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Congenital Esophageal Stenosis Associated with Esophageal Atresia

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Esophageal and Gastric Disorders in Infancy and Childhood

Abstract

Congenital esophageal stenosis (CES) associated with esophageal atresia (EA) is common. The etiology of esophageal dysfunction includes textural abnormalities, excessive dissection, gastroesophageal reflux (GER), and the development of stricture. The muscles may be hypoplastic, distorted by fibrosis, cartilage, and/or respiratory glands. There are abnormalities in the intrinsic and extrinsic innervation of both esophageal pouches. Neuropeptides are also abnormal. Lack of nitric oxide (NO) inhibitory innervation may be an important mechanism in the pathogenesis of stenosis and aperistalsis. CES commonly involves the peri-anastomotic area and may extend distally. Surgical specimens from the tip of the lower esophageal pouch during primary repair of EA helped in the diagnosis of CES and its typing. The presence of cartilage usually requires surgical resection, while the other types may respond to balloon dilatations. Unlike isolated CES, GER is common. The presence of major esophageal dysmotility requires a gastrostomy and a partial anterior wrap. Further multicentric cooperation for histological, histochemical, and ultrastructural studies from both esophageal pouches is required. Results should be correlated clinically and videomanometrically.

The original version of this chapter was revised. An erratum to this chapter can be found at DOI 10.1007/978-3-642-11202-7_131

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Correspondence to Ashraf H. M. Ibrahim FRCSI, MD .

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Ibrahim, A.H.M., Al Malki, T.A. (2017). Congenital Esophageal Stenosis Associated with Esophageal Atresia. In: Till, H., Thomson, M., Foker, J., Holcomb III, G., Khan, K. (eds) Esophageal and Gastric Disorders in Infancy and Childhood. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-11202-7_9

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