Tongue-based Airway Obstruction Research Articles

Syndromic Piere Robbin Sequence- A Rare Presentation in Association with Multiple Heart Defects and Type III Stickler Syndrome.

Pierre Robin Sequence (PRS), a rare congenital disorder, is a triad of micrognathia, glossoptosis, and tongue based airway obstruction (TBSO). It may occur as isolated anomaly (iPRS) or as a part of a syndrome (sPRS), like that seen in association with Stickler Syndrome. Approximately 20% of children with PRS have congenital heart diseases. To the best of our knowledge this case of a one-day old infant is the first one to be reported as having two heart defects; patent ductus arteriosus and patent foramen ovale in Pierre Robbin Sequence child.

MicroNAPS: A Novel Classification for Infants with Micrognathia, Robin Sequence, and Tongue-based Airway Obstruction.

Robin sequence (RS) describes a heterogeneous population with micrognathia, glossoptosis, and upper airway obstruction (UAO). Workup, treatment, outcomes assessment, and research inclusion are widely variable. Despite several classifications and algorithms, none is broadly endorsed. The objective of this investigation was to develop and trial a novel classification system designed to enhance clinical communication, treatment planning, prognostication, and research. This is a retrospective cross-sectional study. A classification system was developed with five elements: micrognathia, nutrition, airway, palate, syndrome/comorbidities (MicroNAPS). Definitions and a framework for "stage" assignment (R0-R4) were constructed. Stage "tongue-based airway obstruction" (TBAO) was defined for infants with glossoptosis and UAO without micrognathia. MicroNAPS was applied to 100 infants with at least 1-year follow-up. Clinical course, treatment, airway, and feeding characteristics were assessed. Descriptive and analytic statistics were calculated and a P value less than 0.05 was considered significant. Of the 100 infants, 53 were male. Mean follow-up was 5.0 ± 3.6 years. R1 demonstrated feeding-predominant mild RS for which UAO was managed nonoperatively but gastrostomy tubes were prevalent. R2 was characterized by airway-predominant moderate RS, typically managed with mandibular distraction or tongue-lip adhesion, with few gastrostomy tubes and short lengths-of-stay. R3 denoted severe RS, with similar UAO treatment to R2, but with more surgical feeding tubes and longer admissions. R4 represented a complex phenotype with 33% tracheostomies, protracted hospitalizations, and delayed palatoplasty. R0 ("at risk") and TBAO groups displayed the most variability. MicroNAPS is easy to use and associated with relevant disease characteristics. We propose its adoption in clinical and research settings.

PLAST Classification of Mandibular Duplication and Associated Anomalies

Mandibular duplication is a rare subtype of craniofacial duplication. It is often associated with other anomalies including cleft palate, lingual duplication, and macrostomia. We present an 8-year-old female who had mandibular duplication presenting soon after birth with tongue-based airway obstruction. She was found to have cardiac abnormalities, split sternum, accessory tongue, cleft palate, and accessory submandibular gland. She underwent surgical correction including resection of the duplicated mandible, tongue, and submandibular gland as well as tongue lip adhesion at 12 days old. Post operatively, we were able to avoid tracheostomy and she was able to undergo successful correction of her cardiac anomaly. Literature review found 29 patients with mandibular duplication. Patients with w-shaped (type I) mandibular duplication are more likely to have airway compromise requiring intervention. Several associated anomalies were seen in patients with mandibular duplication. After analysis, we propose the PLAST classification to describe common findings: cleft Palate, Lip duplication, Airway obstruction, Salivary gland abnormalities, and Tongue duplication. Extra craniofacial anomalies such as cardiac and spinal anomalies were also noted in several patients.

Multimodal Treatment of Robin Sequence Utilizing Mandibular Distraction Osteogenesis and Continuous Positive Airway Pressure.

