Incidence and Recovery of Vocal Fold Immobility Following Pediatric Cardiac Operations.

BACKGROUND: Loss of laryngeal function after congenital cardiac surgery causes morbidity and prolongs hospitalization. Early diagnosis of vocal fold immobility (VFI) and referral to pediatric otolaryngology (pOTO) aids in laryngeal rehabilitation. Understanding the incidence and recovery rates of VFI enables counseling for families of infants undergoing high-risk surgery. METHODS: A retrospective chart review from November 2014 to July 2019 of infants postcardiac surgery where the aortic arch or surrounding structures were manipulated and were screened via flexible fiberoptic laryngoscopy (FFL) at a single institution was performed. Patients were divided into five surgical categories: Norwood procedure, aortic arch augmentation via median sternotomy, arterial switch operation, coarctation repair via lateral thoracotomy, and cardiac surgeries including ligation of a patent ductus arteriosus (PDA). Patients undergoing isolated PDA ligation were excluded. RESULTS: One hundred ninety-nine qualifying operations occurred during this period; 28 patients did not undergo FFL before discharge and were excluded from the analysis. Immediately following cardiac surgery, 34% (58 of 171 patients) had VFI. Follow-up was completed by 38 of 58 patients with VFI. Complete recovery was demonstrated in 63% (24 of 38) of patients by 6 months and in 86% (33 of 38) within 18 months. The highest risk occurred with the Norwood procedure and arch augmentation via median sternotomy. CONCLUSIONS: Infants undergoing surgery involving the aortic arch and surrounding structures have high rates of VFI. Follow-up by pOTO is recommended to optimize laryngeal rehabilitation. Most patients have spontaneous recovery within 18 months of cardiac surgery.

Drug-Induced Sleep Endoscopy Findings in Children With Obstructive Sleep Apnea With vs Without Obesity or Down Syndrome.

Importance: Persistent obstructive sleep apnea after adenotonsillectomy is common in children with Down syndrome or obesity. Drug-induced sleep endoscopy could help to identify anatomic differences in these patients that might affect surgical decision-making. Objective: To assess drug-induced sleep endoscopy findings in surgically naive children with obstructive sleep apnea with obesity or Down syndrome and compare these findings with children without obesity or Down syndrome. Design, Setting, and Participants: This cross-sectional analysis of data from a prospective cohort study of patients enrolled between May 1, 2015, and December 31, 2019, was conducted at an academic tertiary care children's hospital and included a consecutive sample of surgically naive children (age 2-18 years) who underwent drug-induced sleep endoscopy at the time of adenotonsillectomy for sleep-disordered breathing. Indications for sleep endoscopy included severe sleep apnea, age older than 7 years, obesity, African American race, and Down syndrome. Exposures: Drug-induced sleep endoscopy. Main Outcomes and Measures: Sleep endoscopy findings were scored according to the Sleep Endoscopy Rating Scale. Ratings at 6 anatomic levels for children with obesity and those with Down syndrome were compared with controls without obesity or Down syndrome using several measures of effect size (Cohen d, Cramer V, and η2). Results: A total of 317 children (158 girls [50%]; 219 [69%] White, 20 [6%] Black, and 103 [34%] Hispanic; mean [95% CI] age, 9.6 [9.2-10.0] years) were included, of whom 115 (36%) were controls without obesity or Down syndrome, 179 (56%) had obesity without Down syndrome, and 23 (7%) had Down syndrome. The mean apnea-hypopnea index was 16 (95% CI, 13-19), and the mean minimum O2 saturation was 83% (95% CI, 81%-85%). Compared with controls without obesity or Down syndrome, children with Down syndrome demonstrated greater overall obstruction (mean sleep endoscopy rating scale total score of 5.6 vs 4.8; Cohen d, 0.46), and greater tonsillar (percentage of complete obstruction: 65% vs 54%), tongue base (percentage of complete obstruction: 26% vs 12%), and arytenoid obstruction (percentage of at least partial obstruction, 35% vs 6%). Children with obesity had greater tonsillar (percentage of complete obstruction, 74% vs 54%) and less base of tongue obstruction (percentage of complete obstruction, 2% vs 12%) compared with controls. Conclusions and Relevance: In this cohort study, surgically naive children with obesity with obstructive sleep apnea had predominantly tonsillar obstruction, whereas children with Down syndrome demonstrated greater obstruction of the tonsils, tongue base, and arytenoids compared with controls. Routine drug-induced sleep endoscopy should be considered in surgically naive children with Down syndrome to help inform the surgical plan.

