ARRS 2022 Abstracts

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E1601. Congenital Esophageal Atresia and Stenosis: Associations and Complications
Authors
  1. Kristen Lee; Montefiore Medical Center
  2. Mark Messina; Montefiore Medical Center
  3. Jessica Kurian; Montefiore Medical Center
  4. Mark Liszewski; Montefiore Medical Center
  5. Einat Blumfield; Montefiore Medical Center
  6. Terry Levin; Montefiore Medical Center
Background
This exhibit aims to present the classification of esophageal atresia (EA) with common and unusual presentations of this entity as well as its associations. Additionally, we discuss congenital esophageal stenosis secondary to tracheobronchial remnants, which may be seen with EA, as well as tracheobronchial remnants resulting in an esophageal bronchus. The management of these entities and their potential complications, both common and unusual are shown.

Educational Goals / Teaching Points
In this exhibit, we describe the five types of EA (Gross classification and Vogt classification) and its embryogenesis using illustrations, plain radiographs, fluoroscopic studies, and endoscopic images. We also present commonly associated findings including the vertebral-anal-cardiac-tracheoesophageal-renal-limb (VACTERL) association and tracheomalacia anduncommon entities associated with EA, such as congenital esophageal stenosis due to ectopic tracheobronchial remnants in the distal esophagus. Delayed presentation of a congenital esophageal stenosis in association with a bronchus arising from the esophagus is demonstrated. Surgical repair of EA and common complications are presented including post-operative stricture, leak with or without abscess formation, and pneumothorax as well as less common complications such as an extra-pleural air leak resulting in mediastinal shift; long segment distal esophageal stenosis occurring following repair of long gap esophageal atresia without fistula; and esophageal foreign body due to poor distal esophageal motility.

Key Anatomic/Physiologic Issues and Imaging Findings/Techniques
Initial radiographs showing diagnostic chest and abdominal findings are presented. Spine and limb radiographs demonstrate skeletal findings such as vertebral anomalies and radial-ray anomalies. Upper gastrointestinal series is shown to demonstrate H-fistula in a patient with VACTERL as well as congenital esophageal stenosis due to a tracheobronchial remnant in the distal esophagus. A case of esophageal bronchus in association with congenital esophageal stenosis is also shown. Post operative radiographs and fluoroscopic images are provided in patients following repair of EA and tracheoesophageal fistula to demonstrate common and uncommon complications.

Conclusion
Congenital esophageal atresia may be readily recognized clinically and radiographically in the neonatal period. However, the diagnosis of congenital esophageal stenosis and H type tracheal esophageal fistula may be delayed. Awareness of common and uncommon associations and surgical complications of these entities is necessary.