Mandibular distraction osteogenesis (MDO) and continuous positive airway pressure (CPAP) may each have a role in effectively treating tongue-based airway obstruction (TBAO) in Robin sequence (RS). This study describes longitudinal outcomes after treatment of TBAO with CPAP and/or MDO. Retrospective cohort study. Tertiary Pediatric Hospital. A total of 129 patients with RS treated with CPAP and/or MDO from 2009 to 2019 were reviewed. Subjects receiving baseline and at least one follow-up polysomnogram were included. 55 who underwent MDO ± CPAP and 9 who received CPAP-only treatment were included. Patient characteristics, feeding, and polysomnographic data were compared and generalized linear mixed modeling performed. Baseline obstructive apnea–hypopnea index (OAHI) was greater in the MDO-treated group (median x˜ = 33.7 [interquartile range: 26.5-54.5] than the CPAP-treated group (x˜ = 20.3[13.3-36.7], P ≤ .033). There was significant reduction in OAHI following treatment with CPAP and MDO modalities, P ≤ .001. SpO2 nadir after MDO was lower in syndromic (x˜ = 85.0[81.0-87.9] compared to nonsyndromic patients (x˜ = 88.4[86.8-90.5], P ≤ .005.) CPAP was utilized following MDO in 2/24 (8.3%) of nonsyndromic and 16/31 (51.6%) of syndromic subjects ( P ≤ .001,) for a median duration of 414 days. Three patients (5%) underwent tracheostomy, all had MDO. Nasogastric tube feeding at hospital discharge was more common following MDO (44, 80%) than CPAP-only (4, 44.4%, P ≤ .036), but did not differ at 6-month follow-up ( P ≥ .376). CPAP appears to effectively reduce obstructive apnea in patients with RS and moderate TBAO and be a useful adjunct in syndromic patients following MDO with improved but persistent obstruction.

Safety of Mandibular Osteotomies in Infants with Pierre Robin Sequence: Computer-Aided Modeling to Characterize the Risks of Various Techniques.

Mandibular distraction osteogenesis is effective for the correction of severe tongue-based airway obstruction in infants with Pierre Robin sequence. Involved osteotomies may damage developing tooth buds and/or the inferior alveolar nerve. The authors evaluated the theoretical safety of various osteotomy techniques to better define infantile mandibular anatomy using computer-aided modeling. Seven mandibular osteotomy techniques (oblique, inverted-L, multiangular, walking stick, high oblique, vertical/high inverted-L, and horizontal) were simulated using computed tomography studies from infants with Pierre Robin sequence and without other associated conditions. Software was used to manually segment the mandibular bone, inferior alveolar nerve, and tooth buds. Sixty-five computed tomography scans were included, yielding 130 hemimandibles. The horizontal osteotomy pattern had significantly lower theoretical risk of tooth bud (p < 0.001) and inferior alveolar nerve involvement (p < 0.001) than all other patterns. Osteotomies with high vertical components (i.e., vertical, walking stick, and multiangular) had lower theoretical tooth bud involvement than the more proximal oblique and inverted-L osteotomies (p < 0.001). Average lingula location was measured at a point 65 percent of the mandibular width from anterior mandibular border and 63 percent of the mandibular height from the inferior mandibular border. Surgical planning with computed tomography scans can help evaluate an infant's mandibular anatomy to select an osteotomy that reduces morbidity risks. Regardless of technique, tooth buds and the inferior alveolar nerve are often included in osteotomies. The lingula location in this study demonstrates a position more superior and posterior than that previously described. Therapeutic, V.

