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Seediscussions,stats,andauthorprofilesforthispublicationat: Esophageal


Article in BoletíndelaAsociaciónMédicadePuertoRico·January2010







Source:PubMed CITATIONS 4 READS 1,652 2authors ,including: HumbertoLugo UniversityofPuertoRico,MedicalSciencesCampus 55




Review Articles - Artículos de Reseña


Esophageal atresia is the most com- mon congenital anomaly of the esopha- gus in newborns. This review article discusses the incidence, embryologi- cal classification, diagnosis and asso- ciated anomalies of esophageal atre- sia. Emphasis is placed in the current guidelines of standard surgical mana- gement of this congenital condition.

Index words: esophagus, atresia, gui- delines, management



Jessica González-Hernández MS* Humberto Lugo-Vicente MD**

From the * UPR School of Medicine, and ** Section of Pedia- tric Surgery, Department of Surgery, UPR School of Medici- ne, Puerto Rico Health Science Center, San Juan, PR. Address reprints requests to: Humberto Lugo-Vicente MD – PO Box 10426, San Juan, PR 00922. E-mail: titolugo@


Esophageal Atresia (EA) is the most common congenital anomaly of the esophagus. Most cases of EA are associated with tracheo-esophageal fistula (TEF). More than three centuries has elapsed since the first reported case. Following Leven, Ladd and Haight adversities to accomplish successful repair of this de- fect, the morbidity and mortality of EA has improved in response to the development of neonatal intensive care units (NICU), use of parenteral nutrition, better surgical techniques, improved antibiotics and prenatal diagnosis. 1-4


EA occurs in one of every 2500 to 5000 live bir-

ths with a slight male predominance.5-7 Risk of having

a baby with EA increases with the first pregnancy, older mother and twin pregnancy. Familial cases have been

reported. 1, 6, 8, 9 Teratogenic influence has been identi- fied after prolonged use of estrogen, progesterone or thalidomide during pregnancy, and infants born to dia- betic mothers. An experimental model of EA was de- veloped in 1996 by exposing fetal rats to Adriamycin. In 2004, the Adriamycin rat model was used to make

a correlation between EA, amniotic fluid volume varia- tions and other visceral malformations. 10

Chromosomal abnormalities are frequent in children with EA. They include Trisomy 13, 18 (Edward) and 21 (Down) along with the VACTERL association. 7,

11, 12 A deletion of band 11.2 of the long arm of chromo- some 22 has also been associated with EA. 13 Recently,

a deletion in chromosome band 17q22-q23.2 was as-

sociated with esophageal atresia, tracheoesophageal fistula and conductive hearing loss. 14


The esophagus and trachea develop from the foregut bud. During the fourth week of fetal develop- ment the trachea forms as a ventral diverticulum of this bud. Abnormal embryogenesis of the laryngeo-tracheo- esophageal groove and subsequent septum formation allows persistent communication between the trachea and esophagus. The distal esophagus elongation oc- curs with the heart and lung development reaching a maximum length by the seventh week. The proximal- third striated muscle develops from the pharyngeal pouches and its blood supply comes from the thyrocer- vical trunk. Aggressive surgical dissection of this por- tion is permitted due to the submucous blood supply. The middle and distal esophagus has smooth muscle derived from dorsal mesenchymal tissue and its blood supply arise from segmental roots of the aorta. 15 Ag- gressive surgical dissection of this segment leads to ischemia.

Published evidence using the Adriamycin rat model has revealed that EA forms as a blind-ending pouch and the distal TEF develops as the middle branch of a tracheal trifurcation. The distal foregut anlage is switched toward a pulmonary phenotype, it trifurcates and its middle branch grows caudally to fistulized into the stomach. 16 This theory encompassing a respiratory origin to the distal esophagus explains the poor motility observed, the presence of airway cartilage in the histo- logy of the lesion and presence of ectopic esophageal lungs.

Several variations of EA have been described (see Figure 1). Most common variant anomaly (87%) is proximal EA associated with distal TEF. The proximal esophagus is hypertrophied and dilated from amniotic



liquid pressure. Impingement on the trachea by the dilated pouch is the cause of faulty cartilaginous ring development also known as tracheomalacia. Second most common anomaly is pure EA (8%) associated with a poorly developed distal esophagus. Following in frequency is isolated TEF. The other variants are insig- nificant and usually diagnosed intraoperatively. 6


