Long-Gap Esophageal Atresia

Abstract

Esophageal atresia is a rare congenital anomaly that occurs in 1 in 4,500 live births. The expected outcome is close to 100% survival, though this varies depending on birth weight, degree of prematurity, and associated anomalies (especially cardiac). Ideal surgical management consists of a primary end-to-end anastomosis between the upper and the lower esophageal remnants and division of a tracheo-esophageal fistula, if one is present. The vast majority can be corrected without difficulty soon after birth. However, this goal is not always easily achievable with all anatomical variants and the management of long-gap esophageal atresia remains a major challenge to the pediatric surgeon. In addition, there is controversy regarding the definition of a “long gap” and a general lack of consensus regarding which is the best technique. This is perhaps due to the fact that attempts to bridge the gap to allow a delayed anastomosis have led to the introduction of several interesting techniques, none of which are perfect.

Appendices

Summary Points

What constitutes a “long gap” has always been an arbitrary concept: almost all (but not all) type A and B and some type C and D esophageal atresia have a long gap between the two esophageal stumps.

Preserving the native esophagus is almost always possible when the surgeon is committed to this goal and a well defined protocol is followed.

In patients with esophageal atresia with and without fistula, it is important to measure the gap accurately before an operation is undertaken.

Radiographs taken with a radiopaque feeding tube inserted deeply in the upper pouch and a sound or bougie passed into the distal esophagus through the gastrostomy should allow accurate assessment of the distance between the two ends.

Tracheoscopy should always be performed to rule out proximal fistula. It can also be used in combination with fluoroscopy to help define the gap.

With a long-gap esophageal atresia (≥3 vertebral bodies), experienced senior staff should be alerted or the patient referred to a tertiary care center institution.

In types A and B, always delay the primary anastomosis 4–6 weeks (even more if necessary) to allow growth of the hypoplastic distal pouch.

A gastrostomy is useful to immediately start feeding the patient, induce the growth of the lower esophagus and stomach, and allow serial measurement of the gap.

In patients referred after a failed attempt at primary anastomosis, always rigorously measure the gap to define the possibility of a further attempt at esophageal anastomosis.

Esophageal substitution is indicated only if primary anastomosis has proven impossible.

Regardless of the technique used to bridge the gap, patients with long-gap esophageal atresia experience a higher incidence and severity of early and late complications, but this is considered an acceptable trade-off if the native esophagus can be preserved.

Strict long term follow-up of these patients is mandatory.

Editor’s Comment

The take-home message here should clearly be that retaining the native esophagus, be it imperfect or patently flawed, is almost always preferable to any of the various esophageal substitutes that have been described over the years. With experience, meticulous patience and good judgment, we should be able to achieve this goal in the vast majority of patients with esophageal atresia no matter the length of the gap. In most cases, the critical element is time: the esophageal ends will grow, but in some cases this can take many months. There should be no rush to get into the chest to try and bridge a long-gap unless the plan has been thought out very carefully and the advice of experienced pediatric surgeons has been sought.

I look forward to the day when another treasured staple of pediatric surgical history, the cervical esophagostomy, has become a banished relic of a bygone era. This gruesome spectacle of an operation generally precludes salvage of the native esophagus and should only be performed under extraordinary circumstances, never as a primary therapeutic maneuver. The alternative is to control secretions by maintaining a suction cannula in the proximal esophageal pouch for weeks or sometimes months, which usually makes it impossible for the infant to be discharged to home but in the long run is a small price to pay for saving the esophagus.

There are many tricks that have been described to help bridge a long gap. I have found that maximally flexing the neck (chin to chest) helps a great deal. It is also usually safe to mobilize the distal esophagus (and stomach) but this needs to be done carefully so as to preserve the blood supply. Likewise, up to three circumferential myotomies may be performed on the upper pouch, providing a significant amount of length. A common mistake is to begin tying the anastomotic sutures one by one, rather than parachuting them down simultaneously to distribute the tension more evenly. This commonly results in stitches being torn out and the loss of a centimeter or more of esophageal length. I use ringed bowel forceps to grasp each esophageal stump just above and below the respective suture lines and push the ends together so that the entire posterior row of sutures can be tied down under no tension. Silk and other nonabsorbable suture material should not be used on the esophagus because of the risk of foreign body reactions and fistulas, especially since some of the knots usually need to be placed within the lumen. I usually place a small nasogastric tube through the anastomosis to allow gastric decompression and then enteral feeds in the postoperative period, though a tube that is too large can create radial tension and ischemia at the anastomosis, leading to breakdown or stricture. Finally, the technique of gradual lengthening by use of external traction sutures popularized by Foker is clearly a major advance in the field, but I have also seen it fail miserably in inexperienced hands – it is not as easy as it seems and should be guided by someone with direct experience in the technique.

