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 normal life after surgery. That was not true until 1939, when Logan Leven of Minneapolis¹ and William E. Ladd of Boston² independently saved a newborn with esophageal atresia on the same date! The operation consisted of dividing the tracheoesophageal fistula (TEF), marsupializing the blindly ending upper esophageal pouch, and feeding the baby temporarily through a gastrostomy. Later, a multistaged reconstruction was performed to make an antethoracic esophageal substitute, which was placed subcutaneously anterior to the sternum. 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 comprised 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.

An important milestone in surgery for esophageal atresia was an insightful paper by Dr. Thomas Lanman, a colleague of Ladd, in 1940 describing a series of 32 esophageal atresia failures at Children's Hospital Boston.³ Lanman predicted, “Given a suitable case in which the patient is seen early, I feel that with greater experience, improved technique, and good luck, the successful outcome of a direct anastomosis can and will be reported in the near future.” This type of staged repair was abandoned in 1943 when Dr. Cameron Haight⁴ of Ann Arbor, Michigan, was 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 developed subsequently and are also described in this chapter.

Esophageal Atresia and Tracheoesophageal Fistula

The spectrum of pathology in infants with esophageal atresia, with or without TEF, is illustrated in Fig. 51-1. These include esophageal atresia with distal TEF, without TEF, with proximal TEF, and with double (proximal and distal) TEF. Isolated TEF without esophageal atresia, so-called H-fistula that usually occurs in the neck rather than midmediastinum, is also encountered. In most infants, esophageal atresia occurs with a single TEF. This form is observed in approximately 85% of affected infants. The upper esophageal pouch ends blindly, and there is a gap between it and the lower esophageal segment. The fistula from the lower esophageal segment usually enters the trachea just above its bifurcation. The blind upper pouch actually may overlap the nearby lower esophageal segment, or the two ends may be separated by a centimeter or more. Esophageal atresia without TEF is the next most common form and is seen in approximately 8% of infants.