Prenatal diagnosis of EA using ultrasound relies on the finding of a small or absent fetal stomach bubble associated with maternal polyhydramnios. Using these criteria’s, the positive predictive value is 56% and the sensitivity of establishing the diagnosis 42%. 17-18 Po- lyhydramnios is most commonly seen in pure EA. Vi- sualizations of the blind-ending esophagus during fetal swallowing, known as the pouch sign, can also lead to prenatal diagnosis. 17, 19-21 Maternal alpha fetoprotein le- vels has brought inconclusive evidence in the prenatal diagnosis of EA. 22

Most babies born with EA have symptoms since the first hours of birth. They consist of excessive sali- vation (mucosity), first feeding causing reflux, asphyxia and cough followed by cyanosis, respiratory distress and inability to pass an orogastric tube. The abdomen could be either scaphoid or distended depending on the presence of a distal TEF. The diagnosis of EA is confirmed after watching a coiled orogastric tube in the proximal esophagus in simple chest x-ray films. Con- trast studies are rarely needed and of potential disaster (aspiration). If in doubt a very small quantity (0.5 to 1 cc) of diluted barium can be instilled through the tube to delineate the proximal esophageal pouch. A cons- tant distance of 10-11 cm between the nares and proxi- mal esophageal pouch is most often found. Abdominal films should be obtained to rule out the occurrence of associated gastrointestinal anomalies. Other diagnos- tic modalities include esophagoscopy, fluoroscopy and bronchoscopy. The presence of air in the gastrointesti- nal tract suggests that a distal TEF is present, whereas absent air makes the diagnosis of pure EA.1 Due to the high incidence of associate malformations in children born with EA, an initial evaluation should screen for other defects using echocardiogram to exclude ductal- dependent lesions, preoperative renal ultrasound if the child has not voided and chromosomal analysis.

Congenital isolated TEF brings problems du- ring early diagnosis and management. More than H- type is N-type, due to the obliquity of the fistula from the trachea (carina or main bronchi) to esophageal side (see Figure 2) anatomically at the level of the neck root (C7-T1). Pressure changes between both structu- res can cause entrance of air into the esophagus, or esophageal content into the trachea. Thus, the clinical manifestation that we must be aware for early diagno- ses are cyanosis, coughing and choking with feedings, recurrent chest infections, persistent gastrointestinal distension with air, and hypersalivation. 17 Diagnosis is confirmed with an esophagogram, or video-esopha- gogram (high success rates, establish level of the TEF). Barium in the trachea could be caused by as- piration during the procedure. Upon radiologic doubt,

bronchoscopy should be the next diagnostic step. De- lay in surgery is generally due to delay in diagnosis rather than delay in presentation. 23


The pathogenetic mechanisms that produce EA also affect other systems. Between 50 and 70% of children born with EA have associated congenital anomalies that affect prognosis and survival. Cardio- vascular anomalies are the most commonly found (29%), followed by genitourinary (14%), gastrointes- tinal (13%), skeletal (10%) and chromosomal defects (4%). 24 Neurologic anomalies associated include neu- ral tube defects, hydrocephaly, holoprosencephaly and macrophthalmia. Other less common anomalies are choanal atresia, cleft lip, abdominal wall defects and diaphragmatic hernias. The association of two or more system anomalies and the severity of associated ano- malies influence mortality in esophageal atresia. 3, 6, 24,


In 1973, the VACTERL association was descri- bed. VACTERL is the most common association (14- 36%) seen in EA and includes:

Vertebral anomalies

Anal malformations

Cardiac malformations

• Tracheo-Esophageal fistula (must be one of the as-

sociated conditions)

Renal deformities

Limb radial defects.

Other associations included within EA include CHARGE (coloboma, heart disease, retarded develop- ment/growth or central nervous system abnormalities, genital hypoplasia or hypogonadism and ear abnorma- lities or deafness), Downs, Potter, Fryns, Fanconi syn- drome and others. 3, 24, 26

Cardiac defects determine mortality. Risk of death in an infant born with EA and a cardiac defect is 30%. The most common cardiac anomaly is ventricular septal defect. Other cardiac defects include tetralogy of Fallot, patent ductus arteriosus and anomalies of the aortic arch. 8, 27 In infants with non-duct-dependent cardiac anomalies, repair of the esophagus takes pre- cedence whereas in ductal-dependent lesions, tempo- rary control can usually be achieved with prostaglan- din E. 6 Genitourinary anomalies include hypospadia, undescended testis, renal agenesis, hydronephrosis, vesico-ureteral reflux and ambiguous genitalia. The most common gastrointestinal anomalies include im- perforate anus, duodenal atresia, malrotation, annular pancreas and pyloric stenosis.

