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Anatomic and functional abnormalities of the esophagus in infants surviving congenital diaphragmatic hernia
C J Stolar1, J P Levy, P W Dillon
1Division of Pediatric Surgery, Babies Hospital, Columbia-Presbyterian Medical Center, New York, New York 10032.
Insights
Infants surviving congenital diaphragmatic hernia (CDH) often develop esophageal abnormalities, including ectasia and severe gastroesophageal reflux. These conditions, linked to hydramnios, impact growth but are manageable without surgery.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Neonatology
Background:
- Congenital diaphragmatic hernia (CDH) survival rates are increasing, particularly with extracorporeal membrane oxygenation (ECMO) support.
- This improved survival has led to new clinical observations regarding associated conditions in affected infants.
Purpose of the Study:
- To investigate the occurrence and characteristics of esophageal abnormalities in infants with CDH.
- To explore the relationship between CDH, esophageal issues, and outcomes such as growth.
Main Methods:
- Retrospective review of 30 infants with CDH, including ECMO and non-ECMO treated groups.
- Utilized upper gastrointestinal series, contrast studies, and intra-esophageal pH monitoring to assess esophageal function.
- Correlated esophageal findings with amniotic history (hydramnios).
Main Results:
- Esophageal ectasia and severe gastroesophageal reflux were observed in a significant proportion of CDH survivors (14/17 ECMO, 3/7 non-ECMO).
- Intra-esophageal pH monitoring confirmed significant reflux in 69% of tested patients, with abnormal peristalsis suggested by prolonged acid clearance.
- Hydramnios was present in 13/16 infants with available amniotic history, suggesting a correlation.
Conclusions:
- Gastroesophageal reflux and esophageal ectasia represent a newly observed association with CDH.
- These esophageal abnormalities, potentially linked to hydramnios and foregut obstruction, are manageable without surgery.
- While symptoms are treatable, affected children may experience slower weight gain, highlighting the long-term impact on growth.
Abstract:
As more infants with congenital diaphragmatic hernia (CDH) survive by means of extracorporeal membrane oxygenation (ECMO), new clinical observations are being made. We report anatomic and functional abnormalities of the esophagus in these infants. Twenty-two of 30 infants with CDH survived. Three of 7 non-ECMO-treated infants and 14 of 17 ECMO-treated infants had an air- or fluid-filled mediastinal mass that was confirmed by an upper gastrointestinal series as ectatic esophagus. Contrast studies demonstrated severe gastroesophageal reflux. Intra-esophageal pH monitoring confirmed significant reflux (Euler scores greater than 50) in 69% of tested patients. Prolonged acid clearance in most patients implied abnormal peristalsis. Hydramnios was correlated to the observed esophageal abnormalities, being present in 13 of 16 infants with an available amniotic history. All patients had symptomatic gastroesophageal reflux managed without antireflux surgery and were discharged 36 +/- 2 days after initiation of feeding. Follow-up (range: 7 to 56 months; mean: 32 months) showed that weight gain in affected children was slower than in those not affected. All but one are asymptomatic. Repeat upper gastrointestinal series shows persistent ectasia but less gastro-esophageal reflux. Gastroesophageal reflux/ectasia is a new observation with CDH. The associated hydramnios may result from proximal foregut obstruction by kinking the gastroesophageal junction with CDH. The radiographic appearance can be quite unusual. Clinical manifestations of gastro-esophageal reflux are manageable without surgery but may account for the observed compromised growth.