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history of EA/TEF

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[size=20px]A 13-year old female had Type C tracheoesophageal fistula and esophageal atresia (TEF/EA) repair as a child. On her annual visit, she complains of occasional heartburn.

 

At this point, this teenager with a history of EA/TEF should undergo

 

A no diagnostic procedures.

 

B esophagram.

 

C pH probe.

 

D esophageal motility and manometry studies.

 

E esophagogastroduodenoscopy.[/size]

  • 2 weeks later...
E

correct

 

Esophageal metaplasia (gastric and intestinal) arises in 15% to 20% of esophageal atresia (EA) patients with a lag time of development of about 10 years. The youngest patient with adenocarcinoma of the esophagus documented in the literature is below 20 years of age. Although formal recommendations for esophageal endoscopy do not exist, some experts are slowly suggesting that all patients with EA should have screening esophagoscopy at the age of 10 years regardless of symptoms.

 

Recently, the working group of International Network on Esophageal Atresia (comprises members from ESPGHAN/NASPGHAN) was charged with the task of developing uniform evidence based guidelines for the management of gastrointestinal complications in children with EA and recommended all patients with EA should have screening esophagoscopy three times regardless of symptoms: after discontinuing proton pump inhibitors therapy, before 10 years or age and at transition to adulthood. However, follow-up of patients from these more rigid recommendations are currently nonexistent.

 

If patients are on acid suppressive drugs, esophagogastroduodenoscopy (EGD) should be performed while on treatment. EGD should carefully document changes and location in centimeters from the teeth, photographs or video documentation, stepwise 4 quadrant biopsies and staining with alcian blue pH 2.5. If erosive esophagitis is seen or landmarks are unclear, a proton pump inhibitor is started and repeat EGD is performed after three to four months. For Barrett esophagus with or without intestinal metaplasia, repeat endoscopy should be performed in three to five years; if dysplasia is noted, endoscopic mucosal therapies such as mucosal resection or ablation should be performed as formal guidelines warrant.

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