Several deformities of the respiratory system associated with EA can result in a fatal outcome. They include an ectopic superior right bronchus associated with a trifurcated trachea or tracheal bronchi, congenital



bronchial stenosis, tracheal, laryngeal and pulmonary agenesis. 23 Because these abnormalities may be re- lated to the occurrence of respiratory disorders such as tracheomalacia and atelectasis, early bronchosco- pic examination may be useful in patient with predomi- nantly respiratory symptoms.

Chromosomal anomalies such as trisomy 18 and 21 are rarely seen. The correction of EA in children with Trisomy 18 brings challenging ethical questions that should be discussed with the parents.


Preoperative Management

Repair of EA is not an emergency. Time should be taken to optimize the physiologic state of the baby before urgent repair. This includes secure the airway, avoid further aspiration and manage the associated pneumonitis (chest physiotherapy). An orogastric tube (Replogle) set to low intermittent suction can remove secretions from the upper esophageal pouch. The child should be placed in a semi-sitting position to avoid gas- tric content travel from the stomach to the lungs through the TEF. Next, intravenous fluids and broad-spectrum antibiotics should be administered. Lab work-up should include hemogram, serum electrolytes, arterial blood gases, and type and cross match for 20 cc/kg of weight of packed red blood cells. Informed consent should be obtained before surgery.

All patients with esophageal atresia should have an echocardiogram prior to surgery to assess the position of the aortic arch. Operative repair should be done through the contralateral side of the aortic arch. 6

Premature babies with EA and respiratory dis- tress from hyaline membrane disease in need of me- chanical ventilation are a special group. Inefficient ventilation due to preferential escape of air through the TEF in the presence of stiff lungs exposes the child to gastric perforation, abdominal compartment syndrome and hypoxia. Temporary balloon occlusion of the dis- tal esophageal segment has brought conflicting results while emergent ligation of the TEF is the best manage- ment alternative for the affected child (see Figure 3). 6,


Intraoperative Management

As management for infants with esophageal atresia has markedly improved, new surgical techni- ques have been developed. 29 In addition to the conven- tional open repair, a minimally invasive thoracoscopic repair was first successfully done by Lobe and Rothen- berg in 2002. 30-31 Many studies have been conducted to establish the thoracoscopic repair in the new stan- dard of care. 32-34 Studies have concluded that thoracos- copic repair is safe and comparable with conventional open repair of esophageal atresia in terms of short-term outcomes such as operative time, postoperative leak and stricture rates. 32 Long-term benefits of the thoracos- copic repair needs to be further assessed since a pu- blished study has failed to reveal the advantage in terms of postoperative esophageal motor function. 33

in terms of postoperative esophageal motor function. 3 3 Figure 1. Types and Frequency of Esophageal

Figure 1. Types and Frequency of Esophageal Malfor- mations

Figure 1. Types and Frequency of Esophageal Malfor - mations Figure 2. Isolated TEF Figure 3.

Figure 2. Isolated TEF

of Esophageal Malfor - mations Figure 2. Isolated TEF Figure 3. Prematurity, Hyaline Membrane Disease and

Figure 3. Prematurity, Hyaline Membrane Disease and Inefficient ventilation in a child with EA and TEF

Intraoperative management depends on type of defect and associated malformations. The final ob- jective is to establish bowel continuity while preserving the native esophagus of the child.

The standard of care remains the open thora- cotomy repair and at the operating room, the manage- ment of EA with distal TEF entails the following guideli- nes:



1. Thoracic approach should be contralateral to the aortic arch, most often this means a right thora- cotomy. A high axillary skin crease incision allows unrestricted access to the posterior mediastinum, carries minimal morbidity and provides excellent scar cosmesis. 35-36

2. Whenever possible preservation of the latissimus dorsi and serratus anterior muscles should be done (muscle-sparring thoracotomy) to avoid shoulder deformity, scoliosis, and winged scapula.

3. Extrapleural approach to the posterior mediasti- num is preferred since a rib does not have to be removed. Anastomotic leaks using the extrapleural approach are better tolerated.

4. Division of the azygous vein near the superior vena cava entrance to help dissect the TEF is operator dependant since many surgeons prefer not to liga- te the azygous vein. The TEF is watertight ligated with small absorbable sutures (6-0 PDS or Vicryl).

5. Anesthesiologist will help identify the proximal esophageal stump while exerting pressure with an oro-esophageal tube. The proximal esopha- geal stump can be aggressively dissected up the neck, but avoid using the cautery since recurrent laryngeal nerve damage is a possibility. The distal esophageal stump is dissected carefully since its blood supply is more precarious.

