperative fistula. RESULTS: Of 584 palate repairs, 505 (87%) had follow-up, with an overall fistula rate of 10.1% (n = 51). Among single-stage repairs (n = 211), the fistula rate was 6.7%; it was 12.6% in 2-stage repairs (n = 294, P = .03). In the final model utilizing both single-stage and 2-stage patient data, significant predictors of fistula were 2-stage repair (odds ratio [OR]: 2.5, P = .012), surgeon volume, and surgeon. When examining only single-stage patients, higher surgeon volume was protective against fistula. In the model examining 2-stage patients, surgeon and age at hard palate repair were significant; older age at hard palate closure was protective for fistula, with an OR of 0.82 (P = .046) for each additional 6 months in age at repair. CONCLUSIONS: Two-stage surgery, surgeon, and surgeon volume were significant predictors of fistula occurrence in all children, and older age at hard palate repair was protective in those with 2-stage repair.

Ecuadorian Spanish translation and validation of the VELO quality of life instrument.

OBJECTIVES: Adapt the Spanish translation of VPI Effects on Life Outcome (VELO) instrument into Ecuadorian Spanish; test the resulting instrument for reliability and validity. METHODS: A cross-sectional, prospective design, set at a humanitarian mission within a community hospital. Linguistic validation: native Ecuadorian-Spanish speakers modified the Spanish VELO to Ecuadorian Spanish. Cognitive interviews were conducted with children with cleft palate (CP) and their parents (n = 50), guiding instrument modifications. An expert panel reviewed changes, resulting in the VELO-Ecuadorian dialect (VELO-Ec). INSTRUMENT ASSESSMENT: 88 participants with CP (88 parents, 46 children) and 33 non-cleft controls (33 adult, 11 children) completed the VELO-Ec, Spanish-Pediatric Voice Handicap Index (pVHI), and Spanish-Intelligibility in Context Scale (ICS). Internal consistency was assessed with Cronbach's alpha; test-retest reliability was assessed by calculating the intraclass correlation coefficient (ICC); standard error of measurement (SEM) was calculated. Concurrent validity was assessed with Pearson correlations of VELO-Ec with pVHI and ICS. Discriminant validity assessment used an established ICS cutoff. Construct validity was assessed by grouping patients by parent report of hypernasality and early vs. late cleft repair (>24 months) using the Wilcoxon Rank-Sum test. RESULTS: VELO-Ec showed excellent internal consistency (alpha 0.96) and test-retest reliability (ICC = 0.85, 95% CI 0.68-0.93, SEM 5.71). It had strong concurrent validity, correlating with ICS (r = 0.75, p < 0.001) and pVHI (r = -0.79, p < 0.001). Discriminant validity was strong with better VELO-Ec scores among subjects with normal vs. abnormal ICS score (median 95 & 61, p < 0.001). Strong construct validity was identified: those with parent-reported hypernasality had worse VELO-Ec scores than those without (median 59 & 75, p < 0.001). Those with repair before or after 24 months had similar VELO-Ec scores (p = 0.882). CONCLUSION: The VELO-Ec is a valid and reliable measure of VPI-related quality of life, useful to clinicians and researchers treating Ecuadorian CP patients, especially in areas with limited resources such as on humanitarian missions.

Association of Allergic Rhinitis With Change in Nasal Congestion in New Continuous Positive Airway Pressure Users.