Endoscopic ear surgery in children with down syndrome.

INTRODUCTION: Children with Down syndrome (DS) have a high incidence of chronic middle ear disease. Surgery to manage this disease is challenging due to the severity of illness and narrow ear canal dimensions. Endoscopic ear surgery is used to manage tympanic membrane and middle ear disease with the advantages of improved visualization and avoidance of post-auricular incisions. However, its application in children with DS has not been reported. We aim to compare the outcomes of endoscopic versus microscopic ear surgery in children with DS. METHODS: All patients with DS who underwent tympanoplasty without mastoidectomy between 2012 and 2018 were identified, and their charts retrospectively reviewed. Rate of residual perforation, hearing, surgical time, and surgical details were recorded. RESULTS: 37 surgeries in 26 patients were identified that met inclusion criteria. Two subgroups were analyzed. The first included 14 cases that were done using traditional microscopic visualization (MV). The second included 17 cases that had substantial or exclusive use of endoscopic visualization (EES). Due to a learning curve, the number of cases done endoscopically increased over time. The average age in MV was 13.9 years vs 11.0 in EES. The MV cases included 2 with cholesteatoma vs 4 in EES. In cases with adequate follow up, residual perforations were found in 1/13 MV, and 4/17 EES. All of the residual perforation cases in EES used acellular porcine submucosa grafts. None of the cases in MV used this material. Average air bone gap reduction was seen in both groups; 4.2 dB in MV, 9.8 dB in EES. Average surgical time was similar between groups; 124 min in MV, 115 min in EES. All cases in MV required a post-auricular incision and approach to the middle ear. Only four cases in EES required this approach. Six cases in EES did not require any incision outside of the ear canal for either graft harvest or middle ear approach. CONCLUSION: Endoscopic and microscopic ear surgery in children with DS have similar outcomes. There were no statistical differences in hearing results, surgical times, or residual tympanic membrane perforations, although the rate of perforations in the endoscopic group trended higher. Most endoscopic cases did not require conversion to a post-auricular approach. Endoscopic surgery allows some DS patients to avoid any incision outside of the ear canal.

Multicenter Interrater Reliability in the Endoscopic Assessment of Velopharyngeal Function Using a Video Instruction Tool.

OBJECTIVE: Assess interrater agreement of endoscopic assessment of velopharyngeal (VP) function before and after viewing the video instruction tool (VIT). We hypothesized improvement in interrater agreement using the Golding-Kushner scale (GKS) after viewing the VIT. STUDY DESIGN: Prospective study. SETTING: Multi-institutional. METHODS: Sixteen fellowship-trained pediatric otolaryngologists who treat velopharyngeal insufficiency (VPI) rated 50 video segments using the GKS before and after watching the VIT. Raters assessed gap size percentage and lateral pharyngeal wall (LPW), soft palate (SP), and posterior pharyngeal wall (PPW) movement. Intraclass correlation coefficient was estimated for these continuous measures. Raters also indicated the presence of a palatal notch, Passavant's ridge, and aberrant pulsations (categorical variables). Fleiss κ coefficient was used for categorical variables. Wilcoxon signed-rank test was performed on the difference between the pre/post individual video ratings. RESULTS: Reliability improved for all continuous variables after watching the instructional video. The improvement was significant for PPW (0.22-0.30, P < .001), SP (left: 0.63-0.68, P < .001 and right: 0.64-0.68, P = .001), and LPW (left: 0.49-0.54, P = .01 and right: 0.49-0.54, P = .09) but not significant for gap size (0.65-0.69, P = .36). Among categorical variables, agreement on Passavant's ridge significantly improved (0.30-0.36, P = .03). CONCLUSION: Exposure to a video instruction tool improves interrater agreement of endoscopic assessment of VP function. Significant improvement was observed in our primary end points, specifically posterior pharyngeal wall movement, soft palate movement, and lateral pharyngeal wall movement. There was less impact of the VIT on the interrater agreement of the categorical variables, palatal notch, Passavant's ridge, and aberrant pulsations.

Fidget Spinner Ingestions in Children-A Problem that Spun Out of Nowhere.

