Multilevel Airway Obstruction Research Articles

Long-Term Outcomes of Airway Management in 6 Children With Campomelic Dysplasia.

This case series describes the outcomes of airway management, including airway reconstruction, in 6 patients with campomelic dysplasia and tracheostomy/ventilator dependence secondary to multilevel airway obstruction. Case series and clinical guidelines are provided for the airway management of patients with campomelic dysplasia. Average age of individuals is 19.4 years. Mean follow-up was 12.2 years. Four individuals underwent open airway reconstruction and achieved decannulation. One patient underwent airway reconstruction with improvement of a complete subglottic stenosis but remains ventilator dependent due to severe scoliosis. The remaining 2 patients did not require additional airway reconstruction, have been liberated from ventilator support, and are under evaluation for tracheostomy tube decannulation. Although campomelic dysplasia was historically considered a lethal form of congenital skeletal dysplasia, with many patients succumbing to respiratory failure due to tracheobronchomalacia in the neonatal period, airway reconstruction and long-term survivorship is feasible in children with campomelic dysplasia and significant airway disease.

Approaching Saber-Sheath Trachea in a Patient with Bilateral Vocal Cord Abductor Palsy and Post Tracheostomy Tracheal Stenosis.

Human trachea has variable shapes and the most common are circular and oval-shaped. Other shapes are rare to find and usually related to some diseases. Such as the Saber-sheath shaped trachea, which was commonly attributed to patients with chronic obstructive pulmonary disease (COPD). This case is about a patient who was detected to have dual pathology, bilateral vocal cord abductor palsy, and post-tracheostomy complicated with tracheal stenosis for 40 years. Symptoms worsened in the last three years until he was admitted to the ward. However, upon managing this case with multilevel airway obstruction, we have found out that this patient who did not have COPD, has a Saber-sheath trachea shape. This finding may indicate that the shape is not limited to a certain disease only.

Types of Epiglottic Collapse in Breathing Sleep Disorder and Their Impact in Clinical Practice.

Introduction Obstructive sleep apnea (OSA) is a severe form of sleep-disordered breathing (SDB) that is strongly correlated with comorbidities, in which epiglottic collapse (EC) and other contributing factors are involved. Objectives To evaluate the occurrence of EC in OSA patients through drug-induced sleep endoscopy (DISE) and to determine the factors contributing to EC. Methods A retrospective study of 37 adult patients using medical history. Patients were assessed for laryngopharyngeal reflux (LPR) and lingual tonsil hypertrophy (LTH) using reflux symptom index and reflux finding score (RFS); for OSA using polysomnography, and for airway collapse through DISE. An independent t -test was performed to evaluate risk factors, including the involvement of three other airway structures. Results Most EC patients exhibited trap door epiglottic collapse (TDEC) (56.8%) or pushed epiglottic collapse (PEC) (29.7%). Lingual tonsil hypertrophy, RFS, and respiratory effort-related arousal (RERA) were associated with epiglottic subtypes. Laryngopharyngeal reflux patients confirmed by RFS (t(25) = -1.32, p = 0.197) tended to suffer PEC; LTH was significantly associated (X2(1) = 2.5, p = 0.012) with PEC (odds ratio [OR] value = 44) in grades II and III LTH patients; 11 of 16 TDEC patients had grade I LTH. Pushed epiglottic collapse was more prevalent among multilevel airway obstruction patients. A single additional collapse site was found only in TDEC patients. Conclusion Laryngopharyngeal reflux causes repetitive acid stress toward lingual tonsils causing LTH, resulting in PEC with grade II or III LTH. Trap door epiglottic collapse requires one additional structural collapse, while at least two additional collapse sites were necessary to develop PEC. Respiratory effort-related arousal values may indicate EC.

Transtracheal Pressure for Evaluation of Decannulation Readiness.

