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This case report describes a mycobacterial infection in an adult Whippet cross dog. The dog was diagnosed with Mycolicibacterium sediminis infection, a species of mycobacteria that is yet to be reported as a causative agent of infection in humans or domestic animals. The dog was presented for specialist opinion of a 6-month history of severe facial lymphadenopathy that was nonresponsive to antibiotic and immunosuppressive therapy. A necrotic lesion developed on her right antebrachium approximately 10-14 days before presentation. The dog was anaesthetised for computed tomography and nodule and skin biopsies including fresh tissue for mycobacterial polymerase chain reaction (PCR). The nodules contained pyogranulomatous inflammation and perivascular necrosis that are typically found in mycobacterial infections. The mycobacterial PCR isolated Mycolicibacterium sediminis. The dog was prescribed triple antibiotic therapy and tapered off corticosteroids, with noticeable improvement within 4 weeks and resolution of granulomas within 3 months of therapy. Presence of chronic dermal pyogranulomatous inflammation should raise suspicions for mycobacterial disease, and fresh tissue should be submitted for PCR to aid in diagnosis., (© 2024 Australian Veterinary Association.)

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., (© 2025 The Author(s). Pediatric Anesthesia published by John Wiley & Sons Ltd.)

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 (CO 2 ) levels. This study aimed to investigate the effects of the clinical implementation of transcutaneous CO 2 (tcPCO 2 ) 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 tcPCO 2 monitoring were excluded., Interventions: TcPCO 2 monitoring was performed in two groups; blinded before clinical implementation (control group) and visible for ventilation management after clinical implementation (tcPCO 2 group)., Measurements: The total tcPCO 2 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 tcPCO 2 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 tcPCO 2 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 tcPCO 2 group. Spontaneous breathing was associated with lower tcPCO 2 levels. The general satisfaction score of tcPCO 2 monitoring rated by the anesthesiologist was 8.19 (0.96)., Conclusions: TcPCO 2 levels reached approximately twice the upper limit of the normal range during rigid bronchoscopy and MLS. Availability of tcPCO 2 monitoring did not affect these high levels, despite adjustments in strategy. However, tcPCO 2 monitoring provides valuable insight in CO 2 load and applied ventilation strategies., Competing Interests: Declaration of competing interest The authors have declared no conflict of interest. This study was funded by the Erasmus MC Sophia Foundation (grant number: B1603B)., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Introduction: Tracheomalacia (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 Analysis: This 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 Dissemination: Patients 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 Number: NCT06335862., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

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., Competing Interests: Conflicts of interest The authors have no relevant financial or non-financial interests to disclose., (Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.)

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: Pathogenic germline variants in T ransient R eceptor P otential V anilloid 4 C ation C hannel ( 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., Competing Interests: Competing interests: RG and CCG are research fellows at the National Council for Scientific and Technological Development, Brazil., (© Author(s) (or their employer(s)) 2022. Re-use permitted under CC BY. Published by BMJ.)

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., (Copyright © 2021 The Authors. Published by Elsevier B.V. All rights reserved.)

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., Competing Interests: Declaration of Competing Interest None., (Copyright © 2021 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.)

Objectives/hypothesis: Dysphonia 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 Design: Prospective cohort study., Methods: Voice 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., Results: Forty-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)., Conclusions: In 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 Evidence: 3 Laryngoscope, 131:E2402-E2408, 2021., (© 2021 The Authors. The Laryngoscope published by Wiley Periodicals LLC. on behalf of The American Laryngological, Rhinological and Otological Society, Inc.)

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., (Copyright © 2021. Published by Elsevier Inc.)

Objectives/hypothesis: Otolaryngology 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 Design: DELPHI survey., Methods: Twenty-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., Results: The 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., Conclusions: International recommendations have been determined to assist in the creation and standardization of educational surgical videos in otolaryngology and head and neck surgery., Level of Evidence: 5 Laryngoscope, 131:E732-E737, 2021., (© 2020 The Authors. The Laryngoscope published by Wiley Periodicals LLC on behalf of American Laryngological, Rhinological and Otological Society Inc, "The Triological Society" and American Laryngological Association (ALA).)

Rationale: Paediatric 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., Objective: The 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., Methods: Forty-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 Results: Good 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) mm 2 vs 35.1 (21.2-54.7) mm 2 , p=0.03) but not cricoid lumen area (62.3±27.0 mm 2 vs 66.2±34.8 mm 2 , 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., Conclusions: Static 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., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY. Published by BMJ.)

Aims: We 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., Methods: A 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., Results: Inhalations 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., Conclusions: Nebulization 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., (© 2019 The Authors. British Journal of Clinical Pharmacology published by John Wiley & Sons Ltd on behalf of British Pharmacological Society.)