Literature

Clinical Pearls & Morning Reports


Published September 18, 2019

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What is congenital esophageal stenosis? 

In a child who struggles to gain weight, the first step is to determine whether the child has adequate caloric intake and, if he does, whether he is losing weight as a result of vomiting or diarrhea or is not able to use the nutritional intake effectively because of a genetic, endocrine, or metabolic disturbance. Read the latest Case Records of the Massachusetts General Hospital here.

Clinical Pearls

Q: What are the most common causes of vomiting and failure to thrive in infants?

A: Gastroesophageal reflux disease (GERD) and dietary protein allergy are the most common causes of vomiting and failure to thrive in infants. There is no easy way to confidently differentiate between these two conditions, which have overlapping symptoms. Treatment with acid suppression often fails to reduce symptoms attributed to GERD in infants. Recent international guidelines have recommended introducing a hypoallergenic infant formula before starting acid-suppression treatment in infants who have symptoms suggestive of GERD. The natural history of GERD in infants is favorable; by 12 months of age, 95% of infants no longer have emesis.

Q: What is congenital esophageal stenosis?

A: Congenital esophageal stenosis (CES) is a rare condition with an estimated prevalence of 1 in 25,000 to 50,000 live births. The most common subtype is CES with fibromuscular thickening, which accounts for approximately 54% of cases of CES and generally involves the mid-to-lower esophagus. The other two subtypes are CES with tracheobronchial remnants (which typically consist of cartilage or respiratory-type epithelium), which accounts for approximately 30% of cases and involves the lower esophagus, and CES with membranous webs, which accounts for approximately 15% of cases and involves the proximal esophagus.

Morning Report Questions

Q: Is CES associated with a delay in diagnosis?

A: Typical symptoms of CES include vomiting and dysphagia during ingestion of solid foods, which ultimately lead to poor weight gain in 50% of cases. The time from the onset of symptoms to diagnosis is often prolonged, with an average time to diagnosis of 2 years. Although studies of long-term prognosis are lacking, coexisting conditions probably influence outcomes, given that cases of CES with cardiac or other gastrointestinal anomalies have been reported.

Q: Does dysphagia associated with CES persist after therapy in many patients?

A: After endoscopic dilation or surgical resection, dysphagia persists in up to 64% of patients with CES. The persistence of dysphagia may be due to intrinsic esophageal dysmotility, since there is evidence that patients who have CES with fibromuscular thickening or CES with tracheobronchial remnants have abnormal findings on esophageal manometry before the dilation procedure or surgery.

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