Tamara M. Meeker, MSN, CRNP, NNP-BC Section Editor Special Series: Surgical Issues

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14 Advances in Neonatal Care • Vol. 18, No. 1 • pp. 14-21

Special Series: Surgical Issues

D

evelopment of the respiratory diverticulum occurs early in gestation. During the fourth week of development, the embryo forms the laryngotracheal tube that divides into the esophagus and the trachea. ¹ When the laryngotracheal tube fails to bud off and split properly, fistula and/or atresia of the esophagus can occur. Tracheoesophageal fistula (TEF) and esophageal atresia (EA) are congenital anomalies in which an abnormal communication occurs between the trachea and the proximal and/or distal esophagus segments. ^2–4 It is important to note that EA is a frequently occurring anomaly and it can be present with or without a TEF. ⁵ Figure 1 outlines the various types of TEF/

EA based on the Gross classification. ² ^, ⁶

Type A—Pure EA: Absence of the esophagus with no fistula. Neither end of the esophagus is connected to the trachea.

Type B—EA with proximal TEF: Middle section of the esophagus is missing, and there is a fistula on the trachea from the upper end of the esophagus.

Type C—EA with distal TEF: Middle of the esophagus is missing, and the trachea is connected to the lower end of the esophagus with a fistula.

Type D—EA with proximal and distal fistulas:

Absence of a portion of the esophagus with fistula connections to the trachea from both the upper and lower portions of the esophagus.

Type E—H-type fistula: Esophagus is complete with a fistula on the trachea. There may be a nar- rowed area of the esophagus.

STATISTICS

The worldwide prevalence of EA is 2.99 per 10,000. ⁵ ^, ⁷ Sixty percent of newborns with EA will have coexisting anomalies, whereas half of those infants will have Vertebral anomalies (V), Anal atresia (A), Cardiac anomalies (C), Tracheoesophageal fistula (T), Esophageal atresia (E), Renal anomalies (R), and Limb anomalies (L) (VACTERL) association. ⁸ ^, ⁹ Eighty-six percent of infants with EA will have a distal TEF; 2% will have a proximal TEF; 7%

will have no fistulous connection; and 4% will have TEF without atresia. ¹⁰ Infants with TEF/EA are 50%

Basic Knowledge of Tracheoesophageal Fistula and Esophageal Atresia

Sura Lee , MSN, CRNP

ABSTRACT

Background: Tracheoesophageal fistula (TEF) and esophageal atresia (EA) are rare anomalies in neonates. Up to 50%

of neonates with TEF/EA will have Vertebral anomalies (V), Anal atresia (A), Cardiac anomalies (C), Tracheoesophageal fistula (T), Esophageal atresia (E), Renal anomalies (R), and Limb anomalies (L) (VACTERL) association, which has the potential to cause serious morbidity.

Purpose: Timely management of the neonate can greatly impact the infant’s overall outcome. Spreading latest evidence- based knowledge and sharing practical experience with clinicians across various levels of the neonatal intensive care unit and well-baby units have the potential to decrease the rate of morbidity and mortality.

Methods/Search Strategy: PubMed, CINAHL, Cochrane Review, and Google Scholar were used to search key words—

tracheoesophageal fistula, esophageal atresia, TEF/EA, VACTERL, long gap, post-operative management, NICU, pediatric surgery—for articles that were relevant and current.

Findings/Results: Advancements in both technology and medicine have helped identify and decrease postsurgical complications. More understanding and clarity are needed to manage acid suppression and its effects in a timely way.

Implications for Practice: Knowing the clinical signs of potential TEF/EA, clinicians can initiate preoperative manage- ment and expedite transfer to a hospital with pediatric surgeons who are experts in TEF/EA management to prevent long-term morbidity.

Implications for Research: Various methods of perioperative management exist, and future studies should look into standardizing perioperative care. Other areas of research should include acid suppression recommendation, reducing long-term morbidity seen in patients with TEF/EA, postoperative complications, and how we can safely and effectively decrease the length of time to surgery for long-gap atresia in neonates .