Mandibular distraction to correct severe non-isolated mandibular hypoplasia: The role of drug-induced sleep endoscopy (DISE) in decision making

ObjectivesIn patients with mandibular hypoplasia, mandibular distraction osteogenesis (MDO) aims to relieve tongue-based airway obstruction. Drug-induced sleep endoscopy (DISE) provides a dynamic assessment of the upper airway and visualizes anatomical site and cause of airway obstruction. The aim of this study was to evaluate the effect of MDO on tongue-based airway obstruction found by DISE within a non-isolated patient population with severe upper airway obstruction (UAO). Furthermore, we aimed to assess the additional value of DISE in clinical decision making by correlating DISE findings to functional airway outcomes after MDO. MethodsFindings on DISE in children who underwent MDO were retrospectively gathered and evaluated. According to DISE findings, severity of tongue-based obstruction was scored using a 4-step classification similar to the one that is used by Bravo et al.. Intubation conditions were scored according to the Cormack Lehane score (CLS). Pre-and postoperative DISE findings were compared and correlated with functional airway outcomes following MDO. ResultsIn 19 out of 28 MDO procedures, both a pre-and postoperative DISE was available. Tongue-based obstruction scores improved in 13 procedures, which correlated to a functional improvement in seven. Postoperative tongue-based obstruction differed significantly between patients with successful MDO and patients treated unsuccessfully (2.00 ((Interquartile range (IQR) 1.00–2.00) vs. 3.00 (IQR 2.00–4.00), p = 0.028), whereas this difference was not significant for the CLS (1.00 (IQR 1.00–1.50) vs. 2.00 (IQR 1.00–4.00), p = 0.066). If no improvement of tongue-based obstruction was seen, MDO is very unlikely to be successful on the functional airway. ConclusionsDISE provides information on the site and nature of airway obstruction and can visualize the effect of MDO on the severity of tongue-based airway obstruction. Therefore, it can be of additional value in understanding the differences in functional airway outcomes after MDO and aids in deciding appropriate and targeted treatment. Hence, standardized use of DISE, in addition to the clinical assessment of mandibular position and a polysomnography, during MDO management is highly recommended.

The Radiographic "Air Sign" in Neonatal Mandibular Distraction Osteogenesis.

Timing of extubation on post-mandibular distraction osteogenesis (MDO) surgery is critical, given that at baseline these infants have difficult airways and failed extubation requires either re-intubation of an already complex airway with a fragile, recently osteotomized mandible, or adjunctive airway measures such as CPAP that may apply unwanted pressure to the surgical site. Thus, the goal is to plan extubation when the risk of failure is minimal. Currently, there is a void in the literature addressing the timing of extubation post-MDO and no objective sign of extubation readiness has been elucidated. This study describes a simple clinical pearl to assist in the evaluation of extubation readiness in these patients. Postoperatively, we obtain weekly radiographs to assess distractor stability and advancement, and to assess for the "Air Sign". The Air Sign describes a radiolucent space (air) visualized in the oropharynx on lateral radiographs, likely indicating that the tongue based airway obstruction has been relieved by mandibular advancement.

Mandibular Distraction Osteogenesis for Tongue-Based Airway Obstruction Without Micrognathia.

