Slide Tracheoplasty to Repair Stenotic Tracheal Cartilaginous Sleeve with Advanced Surgical Planning.
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ABSTRACT:
SUBMITTER: Zenner K
PROVIDER: S-EPMC7980779 | biostudies-literature | 2020 Aug
REPOSITORIES: biostudies-literature
Publications
Slide Tracheoplasty to Repair Stenotic Tracheal Cartilaginous Sleeve with Advanced Surgical Planning.
Zenner Kaitlyn K Bonilla-Velez Juliana J Johnson Kaalan K Bly Randall A RA
Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery 20200414 2
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Project description:PurposeBecause a tracheal cartilaginous sleeve (TCS) confers a significant mortality risk that can be mitigated with appropriate intervention, we sought to describe the prevalence and associated genotypes in a large cohort of children with syndromic craniosynostosis.MethodsChart review of patients with syndromic craniosynostosis across two institutions.ResultsIn a cohort of 86 patients with syndromic craniosynostosis, 31 required airway evaluation under anesthesia. TCS was found in 19, for an overall prevalence of 22%. FGFR2, TWIST1, and FGFR3 mutations were identified in children with TCS. All five children with a W290C mutation in FGFR2 had TCS, and most previously reported children with W290C had identification of TCS or early death. In contrast, TCS was not associated with other mutations at residue 290.ConclusionThere is an association between TCS and syndromic craniosynostosis, and it appears to be particularly high in individuals with the W290C mutation in FGFR2. Referral to a pediatric otolaryngologist and consideration of operative airway evaluation (i.e., bronchoscopy or rigid endoscopy) in all patients with syndromic craniosynostosis should be considered to evaluate for TCS. Results from genetic testing may help providers weigh the risks and benefits of early airway evaluation and intervention in children with higher-risk genotypes.Genet Med 19 1, 62-68.
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Project description:ObjectivesTo characterize tracheal cartilage morphology in mouse models of fibroblast growth factor receptor (Fgfr2)-related craniosynostosis syndromes. To establish relationships between specific Fgfr2 mutations and tracheal cartilaginous sleeve (TCS) phenotypes in these mouse models.MethodsPostnatal day 0 knock-in mouse lines with disease-specific genetic variations in the Fgfr2 gene (Fgfr2C342Y/C342Y , Fgfr2C342Y/+ , Fgfr2+/Y394C , Fgfr2+/S252W , and Fgfr2+/P253R ) as well as line-specific controls were utilized. Tracheal cartilage morphology as measured by gross analyses, microcomputed tomography (μCT), and histopathology were compared using Chi-squared and single-factor analysis of variance statistical tests.ResultsA greater proportion of rings per trachea were abnormal in Fgfr2C342Y/+ tracheas (63%) than Fgfr2+/S252W (17%), Fgfr2+/P253R (17%), Fgfr2+/Y394C (12%), and controls (10%) (P < .001 for each vs. Fgfr2C342Y/+ ). TCS segments were found only in Fgfr2C342Y/C342Y (100%) and Fgfr2C342Y/+ (72%) tracheas. Cricoid and first-tracheal ring fusion was noted in all Fgfr2C342Y/C342Y and 94% of Fgfr2C342Y/+ samples. The Fgfr2C342Y/C342Y and Fgfr2C342Y/+ groups were found to have greater areas and volumes of cartilage than other lines on gross analysis and μCT. Histologic analyses confirmed TCS among the Fgfr2C342Y/C342Y and Fgfr2C342Y/+ groups, without appreciable differences in cartilage morphology, cell size, or density; no histologic differences were observed among other Fgfr2 lines compared to controls.ConclusionThis study found TCS phenotypes only in the Fgfr2C342Y mouse lines. These lines also had increased tracheal cartilage compared to other mutant lines and controls. These data support further study of the Fgfr2 mouse lines and the investigation of other Fgfr2 variants to better understand their role in tracheal development and TCS formation.Level of evidenceNA Laryngoscope, 131:E1349-E1356, 2021.
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Project description:ObjectivesTo investigate how virtual reality (VR) imaging impacts decision-making in atrioventricular valve surgery.MethodsThis was a single-center retrospective study involving 15 children and adolescents, median age 6 years (range, 0.33-16) requiring surgical repair of the atrioventricular valves between the years 2016 and 2019. The patients' preoperative 3-dimesnional (3D) echocardiographic data were used to create 3D visualization in a VR application. Five pediatric cardiothoracic surgeons completed a questionnaire formulated to compare their surgical decisions regarding the cases after reviewing conventionally presented 2-dimesnional and 3D echocardiographic images and again after visualization of 3D echocardiograms using the VR platform. Finally, intraoperative findings were shared with surgeons to confirm assessment of the pathology.ResultsIn 67% of cases presented with VR, surgeons reported having "more" or "much more" confidence in their understanding of each patient's pathology and their surgical approach. In all but one case, surgeons were at least as confident after reviewing the VR compared with standard imaging. The case where surgeons reported to be least confident on VR had the worst technical quality of data used. After viewing patient cases on VR, surgeons reported that they would have made minor modifications to surgical approach in 53% and major modifications in 7% of cases.ConclusionsThe main impact of viewing imaging on VR is the improved clarity of the anatomical structures. Surgeons reported that this would have impacted the surgical approach in the majority of cases. Poor-quality 3D echocardiographic data were associated with a negative impact of VR visualization; thus. quality assessment of imaging is necessary before projecting in a VR format.
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Project description:Tracheal sleeve pneumonectomy for lung cancer is an old technique, and it is reserved for exceptional cases with tracheal carina involvement. Intra-operative airways management of this operation is incredibly complex, involving thoracic surgeons, anaesthesiologists and pulmonologists. We report a case of a 38-year-old male with no clinical history, referred to our department for an adenoid-cystic carcinoma involving distal trachea, carina and main right bronchus. Tracheal sleeve pneumonectomy was performed using extra-corporeal membrane oxygenation (ECMO). A veno-venous ECMO circuit was established through a heparin-coated percutaneous cannula in the right femoral vein and a heparin-coated percutaneous cannula in the internal right jugular vein by ultrasound assistance. No major complications occurred, and the patient was discharged after 30-day bronchoscopic control, showing the absence of fistula and negativity of the methylene blue test. ECMO-assisted surgery ensures adequate respiratory support, haemodynamic stability, lower risk of bleeding complications with a clean operating field and better brain and myocardial oxygenation.
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Project description:There is a critical need to engineer a neotrachea because currently there are no long-term treatments for tracheal stenoses affecting large portions of the airway. In this work, a modular tracheal tissue replacement strategy was developed. High-cell density, scaffold-free human mesenchymal stem cell-derived cartilaginous rings and tubes were successfully generated through employment of custom designed culture wells and a ring-to-tube assembly system. Furthermore, incorporation of transforming growth factor-β1-delivering gelatin microspheres into the engineered tissues enhanced chondrogenesis with regard to tissue size and matrix production and distribution in the ring- and tube-shaped constructs, as well as luminal rigidity of the tubes. Importantly, all engineered tissues had similar or improved biomechanical properties compared to rat tracheas, which suggests they could be transplanted into a small animal model for airway defects. The modular, bottom up approach used to grow stem cell-based cartilaginous tubes in this report is a promising platform to engineer complex organs (e.g., trachea), with control over tissue size and geometry, and has the potential to be used to generate autologous tissue implants for human clinical applications.
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