The Consumer Product Safety Risk Management System's injury and potential injury database records 13 cases of fidget spinner ingestion since 2016. In addition to a database query, we report 3 additional cases of fidget spinner ingestion to describe patient presentations and subsequent management strategies.

Assessment of pediatric obstructive sleep apnea using a drug-induced sleep endoscopy rating scale.

OBJECTIVES/HYPOTHESIS: Assess the reliability of a Sleep Endoscopy Rating Scale (SERS) and its relationship with pediatric obstructive sleep apnea (OSA) severity. STUDY DESIGN: Retrospective case series of pediatric patients who underwent drug-induced sleep endoscopy (DISE) at the time of surgery for OSA from January 1, 2013 to May 1, 2014. METHODS: Three blinded otolaryngologists scored obstruction on DISE recordings as absent (0), partial (+1), or complete (+2) at six anatomic levels: nasal airway, nasopharynx, velopharynx, oropharynx, hypopharynx, and arytenoids. Ratings were summed for a SERS total score (range, 0-12). Reliability was calculated using a κ statistic with linear weighting. SERS ratings and obstructive apnea-hypopnea index (OAHI) were compared using Spearman correlation. A receiver operating characteristic (ROC) analysis determined the ability of the SERS total score to predict severe OSA (OAHI >10). RESULTS: Thirty-nine patients were included (mean age, 8.3 ± 5.1 years; 36% obese; mean OAHI, 19.1 ± 23.7). Intrarater and inter-rater reliability was substantial-to-excellent (κ = 0.61-0.83) and fair-to-substantial (κ = 0.33-0.76), respectively. Ratings correlated best with OAHI for the oropharynx (r = 0.54, P = .02), hypopharynx (r = 0.48, P = .04), and SERS total score (r = 0.75, P = .002). In ROC analysis, a SERS total score ≥6 demonstrated sensitivity/specificity of 81.8%/87.5%, respectively, and correctly classified 84% of patients. CONCLUSIONS: The SERS can be applied reliably in children undergoing DISE for OSA. Ratings of the oropharynx, hypopharynx, and SERS total score demonstrated significant correlation with OSA severity. A SERS total score ≥6 was an accurate predictor of severe OSA. LEVEL OF EVIDENCE: 4. Laryngoscope, 126:1492-1498, 2016.

Pediatric airway reconstruction with a prefabricated auricular cartilage and radial forearm free flap.

Prefabricated composite free flaps for complex airway reconstruction have been described for an adult series at our institution. We extended this approach to a pediatric patient with lifelong subglottic stenosis who had failed previous open airway reconstructions. A staged procedure was utilized in which a composite graft was created using conchal cartilages and a radial forearm free flap. This reconstruction improved the patency of her airway and decreased her dependency on intermittent airway dilations. Airway reconstruction with prefabricated conchal cartilage composite free flaps may be used as a salvage procedure for complex pediatric airway reconstruction when other methods have failed.

The laryngeal mask airway for pediatric adenotonsillectomy: predictors of failure and complications.

OBJECTIVES: We hypothesize that the laryngeal mask airway (LMA) is a safe technique for airway management in pediatric adenotonsillectomy (T&A). METHODS: After institutional review board (I.R.B.) approval, we conducted a retrospective review of 1199 medical records of children who underwent T&A from 2002 to 2006 at Doernbecher Children's Hospital, a teaching institution in Portland, OR. There were no significant demographic differences between the LMA (n=451), endotracheal tube (ETT) (n=715), and failed LMA groups (n=33). Outcome variables were LMA failure (LMA replaced with endotracheal tube), and any complication. We collected demographic and medical data to determine the incidence and predictors of LMA failure, and to characterize the failed LMA group. RESULTS: The incidence of LMA failure was 6.8%. Patients who underwent adenoidectomy had significantly lower odds of LMA failure compared to patients who had a tonsillectomy or adenotonsillectomy (OR 0.28, 95% CI 0.15-0.52, P<0.0001). One of the surgeons (OR 0.46, 95% CI 0.45-0.48, P<0.0001) was also associated with decreased odds of LMA failure. Controlled ventilation (OR 7.17, 95% CI 4.99-10.32, P<0.0001), and younger patients (OR 1.05 for each year decrease in age, 95% CI 1.03-1.07, P ≤ 0.0001) were associated with increased odds of LMA failure. The complication rate was 14.2% in the LMA group and 7.7% in the ETT group. Increased odds of developing any complication were seen in male patients (OR 1.4, 95% CI 1.01-1.7, P=0.04), and in patients with co-morbidities other than obstructive sleep apnea syndrome or upper respiratory tract infection (OR 4.2, 95% CI 1.03-17.2, P=0.04). The odds of developing a complication were lower in the ETT group compared to the LMA group (0.63, 0.46, 0.8, P=0.005). CONCLUSIONS: LMA use for pediatric T&A is associated with a higher incidence of complications, mainly as a result of airway obstruction following insertion of the LMA or McIvor gag placement. Complications were more likely if tonsillectomy was performed when compared to adenoidectomy alone. Appropriate patient selection, careful insertion, and avoidance of controlled ventilation may decrease the incidence of LMA failure, especially if tonsillectomy is performed. The ability of surgeons to work around the LMA can modify the failure rate significantly.

