BACKGROUND:Robin sequence (RS) is a congenital condition and constitutes the triad of micrognathia, glossoptosis, and upper airway obstruction. While micrognathia is a cardinal feature of RS, its assessment is largely subjective. The aim of the present study is to describe 3D mandibular morphology in patients with RS and age-related mandibular shape variation compared with an age-matched control group. METHODS:3D reconstructions of the mandible were obtained from CT-imaging of children with isolated (iRS) and nonisolated RS (niRS). Principal Component Analysis was used to describe variation in mandibular morphology. Partial Least Squares and multivariate analysis of variance (MANOVA) were used to compare shape differences between patients with RS and 1:1 age-matched control groups. RESULTS:A total of 84 patients with iRS and 48 with niRS were included with a mean age of 5.4±8.4 months versus 11.0±13.9 months ( P -value<0.001). For the iRS and niRS groups, the first principal component primarily constituted allometric shape variation, as a high correlation was noted with age in both groups (Spearman R=0.79). Compared with the control group, both the iRS and niRS mandibles displayed shorter condylar necks, shorter mandibular bodies, and less pronounced, more rounded symphyseal projection (MANOVA, P -value<0.001). For both groups, a persistent difference in age-related shape changes along the first shape variable compared with the age-matched control group is observed. CONCLUSIONS:Variation in mandibular morphology in patients with RS for the included age range is primarily due to allometric shape changes. Patients with RS have distinct mandibular morphology relative to age-matched controls. The differences observed in the comparison of age-related shape changes are suggestive of a persistent dysmorphology for patients with iRS and niRS. Future studies will explore the association of mandibular morphology with clinical parameters.

BACKGROUND:Midface advancements in children with syndromic craniosynostosis present challenges for anesthesiologists and intensive care teams. AIMS:This study reviewed the perioperative airway management protocol for immediate tracheal extubation after midface surgery at our tertiary center over the past 10 years. METHODS:A retrospective cohort study was performed to obtain information on respiratory disorders, surgical and anesthetic management, airway support, and respiratory complications following le Fort III (LF3) and monobloc (MB) with distraction. Patients with a tracheostomy were excluded. RESULTS:Thirty-two patients (12 LF3, 20 MB) were included. All were immediately extubated with a median of 25 min after surgery. Immediate extubation was performed in young patients (n = 8/32, < 5 years old), in patients with severe OSA (n = 6/32, median oAHI 23/h), with difficult airways (n = 5/32, Cormack-Lehane airway grade ≥ 3), with significant intraoperative blood loss (n = 32, median 46 mL/kg), and with long operative times (n = 32, median 223 min). The majority of patients received no or only oxygen support in the first hours after extubation (n = 29/32) and could be discharged from the pediatric intensive care unit to the surgical ward after 1 day (n = 30/32). A 5-month-old patient with MB required intermittent oxygen and Guedel airway throughout his hospitalization due to airway obstruction at the tongue base combined with supine positioning to allow external traction. CONCLUSIONS:Despite the pre-existing airway disorder, the extent of the procedure and the effect of anesthesia on airway tone, all patients were extubated immediately after midface advancement, with only one young patient needing prolonged postoperative support. Immediate extubation is feasible following midface advancement in patients with syndromic craniosynostosis. Further prospective randomized trials are needed to demonstrate superiority to delayed extubation.

