Alessandro de Alarcon

Characterization of congenital anomalies in individuals with choanal atresia.

OBJECTIVE To review a tertiary care pediatric hospitals experience with choanal atresia and stenosis (CA/S) related to associated congenital anomalies (birth defects, including minor abnormalities) and genetic disorders. DESIGN Retrospective case series. SETTING Tertiary care pediatric hospital. PATIENTS Individuals with CA/S. MAIN OUTCOME MEASURES Identification of congenital anomalies, neurologic abnormalities, and developmental disabilities in individuals with CA/S. RESULTS One hundred twenty-nine individuals with CA/S were evaluated between July 1, 1997, and July 1, 2007. Choanal atresia and stenosis was an isolated finding in 34 patients (26.4%) and was associated with other anomalies in 95 patients (73.6%). Specific conditions were diagnosed in 66 patients (51.2%); CHARGE (coloboma, heart defect, atresia choanae, retarded growth, genitourinary abnormalities, and ear anomalies) syndrome was the most common diagnosis (33 patients [25.6%]). Numerous conditions were seen, including chromosomal abnormalities, single-gene defects, deformations, and those caused by teratogens. Choanal atresia and stenosis was unilateral in 62 patients (48.1%) and was bilateral in 60 patients (46.5%). Unilateral cases were more likely to be isolated (30 patients [53.2%]). Bilateral cases were more likely to be associated with specific disorders or multiple congenital anomalies (60 patients [98.4%]). There was no difference in laterality among unilateral cases. CONCLUSIONS Choanal atresia and stenosis is associated with a wide range of disorders. Congenital anomalies, neurologic abnormalities, and developmental disabilities are commonly identified in affected individuals. Bilateral CA/S is more commonly seen in patients in whom specific diagnoses or other congenital anomalies are identified. Unilateral CA/S occurs more frequently in isolated cases. A comprehensive evaluation is recommended in individuals with CA/S to evaluate for other congenital anomalies, neurologic abnormalities, developmental delays, and evidence of a specific underlying disorder.

Endoscopic airway management in children

Purpose of reviewTo discuss the current status of endoscopic airway surgery in children. Recent findingsMore refined endoscopic instruments have been introduced, including balloon dilators, powered debriders, lasers with more exact modes of delivery, and innovative suspension laryngoscopes. The use of balloon dilatation for primary management of acquired subglottic stenosis is promising. Microdebriders are now considered a viable option to the CO2 laser for the management of a number of airway diseases. Knowledge regarding supraglottoplasty continues to evolve. Endoscopic vocal cord lateralization is being successfully used at some centers for the management of bilateral vocal cord paralysis. The da Vinci Surgical Robot (Intuitive Surgical, Inc., Sunnyvale, California, USA) has been introduced to facilitate endoscopic laryngeal cleft repair. SummaryEndoscopic airway surgery is presently regaining its early (1960s) popularity. This trend can be attributed to the availability of new and more sophisticated endoscopic instrumentation, the adjunctive use of new pharmaceuticals, and the realization that open and endoscopic techniques can often be used in a complementary fashion.

Outcomes of Mandibular Distraction Osteogenesis in the Treatment of Severe Micrognathia

IMPORTANCE Patients with severe micrognathia are predisposed to airway obstruction. Mandibular distraction osteogenesis (MDO) is an alternative to tracheotomy that lengthens the mandible in order to improve the retrolingual airway. This study presents outcomes from one of the largest cohorts reported. OBJECTIVE To assess the rate and predictors of surgical success and complications among (1) patients who underwent MDO prior to other airway procedures (MDO first), and (2) patients who required an initial tracheotomy and were subsequently treated with MDO (tracheotomy first). DESIGN, SETTING, AND PARTICIPANTS Retrospective cohort study at a tertiary care pediatric medical center of patients diagnosed as having micrognathia resulting in symptomatic airway obstruction (Pierre Robin sequence) and who underwent MDO from September 1995 to December 2009. INTERVENTIONS Electronic medical records were reviewed. Multivariable regression analysis was used to assess for predictors of outcome. MAIN OUTCOMES AND MEASURES Rates of surgical success (defined as either tracheotomy avoidance or decannulation) and complications. Potential predictors included demographics, syndrome presence, follow-up time, and surgical history. RESULTS A total of 123 patients (61 in MDO-first subgroup, 62 in tracheotomy-first subgroup) underwent MDO during the study period. Median age at time of distraction was 21 months (range, 7 days-24 years). Surgical success and complication rates were 83.6% and 14.8% in the MDO-first subgroup and 67.7% and 38.7% in the tracheotomy-first subgroup. Tracheotomy-first patients were more likely to have a syndromic diagnosis (66.0% vs 43.0%; P = .009) and were older at the time of MDO (median age, 30 months vs 5.1 months; P < .001). Poorer odds of success were associated with the need for 2 or more other airway procedures (odds ratio [OR], 0.14 [95% CI, 0.02-0.82]) in the MDO-first subgroup and craniofacial microsomia or Goldenhar syndrome (OR, 0.07 [95% CI, 0.009-0.52]) in the tracheotomy-first subgroup. CONCLUSIONS AND RELEVANCE Mandibular distraction osteogenesis has a high rate of success in avoiding tracheotomy. Patients who required a tracheotomy before MDO had a lower success rate in achieving decannulation and a higher rate of complications. However, these patients also had a higher rate of syndromic diagnoses and associated comorbidities. Patients with Goldenhar syndrome have a decreased likelihood of surgical success.

