You are in: eMedicine Specialties > Pediatrics: Surgery > General Surgery Congenital Anomalies of the EsophagusArticle Last Updated: May 3, 2006AUTHOR AND EDITOR INFORMATIONSection 1 of 12
  Author: Robert K Minkes, MD, PhD, Staff Pediatric Surgeon, Houston Pediatric Surgeons, Texas Children's Hospital Robert K Minkes is a member of the following medical societies: Alpha Omega Alpha, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, and Phi Beta Kappa Coauthor(s): Alison Snyder, MD, Washington University School of Medicine; Sean E McLean, MD, Consulting Staff, General Surgery, Washington University, Barnes-Jewish Hospital, St Louis Children's Hospital; Mark V Mazziotti, MD, Assistant Professor of Pediatric Surgery, Department of Surgery, Baylor College of Medicine, Texas Children's Hospital; Jacob C Langer, MD, Professor, Department of Surgery, University of Toronto Faculty of Medicine Editors: Robert Kelly, MD, Chairman, Department of Surgery, Departments of Surgery and Pediatrics, Children's Hospital of the King's Daughters; Associate Professor, Eastern Virginia Medical School; Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc; Deborah F Billmire, MD, Associate Professor, Department of Surgery, Indiana University Medical Center; 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: congenital anomalies of the esophagus, esophageal atresia, EA, tracheoesophageal fistula, TEF, esophageal stenosis, esophageal cyst, tracheobronchial remnant, esophageal atresia and tracheoesophageal fistula, EA-TEF, esophageal web, esophageal muscular hypertrophy, esophageal duplications, esophageal rests, columnar epithelium–lined lower esophagus, Barrett esophagus, Barrett's esophagus, laryngotracheoesophageal cleft, LTEC INTRODUCTIONSection 2 of 12
  Congenital anomalies of the esophagus occur in up to 1 per 3000-5000 births, with esophageal atresia (EA) and tracheoesophageal fistula (TEF) being the most common types. Other lesions, such as congenital esophageal stenosis, duplications, and cysts, occur less frequently. For excellent patient education resources, visit eMedicine's Esophagus, Stomach, and Intestine Center. Also, see eMedicine's patient education article, Choking. History of the ProcedureThe recorded history of EA dates back as early as 1670 when Durston described the presence of a blind-ending upper esophageal pouch in a conjoined twin, but surgical therapy for EA was not suggested until 1869. Steele made the first attempt at surgical correction for EA in 1888. He performed a gastrostomy in a patient with pure EA, hoping to perforate what he suspected to be an esophageal membrane. In 1913, Richter proposed fistula ligation with anastomosis of the 2 esophageal ends for EA with TEF. Although he considered primary repair to be the best option, he also acknowledged the impracticality of this procedure at the time. Instead, he ligated the fistula intrathoracically. His patient did not survive long enough to attempt an esophageal anastomosis. The first patient to survive a congenital esophageal anomaly was born in 1931 with a TEF and no atresia. The fistula was repaired with a transtracheal incision in 1935, the same year that the first survivor of EA was born. The infant with EA was treated with gastrostomy feedings and a jejunal interposition. Both of these children had an isolated defect (atresia or fistula), and treatment was successful without a thoracotomy. The treatment for EA with TEF proved to be more difficult. Pneumonia, mediastinitis, poor airway control, and fluid management problems were frequent complications. In 1936, Lanman was the first to perform a repair with an extrapleural approach. The first patient to undergo the technique survived only 3 hours. In 1938, Shaw performed the first fistula ligation and primary anastomosis of the esophagus for EA-TEF. This patient died 12 days postoperatively from a transfusion reaction. In 1939, the first 2 successful treatments of patients with EA-TEF occurred independently, one day apart, by Leven and Ladd. They performed staged repairs involving gastrostomy placement followed by fistula ligation 5 weeks and 4 months later, respectively. Cervical esophagostomies, the use of jejunal interposition, and an antethoracic skin tube for esophageal reconstruction were use in the years to follow. Haight completed the first successful primary repair in 1941. The procedure involved a left extrapleural approach, fistula ligation, and a single-layer esophageal anastomosis. Haight later switched to a right extrapleural approach and modified his technique to a 2-layer telescoping anastomosis in an attempt to diminish leak risk. By 1944, one third of the children with EA-TEF survived primary repair. Advances in preoperative preparation, antibiotic treatment, and intraoperative and postoperative management contributed to more favorable survival rates. Despite the increased success, leaks, strictures, and lower esophageal segment dysmotility were common postoperative problems. ProblemEA and TEF are the most common anomalies and, therefore, receive more emphasis. Congenital stenosis or obstruction is also encountered. Congenital muscular hypertrophy, webs, cysts, and tracheobronchial remnants are observed. Esophageal atresia and tracheoesophageal fistula EA is a condition in which the proximal and distal portions of the esophagus do not communicate. The upper segment of the esophagus is a dilated blind-ending pouch with a hypertrophied muscular wall. The pouch typically extends to the level of the second to fourth thoracic vertebra. In contrast, the distal esophageal portion is an atretic pouch with a small diameter and a thin muscular wall; it usually extends 1-2 cm above the diaphragm. TEF is an abnormal communication between the trachea and esophagus. When associated with EA, the fistula commonly enters the trachea posteriorly just above the carina. However, isolated TEF, or an H-fistula, can occur at any level from the cricoid cartilage to the carina. Several different types of EA and TEF have been described. The frequencies calculated from a summary of 6 long-term studies are provided for each type. The most common abnormality (84%) is