VACTERL association complicated with multiple airway abnormalities

Abstract

Introduction:VACTERL association is an acronym that includes vertebral anomalies (V), anal atresia (A), cardiac defects (C), tracheoesophageal fistula (TEF) or esophageal atresia (EA), renal anomalies (R), and limb defects (L). Airway anomalies have rarely been reported with VACTERL association.Patient concerns:A 10-month-old boy who had been diagnosed with anal atresia and received surgical corrections soon after birth consulted our institution by complaining repeated cough and fever.Diagnosis:Diagnosis of VACTERL association was finally made. Bronchoscopy and chest CT with computed tomography angiography confirmed multiple airway abnormalities including bridging bronchus, airway malacia, and complete tracheal rings.Interventions:Supplemental oxygen was provided and antibiotics was initiated.Outcomes:The patient resolved gradually and was discharged 10 days later. The follow-up showed the patient has remained well just with mild psychomotor retardation.Conclusion:Multiple airway anomalies may be seen in VACTERL association. It is worthwhile to make special note for evaluating the tracheobronchial pulmonary system by chest CT and bronchoscopy, especially patients presenting with breathing anomalies.

VACTERL-associated bilateral bronchial stenosis with concomitant spinal muscular atrophy

“…Longer-term complications of TEF can include tracheomalacia, TEF recurrence, esophageal stricture, and gastroesophageal reflux disease. 9,10 Congenital tracheal stenosis 5 and bronchial stenosis 6 have been reported in two infants with VACTERL association. There should be a low threshold for investigation when patients with VACTERL association present with persistent or severe respiratory symptoms.…”

“…Tracheoesophageal fistula (TEF) occurs in 60-80% of children with VACTERL association. 3,4 In rare cases VACTERL has been complicated by severe bronchial stenosis 5,6 or unilateral pulmonary agenesis. 7 We report a child who developed episodes of oxygen desaturation during sleep.…”

Respiratory failure in a patient with VACTERL association and concomitant spinal muscular atrophy

“…Longer‐term complications of TEF can include tracheomalacia, TEF recurrence, esophageal stricture, and gastroesophageal reflux disease 6,7 . Congenital tracheal stenosis, 8 bronchial stenosis, 9 and pulmonary agenesis 10 have been reported in two infants with VACTERL association. There should be a low threshold for investigation when patients with VACTERL association present with persistent or severe respiratory symptoms.…”

The Importance of the Airway in Children Undergoing Surgery for Congenital Heart Disease

“…3,4 Clinically, there are many reports on the association of congenital and acquired airway abnormalities in children with CHD. [5][6][7][8][9][10] Not infrequently, children with congenital tracheal stenosis and congenital heart disease require simultaneous airway and cardiac surgical repair on cardiopulmonary bypass soon after birth. 11,12 Taking these factors into consideration, the first step in planning the delivery of safe general anesthesia for children with CHD is a detailed airway assessment.…”