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Background  The surgical management of esophageal atresia in extreme-low-birth-weight infants (< 1000 g) is challenging. We report on an extreme-low-birth-weight infant who was extremely preterm (510 g, 25 + 5 weeks) and of prenatally unknown Gross type C esophageal atresia.    Case presentation  After resuscitation and intubation, the tracheoesophageal fistula was closed on the first day of life in the neonatal intensive care unit via an extrapleural approach using a titanium clip. On the sixth day of life, the Caucasian child was extubated. To minimize the operative trauma in the initial neonatal period, we prolonged gastrostomy placement until the 22nd day of life (weight 725 g). At the age of 3 months (weight 2510 g), thoracoscopic esophageal anastomosis was performed. The postoperative course was unremarkable. During the further clinical course, eight esophageal dilations were necessary. Currently, the patient swallows without difficulties at the age of 4 years and thrives well [15 kg (Percentile 28); 100 cm (Percentile 24)].    Conclusions  Our case shows that minimized postnatal surgical trauma with primary tracheoesophageal fistula closure at the bedside, delayed gastrostomy, and minimally invasive esophageal repair after substantial weight gain (> 2.5 kg) is a good strategy for esophageal atresia/tracheoesophageal fistula in extreme-low-birth-weight infants.    Supplementary Information  The online version contains supplementary material available at 10.1186/s13256-021-02951-x.

Surgical treatment of esophageal atresia with lower tracheoesophageal fistula in an extremely preterm infant (510 g, 25 + 5 weeks): a case report