EA with a distal TEF. Isolated atresia with no fistula is the next most common finding (8%), followed by isolated TEF with no atresia (4%). EA with proximal and distal fistulas (3%) and EA with a proximal fistula (1%) are less common. Esophageal stenosis, web, and muscular hypertrophy Congenital esophageal stenosis is a narrowing of a region of the esophagus. A web, or diaphragm, consists of a thin squamous epithelial membrane in the esophageal lumen. It typically causes a partial obstruction in the middle to lower esophagus. Congenital muscular hypertrophy is characterized by submucosal proliferation of smooth muscle and fibrous connective tissue beneath a normal squamous epithelium. Individuals with congenital muscular hypertrophy may be asymptomatic. Esophageal duplications, rests, and cysts An esophageal duplication may be open at both ends (double esophagus), open at one end (diverticulum), or closed (elongated cyst). Rests are areas where embryonic tracheal or esophageal cells reside in mesodermal tissues. These areas may form cysts in the muscular tissues. A choristoma is a distinct cartilaginous cyst that partially or completely encircles a region typically in the lower third of the esophagus. Columnar epithelium–lined lower esophagus The congenital form of this condition is associated with gastroesophageal reflux. Debate exists as to whether this lesion, also called Barrett esophagus, is congenital or acquired. Laryngotracheoesophageal cleft Laryngotracheoesophageal cleft (LTEC) is defined as a midline communication among the larynx, trachea, and esophagus. FrequencyEsophageal atresia has an incidence of 1 in 3000 in the United States. Internationally, EA occurs in 1 per 3000-5000 live births. Males have a slightly increased risk for EA compared to females, and one study in California reported a higher incidence of EA in white populations (1 per 10,000 births) compared to nonwhite populations (0.55 per 10,000 births). True congenital stenosis of the esophagus is rare. It occurs in 1 in 25,000-50,000 births. Incidence is higher in Japan. EtiologyThe etiology of EA is unknown; however, many theories have been proposed. The esophagus and trachea both are derivations of the primitive foregut. The larynx and trachea outpouch from the foregut at 22 days' gestation, and the lung buds are typically formed by the 26th day. Lateral mesodermal ridges form in the proximal esophagus during the fourth week of gestation, and the fusion of these grooves in the midline separates the esophagus from the trachea at approximately 26 days' gestation. The esophageal lumen forms following a process of mucosal proliferation and subsequent vacuole formation. Esophageal anomalies result from failure of these processes. Numerous theories have been postulated concerning the embryogenesis of EA, including asymmetric growth of the esophageal mesenchyme and the epithelial lining, an increased cell proliferation rate in the trachea, a lack of tracheoesophageal separation, notochord abnormalities, delayed or absent apoptosis, and neural crest abnormalities. Several theories have also been suggested for TEF. Failure of lateral ridge fusion or incomplete septation, abnormal epithelial connections that develop between the separated trachea and esophagus, and vascular deficiencies have been proposed to explain EA and TEF. Intestinal atresias have been produced experimentally by interrupting the blood supply to the intestine, but convincing support for this theory has not been demonstrated for EA. The fetal heart begins beating during the fourth week of development, and a vascular accident causing EA would need to occur before the sixth week of development. However, a 9-mm embryo with an established EA-TEF has been reported, indicating that the defect may occur before the vascular tree is fully developed. Many children with EA and TEF have been reported to have an insufficient esophageal blood supply. Furthermore, several reports have shown an association between EA and a single umbilical artery or other abnormalities that may result in vascular compromise. Aberrant vessels and an enlarged heart have also been cited as potential causative agents for tracheoesophageal malformations. These may cause excessive pressure on nearby organs, such as the esophagus and trachea. Other reports suggest that structures of the developing embryo are not rigid enough to be injured by this mechanism. Although genetics has not been found to play a definitive role in the origination of EA-TEF, several chromosomal defects and gene associations have been suggested. Genetic involvement has also been described for conditions in which EA is one of many anomalies, such as oculodigitoesophageoduodenal (ODED) syndrome (ie, Feingold syndrome), trisomy 18, and Down syndrome. Other etiologic factors (eg, vitamin deficiencies; drug exposures; viral, chemical, and physical external events) have been reported to cause tracheoesophageal malformations in experimental models and in humans. Other congenital anomalies One proposition is that esophageal webs result from a failure of esophageal vacuoles to coalesce at days 25-31 of development, which normally leads to complete luminal patency. True esophageal duplications may develop from persistent esophageal vacuoles, whereas cysts result from remnants of the dorsal notochord, abnormal tracheobronchial tree branching, or primitive foregut diverticula. Cysts may be formed when groups of cells that are capable of forming a portion of esophagus, stomach, or pulmonary tree are pinched off from the developing foregut. Congenital rings are thought to result from incomplete separation of respiratory tissue from the esophagus during fetal life. Stenosis results from abnormal rests of respiratory tissue in the esophageal wall or fibromuscular hypertrophy. Laryngotracheoesophageal clefts may result from faulty growth of the foregut folds, resulting in failure of the posterior larynx to close and allowing persistence of the primitive tracheoesophageal space. PathophysiologyBecause the esophagus is discontinuous, an infant with EA cannot swallow and appropriately handle secretions. Infants exhibit persistent