Revision Pediatric Laryngotracheal Reconstruction

Surgeons who make airway reconstruction a major part of their practice inevitably are faced with children in whom initial surgical management fails. Searching for the possible causes of failure and determining how best to minimize the risk of repeated failure is often an exhaustive process. Establishing a framework and guidelines for approaching and managing these failures can improve the chances of success significantly. The aim of this article is to provide such a framework.

Swallowing function after laryngeal cleft repair: More than just fixing the cleft

To evaluate and describe the swallowing function in children after laryngeal cleft repair.

Comparison of Pediatric Voice Handicap Index Scores with Perceptual Voice Analysis in Patients following Airway Reconstruction

Objectives We performed a retrospective review to compare a subjective parental proxy-derived voice handicap survey to an observer-derived method of measuring voice perturbation in children who have undergone airway reconstruction. The main outcome measures were the Pediatric Voice Handicap Index (pVHI) total score and the Overall Severity score on the Consensus Auditory-Perceptual Evaluation of Voice (CAPE-V). Methods The percent Overall Severity CAPE-V score (score divided by 100) and the percent pVHI score (score divided by 92) were calculated. A Wilcoxon signed rank test was used to compare CAPE-V scores with the pVHI total scores. The relationship between the pVHI scores and the CAPE-V scores was investigated with a Spearman correlation. Subgroup analysis was performed to determine the relationship of surgery type to CAPE-V and pVHI scores. Results Fifty subjects with a history of airway surgery who were evaluated between 2005 and 2008 were identified. Forty-two of the 50 subjects had complete data for review. Their median age was 7.1 years (range, 3.3 to 17.9 years). Their pVHI total scores had a median of 30 (range, 1 to 80). Their Overall Severity CAPE-V scores had a median of 50.5 (range, 0 to 98). Their median CAPE-V percent was higher than their median pVHI percent (50.5% versus 32.6%; p = 0.0003). A weak correlation was found between the Overall Severity CAPE-V score and the pVHI total score (rho = 0.41; p = 0.0003). There was a trend toward higher Overall Severity CAPE-V scores in patients who underwent cricotracheal resection. The total number of airway surgeries was significantly correlated with the Overall Severity CAPE-V score (rho = 0.6; p < 0.0001) but not with the pVHI score. Conclusions Children who undergo airway reconstruction often have a resulting voice disturbance that can affect their lives in multiple dimensions. The results of this study revealed a weak-to-fair correlation between the parent-reported pVHI total score and expert ratings of voice quality using the CAPE-V. In this patient population, both of these tools provided important information regarding the relationship of the severity of voice disturbance to its handicapping effects.

Characterization of supraglottic phonation in children after airway reconstruction.

Objectives We examined select acoustic (signal type), aerodynamic, and perceptual measures and associated surgical data in a cohort of children who were endoscopically identified as using supraglottic phonation after undergoing airway reconstruction. Methods Twenty-one children (4 to 18 years of age) who were seen in the Cincinnati Childrens Hospital Medical Center for Pediatric Voice Disorders and identified as using supraglottic phonation were included in this study. According to standard protocol, each of these children underwent acoustic, aerodynamic, and perceptual analyses and laryngeal imaging. Their medical records were reviewed for surgical history. Results Four primary supraglottic compression patterns and 3 distinct sound sources for voice were identified. Signal type classification revealed that 20 of 21 voice signals were either type II or type III. Signal type was moderately associated with compression pattern (p = 0.01). No statistically significant findings were found in testing the Consensus Auditory Perceptual Evaluation of Voice (CAPE-V) Overall Severity score against compression patterns and vibration source. The mean Strain scores for participants who used a combined source of vibration were significantly higher than for those who used their ventricular folds. Conclusions The compensatory compression patterns and alternate sources of vibration used by these children resulted in moderate to severe dysphonias. How children compensate after undergoing airway reconstruction has important implications for behavioral and surgical interventions aimed at improving voice quality. Not all aspects of traditional voice evaluation are suitable for this population.

