Bronchial anomalies in VACTERL association

Abstract

VACTERL association is an acronym made of associated defects including vertebral anomalies, anal atresia, cardiac, tracheal-esophageal fistula, and renal/radial limb anomalies. Tracheal bronchus is a condition characterized by ectopic location of the right upper lobe bronchus at the mid to distal trachea. This condition is associated with congenital anomalies and has been reported in one previous case of VACTERL. We report another infant with VACTERL presenting with respiratory complications due to presence of tracheal bronchus. She also had a narrowed segment of her right main stem bronchus.

Congenital Trifurcation of the Trachea

“…The relationship between esophageal atresia and tracheal bronchus has been demonstrated in the Adriamycin rat model, in which approximately one-fifth had a tracheal bronchus. 7 This corresponds with case reports of VACTERL children with tracheobronchial malformations 8 and highlights the need for comprehensive evaluation of the tracheobronchial tree in esophageal atresia before repair is undertaken. 9 Although it is suspected that these malformations may carry an increased malignant risk this has not been quantified in the literature.…”

VACTERL association complicated with multiple airway abnormalities

“…A few airway anomalies have been reported with VACTERL association including tracheal bronchus, ectopic apical segment of right upper lobe bronchus arising from the proximal right main bronchus or from the esophagus, BB, tracheal stenosis, horseshoe lung, and pulmonary agenesis. [9–11] Our patient presented with multiple airway anomalies including BB, airway malacia, and congenital tracheal stenosis (CTS) which had never been reported before. These anomalies are commonly associated with recurrent pneumonia and stridor.…”

“…This highlights the need to completely evaluate the tracheobronchial pulmonary system in individuals with VACTERL association. [9] Bronchoscopy allows direct visualization of airway anomalies. With respect to some airway abnormalities, such as tracheomalacia and cartilage rings, bronchoscopy persists as the “gold” standard method of diagnosis.…”

Spanish multicentre study on morbidity and pathogenicity of tracheal bronchus in children

“…From an embryological standpoint, TRB develops between the 3rd and 7th week of gestation from an anomalous tracheal sprout which does not undergo regression or through implantation of bronchial mesenchyma within the trachea . It is usually associated with a wide variety of congenital anomalies or syndromes . As far as we know, this series is the most extensively described to date since most of the published data on TRB corresponds to case reports or small series of patients .…”