drooling and aspiration or regurgitation of food after attempted feedings. Patients who have EA with distal TEF are at risk for additional complications related to the tracheoesophageal communication. When infants with this anomaly strain, cough, or cry, air enters the stomach through the fistula. As a result, the stomach and small intestine become dilated, elevating the diaphragm and making respiration more difficult. Gastric secretions may also reflux retrograde through the fistula into the tracheobronchial tree, contributing to pneumonia and atelectasis. Abnormal esophageal motility is common in children with congenital anomalies of the esophagus. ClinicalThe earliest clinical sign of an infant with EA is polyhydramnios resulting from the infant's inability to swallow and absorb amniotic fluid through the gut. The infant may have a small or absent stomach on ultrasonographic study. Note that polyhydramnios is observed in infants with many diagnoses. Only 1 in 12 infants with polyhydramnios has EA. Polyhydramnios is observed in 95% of infants with EA and no fistula and in 35% of patients who have EA with a distal fistula. Increased pressure because of the amniotic fluid accumulation results in a higher number of premature births and newborns with low birth weight. One third of infants with EA weigh less than 2250 g. Postnatally, infants with pure EA become symptomatic within the first few hours of life. Children with an isolated TEF have more subtle symptoms that may not be initially recognized. Excess salivation and fine frothy bubbles in the mouth and sometimes nose result from an inability to swallow. Any attempts at feeding result in choking, coughing, cyanotic episodes, and food regurgitation. The presence of a fistula increases the risk of aspiration of gastric secretions into the trachea and lungs. Pneumonitis and atelectasis develop quickly in these neonates, and rattles heard during respirations are common. Fistulas also allow air to enter into the stomach and intestines, which can lead to abdominal distension. Gastric perforations occur, especially in the presence of imperforate anus. In the presence of atresia alone, the abdomen appears scaphoid. Many anomalies are associated with EA, and 50-70% of children with EA have some other defect. The VACTERL association describes the following more commonly associated combination of defects: vertebral, anorectal, cardiac, tracheal, esophageal, renal, and limb. Cardiac abnormalities are the most common, especially ventricular septal defects and tetralogy of Fallot. Imperforate anus and skeletal malformations may also be found on examination. In the absence of such associated anomalies, the physical examination findings of infants with EA are fairly unremarkable. Symptoms of congenital esophageal stenosis related to membranous webs, diaphragm muscular hypertrophy, or tracheobronchial remnants occur in infancy with progressive dysphagia and vomiting. Most patients present after semisolid or solid foods are introduced. More rarely, patients with congenital stenosis present with regurgitation and aspiration as newborns. A foreign body in the esophagus may be the first symptom. Cysts may be identified on chest radiography or CT scanning obtained for recurrent pneumonia or unrelated reasons. INDICATIONSSection 3 of 12
All children with EA and many with congenital stenosis require surgical intervention. The diagnosis of EA or TEF can be made prenatally and after birth by clinical signs and supportive findings on imaging studies. Prenatally, an ultrasonographic finding of a small or absent stomach bubble suggests EA with 42% sensitivity. Recently, prenatal MRI was used to evaluate the esophagus of fetuses with a small or absent stomach bubble on ultrasonographic evaluation. Positive findings on prenatal MRI had a sensitivity of 100% and specificity of 80%. After birth, failure of passage of a rigid radiopaque 10F catheter from the mouth to the stomach suggests EA. The diagnosis is typically confirmed with plain radiography. The presence of air in the stomach and intestines indicates EA with a distal fistula; the absence of abdominal gas suggests pure atresia, EA with a proximal fistula, or on rare occasions, EA with an occluded distal fistula. A small upper esophageal pouch is suggestive of a proximal fistula, and the presence of a proximal TEF can be confirmed with fluorography, endoscopy, bronchoscopy, or upper esophageal contrast studies. An isolated TEF may be detected by barium esophagraphy, cinefluoroscopy, bronchoscopy, or esophagoscopy. Because of the risk of aspiration, the use of contrast for visualization of congenital esophageal anomalies must be approached with extreme care and performed only by an experienced radiologist. Although a diagnosis of EA or TEF is no longer considered a surgical emergency because of improvements in neonatal intensive care, respiratory problems still may develop and progress rapidly. A period of 24-48 hours between diagnosis and surgical repair allows for a thorough assessment of the neonate and treatment of any pulmonary complications. In general, vigorous infants weighing more than 1300 g and without pulmonary insufficiency or major associated anomalies should be considered for repair. Historically, prognostic risk classification was based on birth weight, the presence and severity of pneumonia, and congenital anomalies for infants with EA. More recently, ventilator dependence and severe anomalies, not birth weight, have been linked to mortality. Regardless of the classification used, stronger infants with fewer concomitant disorders have lower risks associated with the surgical repair. Intervention for esophageal stenosis, webs, and tracheobronchial remnants is indicated based on diagnosis and the presence of symptoms. RELEVANT ANATOMYSection 4 of 12
