eMedicine Specialties > Pediatrics: Surgery > General Surgery
Esophageal Atresia With or Without Tracheoesophageal Fistula: Treatment
Updated: Apr 30, 2008
Treatment
Medical Therapy
The preparation of a 1-day-old neonate for surgery includes the following measures:
- Intravenous fluid containing an adequate glucose concentration (ie, 10% glucose) is administered at a rate appropriate for the neonate's gestational age and weight.
- Prophylactic broad-spectrum antibiotics (eg, ampicillin, gentamicin) are intravenously administered.
- The neonate is kept warm by using an incubator or overhead warmer and is positioned supine in the Fowler position, with the head elevated by approximately 45°.
- A 10F Replogle tube is placed nasally or orally well into the upper pouch and is connected to a continuous suction device. Every 30 minutes, the tube is checked for patency first by suctioning with an empty syringe and then by gently injecting 5 mL of air. (Never use water.) In small infants, an 8F double-lumen tube may be used instead.
- The parents should be fully briefed about the nature of the congenital anomaly. A diagram is invaluable for explaining not only the pathologic anatomy and intended repair but also the possible complications. Their consent for treatment should be obtained, and the discussion with them should be documented in appropriate detail on the baby's medical record.
Surgical Therapy
This section provides some details about surgical approaches for the repair of the most common type of esophageal atresia (ie, esophageal atresia with distal tracheoesophageal fistula [TEF]) in low-risk patients. Surgical techniques vary according to surgeons' preferences and variations in pathologic anatomy. Modifications for special anatomic challenges are briefly discussed. In particular, infants born with esophageal atresia without fistula represent a specific and challenging subgroup. These babies should undergo an early gastrostomy procedure in the newborn period. (see discussion about delays in esophageal atresia repair in Relevant Anatomy). A gap-o-gram should be performed to assess the prospects for anastomotic repair.
In infants with atresia without fistula, surgical decisions must be made regarding the length of time to wait for the ends to grow closer; whether to perform one of numerous esophageal lengthening procedures such as the Kimura, Livaditis, Scharli, or Foker procedures; whether to perform an esophageal substitution procedure, with or without the formation of a cervical esophagostomy; and whether to use a gastric tube (reversed and proximally based or antegrade and distally based). See Media file 5.
The use of colonic (left chest or substernal), gastric pull-up, or jejunal vascularized graft segments is difficult and should be based on the condition of the infant, the pathologic anatomy, associated defects (eg, gastric pull-up is usually contraindicated in significant cardiac disease, colonic esophageal replacement is usually contraindicated with concomitant imperforate anus), and the surgeon's experience.
As a rule, a child's own esophagus is better than any substitution. Recent favorable reports of the Foker technique used for serial dynamic lengthening in cases of long gap suggest that advantage.12,13,14 The Foker technique involves 2 thoracotomies. First, anchoring sutures are placed securely at the 2 ends of the atretic esophagus and are brought out diagonally to the chest wall. Over a period of days to weeks, the 2 ends are brought closer together by a series of daily lengthenings by traction on the exposed sutures. The closure of the gap is monitored radiologically with radio-opaque markers at the atretic ends. A second thoracotomy is then performed to effect a tension-free anastomosis.
Preoperative Details
Bronchoscopy performed just prior to repair of the esophageal atresia may enable the following:- Detection of a upper pouch fistula
- Localization of the distal fistula, which usually lies at a level just above that of the carina
- Detection of an aberrant right upper lobe bronchus emanating from the trachea, which is not uncommon in cases of esophageal atresia
- Early assessment of the cross-sectional shape of the trachea, which may help in determining the risk of significant postoperative tracheomalacia
- Assessment of specific vascular anomalies (eg, right-sided aortic arch, aberrant right subclavian artery [for which one looks for the pattern of pulsation on the tracheal wall])
Identification of a laryngotracheoesophageal cleft
The infant is endotracheally intubated without paralysis. The anesthesiologist must be mindful of the distal fistula. With skill, the long end of the distal endotracheal tube bevel may be positioned over the fistula to decrease the passage of gases into the stomach. This maneuver helps prevent gastric distension, maximizes ventilation, and minimizes the chances of a gastric perforation. As much as possible, the baby should be allowed to spontaneously breathe until the fistula is occluded. In reality, and especially because the chest is open and the lung is retracted, the anesthesiologist manually assists with the baby's ventilation. However, mechanical ventilation should be avoided until the fistula is controlled. This procedure requires great skill, experience, and focus on the part of an anesthesiologist in caring for these babies in the operating room.