Importance: Nasal congestion occurring after continuous positive airway pressure (CPAP) treatment initiation impairs CPAP adherence. Allergic rhinitis is associated with worsening nasal congestion in patients who are exposed to nonallergic triggers. Use of CPAP presents potential nonallergic triggers (eg, humidity, temperature, pressure, and airflow). Objective: To compare nasal congestion among CPAP users with allergic rhinitis, nonallergic rhinitis, and no rhinitis. We hypothesize that CPAP patients with baseline allergic rhinitis are more likely to experience a worsening of nasal congestion (or less improvement in nasal congestion) compared with patients with no baseline rhinitis. Design, Setting, and Participants: This prospective cohort study included consecutive patients newly diagnosed with obstructive sleep apnea in a tertiary sleep center who were using CPAP therapy 3 months after diagnosis. Baseline rhinitis status was assigned as allergic rhinitis, nonallergic rhinitis, or no rhinitis, based on questionnaire responses and past allergy testing. Data were collected from 2004 to 2008 and analyzed from July 2019 to February 2020. Main Outcomes and Measures: At baseline before CPAP exposure and again 3 months later, subjective nasal congestion was measured with the Nasal Obstruction Symptom Evaluation (NOSE) scale and a visual analog scale (VAS), each scored from 0 to 100 (100 = worst congestion). Changes in nasal congestion were tested over 3 months for the whole cohort, within each rhinitis subgroup (paired t test), and between rhinitis subgroups (multivariate linear regression). Results: The study cohort comprised 102 participants, of whom 61 (60%) were male and the mean (SD) age was 50 (13). The study included 23 (22.5%) participants with allergic rhinitis, 67 (65.7%) with nonallergic rhinitis, and 12 (11.8%) with no rhinitis. Nasal congestion improved from baseline to 3 months in the whole cohort (mean [SD] NOSE score, 38 [26] to 27 [23], mean [SD] change, -10 [23]; 95% CI, -15 to -6; mean [SD] VAS score, 41 [27] to 32 [28]; mean [SD] change, -10 [26]; 95% CI, [-15 to -4]) and in each rhinitis subgroup. Adjusted improvement in nasal congestion at 3 months was significantly less in the allergic rhinitis subgroup compared with the no rhinitis subgroup (positive difference means less improvement) compared with baseline: NOSE score 14 (95% CI, 1 to 28) and VAS score 15 (95% CI, 0 to 30). Conclusions and Relevance: Initiation of CPAP was associated with improved subjective nasal congestion, but less improvement in patients with baseline allergic rhinitis. Baseline allergic rhinitis may predict which patients are more vulnerable to potential congestive effects of CPAP.

Family Experience With Pierre Robin Sequence: A Qualitative Study.

OBJECTIVE: To identify concepts and constructs important to parents of children with Pierre Robin Sequence (PRS). DESIGN: Qualitative study. SETTING: All children received some care at a tertiary hospital with additional care at outside facilities. Interviews were conducted in nonclinical locations, including remote locations. PARTICIPANTS: Parents of children <5 years old with a diagnosis of PRS. Prior treatments included observation, positioning, nasal trumpet, mandibular distraction osteogenesis, tracheostomy, and gastrostomy. INTERVENTION: Semi-structured interviews with individuals (4) and with groups (focus groups, 4) were conducted using open-ended questions and non-leading prompts. Transcripts were analyzed with iterative open and axial coding. Concepts and constructs were identified and refined into codes and central themes. Interviews were conducted until thematic saturation was achieved. RESULTS: Sixteen parents were interviewed. Their experiences were coded into 5 main themes, which can be summarized as: (1) child's symptoms/well-being, (2) parents' grief/isolation, (3) family stress, (4) relationships with providers, and (5) psychological and technical growth. Difficulty with feeding, weight gain, and breathing problems were core physical issues described by participants with associated intense fear. Participants described frustration from not only lack of care coordination, slow diagnoses, and poor communication but also gratitude for providers who served as advocates. Participants described gradual development of knowledge/competencies. CONCLUSIONS: Families of children with PRS have experiences that profoundly affect their lives. Child's physical symptoms/well-being and parents' psychosocial well-being provide content for a future PRS-specific quality-of-life instrument. Concepts that emerged also provide a framework to improve parents' experience and enhance their children's quality of care.

The Case of the Missing Nose: Congenital Arhinia Case Presentation and Management Recommendations.

OBJECTIVES: To discuss the presentation and management of infants with arhinia or congenital absence of the nose. METHODS: This case report describes an infant with arhinia that was diagnosed prenatally. In addition to a discussion of the case, a review of the literature was completed to define appropriate postnatal work-up and management. RESULTS: The patient is a term male infant, diagnosed with arhinia on ultrasound and magnetic resonance imaging (MRI) performed at 21-weeks gestational age. Upon birth, the patient was subsequently intubated, followed by tracheostomy due to complete nasal obstruction. Through a genetics evaluation, the patient was found to be heterozygous for the SMCHD1 gene, with hypomethylation at the D4Z4 locus. Plans for reconstruction will be based on future imaging and the development of any nasal patency, however, the patient's family plans to utilize a prosthetic nose until the patient is older. CONCLUSION: Arhinia is a rare condition causing respiratory distress in the neonatal period. While stabilization of the airway is the first priority, further management is not clearly defined given the rarity of the malformation. This case discusses stabilization of the airway with a review of treatment and reconstructive options.