OBJECTIVE:To reflect upon our non-surgical respiratory management by evaluating clinical outcomes regarding airway, feeding, and growth during the first year of life in patients with Robin Sequence. DESIGN:Prospective study. SETTING:Sophia Children's Hospital, Rotterdam, the Netherlands. PATIENTS/ PARTICIPANTS:36 patients with Robin Sequence who were treated between 2011 and 2021. INTERVENTIONS:Positional therapy and respiratory support. MAIN OUTCOME MEASURE(S):Data on respiratory outcomes included polysomnography characteristics and capillary blood gas values. Feeding outcomes were based on the requirement of additional tube feeding. Outcomes on growth were expressed as standard-deviation-scores (SDS) for weight-for-age (WFA) and height-for-age (HFA). RESULTS:Twenty patients were treated with positional therapy (PT), whilst the other 16 patients required respiratory support. Twenty-two patients presented with non-isolated Robin Sequence (RS). During the first year of life, obstructive apnea hypopnea index decreased, oxygen levels enhanced, and capillary blood gas values improved. Eighty-six percent (31/36) experienced feeding difficulties, which completely resolved in 71% (22/31) during their first year of life. From start treatment, to stop treatment, to the age of 1 year, the SDS WFA worsened from -0.40 to -0.33 to -1.03, respectively. CONCLUSIONS:Non-surgical respiratory treatment resulted in an improvement of respiratory outcomes to near normal during the first year of life in patients with RS. These patients often experience feeding difficulties and endure impaired weight gain up to 1 year of age, despite near normalization of breathing. The high prevalence of feeding difficulties and impaired weight for age indicate the urgency for early recognition and adequate treatment to support optimal growth.

Background: Oesophageal atresia (OA) is often accompanied by tracheomalacia (TM). The aim of this study was to evaluate its presence in OA patients during routine rigid tracheobronchoscopy (TBS) before primary correction and compare this to postoperative TBS and clinical signs of TM. Methods: This retrospective cohort study included patients born with OA between June 2013 and December 2022 who had received a TBS before OA correction and had been followed for at least twelve months. Definite TM was postoperatively diagnosed through TBS, and probable TM was defined as having symptoms of TM. Results: We analysed data from 79 patients, of whom 87% with OA type C. Preoperatively, TM was observed in 33 patients (42% of all patients), seven of whom had severe TM. Definite TM was observed in 21 patients (27%), of whom 15 had severe TM. Forty-one patients (52% of all patients) had developed symptoms of TM within twelve months, including harsh barking cough (n = 15), stridor and/or wheezing (n = 20), recurrent respiratory insufficiency (n = 11), or needing airway surgery (n = 7). The sensitivity of preoperative TBS for the presence of postoperative (definite and probable combined) TM is 50.0%, 95% CI [35.2-64.8], and the specificity 67.6%, 95% CI [51.7-81.1]. Clinical characteristics did not differ between the patients with or without postoperative TM. Conclusions: More than half of the studied patients with OA experienced symptoms of TM. While preoperative TBS is routinely performed prior to surgical OA correction, its predictive value for the presence of postoperative TM remains limited. Level of Evidence: Level II. Type of Study: Study of Diagnostics Test. (c) 2024 The Author(s). Published by Elsevier Inc. This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).

IntroductionTracheomalacia (TM) often occurs in children with oesophageal atresia (OA), leading to recurrent respiratory symptoms and in severe cases to blue spells or ultimately respiratory arrest. In some patients, a secondary posterior tracheopexy may then be indicated. This secondary surgery, as well as respiratory morbidity, may be prevented by performing a primary posterior tracheopexy (PPT) concurrent with primary OA correction. The aim of this trial is to determine if a PPT can decrease—or prevent—tracheal collapse in newborns with OA and TM. Additionally, the trial aims to determine whether the potential observed effect of PPT on tracheal stability is sustained over time.Methods and analysisThis is an international multicentre double-blind randomised controlled trial. Seventy-eight children with OA type C will be randomised 1:1 into the no-PPT group or PPT group. Randomisation will be stratified by centre. The degree and location of TM are assessed during preoperative, intraoperative and two postoperative tracheobronchoscopies. The occurrence of TM will be evaluated during three routine follow-up consultations until the age of 6 months. The primary outcome is the degree of collapse of the tracheal wall during the intraoperative tracheobronchoscopy (after performing the PPT/no-PPT), measured in percentages. The difference in the mean degree of collapse will be compared between the no-PPT and the PPT groups using linear regression, adjusting for centre and the preoperative degree of tracheal collapse at baseline. The adjusted mean difference will be reported as effect size together with its 95% CI.Ethics and disseminationPatients will be included after written parental informed consent. The risks and burden associated with the trial are minimal. The institutional review board of the University Medical Center Utrecht has approved this protocol (METC-number 23-256/A). Results will be shared in a peer-reviewed scientific journal and presented at international conferences.Trial registration numberNCT06335862.

