Is it likely that there are other anomalies associated with the TEF/EA? What anomalies are associated with this disorder? What additional tests or information would be critical to obtain? Why?

Of what importance is pulmonary insufficiency on the conduct of this case?

How would you counsel the parents of this child about the likelihood of further operations? Is this likely to be the only operation or procedure this child needs? Are there likely to be more procedures in the future – if so, what might they be and how might you predict this?

TEF/EA has an incidence of 1:2,500–3,000 and is associated with other congenital anomalies in 50 % of cases. The most common form of the EA/TEF anomaly is esophageal atresia associated with a distal fistula (C/IIIb). The diagnosis is rarely made prenatally but may be suspected due to the associated findings such as polyhydramnios and an absent or small stomach bubble. Cardiac anomalies occur in 29 % of EA/TEF patients including ventricular septal defect (VSD), patent ductus arteriosus (PDA), tetralogy of Fallot (TOF), atrial septal defect (ASD), and right-sided aortic arch. Gastrointestinal anomalies including duodenal atresia, imperforate anus, intestinal malrotation, pyloric stenosis, and omphalocele are associated. Genitourinary issues including renal agenesis, hypospadias, horseshoe/polycystic kidney, and ureteral/urethral abnormalities occur in 10 %. Musculoskeletal anomalies including radial limb abnormalities, polydactyly, lower limb defects, hemivertebrae, rib defects, and scoliosis are found in 10 % as is the confluence of these findings designated as VACTERL syndrome. Overall, genetic syndromes such as trisomy 21, trisomy 18, and 12Q deletion are present in 4 %. An echocardiogram and ECG are important to obtain prior to starting anesthesia. An abdominal ultrasound to determine renal anatomy is also helpful as is a plain film of the chest and abdomen, which would reveal vertebral anomalies. Presence of air below the diaphragm in any of these tests confirms the presence of a fistula in addition to the esophageal atresia.

The presence of pulmonary insufficiency could be caused by prematurity (30 % of EA/TEF patients are premature) or aspiration due to the fistula itself. Most often these patients are actually doing well on room air when you meet them. If this is the case, induction and intubation usually proceed without incident although there are specific issues to be addressed. Poor lung compliance changes this situation significantly. Poor compliance increases the chance that air will be forced into the stomach on mask ventilation and will increase gastric distension which in turn will make ventilation much more difficult. Any positive pressure ventilation (by mask or ET tube) that occurs without isolation of the fistula is very likely to continue to distend the stomach in these cases.

The prognosis for further procedures in patients with T-E fistula is such that one would predict the majority will need several further procedures. The exact likelihood depends on the nature of the fistula and the trachea prior to surgery. Longer “gaps” between the segments of atretic esophagus portend more procedures. Patients with particularly “long gap” esophageal atresia cannot be primarily repaired and require staged procedures. Even patients with relatively favorable “shorter” gaps frequently require dilations of the esophagus where the anastomosis was made. When the anomaly occurs in patients with VACTERL syndrome, it is assured that the patient will need many surgeries during childhood and parents should be given appropriate counseling prior to beginning this process.

How would you proceed with the induction of anesthesia? Why would you choose this technique? What is the advantage of keeping the child breathing spontaneously vs. controlling ventilation?

What monitors are required for this procedure? Is a central venous catheter required?

What will your maintenance of anesthesia technique be? Why? Will you use nitrous oxide? Why/why not? What is your choice of muscle relaxant? Why?

During surgery, the patient’s SpO₂ suddenly decreases to 82 % from 99 % and the inspiratory pressure increases from 25 to 45 cm H₂O. What do you think is going on? What would you do? Why? How would you manage her depth of anesthesia? Why? How can her inhaler be administered intraoperatively? How much would you administer through the endotracheal tube? Why?