INTRODUCTION

Esophageal atresia (EA) occurs in approximately 1 in every 3000 to 4500 live births and has no described sex predilection. EAs with and without tracheal fistulas have been classified into five types: (1) EA with distal tracheoesophageal fistula (TEF), (2) EA without TEF, (3) EA with proximal TEF, (4) EA with proximal and distal fistula, and (5) isolated TEF (H type). EA with a TEF between the distal esophagus and the trachea occurs in approximately 86% of cases.¹^–³ EA occurs with other significant congenital anomalies in 30% to 76% of children.^2,⁴ Importantly, these associated congenital anomalies are the major source of morbidity and mortality associated with EA repair.¹ The most common congenital anomaly is congenital heart disease, which is found in up to 20% of children.⁵ The acronym VACTERL groups associated defects into vertebral, anal, cardiac, tracheoesophageal, renal, and limb abnormalities.

Children present at various times depending on the type of anomaly. EA prevents the child from swallowing amniotic fluid, with a resultant polyhydramnios. Prenatal ultrasound can demonstrate polyhydramnios, absent or small stomach bubble, and an esophageal pouch.^6,⁷ Those not detected prenatally become symptomatic soon after birth with drooling, choking, and the inability to tolerate feeding. In contrast, patients with an H-type TEF may not be diagnosed until later in life. The diagnosis should be suspected in a child with recurrent episodes of aspiration pneumonia, choking, and coughing with feedings.

The diagnosis of EA is demonstrated on chest radiograph showing a curved catheter in the proximal esophageal pouch. In patients with an isolated EA, other findings include a gasless abdomen. Other studies in the work-up may include contrast esophagogram and bronchoscopy. Evaluation should also include echocardiogram, renal ultrasound, and vertebral films to rule out major cardiac, renal, and vertebral anomalies. In addition, the echocardiogram will evaluate for the location of the aortic arch to aid in planning the operation.

INDICATIONS

• Newborn with blind ending upper esophageal pouch

• Newborn with a gasless abdomen on plain x-ray

OPERATIVE PROCEDURE

With the significant association with congenital cardiac anomalies, the location of the aortic arch is right-sided in up to 5.4% of children.⁸^–¹⁰ The location in the opposite chest makes the already challenging anastomosis more difficult.⁸ The aorta obscures the esophagus and fistula when approaching from the right chest. Proceeding with the operation through the right chest leads to a higher leak rate (42%) and higher morbidity and mortality.⁹ Of significance, the finding of a right-sided aortic arch (RAA) should raise the suspicion for the finding of a long-gap atresia. Long-gap atresias are found in up to 42% of patients with RAA.⁸

Grade 3 complication

• Repair

Babu and coworkers ⁸ proposed the approach to management of RAA tracheoesophageal repairs. All infants should have a preoperative echocardiogram, and if RAA is suspected, the echocardiogram should be repeated; alternatively, magnetic resonance imaging (MRI) is performed. When an unsuspected RAA is found at the time of the initial exploration, the decision to proceed is based on the anatomy and the ease of dissection. If the dissection is difficult, a delayed left thoracotomy should be performed after a complete evaluation of the vascular anatomy.

• Prevention

Preoperatively, all patients should undergo an extensive work-up to diagnose an RAA including echocardiogram. If identified, a repeat echocardiogram or MRI should be performed to confirm the diagnosis. The alternate vascular abnormality of a double aortic arch would not change the approach on the right side.

Long Thoracic Nerve Injury

• Consequence

In 89 patients undergoing thoracotomy for TEF repair, 29 (33%) had significant musculoskeletal abnormalities, and 24% demonstrated long thoracic nerve injury.¹¹ The long thoracic nerve is purely motor and originates from the fifth to seventh cervical roots and supplies the serratus anterior. The serratus anterior is responsible for the abduction and elevation of the superior limb and can act as an accessory muscle during inspiration. Paralysis of the muscle causes “winged scapula,” in which the scapula moves away from the thoracic wall, the shoulder falls down, and the arm cannot be lifted higher than 90° when stretched outward.

Grade 4 complication

• Repair

In most cases, serratus anterior paralysis secondary to thoracotomy will resolve over 6 months.⁸ If conservative treatment is unsuccessful, the scapula will require loose fixation to the chest wall.¹²

• Prevention

Bianchi and associates ¹³ proposed the use of an axillary skin crease muscle-sparing incision through the third or fourth intercostal space. Exposure was not restricted, scar aesthetic was excellent, and no significant difference was found regarding duration of operation, postoperative ventilation, or the incidence of anastomotic stricture.¹⁴

Thoracoscopic repair has been shown to be a safe alternative when performed by experienced surgeons.¹⁵ The thoracoscopic approach decreases the morbidity associated with the thoracotomy with no subsequent increase in morbidity and/or mortality.