Development of a Sleep Apnea-Specific Health State Utility Algorithm.

Importance: With the increasing emphasis on economic evaluations, there is a need for additional methods of measuring patient utility in the obstructive sleep apnea population. Objective: To develop and validate a utility scoring algorithm for a sleep apnea-specific quality-of-life instrument. Design, Setting, and Participants: Development and validation were conducted at 2 tertiary referral sleep centers and associated sleep clinics and included patients with newly diagnosed obstructive sleep apnea from a randomized clinical trial and an associated observational cohort study. Baseline participants were randomly divided into a model development group (60%) and a cross-validation group (40%). Main Outcomes and Measures: Utility scoring of the Symptoms of Nocturnal Obstruction and Related Events (SNORE-25) was mapped from the SF-6D utility index through multiple linear regression in the development sample using the Akaike information criterion to determine the best model. Results: A total of 500 participants (development, n = 300; validation, n = 200) were enrolled; the analyzed sample of 500 participants included 295 men (59%), and the mean (SD) age was 48.6 (12.8) years, with a range of 18 to 90 years. The mean (SD) SF-6D utility among participants with untreated sleep apnea was 0.61 (0.08; range, 0.40-0.85) with similar utility across sleep apnea severity groups. The best-fit model (the SNORE Utility Index) was the natural log conversion of the instrument subscales (r2 = 0.32 in the development sample). The SNORE Utility Index retained this association within the validation sample (r2 = 0.33). Conclusions and Relevance: The SNORE Utility Index provides a validated, disease-specific, preference-weighted utility instrument that can be used in future studies of patients with obstructive sleep apnea.

Brazilian-Portuguese Linguistic Validation of the Velopharyngeal Insufficiency Effects on Life Outcome Instrument.

BACKGROUND: The purpose of this study was to conduct a linguistic validation of the velopharyngeal insufficiency (VPI) Effects on Life Outcome (VELO) instrument for use in Brazilian-Portuguese patients with VPI. METHODS: The original English version of the VELO instrument was translated into Brazilian-Portuguese, back-translated, and adapted among the Brazilian patients (n = 21) with VPI and their parents, based on the standardized guidelines for the cross-culture adaption process. Discrepancies in the forward and backward translation steps were computed. Comprehension rates were captured for each debriefing interview. The content validity index (CVI) per item (I-CVI) and of the scale (S-CVI universal agreement [S-CVI/UA] and averaging [S-CVI/Ave]) were calculated. RESULTS: Reconciliation of the 2 forward translations and the comparison between the back translation and the original VELO version resulted in some item wordings with discrepancies which were reviewed by the research team (translators, expert committee, and original developers of instrument). Three rounds of cognitive interviews also led to some revisions of wording. Comprehension rates of patients and their parents were 60% to 100%, 80% to 100%, and 100% in the first, second, and third rounds of cognitive interviews, respectively. The I-CVI, S-CVI/AU, and SCI/Ave for the Brazilian-Portuguese VELO version were 0.83 (or higher), 0.83 (or higher), and 0.97, respectively. CONCLUSIONS: The linguistic validation process of the VELO instrument created a cross-culturally equivalent Brazilian-Portuguese version for use in Brazilian-Portuguese speaking patients with VPI.

Measuring Patient-Reported Health-Related Quality of Life in Velopharyngeal Insufficiency: Reliability and Validity of the Brazilian Portuguese Version of the VELO Instrument.