Pediatric tracheostomy decannulation protocols vary among institutions and may include toleration of Passy Muir Valve (PMV), microlaryngoscopy and bronchoscopy (MLB) findings, and polysomnography evaluation. Transtracheal pressure (TTP) is an objective measurement utilized to evaluate PMV toleration. We aimed to investigate the role of TTP in decannulation candidates and compare TTP measurements with polysomnography and MLB findings. A retrospective cohort study of children who underwent TTP measurement during PMV trial between December 2012 and November 2022. A total of 79 patients underwent TTP measurement and MLB evaluation; of these, 16 (20.3%) patients had a capped polysomnography. Twenty-eight (35.4%) patients had TTPs ≤10 cm H2O, and 51 (64.6%) patients had TTPs >10 cm H2O. The most common indication for tracheostomy was upper airway obstruction (n = 41, 51.9%), followed by a need for mechanical ventilation (n = 24, 30.4%). Twenty-five (31.6%) patients were decannulated. Patients with TTPs ≤10 cm H2O had a mean Apnea-Hypopnea Index of 0.17 ± 0.26/h compared with 6.93 ± 7.67/h in those with TTPs >10 cm H2O, p = 0.0365. Patients with TTPs >10 cm H2O were found to have a significantly higher occurrence of airway obstruction (96.1% vs. 46.4%, p < 0.0001) and multilevel airway obstruction (70.6% vs. 21.4%, p < 0.0001) on MLB. Neither TTP measured at time of PMV assessment nor capped polysomnography was associated with successful decannulation. TTP measurements at time of PMV evaluation are associated with polysomnography and MLB findings. One-time PMV measurements were not indicative of decannulation success. 4 Laryngoscope, 134:3377-3383, 2024.

Decannulation in Trisomy 21 patients undergoing laryngotracheal reconstruction.

Down Syndrome (DS) patients are more susceptible to either congenital or acquired subglottic stenosis (SGS). This often creates a multilevel airway obstruction and can lead to tracheostomy dependence early in life. As a result, they may require Laryngotracheal Reconstruction (LTR) to achieve decannulation. The primary objective of this study was to assess decannulation rates, time to decannulation and potential barriers to decannulation in DS patients undergoing LTR. We performed a retrospective chart review from 2008 to 2021 of 193 children who underwent LTR for treatment for laryngotracheal stenosis at a stand-alone tertiary children's hospital. The relationship between clinical data and decannulation status was evaluated using multivariable logistic regression and Fisher exact tests. Time to decannulation analysis was performed using Kaplan Meier analysis and evaluated with log-rank and Cox proportional hazards regression. We determined that DS patients carry an inherit risk for decannulation failure compared to the general population (OR: 6.112, P=. 044, CI 1.046-35.730). Of the 8 patients with DS only three were decannulated. Overall, patients with Trisomy 21 had a significantly increased time to decannulation when compared to all LTR patients (P=.008, Log-rank). We found that these patients are more likely to have both suprastomal collapse (P=.0004, Fischer's Exact) and Tracheomalacia (P=.034, Fischer's Exact) compared to all other LTR patients. While post-operative tracheomalacia did not significantly affect decannulation failure (P=.056, Fischer's Exact) it did significantly prolong decannulation in all LTR patients (P=.018, Log-rank). Trisomy 21 patients are at an increased risk for decannulation failure. Our study illustrates that these poor outcomes are likely a result of conditions more commonly found in this cohort including: narrow tracheal caliber, tracheomalacia and hypotonia.

Effect of modified genioglosuss advancement on hyoid bone position: Cephalometric study

ObjectiveTo assess Hyoid bone position and retrolingual airway space after Modified Genioglossus Advancement Surgery by cephalometry in patients with obstructive sleep apnea (OSA). Study designProspective study. SettingZagazig University Hospital. MethodsEighteen patients with moderate to severe OSA having multilevel airway obstruction confirmed by fiberoptic endoscopy during Muller's maneuver and DISE. All patients underwent modified genioglossus advancement surgery associated with antrolateral advancement pharyngoplasty. Beside Polysomnography and Drug induced sleep endoscopy, Cephalometry was done preoperatively and 6 months postoperative. ResultsImproved Polysomnography parameters as Postoperative mean ± SD apnea hypopnea index decreased from 52 ± 17.1 to 17 ± 3 (P < 0.001, 95% confidence interval 27.71 to 42.41). LOS increased from 79.89 ± 4.43% to 83 ± 4.05% (P 0.07, 95% confidence interval −0.31 to 6.97).Cephalometry analysis showed a significant difference between preoperative and postoperative findings, including: Retrolingual airway space at three levels significantly increased; Level 1 from 6.1 ± 1.6 to 8.5 ± 1.7, Level 2 from 10.5 ± 2.4 to 13.9 ± 2.1, Level 3 from 15.7 ± 3.1 to 21 ± 4, H-GN decreased from 51 ± 7 to 39 ± 8, H-MP decreased from 31.6 ± 7.7 to 24.9 ± 7.3, HS decreased from 121 ± 15 to 102 ± 12, H-PH increased from 29 ± 8 to 43 ± 9. With a success rate defined as AHI <20 and a 50% decrease in AHI of the preoperative value, the surgical success rate was 83.33%. ConclusionThis study showed that Modified genioglossus advancement procedures done for OSA patients significantly changed the position of hyoid bone into a more anterior and superior position and this was reflected in the postoperative Polysomnography.

