What is Tracheoesophageal Atresia?
Esophageal atresia is a congenitally interrupted esophagus, often with one or more fistulae between the malformed esophagus and the trachea. It was first described anecdotally in the 17th century, and Gross of Boston later set out the classification system that is most often cited.
Pathophysiology
Under the Gross system, the types of esophageal atresia and their approximate incidence among infants born with esophageal anomalies are: Type A, esophageal atresia without fistula (pure esophageal atresia), 10%; Type B, esophageal atresia with proximal TEF, <1%; Type C, esophageal atresia with distal TEF, 85%; Type D, esophageal atresia with proximal and distal TEFs, <1%; Type E, TEF without esophageal atresia (H-type fistula), 4%; and Type F, congenital esophageal stenosis, <1%.
A fetus with esophageal atresia cannot effectively swallow amniotic fluid, especially when no TEF is present; with a distal TEF, some amniotic fluid presumably flows through the trachea and down the fistula to the gut. The neonate cannot swallow and drools copious saliva. Air from the trachea can pass down the distal fistula when the baby cries, strains, or is ventilated, which can cause an often-lethal acute gastric perforation. Manometric studies before repair show the distal esophagus is essentially dysmotile, with poor or absent peristalsis.
Causes
No human teratogens are known to cause esophageal atresia. Familial cases occur, with a 2% recurrence risk when a sibling is affected, and the occasional association with trisomies 21, 13, and 18 suggests a genetic contribution. Several embryologic theories exist. O'Rahilly (1984) proposed a fixed cephalad point of tracheoesophageal separation with elements elongating caudally, explaining TEF as a breakdown of esophageal mucosa when linear growth outpaces epithelial cell division. Kluth (1987) attributed it to faulty development of the already-differentiated trachea and esophagus, with a dorsal fold lying too far ventrally so the early tracheoesophagus stays undivided. Spilde et al (2003) reported esophageal atresia-TEF in doxorubicin-induced rat embryos with specific absences of fibroblast growth factor elements (FGF1 and the IIIb splice variant of the FGF2R receptor). Orford et al (2001) proposed that an ectopic, ventrally displaced notochord at 21 days' gestation disrupts sonic hedgehog-signaled apoptosis in the developing foregut.
Statistics and Incidences
The incidence of esophageal atresia is roughly 1 case in 3000 to 4500 births, often cited as about 1 in 3500 (StatPearls). The highest reported incidence is in Finland. Type C, esophageal atresia with a distal tracheoesophageal fistula, accounts for about 85% of cases.
Clinical Manifestations
The newborn drools and produces substantial mucus and excessive oral secretions. If allowed to suckle, the baby chokes, may struggle to maintain an airway, and can develop significant respiratory distress. A sonorous seal-bark cough in the delivery room points to concomitant tracheomalacia. When an oral tube is placed to suction the stomach, it characteristically becomes blocked 10-11 cm from the lips.
Assessment and Diagnostic Findings
Draw baseline labs: CBC, electrolytes, venous gas, BUN and serum creatinine, blood glucose, and serum calcium. Prenatal ultrasonography may show the gastric bubble size, polyhydramnios, and VACTERL (vertebral defects, anorectal malformations, cardiovascular defects, tracheoesophageal defects, renal anomalies, limb deformities) anomalies suggesting esophageal atresia. Echocardiography is indicated early when there are signs of cardiovascular disease. Chest radiography is mandatory and should be done as soon as esophageal atresia is suspected.
Medical Management
Individualize the plan. For delayed repair, place a 10-French Replogle double-lumen tube through the mouth or nose well into the upper pouch for continuous suction of pooled secretions, position the baby in the 45° sitting position, and consider prophylactic broad-spectrum antibiotics (ampicillin and gentamicin). If no distal TEF is present, a gastrostomy may be created (the stomach is small, so laparotomy is required); when the baby is ventilated at high pressures, the gastrostomy can become a low-resistance route that vents gases through the distal fistula. When a distal fistula keeps soiling the lung, distal TEF ligation is considered, performed through a right-side thoracotomy, ideally extrapleural.
