Chapter 51: Congenital Disorders of the Esophagus in Infants and Children

INTRODUCTION

This chapter presents the most common congenital malformations of the esophagus that require surgical correction in infants and children. Today, most of these entities can be corrected, and a child can lead a near normal life after surgery. That was not true until 1939, when Logan Leven of Minneapolis¹ and William E. Ladd of Boston² independently each saved a newborn with esophageal atresia on the same date! The operation consisted of dividing the tracheoesophageal fistula (TEF), marsupializing the blind-ending upper esophageal pouch as an esophagostomy, and feeding the baby temporarily through a gastrostomy. Later, a multistaged reconstruction was performed to make an ante-thoracic esophageal substitute, which was placed subcutaneously anterior to the sternum.³ The lower two-thirds of this conduit consisted of a Roux-en-Y loop of upper jejunum that bypassed the stomach and duodenum. The upper third of the conduit was a tubularized full-thickness graft composed of skin and subcutaneous tissue. This was used to bridge the gap between the upper esophageal segment, which had been marsupialized in the neck, and the Roux-en-Y loop, which was brought up to the level of the upper sternum.³

This type of staged repair was abandoned in 1943 when Dr. Cameron Haight⁴ of Ann Arbor, Michigan, was the first to report the primary definitive repair of esophageal atresia and TEF in the neonate. This was an important step in thoracic surgery for infants and children. Surgical treatments for other congenital esophageal anomalies were subsequently developed and also are described in this chapter.

ESOPHAGEAL ATRESIA AND TRACHEOESOPHAGEAL FISTULA

The incidence of esophageal atresia is approximately 1 in 2500–4000.^5,6 The spectrum of pathology in infants with esophageal atresia, with or without TEF, is illustrated in Fig. 51-1. These include esophageal atresia without TEF (A), with proximal TEF (B), with distal TEF (C), and with double (proximal and distal) TEF (D). Isolated TEF without esophageal atresia (E), so-called H-fistula that usually occurs in the neck rather than mid-mediastinum, also is encountered (Fig. 51-1A–E).⁷ In most infants, esophageal atresia occurs with a blind-ending upper pouch and a single TEF connected to the trachea near the level of the carina.⁷ This form, Gross type C, is observed in approximately 85% of affected infants. The gap between the lower esophageal segment and blind upper pouch can range from more than 6 cm centimeters apart to nearly overlapping. Esophageal atresia without TEF (type A, or pure esophageal atresia) is the next most common form and is seen in approximately 8% of infants.⁷ Most commonly, the infants have a long gap separating the lower and upper esophageal ends with the distal segment often being quite short and thin.