Managing an infant with premature lungs
In positioning the baby in full right thoracotomy position, the surgeon must ensure that the anesthesiologist has full and easy access to the infant's nose and to the Replogle tube, which is not taped so that it can move in or out. If a right-sided aortic arch is detected preoperatively, controversy exists about whether a left thoracotomy provides easier access. A left-sided approach has its merits, but in this instance, the esophagus is still a right-sided structure, and access from the right is best.
Lastly, the baby is covered with antiseptic solution, and drapes are placed with the areas from the nipple to mid back and from the axilla to the 10th rib exposed.
Intraoperative Details
The surgeon should wear magnification loupes. The assistants and nurses should be briefed about their duties and about special points of care regarding the delicate nature of the procedure and the baby's tissues.
The procedure is performed as follows: A transverse right thoracotomy incision is made from the anterior axillary line to approximately one finger's breadth posterior to the posterior axillary line at a level 1 cm inferior to the palpable tip of the scapula. The latissimus dorsi is divided with the coagulating current of the electrocautery device. The fascia lying just posterior to the posterior margin of the serratus is divided with electrocautery, and the serratus is retracted anteriorly. Usually, an incision in the serratus is not needed. The scapula is then lifted away from the chest wall, and the ribs are counted from the first to the fourth. Ideally, the chest is entered through the fourth interspace. With careful use of forceps and the electrocautery device, the outer and innermost intercostal muscles are divided in this interspace down to the parietal pleura.
By using either moist sponges or peanut gauze on the forceps, the parietal pleura is dissected away from the chest wall, proceeding posteriorly but also dissecting somewhat superiorly and inferiorly as well. A small mechanical Finochietto-type rib retractor is placed in the open thoracotomy site, and the pleural dissection proceeds to a point medial to the azygos vein. The azygos vein is ligated and divided with fine silk. This extrapleural dissection then allows retropleural repair of the esophagus. If an anastomotic leak occurs, it tends to be more contained compared with the empyema that results if the repair is performed transpleurally.
At this point in the dissection, the anatomy is defined first by having the anesthesiologist push on the indwelling Replogle tube; this action usually reveals the upper pouch that rhythmically bulges out in the apex of the right chest cavity. The distal fistula is at the level of the carina and usually lies just beneath the divided azygos vein. It expands slightly with each inspiration. One must take great care not to mistake the aorta for the fistula. Mistaken ligation of the aorta is possible; in case of doubt, a 25-gauge needle can be passed into the structure to check.
Gaining control of the fistula now relieves the anesthesiologist. A silicone rubber vessel loop can be passed around the fistula at a convenient level near the trachea. Gentle retraction on this occludes the fistula. Most advise dividing the fistula with suturing of its tracheal aspect. This division can be accomplished by cutting into the fistula as it enters the back wall of the trachea in short snips and by oversewing the tracheal aspect as it opens in stages. Usually, about 4 interrupted sutures suffice. Most advocate the use of an absorbable synthetic suture material such as polyglactin. This sutured fistula site may be covered with an azygos or pleural patch for extra security.
The fistula closure should be checked by covering the closure in saline and manually ventilating the patient for a Valsalva test. If bubbles appear, the closure is leaking and must be resutured. Turning his or her attention to the upper pouch, the anesthesiologist can again push on the Replogle tube to facilitate placement of a traction suture into the distal end of the upper pouch. The upper pouch is then dissected superiorly to increase its length. Blood supply to the upper portion is linearly arrayed from the cervical and subclavian vessels; ischemia is not a concern. This dissection must be carefully performed between the pouch and the trachea while the presence of an upper pouch fistula that emanates from the side, not the end, of the pouch is determined. Also, the back wall of the trachea may be inadvertently entered. This condition is repairable with absorbable sutures.
