左肺動脈右肺動脈起始症

Pulmonary developmental disorders comprise a heterogeneous group of congenital abnormalities involving the airways, lung parenchyma, pulmonary vasculature, or overlapping developmental pathways. Although these conditions commonly present during infancy or childhood, many remain clinically silent until adulthood, when they are discovered incidentally or manifest with recurrent respiratory symptoms such as infection, hemoptysis, or progressive dyspnea. These abnormalities may be broadly categorized into airway, vascular, parenchymal, and mixed developmental anomalies. Airway abnormalities include congenital tracheobronchomegaly, branching anomalies of the tracheobronchial tree, bronchial atresia, bronchogenic cysts, congenital pulmonary airway malformations (CPAM), and bronchopulmonary sequestration (BPS). Vascular anomalies include pulmonary artery sling, proximal interruption of the pulmonary artery, Scimitar syndrome, and meandering pulmonary veins. Parenchymal disorders encompass congenital lobar overinflation and pulmonary hypoplasia. Increasing recognition of overlapping lesions, particularly combinations of CPAM, sequestration, and bronchial atresia, has challenged traditional compartmental classification systems and supports a developmental spectrum model. This review synthesizes contemporary concepts regarding embryologic mechanisms, pathogenesis, imaging findings, and management of these entities in adults. Particular emphasis is placed on imaging evaluation with CT and MRI, updated terminology, evolving molecular understanding of CPAMs, including lesions with malignant potential, and distinctions between congenital sequestration and pseudosequestration. Recognition of these disorders and their associated anomalies is essential for accurate diagnosis, appropriate management, and surgical planning in adult patients.

An infant presented with persistent stridor and recurrent respiratory distress since birth. Imaging revealed a left pulmonary artery sling causing significant tracheal compression. Surgical reimplantation of the left pulmonary artery to the main pulmonary artery with concurrent slide tracheoplasty resulted in full recovery. This case highlights the importance of early recognition of vascular anomalies in infants presenting with unexplained airway obstruction.

Pulmonary artery sling is a rare congenital anomaly in which the left pulmonary artery arises anomalously from the right pulmonary artery. With rigorous pre-operative assessment, we successfully repaired isolated pulmonary artery sling using left thoracotomy in a highly selected group of children. In this modern series, we have achieved satisfactory surgical outcomes without using cardiopulmonary bypass support.

OBJECTIVE: Patients with a left pulmonary artery (LPA) sling often require surgical reimplantation to address pulmonary flow maldistribution and tracheal compression. We developed a virtual modeling workflow to determine what length of LPA should be retained for reimplantation and provided these data for intraoperative guidance. METHODS: From March 2024 to May 2025, 5 patients diagnosed with an LPA sling with (n = 3) or without (n = 2) tracheal stenosis underwent surgical LPA reimplantation with or without slide tracheoplasty at median age 1.8 years [range, 0.4-3.6 years]. All patients had preoperative surgical planning using measurements from a segmented 3-dimensional model using a digital workflow. Model measurements included distances from the main pulmonary artery reimplantation site to nearby landmarks and the length of pressurized, prestretched LPA that should be retained for reimplantation based on the distance between the reimplant site and anticipated extent of distal LPA mobilization. These measurements were used as intraoperative guidance. RESULTS: The planned main pulmonary artery reimplant site varied across surgeons, and recommended LPA length ranged from 42% to 71% of the total native length. Model recommendations were closely followed in all patients with LPA sling. Median hospital length of stay was 7 days [6-15]. At discharge, 3 patients had no/trivial LPA stenosis and 2 had mild (peak gradient 23 mm Hg [20, 26]). At median follow-up of 17.6 months [6.5-22.1], all patients had no/trivial stenosis and were asymptomatic with 100% freedom from reintervention. Postoperative lung-perfusion and computed tomography scans demonstrated left/right split of 40/60% ± 2% (n = 3) and LPA diameter z score of -0.23 [-0.82, +0.31] (n = 2). CONCLUSIONS: Three-dimensional models provide a platform for quantitative surgical planning to help achieve targeted reconstruction with good initial clinical follow-up.

Congenital tracheobronchial variants (CTBVs) may distort airway landmarks, alter carinal recognition, and complicate ventilation during pediatric endotracheal intubation. Although uncommon, these anomalies can create important peri-intubation risks that are often underrecognized. This scoping review was conducted in accordance with the Joanna Briggs Institute methodology and Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for Scoping Reviews (PRISMA-ScR) guidance. Using a Population-Concept-Context framework, we identified human case reports and small case series describing congenital tracheal or bronchial anomalies relevant to intubation, bronchoscopy, ventilation, or airway planning. Searches were performed in PubMed, Scopus, and Web of Science, with additional studies identified through manual searching. Sixty-six records were identified. After removal of one duplicate, 61 records from the database search were screened, 42 were excluded, and 23 full-text reports were assessed. Twenty studies were included, comprising 18 case reports and two small case series. Six studies described tracheal agenesis or atresia, four bronchial atresia, three tracheal bronchi, three bridge bronchi with or without pulmonary artery sling, and four bronchial isomerism or laterality defects. Cases ranged from 21 weeks of gestation to 15 years of age. Tracheal agenesis or atresia produced the most severe cannot-intubate presentations. Fixed stenotic lesions increased risks during tube passage and sizing, whereas distal anomalies more commonly mimicked selective intubation, mucus plugging, or refractory wheeze. CTBVs have important implications for pediatric airway management. Pre-intubation anatomical assessment and early bronchoscopic confirmation may reduce avoidable complications and support safer individualized airway planning.