VACTERL Association

Airway below the cords - The airway problem in VACTERL sits distal to the larynx. Tracheomalacia and bronchomalacia occur in 10% to 20% of infants with tracheoesophageal fistula and can produce life-threatening airway obstruction and failure to wean from the ventilator. All infants get laryngoscopy and rigid bronchoscopy before an open repair, which identifies the fistula level plus tracheomalacia, posterior laryngeal cleft, laryngomalacia, and vocal cord dysfunction — read that report before you induce, because it also tells you where the fistula sits relative to the carina.

Cardiac lesion - Congenital heart disease accompanies tracheoesophageal fistula in about 32% of patients. A ventricular septal defect is commonest (22% in combination with other defects, 7% isolated); cyanotic disease is uncommon at about 4.5% and is essentially all tetralogy of Fallot. The preoperative echocardiogram is not optional — it also identifies a right-sided aortic arch, present in 2.5%, which raises morbidity and moves the thoracotomy to the left side.

Vertebral anomalies and neuraxial - Vertebral defects appear in about 24% and bring rib anomalies and tethered cord with them. Spinal ultrasound is part of the standard VACTERL workup; look at it before planning a caudal or any neuraxial technique, and before positioning for a lateral thoracotomy. Axial spine abnormalities are also associated with a higher esophageal anastomotic leak rate, thought to reflect a wider gap and more tension at the anastomosis.

Vascular access - Radial ray and other limb lesions restrict where a peripheral IV or an arterial line will go. Map the usable limbs and plan the arterial site before the patient is prepped and draped, not after.

Drug handling - Renal and distal urinary tract anomalies are part of the definition of the association, so confirm the renal ultrasound and a baseline creatinine before dosing renally cleared drugs or NSAIDs. Low birth weight is one of the two variables in the Spitz risk classification alongside cardiac disease — survival is about 97% in infants without major cardiac anomalies but falls to 22% when low birth weight and cardiac anomalies coexist. Dose to actual weight and expect immature clearance.

Aspiration before fistula ligation - Until the fistula is ligated this is a stomach that refills through the chest. Reflux of gastric contents up the fistula tract causes aspiration pneumonia, and gastric distension is the classic complication of a fistula between the trachea and the distal esophagus — the same route that lets ventilating gas preferentially fill the stomach. Keep the blind upper pouch on continuous suction, keep the head up, and keep airway pressures as low as the case allows until the fistula is controlled.

Anal or GI atresia - Atresia means a distended abdomen splinting the diaphragm and a neonate who is volume-depleted and electrolyte-deranged by the time the case is booked. Correct the deficit against the measured electrolytes before induction rather than chasing it afterwards.

Postoperative respiratory course - Recurrent bronchitis and pneumonia affect up to two-thirds of these children in the first few years, wheezing persists in about 40% of survivors and does not improve with age, and abnormal pulmonary function is found in up to 40%. Late mortality after repair is respiratory, not surgical. Plan for an airway that stays instrumented longer than the operation would suggest, and expect reflux — an expert panel recommends a proton pump inhibitor for at least a year after repair.

After the repair - Do not blindly pass or reposition a nasogastric tube after an esophageal anastomosis; the surgeon places it and it stays where it is put.

VACTERL is a non-random cluster of congenital anomalies rather than a single-gene disease. The complex refers to anomalies of the vertebrae (V), anal or gastrointestinal tract atresia (A), congenital cardiac defects (C), tracheoesophageal defects (TE), renal and distal urinary tract anomalies (R), and limb lesions (L). Sonic hedgehog (SHH) signaling is implicated — animals with disordered SHH regulation reproduce the pattern. Perioperatively it is the C and the TE that kill. Among infants with tracheoesophageal fistula, congenital heart disease is present in about 32% once patent foramen ovale and patent ductus are excluded, and mortality with an associated cardiac lesion is 42% versus 12% without. Vertebral anomalies appear in about 24% and rarely travel alone. Finding one component anomaly is the reason to hunt for the rest.