Browsing by Author "Saba, Thomas G."

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    A multi-disciplinary, comprehensive approach to management of children with heterotaxy
    (BMC, 2022-09-09) Saba, Thomas G.; Geddes, Gabrielle C.; Ware, Stephanie M.; Schidlow, David N.; del Nido, Pedro J.; Rubalcava, Nathan S.; Gadepalli, Samir K.; Stillwell, Terri; Griffiths, Anne; Bennett Murphy, Laura M.; Barber, Andrew T.; Leigh, Margaret W.; Sabin, Necia; Shapiro, Adam J.; Medical and Molecular Genetics, School of Medicine
    Heterotaxy (HTX) is a rare condition of abnormal thoraco-abdominal organ arrangement across the left-right axis of the body. The pathogenesis of HTX includes a derangement of the complex signaling at the left-right organizer early in embryogenesis involving motile and non-motile cilia. It can be inherited as a single-gene disorder, a phenotypic feature of a known genetic syndrome or without any clear genetic etiology. Most patients with HTX have complex cardiovascular malformations requiring surgical intervention. Surgical risks are relatively high due to several serious comorbidities often seen in patients with HTX. Asplenia or functional hyposplenism significantly increase the risk for sepsis and therefore require antimicrobial prophylaxis and immediate medical attention with fever. Intestinal rotation abnormalities are common among patients with HTX, although volvulus is rare and surgical correction carries substantial risk. While routine screening for intestinal malrotation is not recommended, providers and families should promptly address symptoms concerning for volvulus and biliary atresia, another serious morbidity more common among patients with HTX. Many patients with HTX have chronic lung disease and should be screened for primary ciliary dyskinesia, a condition of respiratory cilia impairment leading to bronchiectasis. Mental health and neurodevelopmental conditions need to be carefully considered among this population of patients living with a substantial medical burden. Optimal care of children with HTX requires a cohesive team of primary care providers and experienced subspecialists collaborating to provide compassionate, standardized and evidence-based care. In this statement, subspecialty experts experienced in HTX care and research collaborated to provide expert- and evidence-based suggestions addressing the numerous medical issues affecting children living with HTX.
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    Incorporating a Three-Dimensional Printed Airway into a Pediatric Flexible Bronchoscopy Curriculum
    (American Thoracic Society, 2024-01-30) Painter, Natalia; Monovoukas, Demetri; Delecaris, Angela O.; Coverstone, Andrea M.; Zopf, David A.; Saba, Thomas G.; Pediatrics, School of Medicine
    Background: Although hands-on simulation plays a valuable role in procedural training, there are limited tools available to teach pediatric flexible bronchoscopy (PFB). Fellowship programs rely on patient encounters, with inherent risk, or high-cost virtual reality simulators that may not be widely available and create education inequalities. Objective: Our objective was to study the educational value and transferability of a novel, low-cost, three-dimensional-printed pediatric airway model (3D-AM) for PFB training. Our central hypothesis was that the 3D-AM would have high educational value and would be easily transferrable to learners at different teaching hospitals. Methods: The 3D-AM was designed to teach technical bronchoscopy skills, airway anatomy, airway pathology, and bronchoalveolar lavage (BAL). The curriculum was offered to incoming fellows in pediatric pulmonology, pediatric surgery, and pediatric critical care across three different teaching institutions. After course completion, each participant assessed the simulation model(s) with a 5-point Likert scale across six domains: physical attributes, realism of experience, ability to perform tasks, value, relevance, and global impression. The expert instructors assessed the learners' competency using a modified version of the Bronchoscopy Skills and Tasks Assessment Tool. Results: A total of 14 incoming fellows participated in the course. The mean scores for the 3D-AM across all six domains and across the three institutions was between 4 and 5, suggesting that learners generally had a favorable impression and a similar experience across different institutions. All learners "agreed" or "strongly agreed" that the course was a valuable use of their time, helped teach technical skills and airway anatomy, and would be useful for extra training during fellowship. Most of the learners correctly identified anatomy, bronchomalacia, and performed a BAL. Wall trauma was observed in 36% of learners. Conclusion: The utility, low cost, and transferability of this model may create opportunities for PFB training across different institutions despite resource limitations in the United States and abroad.