From the Guidelines
Esophageal atresia presents with several characteristic signs and symptoms that typically appear shortly after birth, including excessive drooling, feeding difficulties, choking, coughing, regurgitation, and cyanosis, as well as respiratory distress and a scaphoid abdomen, and long-term complications such as gastroesophageal reflux disease (GERD), dysphagia, and eosinophilic esophagitis (EoE) may occur in adolescents and adults 1. The most common signs and symptoms of esophageal atresia (EA) include:
- Excessive drooling and bubbling of saliva from the mouth and nose
- Feeding difficulties, with choking, coughing, and regurgitation occurring when attempts are made to feed the infant
- Cyanosis (bluish discoloration of the skin) may develop during feeding attempts
- Respiratory distress is common, particularly if there is an associated tracheoesophageal fistula allowing stomach contents to reflux into the lungs
- A scaphoid (sunken) abdomen may be present if there is no connection between the esophagus and stomach, as air cannot enter the gastrointestinal tract
- Long-term complications such as GERD, dysphagia, and EoE may occur in adolescents and adults, with symptoms including vomiting, dysphagia, and food bolus impaction 1. Diagnostic confirmation typically involves attempting to pass a nasogastric tube, which meets resistance, and radiographic studies showing the tube coiled in the upper esophageal pouch 1. It is essential to evaluate symptomatic adolescent and adult patients with EA-TEF with endoscopy and biopsy to diagnose and manage long-term complications such as EoE, which can adversely affect feeding and nutrition 1. Early recognition and prompt surgical intervention are crucial to establish esophageal continuity and prevent complications such as aspiration pneumonia and malnutrition 1.
From the Research
Signs and Symptoms of Esophageal Atresia (EA)
The signs and symptoms of esophageal atresia (EA) can vary, but some common presentations include:
- Inability to pass an orogastric tube, respiratory distress, or an inability to tolerate feeding in infants with EA 2
- Coughing, recurrent pneumonia, or choking during feedings in cases of isolated trachea-esophageal fistula (TEF) 2
- Dysphagia, which is a common symptom in patients with EA, usually related to stricture, dysmotility, or peptic esophagitis 3
- Feeding difficulties in early infancy, which may interrupt maternal expectations of becoming providers of nutrition to their infants and influence the infant's development of sensory motor skills and parent-child relationships 4
Associated Conditions
EA may be associated with other major congenital anomalies, such as:
- VACTERL association (vertebral, anal, cardiac, tracheo-esophageal, renal/radial) 2, 5
- Trisomy 18 or 21 5
- CHARGE syndrome 5
- Eosinophilic esophagitis (EoE), which may be a frequent concomitant problem in patients with a history of congenital esophageal deformities 3
Disease Severity and Survival Rates
The severity of EA can vary, and disease severity stratification can be done using scores such as the American Society of Anesthesiologists (ASA) and Pediatric Risk Assessment (PRAm) scores 6