Key Words: anastomosis , esophageal atresia , long gap , NICU , pediatric surgery , postoperative management , recurrent fistula , tracheoesophageal fistula , VACTERL

Author Affiliation: Newborn/Infant Intensive Care Unit, Division of Pediatric General, Thoracic, and Fetal Surgery, Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania.

The author declares no conflict of interest.

Correspondence: Sura Lee, MSN, CRNP, Newborn/Infant Intensive Care Unit, Division of Pediatric General, Thoracic, and Fetal Surgery, Children’s Hospital of Philadelphia, 3401 Civic Center Blvd, Philadelphia, PA 19104 ( [email protected] ).

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more likely to have congenital anomalies, with VAC- TERL being the most common association. ¹¹

VACTER/VACTERL

Quan and Smith ¹² first described the nonrandom occurrences of anomalies: (V) vertebral, (A) anorectal, (T) tracheoesophageal, (E) esophageal, and (R) renal and/or radial abnormalities. Over time, the incidence of cardiac and limb anomalies increased, having to expand VATER to VACTERL. (V) Vertebral anomalies occur in 60% to 90% of individuals with VAC- TERL association. ¹³ These anomalies may include segmentation defects, rib anomalies, abnormal spinal curvatures, costovertebral anomalies, and tethered spinal cord. ¹³ Obtaining radiographic images will help determine the need for surgical interventions. ¹³ (A) Anorectal malformation occurs in 55% to 90% of

individuals; thorough physical examination is key in identifying imperforate anus and other genital anomalies. ¹³ (C) Cardiac malformation have been reported in 40% to 80% of infants with VACTERL association. ¹³ Anomalies may include congenital heart dis- ease, situs anomalies, vascular anomalies, and arrhythmias. ¹³ Echocardiogram should be obtained early in life to identify any anomalies since it may impact the overall medical and surgical management of the infant. (TE) TEF/EA occurs in 50% to 80% of individuals, monitoring oral intake and respiratory status is paramount in evaluating an infant for potential TEF/EA diagnosis. ¹³ (R) Renal anomalies affect 50% of individuals; careful monitoring of urine out- put and renal function should be maintained. ¹³ Renal ultrasound scan and voiding cystourethrogram can help detect and manage infection and vesicoureteral reflux. (L) Limb anomalies occur in 40% to 55% of FIGURE 1

Gross-Vogt classification of TEF/EA outlines various types of TEF/EA, their incidence, and a picture showing the difference. TEF indicates tracheoesophageal fistula; EA, esophageal atresia.

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www.advancesinneonatalcare.org

affected individuals. Physical examination may identify radial and thumb anomalies, polydactyly, hypoplasia, and length discrepancy that may warrant further radiologic images. ¹³ Table 1 provides common VACTERL anomalies and their incidence. ¹³

To be diagnosed with VACTERL, 2 to 3 anomalies must be present. ¹³ Having a VACTERL diagnosis can complicate the treatment and subsequent outcomes in neonates with TEF/EA because multiple body systems are impacted. Neonates with TEF/EA should be evaluated for VACTERL association, in addition to genetic and chromosomal anomalies.

PRENATAL DIAGNOSIS

Advances in medicine and technology have made early prenatal counseling possible, which can improve postnatal outcomes. Routine fetal anatomy ultrasound scans that typically occur around approximately 20 weeks’ gestation can detect congenital anomalies such as TEF and EA. ¹¹ Polyhydramnios in the setting of small or absent stomach raises concern for TEF/EA; however, these ultrasound features can be nonspecific, subjective, and occasionally transient in nature. ¹¹ Bradshaw et al ¹¹ found that the overall sensitivity of prenatal ultrasound scan when identifying TEF/EA was 26% with 99% specificity and 35%

positive predictive value with no significant postnatal outcome. The accuracy and sensitivity of prenatal

ultrasound scans increase to 57% when completed in tertiary centers. ¹¹ Radiologists interpreting the images and the ultrasound technicians performing the studies in tertiary centers are specialized in congenital anomalies and thus accuracy increases. Some centers have the capability of performing fetal magnetic resonance imaging (MRI), which can be highly predictive for the diagnosis of TEF/EA if an upper esophageal pouch is present. However, fetal MRI has been criticized for its poorer feasibility and cost-effectiveness. ¹¹