Generation of consensus in the application of a rating scale to nasendoscopic assessment of velopharyngeal function.

OBJECTIVE: To generate consensus ratings of velopharyngeal function on nasendoscopy (NE) with the goal of creating a video instruction tool. METHODS: The American Society of Pediatric Otolaryngology Velopharyngeal Insufficiency Study Group convened to identify NE segments to be included in an instructional video. Of 24 segments reviewed, 11 were selected based on the quality of the examinations and spectrum of closure patterns. Participating otolaryngologists independently rated NE segments using the Golding-Kushner scale. The participants then convened and rated each of the NE segments as a group. Thirty-nine members of the American Society of Pediatric Otolaryngology met and agreed with the group ratings, creating a consensus standard. RESULTS: Individual scores for palate and lateral wall motion showed high variability, ranging from 0 to 6 points difference from the consensus. Variability was also seen for the following qualitative findings: the Passavant ridge, aberrant pulsations, and dorsal palatal notch. The individual ratings are presented graphically to demonstrate the range of individual responses as well as to compare responses to the consensus ratings. No further changes were made to the proposed consensus ratings when reviewed by the larger group. CONCLUSIONS: Rating of NE evaluations of velopharyngeal function was variable among a group of pediatric otolaryngologists experienced in treating velopharyngeal insufficiency. These results highlight the need to develop a standardized method of reporting NE findings for velopharyngeal insufficiency. Despite this, consensus ratings were achieved that will facilitate development of a video instruction tool.

Effects of oropharyngeal surgery on velopharyngeal competence.

PURPOSE OF REVIEW: This article addresses the question of whether it is safe to perform pharyngeal surgery on children who are at risk for speech disorders. Patients with orofacial clefts or craniofacial disorders often have speech and resonance disorders that affect their understandability. They are also prone to develop sleep apnea or other diseases that affect Waldeyer's ring. RECENT FINDINGS: There is increasing recognition of sleep disordered breathing and obstructive sleep apnea syndrome (OSAS) in the group of children who have, or are at risk of developing, speech disorders. Often, these children have a dysfunctional velopharyngeal valve which results in velopharyngeal insufficiency (VPI) and unintelligible speech. There are also children, otherwise thought to be normal, who develop VPI after adenoidectomy or tonsillectomy. Greater knowledge of the causes for VPI has led to the strategies limiting the risk of speech disorders after tonsillectomy or adenoidectomy. SUMMARY: Children at risk for VPI who have OSAS may not experience deterioration of their speech understandability when a careful tonsillectomy with or without partial adenoidectomy is done. Coordination between the treating providers is paramount for successful outcomes for both the problems.

Innate immunity gene single nucleotide polymorphisms and otitis media.

OBJECTIVE: Toll-like receptors (TLR) activate the innate immune system. Single nucleotide polymorphisms (SNPs) in TLR genes are linked to increased susceptibility to infections. TLR4-deficient mice have increased incidence and duration of otitis media. We hypothesize that SNPs in TLR genes are more common in otitis-prone children than in children without a history of otitis media. METHODS: Cases (n=70) included children undergoing surgery for otitis media. Control subjects (n=70) included children undergoing surgery for non-otologic indication. Genomic DNA was extracted from blood samples. RT-PCR genotyping was performed for TLR2 (rs5743708), TLR4 (rs4986790 and rs4986791), TLR9 (rs5743836 & rs187084), and CD14 (rs2569190). RESULTS: There were no significant differences between the groups in family history, day care, smoke exposure, allergies or prevalence of the SNPs. The most common pre-op diagnosis in control subjects was obstructive sleep apnea (OSA). CONCLUSIONS: TLR2, TLR4, TLR9 and CD14 gene SNPs were not more prevalent in otitis-prone children.