OBJECTIVE: To test the Brazilian Portuguese velopharyngeal insufficiency (VPI) Effects on Life Outcome (VELO) instrument for reliability and validity. DESIGN: Cross-sectional methodological study. SETTING: Tertiary craniofacial medical center. PARTICIPANTS: Participants with VPI (VPI group, n = 60), with cleft and without VPI (no VPI/cleft group, n = 60), and with no cleft nor VPI (no VPI/no cleft group, n = 60) and their parents (n = 180). INTERVENTIONS: All patients with VPI 8+ years old and their parents completed the Brazilian-Portuguese VELO instrument and other questionnaires (Pediatric Quality of Life Inventory4.0, PedsQL4.0; Pediatric Voice-Related Quality of Life, PVRQOL; and Intelligibility in Context Scale, ICS) at baseline; patients with VPI and their parents completed the VELO instrument again 2 weeks later. MAIN OUTCOME MEASURES: The VELO instrument was tested for internal consistency, test-retest reliability, discriminant validity (participants with VPI against participants with no VPI), concurrent validity against other questionnaires, criterion validity against hypernasality severity, and construct validity against nasal air emission and overall velopharyngeal competence (speech construct) and velopharyngeal gap (anatomic construct). RESULTS: The VELO had excellent internal consistency (Cronbach α 0.99 for parents and 0.98 for participants with VPI) and test-retest reliability (all intraclass correlation coefficient > 0.87). The VELO discriminated well between VPI group and unaffected groups (all P < .05). The VELO was significantly correlated with the PedsQL4.0, PVRQOL, and ICS (-r > 0.75; P < .001). The VELO met criterion validity, speech construct validity, and anatomic construct validity (r > 0.7; P < .001). CONCLUSIONS: The Brazilian-Portuguese VELO instrument demonstrated reliability (internal consistency and test-retest) and validity (discriminant, concurrent, criterion, and construct).

Validation of the Chinese Velopharyngeal Insufficiency Effects on Life Outcomes Instrument.

OBJECTIVES/HYPOTHESIS: To translate the Velopharyngeal Insufficiency Effects on Life Outcomes (VELO) instrument into Chinese and test its psychometric properties. STUDY DESIGN: Quality of life instrument translation and validation. METHODS: The original English version of the VELO instrument was translated into Mandarin, back-translated, and adapted among the Chinese population, based on the standardized guidelines for the cross-culture adaption process. Velopharyngeal insufficiency (VPI) patients were identified by a professional speech and language pathologist. Internal reliability of the VELO instrument was assessed by the Cronbach's α coefficient. Discriminant validity was tested by the Mann-Whitney U test. Construct validity was assessed by factor analysis. RESULTS: A total of 113 patients with VPI and 72 parents of the patients were enrolled. The mean age of the VPI patients was 14.8 years. Internal reliability was excellent; Cronbach's α coefficients were 0.92 and 0.94 for VPI patients and their parents, respectively. The Chinese VELO discriminated well between the VPI group and the controls, with a mean (standard deviation) score that was significantly lower for the VPI group (74.8 [25.7]) than the control group (98.0 [15.9]) (P < .001). The total scores and scores in the emotional domain or perception domain showed differences between VPI patients and their parents. Similar to the original study, the factor loading after rotation followed hypothesized domains largely, in spite of items from several domains loaded on the same factor. CONCLUSIONS: The translated Chinese version of the VELO instrument demonstrated an acceptable reliability, discriminant validity, and construct validity. These psychometric properties suggested theoretical evidence for the further use of the VELO instrument among Chinese patients with VPI. LEVEL OF EVIDENCE: 3b Laryngoscope, 129:E395-E401, 2019.

Unilateral Hearing Loss in Youth: Development of Candidate Items for a Condition-Specific Validated Instrument.

