Congenital esophageal pathology

Abstract

Congenital esophageal stenosis is an intrinsic stenosis of the esophagus, present at birth, which is caused by congenital malformation of the esophageal wall architecture. Three forms of congenital esophageal stenosis exist: a membranous web or diaphragm occluding the esophageal lumen, fibromuscular hypertrophy of the esophageal wall, and tracheobronchial remnants embedded in the esophageal wall (Nihoul-Fekete et al., Pediatr Surg Int 2:86-92, 1987). The membranous web or diaphragm, the rarest of the three forms, consists of a partially obstructing lesion with an eccentric opening covered on both sides with squamous epithelium. These are located in the mid to distal esophagus. Stenosis due to fibromuscular hypertrophy exhibits normal squamous epithelium overlying a proliferation of smooth muscle fibers and fibrous connective tissue similar to that seen in hypertrophic pyloric stenosis, leading to narrowing in the mid to distal esophagus. This tapering is more gradual than the narrowing seen with tracheobronchial remnants. Tracheobronchial remnants, the most common congenital esophageal stenosis, results from abnormal separation of the esophagus and tracheobronchial tree during embryologic development resulting in cartilaginous remnants ectopically located in the esophageal wall. The narrowing occurs in the distal esophagus, is covered by normal mucosa, and is often abrupt in appearance due to a ring of cartilage in the esophageal wall. The cartilaginous remnants may or may not form a complete ring.