Efficacy of tonsillectomy for pediatric patients with Dysphagia and tonsillar hypertrophy.

OBJECTIVE: To determine the effectiveness of tonsillectomy for the treatment of dysphagia related to tonsillar hypertrophy. DESIGN: Prospective cohort study. SETTING: Tertiary care pediatric otolaryngology practice. PARTICIPANTS: Eighty-five children aged 2 to 14 years referred for tonsillectomy owing to dysphagia related to tonsillar hypertrophy (dysphagia cohort) or for other indications (control cohort). INTERVENTIONS: Swallowing Quality of Life (SWAL-QOL) dysphagia questionnaires were administered at the initial clinic visit, on the day of surgery, and at 1 month and 6 months after surgery. Patients were weighed on the day of surgery and at 1 month after surgery. MAIN OUTCOME MEASURES: The primary outcome measure was the SWAL-QOL score. Secondary outcome measures were the type of diet consistency patients tolerated at home and the weight percentile for age. RESULTS: Of 85 patients enrolled, 57 went on to have surgery, completed at least 1 postoperative questionnaire, and were included in the data analysis. At 1 month after tonsillectomy, the dysphagia cohort (n = 18) demonstrated improved SWAL-QOL scores (mean [SD], 58.4 [4.8] before surgery vs 82.4 [5.3] after surgery; P < .001), more patients tolerating a regular diet (12 of 37 patients [33.3%] before surgery vs 22 of 36 [60.0%] after surgery, P = .01), and increased weight percentile for age (mean [SD], 36.5 [10.7] before surgery vs 50.0 [10.6] after surgery; P = .01). Similarly, at 1 month after tonsillectomy, the control cohort (n = 39) demonstrated improved SWAL-QOL scores (mean [SD], 80.8 [2.6] before surgery vs 91.7 [1.8] after surgery; P < .001), more patients tolerating a regular diet (30 of 37 patients [81.1%] before surgery vs 34 of 36 patients [94.4%] after surgery, P = .04), and increased weight percentile for age (mean [SD], 62.8 [5.4] before surgery vs 70.4 [5.1] after surgery; P = .003). CONCLUSIONS: Dysphagia related to tonsillar hypertrophy is a significant problem not only among children with dysphagia with a primary complaint but also among a large subset of patients referred for tonsillectomy for other indications. Following tonsillectomy, both groups experience significant improvement in swallowing-related quality of life, ability to tolerate a regular diet, and weight percentile for age. Tonsillectomy is an effective treatment for the management of dysphagia related to tonsillar hypertrophy in children.

The effect of the palatoplasty method on the frequency of ear tube placement.

OBJECTIVE: To determine whether the type of palate repair affects the frequency of subsequent ventilation tube placement. DESIGN: Combined retrospective and prospective cohort with more than 2 years clinical follow-up after palatoplasty. SETTING: Tertiary care children's hospital and clinic. PATIENTS: A total of 170 patients with cleft palate (with or without cleft lip) underwent palatoplasty between 1995 and 2003. Sixty-nine patients with less than 2 years of follow-up visits and 1 patient who did not require ear tubes were excluded from this analysis. INTERVENTIONS: Either traditional 2-flap palatoplasty (group A) or double-opposing Z-plasty (group B) was performed. The type of palatoplasty performed was based on the reconstructive surgeon's clinical decision. Ventilation tubes were placed for otitis media, conductive hearing loss, or eustachian tube dysfunction. Patients received routine follow-up care every 6 months or whenever acute problems arose. Data were analyzed with independent t tests, chi(2) tests, and Fisher exact tests. MAIN OUTCOME MEASURES: Number of ear tubes placed after palatoplasty in each group. RESULTS: Group A had a mean (SE) of 2.9 (0.2) sets of tubes placed, while group B had a mean (SE) of 1.8 (0.2) sets of tubes. Group A had significantly more sets of ventilation tubes placed (P < .001) than group B. Subgroup analysis based on type of cleft was performed. CONCLUSION: Children with cleft palate who underwent double-opposing Z-plasty had fewer sets of ear tubes placed postoperatively than patients who had traditional repair.