Avoid extensive dissection of the distal end because its blood supply is segmental from the aorta, and it can easily become ischemic. A gap between the ends may seem to be present. If it is very lengthy, the muscular covering of the upper pouch may be cut without entry into the lumen to achieve an extra 1 cm or so. Distal dissection may be performed; the risk of ischemia should be recognized. If absolutely necessary, the 2 ends may be simply bridged using 2 stout silk sutures in the hopes that they form a fistula and that they can be dilated to form a functional esophagus. More commonly, the 2 ends are reasonably close, and an anastomosis is possible.
The distal portion of the upper pouch is cut off, and the proximal portion of the distal segment is trimmed. Both the mucosal and muscularis layers of the esophagus should be carefully sutured in a single layer to form an anastomosis with simple interrupted stitches. Once again, most advocate the use of an absorbable synthetic suture with a caliber of approximately 5-0 (eg, braided polyglactin). The back wall is sutured, and the upper pouch tube is passed through the half-completed anastomosis into the stomach to help rule out a distal stricture and to empty the stomach of accumulated gas.
This tube is left in place as the anterior wall of the anastomosis is completed. The tube is then gently withdrawn from the body. Some advocate leaving the transanastomotic tube to act as a stent, although this tube may be partially moved, potentially injuring the anastomosis. A small-caliber 10F chest tube may be left in place as an extrapleural chest drain. The ribs are closed by encircling them with two 3-0 absorbable sutures and by restoring their normal anatomic position. The muscles and skin are closed in layers with absorbable sutures.
In some pediatric surgical centers, surgeons are gaining experience in repairing esophageal atresia using a minimally invasive thoracoscopic approach.15,16,17,18 This approach should be undertaken only by those who have extensive experience in pediatric thoracoscopic surgery.
Postoperative Details
The intubated patient is transported to the neonatal intensive care unit. Antibiotics are continued until the chest drain is removed, and the endotracheal tube is suctioned as necessary. Oral suctioning to a depth of no more than 7 cm from the lips is performed every half hour for the first day, then every hour or more frequently as necessary on the second day. Thereafter, it is performed as needed. Suctioning is required to handle the sometimes copious oral secretions that can build up in the first day or so after surgery. As the swelling of the esophagus settles, the secretions taper.
The chest draining tube is placed in 2 cm of water only to seal it; it is not connected to a suction device, which could encourage an anastomotic leak. Morphine is infused as necessary for the patient's comfort, and peripheral parenteral nutrition should be commenced. The endotracheal tube should remain until weaning from ventilation is ensured, usually after 1-2 days. Premature extubation and subsequent intubation in the setting of a freshly closed tracheal fistula invites reopening of the fistula.
Watch for saliva exiting out the chest drain; this is a signal of anastomotic leakage. Often, it is accompanied by visible distress. Signs of sepsis may or may not be present. A chest radiograph should be obtained. Provided that the baby is stable, a contrast-enhanced study of the esophagus with a water-soluble isotonic medium may be performed on day 6 or 7 to assess for leaks and to view the caliber of the repair (see Media file 6). If the esophagus is patent and reasonably sized, the baby may be orally fed; starting with expressed breast milk is ideal. Then, the chest tube is removed. As soon as the baby is feeding well, the intravenous line is discontinued, and the baby can be discharged. Oral ranitidine is prescribed for 6 months because of the propensity for gastroesophageal reflux in this group of patients and because of the risk of stricture as a secondary effect.
Follow-up
If all is well with the patient and if the parents have been briefed on what to look for, a reasonable follow-up regimen may include the following steps:
- Make contact with the community physician who is responsible for the general medical care of the child and ensure that he or she is briefed on the baby's history, condition, and expected outcome.
- The nurse on the surgical team should follow up by telephone in one week.
- The surgeon should follow up in one month to interview the parents and generally assess the child's condition, growth, and healing at the surgical site.
- The patient should return at 3 months for a similar assessment.
- At a 1-year follow-up and general assessment, swallowing function, respiratory issues, and other factors should be addressed.
Radiologic assessment of the esophagus is required only if a significant history of choking, cyanosis, regurgitation, dysphagia, growth failure, coughing, or wheezing is noted. Subsequent endoscopic evaluation can be performed as indicated.