Mandibular distraction osteogenesis (MDO) effectively treats tongue-based airway obstruction (TBAO) in micrognathic patients with Robin Sequence. Mandibular distraction osteogenesis may also address TBAO in certain nonmicrognathic patients who have severe obstructive apnea, although there is no current literature to guide MDO use in these atypical patients. This study describes outcomes of MDO in a series of patients with TBAO without micrognathia. Patients who underwent MDO for TBAO from 2013-20 were reviewed, and patients with micrognathia were excluded. Study subjects received baseline/follow up polysomnography. Polysomnography variables, including Obstructive Apnea Hypopnea Index, oxyhemoglobin saturation nadir (SpO2 nadir), percent sleep time end tidal CO2 greater than 50 mm Hg (%ETCO2 > 50), and respiratory-related arousals were compared before and after MDO. Demographics, syndromic/cleft palate status, airway anomalies, respiratory support, and feeding outcomes were collected. One hundred and twenty-four patients underwent MDO during this study period; 5 were nonmicrognathic and included in analysis. Sixty percent (n = 3) of the cohort was syndromic: 1 patient each had Trisomy 9, Beckwith Wiedemann syndrome, and duplicated pituitary gland plus syndrome. Forty percent (n = 2) of patients had a cleft palate, 60% (n = 3) had laryngomalacia, and 40% had tracheomalacia. Median (range) age at MDO was 53 days (47-167 days), and median length of distraction was 16 mm (14-20 mm). After MDO, median Obstructive Apnea Hypopnea Index decreased from x̃ = 60.7/h (11.6-109.4) to x̃ = 5.3/h (3.5-19.3) (P = 0.034). SpO2 nadir increased (69% [58-74] to 85% [80-88], P = 0.011), and median %ETCO2 > 50 mm Hg decreased (5.8% [5.2-30.1] to 0.0% [0.0-1.3], P ≤ 0.043). Continuous positive airway pressure was used by all patients immediately after MDO, and at 6 months postoperatively, 1 patient remained on continuous positive airway pressure and 1 patient required supplemental oxygen. At last follow up, no patients had significant residual airway obstruction or required a tracheostomy. Mandibular distraction osteogenesis can effectively treat severe TBAO in some patients without micrognathia that would otherwise be candidates for tracheostomy. When used in select patients, MDO significantly improves obstructive sleep apnea and reduces need for ventilatory support, although feeding support is still needed in most patients at 6 months. Further study in a larger cohort will help identify appropriate candidates for MDO and characterize outcomes of unique patient populations.

Incidence of mandibular distraction osteogenesis in Stickler Syndrome: Variation due to COL2A1 and COL11A1

ObjectiveTo determine whether the two most common genetic mutations seen in Stickler Syndrome (SS) (COL2A1 and COL11A1) affect the incidence of mandibular distraction osteogenesis (MDO) and what impact Robin sequence (RS) has on diagnosis. SS is an autosomal dominant connective tissue disorder characterized by almost complete penetrance. COL2A1 and COL11A1 are the two most common mutations seen in SS patients. SS often presents at birth with RS, which is characterized by the triad of micrognathia, glossoptosis, and tongue-based airway obstruction. MDO is one surgical intervention that has been shown to be successful in relieving tongue base obstruction and is the surgical intervention of choice for this condition. MethodsA retrospective chart review was performed on all patients with a diagnosis of SS at a tertiary pediatric hospital between January 1, 2003 and December 31, 2018. The included patient charts were reviewed for demographic information, SS mutation, and history of MDO. Forty-six patients had a clinical diagnosis of SS. Of those, 31 met inclusion criteria which involved having a molecular diagnosis of SS and sufficient follow up information to determine if MDO was indicated or performed. Twenty-two of the 31 included patients had a diagnosis of RS (70.96%). Thirteen of the 31 patients (41.94%) included in this study required MDO as a neonate. ResultsFifty-percent of patients with type I (COL2A1) required MDO as a neonate compared to only 31% of patients with type II (COL11A1), though the difference between the two groups was not statistically significant. ConclusionThe findings of this study suggest that patients with type I mutation may have a higher incidence of MDO than patients with a type II mutation, though further research with larger sample sizes is needed. This information is helpful in counseling those with SS or family history of SS about what they can expect related to RS and need for MDO based on genetic findings. Level of Evidence3

Polysomnography in the Early Consolidation Phase of Mandibular Distraction as a Tool to Identify Residual Tongue-Based Airway Obstruction and Need for Further Distraction: A Follow-Up Study