Patterns of airway obstruction of non-acquired origin in children with and without major congenital anomalies

Major congenital anomalies are known to play a role in the management and prognosis of airway obstruction. Most studies assess acquired forms of airway obstruction. Data on congenital or otherwise non-acquired forms of airway obstruction is sparse. In this retrospective, single-institution cohort study, we sought to evaluate and compare the patterns of airway obstruction in children with and without major congenital anomalies, and to assess the impact of management and outcome, irrespective of aetiology. Fifty-five patients were included, 23 with and 32 without underlying major congenital anomalies. Multilevel airway obstruction (usually affecting the nasopharynx, oropharynx, and the trachea) was more common in children with congenital anomalies (91% vs. 41%, p < .001). Consequently, these children required more frequent and earlier surgical management, especially tracheostomy and adenotonsillar surgery.Conclusions: Major congenital anomalies are associated with multilevel airway obstruction and poor functional prognosis. A simple clinical definition considering impact of major congenital anomalies on development and growth may help guide management plans following endoscopic evaluation of the entire airway and flanked by multidisciplinary discussions.What is Known:• Children with major comorbidities display increased disease severity and more prevalent multilevel airway obstruction• Previous studies include both children with acquired and non-acquired forms of airway obstruction; therefore, the actual impact major comorbidities in children with non-acquired causes of airway obstruction remain unclear.What is New:• A total of 42% children in this study population had major comorbidities with and impact on growth and/or psychomotor development, with a higher prevalence of multilevel airway obstruction and worse rates of functional improvement/recovery.• Children with major comorbidities require tracheostomy more often and earlier than those without major comorbidities, and remain tracheostomy-dependent for a longer time.

Complex Airway Management in Patients with Tracheal Cartilaginous Sleeves.

A tracheal cartilaginous sleeve (TCS) is a rare anomaly characterized by anterior fusion of tracheal cartilages. TCS is associated with syndromic craniosynostoses including Apert, Crouzon and Pfeiffer syndromes and FGFR2, FGFR3, and TWIST1 variants. This study presents a 30-year review of patients with syndromic craniosynostosis and TCS and describes diagnostic methods, genetic variants, surgical interventions, and long-term outcomes. Retrospective, single-institution review. This review included patients with syndromic craniosynostosis and TCS treated at Seattle Children's Hospital from 1990 to 2020. Tracheostomy, genetic variants, and additional surgery were primary measures. Fisher's exact test compared need for tracheostomy in patients with proposed high-risk (FGFR2 p.W290 or FGFR2 p.C342) versus low-risk genetic variants. Thirty patients with TCS were identified. Average age at diagnosis was 12 months (range 2-weeks to 7.9-years; standard deviation 19.8 months). Syndromes included Pfeiffer (37%), Apert (37%), and Crouzon (26%). Severe obstructive sleep apnea was present in 76% of patients. Tracheostomy was performed in 17 patients (57%); five were successfully decannulated. Additional interventions included adenotonsillectomy (57%), nasal (20%), laryngeal (17%), and craniofacial skeletal surgery (87%). All patients with Pfeiffer syndrome and FGFR2 p.W290C variants and 83% of patients with FGFR2 p.C342 variants required tracheostomy, differing from other variants (P=.02, odds ratio 33, 95% confidence interval 1.56-697.96). One patient (3%) died. TCS contributes to multilevel airway obstruction in patients with syndromic craniosynostosis. Genetic testing in patients with FGFR2-related syndromic craniosynostoses may identify those at risk of TCS and facilitate early intervention. A better understanding of this patient population may foster individualized airway management strategies and improve outcomes. 4 Laryngoscope, 132:215-221, 2022.