Follow-up care when the child is older can be performed as needed. Specific reassessment with esophageal endoscopy and biopsy when the patient is aged approximately 12 years has been advised by some who also advise follow-up with periodic endoscopy every few years until the patient is an adult. Although Barrett esophagus and subsequent malignant change has been described in this condition, presumably because of gastroesophageal reflux, whether endoscopic surveillance is necessary in patients with repaired esophageal atresia remains unclear.19,20
Complications
Early complications
Early complications may include an anastomotic leak,21 recurrent tracheoesophageal fistula (TEF), and anastomotic stricture.
An anastomotic leak tends to occur 3-4 days after surgery. This leak has been reported in approximately 15% of cases. Pain and distress are often evident. Signs of sepsis may be present. The chest tube drains saliva. Treatment is supportive; appropriate antibiotics should be used, and the child should be given nothing by mouth. Surgery is not indicated, even with huge leaks. If the leak persists, esophagography may be performed with water-soluble contrast material to assess its magnitude. The usual protocol is to wait and let the leak close. If an extrapleural approach was used, the child is usually less ill than with other approaches, and the resultant esophagocutaneous fistula closes within days. If a transpleural approach was used, then the child is more ill and has an empyema that may require further treatment and drainage. No absolute evidence indicates that postoperative leaks lead to anastomotic stenoses.
Recurrent TEF may occur within days; most often, it occurs weeks later. Its incidence has been variously reported as 3-14%. Its first manifestation may be pneumonia, although the child may cough and have respiratory distress with feeding. The diagnosis is made by means of an esophagography performed with water-soluble contrast material under fluoroscopic guidance with the child prone. The contrast material is slowly injected through a catheter in the esophagus as the tube is slowly withdrawn, and lateral views are obtained by means of videofluoroscopy.
The recurrent fistula is observed as a wisp of contrast material that suddenly crosses over to the trachea. This so-called pull-back esophagraphy is the most accurate method for diagnosing a recurrent fistula. Bronchoscopy and esophagoscopy may provide supplementary information. One endoscopic technique is to inject 0.5 mL of methylene blue into the endotracheal tube and through the esophagoscope while watching for it to come through the fistula. Historically, these fistulae were believed to require surgical repair by means of repeat right-sided thoracotomy; however, the authors have been successful in a minority of cases of fistulae by allowing them to close spontaneously while maintaining the nothing by mouth restriction and while administering antibiotics for one week. Endoscopic cautery and fibrin glue have also been reported to be occasionally successful.
Anastomotic stricture has been reported in as many as 50% of cases, but the rate partially depends on the definition of stricture. Essentially, 100% of babies have a waist at the anastomotic site, but this may not be functionally significant. In cases in which the stricture appears to be functionally significant on oral contrast-enhanced studies, esophageal dilation is best and is most safely performed by means of a Grüntzig balloon technique under fluoroscopic control (in the authors' opinion). This procedure should be performed by an experienced radiologist who can monitor the balloon pressure, position, and inflation diameter. In newborns, this technique of dilatation would best be deferred until the child is aged at least 6 weeks, and at least 4 weeks after anastomosis.
Other methods involve the passage of tapered dilators of various sorts (eg, Tucker and Maloney dilators). Certainly, the methods can be effective but are performed in essentially a blind manner unless done under fluoroscopic control. They also involve longitudinal and radial force vectors, as opposed to the pure radial force vectors of the Grüntzig technique. Repeat dilations are often necessary. Histamine 2 (H2)-receptor blockade should be started because acid reflux can be both an aggravating and a causative factor in stricture formation.
Other factors to consider include the surgical technique; the type of suture used; the length of the atretic gap; ischemia of the distal portion; and, possibly, whether an anastomotic leak has occurred. Strictures resistant to a few dilations need more aggressive treatment, which may include an antireflux operation, stricture resection, or both; rarely, they require esophageal replacement. Stents have been used but are still investigational. Surprisingly, parents can be taught to perform regular Maloney dilations at home in selected cases.
Late complications
Late complications may include gastroesophageal reflux, esophageal dysmotility, and tracheomalacia. Some of these complications may appear early.