Background: The objective of this study was to analyze and report our experience with using polysomnography (PSG) during the early consolidation phase of mandibular distraction osteogenesis (MDO) in patients with Pierre Robin Sequence and associated tongue-based airway obstruction with management decisions informed by the results. The secondary objective was to determine whether pre-operative PSG can predict which patients will have persistent obstructive sleep apnea (OSA) and require additional distraction. Methods: A single-surgeon, retrospective review was performed of all patients who underwent MDO for treatment of tongue-based airway obstruction over an 11-year period. Pre-operative and post-distraction 16-channel overnight bedside PSG was performed on all patients and scored according to the AASM Scoring Manual. Patients who demonstrated persistent OSA underwent further distraction and were reevaluated by PSG after final distraction length was achieved. Results: Pre-operative and post-distraction PSG was obtained and available for analysis in 37 patients. Apnea-Hypopnea Index (AHI) improved from 45.85 ± 34.41 to 9.73 ± 10.85 ( P &lt; .001). There was evidence for persistent, severe OSA in 14 patients (38%) and persistent, moderate OSA in 6 patients (16%) in the early consolidation phase of MDO. Nine of these patients underwent additional distraction and demonstrated symptomatic resolution and quantitative improvement in all PSG measures. The group of patients that underwent additional distraction had significantly more severe baseline OSA as defined by preoperative AHI, oxygen desaturation index (ODI), and percentage of time spent below 90% SpO2. There was no significant difference in patient characteristics or pre-operative symptoms between the 2 groups. Conclusions: PSG can be performed at the bedside at the end of the distraction phase of MDO to assess for residual airway obstruction and inform the need for further distraction. Pre-operative PSG measures can help predict the need for further distraction and aid in operative planning and distractor placement.

Laryngoscopy Grade Improvement and Difficult Airway Resolution in Infants With Robin Sequence Undergoing Mandibular Distraction Osteogenesis: A Multi-Institutional Study.

Mandibular distraction osteogenesis (MDO) aims to relieve tongue-based airway obstruction in Robin Sequence (RS). We investigated direct laryngoscopy grade (DLG) improvement and difficult airway (DA) resolution following MDO. Retrospective cohort analysis. Three tertiary care institutions. Sixty-four infants with RS who underwent a single MDO procedure in their first year of life were identified from January 2010 to January 2019. The primary outcome was DLG pre- and post-MDO. Secondary outcomes included DA designation, pre- and post-MDO polysomnographic assessment for obstructive sleep apnea (OSA), length of stay, need for gastrostomy, and major/minor adverse events. Median DLG improved from II pre-MDO to I at the time of distractor removal (n = 43, P < .001). No significant change was seen in patients with a third recorded time point (eg, palatoplasty; n = 78, P = .52). Twenty-six (47%) of 55 patients were designated as DA pre-MDO, and 10 (18%) of 55 patients retained the label post-MDO (P < .01). Five (50%) of these 10 patients appeared to be inappropriately retained. Median obstructive apnea-hypopnea index improved from 38.6 (range 31.2-62.8) pre-MDO to 2.9 (range 1-3.9) post-MDO (n = 12; P = .002). Mandibular distraction osteogenesis allowed for DLG improvement that was stably maintained as well as functional improvement in OSA, with minimal morbidity. Difficult airway designation persisted in the electronic record of some infants despite clinical resolution.

Novel Approach of Mandibular Distraction to Avoid Tracheostomy in KAT6B-related Gene Disorders

Previously, severe upper airway obstruction in patients with retrognathia and glossoptosis has been managed with tracheostomy. However, tracheostomy is associated with significant morbidity. In recent years, mandibular distraction has become an alternative management strategy in infants, but these applications have been limited to patient populations with retrognathia and glossoptosis. The authors present 2 unique cases of patients with KAT6B-related gene disorders, who present with a paradox of tongue-based airway obstruction in the absence of retrognathia. In both cases mandibular distraction osteogenesis with an obliquely oriented vector was successfully performed and both children avoided the need for tracheostomy.

Neonatal Mandibular Distraction Osteogenesis in Infants With Pierre Robin Sequence.