STUDY OBJECTIVE:During rigid bronchoscopies and microlaryngeal surgery (MLS) in children, there is currently no reliable method for managing ventilation strategies based on carbon dioxide (CO2) levels. This study aimed to investigate the effects of the clinical implementation of transcutaneous CO2 (tcPCO2) monitoring during rigid bronchoscopies or MLS. DESIGN:Prospective observational study. SETTING:Operating theatre of a tertiary pediatric hospital, from January 2019 to March 2021. PATIENTS:Children with an age < 18 years, undergoing rigid bronchoscopy or MLS, were eligible for inclusion. Children with tracheostomy and/or skin conditions limiting tcPCO2 monitoring were excluded. INTERVENTIONS:TcPCO2 monitoring was performed in two groups; blinded before clinical implementation (control group) and visible for ventilation management after clinical implementation (tcPCO2 group). MEASUREMENTS:The total tcPCO2 load outside of the normal range (35-48 mm Hg) was calculated as the area under the curve (AUC) and compared between the groups. Anesthesiologists in the tcPCO2 group received a questionnaire after each procedure. MAIN RESULTS:A total of 120 patients were included. No significant differences were found between the two groups in the AUC during the procedure (19,202 (7,863-44,944) vs 17,737 (9,800-47,566) mm Hg · s, P = 0.84) or between different ventilation strategies. The maximal tcPCO2 level was 69.2 (62.1-81.2) mm Hg in the control group and 71.1 (62.8-80.8) mm Hg, (P = 0.85) in the tcPCO2 group. Spontaneous breathing was associated with lower tcPCO2 levels. The general satisfaction score of tcPCO2 monitoring rated by the anesthesiologist was 8.19 (0.96). CONCLUSIONS:TcPCO2 levels reached approximately twice the upper limit of the normal range during rigid bronchoscopy and MLS. Availability of tcPCO2 monitoring did not affect these high levels, despite adjustments in strategy. However, tcPCO2 monitoring provides valuable insight in CO2 load and applied ventilation strategies.

Introduction: Robin Sequence (RS) is characterized by retrognathia, glossoptosis, and upper airway obstruction (UAO). Nonetheless, the exact role of retrognathia in RS remains unclear. The aim of this study was to evaluate if measuring retrognathia could be of use next to the polysomnography (PSG) in the diagnosis of RS by differentiating RS patients and controls. Furthermore, this study endeavored to evaluate mandibular growth over time. Methods: A retrospective study was performed on RS patients without life-threatening UAO and controls. Mandibular length was assessed by using the Nasion-Mandibula Ratio (NMRatio) at the following three time points: 0 to 3 months, 1 year, and 4 years of age. Results: A total of 107 patients were included of whom 38 were diagnosed with RS. Thirty-two patients presented with clinical retrognathia but without obstructive sleep apnea (OSA), whilst 37 patients had an isolated cleft palate (CP). At 0 to 3 months, 1 year, and 4 years of age, significant higher NMRatios were found in RS patients compared to isolated CP patients, whilst no significant differences were found compared to retrognathia-only patients. Within RS patients, no significant differences were found between isolated versus non-isolated and invasively versus non-invasively treated patients. In all three patient groups, a significant decrease of the NMRatio was seen at the age of 4 years compared to 0 to 3 months and 1 year of age. Conclusions: Although the NMRatio differs between RS and isolated CP patients, a seemingly small mandible (in a-p direction) does not reflect functional outcomes in RS patients and a PSG should always be performed to confirm the diagnosis. Despite that mandibular length significantly increased after the first year of life, mandibular size of RS patients does not seem to reach values of normal infants. Accordingly, mandibular catch up growth, that is suggested to be typically present in RS patients, was not found by our study.

OBJECTIVES:In 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. METHODS:Findings 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. RESULTS:In 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. CONCLUSIONS:DISE 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.