A Case of Multilevel Airway Obstruction Secondary to Paradoxical Reaction to Anti TB Drugs in a Non HIV Patient with Tracheobronchial and Cervical Tuberculous Lymphadenitis

A case report of a young male diagnosed as cervical tuberculous lymphadenitis presenting with rapidly enlarging neck nodes causing airway obstructing secondary to paradoxical reaction to antituberculous drugs. This phenomenon is rare in HIV negative patient. He also had myriad of symptoms and multilevel obstructions. In this case report we discuss the presentation of patient to our center with the management and possible pitfalls.

Sleep Outcomes in Neonates with Pierre Robin Sequence Undergoing External Mandibular Distraction: A Longitudinal Analysis.

Robin sequence is a common cause of upper airway obstruction in newborns. Herein, we report sleep outcomes in neonates undergoing external mandibular distraction osteogenesis. In this retrospective, 14-year, single-institution study of neonates with Robin sequence undergoing mandibular distraction osteogenesis, we compare respiratory parameters and sleep architecture before versus after surgery. Thirty-one neonates were included; age was 13 days (interquartile range, 5 to 34 days) at preoperative polysomnography and 80 days (interquartile range, 50 to 98 days) at postoperative polysomnography. All neonates had severe obstructive sleep apnea at baseline (defined as pre-operative obstructive apnea hypopnea index ≥ 10). Postoperatively, there was a significant reduction in obstructive apnea hypopnea index [38.3 (interquartile range, 23.4 to 61.8) preoperatively versus 9.4 (interquartile range, 5.3 to 17.1) postoperatively; p < 0.0001], and a significant improvement in sleep efficiency and oxygen saturation nadir. Although 26 neonates (84 percent) had a 50 percent reduction in obstructive apnea hypopnea index postoperatively, all neonates had obstructive sleep apnea, and 15 neonates (48 percent) had persistent severe obstructive sleep apnea following surgery. We report the largest cohort of sleep outcomes in neonates with Robin sequence and severe obstructive sleep apnea undergoing external mandibular distraction osteogenesis. Although the severity of obstructive sleep apnea improves postoperatively, the disease persists in all neonates. We propose that neonates undergo polysomnography before and soon after mandibular distraction osteogenesis to objectively assess improvement in obstructive sleep apnea, as they may require additional evaluation for sites of multilevel airway obstruction and treatment. Therapeutic, IV.

Management of A‐Frame Tracheal Deformity in Children With Endoscopic Resection Tracheoplasty

Tracheal A-frame deformity is a known consequence of tracheostomy that may lead to obstruction after decannulation. The goal of this study is to demonstrate the feasibility and success of endoscopic carbon dioxide (CO2 ) laser-assisted tracheoplasty of tracheal A-frame deformity in children. Retrospective case series of symptomatic children with tracheal A-frame deformity with no other site of airway obstruction (2016-2018). All patients underwent CO2 -laser assisted endoscopic resection tracheoplasty. Eight patients (six male) were included with a median age of 15.4 (IQR 12.3-17.9) years. Patients had a median of two previous open airway surgeries (IQR 1-2.5) and all patients had a history of tracheostomy with successful decannulation. Tracheal A-frame deformity presented as dyspnea on exertion for all patients (n = 8, 100%). Obstructive sleep apnea was confirmed for all patients who underwent polysomnography (4/4, 100%). Median interval from decannulation to development of symptoms was 8.7 years (IQR 5.8-9.3). All patients sized with an age-appropriate endotracheal tube despite the deformity. Endoscopic A-frame tracheoplasty was successful for 7/8 (87.5%) patients and was performed with overnight observation for these patients (8/8, 100%). Unilateral A-frame tracheoplasty was performed successfully for five patients (62.5%), bilateral A-frame tracheoplasty was performed successfully for two patients (25.0%), and one patient (12.5%) did not have complete resolution of symptoms after bilateral A-frame tracheoplasty due to multi-level airway obstruction. CO2 laser-assisted tracheoplasty is an innovative endoscopic technique to relieve symptoms of airway obstruction for selected patients with tracheal A-frame deformity although avoiding the morbidity and hospital stay duration associated with tracheal resection. 4 Laryngoscope, 131:E719-E723, 2021.

Sleep-disordered breathing in children with pycnodysostosis.