The esophagus can be divided into several segments based on its blood supply. The cervical portion of the esophagus is well vascularized and thought to have good intramural vascular communications. The cervical esophagus is supplied by the inferior thyroid artery and accessory vessels derived from the common carotid, subclavian, vertebral, ascending pharyngeal, superficial cervical, and costocervical arteries. Mobilization of the upper esophagus is generally well tolerated. The thoracic portion of the esophagus has a segmental blood supply. The connections in this region are the most tenuous, and care should be taken during mobilization of this segment to reduce the risk of ischemia. The bronchial arteries provide the main vascular supply at this level, and 1-3 bronchial branches enter the esophagus at the level of the tracheal bifurcation. Variable branches originating directly from the aorta may also be present in this region. The lower thoracic esophagus is supplied by 3 unpaired esophageal branches arising directly from the aorta. These branches may anastomose with branches from the intercostal and bronchial arteries. Branches from the internal mammary and carotid arteries may also be present here. The abdominal esophagus is supplied by the ascending branch of the left gastric artery and branches of the left inferior phrenic artery. Before surgery, the position of the aortic arch should be confirmed, preferably by echocardiography. The surgical approach should be performed on the side opposite of the aortic arch. A right-sided aortic arch occurs in 5% of infants with EA. Although the venous drainage of the esophagus is not described here, the azygos vein serves as a good landmark during surgery. The esophageal ends, particularly the distal segment in a TEF, are often readily visualized once the azygos vein has been divided or reflected. The esophagus is innervated largely by the autonomic nervous system. Sympathetic innervation plays a minor role and arises from the pharyngeal plexus in the upper esophagus and the stellate ganglia in the lower cervical and upper thoracic portions. The aortic plexus, sympathetic chain, and splanchnic nerves supply the remainder of the thoracic esophagus. In the abdominal segment, fibers from the celiac ganglion pass around the left gastric and inferior phrenic arteries to innervate the esophagus. Parasympathetic innervation to the esophagus is provided by the vagus. Parasympathetic function includes stimulation of smooth muscle and secretory activity. The vagus also aids the sphincteric function of the lower esophagus. The recurrent laryngeal nerves pass cranially in a groove between the esophagus and trachea, supplying the cervical and upper one third of the thoracic esophagus. The vagus nerves descend caudally, arborize to form the esophageal plexus, and then coalesce into the left and right vagal trunks, which overlie the anterior and posterior lower esophagus, respectively. Because of its course along the esophagus, the vagus is another helpful landmark during operative esophageal procedures. Disruption or injury to the vagus nerves during surgical manipulation has been proposed as a mechanism of dysmotility following esophageal repair. CONTRAINDICATIONSSection 5 of 12
The selection and timing of surgical treatment for congenital esophageal anomalies depend on the type of lesion, the presence and severity of associated anomalies, the vigor of the infant, pulmonary status, and the presence of infection. Prematurity, life-threatening congenital anomalies, sepsis, and respiratory compromise may necessitate delay of surgical treatment. Lung infiltrates, particularly those involving the left lung, usually require treatment before the operation. In general, the overall health of the infant should be considered when preparing for surgery, and the most serious abnormalities should be corrected first. WORKUPSection 6 of 12
Lab Studies
Imaging Studies
Diagnostic Procedures
Histologic Findings
TREATMENTSection 7 of 12
Medical therapyTo prevent mucous accumulation, aspiration, and respiratory deterioration continuous or intermittent low-pressure suction of the upper esophageal pouch should be initiated with a double-lumen Replogle catheter. In small infants, intermittent suction may be better. The infant should be positioned to minimize gastric fluid reflux. The infant is typically positioned in a 45-degree sitting position. In addition, infant handling should be minimized because excess disturbance may lead to further respiratory complications, increased oxygen consumption, cold stress, and increased regurgitation of gastric contents. Oxygen therapy should be administered as needed to maintain oxygen saturation. Endotracheal intubation is not performed routinely, but it may be required based on the infant's respiratory status. Bag-mask ventilation should be avoided because it may cause gastric distension leading to increased reflux. Intravenous fluid therapy consisting of 10% dextrose and hypotonic sodium chloride solution is used to maintain fluid, electrolyte, and glucose balance. Broad-spectrum antibiotics should be administered at the time of diagnosis or after cultures are obtained. A vitamin K analog should also be administered before surgery. Under no conditions should the infant be fed orally. If surgical treatment is delayed more than a few days, total parenteral nutrition is used. In addition, the infant should be transferred to a tertiary care pediatric institution with a neonatal intensive care unit and a pediatric surgery team. Surgical therapyThe operative procedure for infants with congenital esophageal abnormalities depends on the specific type of anomaly present, the condition of the infant, and the presence of other congenital anomalies. Gastrostomy The staged approach for patients with pure EA and in some infants with EA and TEF includes initial placement of a Stamm gastrostomy, followed later by fistula division and later esophageal reconstruction. Gastrostomies may cause problems in infants with EA and TEF, especially in premature infants with severe respiratory distress syndrome requiring positive-pressure ventilation. Because of the TEF, the infant's respiratory and upper gastrointestinal tracts function as a single unit. Therefore, the sudden decrease in intragastric pressure may result in preferential airflow through the fistula. Fistula ligation, occlusion of the fistula with a Fogarty catheter, and an underwater seal for the gastrostomy tube are methods used to