You are in: eMedicine Specialties > Pediatrics: Surgery > General Surgery Esophageal Atresia With or Without Tracheoesophageal FistulaArticle Last Updated: Apr 30, 2008AUTHOR AND EDITOR INFORMATIONSection 1 of 12
  Author: Amulya K Saxena, MD, Attending Pediatric Surgeon, Department of Pediatric Surgery, Medical University of Graz, Austria Amulya K Saxena is a member of the following medical societies: European Pediatric Surgeons Association, German Society of Pediatric Surgery, German Society of Surgery, and International Pediatric Endosurgery Group Coauthor(s): Geoffrey Blair, MD, Clinical Professor of Pediatric General Surgery, Department of Pediatric Surgery, University of British Columbia; Head, British Columbia's Children's Hospital; David E Konkin, MD, Staff Physician, Department of Surgery, Royal Columbian Hospital, University of British Columbia Editors: Kurt D Newman, MD, Vice Chairman, Department of Pediatric Surgery, Children's National Medical Center; Professor, Departments of Surgery and Pediatrics, George Washington University School of Medicine; Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc; Michael G Caty, MD, Professor of Surgery and Pediatrics, State University of New York at Buffalo; Consulting Staff, Department of Pediatric Surgery, Children's Hospital of Buffalo; H Biemann Othersen Jr, MD, Professor of Surgery and Pediatrics, Emeritus Head, Division of Pediatric Surgery, Medical University of South Carolina; Marleta Reynolds, MD, Professor of Surgery, Feinberg School of Medicine, Northwestern University; Interim Head, Division of Pediatric Surgery, Department of Surgery, Children's Memorial Hospital of Chicago Author and Editor Disclosure Synonyms and related 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 INTRODUCTIONSection 2 of 12
  Esophageal atresia refers to a congenitally interrupted esophagus. One or more fistulae may be present between the malformed esophagus and the trachea. History of the ProcedureThe condition was first described anecdotally in the 17th century. In 1670, Durston described the first case of esophageal atresia in one conjoined twin. In 1696, Gibson provided the first description of esophageal atresia with a distal tracheoesophageal fistula (TEF). In 1862, Hirschsprung (a famous pediatrician from Copenhagen) described 14 cases of esophageal atresia. In 1898, Hoffman attempted primary repair of the defect but was not successful and resorted to the placement of a gastrostomy. At the start of the 20th century, surgeons were theorizing about how the lesion could be repaired. In 1939 and 1940, Ladd of Boston and Lever of Minnesota first achieved surgical success in stages; success meant that the affected children survived and skin-lined pharyngogastric conduits were eventually constructed. In 1941, Haight of Michigan successfully repaired esophageal atresia in a 12-day-old baby using a primary single-stage left-sided extrapleural approach. Subsequent to that child's survival and with advances in surgical and anesthetic techniques, esophageal atresia is now regarded as an eminently correctable congenital lesion. ProblemThe lack of esophageal patency prevents swallowing. In addition to preventing normal feeding, this problem may cause infants to aspirate and literally drown in their own saliva, which quickly overflows the upper pouch of the obstructed esophagus. If a TEF is present, fluid (either saliva from above or gastric secretions from below) may flow directly into the tracheobronchial tree. FrequencyThe incidence of esophageal atresia is 1 case in 3000-4500 births. This frequency may be decreasing for unknown reasons. Internationally, the highest incidence of this disorder is in Finland, where it is 1 case in 2500 births. EtiologyNo human teratogens that cause esophageal atresia are known. Esophageal atresia that occurs in families has been reported. A 2% risk of recurrence is present when a sibling is affected. The occasional association of esophageal atresia with trisomies 21, 13, and 18 further suggests genetic causation. Also, twinning occurs about 6 times more frequently in patients with esophageal atresia than in those without the condition. Currently, most authorities believe that the development of esophageal atresia has a nongenetic basis. Debate about the embryopathologic process of this condition continues, and little about it is known. The old His theory that lateral infoldings divide the foregut into the esophagus and trachea is attractively simple, but findings from human embryology studies do not support this theory. In 1984, O'Rahilly proposed that a fixed cephalad point of tracheoesophageal separation is present, with the tracheobronchial and esophageal elements elongating in a caudal direction from this point.1 This theory does not easily account for esophageal atresia but explains TEF as a deficiency or breakdown of esophageal mucosa, which occurs as the linear growth of the organ exceeds the cellular division of the esophageal epithelium. In a 1987 report, Kluth eschews the concept that tracheoesophageal septation has a key role in the development of esophageal atresia.2 Instead, he bases the embryopathologic process on the faulty development of the early, but already differentiated, trachea and