Objectives/HypothesisDysphonia is a common problem at long‐term follow‐up after airway surgery for laryngotracheal stenosis (LTS) with major impact on quality of life. Dysphonia after LTS can be caused by scar tissue from initial stenosis along with anatomical alterations after surgery. There is need for a modality to noninvasively image structure and function of the reconstructed upper airways including the vocal cords to assess voice outcome and possible treatment after LTS. Our objective was to correlate vocal cord structure and function of patients after airway reconstruction for LTS on static and dynamic magnetic resonance imaging (MRI) to voice outcome.Study DesignProspective cohort study.MethodsVoice outcome was assessed by voice questionnaires ((pediatric) Voice Handicap Index (p)VHI)) and the Dysphonia Severity Index (DSI). Postsurgical anatomy, airway lumen, and vocal cord thickness and movement on multiplanar static high‐resolution MRI and dynamic acquisitions during phonation was correlated to voice outcome.ResultsForty‐eight patients (age 14.4 (range 7.5–30.7) years) and 11 healthy volunteers (15.9 (8.2–28.8) years) were included. Static MRI demonstrated vocal cord thickening in 80.9% of patients, correlated to a decrease in DSI (expected odds 0.75 [C.I. 0.58–0.96] P = .02). Dynamic MRI showed impaired vocal cord adduction during phonation in 61.7% of patients, associated with a lower DSI score (0.65 [C.I. 0.48–0.88] P = .006).ConclusionsIn LTS patients, after airway reconstruction MRI can safely provide excellent structural and functional detail of the vocal cords correlating to DSI, with further usefulness expected from technical refinements. We therefore suggest MRI as a tool for extensive imaging during LTS follow‐up.Level of evidence3 Laryngoscope, 131:E2402–E2408, 2021

Children with a tracheostomy often present with swallowing disorders. Assessing the impact the presence of the tracheostomy tube has on swallowing function next to the underlying pathology can be very challenging. This article gives an overview of normal swallowing physiology and development, swallowing difficulties as encountered in various airway pathologies and addresses the mechanism by which the tracheostomy tube impacts swallowing. We discuss methods of investigating swallowing disorders and offer tools for management in everyday practice.

Objectives/HypothesisOtolaryngology instructional videos available online are often of poor quality. The objective of this article was to establish international consensus recommendations for the production of educational surgical videos in otolaryngology.Study DesignDELPHI survey.MethodsTwenty‐seven international respondents participated in this study from 12 countries. Consensus was reached after three rounds of questionnaires following the Delphi methodology. The proposals having reached the 80% agreement threshold in the third round were retained.ResultsThe main recommendations are as follows: 1) Ethics: patients must be anonymized and unrecognizable (apart from plastic surgery if necessary). A signed authorization must be obtained if the person is recognizable. 2) Technical aspects: videos should be edited and in high‐definition (HD) quality if possible. Narration or subtitles and didactic illustrations are recommended. 3) Case presentation: name of pathology and procedure must be specified; the case should be presented with relevant workup. 4) Surgery: surgical procedures should be divided into several distinct stages and include tips and pitfalls. Pathology should be shown if relevant. Key points should be detailed at the end of the procedure. 5) Organ‐specific: type of approach and bilateral audiometry should be specified in otology. Coronal plane computed tomography scans should be shown in endonasal surgery. It is recommended to show pre‐ and postoperative videos in voice surgery and preoperative drawings and photos of scars in plastic surgery, as well as the ventilation method in airway surgery.ConclusionsInternational recommendations have been determined to assist in the creation and standardization of educational surgical videos in otolaryngology and head and neck surgery.Level of Evidence5 Laryngoscope, 131:E732–E737, 2021