POSTNATAL DIAGNOSIS AND MANAGEMENT

Undiagnosed TEF/EA is fairly common since prenatal ultrasound scan has a 26% sensitivity. ¹¹ Detailed physical evaluation of the newborn can identify clinical signs that can lead to early diagnosis of TEF/EA.

Clinical signs that raise suspicion of TEF/EA are excessive salivations, worsening oral feeds with choking and coughing, cyanosis, and respiratory distress. ² ^, ¹⁴ Once TEF/EA is suspected, confirmation can be made by inserting a 10F Salem Sump or Replogle tube. Salem Sump and Replogle tubes are double- lumen tubes in which one lumen drains secretions under continuous suction and the other lumen serves as an air vent. The air vent prevents the suction from becoming attached to the mucosa as long as the air vent is patent. Although both tubes have holes distally

TABLE 1. VACTERL Associated Anomalies ^a

Incidence Systems Affected Examples

60%-90% Vertebral anomalies (V) • Butterfl y vertebrae, hemivertebrae, vertebral fusions • Sacral agenesis/dysgenesis

• Rib anomalies

• Abnormal spinal curvatures • Tethered spinal cord

55%-90% Anal atresia (A) • Imperforate anus

• Anal atresia

• Perineal or gastrocutaneous fi stula • Genitourinary anomalies

40%-80% Cardiac anomalies (C) • Congenital heart defects • Situs anomalies • Vascular anomalies • Arrhythmias 50%-80% Tracheoesophageal fi stula with or

without Esophageal atresia (TE)

• Tracheoesophageal fi stula • Esophageal atresia

50%-80% Renal anomalies (R) • Renal agenesis

• Cystic and/or dysplastic kidneys • Renal fusions

• Ureteral anomalies 40%-55% Limb anomalies (L) • Radial anomalies

• Isolated thumb anomalies • Polydactyly

• Limb or digit hypoplasia • Limb length discrepancy

a Table adapted from Solomon et al. ¹³

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for suction, Replogle tube is preferred in infants with EA because of the 2 holes at the end of the tube. If the tube does not pass beyond approximately 10 cm and coils in the upper thoracic region, the diagnosis of TEF/EA should be considered. Failed passage of gastric tube on the anteroposterior chest radiograph is the gold standard for diagnosis of TEF/EA.

Some classic radiographic examples of TEF/EA are presented. In Figure 2 , tip of the catheter terminates within the upper esophageal pouch. The stomach and the intestines have gas present, which identi- fies that a distal TEF is present. ³ ^, ¹⁵ In Figure 3 , tip of the catheter terminates within the upper esophageal pouch. There is an absence of gastrointestinal gas, which indicates an isolated atresia. ³

PREOPERATIVE MANAGEMENT

Preoperative management of a neonate with TEF/EA is crucial before transferring the infant to a surgical neonatal intensive care unit ( Table 2 ). Once TEF/EA has been identified, ensure that the infant has nothing by mouth (NPO) and start intravenous fluids to maintain euvolemia and normoglycemia. Position of the infant’s head should be 30 ° to 40 ° to prevent any gastric content refluxing up to the esophagus and into the trachea. Maintain a patent Replogle tube or a Salem Sump to prevent aspiration. If available, an echocardiogram should be obtained to evaluate cardiac anomalies, especially the position of the aortic arch prior to surgery. Transfer the patient immediately to a surgical intensive care unit.