Upper airway obstruction is a common feature in pycnodysostosis and may cause obstructive sleep apnea (OSA). The aim of our study was to analyze sleep-disordered breathing and respiratory management in children with pycnodysostosis. A retrospective review of the clinical charts and sleep studies of 10 consecutive children (three girls and seven boys) with pycnodysostosis seen over a time period of 10 years was performed. Six patients had severe OSA and/or nocturnal hypoventilation and were started on continuous positive airway pressure (CPAP) as a first treatment at a median age of 3.4 ± 2.6 years, because of the lack of indication of any surgical treatment. Three patients could be weaned after several years from CPAP after spontaneous improvement (two patients) or multiple upper airway surgeries (one patient). Three patients had upper airway surgery prior to their first sleep study with two patients still needing CPAP during their follow-up. Only one patient never developed OSA. Patients with pycnodysostosis are at a high risk of severe OSA, underlying the importance of a systematic screening for sleep-disordered breathing. Multidisciplinary care is mandatory because of the multilevel airway obstruction. CPAP is very effective and well accepted for treating OSA.

Transnasal adenoidectomy in mucopolysaccharidosis

BackgroundMucopolysaccharide (MPS) diseases are a heterogeneous group of inherited, metabolic disorders characterized by accumulation of partially degraded glycosaminoglycans (GAG) in multiple organ systems. Due to accumulation in the airway, patients often present with multilevel airway obstruction and obstructive sleep apnoea (OSA). Adenotonsillar surgery leads to a significant improvement in the severity of OSA in MPS patients. However, access to secure the airway and for conventional surgery can be challenging, due to limited neck extension, macroglossia and reduced mouth opening. This study was undertaken to evaluate the role of transnasal microdebridement and radiofrequent plasma ablation (Coblation) in adenoidectomy to treat OSA in patients with MPS and restricted airway access. MethodsA retrospective case review was performed including patients with MPS undergoing adenoidectomy for OSA in the period between June 2015 and March 2017. In all cases, either a microdebrider (Gyrus Diablo) or a Coblation wand (EVAC70, Smith&Nephew) was used via a transnasal approach guided by nasendoscopy. The primary outcome was effect upon OSA, measured by sleep oximetry and parental report of benefit. The secondary outcomes were surgical complications and risk factors for persistent OSA after surgery. ResultsA total of nine patients were identified with a mean age of 9 years (range 3–14 years) at surgery. Post-operative sleep study data was available for eight patients (8/9). Six patients (6/8) had improvement in 4% oxygen desaturation index (ODI-4) with a mean of 8.11 pre-operatively (range 2.69–14.0) and 4.99 postoperatively (range 0.68–8.48). ODI-4 did not improve in two (2/8) patients. Irrespective of sleep oximetry results, improvement in OSA-related symptoms was noted by all parents postoperatively. No risk factors for persistent OSA were identified. Furthermore, no complications were noted in this cohort. ConclusionTransnasal Coblation and Microdebrider adenoidectomy is a safe and effective surgical treatment for OSA in patients with Mucopolysaccharidosis and adenoidal hypertrophy. As lifespan increases for patients with the Mucopolysaccharidoses, greater emphasis is being given to optimising airway management over the longer-term. This technical note describes the novel application of endoscopic techniques for the management of primary adenoidal hypertrophy when transoral access is restricted, or to debulk recurrent disease that would be challenging to remove via the standard transoral route.

Anesthesia Risk and the Mucopolysaccharidoses: A Challenging and Changing Landscape

The mucopolysaccharidoses (MPS) are well known to present a high risk of anesthesia. Predominantly, this risk is not only related to upper airway issues but also to multilevel airway obstruction, respiratory morbidity, and cardiac issues. Before disease-specific treatment became available, the management of the untreated patients with MPS was regarded as the worst airway problem in pediatric anesthetic practice. The last 20 to 30 years have seen great advances in the treatment of the different types of MPS with hematopoietic stem cell transplantation (HSCT) being offered to children with MPS I Hurler sundrome since the 1980s, and more recently enzyme replacement therapy (ERT) becoming available for many MPS subtypes.These treatments have undoubtedly affected the relative risk involved with an anesthetic. HSCT improves markedly the upper airway and cardiac myocardial function and there is evidence that airway management is much more straightforward. ERT, on the other hand, improves obstructive sleep apnea and exercise tolerance, but the overall effect on the risk of anesthesia is less obvious and airway complications during anesthesia remain at a high level. The impact of improved anesthetic management in the same time frame is borne out by a reduced failed intubation rate in the literature.The management of the MPS patients necessitates a multidisciplinary approach presently widely practiced in pediatric centers, but successful modes of therapy increasing their life span have pushed many of the extreme anesthetic challenges into adult practice where most anesthetists will be unfamiliar with the problem. This is a problem that needs to be addressed, and solutions to this issue could be collaborative working between pediatric and adult practitioners or an international network of experts available for advice.