Multicenter interrater and intrarater reliability in the endoscopic evaluation of velopharyngeal insufficiency.

OBJECTIVE: To explore interrater and intrarater reliability (R (inter) and R (intra), respectively) of a standardized scale applied to nasoendoscopic assessment of velopharyngeal (VP) function, across multiple centers. DESIGN: Multicenter blinded R (inter) and R (intra) study. SETTING: Eight academic tertiary care centers. PARTICIPANTS: Sixteen otolaryngologists from 8 centers. MAIN OUTCOME MEASURES: Raters estimated lateral pharyngeal and palatal movement on nasoendoscopic tapes from 50 different patients. Raters were asked to (1) estimate gap size during phonation and (2) note the presence of the Passavant ridge, a midline palatal notch on the nasal surface of the soft palate, and aberrant pulsations. Primary outcome measures were R (inter) and R (intra) coefficients for estimated gap size, lateral wall, and palatal movement; kappa coefficients for the Passavant ridge, a midline palatal notch on the nasal soft palate, and aberrant pulsations were also calculated. RESULTS: The R (inter) coefficients were 0.63 for estimated gap size, 0.41 for lateral wall movement, and 0.43 for palate movement; corresponding R (intra) coefficients were 0.86, 0.79, and 0.83, respectively. Interrater kappa values for qualitative features were 0.10 for the Passavant ridge; 0.48 for a notch on the nasal surface of the soft palate, 0.56 for aberrant pulsations, and 0.39 for estimation of gap size. CONCLUSIONS: In these data, there was good R (intra) and fair R (inter) when using the Golding-Kushner scale for rating VP function based on nasoendoscopy. Estimates of VP gap size demonstrate higher reliability coefficients than total lateral wall, mean palate estimates, and categorical estimate of gap size. The reliability of rating qualitative characteristics (ie, the presence of the Passavant ridge, aberrant pulsations, and notch on the nasal surface of the soft palate) is variable.

Pediatric vocal fold paralysis after cardiac surgery: rate of recovery and sequelae.

OBJECTIVE: To determine the rate of recovery of pediatric vocal fold paralysis (VFP) after cardiac surgery. STUDY DESIGN AND SETTING: Retrospective case series from January 2000 to 2005 at 4 tertiary care pediatric hospitals. RESULTS: A total of 109 children with VFP were identified. Of 80 patients with follow-up >3 months, 28 (35%) recovered vocal fold function with a median time to diagnosis of recovery of 6.6 months. Fifty-two (65%) patients had persistent vocal fold paralysis with a median follow-up time of 16.4 months. Twenty-five (45%) of 55 patients demonstrated aspiration or laryngeal penetration with modified barium swallow. Twenty-nine (27%) of the 109 patients underwent surgical intervention for their airway, feeding, or voice. CONCLUSIONS: Pediatric VFP is not an uncommon complication after cardiac surgery and can result in serious sequelae. This study demonstrates a 35% rate of recovery, 45% rate of aspiration, and 27% rate of complications that require surgical intervention.

Surgical outcomes for velopharyngeal insufficiency in velocardiofacial syndrome and nonsyndromic patients.

OBJECTIVE: To compare speech outcomes after operative intervention for velopharyngeal insufficiency between velocardiofacial syndrome patients and nonsyndromic patients. DESIGN: Retrospective cohort study. SETTING: Tertiary academic center. PATIENTS: Cohorts of 14 velocardiofacial syndrome and 15 nonsyndromic patients without overt cleft palate who underwent operative procedures to correct velopharyngeal insufficiency. All velocardiofacial syndrome patients were positive for 22q11.2 microdeletion by fluorescent in situ hybridization and possessed phenotypic features of velocardiofacial syndrome. INTERVENTIONS: Operative procedures, including sphincter pharyngoplasty, Furlow palatoplasty, or both, were selected based on preoperative endoscopic assessments of velopharyngeal motion and residual gap size and shape, as well as velocardiofacial syndrome status. Five single and 9 combined procedures were performed in the velocardiofacial syndrome group, whereas 13 single and 2 combined procedures were performed in the nonsyndromic group. OUTCOME MEASURES: Pre- and post-op evaluation was conducted by a speech pathologist. Assessment parameters scored on a numerical scale included speech intelligibility, resonance, nasal air emissions, and overall severity of velopharyngeal insufficiency. Postoperative complications were recorded. RESULTS: Most velocardiofacial syndrome patients and nonsyndromic patients demonstrated significant improvements in all parameters. Comparison of the two groups demonstrated similar improvements in both. Changes in speech resonance were significantly different between the two groups, whereas other speech parameters did not reach significance. There was no difference in airway complications between groups. CONCLUSIONS: Velocardiofacial syndrome patients may have comparable outcomes to nonsyndromic patients in selective surgical management of velopharyngeal insufficiency. In addition, the data demonstrate the efficacy of a single-stage combined procedure without increased morbidity.

