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Congenital esophageal stenosis associated with esophageal atresia/tracheoesophageal fistula: clinical and radiologic features

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Abstract

Background

Congenital esophageal stenosis (CES) can be associated with esophageal atresia/tracheoesophageal fistula (EA/TEF). Because there are a variety of degrees of obstruction and symptoms of CES, it is frequently difficult to make a pre- and post-operative diagnosis of the distal CES associated with EA/TEF.

Objective

To evaluate the clinical and radiologic features of congenital esophageal stenosis associated with esophageal atresia/tracheoesophageal fistula.

Materials and methods

We retrospectively reviewed postoperative esophagograms and medical records of 187 children (107 boys, 80 girls) who had primary repair of EA/TEF from 1992 to 2009 at our institution. We evaluated the incidence of CES, clinical findings, radiologic features and management of CES in these children.

Results

CES was diagnosed in 22 of 187 EA/TEF children (12%); one child had double CES lesions, for a total of 23 lesions. Ten of those 22 children (45%) had presented with significant symptoms of esophageal obstruction. The diagnosis of CES was delayed in 10 children (45%) until 1–10 years of age. On esophagogram, CES (n = 23) was located in the distal esophagus (n = 20, 87%) or mid-esophagus (n = 3, 13%). The degree of stenosis was severe (n = 6, 26%), moderate (n = 10, 43%), or mild (n = 7, 30%). Eight children, including two with unsuccessful esophageal balloon dilatation of CES, were treated surgically. Histologic examination revealed tracheobronchial remnant (n = 7) or fibromuscular hyperplasia (n = 1). One child with surgically treated CES developed achalasia at the age of 3 years 9 months.

Conclusion

Esophagography after EA/TEF repair should be performed with a high index of suspicion for the presence of distal CES, because the diagnosis and adequate management of CES can often be delayed.

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Author notes

  1. So-Young Yoo

    Present address: Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea

Authors and Affiliations

  1. Department of Radiology, Seoul National University College of Medicine and the Institute of Radiation Medicine, 101 Daehangno, Jongno-Gu, Seoul, 110-744, South Korea

    Hye Jin Yoo, Woo Sun Kim, Jung-Eun Cheon, So-Young Yoo, Su-Mi Shin, In-One Kim & Kyung Mo Yeon

  2. Department of Pediatric Surgery, Seoul National University Children’s Hospital, Seoul, South Korea

    Kwi-Won Park & Sung-Eun Jung

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  1. Hye Jin Yoo
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Correspondence to Woo Sun Kim.

Additional information

A related editorial can be found at doi:10.1007/s00247-010-1601-2.

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Yoo, H.J., Kim, W.S., Cheon, JE. et al. Congenital esophageal stenosis associated with esophageal atresia/tracheoesophageal fistula: clinical and radiologic features. Pediatr Radiol 40, 1353–1359 (2010). https://doi.org/10.1007/s00247-010-1603-0

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  • DOI: https://doi.org/10.1007/s00247-010-1603-0

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