esophagus, in which a dorsal fold comes to lie too far ventrally; thus, the early tracheoesophagus remains undivided. He also suggests that esophageal vascular events, ischemic events, or both may be causes in cases of esophageal atresia without fistula. In 2003, Spilde et al reported esophageal atresia-TEF formations in the embryos of rat models of Adriamycin-induced teratogenesis.3 Specific absences of certain fibroblast growth factor (FGF) elements have been reported, specifically FGF1 and the IIIb splice variant of the FGF2R receptor.4 These specific FGF-signaling absences are postulated to allow the nonbranching development of the fistulous tract from the foregut, which then establishes continuity with the developing stomach. In 2001, Orford et al postulated that the ectopic, ventrally displaced location of the notochord in an embryo at 21 days' gestation can lead to a disruption of the gene locus, sonic hedgehog-signaled apoptosis in the developing foregut, and variants of esophageal atresia.5 This situation may be due to various early gestation teratogenic influences such as twinning, toxin exposure, or possible abortion. More studies are required. PathophysiologyThe variants of esophageal atresia have been described using many anatomic classification systems. To avoid ambiguity, the clinician should use a narrative description. Nevertheless, Gross of Boston described the classification system that is most often cited (see Media file 1).6 According to this system, the types of esophageal atresia and the approximate incidence in all infants born with esophageal anomalies is as follows:
A fetus with esophageal atresia cannot effectively swallow amniotic fluid, especially when TEF is absent. In a fetus with esophageal atresia and a distal TEF, some amniotic fluid presumably flows through the trachea and down the fistula to the gut. Polyhydramnios may be the result of this change in the recycling of amniotic fluid through the fetus. Polyhydramnios, in turn, may lead to premature labor. The fetus also appears to derive some nutritional benefit from the ingestion of amniotic fluid; thus, fetuses with esophageal atresia may be small for their gestational age. The neonate with esophageal atresia cannot swallow and drools copious amounts of saliva. Aspiration of saliva or milk, if the baby is allowed to suckle, can lead to an aspiration pneumonitis. In a baby with esophageal atresia and a distal TEF, the lungs may be exposed to gastric secretions. Also, air from the trachea can pass down the distal fistula when the baby cries, strains, or receives ventilation. This condition can lead to an acute gastric perforation, which is often lethal. Prerepair esophageal manometric studies have revealed that the distal esophagus in esophageal atresia is essentially dysmotile, with poor or absent propagating peristaltic waves. This condition results in variable degrees of dysphagia after the repair and contributes to gastroesophageal reflux. The trachea is also affected by the disordered embryogenesis in esophageal atresia. The membranous part of the trachea, the pars membranacea, is often wide and imparts a cross-sectional D shape to the trachea, as opposed to the usual C shape. These changes cause secondary anteroposterior structural weakening of the trachea, or tracheomalacia. This weakening can result in a sonorous cough as the intrathoracic trachea resonates and partially collapses with forceful expiration. Secretions can be difficult to clear and may lead to frequent pneumonias. Also, the trachea can partially collapse during feeding, after repair, or with episodes of gastroesophageal reflux; this partial collapse can lead to ineffective respiration; hypoxia; and, somewhat inexplicably, apnea. ClinicalA mother who is carrying a fetus with esophageal atresia may have polyhydramnios, which occurs with approximately 33% of mothers with fetuses with esophageal atresia and distal TEF and with virtually 100% of mothers with fetuses with esophageal atresia without fistula. Characteristically, the neonate born with esophageal atresia drools and has substantial mucus, with excessive oral secretions. If suckling at the breast or bottle is allowed, the baby appears to choke and may have difficulty maintaining an airway. Significant respiratory distress may result. In the delivery room, the affected infant may have the sonorous seal-bark cough that indicates concomitant tracheomalacia. If an oral tube is placed to suction the stomach, as it is in some delivery rooms, it characteristically becomes blocked 10-11 cm from the lips. Vertebral defects, anorectal malformations, cardiovascular defects, tracheoesophageal defects, renal anomalies, and limb deformities (VACTERL) are associated anomalies that should be readily apparent upon physical examination. If any of these anomalies are present, the presence of the others must be assessed. The VACTERL syndrome occurs when 3 or more of the associated anomalies are present. This syndrome occurs in approximately 25% of all patients with esophageal atresia. Anomalies in this syndrome include the following:
Other associated conditions include coloboma, heart defects, atresia choanae, developmental retardation, genital hypoplasia, and ear deformities (CHARGE).