maintain ventilatory pressure in these cases. Most surgeons perform a gastrostomy in the first 24 hours of life in an infant with pure EA. This allows enteral feedings while the child grows and the esophageal gap shortens. A gastrostomy may be used in premature or unstable infants with EA and TEF. Esophageal atresia with tracheoesophageal fistula Fistula division with primary anastomosis is the surgical treatment for EA with TEF. A posterolateral thoracotomy on the side opposite the aortic arch is used. The patient is typically positioned on the left side with a small axillary roll. The right arm is extended above the head with the neck slightly flexed. Typically, manual ventilation control is employed until the fistula is ligated. For infants in whom ventilation is difficult because of the passage of air through the fistula into the stomach, insertion of a Foley catheter through the fistula into the lower esophagus may be helpful. This can be done through a bronchoscope or performed retrograde through the stomach accessed with a laparotomy incision. A fourth intercostal extrapleural approach is employed through a transverse incision along the inferior angle of the scapula, from the anterior axillary line posteriorly to the paravertebral region. Although some surgeons prefer the transpleural technique for its speed, the extrapleural approach provides added protection against empyema should an anastomotic leak occur. The latissimus dorsi is divided, and the serratus anterior can be divided or reflected to protect its innervation. A muscle-sparing approach can be performed. The thorax is entered through the fourth intercostal space (ICS) by dividing the intercostal muscles. The extrapleural dissection begins posteriorly and proceeds superiorly, inferiorly, and finally anteriorly, where the risk of a pleural tear is highest. Wet cotton-tipped applicators, gauze swabs, moist peanut dissectors, or gentle finger dissection facilitates the pleural dissection from the chest wall. The azygous vein is identified, ligated and divided, or retracted (see Image 11). The lung and pleura are retracted medially. The distal esophageal segment is identified by following the right vagus nerve inferiorly, and the connection of the esophagus to the trachea is located. The lower esophagus is dissected circumferentially near the fistula taking care to not damage the vagal fibers or vascularization. Extensive mobilization of the distal pouch is not recommended because of its segmental blood supply. However, distal pouch mobilization may be necessary to achieve a primary anastomosis. The fistula is divided close to the trachea. A 1- to 2-mm esophageal cuff should be left on the trachea to minimize the risk of postoperative tracheal stricture. Interrupted 5-0 or 6-0 silk or polypropylene sutures are used to close the fistula. The air-tightness of the tracheal closure should be assessed by filling the chest with saline and looking for any bubbles when positive-pressure is applied by the anesthesiologist. Atraumatic handling of the distal esophagus is important. Two fine-stay sutures in the distal esophagus allow for mobilization and gentle traction without the use of forceps. The proximal segment is identified by gently advancing the Replogle tube, and traction sutures may be placed. The upper pouch can and should be mobilized all the way to the thoracic inlet. Circumferential mobilization to the thoracic inlet aids in identifying proximal fistulas. Care must be taken during the dissection of the proximal esophagus to avoid injury to the trachea or recurrent laryngeal nerve. A primary anastomosis should be performed whenever possible. If the esophageal segments cannot be joined without undue tension after mobilization of both ends, a circular myotomy may be performed on the proximal segment. A primary anastomosis is accomplished by opening the proximal pouch at the lower-most point. The opening created in the upper pouch should approximate that of the lower pouch. The distal segment is incised only if it is clearly devascularized or narrow and fibrous. Interrupted absorbable sutures are used to perform the anastomosis. Lateral stay sutures are placed. Ensuring mucosal apposition during the anastomosis is important. Usually, 5-6 sutures are needed to complete the posterior row. After all posterior sutures have been placed, the knots are tied on the inside of the esophageal lumen to prevent subsequent twisting of the esophagus. As the sutures are tied, tension gradually is distributed to the tied sutures. The lateral stay sutures are not tied. A small feeding tube can be passed across the anastomosis into the stomach to ensure luminal patency and protect the posterior wall of the anastomosis while the anterior wall is sutured. Some surgeons leave the tube in postoperatively to decompress the stomach and provide a postoperative feeding route (see Image 20). The anterior suture layer of the anastomosis is then completed over the tube, and the knots are tied on the outside (see Image 13). A muscle or tissue flap may also be placed between the anastomosis and the repaired trachea to decrease the risk of fistula recurrence. Most surgeons place a retropleural chest tube with the tip positioned near, but not touching, the anastomosis (see Image 21). The tube is placed to water seal drainage to avoid an extrapleural pneumothorax. Suction applied to the tube may disrupt the anastomosis. Intubation should be maintained as is clinically required. Premature extubation that results in aggressive bag-mask ventilation and reintubation can be disastrous to the repair. Premature and medically unstable children can be maintained on total parenteral nutrition with a tube in the proximal pouch until surgery. Emergent thoracotomy and fistula ligation is used for infants with respiratory distress when nonoperative management is unsuccessful. Recently, thoracoscopic techniques have been used to repair tracheoesophageal atresia and distal fistula. Esophageal atresia with proximal fistula The repair for a proximal fistula is the same as that described above. If a proximal fistula exists with no distal fistula, a cervical approach