6. A primary esophageal anastomosis between both stumps can be accomplished most often using one layer full thickness interrupted absorbable sutures. Around eight stitches are needed. The surgeon must develop judgement on the tension exerted when performing the anastomosis. A small 5 Fr. trans-anastomotic tube is placed in the stomach for drainage and early gavage feeding. Once finished a chest drain is left near the anastomosis and pla- ced to underwater sealing suctioning (see Figure


7. Intercostal closure and muscle apposition follows with transfer of the baby to the NICU.

Management of the baby born with pure esopha- geal atresia remains a challenge since it depends on the distance (gap) between the esophageal stumps. There are different alternatives of treatment but an in- dividually decision should be made for each patient. Initially, a gastrostomy is done. This will help start fee- dings and permit measurement of the gap distance. The proximal esophageal stump can be serially dilated using Bakes metal dilators, while bolus gastrostomy feeding will help the distal esophageal stump to grow (see Figure 5). Meticulous nursing care is needed to avoid respiratory problems from chronic saliva aspira- tion. Once a gap of one centimeter is obtained between the esophageal stumps, an anastomosis can be done.

Several techniques are described to gain leng- th and accomplish a tension free anastomosis during repair, such as circular or spiral myotomy, flap esopha- goplasty or extrathoracic extension, all performed on the proximal esophageal pouch. 6, 8 Esophageal repla- cement can be accomplished using preferably colon, stomach or jejunum discussion of which is beyond the scope of this review. It has been proved that patient un- dergoing primary repair had generally better outcomes

un- dergoing primary repair had generally better outcomes Figure 4. Surgical Repair of EA with TEF

Figure 4. Surgical Repair of EA with TEF

better outcomes Figure 4. Surgical Repair of EA with TEF Figure 5. Long gap EA, suction

Figure 5. Long gap EA, suction catheter in upper pouch and gastrostomy

than those who underwent an esophageal replace- ment. 37 Primary repairs in long gap esophageal atresia would cause severe anastomotic tension and would increase the risk of leakage and stricture formation. A new approach has surged for these patients, “growth induction” of the esophageal stumps by internal or ex- ternal traction. A staged esophageal lengthening has been achieved requiring three operations but resulting in an esophagus-only repair with the gastroesophageal junction below the diaphragm. 38 Further studies have demonstrated the efficacy of the growth procedure to treat the full spectrum of EA including those with most rudimentary lower segments and the longest gaps. The long gap-EA patients treated by the growth procedure and an early active course of dilations and treatment of GE reflux seem indistinguishable from the much more favorable short gap EA/TEF patients. 39

Isolated TEF management consists of surgi- cal closure through a right cervical approach. A small guide-wire threaded through the fistula during bron- choscopy helps identified the fistulous tract. Working in the tracheo-esophageal groove can cause injury to the recurrent laryngeal nerve with subsequent vocal cord paralysis. Recurrence after closure is rare. 40



Postoperative Care

With moderate or severe tension on the repair, the child benefits from postoperative prophylactic pa- ralysis and mechanical ventilation to avoid neck move- ment, further tension, breakage or leak of the anasto- mosis. 6 The chest tube is left until it clogs or patency of the esophagus is documented. The presence of pneumothorax or saliva in the chest tube suggests an anastomotic leak. Gavage feedings through the tran- sanastomotic tube can be started after the third posto- perative day. A barium swallow is performed seven to ten days after the repair to document the presence of leak, stricture, dysmotility and gastroesophageal reflux (GER).

Since esophageal stricture is the one most fea- red complication after esophageal repair, published studies have tried to determine whether routine ba- lloon dilatation of the anastomosis after repair of an esophageal atresia with distal fistula is superior to wait and see policy with dilatation only when symptoms ari- se. Studies have reported that dilatation on demand results in fewer procedures, fewer hospitals days and likely lower cost. 41-42


Repair of EA and TEF can be plagued with early and late morbidity. The three most common anastomo- tic complications are in order of frequency: stricture, leakage and recurrent TEF. Late complications include gastroesophageal reflux (GER), tracheomalacia and dysmotility. 43

Anastomotic strictures (or stenosis) are a common finding after repair of EA. Incidence can be as high as 50%. Predisposing factors include the use of breaded silk, thepresence of GER and leakage of the anastomosis. Strictures are associated with cho- king, frequent respiratory infections and foreign body impaction. Most children respond to dilatation. Tho- se associated to GER should be fundoplicated. 4, 17, 44

Esophageal leaks occur in 10-15% of all ca- ses. Leaks that occur during the first 72 hours after repair present as tension pneumothorax. An immediate esophagogram should be done with water-soluble ma- terials to exclude a complete esophageal breakdown that might need repair, esophageal exclusion and/or gastrostomy. Late leaks are usually small, present with saliva in the chest drain and close spontaneously with proper drainage and nutrition. 17