Also, trisomies 13, 21, or 18 and Fanconi syndrome may be present. The overall incidence of associated anomalies is approximately 50%. Cardiovascular anomalies occur in 35% of cases, genitourinary anomalies occur in 20% of cases, and associated gastrointestinal anomalies occur in approximately 20% of cases. A tethered cord is usually detectable with ultrasonography in the newborn period or later in life with MRI (or less desirably with CT scanning) if findings are equivocal. INDICATIONSSection 3 of 12
The indication and timing of surgical repair may be determined by using the Waterston, Spitz, or Poenaru prognostic classification system. In 1962, Waterston developed a prognostic classification system for esophageal atresia that is still used today.7 Category A includes patients who weigh more than 5.5 lb (2.5 kg) at birth and who are otherwise well; category B includes patients who weigh 4-5.5 lb (1.8-2.5 kg) and are well or who have higher birth weights, moderate pneumonia, and congenital anomalies; and category C includes patients who weigh less than 4 lb (1.8 kg) or have higher birth weights, severe pneumonia, and severe congenital anomalies. Management strategies are as follows:
In 1994, after analyzing findings in 387 patients, Spitz et al recognized that the presence or absence of cardiac disease is a proven major prognostic factor.8 Spitz et al suggested the following groups, which are analogous to those in the Waterston classification system:
In 1993, Poenaru proposed a simpler, 2-group classification system based on logistic regression analysis findings in 95 patients.9 Note that birth weight is not a factor. Class I includes patients who are low risk and do not meet criteria in class II, and class II includes patients who are high risk and ventilator-dependent or who have life-threatening anomalies, regardless of pulmonary status. In 1989, Randolph et al refined the Waterston classification and reported a clinically helpful system that used a patient's physiologic status to determine the surgical management (ie, immediate repair, delayed primary repair, or staged repair).10 Weight, gestational age, and pulmonary condition were not considered. If the patient's physiologic parameters were good, they were managed with immediate repair. Staged repairs were used for infants who were severe compromised infants, especially those with severe cardiac anomalies. In this group, the survival rate was 77%, and the overall survival was 90%. The above prognostic groupings can allow for the stratification of high-risk patients with esophageal atresia in planning for delayed repair, staged repair, or both; low-risk babies can usually undergo early (first 24-48 h) primary single-stage repair. For instance, a 2-kg baby with esophageal atresia and distal tracheoesophageal fistula (TEF) who also has tetralogy of Fallot is in Waterston category C, Spitz group II, and Poenaru class II; in this patient, delayed or staged repair may be best. RELEVANT ANATOMYSection 4 of 12
The treatment plan for each baby must be individualized. The prognostic classifications can provide guidance in patients with multiple problems, but decisions in identifying the most life-threatening anomaly must be made early. Management plans for a delayed repair of the esophageal atresia may include placing a 10F Replogle double-lumen tube through the mouth or nose well into the upper pouch to provide continuous suction of pooled secretions from the proximal portion of the atretic esophagus. The baby may be positioned in the 45° sitting position. Prophylactic broad-spectrum antibiotics such as ampicillin and gentamicin may be used. General supportive care and total parenteral nutrition are needed. With careful bedside attendance, these measures may permit a delay of days to perhaps weeks. Some have described cases in which the baby was discharged home with a Replogle tube in situ while waiting for staged repair of an esophageal atresia. However, deaths have been reported in infants in whom the tube did not maintain an empty upper pouch. A gastrostomy, distal tracheoesophageal fistula (TEF) ligation, or cervical esophagostomy may permit longer delays in the esophageal atresia repair. However, each intrusion carries a price. A gastrostomy may be created if no distal TEF is present. In such cases, the stomach is small, and laparotomy is required. In all cases of esophageal atresia in which a gastrostomy is created, care should be taken to place it near the lesser curve to avoid damaging the greater curve, which can be used in the formation of an esophageal substitute. When a baby is ventilated with high pressures, the gastrostomy may offer a route of decreased resistance, causing the ventilation gases to flow through the distal fistula and out the