先天性気管狭窄症／先天性声門下狭窄症

We report the case of a child with right pulmonary agenesis, congenital tracheal stenosis, and atrial septal defect (ASD). At age 2 years, an intrathoracic tissue expander was placed to reduce tracheal compression from mediastinal shift and to enable subsequent atrial septal defect closure. At age 5 years, a right atrial mass was incidentally detected along with right atrial compression caused by the expander. Emergency mass resection was performed, and the mass was diagnosed as a thrombus. The tissue expander was subsequently deflated and removed to prevent further compression. The patient had no recurrence of thrombus or respiratory symptoms during 8-year follow-up.

BACKGROUND: To explore targeted nursing strategies for managing postoperative complications in children with congenital tracheal stenosis (CTS) following tracheoplasty. METHODS: A total of 263 children with CTS who underwent tracheoplasty were retrospectively analyzed. Key aspects of postoperative nursing included body positioning, endotracheal intubation management, invasive mechanical ventilation management, airway care, and the identification and management of common postoperative complications. RESULTS: Twelve children died during hospitalization, yielding an overall mortality rate of 4.5%. Granulation tissue hyperplasia was observed in 18 cases and was treated with endoscopic laser cauterization and/or forceps removal. Anastomotic scar contracture occurred in 34 cases and was treated with endoscopic balloon dilatation. Severe tracheomalacia affecting ventilation was identified in 43 patients, among whom 9 required tracheal silicone stent implantation. A total of 251 surviving children were followed up for 1-56 months (median: 13 months). Two children with preoperative bronchiolitis obliterans remained dependent on intermittent oxygen therapy at the final follow-up. Respiratory symptoms were significantly improved in the remaining survivors. CONCLUSION: Meticulous postoperative airway nursing care in children with CTS can improve surgical outcomes, reduce complications, and facilitate postoperative recovery.

Congenital tracheal stenosis (CTS) from complete tracheal rings is a rare and potentially life-threatening condition necessitating complex multidisciplinary management. This clinical review explores the anatomic patterns of CTS and discusses diagnostic nuances, emphasizing the use of ultrasmall instrumentation to avoid mucosal trauma. We detail the historical failure of graft-based repairs and the paradigm shift brought by slide tracheoplasty. Current surgical management for long-segment and short-segment disease is discussed, emphasizing the narrowest point philosophy, 45-degree beveled incisions, and internal Grillo sutures. Finally, we address the management of technical complications and the coordinated aerodigestive model of care.

BACKGROUND: Conventional diagnostic assessment of congenital tracheal stenosis does not always closely correlate with clinical status, especially in asymptomatic children. Clinical decision making is often difficult in these circumstances. Computational fluid dynamics (CFD), in which computed tomography scans are reconstructed to map dynamic flow in the body, has shown promise as a tool for assessing airflow in children with congenital tracheal stenosis when traditional imaging modalities are limited. We sought to establish proof of this concept by applying CFD to congenital tracheal stenosis. METHODS: A retrospective single-center cohort study was performed for patients with congenital tracheal stenosis treated with slide tracheoplasty. A total of 9 children (age 3 months-12 years; weight 3-42 kg) with tracheal stenosis were assessed, including 2 with isolated stenosis, 2 with stenosis with a left pulmonary artery sling, 2 with stenosis with tracheal bronchus, and 3 with stenosis with single lung. Preoperative and postoperative computed tomography scans were obtained retrospectively and then used to construct 3-dimensional tracheal models and assess airflow using CFD. The 3-dimensional tracheal geometries were segmented to extract centerlines and prepare inlet/outlet planes, a steady velocity was imposed at the inlet, and zero pressure was imposed at the outlets. RESULTS: CFD simulations were successfully run to visualize velocity and pressure fields, which in turn were used to calculate pressure gradients across areas of obstruction and energy loss in the system. Postrepair velocities at the areas of maximum obstruction were lower than pre-repair velocities at the mid-trachea or carina for all 9 patients. Higher velocity, lower pressure, and increased wall stress corresponded to areas of stenosis across CFD simulations. CONCLUSIONS: Clinicians have limited functional data to inform decision making in the surgical repair of congenitally malformed tracheas. CFD using our methodology can virtually simulate flow in pre-repair and postrepair scenarios in several different malformations. The results of this study support CFD as a promising method for objectively assessing fluid dynamic metrics in congenital tracheal stenosis and correlating with clinical presentation and prognosis.

Partial anomalous left pulmonary artery sling is an exceptionally rare congenital anomaly, and its coexistence with ventricular septal defect and right lung agenesis has not been reported. We describe a neonate presenting with right lung agenesis, a large ventricular septal defect, partial anomalous left pulmonary artery sling, and congenital tracheal stenosis. Following surgical repair of the ventricular septal defect at 73 days of age, the patient showed marked improvement in respiratory status and was discharged home without respiratory support.