We experienced a case of a female infant with a double aortic arch (DAA) which formed an aortoesophageal fistula, leading to hemorrhagic shock. The patient had severe dyspnea at birth, and was intubated and tube-feeding was started through a nasogastric tube immediately after birth. A DAA was diagnosed by contrast-enhanced computed tomography. Due to abdominal organ malformation, we proceeded with abdominal surgery. Forty-nine days after birth, she suddenly developed massive hematemesis and went into hemorrhagic shock. The bleeding was stopped using an endoscope and was shown to have originated from the esophagus membrane. Compression of the esophageal wall by both the inserted nasogastric tube and vascular ring led to the development of ulceration, resulting in a fistula associated with massive hematemesis. An operation for a DAA was performed on the 53rd day after birth. The inferior side of the DAA was cut, to decompress the bronchus and esophagus and close the fistula. The patient's postoperative course was good and there was no further bleeding. In severe cases of a DAA who require respiratory intubation and tube feeding from a nasogastric tube it is important to carry out surgery as soon as possible.

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