may be used instead. Pure esophageal atresia Most children with pure EA undergo gastrostomy placement followed by delayed repair. Following a period of growth, repair of EA without fistula consists of a thoracotomy with retropleural dissection, mobilization of the 2 esophageal segments, and a primary anastomosis (see Images 12-13). Esophageal continuity can usually be achieved without an esophageal replacement. Several techniques are used to lengthen the esophageal ends to achieve a primary anastomosis. Bougienage is the most common mechanical lengthening procedure. Upper pouch bougienage is performed by passing a weighted bougie through the mouth into the upper pouch and applying forward pressure 1-2 times daily. This procedure is performed for 6-12 weeks followed by a delayed primary repair. Both internal and external traction sutures have been used by some surgeons. During surgery, lengthening techniques may also be used. Myotomy is a common method that provides a 1-cm increase in length. The muscular layers are divided to create a plane between the muscularis propria and submucosa. Circular myotomies involve a circumferential division, while spiral myotomies preserve the muscular continuity of the proximal pouch and maintain closed submucosal layers. Up to 3 myotomies can be performed. The most proximal myotomy should be performed first. This procedure can be performed on both esophageal segments, but myotomies performed on the distal stump may increase the incidence of gastroesophageal reflux. When additional length is necessary, a portion of the stomach may be brought up through the diaphragmatic hiatus. This procedure has been used successfully, even in infants with low birth weight. A Collis lengthening procedure may also be used. A cervical esophagostomy is used when an anastomosis is impossible and in cases of failed surgery. For this procedure, a left transverse incision is performed 1 cm above and parallel to the medial third of the clavicle. The incision is deepened through the platysma. The sternal head of the sternocleidomastoid is divided, the sternothyroid muscle is divided or reflected, and the carotid sheath is retracted. The esophagus is mobilized circumferentially and dissected distally. The esophageal end is brought out to the lateral end of the skin incision and sutured with interrupted absorbable sutures. This procedure allows the child to swallow normally. Sham feeds are administered to stimulate lengthening by a natural bougienage effect and to avoid oral aversion. The infant may be discharged home on gastrostomy feedings until esophageal repair or replacement can be performed. A variant of this method is the multistaged extrathoracic elongation, which has also been used in long-gap treatment. In this procedure, the upper esophagus is initially mobilized and brought out as an end cervical esophagostomy. Over a period of weeks to months, the esophagus and stoma are progressively translocated down the anterior chest until adequate length is achieved to permit an end-to-end anastomosis. Again, the child is able to swallow and can be discharged home during the intervening period. Recently, the use of short-term traction sutures to narrow the esophageal gap has been described. Esophageal replacement Esophageal substitutions are used to restore esophageal continuity when the patient's native esophagus is not an option. No esophageal replacement is ideal, and a poorly functioning esophagus may even be preferable to any esophageal substitute. Because of advancements in the surgical techniques to treat EA, the need for esophageal replacement has diminished; however, conditions that necessitate an esophageal substitution still exist. Many types of conduits have been used, including colon, stomach, gastric tubes, and jejunum. The choice of substitute to be used is influenced by the length and segment of esophagus to be replaced, the presence of any associated anomalies, and the vascular adequacy of the proposed replacement. Most often, the surgeon's preference and experience contributes to the selection.
Isolated tracheoesophageal fistula H-fistulas or N-fistulas are typically located at the T1-T3 level, coursing downward from the trachea to the esophagus. Most can be repaired through a cervical approach. An undiagnosed H-type fistula may also be identified while mobilizing the proximal esophagus during surgery for EA (see Image 15). A right-sided supraclavicular incision 1-1.5 cm above and parallel to the right clavicle is made to minimize risk of injury to the thoracic duct. The sternocleidomastoid muscle is retracted posteriorly, and the sternal head is divided if necessary. The inferior thyroid artery and middle thyroid vein are divided if needed to expose the plane between the trachea and the esophagus. Care should be taken to clearly identify and preserve the recurrent laryngeal nerves, vagal nerve fibers, and posterior trachea. The fistula may be located higher than might be expected. The esophagus is encircled with rubber vessel loops to facilitate mobilization. The fistula is also encircled when it is identified (see Image 15). The fistula is divided close to the esophagus, leaving a 2-mm esophageal cuff on the trachea. Interrupted sutures are used to close the esophagus and trachea (see Image 16). A muscle flap may be interposed between the 2 suture lines to decrease the likelihood of a recurrent fistula. Fistula ligation without division should not be performed. Wound drainage is typically not necessary for an H-fistula repair, and the endotracheal tube should be left in because tracheal swelling is a frequent postoperative occurrence. Congenital esophageal stenosis, webs, and tracheobronchial remnants Treatment should relieve the obstructive symptoms and maintain the antireflux mechanism of the gastroesophageal junction. Bougienage or balloon dilation may successfully treat fibromuscular hypertrophy. Membranous webs, complete occlusion, and tracheobronchial remnants usually require surgical excision (see Images 18-19). A right thoracotomy is usually used. Lesions in the abdominal esophagus can be approached through the abdomen. A segmental resection and