Computed Tomography Characterization and Comparison With Polysomnography for Obstructive Sleep Apnea Evaluation

We hypothesized that computed tomography (CT) combined with portable polysomnography (PSG) might better visualize anatomic data related to obstructive sleep apnea (OSA). The present study evaluated the CT findings during OSA and assessed their associations with the PSG data and patient characteristics. We designed a prospective cross-sectional study of patients with OSA. The patients underwent scanning during the awake state and apneic episodes. Associations of the predictor variables (ie, PSG data, respiratory disturbance index [RDI]), patient characteristics (body mass index [BMI], neck circumference [NC], and waist circumference [WC]), and outcome variables (ie, CT findings during apneic episodes) were assessed using logistic regression analysis. The CT findings during apneic episodes were categorized regarding the level of obstruction, single level (retropalatal [RP] or retroglossal [RG]) or multilevel (mixed RP and RG), degree of obstruction (partial or complete), and pattern of collapse (complete concentric collapse [CCC] or other patterns). A total of 58 adult patients with OSA were scanned. The mean±standard deviation for the RDI, BMI, NC, and WC were 41.6±28.55, 27.80±5.43kg/m2, 38.3±4.3cm, and 93.8±13.6cm, respectively. No variables distinguished between the presence of single- and multilevel airway obstruction in the present study. A high RDI (≥30) was associated with the presence of complete obstruction and CCC (odds ratio 6.33, 95% confidence interval 1.55 to 25.90; and odds ratio 3.77, 95% confidence interval 1.02 to 13.91, respectively) compared with those with a lesser RDI. An increased RDI appears to be an important variable for predicting the presence of complete obstruction and CCC during OSA. Scanning during apneic episodes, using low-dose volumetric CT combined with portable PSG provided better anatomic and pathologic findings of OSA than did scans performed during the awake state.

Acoustic-integrated dynamic MR imaging for a patient with obstructive sleep apnea

Obstructive sleep apnea syndrome (OSAS) is caused by multi-level upper airway obstruction. Anatomic changes at the sites of obstruction may modify the physical or acoustic properties of snores. The surgical success of OSA depends upon precise localization of obstructed levels. We present a case of OSAS who received simultaneous dynamic MRI and snore acoustic recordings. The synchronized image and acoustic information successfully characterize the sites of temporal obstruction during sleep-disordered breathing events.

Obstructive Sleep Apnea in MPS

The mucopolysaccharidoses (MPSs) are a group of inherited, metabolic disorders characterized by progressive multisystem accumulation of partially degraded glycosaminoglycans. This manifests with multilevel airway obstruction, presenting with obstructive sleep apnea (OSA). We systematically reviewed the literature to determine the severity and prevalence of OSA in MPS based on polysomnography analysis. Fifteen studies with 294 participants met the inclusion criteria for review. The pretreatment prevalence of OSA in MPS was 81% with a mean apnea–hypopnea index (AHI) of 10.4. Patients with MPS I are most significantly affected, with 75% suffering with moderate to severe OSA (mean AHI, 16.6). Enzyme replacement therapy (ERT) results in an almost significant reduction in OSA in MPS I (P = .06), while adenotonsillar surgery significantly improves AHI (P = .002). Obstructive sleep apnea least affects MPS III. There is a lack of long-term post-ERT and hematopoietic stem cell transplant data relating to OSA outcom...

Role of polysomnography in the development of an algorithm for planning tracheostomy decannulation.