In May 2017 the FDA approved the Flourish Pediatric Esophageal Atresia Anastomosis (Cook Medical) for infants up to 1-year-old who have no teeth and no TEF (or a repaired TEF); it uses magnets to pull the upper and lower esophageal segments together and is not indicated when the gap between segments is 4 cm or greater. Some centers repair esophageal atresia by minimally invasive thoracoscopy, which should be done only by surgeons with extensive pediatric thoracoscopic experience. The chest drain is placed in 2 cm of water only to seal it; it is not connected to suction, which could encourage an anastomotic leak.
Nursing Management
Nursing Assessment
A mother carrying a fetus with esophageal atresia may have polyhydramnios, present in approximately 33% of mothers when there is a distal TEF and in virtually 100% when there is esophageal atresia without fistula. On exam, look for VACTERL anomalies; if one is present, assess for the others. VACTERL syndrome exists when three or more associated anomalies are present and occurs in approximately 25% of patients with esophageal atresia.
Nursing Diagnosis
Based on the assessment data, major nursing diagnoses include impaired gas exchange related to the abnormal opening between esophagus and trachea (evidenced by cyanosis); impaired swallowing related to mechanical obstruction; risk for injury related to the surgical procedure; anxiety related to difficulty swallowing and surgical discomfort; altered family processes related to a child with a physical defect; and risk for aspiration related to difficulty swallowing.
Nursing Care Planning and Goals
The infant is free of signs of aspiration with decreased aspiration risk, swallows and digests oral, nasogastric, or gastric feeding without aspiration, and shows safe swallowing (no coughing or choking during feeding, no oral stasis after eating, able to ingest foods and fluids). The infant remains free of injury, and family caregivers describe their anxiety and coping patterns and identify strategies to reduce anxiety.
Nursing Interventions
Keep suction equipment at the bedside and suction as needed; ensure nutrition by consulting the physician for enteral feedings, usually a PEG tube. To prevent aspiration, confirm tube placement before feeding using tube markings, x-ray (most accurate), gastric fluid pH, and aspirate color; if ordered, add a few drops of blue or green food coloring to feedings to flag aspiration, and test tracheobronchial secretions for glucose to detect aspirated feedings. Elevate the head of bed to 30 to 45 degrees during feeding and for 30 to 45 minutes afterward with intermittent feeds, and teach the signs of aspiration.
To reduce anxiety, let family caregivers talk through anxious feelings and identifiable triggers, help them build anxiety-reducing skills (relaxation, deep breathing, positive visualization, reassuring self-statements), and explain all activities and procedures in nonmedical terms with calm, slow speech ahead of time, validating their understanding.
Evaluation
Goals are met when the infant stays free of aspiration signs with reduced risk, swallows and digests feedings without aspiration, demonstrates safe swallowing, remains free of injury, and family caregivers describe their anxiety and coping patterns and identify strategies to reduce anxiety.
Documentation Guidelines
Document individual findings including contributing factors, interactions, and specifics of behavior; intake and output; signs of infection; cultural and religious beliefs and expectations; the plan of care; the teaching plan; responses to interventions and teaching; and progress toward desired outcomes.
Frequently Asked Questions
What is tracheoesophageal atresia? It is a congenital malformation in which the esophagus ends in a blind pouch instead of connecting to the stomach, often with one or more abnormal connections (fistulae) between the esophagus and trachea. It is a surgical newborn emergency.
What is the classic early sign? A newborn who drools, produces excessive frothy secretions, and chokes, with a feeding or suction tube that cannot be passed and characteristically stops about 10 to 11 cm from the lips. Recognize it before the infant is fed.
How common is it, and which type is most frequent? Esophageal atresia occurs in roughly 1 in 3500 births. Type C, esophageal atresia with a distal tracheoesophageal fistula, is by far the most common, accounting for about 85% of cases (StatPearls).
Why must the baby be kept NPO with continuous suction? Anything by mouth, and pooled saliva in the blind pouch, can be aspirated into the lungs. A Replogle double-lumen tube provides continuous suction of the upper pouch, and elevating the head reduces reflux of gastric contents through a distal fistula.
What other anomalies are associated with it? The VACTERL association (vertebral, anorectal, cardiac, tracheoesophageal, renal, and limb defects). VACTERL is diagnosed when three or more of these occur together and is present in about 25% of infants with esophageal atresia, so finding one anomaly should prompt a search for the others.
How is it repaired? Treatment is surgical, joining the esophageal segments and closing any fistula, with timing individualized to the infant's stability and anatomy (StatPearls). Before surgery, care centers on airway protection, suction, NPO status, and stabilization.