Never quite right

After esophageal atresia (EA) repair, problems with reflux and dysphagia effect up to 75-100% of patients.  A new study, JPGN 2013; 56: 609-14, helps provide some understanding why the esophagus is never quite right in these patients.

High resolution esophageal manometry (HREM) was performed in 40 patients with a median age of 8 years at three centers. Data was obtained primarily by chart review; in addition, symptomatology at the time of HRE#M was evaluated through a self-assessment questionnaire completed by the child or his primary caregiver.  35 patients had type C EA which typically accounts for 80-85% of all EA cases. Type C EA refers to a proximal esophageal pouch and a distal tracheoesophageal fistula (TEF).  5 patients had type A. Type A EA is an EA without a distal TEF. At the time of the HREM, 7 (18%) were considered asymptomatic.

Findings:

  • Three different motility patterns were identified: aperistalsis in 15 (38%), pressurization in 6 (15%), and distal contractions in 19 (47%).
  • Aperistalsis occurred primarily in patients with long-gap defects and/or following anastomotic leaks. 8 of 15 patients with aperistalsis had undergone fundoplication.
  • Pressurization (as shown in Figure 1) was when contraction of the entire esophageal body  occurred at once rather than in a progressive manner from proximal to distal esophagus. Distal contraction pattern indicated an absence of proximal esophageal contractions. 4 of 6 patients with pressurization pattern had undergone previous fundoplication.
  • Motility patterns were not predictive of symptoms.  Asymptomatic patients were noted with all three patterns.  However, gastroesophageal symptoms predominated in the aperistalsis group.  Dysphagia was frequent in all three groups.

Study limitations included retrospective data, and small numbers of patients. Furthermore, in patients with long-standing esophageal problems, “asymptomatic” may be related to the patient not knowing what “normal” feels like and may be related to compensatory behaviors.

While HREM explains the pathophysiology in EA patients, given the lack of effective medical treatments for motility disturbances, upper endoscopy is likely to be more useful for clinical management by identifying esophagitis and possibly Barrett’s esophagus.

Related blog post:

2 thoughts on “Never quite right

  1. Pingback: Endoscopic Surveillance after Esophageal Atresia: Low Yield In Pediatrics | gutsandgrowth

  2. Pingback: Guidelines for Esophageal Atresia-Tracheoesophageal Fistula | gutsandgrowth

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