OBJECTIVE: This study interviewed youth with unilateral hearing, utilizing their responses to generate candidate items for a condition-specific patient-reported instrument. STUDY DESIGN: Mixed methods, cross-sectional. SETTING: Tertiary care children's hospital. PARTICIPANTS AND METHODS: Youth with unilateral hearing loss and normal hearing in the contralateral ear were identified and recruited for participation through query of an audiometric database and through hearing loss clinics. Interviews with the youth were qualitatively analyzed to identify common themes and generate items related to functional impact. A multi-institutional expert panel reviewed items with prespecified item selection criteria. Participants rated items for impact on daily life. For preliminary criterion validity assessment, statistical analyses explored correlations between functional scores and type and severity of hearing loss. RESULTS: Thirty-nine youth aged 9 to 18 years with unilateral hearing loss participated; 31% used a hearing device. Fifteen youth participated in interviews; thematic analysis, item crafting, and expert panel item review resulted in 41 items. Twenty-six youth responded to the items, reporting low functional scores in the domains of sound localization, ear positioning, and noise environment. They reported better levels of function in carrying out group conversations, focusing on schoolwork, and feeling safe during activities. Multivariate linear regression found that youth scored 0.4 points (or approximately 8%) lower on the functional impact scale with every 20-dB HL increase in pure tone average in the abnormal ear. CONCLUSION: Youth with unilateral hearing loss report functional impact, particularly related to sound localization, ear positioning, and noise environment; therefore, they may benefit from a condition-specific functional assessment instrument.

Lingual tonsillectomy in children with Down syndrome: Is it safe?

OBJECTIVE: Evaluate peri-operative course and morbidity in children with Down syndrome (DS) who underwent a lingual tonsillectomy (LT) for residual obstructive sleep apnea (rOSA). METHODS: Retrospective case series for children with DS who underwent LT for rOSA from April 2011 to July 2016. Our primary outcomes were length of stay, readmission and complications. Surgical effectiveness was evaluated by change in the obstructive apnea-hypopnea-index(OAHI) and oxygen saturation nadir. RESULTS: Thirty-nine patients underwent LT. The mean length of stay was 1.3 days with n = 21(72%) staying one night. One subject (2.6%) had a post-operative bleed that did not require operative intervention. No other major complications occurred. In terms of effectiveness of surgery, twenty-nine children had sufficient data for inclusion. Median OAHI did not appreciably change (p = 0.07) from before surgery. Five subjects (17%) were cured of OSA (OAHI < 2/hour) and a mix of improvement and worsening was identified. The lowest oxygen saturation improved from 78% (SD = 7) before surgery to 82% (SD = 6) after surgery (p = 0.003). CONCLUSION: LT has a favorable post-operative course but its effectiveness at curing rOSA in the DS population has not been established/proven. Further research is indicated to determine optimal surgical management for DS children with LTH. LEVEL OF EVIDENCE: 4.

Spanish Linguistic Validation of the Velopharyngeal Insufficiency Effects on Life Outcomes: VELO-Spanish.

BACKGROUND: Quality of life (QOL) assessments are useful tools that measure a patient's health status and monitor patient-reported outcome measures. This study highlights the process of linguistic validation of a QOL assessment to serve Spanish-speaking families and ultimately help decrease language barriers in the treatment of velopharyngeal insufficiency (VPI). METHODS: The standardized linguistic validation process included forward and backward translation, reconciliation, and cognitive interviews with patients and families. Preliminary instrument test-retest measurement was assessed. Eligibility for cognitive interviews included families with familiarity of velopharyngeal insufficiency. Exclusion criteria included illiteracy and parent and child respondents who do not speak Spanish. Reliability was tested by intraclass correlation (ICC) on VPI Effects on Life Outcomes (VELO)-Spanish instruments completion on 2 measurements from the medical record. RESULTS: The instrument was optimized through a standardized forward and backward translation process. Further problematic language was identified during cognitive interviews with families and their children. In the second interview, only minimal changes were needed. Twenty-one patients (8 males and 13 females) were included. Mean (SD) age was 8.0 (5.3) years (range, 3-21 years). The mean (SD) Velo-Spanish score was 65 (22.1); range 32.7-100. The VELO-Spanish instrument demonstrated excellent test-retest reliability [ICC = 0.91; n = 21 and internal consistency (α = 0.96)]. CONCLUSIONS: The Spanish VELO has been developed and refined for use in Spanish-speaking populations as a VPI-specific QOL instrument. The linguistic validation process including cognitive interviews and initial reliability testing. The instrument may improve the understanding of patient-reported outcomes and potential disparities from linguistic and cultural barriers in VPI treatment.

Association of Velopharyngeal Insufficiency With Quality of Life and Patient-Reported Outcomes After Speech Surgery.

