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[Ring-sling complex association with pig bronchus: a pediatric case report].

María L Bergamini1, María C Martínez Riccetti2, Juan A Kiang3

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Summary

The ring-sling complex, a rare association of pulmonary artery sling and congenital tracheal stenosis, presents diagnostic challenges. This case highlights the importance of comprehensive imaging and endoscopy for timely diagnosis and surgical repair of these congenital anomalies.

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Area of Science:

  • Cardiology
  • Pediatric Surgery
  • Medical Imaging

Background:

  • Pulmonary artery sling is a rare congenital heart defect involving an aberrant left pulmonary artery originating from the right pulmonary artery.
  • Congenital tracheal stenosis is a rare airway malformation characterized by a narrowed trachea.
  • The ring-sling complex represents an association between these two conditions, posing significant diagnostic and therapeutic challenges.

Observation:

  • A pediatric patient presented with inspiratory stridor and respiratory distress.
  • Initial evaluation revealed extrinsic tracheal compression, suspected to be a vascular ring.
  • CT angiography confirmed pulmonary artery sling, while subsequent surgical endoscopy identified congenital tracheal stenosis and a right accessory bronchus.

Findings:

  • The patient was diagnosed with the ring-sling complex, encompassing pulmonary artery sling, congenital tracheal stenosis, and a right accessory bronchus ('pig bronchus').
  • Surgical intervention successfully repaired all identified congenital anomalies, including the vascular ring and tracheal stenosis.

Implications:

  • This case underscores the critical role of integrated diagnostic approaches, including advanced imaging and direct airway visualization, for managing complex congenital anomalies.
  • Simultaneous surgical repair of pulmonary artery sling and congenital tracheal stenosis is feasible and effective.
  • Early and accurate diagnosis of the ring-sling complex is essential for preventing severe respiratory complications in affected children.