Pierre Robin sequence is the constellation of micrognathia, glossoptosis, and tongue-based airway obstruction. When airway obstruction is severe, feeding, growth, and respiratory demise are at risk. Neonatal mandibular distraction osteogenesis is a technique which improves tongue-based airway obstruction and avoids tracheostomy in patients with severe expressions of Pierre Robin sequence. Its efficacy in relieving airway obstruction is well documented, and it has become the surgical intervention of choice at many craniofacial centers. However, this is an uncommon procedure which can be performed within the first weeks of life, offering little space for a learning curve. The success of neonatal distraction and avoidance of complications is highly dependent on proper surgical technique. This report provides a brief overview of the disease, details the technique of the senior surgeon with captioned videos, describes the protocol used at our institution and reports long-term outcomes with a case description.

Optimal duration of mechanical ventilation and influencing factors following mandibular distraction osteogenesis in infants with Pierre Robin sequence.

Mandibular distraction osteogenesis (MDO) is an effective treatment for tongue-based airway obstruction in infants with severe Pierre Robin sequence (PRS). Most infants receiving MDO require postoperative mechanical ventilation (MV) to assist breathing. Optimal MV time for each individual patient and factors influencing the time must be identified to guide clinical decision-making.A retrospective analysis was performed on 75 infants with PRS receiving MDO from November 2016 to August 2018. Twenty-six were females and 47 were males. Data extracted from the hospital information system included sex, age, weight, history of preterm labor, preoperative pulmonary infection, laryngomalacia/tracheomalacia, laryngoscope exposure classification, anesthesia duration, operation duration, postoperative treatment site, situation of distraction, postoperative complications and MV duration. Statistical analyses were conducted to investigate the potential associations of these factors with MV time.Seventy-three PRS syndrome patients received anesthesia for MDO device procedures were considered eligible for study. Patient sex, history of preterm labor, preoperative pulmonary infection, laryngomalacia/tracheomalacia, laryngoscopy exposure difficulty, postoperative treatment site (neonatal or pediatric intensive care unit), ventilator-associated pneumonia, age, weight, anesthesia duration, and operation duration had no significant influence on postsurgical MV time (P > .05). Amount of distraction at the time of extubation had statistically significant influence on postoperative MV time (P < .05). In addition, scatter plots revealed linear relationships between postoperative MV time and amount of distraction at extubation.According to this analysis, amount of distraction was associated with MV time following MDO for severe PRS and roughly 6 days post-surgery is a generally safe extubation time.

S7B-04 SESSION 7B: HFM ROBIN - PART II RATE AND RISK FACTORS FOR REPEATED MANDIBULAR DISTRACTION OSTEOGENESIS IN INFANT PATIENTS WITH TONGUE BASED AIRWAY OBSTRUCTION

Introduction: Mandibular distraction osteogenesis (MDO) has demonstrated efficacy for tongue-based airway obstruction in infant Pierre Robin Sequence (PRS). The purpose of this study is to identify risk factors for patients who require repeat distraction after MDO in early infancy for PRS. Methods: Consecutive patients who underwent MDO from 2004 to 2018 at a single institution were retrospectively reviewed. Patient characteristics including age at initial MDO, comorbidities, polysomnographic parameters, and distraction data were collected. Patients whose initial surgery was before 2 months of age were identified and divided into cohorts: single versus repeat distraction. Results: 103 patients were identified during the study period - 95 had a single distraction procedure and 8 required repeat distraction. Indications for repeat distraction were recurrent airway obstruction (6), failure to extubate (1), and persistent micrognathia (1). There was no difference in age or weight at initial distraction, duration of distraction, or distraction length between single and repeat distraction groups. Overall, average AHI was 39.9 pre-operatively and 10.1 post-operatively; neither varied between groups. Genetic anomalies and syndromic diagnoses were more common in the repeat distraction group (75.0% vs. 40.0% single distraction, p=0.06). More patients had laryngomalacia in the repeat distraction group (75.0% vs. 33.7% single distraction, p=0.02). Conclusion: MDO has been accepted as efficacious in PRS but the rate of repeat distraction has not been described. In this study 7.8% of infants required redistraction and the identified risk factors were genetic anomaly or syndromic diagnosis, pre-operative laryngomalacia, or recurrent airway obstruction.