Gastroesophageal reflux is particularly problematic in patients with esophageal atresia because of congenital distal dysmotility of the esophagus, dysfunction of the physiologic antireflux barrier, possible partial vagotomy during surgery, or essential vagal dysfunction that can lead to delayed gastric emptying. Essentially all babies with esophageal atresia have detectable gastroesophageal reflux. Patients who require treatment must be carefully identified.
All babies with esophageal atresia should be prophylactically treated with ranitidine until they are aged 6 months. Failure to thrive, coughing, choking spells, wheezing and asthma, recurrent pneumonias, vomiting, cyanosis, dying spells, excessive drooling, and apparent dysphagia are all indications to investigate the degree of gastroesophageal reflux. Oral contrast material should be administered, and endoscopy should be performed. Strictures should be dilated. A pH probe study may help if the probe is placed below any present stricture. A gastric emptying scan should be obtained. All factors should be carefully considered.
Surgical approaches to helping the child may include an antireflux operation. A partial-wrap fundoplication is usually preferred because of the dysmotility of the repaired esophagus. Dysphagia after even a very loose wrap is not uncommon. If the stomach has delayed emptying, balloon pyloroplasty or surgical pyloroplasty may be considered to speed emptying. The authors have used a surgically conservative approach in children with this condition; the authors prefer to treat the reflux medically, with H2-receptor blockade or proton pump inhibition when possible. However, certainly some patients require a surgical approach for later complications.
Esophageal dysmotility is an ongoing problem. It has various dysphagic manifestations. The children eventually learn that they must masticate thoroughly and drink fluids when eating. Food bolus obstructions, even without a significant stricture, are not uncommon in toddlers. Parents must be mindful of this possibility and choose their child's foods accordingly. The use of motility agents such as domperidone may help.
Tracheomalacia is the manifestation of disordered embryogenesis. In its severe form, which occurs in approximately 10% of patients, dramatic signs include an inability to wean the patient from a ventilator and the classic dying spells in which the patient becomes pale and limp and, usually, apneic and cyanotic for a short time. Children with this condition require examination and treatment. Milder cases of tracheomalacia may cause recurrent pneumonias or asthma attacks, and in general respiratory ailments are common in these children.
Bronchoscopy performed while the patient is spontaneously breathing reveals a trachea that significantly collapses, flattens, or closes upon expiration. Treatment consists of aortopexy,22 which suspends the aortic arch to the underside of the sternum and, thus, secondarily suspends the anterior tracheal wall anteriorly, preventing its collapse. If this is unsuccessful, stent placement may help, but this option is controversial. Tracheostomy is the final management option. Fortunately, tracheomalacia tends to improve with time, growth, and maturation.
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Further Reading
Keywords
esophageal atresia, EA, tracheoesophageal fistula, TEF, congenitally interrupted esophagus, malformed esophagus, tracheoesophageal defects, trisomy 21, trisomy 13, trisomy 18, tracheoesophageal separation, esophageal atresia without fistula, pure esophageal atresia, proximal TEF, distal TEF, H-type fistula, congenital esophageal stenosis, polyhydramnios, aspiration pneumonitis, acute gastric perforation, dysphagia, gastroesophageal reflux, tracheomalacia, pneumonia, respiratory distress, VACTERL, vertebral defects, anorectal malformations, cardiovascular defects, tracheoesophageal defects, renal anomalies, limb deformities, hemivertebrae, scoliosis, rib deformities, imperforate anus, cloacal deformities
ventricular septal defect, tetralogy of Fallot, patent ductus arteriosus, atrial septal defects, atrioventricular canal defects, aortic coarctation, right-sided aortic arch, single umbilical artery, Potter syndrome, bilateral renal agenesis, horseshoe kidney, polycystic kidneys, urethral atresia, ureteral malformations, radial dysplasia, absent radius, radial-ray deformities, syndactyly, polydactyly, lower-limb tibial deformities, coloboma, heart defects, atresia choanae, developmental retardation, genital hypoplasia, ear deformities, CHARGE, neural tube defects, hydrocephalus, tethered cord, holoprosencephaly, duodenal atresia, ileal atresia, hypertrophic pyloric stenosis, omphalocele, malrotation, Meckel diverticulum, unilateral pulmonary agenesis, diaphragmatic hernia, undescended testicles, ambiguous genitalia, hypospadias, Fanconi syndrome