Repair of the EA is urgent in infants with respiratory distress requiring ventilatory support. ³ ^, ⁷ Ventila- tory support causes air to become trapped in the stomach. This air can potentially leak through the distal fistula, as seen in types C and D, worsening respiratory function, and increases the risk for gastric perforation. Decompression of the stomach and repair of the TEF/EA may be necessary as soon as the

neonate is born to prevent gastric perforation. To prevent gastric perforation, an emergency gastrostomy tube placement may be needed. Endotracheal tube should be placed distal to the fistula to prevent gastric distension. ⁸ Term infants may tolerate primary repair of TEF/EA well; however, preterm infant who are extremely low birth weight (ELBW) or very low birth weight (VLBW) may be too fragile. Spitz ³ classified the survival of infants with EA by weight, since birth weight is a risk factor for neonatal survival ( Table 3 ).

Spitz ³ predicted that neonates with low birth weights with a cardiac anomaly had the lowest survival rate.

Schmidt et al ¹⁶ evaluated the surgical outcome of primary repair of type C TEF/EA neonates based on their weight. The authors concluded that primary repair of type C TEF/EA in ELBW and VLBW neonates was not associated with increased intra- and postoperative complications compared with neonates with higher birth weight. ¹⁶ Associated anomalies play a significant role in dictating neonatal outcomes when compared with weight alone.

SURGICAL MANAGEMENT

Across institutions, different methods are used to identify various types of TEF/EA and to identify the location of the fistula. ¹⁷ Lal et al ¹⁸ performed a retrospective study looking at 11 children’s hospitals over 5 years to identify common perioperative management of TEF/EA. ¹⁸ Perioperative bronchoscopy ranged from 0% to 100%; perioperative proximal esophageal pouch contrast ranged from 0% to 69%;

and thoracoscopic repair ranged from 0% to 21%. ¹⁸ The authors found wide variation in care across the institutions, with no standardized, evidence-based best practice.

TEF/EA repairs consist of TEF ligation and/or EA anastomosis. Postoperatively, patients routinely return with a secure artificial airway in place and taking potentially paralytics to prevent shearing or

FIGURE 2

Type C depicts the blind pouch with gas noted in the stomach and intestine to identify a distal tracheoesophageal fistula.

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distracting the anastomotic site that may be under tension. ⁸ ^, ¹⁹ Chin-to-chest position is critical to prevent hyperextension of the neck to prevent endotracheal tube dislodgement and trauma to the repair site. Patients return from the operating room (OR) NPO until enteral feeds may be initiated in 1 to 2 days via a transanastomotic feeding tube placed in the OR. ⁸ If enteral feeds are withheld for any reason, total parental nutrition may be started. All patients returning from the OR after their TEF/EA repair should have radiographs of their chest and abdomen to ensure proper placement of the endotracheal tube and the transanastomotic tube. Antibiotics are typically given for 24 hours postoperatively, and acid suppression medication is started. ¹⁹

A chest tube is often placed in the OR to evacuate air and fluid within the pleural space that may accu- mulate after the surgery. ¹⁹ The chest tube should be connected to the drainage system with the suction regulator at − 10 cm of H 2 O and placed on continuous wall suction at 80 mm Hg for 24 to 48 hours. A chest radiograph should be obtained to evaluate for any air or fluid accumulation. After 24 to 48 hours, the chest tube is placed to water seal. Once the infant is extubated and clinically stable for oral feeds, an esophagram study should be completed. ¹⁹ An esophagram or barium swallow test evaluates the integrity

of the esophagus by placing water-soluble barium into the transanastomotic gastric tube just above the anastomotic site under fluoroscopy. The purpose of an esophagram is to evaluate for any leak from the anastomotic site. If there is no leak, the chest tube can safely be removed and oral feedings may be initiated. ¹⁹

LONG-GAP EA

According to the International Network of Esopha- geal Atresia (INoEA), long-gap atresia occurs in 10% of the EA spectrum. ²⁰ Long gap is defined as an anatomic distance of 3 or more vertebral bodies between the proximal and distal esophageal segments. ¹⁸ To identify the vertebral body distance, the proximal and distal esophagus segments are mea- sured by injecting contrast from the gastrostomy site into the distal esophagus and into the proximal esophagus. ³ Once the measurement is confirmed, a primary anastomosis can be performed if the gap measurement is 2 vertebral bodies or less. ⁷ If the measurement is greater than 2 vertebral bodies, it requires a delayed repair. The goal is to preserve the native esophagus, thus the delayed repair. ²⁰ While awaiting growth, feeds are initiated via a gastrostomy tube.