primary anastomosis can be achieved in most instances (see Images 18-19). The phrenic and vagus nerves should be identified and preserved. Esophageal replacement may be needed for long segments of fibromuscular hypertrophy. Postoperative detailsPostoperative care of an infant with EA involves a team approach, typically in the neonatal intensive care unit. A chest radiograph is obtained immediately following surgery (see Images 20-21). The infant should be kept in a semiupright position, and the patient's head should be supported to prevent neck extension, which may disrupt the anastomosis. Intravenous fluid replacement should be maintained, and prophylactic antibiotic treatment should be continued. The pharynx should be frequently suctioned to prevent respiratory infection, but deep suctioning should be avoided. Appropriate temperature, humidity, and oxygen atmospheric control are essential. If the anastomosis was performed under extensive tension, some surgeons recommend elective paralysis and mechanical ventilation for several days postoperatively. Otherwise, the patient is weaned from the ventilator as soon as possible. Contrast esophagraphy is performed postoperatively to assess for esophageal leak, stricture, motility, and gastroesophageal reflux (see Image 22). The swallowing reflex and positions of the duodenum and ligament of Treitz should also be examined. The timing of the initiation of feeding varies. Some advocate starting gastrostomy or nasogastric feedings on the first or second postoperative day in uncomplicated cases. Other surgeons advise against gastrostomy or nasogastric tube feedings because of the potential for acid reflux and injury to the anastomosis. Total parenteral nutrition should be used when enteral feedings are not started. If no leaks are observed on the postoperative contrast study, feedings are initiated and the chest tube is removed. The child may be discharged when feedings are tolerated and appropriate weight gain is observed. Follow-upFrequent follow-up visits are necessary during the first year after repair. If the child is doing well, visits can be decreased to 1-2 times per year until school age. Because of scarring at the anastomosis, the child may tolerate only pureed food up to age 12-18 months and then minced food until age 5 years. At age 5 years, the child has typically learned to chew well before swallowing and has developed sufficient teeth to aid in this task. The child's parents should be informed about the signs of gastroesophageal reflux, recurrent fistula, tracheomalacia, and other complications. COMPLICATIONSSection 8 of 12
The severity of complications following esophageal surgery is often dictated by the extent of the repair. Anastomotic tension is involved in 79% of complications, and the most common complications include anastomotic leak, recurrent fistula, stricture, and gastroesophageal reflux. Anastomotic leakage occurs in anywhere from 14-21% of children that have undergone a surgical EA repair (see Image 23). Leaks result from the small friable lower segment, ischemia of the esophageal ends, excess anastomotic tension, sepsis, poor suturing techniques, and inaccurate mucosal apposition. Early extubation with reintubation also puts infants at increased risk of anastomotic leakage. Most leaks are small, occur late after the first 48 hours, and require only conservative management. Chest tube drainage, antibiotics, and time allow most to heal. Spontaneous healing occurs in 95% of leaks when a mediastinal drain is present. A repeat esophagraphy is performed each week until the leak has resolved. More significant leaks occur early, within the first few days, and should be explored immediately in most cases. Major anastomotic disruptions account for only 3-5% of leaks. Large leaks can be fatal or may lead to fistularecurrence. Fistula recurrence is observed in 3-14% of patients treated for EA-TEF or isolated TEF. Fistulas usually recur within a few months, but they may be found as late as 2 years postoperatively. Fistula recurrence is caused by anastomotic leak with local inflammation and erosion at the previous repair site, ischemia, and surgical dissection too near the trachea. Recurrent TEF should be suspected when choking episodes occur during feeding and/or recurrent pneumonia is observed. Esophagography under video fluoroscopy with the patient in the prone position or bronchoscopy provide the best methods of diagnosis. Routine contrast swallows do not reveal 50% of recurrent TEFs. Fistulas do not close spontaneously and require surgical division and suturing. Recently, attempts have been made to close recurrent fistulas with fibrin glue administered into the fistula. During surgical repair, a tissue flap should be interposed between the suture lines of the trachea and esophagus. Recurrence rates remain in the 10-20% range when repeat surgery is needed. Esophageal strictures are also common following esophageal surgery. Anastomotic strictures occur in up to 40% of children with an EA repair (see Images 24-29). Strictures can result from the natural healing process, the different sizes of the 2 anastomosed segments, tension, gastroesophageal reflux, and leaks. Asymptomatic narrowing observed on initial esophagography can improve over time without the need for intervention (see Images 24-26). Strictures are suspected clinically, and the diagnosis can be confirmed via contrast esophagraphy and esophagoscopy (see Images 27-29). Most strictures can be managed with serial dilatations. Multiple dilatations over several months are needed in many cases. Strictures unresponsive to dilation require surgical resection. Gastroesophageal reflux is a common complication of esophageal surgery, occurring in 40-70% of patients undergoing EA repair. Symptoms of gastroesophageal reflux include coughing, apnea, recurrent pneumonia, failure to thrive, and stricture formation (see Images 28-29). Reflux is thought to be related to tension, dysmotility of the lower esophagus, and an altered angle of His from distal esophageal mobilization. Tracheomalacia is a condition in which weakness of the trachea results in compression of the anterior