is a rare diffuse cystic lung disease that almost exclusively affects women (1). LAM can occur sporadically, or be associated with tuberous sclerosis complex (TSC), a heritable tumor suppressor syndrome associated with hamartomas in multiple organs, seizures, and cognitive impairment (2-4). Cystic changes consistent with LAM have been reported to occur in 26-49% of women with TSC (2, 5-7), and most female patients with TSC have at least a few pulmonary cysts by age 40 years (5). Current Tuberous Sclerosis Alliance guidelines recommendations include screening women with TSC for LAM with high-resolution computed tomography starting at the age of 18 years (8). The National Heart, Lung, and Blood Institute (NHLBI) LAM Registry Committee reported that the 5-, 10-, 15-, and 20-year transplant-free survival in LAM is 95%, 85%, 75%, and 64%, respectively (9). TSC-LAM is believed to be milder and less progressive than sporadic LAM, but the natural history of TSC-LAM and its impact on mortality is less well understood (9). The purposes of this study were to better understand the long-term prognosis of LAM in patients with TSC and to establish the leading causes of death in this population. MethodsAuthor disclosures are available with the text of this letter at www.atsjournals.org.
Introduction: Family-centered rounds (FCR) are a common method for daily communication between families and providers in pediatric hospitals. Traditionally, medical education teaches students fundamental oral presentation skills. However, students frequently struggle with synthesizing pertinent information for communication to patients and families, especially without the use of medical jargon. To address this deficit, we created a workshop for third-year medical students on their pediatric clerkship to provide expectations for FCR presentations and allow for directed practice with immediate feedback. Methods: This 1-hour workshop begins with a preworkshop survey and PowerPoint introductory presentation on FCR. Participants then watch videos of both good and bad history and physical (H&P) performances and discuss the differences. The workshop ends with 30 minutes for small-group H&P practice. Results: This workshop has increased medical students' perceived comfort with oral presentations on family-centered rounds, as well as their ability to identify and synthesize pertinent information. Discussion: The workshop has created a solid foundation for the introduction of medical students to family-centered rounds presentations; however, further development of educational and feedback tools would continue to enhance this learning experience. The creation and maintenance of the workshop relied heavily upon senior resident involvement to serve as small-group facilitators.
ObjectivesBronchial anomalies are rare but challenging conditions to treat in children, encompassing a variety of structural abnormalities that could compromise airway patency. This includes complete rings, absent cartilage, traumatic avulsions, bronchoesophageal fistulas, and cartilaginous sleeves. The objective of this study is to describe the characteristics and outcomes of a series of pediatric cases of bronchial anomalies that were treated by slide tracheobronchoplasty.MethodsThis is a single‐institution retrospective case series of pediatric patients with bronchial anomalies who underwent surgical treatment between February 2004 and April 2020. Data extracted from electronic medical records included patient demographics, comorbidities, and surgical outcomes.ResultsThere were a total of 29 patients included in the study, of which 14 had complete bronchial rings, 8 had absent bronchial rings, 4 had traumatic bronchial avulsions, 2 had bronchoesophageal fistulas, and one had a cartilaginous sleeve. Median follow‐up time was 13 months (with a range of 0.5–213 months). The overall mortality rate was 17.2% (5 patients), all of whom had complete bronchial rings. Patients with complete bronchial rings also had a higher rate of not only cardiac (85.7%) and pulmonary comorbidities (85.7%) but also secondary airway lesions (78.6%).ConclusionThis is the largest series to date describing surgical treatment for bronchial anomalies. Complete bronchial rings were the most common anomaly treated, followed by absent rings and trauma. Surgical treatment can be successful but mortality rates are higher in patients with complete bronchial rings, possibly due to higher rates of pulmonary and cardiac comorbidities.Level of Evidence4 Laryngoscope, 2023
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