FIGURE 3

Type A depicts the blind pouch without gas in the stomach and intestine to identify an isolated atresia.

TABLE 2. Preoperative Management of an Infant With Suspected TEF/EA

NPO, IVF for euvolemia and normoglycemia

• Protect the lungs by evacuation of the proximal esophageal pouch by placing an indwelling Replogle tube or Salem Sump (10F) attached to continuous low suction

• Minimize refl ux of gastric contents up the esophagus into the trachea by placing the infant’s head of bed 30 ° -45 ° • Antibiotics if there is suspicion of aspiration or if perinatal risk factors are present

• Echocardiogram is obtained to rule out right-sided aortic arch or other cardiac anomalies prior to surgery • Transfer to a surgical neonatal intensive care unit

Abbreviations: EA, esophageal atresia; IVF, intravenous fl uid; NPO, nothing by mouth; TEF, tracheoesophageal fi stula.

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POSTOPERATIVE COMPLICATIONS Timing of the gastrostomy tube placement will depend on the ability to decompress the dilated stomach. ⁸ A Replogle catheter to continuous suction should remain in the upper pouch to prevent aspiration of saliva until repair is completed. ⁷ Continuous suction should be used cautiously to prevent damage or irritation to the lining of the esophageal pouch and not go beyond 30 mm Hg. Prior to surgery, an echocardiogram should be completed. The importance of identifying a right aortic arch on an echocardiogram in infants with long-gap atresia is because they have a 5-fold increase in developing postoperative complications such as a leak or stricture. ²¹

Postoperative complication that typically occurs within 48 hours is anastomotic leak. The retrospective study by Shah et al ²² concluded that using continuous positive airway pressure immediately after TEF/EA surgery did not increase the risk of

anastomotic leak or recurrence of the fistula. Clini- cally, anastomotic leak can be identified by frothy saliva seen in the chest tube, which requires suction or water seal depending on the surgeon. Infants should be carefully monitored until the site is healed and frothy saliva is no longer seen within the chest tube. Antibiotics should be given to cover oral flora that was leaking through the fistula.

Infants who are symptomatic with dysphagia or respiratory distress with oral feeds may have an anastomotic stricture. It is the most common type of complication requiring esophageal dilatation. Balloon dilatation for anastomotic stricture post–esophageal repair is the preferred method since it is safe and associated with low perforation and mortality rates. ²³ Frequency of dilatation vary among infants; however, the median number of dilatations has been found to be 3, with an average of 177 days since the last dilatation. ²³ To prevent stricture formation, infants who had repairs of TEF/EA were prescribed TABLE 3. Spitz Risk Classification for Neonatal Survival ^a

Group Birth Weight % Survival

Group 1 Birth weight > 1500 g without major cardiac anomaly 98

Group 2 Birth weight < 1500 g or major cardiac anomaly 82

Group 3 Birth weight < 1500 g and major cardiac anomaly 50

a Table adapted from Spitz. ³

TABLE 4. Common Complications ^a

Type of

Complications Incidence Facts Signs/Symptoms Treatment Anastomotic leak 10%-20% 3%-5% account for major

leaks that typically occur within 48 h of repair

• Tension pneumothorax • Sepsis

• Mediastinitis • Frothy saliva in the

chest tube

• NPO

• Chest tube to suction or water seal • Antibiotics covering

oral fl ora Anastomotic stric-

tures

30%-40% • Diffi culty feeding

• Respiratory distress

• Esophageal dilatation(s) • Acid suppression

Recurrent TEF 5%-14% • Coughing/choking with

feeds

• Apnea/cyanotic spells • Recurrent respiratory

infections (pneumonia)

