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Background <p>Esophageal atresia with tracheoesophageal fistula (EA/TEF) and tetralogy of Fallot (TOF) are both life-threatening congenital malformations in the neonatal period. Their coexistence poses substantial challenges for treatment planning, particularly in low-birth-weight infants, for whom no consensus management strategy has yet been established.</p> Case presentation <p>We report the diagnostic and therapeutic courses of two preterm, low-birth-weight infants with concomitant EA/TEF and TOF. Case 1 was a male neonate with a birth weight of 1.7&#xa0;kg. Intraoperative exploration revealed an approximately 3-cm gap between the proximal and distal esophageal segments, rendering primary end-to-end esophageal anastomosis excessively tensioned. A staged surgical approach was therefore adopted. In the first stage, ligation of the tracheoesophageal fistula and gastrostomy were performed. At 2.5 months of age, when the infant weighed 2.8&#xa0;kg, he underwent complete repair of TOF, correction of a complete vascular ring, and closure of an atrial septal defect under cardiopulmonary bypass. Seventeen days later, definitive repair of the esophageal atresia was successfully completed. The patient recovered well and was ultimately discharged. Case 2 was a male neonate with a birth weight of 2.2&#xa0;kg, in whom primary esophageal anastomosis was successfully achieved during the initial operation. Three months after discharge, he was readmitted with cough and cyanosis. He subsequently underwent complete repair of TOF, closure of the atrial septal defect, and placement of a bioresorbable external tracheal splint, and recovered uneventfully.</p> Conclusions <p>The successful management of these two low-birth-weight infants with EA/TEF and TOF suggests that surgical strategies should be individualized according to anatomical characteristics and overall clinical condition. Primary esophageal anastomosis may be appropriate in patients with favorable anatomy, whereas a three-stage approach consisting of TEF ligation, complete TOF repair, and delayed esophageal anastomosis may provide a feasible alternative for selected patients with a long esophageal gap and excessive anastomotic tension. In addition, low birth weight alone should not be regarded as an absolute contraindication to surgery. With aggressive nutritional support and meticulous perioperative management, some low-birth-weight infants may tolerate complex surgical interventions. Timely recognition and management of postoperative complications are also critical to achieving favorable outcomes.</p>

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Balancing two defects: surgical decision-making in low-birth-weight neonates with esophageal atresia and tetralogy of Fallot—a case series

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  • 发表日期：

    2026年4月11日

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  • Ming-tang Ye,
  • Yu-zhong Yang,
  • Ji-rong Qi,
  • Xu-ming Mo

摘要

Background

Esophageal atresia with tracheoesophageal fistula (EA/TEF) and tetralogy of Fallot (TOF) are both life-threatening congenital malformations in the neonatal period. Their coexistence poses substantial challenges for treatment planning, particularly in low-birth-weight infants, for whom no consensus management strategy has yet been established.

Case presentation

We report the diagnostic and therapeutic courses of two preterm, low-birth-weight infants with concomitant EA/TEF and TOF. Case 1 was a male neonate with a birth weight of 1.7 kg. Intraoperative exploration revealed an approximately 3-cm gap between the proximal and distal esophageal segments, rendering primary end-to-end esophageal anastomosis excessively tensioned. A staged surgical approach was therefore adopted. In the first stage, ligation of the tracheoesophageal fistula and gastrostomy were performed. At 2.5 months of age, when the infant weighed 2.8 kg, he underwent complete repair of TOF, correction of a complete vascular ring, and closure of an atrial septal defect under cardiopulmonary bypass. Seventeen days later, definitive repair of the esophageal atresia was successfully completed. The patient recovered well and was ultimately discharged. Case 2 was a male neonate with a birth weight of 2.2 kg, in whom primary esophageal anastomosis was successfully achieved during the initial operation. Three months after discharge, he was readmitted with cough and cyanosis. He subsequently underwent complete repair of TOF, closure of the atrial septal defect, and placement of a bioresorbable external tracheal splint, and recovered uneventfully.

Conclusions

The successful management of these two low-birth-weight infants with EA/TEF and TOF suggests that surgical strategies should be individualized according to anatomical characteristics and overall clinical condition. Primary esophageal anastomosis may be appropriate in patients with favorable anatomy, whereas a three-stage approach consisting of TEF ligation, complete TOF repair, and delayed esophageal anastomosis may provide a feasible alternative for selected patients with a long esophageal gap and excessive anastomotic tension. In addition, low birth weight alone should not be regarded as an absolute contraindication to surgery. With aggressive nutritional support and meticulous perioperative management, some low-birth-weight infants may tolerate complex surgical interventions. Timely recognition and management of postoperative complications are also critical to achieving favorable outcomes.