A new approach for the management of esophageal atresia without tracheo-esophageal fistula.

Long gap esophageal atresia (OA) is a challenging condition. While discussing the various methods of management available to us, we report the use of magnetic resonance imaging in a case of pure OA to judge the gap between two ends of the esophagus.

INTRODUCTION

Esophageal atresia (OA) with and without trachea-esophageal fistula (΁TOF) is a common group of anomalies which are evenly distributed worldwide with an incidence of one in 2500-3000 live births.[1] Many classifications of this anomaly by various authors[1234] are available. We are following the most comprehensive classification by Gross,[4] according to which OA without TOF is classified as Type A. Its incidence is 7% of all OAs; yet it is the second most common form of OA. The most common being Type C (86%).

OA Type A has a wider gap between the two ends of the esophagus as compared to OA Type C. A gap of more than four vertebral bodies by some[5] and more than six vertebral bodies by others,[1] that is, ≥5 cm is considered a wide gap. Various approaches have been utilized to solve this life-threatening problem. Currently, the approaches available to us are primary repair, delayed primary repair, or esophageal replacement. Primary repair is the ideal situation where the native esophagus is retained. This is possible if the gap can be covered by esophageal dissection. In the event that the gap cannot be bridged, many centers[156] are now advocating delayed primary repair, allowing the esophageal ends to grow sufficiently to be brought together. This wait is carried out in hospital with regular upper pouch suction and a feeding gastrostomy to deliver nutrition. This usually takes about 3 months or when the child attains 4 kg weight.[15] Alternatively, a draining esophagostomy in the neck and a feeding gastrostomy will be made, and the child will be sent home to come later for an esophageal replacement surgery.

We report a case of OA Type A with a long upper pouch where we decided to see if we could judge the gap with magnetic resonance imaging (MRI) so as to facilitate taking a suitable decision for surgery.

MATERIALS AND METHODS

A full-term male child, weighing 2.75 kg and 4 h old presented with a diagnosis of OA made by the pediatrician attending the delivery. The plain X-ray [Figure 1] showed the nasogastric tube coiling in the upper esophageal pouch at the level of the 4th thoracic vertebra (D4). He had mild respiratory distress with pneumonia. Within 24 h, the pneumonia started responding to throat suction, antibiotics, and chest physiotherapy. The child was fit for surgery on the 3rd day of life. The upper pouch was as low as D4 and we felt that if the lower end could be visualized and the gap distance quantified, we could then take the right decision on whether to attempt a primary repair or proceed with esophagostomy and gastrostomy. We decided to do an MRI of the chest to see if the lower end of the esophagus could be identified in the mediastinum.

Figure 1

Chest X-ray showing a feeding tube coiling in the upper esophageal pouch, reaching as low as the fourth thoracic vertebra (black arrow). There is absence of gas in the abdomen and signs of pneumonia on both lungs

The machine used is a 1.5 Tesla GE Signa Excite MRI. A sagittal T2-weighted image showed a dilated proximal esophageal pouch with air-fluid level within it and its lower margin ending at the upper border of D4 vertebra [Figure 2]. The distal blind pouch could be traced in the mediastinum and its upper margin was at the upper border of D8 vertebra. The distance between the two ends of the esophagus was reported to be 2.6 cm. We proceeded with a primary repair of the esophagus through the right thoracotomy via 4th intercostal space using extrapleural approach. The gap was measured and it was 2.5 cm long. Postoperative recovery was good and the child went home on the 8th postoperative day. He has been in follow-up for over 2 years now and is thriving.

Figure 2

Magnetic resonance imaging of the chest showing a sagittal view of the mediastinum labeling the upper and lower esophageal pouches with a line (marked 1) between the two ends measuring 2.6 cm

DISCUSSION

In the earlier days, various methods were devised and used to cover the long gap in OAs. Circular myotomies of the upper pouch to lengthen it and tubularization of the upper pouch after creating a flap were the commonly reported techniques used.[1] Both techniques are fraught with possible complications such as inability to get enough length, not enough width of the upper pouch to form a flap and tubularize it, mucosal injury, and anastomotic leaks as well as dysmotility and stricture formation. In developed countries today, most centers are doing delayed primary repair of the esophagus as it is now clear that the native esophagus is a better option than replacements. It is a prerequisite to primary repair that an esophagostomy should be avoided. This improves the chances of growth of the upper end in the right direction. Growth of both ends allows delayed primary repair of the esophagus. To assist this event, many centers were, at first, doing gentle bougieing of both ends with the idea of stretching and stimulating growth. Most centers now wait for 3 months or till the child is grown to 4 kg, whichever happens first, and then assess the gap for a delayed primary repair.

Assessing the gap requires insertion of radio-opaque tubing or bougie at both ends of the esophagus and taking a radiograph or seeing by fluoroscopy and measuring the distance between the two ends. This is only possible after performing a gastrotomy for the lower end of the esophagus. As assessment is required at regular intervals, the feeding gastrostomy tube is removed each time and reinserted after the procedure. Gentle pressure is applied at the point of resistance on the bougies, and the distance between the two ends is measured. If the gap is more than 5 cm after 3 months, then it is unlikely that it will reduce further in time and an esophageal replacement can be planned. If the gap is <5 cm, a primary repair is attempted. If the gap is at a borderline of 4-5 cm and the surgeon feels unhappy about the proceeding, he can wait a while longer for the gap to reduce. The procedure is repeated and a decision will be taken.

During this period of waiting for the gap to reduce with growth of the two ends, it is imperative that the child's nutrition is maintained via gastrostomy feeds and that the child is protected from aspiration of secretions which can cause pneumonia or sudden respiratory distress leading to cardio-respiratory arrest. Pneumonia can also lead to septicemia, which can be life-threatening. There is an added danger of hospital-acquired infections, which are often multi-drug resistant. Keeping the upper pouch dry of secretions and maintaining the child on gastrostomy feeds require skilled personnel, necessary equipment, and sterile conditions. An attending nurse is required round the clock. The child remains in hospital while waiting for the assessment of esophageal growth. This puts a huge burden of expense on young parents, and insurance companies in India do not cover congenital malformations. If the parents are able to afford the huge expense, the hospitals are overburdened with patient load; under staffed and under equipped to cope with that load. Resources cannot be focused on one child alone at a given point of time. Breakthrough pneumonias will require treatment and possibly ventilation. The risk of sepsis remains at all times.

On the other hand, if esophagostomy is done in all infants with a gastrostomy for pure OA, there would be a certain number of infants who did not have a long gap and in whom a primary repair was possible. There are a definite number of infants in whom the native esophagus can be retained, but is being sacrificed. The cost of an MRI is much less than the phenomenal cost of waiting in hospital for a long period of time. We measured a difference of 1 mm suggesting that the MRI was very accurate and should be a mandatory investigation in all cases of OA without TOF so that an early decision can be taken regarding the possibility of a primary repair.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.