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Congenital Esophageal Stenosis pp 39–46Cite as

The Spectrum of CES

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Abstract

CES is defined as esophageal narrowing present at birth, but not essentially symptomatic, due to intra-luminal membranous disease (MD), intrinsic disease with textural abnormalities or extramural compression. The intrinsic causes involve a type with tracheobronchial remnant (TBR), another with segmental hypertrophy of the muscularis and diffuse fibrosis of the submucosa (FMD). A definitive incidence of subtypes cannot be determined. CES represents a wide spectrum of diseases. It can be associated with esophageal atresia (EA) or it can be an isolated lesion. The association of CES and EA ranges from 0.4 to 14%. CES may have associated EA in 20–60% of cases. CES associated with EA may affect the anastomotic site or distal to or it may involve the whole lower esophageal pouch. CES distal to the anastomotic site may be proximal to the gastroesophageal junction (GEJ) allowing free GER. An isolated CES can affect any part of the esophagus; upper, middle, lower esophagus or even the cardiac end mimicking cardiac achalasia. The MD may be an isolated lesion or associated with EA. It may be a complete diaphragm or a perforated one. Associated anomalies with CES may be as high as 17–52.5%.

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Author information

Authors and Affiliations

  1. Consultant Pediatric Surgeon, Armed Forces Hospital, Southern Region, King Fahad Military Hospital, Khamis Mushait, Saudi Arabia

    Ashraf Ibrahim

  2. Senior Consultant Pediatric and Neonatal Surgeon, Alhada Military Hospital, Vice President for D&Q, Taif University, Taif, Saudi Arabia

    Talal Al-Malki

Authors
  1. Ashraf Ibrahim
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  2. Talal Al-Malki
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Ibrahim, A., Al-Malki, T. (2019). The Spectrum of CES. In: Congenital Esophageal Stenosis. Springer, Cham. https://doi.org/10.1007/978-3-030-10782-6_4

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  • DOI: https://doi.org/10.1007/978-3-030-10782-6_4

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-10781-9

  • Online ISBN: 978-3-030-10782-6

  • eBook Packages: MedicineMedicine (R0)

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