INTRODUCTION:Obstructive sleep apnea (OSA) is frequently present in patients with syndromic craniosynostosis. The aim of this study is to determine the long-term effectiveness of our OSA treatment protocol in our tertiary center in a cohort of children with syndromic craniosynostosis.METHODS:Children with syndromic craniosynostosis born between January 2005 and December 2013 were eligible for inclusion (n = 114). Data from ambulatory and inhospital polysomnographies were used. The obstructive-apnea/hypopnea index was used for OSA classification.RESULTS:Polysomnographies were performed in 83 patients. Mild OSA was diagnosed in 19, moderate in six, and severe in seven children. Of the 32 patients with OSA, 12 patients (37.5%) initially received expectant care of which OSA resolved spontaneously in nine without recurrence. Twenty patients were surgically treated. Adenotonsillectomy (ATE) had a 90% success rate with no OSA recurrence. Monobloc surgery was performed in four patients with mild OSA, although not OSA-indicated. Monobloc was performed for moderate or severe OSA in six patients, in four patients in combination with ATE and with mandibular distraction in one. Monobloc surgery for moderate or severe OSA had a 100% success rate in treating OSA and decannulation.CONCLUSION:Expectant care is often sufficient to resolve mild OSA in patients with syndromic craniosynostosis, and should also be considered in patients with moderate OSA with close follow-up. ATE has an important role in the OSA treatment protocol. Monobloc surgery, combined with mandibular distraction on indication, is effective in resolving moderate to severe OSA with a stable long-term result.

Background Pathogenic germline variants in Transient Receptor Potential Vanilloid 4 Cation Channel (TRPV4) lead to channelopathies, which are phenotypically diverse and heterogeneous disorders grossly divided in neuromuscular disorders and skeletal dysplasia. We recently reported in sporadic giant cell lesions of the jaws (GCLJs) novel, somatic, heterozygous, gain-of-function mutations in TRPV4, at Met713. Methods Here we report two unrelated women with a de novo germline p.Leu619Pro TRPV4 variant and an overlapping systemic disorder affecting all organs individually described in TRPV4 channelopathies. Results From an early age, both patients had several lesions of the nervous system including progressive polyneuropathy, and multiple aggressive giant cell-rich lesions of the jaws and craniofacial/skull bones, and other skeletal lesions. One patient had a relatively milder disease phenotype possibly due to postzygotic somatic mosaicism. Indeed, the TRPV4 p.Leu619Pro variant was present at a lower frequency (variant allele frequency (VAF)=21.6%) than expected for a heterozygous variant as seen in the other proband, and showed variable regional frequency in the GCLJ (VAF ranging from 42% to 10%). In silico structural analysis suggests that the gain-of-function p.Leu619Pro alters the ion channel activity leading to constitutive ion leakage. Conclusion Our findings define a novel polysystemic syndrome due to germline TRPV4 p.Leu619Pro and further extend the spectrum of TRPV4 channelopathies. They further highlight the convergence of TRPV4 mutations on different organ systems leading to complex phenotypes which are further mitigated by possible post-zygotic mosaicism. Treatment of this disorder is challenging, and surgical intervention of the GCLJ worsens the lesions, suggesting the future use of MEK inhibitors and TRPV4 antagonists as therapeutic modalities for unmet clinical needs.

RationalePaediatric laryngotracheal stenosis (LTS) is often successfully corrected with open airway surgery. However, respiratory and vocal sequelae frequently remain. Clinical care and surgical interventions could be improved with better understanding of these sequelae.ObjectiveThe objective of this cross-sectional study was to develop an upper airway MRI protocol to obtain information on anatomical and functional sequelae post-LTS repair.MethodsForty-eight patients (age 14.4 (range 7.5–30.7) years) and 11 healthy volunteers (15.9 (8.2–28.8) years) were included. Spirometry and static and dynamic upper airway MRI (3.0 T, 30 min protocol) were conducted. Analysis included assessment of postoperative anatomy and airway lumen measurements during static and dynamic (inspiration and phonation) acquisitions.Main resultsGood image quality without artefacts was achieved for static and dynamic images in the majority of MRIs. MRI showed vocal cord thickening in 80.9% of patients and compared with volunteers, a significant decrease in vocal cord lumen area (22.0 (IQR 17.7–30.3) mm2vs 35.1 (21.2–54.7) mm2, p=0.03) but not cricoid lumen area (62.3±27.0 mm2vs 66.2±34.8 mm2, p=0.70). Furthermore, 53.2% of patients had an A-frame deformation at site of previous tracheal cannula, showing lumen collapse during inspiration. Dynamic imaging showed incomplete vocal cord abduction during inspiration in 42.6% and incomplete adduction during phonation in 61.7% of patients.ConclusionsStatic and dynamic MRI is an excellent modality to non-invasively image anatomy, tissue characteristics and vocal cord dynamics of the upper airways. MRI-derived knowledge on postsurgical LTS sequelae might be used to improve surgery.