To examine the role of polysomnography (PSG) in helping determine readiness of tracheostomized patients for decannulation. Case series with chart review of pediatric patients who underwent PSG with tracheostomy tube in place with the goal of decannulation. Tertiary care pediatric center. Twenty-eight tracheostomized patients who underwent PSG from January 2006 to March 2012 were included. Outcome measures were successful decannulation, PSG results, surgical procedures, and medical comorbidities. Of the 28 patients, 20 (71.4%) were decannulated and 8 (28.6%) were not. One (3.6%) patient failed long-term decannulation. The average apnea-hypopnea index (AHI) with a capped tracheostomy for those successfully decannulated was 2.75 (range, 0.6-7.6), while the AHI for those not decannulated was 15.99 (range, 3.2-62). Factors associated with success or failure to decannulate were assessed, and an algorithm was developed to plan for successful decannulation. Laryngotracheal reconstruction was a significant factor in those successfully decannulated. Those who were not decannulated had multiple medical comorbidities, multilevel airway obstruction, need for additional surgery, or chronic need for pulmonary toilet. Polysomnography may be a useful adjunctive study in the process of determining a patient's readiness for decannulation. Our current algorithm for decannulation includes upper airway endoscopy with identification of levels of obstruction, followed by surgical correction of those obstructions; capped PSG to determine patency of the airway and help assess lung function; and overnight intensive care unit admission for capping trial, with decannulation the following day if well tolerated.

Outcomes Analysis of Neonates Treated for Pierre Robin Sequence

BACKGROUND: Pierre Robin Sequence (PRS) is caused by micrognathia, which leads to glossoptosis and airway obstruction. Multiple treatment modalities are described including conservative treatment, bypass of the obstruction, airway repositioning and correction of the anatomic deficiency. This study presents an outcomes analysis of the largest series of neonates with PRS. METHODS: An IRB approved, 18-year retrospective review of all neonates treated with PRS was performed. Examined variables included patient demographics, syndromic and neurologic status, feeding outcomes, and polysomnography and microlaryngoscopy data. RESULTS: 140 neonates were identified and divided into three cohorts based on initial treatment: conservative management (n=22), external mandibular distraction (MD, n=68) and tracheostomy (n=50). Neonates initially receiving tracheostomy were more likely to be syndromic (OR=2.62, p=0.01), have neurologic impairment (OR=2.85, p=0.02), GE reflux (OR=2.1, p=0.05) and require intervention within 5 days of birth (OR=60.65, p=0.005) compared with those receiving MD. Polysomnograms obtained pre- and post-intervention had similar significantly improved profiles for patients receiving both MD (obstructive index (OI) decrease 43.8 (p=0.03)) and tracheostomy (OI decrease 44.7 (p=0.008)). However those receiving MD had significantly greater avoidance of gastrostomy (19.1 vs 83.7%, p<0.0001) and higher success in exclusive oral diet (85.7% vs 38.9%, p<0.0001) compared to the tracheostomy patients. As shown in Table 1, four factors were associated with failure of MD: low birth weight, syndromic status, neurologic impairment and poor post-intervention polysomnogram. We demonstrate that combined these factors may be used to predict those at risk for failure of MD with high sensitivity and specificity (Table 2). Lastly, the presence of multilevel obstruction identified on microlaryngoscopy was not associated with need for tracheostomy (38.6% vs 26.9% for MD group, OR 1.77, p=0.22); and when present in patients receiving MD multilevel obstruction was not associated with higher failure rates (p=0.71).Table 1: Factors predicting failure of mandibular distraction for treatment of micrognathia/tongue-based airway obstruction. (Failure defined by need for subsequent tracheostomy.)Table 2: Mandibular distraction failure prediction testing, based upon the four criteria in Table 1.CONCLUSIONS: Mandibular distraction is an efficacious treatment modality for neonates with PRS, and should be considered the first line intervention to avoid tracheostomy. Patient variables including birth weight, syndromic status, neurologic impairment and multi-level airway obstruction do not preclude the utilization of MD; however, predicting the future necessity of tracheostomy and the ability to decannulate following MD of these patients is less reliable.

Successful Neonatal Mandibular Distraction Osteogenesis in Patients with Concomitant Laryngomalacia

8 CONCLUSIONS: Mandibular distraction is an effi cacious treatment modality for neonates with PRS, and should be considered the fi rst line intervention to avoid tracheostomy. Patient variables including birth weight, syndromic status, neurologic impairment and multi-level airway obstruction do not preclude the utilization of MD; however, predicting the future necessity of tracheostomy and the ability to decannulate following MD of these patients is less reliable.