Congenital esophageal stenosis

Shintaro Amae, Masaki Nio, Yutaka Hayashi, Ryoji Ohi

Research output: Chapter in Book/Report/Conference proceedingChapter


The difficulties in obtaining the differential diagnosis of congenital esophageal stenosis (CES) from achalasia and secondary esophageal stenosis, especially stricture due to reflux esophagitis,1 has resulted in various clinical problems in its treatment. CES is defined as an intrinsic stenosis of the esophagus caused by the congenital malformation of the esophageal wall architecture. This malformation may result from: • Tracheobronchial remnants in the esophageal wall2-16 • Fibromuscular thickening of the esophageal wall1,11,17-21 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 tracheobronchial 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.11,26-30 In general, the stenotic area in cases with tracheobronchial remnants is usually identified, 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.31-33 In the stenotic segment caused by tracheobronchial remnants, mature or immature cartilage, the seromucous tracheobronchial glands and ciliated epithelium are generally found microscopically. Tracheobronchial remnants are believed to be the result of failure in the normal separation of the embryonic respiratory tract from the foregut. In fibromuscular cases, circumferential proliferation of smooth muscle fibers with moderate fibrosis was revealed.11,21,34 Singaram et al. reported a significant reduction of myenteric nitrinergic neurons and fibers in the muscle layer of two young adults diagnosed with CES.35 Lack of submucosa27 and loose, vascular connective tissue and diffuse lymphocytes26 were observed microscopically in specimens of membranous web.

Original languageEnglish
Title of host publicationNewborn Surgery, Second Edition
PublisherCRC Press
Number of pages6
ISBN (Electronic)9781444113891
ISBN (Print)9780340761441
Publication statusPublished - 2003 Jan 1

ASJC Scopus subject areas

  • Medicine(all)


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