Introduction: Patients with Treacher Collins or Nager syndrome have a significant risk on obstructive sleep apnea (OSA): 25 to 41 % have severe OSA, without a natural tendency for improvement. These patients are not always directly referred to a craniofacial center of expertise and may be treated for cleft palate in a cleft team. If these care providers are not aware of the risk of upper airway obstruction following cleft repair, serious complications can result. Methods: In this presentation, an overview of Treacher Collins or Nager patients with severe OSA is given, including their treatment and outcome, treated at our clinic between 2010 and 2019. Results: A total of 19 patients were treated: 17 Treacher Collins and 2 Nager syndrome. Eight (42%) had severe OSA for which 5 were treated with a tracheal canula (2 immediately following palatal closure), 2 with nasopharyngeal tube and 1 with CPEP. Four children died: parents of a newborn with Treacher Collins and severe OSA refused placement of a canula or other respiratory support, a 16-year old patient (without OSA) committed suicide and 2 patients died of acute respiratory obstruction. These last two children both had severe OSA for which mandibular distraction was performed, followed by palatal repair and decanullation. One child died at 2 years of age following a viral upper airway infection and aspiration, and the other at 4 years of age after chooking in a sweet. One patient had several mandibular distraction procedures and TMJ surgery followed by decanulation elsewhere, but returned with severe OSA and a pinpoint opening of the trachea at 17 years of age. One child had an unsuccesfull mandibular distraction at 2 years of age and a repeated distraction at 6, and decanulated is now prepared. Distraction at 3.5 years of age resulted in decanulation in another patient, but OSA recurred for which a second distraction was performed at 9 years of age, which solved the OSA. Another child just had her mandibular distraction at 3 years of age, while the last patient, aged 22, is scheduled for surgery. Conclusion: Treacher Collins syndrome patients have a high risk on severe OSA, but succesfull early treatment does not normalize their upper airway. OSA might reoccur and swallowing problems are more common. More awareness of cleft surgeons is necessary to prevent palatal closure that results in severe airway obstruction.

Background: Laryngotracheal stenosis(LTS) is often successfully corrected with open airway surgery. However, majority of patients have respiratory and vocal sequelae. Better understanding of these sequelae is needed to improve clinical care and surgical interventions. Aim: To assess the larynx in pediatric patients post LTS with Magnetic Resonance Imaging(MRI). Methods: Forty-six patients(mean age 15.4, range 7.5-30.6 years) and 8 healthy volunteers (age 10.4,8.1-28.7 years) underwent static and dynamic laryngeal MRI(3.0T, 6 channels carotid coil). Diffusion Weighted Imaging(DWI) was included to assess inflammation/fibrosis. Image quality was scored as poor to excellent(1-5). Laryngeal abnormalities were classified according to anatomical location. Vocal cord dysfunction was defined as impaired abduction during respiration or adduction during phonation, or vocal cord paralysis. Results: A 30 min MRI protocol was developed with high spatial(0.5x0.5x2.0mm3) and temporal(240 ms) resolution. Image quality for static and dynamic images was good(median 4(IQR 4-4) and 4(IQR 4-4)). Poor image quality was due to inadequate coil positioning or motion artefacts. MRI showed vocal cord and arytenoid thickening in 40/46(87%) and 15/46(32.6%) of patients. Tracheal deformation due to prior tracheal cannula was seen in 32/46(69.6%). DWI showed inflammation/fibrosis at vocal cords or arytenoids in 19/43(44.2%). Vocal cord dysfunction was seen in 33/45(77.3%). Conclusion: Static and dynamic MRI is suitable for evaluating post LTS children. Further data analysis including airway measurements and correlations with pulmonary and vocal function will be the basis for improving LTS surgical interventions.