and posterior walls between the aorta and dilated esophagus during expiration or coughing. This complication occurs more frequently in the presence of a fistula and is present in 10-20% of infants after an EA-TEF repair. The region of compression is typically located at or just above the level of the original fistula but may involve the entire trachea. The marked tracheal anterior-posterior collapse is observed easily during bronchoscopy performed while the patient is awake. Tracheomalacia usually improves slowly with time. In some cases, tracheomalacia may prevent extubation, and intervention with aortopexy, tracheostomy, or tracheal stenting is needed. Esophageal dysmotility is frequent following surgery for congenital esophageal lesions. Food impaction can occur at the level of the anastomosis, especially if a stricture is present (see Image 30). Altered esophageal peristalsis has been documented with manometric and radionuclide studies, video fluoroscopy, scintigraphy, and cine esophagraphy after EA repair. Discontinuity of peristaltic function is observed above and below the surgical anastomosis. Dysmotility appears to persist and has been reported in 32-year follow-up studies. Children learn to compensate for the dysmotility by eating in an upright position and drinking frequently during eating. Esophageal diverticula may develop at the anastomosis or a site where a circular myotomy was performed. The myotomy site may balloon progressively over time and cause ventilatory obstruction and dysphagia. OUTCOME AND PROGNOSISSection 9 of 12
The survival rate of patients with EA and/or TEF has improved immensely since Haight's first successful repair in 1941. Early diagnosis and advancements in neonatal anesthesia, surgical technique, treatment of associated anomalies, and intensive care management have improved the prognosis. Most children treated for EA have a normal lifespan. Despite an increased number of patients with severe congenital anomalies, survival rates have been reported as high as 95%. In uncomplicated cases, survival rates are virtually 100%. Traditionally, prognosis for children with EA-TEF was based on birth weight and the presence of pneumonia and associated congenital anomalies. Because of advancements in neonatal care, birth weight does not affect survival rate unless it is severely low, and pneumonia may be treated successfully. Currently, cardiac and chromosomal abnormalities are the most significant causes of death. Infants with a birth weight less than 1500 g, major congenital cardiac abnormalities, severe associated anomalies, preoperative ventilator dependence, and/or long gap are at increased risk. Dysphagia, frequent night coughs, dyspepsia, and recurrent respiratory infections are frequent results of the less distensible esophagus and gastroesophageal reflux. Gastroesophageal reflux occurs in up to one half of these patients and many require antireflux operations. Feeding difficulties also are common, particularly during the first several years after repair. Choking, vomiting, and food impaction occur. These symptoms, like many following EA repair, diminish over time, and 70-80% of adolescents report no or only occasional swallowing impairment. Most patients who have undergone EA repair have abnormal peristalsis with decreased contractile activity and inefficient clearance capacities. In one series, after an average of 8.8 years of follow-up care, all patients were reported to eat excellently or satisfactorily, with more than 90% eating no differently than their siblings. Normal respiratory function is observed in half of patients 3 months postoperatively. Tracheomalacia, vascular rings, and decreased lung volumes account for the abnormal respiratory function in the other children. Tracheomalacia occurs in 10% of patients with TEF. Most outgrow this problem; however, some children require more aggressive therapy. Growth retardation has been observed in some children who have had EA repair, but this observation is variable. Patients treated for EA-TEF are at higher risk for developing esophagitis and Barrett epithelium. Reports of esophageal carcinoma decades after EA-TEF repair are becoming more frequent as the first generation of survivors progresses through adulthood. Surveillance esophagoscopy has been proposed to provide early detection for esophageal abnormalities. Despite the complications, the results of EA-TEF repair have improved dramatically. Many symptoms are alleviated with time, and most children and adults enjoy normal lifestyles and have no complaints concerning their quality of life or eating habits. Even by school age, children who had many complications in infancy reported few restrictions at school or in participation in sports with little or no effect on school attendance and social activities. The outcome for these children and children treated for other congenital lesions is generally good. FUTURE AND CONTROVERSIESSection 10 of 12
The prognosis and treatment course for infants with EA and/or TEF and other congenital lesions has improved over the past 60 years. Advances in perinatal and neonatal care have been paramount in reducing the morbidity and mortality rates associated with these conditions. Currently, associated congenital anomalies and pulmonary complications contribute most significantly to adverse outcomes. Infants with very low birth weight or serious cardiac abnormalities are at increased risk for poor outcome. Improvements in the prevention and management of these high-risk infants would improve outcome and survival rates. In addition, enhanced prenatal detection of EA and/or TEF and other congenital anomalies allows for better prenatal counseling and preparation for the delivery at a tertiary medical center. Esophageal defects are currently repaired with thoracoscopy; robotic-assisted surgery may be used in the future. Tissue engineering for esophageal replacement, in utero intervention, and minimally invasive techniques such as thoracoscopy and robotic assistance may be used in years to come to improve treatment of these infants further. MULTIMEDIASection 11 of 12
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