This study aimed to report the case of a patient diagnosed with pulmonary sequestration accompanied by an intermittent haematemesis as the initial and life-threatening symptom. Emergent surgical intervention finally confirmed a rare direct fistula formation between the arterial blood supply of pulmonary sequestration and the oesophagus, which led to intermittent upper gastrointestinal haemorrhage. To our knowledge, this is the first case reported with this kind of fistula formation.
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