Esophageal atresia (EA) is one of the most common life-threatening congenital anomalies of the newborn and occurs in 2.44 in 10,000 births. The current survival rate of EA with Tracheoesophageal fistula (TEF) in term neonates with birth weight more than >2500 gm - normal birth weight (NBW) and >1500 g to <2500 g - low birth weight (LBW), without major congenital cardiac anomaly, is 95% and can be achieved with early primary repair.^[1] The very low birth weight (VLBW) babies (>1000 g to <1500 g) had poor survival with a mortality ranging from 100% in the early fifties to 20% in the late nineties which was attributed to intrauterine growth retardation, respiratory distress syndrome (RDS), associated anomalies, cardio-pulmonary vulnerability, necrotizing enterocolitis, intracranial hemorrhage, and immature immune system.^[2] Since the late nineties, the survival in this group has increased to 87%.^[3] VLBW babies with EA are more commonly associated with isolated EA (10%) than EA with TEF. VLBW babies with isolated EA have better survival. VLBW babies with EA and TEF are a special group because of the intrauterine growth retardation, related RDS, the risk of gross air-leak through the fistula into the stomach, and its complication- the gastric perforation, which carries a mortality rate as high as 70%.^[2] The iatrogenic injuries caused during the preoperative period of management in preterm babies with TEF are uncommon, life-threatening, under-recognized, and had poor survival. This report is about a baby belonging to such a special group resulting in a gross air leak and cardiovascular collapse requiring repositioning of the endotracheal tube (ETT). Several attempts to replace ETT, suctioning, and replacement of Replogle tube resulted in complex tracheoesophageal trauma and pneumothorax.

A preterm female baby born by emergency LSCS for decreased fetal movements at 32 weeks weighing 1.1 kg with EA/TEF was transported on ventilatory support to our institution at three days of age. The baby developed poor chest rise, desaturation, and bradycardia due to a large air leak into the stomach through the fistula. Initial attempts by adjusting the position of the ETT failed. Further attempts by changing the ETT and subsequently with a large volume low pressure cuffed tube as well as replacing the Replogle tube ended up in pneumothorax (Fig. 1). Intercostal drainage (ICD) was inserted, and the baby was shifted to OR for emergency surgery. Preoperative laryngo-tracheoscopy (PLT) showed a fistulous opening at the usual site proximal to the carina. The opening was wide and the edges were irregular suggestive of trauma (Fig.2a,2b & 2c). A Fogarty catheter was introduced into the fistulous opening and the balloon was placed in the stomach, which was verified by fluoroscopy (Fig. 2d). PLT with a cystoscope with an offset lens and placement of Fogarty catheter has been routinely carried out by us for the last five years.[4] Right posterolateral thoracotomy by trans-pleural approach was carried out. The feeding tube passed into the upper pouch at the time of induction replacing the Replogle tube had come out through a tear in the upper pouch into the pleural cavity. The fistula was torn along its entire length except for a sliver of tissue medially holding the trachea and the lower pouch exposing the Fogarty catheter. The endotracheal tube could be seen through a tear extending into the trachea from the proximal end of the fistula (Fig. 3a). After a careful dissection with the help of the exposed stent which was seen bridging across the tracheal end of the fistula and lower pouch through the torn fistulous tract, the remaining fistulous tract was disconnected from the trachea, and the lower pouch and was excised. The fistulous opening and the adjacent tear on the trachea were closed. The torn tissues of the lower end of the upper pouch were trimmed. The anastomosis of the upper and lower pouches was carried out with slight tension (Fig. 3b). To tide over the tension at the anastomosis, a trans anastomotic nasogastric 8F Silastic Foley balloon catheter was placed as a stent. The balloon inside the stomach was inflated and gentle traction was given at the nasal end of the Foley catheter till a slackness was felt at the anastomosis and the catheter was fixed to the nose. This technique to reduce the tension at the anastomosis has been used by us for the last 10 years.^[5] There was no leak in the postoperative contrast esophagogram (Fig. 3c). The baby required prolonged CMV and TPN. The baby was discharged at the age of 4 months with adequate weight (2.94 kg) for the age and normal oral feeding and is doing well 14 months after the surgery.

The survival of VLBW babies has improved in recent years. This improved survival is related to the consideration of early primary repair in stable cases and delayed esophageal anastomosis or staged surgery in unstable cases, availability of endotracheal intubation, and advances in neonatal, respiratory, surgical, and anesthetic care. There are controversies in the method of management of the VLBW babies with EA and TEF between primary repair and delayed primary anastomosis and staged surgery among the institutions.^{[2], [3], [6]}

VLBW babies with EA with TEF are a special group and may require methods of surgical management other than the primary anastomosis which was first performed by Haight in 1943. This group is considered special because of the intrauterine growth retardation, related RDS, and the risk of progressive atelectasis, loss of functional residual capacity, increased pulmonary vascular resistance, and frequent requirement of continuous mechanical ventilation (CMV). High pressures in the pulmonary parenchyma and the relatively lower pressure of the fistula creates a ventilatory leak through the TEF. This worsens the respiratory distress secondary to the loss of ventilation and resultant gastric distension leading to cardiovascular collapse and gastric perforation which carries a mortality rate as high as 70%.^{[2], [7]} The aggressive non-surgical methods adopted to tackle the air leak through the fistula into the stomach before surgical correction are positioning of ETT tip distal to the fistulous opening, positioning with affected side down, placement of Fogarty catheter either by bronchoscopy or by laparotomy to occlude the fistula with the balloon, banding of the esophagus, gastric division, needle paracentesis of the abdomen, high-frequency ventilation, ECMO, and gastrostomy with underwater seal. The results of these procedures are unreliable, less consistent, and time taking than the emergency fistula ligation which gives an opportunity to carry out primary repair also.^[7] Isolated iatrogenic tracheal or bronchial injury has been reported in 15 neonates.^[6] Death usually occurs at the time of injury but in rare cases where the baby survives, the conservative methods showed higher mortality (88%) than that for the surgical treatment (55%).^[7] Thirty-six cases of GI perforations related to air leak published since 1980, shows overall mortality of 50%.^{[7], [8], [10]} Iatrogenic injuries caused during the management of this special group of VLBW babies with TEF had brought poor survival in the past (Table. 1). ^{[9], [10], [11], [12], [13], [14]} The sites of injury in these 5 cases were the upper pouch in 3, posterior pharyngeal wall in 1, and trachea at the level of fistula in 1. The case presented had a complex tracheoesophageal injury involving the upper pouch, trachea, and fistula which has not been published. Aggressive non-surgical management of air-leak resulted in this injury. Emergency thoracotomy, repair of the injury gave an opportunity to carry out a primary repair. The baby survived the prolonged ventilatory support required for the lung hypoplasia of prematurity. Such special groups of VLBW babies with TEF are advised to be managed on the following lines. 1. Emergency thoracotomy and primary repair before the complications of RDS sets in. 2. If the complications set in very early after birth, emergency thoracotomy, fistula ligation, and primary anastomosis should be attempted and 3. In cases unstable after the fistula ligation, delayed primary anastomosis or staged repair can be planned.