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The underlying principle of treatment is stabilization before surgery, even in the absence of need for transfer to a specialist centre. This involves nasogastric aspiration, ventilation and bicarbonate infusion to correct acid-base and blood gas abnormalities.

Surgery involves a subcostal approach with reduction of abdominal contents and repair of the residual diaphragm with non-absorbable sutures. Prosthetic material may be used to bridge large gaps in the diaphragm.

Postoperative ventilation and early oral feeding are recommended. Extracorporeal membrane oxygenation is being investigated as a measure to encourage lung growth.

There may be a role for repair of the defect in the fetus.

Last reviewed 01/2018