Abstract Introduction VACTERL is an uncommon association of congenital anomalies that stands for vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula, renal anomalies, and limb abnormalities. In addition to these classical features, patients may present with airway abnormalities such as bronchial stenosis or bronchomalacia, which can lead to significant respiratory compromise. Severe bronchial stenosis is usually treated with open airway reconstruction or lung transplantation; however, this is associated with substantial risks, especially in patients with complicated congenital conditions. Interventional bronchoscopic techniques, such as paclitaxel-coated balloon dilatation, allow a less invasive alternative for restoring airway patency and reducing restenosis. We describe the case of a patient with VACTERL association and severe bronchial stenosis successfully treated with serial paclitaxel-coated balloon dilatations, avoiding the need for lung transplantation. Case description A 27-year-old male with VACTERL association presented with symptomatic left mainstem bronchial stenosis and complete right lung collapse. Bronchoscopic intervention was performed over lung transplantation because of the patient’s young age and high surgical risk. It revealed complete fibrotic obstruction of the right main bronchus and proximal stenosis of the left mainstem bronchus, measuring approximately 5-6 mm in diameter. Performing paclitaxel-coated balloon dilation showed progressive airway enlargement and clinical improvement. Under bronchoscopic guidance using a drug-coated balloon (diameter 8-10 mm, length 40 mm), which was placed in the narrowed segment and inflated for 2 minutes to expand the airway and deliver paclitaxel locally, thereby inhibiting cell proliferation and scar tissue formation. Subsequent follow-ups showed that the left mainstem bronchus diameter increased to 8-9 mm, with improvement in the airway patency and respiratory function, without any significant complications. Discussion Although the use of paclitaxel-coated balloon dilation for bronchial stenosis is still limited, this case illustrates its potential benefits as an adjunctive interventional therapy in patients with VACTERL association. The serial dilatations resulted in a progressive, sustained increase in airway diameter, from an initial 5-6 mm to approximately 8-9 mm at final follow-up in our patient (Figure 1). Compared with lung transplant, this intervention offers lower complication rates and the avoidance of chronic graft rejection and immunological complications, which are particularly relevant for young patients. This case report shows the potential of paclitaxel-coated balloon dilation as a safe, effective, and minimally invasive therapeutic option for managing bronchial stenosis in patients with complicated congenital disorders. This abstract is funded by: None
Zapata et al. (Fri,) studied this question.