• Treat symptoms • Prevention • Surgery

Tracheomalacia 10% Collapse of trachea during expiration

• Barking cough • Cyanosis/apnea

• Severe cases require aortopexy Gastroesophageal

refl ux

40% Antirefl ux medication is successful in 50% of cases

• Vomiting • Arching

• Fundoplication

Thal (partial)

Nissen (fl oppy wrap)

Respiratory infections

Common in infancy and fi rst 3 y of life

• Respiratory symptoms • Treat symptoms • Prevention Abbreviations: NPO, nothing by mouth; TEF, tracheoesophageal fi stula.

a Table adapted from Spitz, Stoll et al, ⁷ and Hunt et al. ⁸

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acid suppression drugs. ²⁴ Systemic review by Shawyer et al ²⁵ found no clear consensus whether infants should be treated only if symptoms develop or empir- ically to prevent strictures. More recently, Hunt et al ⁸ state that acid suppression is key to minimizing risk of stricture formation at the anastomotic site.

The least common postoperative complications are recurrent fistula and/or tracheomalacia. Infants with these complications may present with coughing (barking), apnea, cyanotic spells, and respiratory distress. If symptom management does not relieve the problem, further evaluation is required to assess the need for additional surgery. Severe cases of tracheomalacia may require aortopexy. Surgical advances have decreased the incidence of postoperative complications; however, complications do occur. These complications should be managed by pediatric surgeons with expertise in the field to reduce the risk of long-term morbidity.

Table 4 exhibits the most common complications. ³ ^, ⁷ ^, ⁸

CONCLUSION

The outcome of newborns with TEF with and without EA has improved considerably, with an overall survival rate of 85% to 95%. ²⁶ The prognosis can significantly change when it is associated with anomalies, which can affect the quality of life. Acher et al ⁴ completed a survey to evaluate the long-term outcomes of patients with TEF/EA in a retrospective study. Regardless of the type of repair, occurrence of esophageal dysmotility, dysphagia, reflux, and strictures adversely affected the patient’s quality of life. ⁴ Future studies should evaluate how we can minimize these long-term effects of TEF/EA.

While the incidence of TEF/EA may be minimal when compared with other congenital anomalies, there is a high incidence of VACTERL association, which can lead to significant morbidity. Recognizing early signs and symptoms of newborns with TEF/EA has the potential to expedite their medical management and transfer to a hospital where pediatric surgeons with

expertise in and experience with TEF/EA are available.

Delay in appropriate treatment can lead to poorer outcomes such as aspiration, gastric perforation, respiratory failure, and long-term complications.

References

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Summary of Recommendations for Practice and Research

What we know: • 50%-60% of newborns with TEF/EA will have coexisting anomalies.

• Urgent repair of TEF/EA may be warranted despite weight. Associated anomalies play a signifi cant role in dictating neonatal outcome, not the weight alone.

• Postoperative complications exist despite surgical advances.

What needs to be studied: • Standardize perioperative management.

• Reducing long-term morbidity seen in patients with TEF/EA.

• Reducing postoperative complications.

• Acid suppression recommendations.

• Care of infants with long-gap atresia. How can we safely and effectively decrease their length of hospital stay and increase their time to surgery.

What we can do today: • Know the clinical signs of potential TEF/EA diagnosis.

• Transfer to hospitals with pediatric surgeons who are experts in TEF/EA to prevent long-term morbidity.

• Meticulous postoperative management of TEF/EA.

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a systematic review . Pediatr Radiol . 2013 : 43 : 898 – 901 .

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25. Shawyer AC , D’Souza J , Pemberton J , et al. The management of postoperative reflux in congenital esophageal atresia-tracheoesophageal fistula: a systematic review . Pediatr Surg Int . 2014 : 30 : 987 – 996 .

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