Recurrent TEF after surgical repair for EA oc- curs in approximately 3-15% of cases. Tension on the anastomosis followed by leakage may lead to local in- flammation with breakage of both suture lines enhan- cing the chance of recurrent TEF. Once established, the fistula allows saliva and food into the trachea, therefore clinical suspicion of this diagnosis arises with recurrent respiratory symptoms associated with feedings after repair of EA. Diagnosis is confirmed with cineradio- graphy of the esophagus or bronchoscopy. A second thoracotomy is very hazardous, but is the most effecti- ve method to close the recurrent TEF. Either a pleural

or pericardial flap will effectively isolate the suture line. A pericardial flap is easier to mobilize, provides suffi- cient tissue to use and serves as template for ingrowth of new mucosa should leakage occurs. Other alternati- ves are endoscopic diathermy obliteration, endoscopic chemocautherization, laser coagulation, or fibrin glue deposition. 40, 45-47

GER is commonly identified after successful re- pair of EA. Constant gastric bathing of an esophageal anastomosis causes inflammation, edema and strictu- re formation. GER has been implicated in development of esophageal leaks in the immediate postop period. The presence of GER in EA is associated to shortening of the esophagus and vagal nerve denervation during repair. Stricture development, recurrent pneumonia, failure to thrive, Barrett epithelium and severe esopha- gitis are indications for fundoplication. 4, 17, 48-51

Tracheomalacia refers to a structural/functio- nal generalized or localized weakness of the tracheal rings' support resulting in partial respiratory obstruction. Most cases are associated with EA and as such flaccid tracheal development after external pressure from the dilated proximal blind esophageal segment has been proposed as pathogenetic mechanism. Most cases develop expiratory obstruction since only the intratho- racic trachea if affected. The harsh barking cough is the most characteristic initial symptom. Nutritional pro- blems are the result of difficulty breathing as cyanotic attacks might occur during feeding. Other incitatory elements are intercurrent respiratory infections and as- piration. Severe forms are characterized by life-threa- tening apneic spells, inability to extubate the airways, and episodic pneumonia. A cough and wheeze may progress to complete airway obstruction and cyanosis. Diagnosis is obtained with simple lateral thoracic films (narrow slit-like appearance), bronchoscopy during spontaneous breathing (antero-posterior narrowing in expiration), cinetracheobronchography (allows extent of tracheal collapse) or cine CT studies. Reflux must be rule out and manage aggressively. For mild to mo- derate symptoms no management is necessary as the child will improve with time. For severe life threatening tracheomalacia aortopexy must be undertaken. Failed aortopexy may need tracheal reinforcement with auto- logous cartilaginous grafts. 6, 8, 52


Survival of EA has improved dramatically du- ring the past fifty years. A general survival rate of 80 to 95% can be accomplished when children with severe associated malformations are excluded. The original Waterston’s risk classification has yielded to several modifications. High-risk patients are those with very low birth weight (less than 1500 grams), associated cardiac anomalies, chromosomal defects, ventilator dependant and long gap.

Although the long-term outcome of EA patients seems favorable, respiratory and gastrointestinal symp- toms plus functional abnormalities remain frequent. One third of long-term patients report having impaired qua- lity of life because of respiratory infections, dyspnea,



difficulties in swallowing and coughing at night. 53 Al- most 20% of patients have GER symptoms. The rate of symptoms decreases with age. Gastric metaplasia and esophagitis and the high rate of tracheal, esophageal, and gastric inflammation suggest a need for long-term follow-up. Gastric metaplasia is a premalignant lesion of the esophagus. 51, 54 Results can be classified as ex- cellent if the child is totally asymptomatic, good if he has occasional difficulty eating some foods. Children with frequent deglutition or respiratory problems are rated as regular results.

Growth and development after successful EA repair remains within the 50th percentile. Children with EA have more learning, emotional, and behavioral pro- blems than children in the general population. A high- risk group of children with major associated congenital anomalies, who had been ventilated as a newborn, were at special risk for cognitive problems. 55 Children with esophageal replacement procedures fare worst that primarily repair cases. These children develop anastomotic strictures, ulceration, bleeding, redundan- cy and bezoar formation more commonly.


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Atresia esofágica es la condición congénita más co- mún del esófago en recién nacidos. Este articulo de reseña discute la incidencia, clasificación embrioló- gica, diagnostico y anomalías asociadas en atresia del esófago. Se establece énfasis en los principios actualizados del manejo quirúrgico de esta condi- ción congénita.