Differential Diagnosis

• Laringotracheal or laryngo-bronchial cleft

Diagnostic Studies

• Chest and abdominal radiographs

• Cardiac ultrasound

• Bronchoscopy under fluoroscopy, as the last diagnostic step before starting operation

• Serial gap measurement (measured in vertebral bodies) with rigid instruments, with and without pressure (repeat every 2 weeks)

Parental Preparation

This procedure carries a much higher risk when compared to EA without a long gap.

Multiple-step or delayed procedures might be necessary, depending on the type of EA and whether repair has been attempted previously.

Several refinements have become available, which seem to have improved the success rate of the technique.

After repair, expect a long period (at least 6 days) of sedation, paralyzation, and mechanical ventilation.

Final repair might be delayed even longer (up to 14 days) when an extrathoracic esophageal elongation technique is utilized.

There is the possibility that subsequent esophageal substitution will become necessary if primary repair is not achievable.

Most patients have a good quality of life despite an early stormy course.

Preoperative Preparation

• Chest and abdominal radiographs visualizing the ends of esophageal stumps (even to exactly define the best intercostal space for operative approach)

• Cardiac ultrasound confirming left aortic arch

• Continuous suction of the upper pouch

• Before delayed primary repair: rigorous definition/visualization the true gap length

• Type and crossmatch

• Informed consent

Technical Points

Tracheoscopy first: to rule out proximal fistula, to define the gap even in type C and D, and to exclude wide laringo-tracheo-esophageal cleft.

Measure the gap precisely, both with and without tension before deciding the timing of surgical procedure.

Always avoid primary cervical esophagostomy (waste of time and esophageal length).

Choose the best intercostal space for thoracotomy (looking at “gapogram” and the vertebral body where the gap falls), use a muscle-splitting technique, use a retropleural approach when possible, preferable through e subperiosteal approach.

Use magnification.

Don’t try to perform the anastomosis immediately; perform a gastrostomy and close proximal fistula (type B). Define initial gap and re-measure it every 15 days.

Gap <3 vertebral bodies:

• Ready to do the anastomosis, alert senior staff always.

• At anastomosis, use a Hegar dilator (4–5 mm) trough the gastrostomy into the lower esophageal stump to help intra-operative identification of the lower pouch.

• Always attempt primary anastomosis.

Gap ≥3 vertebral bodies:

• Alert senior staff

• Close and divide the fistula

• Re-measure the gap rigorously

If a left esophagostomy has already been done, move it to the right neck (when right thoracotomy and esophageal re-anastomosis is considered possible and planned).

Handle tissues very gently: Use stay sutures on both esophageal ends; don’t clamp esophagus with forceps.

Mobilize both upper and lower esophageal segments extensively.

Verify the gap is bridged before opening either lower or upper esophageal pouch.

If the gap can be bridged, do the anastomosis.

If the gap is still “unbridgeable,” consider:

• Intra-operative traction on each end for 10 min

• Upper pouch sliding

• Further dissection of upper pouch through a cervical approach

• Upper esophageal flap (as small as possible)

• Esophageal lengthening with external traction and redo thoracotomy 6 days later

Doing the anastomosis:

• Posterior row with 3–4 sutures (5-0 or 6-0 polypropylene) placed without tying, and then tied simultaneously

• Remove chest bump

Drain the chest (even if extrapleural approach); avoid costal synostosis.

Maintain the patient sedated and paralyzed for at least 6 days.

Don’t move, rotate, or hyperextend the neck during patient’s transportation and for at least 6 days.

Check the chest drain daily to exclude drainage of saliva: white with foam instead of serous or seroanguinous.

Contrast study on post operative day 6–7, to rule out leak, before starting feeding.