Otolaryngologic manifestations of PHACE syndrome.

Two case reports of PHACE syndrome (posterior fossa malformations (P), hemangiomas (H), arterial anomalies (A), coarctation of the aorta and cardiac defects (C), and eye abnormalities (E)) are presented. Clinical characteristics consisted of cutaneous and airway hemangiomas, aortic coarctation, and left superior vena cava in one child and cutaneous and airway hemangiomas, sternal clefting, and supraumbilical raphe in the other child. Treatment modalities included systemic and intralesional steroids for cutaneous and airway hemangiomas, submucosal resection and laryngotracheal reconstruction for airway hemangiomas, repair of aortic coarctation, and laser treatment of cutaneous hemangiomas. PHACE syndrome poses a significant potential for airway compromise from hemangiomas, which may require multimodality treatment.

Intravenous sedation vs general anesthesia for pediatric otolaryngology procedures.

OBJECTIVE: To compare efficacy, safety, and hospital charges for common pediatric otolaryngology procedures with the use of intravenous sedation (IVS) vs general anesthesia (GA). DESIGN: Retrospective chart study. SETTING: Hospital-based pediatric otolaryngology practice. PATIENTS: Patients younger than 18 years who underwent tympanostomy tube removal and/or patch myringoplasty with absorbable gelatin sponge, nasal ciliary biopsy, fine-needle aspiration, or other minor procedures between September 1, 1998, and August 31, 2001. INTERVENTIONS: Procedures performed in 2 settings: outpatient clinic with IVS or operating room with GA. MAIN OUTCOME MEASURES: Procedure completion rate, tympanic membrane perforation rate after ear procedures, complications, and hospital charges. RESULTS: Of 103 procedures, 54 were performed with IVS and 49 with GA. Within the GA group, 32 of 49 patients had additional operations performed and were excluded from analysis of safety and hospital charges. Procedure completion rate was 100% in both groups. The most common procedure was tympanostomy tube removal with patch myringoplasty (IVS, 52 ears; GA, 42 ears). The rate of persistent tympanic membrane perforation was similar between these groups (IVS, 7 [16%] of 45 ears; GA, 5 [15%] of 33; P =.96). All complications were minor and occurred at similar rates (IVS, 10 [19%] of 54 ears; GA, 3 [18%] of 17; P =.94). These events included hypoxia, airway obstruction, and bradycardia, all of which resolved spontaneously or responded to noninvasive interventions such as oxygen or repositioning. Average hospital charges were significantly higher for the GA group (IVS, $356.22; GA, $1516.55; P<.001). CONCLUSION: Various procedures can be performed safely, effectively, and with decreased hospital charges with the use of IVS administered by a pediatric sedation service.

Intralesional cidofovir for the treatment of severe juvenile recurrent respiratory papillomatosis: long-term results in 4 children.

OBJECTIVE: We sought to determine the efficacy of intralesional injection of cidofovir in improving resolution of recurrent respiratory papillomatosis (RRP). Study design and setting We conducted a prospective, observational trial at an academic tertiary children's hospital. RESULTS: Four children with RRP requiring more than 6 surgical excisions per year were treated with intralesional cidofovir. Cidofovir (5 mg/mL) was injected into airway sites where papillomas had just been excised using sharp technique. Each patient had 6 treatments performed 6 to 8 weeks apart. Biopsies confirmed benign papilloma lesions in all cases. During treatment with intralesional cidofovir there was diminished growth of the papillomas in each patient. Once cidofovir treatment was stopped, the rate of regrowth and frequency of surgical excision returned to pretreatment levels in 3 of the 4 patients. CONCLUSIONS: Intralesional cidofovir may provide benefit in reducing the rate of RRP growth while under treatment, but RRP severity returned to pretreatment levels once cidofovir treatment was stopped using this treatment program.