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Congenital Esophageal Stenosis

  • Michela MaffiEmail author
  • Mario Lima
Chapter

Abstract

Congenital esophageal stenosis (CES) is a rare entity with an estimated incidence of 1:25,000–50,000 live births. Three causes for CES have been described: tracheobronchial remnant, segmental fibromuscular hypertrophy, and membranous web. CES can be associated with other anomalies such as esophageal atresia, chromosomal anomalies, cardiac anomalies, intestinal atresia, anorectal malformation, and trisomy 21.

Main symptoms are dysphagia, vomiting during meals, regurgitation of undigested food, insufficient growth, and relapsing pneumonia due to aspiration and typically occur when solid foods are introduced in diet.

Diagnosis of esophageal stenosis is achieved with barium esophagogram and endoscopic study, but differential diagnosis among the three forms of CES can be difficult. Endoscopic ultrasonography may help in identifying correct diagnosis of cartilaginous remnant.

First-line treatment consists of esophageal dilations which lead to resolution of stenosis in most of the cases. Tracheobronchial remnants typically are unresponsive to dilations and require resection of the stenotic tract followed by end-to-end anastomosis. The most serious complication is the esophageal perforation after dilation.

Despite various therapeutic options, a consistent part of patients continues to complain of dysphagia and requires a follow-up for a long time.

Keywords

Esophageal Stenosis Esophageal Atresia Esophageal Perforation Anorectal Malformation Cartilaginous Tissue 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

References

  1. 1.
    McCann F, Michaud L, Aspirot A, Levesque D, Gottrand F, Faure C (2015) Congenital esophageal stenosis associated with esophageal atresia. Dis Esophagus 28(3):211–215CrossRefPubMedGoogle Scholar
  2. 2.
    Rebelo PG, Ormonde JV, Ormonde Filho JB (2013) Congenital esophageal stenosis owing to tracheobronchial remnants. Rev Paul Pediatr 31(3):406–410CrossRefPubMedPubMedCentralGoogle Scholar
  3. 3.
    Appignani A (1993) Stenosi esofagee congenite. In: Dòmini R, Lima M (eds) Chirurgia delle malformazioni digestive. Piccin, BolognaGoogle Scholar
  4. 4.
    van Poll D, van der Zee DC (2012) Thoracoscopic treatment of congenital esophageal stenosis in combination with H-type tracheoesophageal fistula. J Pediatr Surg 47(8):1611–1613CrossRefPubMedGoogle Scholar
  5. 5.
    Nihoul-Fèkètè C, De Backer A, Lortat-Jacob S et al (1987) Congenital esophageal stenosis: a review of 20 cases. Pediatr Surg Int 2:86–92CrossRefGoogle Scholar
  6. 6.
    Vasudevan SA, Kerendi F, Lee H, Ricketts RR (2002) Management of congenital esophageal stenosis. J Pediatr Surg 37(7):1024–1026CrossRefPubMedGoogle Scholar
  7. 7.
    Michaud L, Coutenier F, Podevin G et al (2013) Characteristics and management of congenital esophageal stenosis: findings from a multicenter study. Orphanet J Rare Dis 8:186CrossRefPubMedPubMedCentralGoogle Scholar
  8. 8.
    Bocus P, Realdon S, Eloubeidi MA, Diamantis G, Betalli P, Gamba P, Zanon GF, Battaglia G (2011) High-frequency miniprobes and 3-dimensional EUS for preoperative evaluation of the etiology of congenital esophageal stenosis in children (with video). Gastrointest Endosc 74(1):204–207CrossRefPubMedGoogle Scholar
  9. 9.
    Romeo E, Foschia F, de Angelis P et al (2011) Endoscopic management of congenital esophageal stenosis. J Pediatr Surg 46(5):838–841CrossRefPubMedGoogle Scholar
  10. 10.
    Jones DW, Kunisaki SM, Teitelbaum DH, Spigland NA, Coran AG (2010) Congenital esophageal stenosis: the differential diagnosis and management. Pediatr Surg Int 26(5):547–551CrossRefPubMedGoogle Scholar
  11. 11.
    Chao HC, Chen SY, Kong MS (2008) Successful treatment of congenital esophageal web by endoscopic electrocauterization and balloon dilatation. J Pediatr Surg 43(1):e13–e15CrossRefPubMedGoogle Scholar
  12. 12.
    Takamizawa S, Tsugawa C, Mouri N, Satoh S, Kanegawa K, Nishijima E, Muraji T (2002) Congenital esophageal stenosis: therapeutic strategy based on etiology. J Pediatr Surg 37(2):197–201CrossRefPubMedGoogle Scholar
  13. 13.
    Amae S, Nio M, Kamiyama T, Ishii T, Yoshida S, Hayashi Y, Ohi R (2003) Clinical characteristics and management of congenital esophageal stenosis: a report on 14 cases. J Pediatr Surg 38(4):565–570CrossRefPubMedGoogle Scholar
  14. 14.
    Maeda K, Hisamatsu C, Hasegawa T, Tanaka H, Okita Y (2004) Circular myectomy for the treatment of congenital esophageal stenosis owing to tracheobronchial remnant. J Pediatr Surg 39(12):1765–1768CrossRefPubMedGoogle Scholar
  15. 15.
    Saito T, Ise K, Kawahara Y, Yamashita M, Shimizu H, Suzuki H, Gotoh M (2008) Congenital esophageal stenosis because of tracheobronchial remnant and treated by circular myectomy: a case report. J Pediatr Surg 43(3):583–585CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing Switzerland 2017

Authors and Affiliations

  1. 1.Pediatric SurgeryS. Orsola-Malpighi Hospital, Bologna UniversityBolognaItaly

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