AimsWe aimed to compare the pharmacokinetics (PK) and safety profile of tobramycin inhalation solution (TIS) using the I‐neb device to the standard PARI‐LC Plus nebulizer in children with cystic fibrosis.MethodsA randomized, open‐label, crossover study was performed. In 2 separate study visits, blood samples from 22 children were collected following TIS nebulization with I‐neb (75 mg) and PARI‐LC Plus (300 mg). Study visits were separated by 1 month, in which 1 of the study nebulizers was used twice daily. Tobramycin PK for both nebulizers was established using measured tobramycin concentrations and Bayesian PK modelling software. Hearing and renal function tests were performed to test for aminoglycoside associated toxicity. In addition to standard estimated glomerular filtration rate values, biomarkers for tubular injury (KIM‐1 and NAG) were measured. Patient and nebulizer satisfaction were assessed.ResultsInhalations were well tolerated and serum trough concentrations below the predefined toxic limit were reached with no significant differences in PK parameters between nebulizers. Results of audiometry and estimated glomerular filtration rate revealed no abnormalities. However, increased urinary NAG/creatinine ratios at visit 2 for both nebulizers suggest TIS‐induced subclinical tubular kidney injury. Nebulization time was 50% shorter and patient satisfaction was significantly higher with the I‐neb.ConclusionsNebulization of 75 mg TIS with the I‐neb in children with cystic fibrosis resulted in comparable systemic exposure to 300 mg TIS with the PARI‐LC Plus and was well tolerated and preferred over the PARI‐LC Plus. Long‐term safety of TIS nebulization should be monitored clinically, especially regarding the effects on tubular kidney injury.

BackgroundMagnetic Resonance Imaging (MRI) techniques to image the larynx have evolved rapidly into a promising and safe imaging modality, without need for sedation or ionizing radiation. MRI is therefore of great interest to image pediatric laryngeal diseases. Our aim was to review MRI developments relevant for the pediatric larynx and to discuss future imaging options. MethodsA systematic search was conducted to identify all morphological and diagnostic studies in which MRI was used to image the pediatric larynx, laryngeal disease, or vocal cords. ResultsFourteen articles were included: three studies on anatomical imaging of the larynx, two studies on Diffusion Weighted Imaging, four studies on vocal cord imaging and five studies on the effect of anaesthesiology on the pediatric larynx. MRI has been used for pediatric laryngeal imaging since 1991. MRI provides excellent soft tissue contrast and good visualization of vascular diseases such as haemangiomas. However, visualization of cartilaginous structures, with varying ossification during childhood, and tissue differentiation remain challenging. The latter has been partly overcome with diffusion weighted imaging (DWI), differentiating between benign and malignant masses with excellent sensitivity (94-94.4%) and specificity (91.2-100%). Vocal cord imaging evolved from static images focused on vocal tract growth to dynamic images able to detect abnormal vocal cord movement. ConclusionMRI is promising to evaluate the pediatric larynx, but studies using MRI as diagnostic imaging modality are scarce. New static and dynamic MR imaging techniques could be implemented in the pediatric population. Further research on imaging of pediatric laryngeal diseases should be conducted.

Obstructive sleep apnea (OSA) is present in 70% of children with Apert or Crouzon/Pfeiffer syndrome, ranging from mild to severe OSA. Treatment of OSA includes nasal corticosteroids, oxygen during the night, CPAP, BiPAP, nasopharyngeal tube, tracheal canula, adenotonsillectomy, palatal widening, midface advancement, and mandibular lengthening. The choice of treatment depends on age of the patient, severity of the OSA and other features that impair the use of certain treatment options. For instance, new-borns are reluctant to accept CPAP or BiPAP and in patients with severe exorbitism, airflow may jeopardize the corneas. For patients with Apert and Crouzon syndrome, midface advancement is generally regarded as the best suited procedure to resolve OSA. In most cases, breathing is strongly improved following a midface procedure, but not completely resolved in all. OSA has been shown to have multilevel causes with the obstructions situated within the nose, nasopharynx, oropharynx, tongue base, hypopharynx or tracheal level. The optimal treatment of OSA thus requires an endoscopic assessment of the upper airway to detect the involved levels of obstruction, next to the sleep studies. In this presentation, the various anatomic obstruction of the upper airway are presented, along with the short-term and long-term outcome on breathing of the various treatment options.