gastrostomy site. This condition may complicate the use of ventilation. In cases such as those above or in cases in which a distal fistula continues to cause lung soiling, consider distal TEF ligation. This ligation is performed by means of a right-sided thoracotomy, ideally performed via an extrapleural approach. The fistula may be clipped or simply ligated. If it is ligated and divided, subsequent staged repair of the esophageal atresia may be difficult because the distal esophageal segment tends to retract inferiorly to a substantial degree when it is detached from its tracheal mooring. However, simple fistula ligation may allow subsequent reopening of the fistula. Division of the fistula and attempts to anchor it at the mid chest with sutures are usually unsuccessful. A cervical esophagostomy or spit fistula may be constructed in the right or left side of the neck, depending on the choice for subsequent esophageal substitution. It allows drainage of the upper pouch and precludes aspiration from the upper pouch. Sham feeding may be commenced in cases in which a long delay to repair is anticipated. This feeding may prevent subsequent oral aversion, which is a real problem in babies who have not been fed by mouth in their early weeks to months of life. However, cervical esophagostomy usually dooms the child to some form of esophageal substitution. Please see Preoperative details for a discussion of aortic arch position and surgery. CONTRAINDICATIONSSection 5 of 12
Potter syndrome is bilateral renal agenesis and has a 100% mortality rate; therefore, repair of esophageal atresia is contraindicated. WORKUPSection 6 of 12
Lab StudiesIn babies with esophageal atresia, samples should be obtained to determine baseline values of the following:
Imaging Studies
TREATMENTSection 7 of 12
Medical TherapyThe preparation of a 1-day-old neonate for surgery includes the following measures:
Surgical TherapyThis 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 DetailsBronchoscopy performed just prior to repair of the esophageal atresia may enable the following:
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 DetailsThe 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 DetailsThe 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-upIf 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:
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 COMPLICATIONSSection 8 of 12
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. OUTCOME AND PROGNOSISSection 9 of 12
Statistics regarding mortality rates in esophageal atresia are constantly changing and improving.23 One must consider the classification system used in reporting such statistics.
Fetuses with prenatal diagnoses of esophageal atresia seem to have a worse prognosis.25 The cohort of babies in whom esophageal atresia is detected prenatally has a 75% mortality rate, whereas the cohort of babies in whom esophageal atresia is not detected prenatally has a 21% mortality rate. Babies who survive have varied morbidities related to any of the associated anomalies and complications. However, most children who undergo a successful repair of esophageal atresia are relatively healthy. FUTURE AND CONTROVERSIESSection 10 of 12
The future is bright, with the following considerations: More accurate prenatal diagnosis and prenatal treatment may be possible. Minimally invasive techniques for repair with thoracoscopic surgery are now used in some centers, with good results. A better understanding of the pathoembryologic processes of this condition may reveal its causative agents or genetic factors. This knowledge, in turn, may lead to specific prenatal treatments or preventive techniques. Recently, the incidence of this disorder has decreased, perhaps because of increased usage of prenatal folic acid supplements. Debates continue about the best operative technique (eg, right-sided or left-sided thoracotomy) for patients with right-sided aortic arches, suture type and technique, esophageal lengthening strategies, and procedures for mobilizing the distal esophagus. Other discussions include when to use cervical esophagostomy and the choice of esophageal replacement. The advent of esophageal atresia repairs that combine both minimally invasive and radiologic interventional techniques may be near. The management of gastroesophageal reflux in esophageal atresia is particularly challenging; some advocate aggressive fundoplication, and others prefer more conservative medical treatment. In addition, the true incidence and treatment of tracheomalacia continues to be the subject of debate. Lastly, the proper evidence-based guidelines for long-term follow-up are still elusive. MULTIMEDIASection 11 of 12
REFERENCESSection 12 of 12
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