B60-12 Optimizing Respiratory Support in Congenital Rib Agenesis - A Case of Early Tracheostomy and Mechanical Ventilation

Abstract Introduction Congenital absence of the ribs is a rare developmental anomaly that may occur in isolation or as part of a broader syndromic condition. The absence or malformation of ribs reduces chest wall compliance resulting in impaired ventilatory mechanics, and, in severe cases, chronic respiratory failure requiring ventilatory support. We present the case of an infant born with extensive rib agenesis and thoracic insufficiency, which was managed with early tracheostomy and mechanical ventilation to promote growth and respiratory stability until definitive corrective surgery could be performed. Clinical Vignette A full-term male infant with known thoracic scoliosis and associated vertebral and rib abnormalities was born with hypoxemia and respiratory distress. Initial management with continuous positive airway pressure (CPAP) and supplemental oxygen were insufficient so he promptly required intubation. Physical examination revealed right-sided flail chest. Imaging demonstrated marked thoracic asymmetry with multiple hypoplastic and/or fused ribs on the right side (Figure 1).He was extubated to CPAP at 6 days of life without respiratory acidosis but had persistent tachypnea and failure to thrive. Due to continued poor growth despite bilevel positive airway pressure, he underwent tracheostomy at 23 days of life. His tracheostomy was uneventful, and subsequent imaging showed improved expansion of the right lung with mechanical ventilation. His post-tracheostomy course was complicated by tracheitis and viral infections requiring transient high frequency oscillatory ventilation. Despite this, he has demonstrated excellent weight gain and is now approaching the 50th percentile. Discussion Our case highlights the importance of individualized respiratory management in patients with congenital chest wall anomalies, who often face significant challenges related to altered lung mechanics and increased work of breathing. In this patient, early tracheostomy with mechanical ventilation was instrumental in stabilizing his respiratory status, supporting weight gain and stamina. His chest imaging demonstrated improved lung inflation and reduced atelectasis, suggesting that the mechanical ventilation enhanced alveolar recruitment and improved ventilation-perfusion matching, as well as improved growth. Compared with CPAP, the tracheostomy significantly decreased his work of breathing, which likely reduced his caloric expenditure and allowed for effective growth. Our case illustrates that early tracheostomy with mechanical ventilation can serve as an effective bridge to definitive surgical repair by improving pulmonary mechanics, optimizing nutritional status, and supporting the growth and development of infants with thoracic insufficiency even without initial respiratory acidosis. This abstract is funded by: None