Risk Factors for Perioperative Respiratory Failure following Mandibular Distraction Osteogenesis for Micrognathia: A Retrospective Cohort Study.

The frequency of respiratory events in the perioperative period, and optimal duration of intubation during early mandibular distraction osteogenesis activation, are poorly understood. This study assesses potential risk factors associated with perioperative respiratory events, particularly the need for reintubation, following mandibular distraction osteogenesis surgery. A retrospective review was conducted for infants (younger than 1 year) undergoing mandibular distraction osteogenesis for tongue-based airway obstruction between November of 2010 and December of 2017. Univariate and multivariate analyses of sentinel events and outcomes were performed. Ninety infants (median age, 35 days) were included (50 percent were syndromic). Twenty-seven subjects (30 percent) experienced a respiratory event requiring intervention before discharge, including 14 subjects who failed initial extubation. Subjects extubated earlier than postoperative day 5 failed extubation more frequently (33%) compared to those extubated later (9%; p = 0.005). Respiratory events occurred more frequently when extubation was attempted at distraction lengths of 5 mm or less (42 percent) compared to greater than 5 mm (21 percent; p = 0.032). Logistic regression modeling showed that syndromic status (OR, 14.8) and secondary airway anomaly (OR, 6.1) were significant predictors for respiratory events, whereas greater length of distraction at the time of extubation was protective (OR, 0.8; p < 0.05). Postoperative intubation of at least 5 days with associated mean distraction of 5 mm appears to be associated with successful extubation trial following mandibular distraction osteogenesis surgery. Patients with congenital syndromes and secondary airway anomalies are more likely to experience perioperative respiratory events. Risk, III.

Pierre Robin Sequence: Two Case Reports

Pierre Robin sequence was first described as a syndrome; however, it is currently recognized as a sequence that includes micrognathia, glossoptosis, and paroxysmal respiratory obstructions. Airway long-term treatment is necessary to avoid serious complications of this condition. Mandibular distraction osteogenesis (MDO) is an effective treatment for tongue-based airway obstruction in children with severe Pierre Robin sequence. The authors described 2 cases of new-born children diagnosed with severe Pierre Robin sequence, treated according to a fixed protocol, that is, following diagnosis, the children were taken to the surgical center for tracheofibroscopy and tracheostomy in cases of tongue impingement. Following general anesthesia, the patients were submitted to MDO using a 20 mm pediatric distractor, bilaterally. After 1 mm of distraction daily and adequate bone healing, distractors were removed and the airway evaluated. The patients showed an improvement of the airway, allowing tracheostomy removal. In conclusion, mandibular distraction is an effective procedure for Pierre Robin sequence.

Management of Airway Obstruction in Infants With Pierre Robin Sequence.

Pierre Robin sequence (PRS)-related airway obstruction is often treated surgically; however, objective measures predicting the need for surgery are poorly defined. A retrospective chart review was performed on 171 neonates with PRS. Infants were grouped based upon intervention modality: nonsurgical (conservative) or surgical [mandibular distraction osteogenesis (MDO) or tracheostomy]. Demographic data, physical examination findings, and study results were compared between groups to determine risk factors for surgical intervention, and to predict long-term success or failure of those interventions. The most significant, objective risk factor among those receiving surgery was a poor preintervention sleep study [obstructive index (OI): 42.4 versus 12.9 for the conservative treatment group; P < 0.001]. Only 11% of those treated conservatively had an OI >20, whereas 67.5% of those treated surgically met this severity measure. Of those receiving surgery, tracheostomy was associated with neurologic impairment (P = 0.030) and low birth weight (P = 0.046) compared with the MDO group. Together with syndromic status, these risk factors were useful for predicting failure of MDO to avoid subsequent tracheostomy (test sensitivity and specificity were 64.2% and 100.0%, respectively). No long-term differences in speech or micrognathia were detected between the 3 groups; however, those treated conservatively or with MDO had improved long-term feeding and airway obstruction outcomes compared with the tracheostomy group. Surgical intervention for PRS-related tongue-based airway obstruction should be strongly considered with an OI >20. Tracheostomy should be reserved for complex patients with concomitant syndromic diagnosis, neurologic impairment, and low birth weight.