Risk factors for recurrent sinus surgery in cystic fibrosis: review of a decade of experience.

Background Patients with cystic fibrosis (CF) who undergo endoscopic sinus surgery often require multiple revision procedures. Our objective was to identify risk factors for revision sinus surgery in patients with CF, to better identify this subset of patients who might be better suited for alternative interventions at their initial procedure. Methods Patients with CF who presented to our academic tertiary care sinus clinic between 1994 and 2003 were reviewed. Data were collected from CT scans using the Lund-Mackay scale. Data are collected on demographics, comorbidities, CF genotype, number and type of sinus surgeries, and pulmonary function tests. Results Eighty-one patients met inclusion criteria. Fifty patients were <18 years old at presentation. Forty-one patients were Δ508 homozygotes, 32 patients were Δ508 heterozygotes, and 5 patients were non-Δ508 heterozygotes. Respiratory comorbidities were asthma alone (28%) and aspirin triad (5%). Eighteen (22%) patients either smoked or lived with smokers. The mean Lund-Mackay score before the initial surgery was 16. Twenty patients were treated with medication only; 35 patients underwent 1 surgery; 14 patients underwent 2 surgeries; 8 patients underwent 3 surgeries; 2 patients underwent 4 surgeries; and 2 patients underwent 5 surgeries. Patients with higher Lund-Mackay scores at their initial CT were more likely to undergo repeat surgeries (p < 0.05). Conclusion CF patients with high Lund-Mackay scores at their initial surgery are more likely to undergo several revision surgeries. These patients should be considered for more alternative initial management of their sinuses.

High-Dose Sublesional Bevacizumab (Avastin) for Pediatric Recurrent Respiratory Papillomatosis

Objectives: We review and report the use of high-dose bevacizumab for the treatment of recurrent respiratory papillomatosis (RRP) in pediatric patients. Methods: We included all patients with pediatric-onset RRP who underwent bevacizumab (25 mg/mL) injections by a single practitioner. A series of 5 consecutive subepithelial injections were administered at 4- to 6-week intervals with concomitant 532 nm KTP laser ablation. The lesions were staged according to the Derkay staging system. The outcomes included pretreatment and posttreatment Derkay scores, the time interval between procedures, and voice outcomes. The demographic data extracted included sex, age at diagnosis, and current age. Results: Nine patients were included in this study, with 1 patient lost to follow-up; their median age was 8 years (range, 3 to 21 years). The mean bevacizumab dose was 14.25 mg (range, 5 to 45 mg). There was a median Derkay score of 11.5 (range, 4 to 23) at the time of diagnosis and a median 58% improvement following therapy. All patients demonstrated an increased time interval between injections, for a median improvement of 2.05× (range, 1.6× to 3.25×). Conclusions: Evidence exists in support of vascular endothelial growth factor as an important factor in the development of RRP. Although some variability in response is demonstrated by this study, high-dose bevacizumab appears to yield promising results for pediatric patients with RRP.

Cervical slide tracheoplasty.

OBJECTIVE To describe our experience with cervical slide tracheoplasty (CST) in managing complex laryngotracheal disorders. DESIGN Retrospective analysis. SETTING Quaternary care pediatric institution. PATIENTS The study included 29 patients who underwent CST without cardiopulmonary bypass at our institution from January 2003 to January 2011. MAIN OUTCOME MEASURE Surgery-specific and overall operative success. RESULTS The most common airway lesion in our cohort of 29 patients (mean age, 10.7 years) was tracheal stenosis (n = 18); 10 of 18 patients had long-segment acquired tracheal stenosis. Operation-specific success was achieved in 23 of 29 patients (79%), including all 10 patients with long-segment acquired tracheal stenosis. Six patients failed initial CST and required additional surgical procedures. Overall success was achieved in 3 of these patients. Patients with subglottic stenosis (n = 7), concomitant glottic stenosis (n = 4), and multilevel airway lesions (n = 10) had lower operation-specific and overall operative success than did patients with other airway lesions. Four patients (14%) experienced complications. CONCLUSIONS Cervical slide tracheoplasty is a valuable technique that should be added to the surgical armamentarium for patients requiring open airway reconstruction. This technique yields a high success rate in treating patients with a broad spectrum of complex laryngotracheal disorders.