IMPORTANCE: Patients with cleft palate and other causes of velopharyngeal insufficiency (VPI) suffer adverse effects on social interactions and communication. Measurement of these patient-reported outcomes is needed to help guide surgical and nonsurgical care. OBJECTIVES: To further validate the VPI Effects on Life Outcomes (VELO) instrument, measure the change in quality of life (QOL) after speech surgery, and test the association of change in speech with change in QOL. DESIGN, SETTING, AND PARTICIPANTS: Prospective descriptive cohort including children and young adults undergoing speech surgery for VPI in a tertiary academic center. Participants completed the validated VELO instrument before and after surgical treatment. MAIN OUTCOMES AND MEASURES: The main outcome measures were preoperative and postoperative VELO scores and the perceptual speech assessment of speech intelligibility. The VELO scores are divided into subscale domains. Changes in VELO after surgery were analyzed using linear regression models. VELO scores were analyzed as a function of speech intelligibility adjusting for age and cleft type. The correlation between speech intelligibility rating and VELO scores was estimated using the polyserial correlation. RESULTS: Twenty-nine patients (13 males and 16 females) were included. Mean (SD) age was 7.9 (4.1) years (range, 4-20 years). Pharyngeal flap was used in 14 (48%) cases, Furlow palatoplasty in 12 (41%), and sphincter pharyngoplasty in 1 (3%). The mean (SD) preoperative speech intelligibility rating was 1.71 (1.08), which decreased postoperatively to 0.79 (0.93) in 24 patients who completed protocol (P < .01). The VELO scores improved after surgery (P<.001) as did most subscale scores. Caregiver impact did not change after surgery (P = .36). Speech Intelligibility was correlated with preoperative and postoperative total VELO score (P < .01) and to preoperative subscale domains (situational difficulty [VELO-SiD, P = .005] and perception by others [VELO-PO, P = .05]) and postoperative subscale domains (VELO-SiD [P = .03], VELO-PO [P = .003]). Neither the VELO total nor subscale score change after surgery was correlated with change in speech intelligibility. CONCLUSIONS AND RELEVANCE: Speech surgery improves VPI-specific quality of life. We confirmed validation in a population of untreated patients with VPI and included pharyngeal flap surgery, which had not previously been included in validation studies. The VELO instrument provides patient-specific outcomes, which allows a broader understanding of the social, emotional, and physical effects of VPI. LEVEL OF EVIDENCE: 2.

Anatomic distribution of cervicofacial lymphatic malformations based on lymph node groups.

OBJECTIVES: To evaluate radiographic characteristics and to identify locations of cervicofacial lymphatic malformations in children based on known lymph node groupings. METHODS: Retrospective chart review of pediatric patients with cervicofacial lymphatic malformations who underwent imaging with magnetic resonance imaging (MRI), computed tomography (CT) or ultrasonography (US). Ninety charts were reviewed from November 2005 to June 2015. Demographic information and imaging characteristics were evaluated. RESULTS: Ninety children were included. The average age at presentation was 52 months (range, 1 day to 170 months). Imaging modalities were MRI in 73 (81%), CT in 7 (8%), US in 6 (7%), and multimodality imaging in 4 (4%) cases. Nearly half (49%) of lesions were found in the parotid and submandibular nodal group, 32% in the cervical group, and 19% in the midline face and oral cavity group. The lymphatic malformations were found on the left in 39 (43%) of cases, on the right in 30 (33%) of cases, and were bilateral in 21 (23%) cases. Nineteen (21%) lesions were macrocystic, twenty-two (24%) were microcystic, and forty-nine (49%) had mixed features. Mixed lesions were more likely to be extensive and involve multiple lymph node groups (P = 0.0005). Adjacent lymphadenopathy was present in 20 (22%) among all subjects, with an average size of 1.22 (± 1.92) cm in the short-axis. CONCLUSION: The results of this study demonstrate three lymph node groupings in which LM are commonly identified. The midline face and oral cavity lesions are predominantly microcystic, the parotid and submandibular lesions are predominately of mixed morphology, and the cervical lesions are predominately macrocystic and mixed. Further studies are needed to determine if such a classification system demonstrates clinically significant difference in disease progression and response to therapy.