Airway Volume Simulation in Virtual Mandibular Distraction: A Cohort Study.

The authors investigated the accuracy of virtual surgical planning in predicting airway volume changes after mandibular distraction in patients with Pierre Robin sequence and associated tongue-based airway obstruction. The authors completed a single-institution retrospective review of patients for whom virtual surgical planning was used during mandibular distraction osteogenesis for treatment of tongue-based airway obstruction. Preoperative airway volume, virtual surgical planning-predicted airway volume, and postoperative airway volume were calculated from three-dimensional computed tomographic scans using industry software. A blinded institutional radiologist also calculated pre- and post-operative airway volumes. Pre- and post-operative polysomnography was used to titrate the endpoint of mandibular lengthening. Eleven patients were included in the study. Mean apnea-hypopnea index (5.42 ± 4.53 versus 44.96 ± 20.57; p < 0.001) and mean nadir oxygen saturation (70.3 ± 9.72 percent versus 82.9 ± 9.62 percent; p = 0.003) improved with mandibular distraction. There was moderate correlation between predicted and actual mandibular distraction lengths (R = 0.65; p = 0.003). There was a strong correlation between predicted and industry-calculated actual post-distraction airway volume (R = 0.99; p < 0.001). There was no significant correlation between actual mandibular distraction length and industry-calculated actual post-distraction airway volume for the entire cohort (R = 0.05; p = 0.49), but correlation approached significance by institutional calculations. No significant correlation existed between industry and institutional-calculated percentage change in post-distraction airway volume (R = 0.06; p = 0.57). Predictive airway volume calculation may be an effective adjunct to determine anatomic endpoint of mandibular distraction but small sample size, operator and software variability, and patient airway morphology may confound firm conclusions. Further studies are warranted.

Logistic regression analysis of Pierre Robin sequence patients requiring surgical intervention.

Determine predictive patient characteristics that guide the decision to proceed with surgical management of tongue-based airway obstruction (TBAO) in Pierre Robin sequence (PRS) patients. Retrospective review of PRS patients between 2005 and 2014 requiring observation in the neonatal intensive care unit (NICU). Patient charts were reviewed for prenatal diagnoses, clinical course, and need for surgical intervention (tracheotomy or mandibular distraction osteogenesis), and the nonsurgical and surgical group were compared with a logistic regression model. Thirty-eight PRS patients who were identified with TBAO and required NICU observation had an average follow-up of 5.4 years. Associated anomalies identified in the PRS patients included neurologic disease (n = 6), renal abnormalities (n = 5), limb abnormalities (n = 4), and cardiac abnormalities (n = 12). Nonsurgical management of PRS TBAO included side (n = 12)/stomach positioning (n = 15), oral airway (n = 6), nasopharyngeal airway (NPA) (n = 14), and intubation (n = 12). Surgical intervention occurred in 13 patients, with tracheotomy in eight due to unstable airway, and mandible distraction in five due to NPA dependence. Factors with significant uncontrolled correlations with the need for surgical airways included presence of cardiac disease (P = .03), cardiac disease severity (P = .03), neurologic disease (P = .01), and continuous positive airway pressure (CPAP)/bilevel positive airway pressure (BiPAP) use (P = .006). Further, stepwise regression showed strong predictive value for CPAP/BiPAP use (odds ratio [OR]: 10.43) and presence of neurological disease (OR: 9.16). PRS TBAO patients required multiple modalities of noninvasive surgical intervention to stabilize their airway. This study identified patient characteristics predictive of progression to a surgical airway to address TBAO, which may decrease healthcare utilization and improve quality of life for these families. 4 Laryngoscope, 127:945-949, 2017.