Congenital esophageal stenosis

Difficulties in obtaining a differential diagnosis of congenital

esophageal stenosis (CES) from achalasia and secondary

esophageal stenosis, especially stricture due to reflux esopha-

gitis,1 have resulted in various clinical problems in its

treatment. CES is defined as an intrinsic stenosis of the

esophagus caused by congenital malformation of the esopha-

geal wall architecture. This malformation may result from:

= tracheobronchial remnants in the esophageal wall;216

= fibromuscular thickening of the esophageal wall;1,11,1721

= membranous mucosal diaphragm or web.8,11,2230

Achalasia, inflammatory esophagitis, and stenosis caused by

tumor or extrinsic compression of the esophagus should be

excluded from this category. The localization of the stenosis

varies with the type of pathology. Stenosis due to tracheo-

bronchial remnants is the most common type of pathological

anomaly, and is localized to the distal esophagus,12 whereas

fibromuscular thickening is generally found in the middle or

lower portions of the esophagus.11,21 Membranous webs are

normally observed in the upper or middle levels of the

esophagus.11,2630 In general, the stenotic area in cases with

tracheobronchial remnants is usually localized, and that of

fibromuscular stenosis varies from one to several centimeters

in length with circular thickening of the esophageal wall. In

almost all cases of membranous web, a single web is found

in children;26,28,30 however plural webs, termed multiple

trachea-like rings, are observed in young adults.3133

In the stenotic segment caused by tracheobronchial rem-

nants, mature or immature cartilage, the seromucous

tracheobronchial glands and ciliated epithelium are generally

found microscopically. Tracheobronchial remnants are be-

lieved to be the result of failure in the normal separation of

the embryonic respiratory tract from the foregut. In fibro-

muscular cases, circumferential proliferation of smooth

muscle fibers with moderate fibrosis was revealed.11,21,34

Singaram et al.35 reported a significant reduction of myen-

teric nitrinergic neurons and fibers in the muscle layer of two

young adults diagnosed with CES. Lack of submucosa27 and

loose, vascular connective tissue, and diffuse lymphocytes26

were observed microscopically in specimens of membranous

web.