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Congenital Esophageal Stenosis

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Abstract

The definition proposed by Fékété needs to be modified to include the whole spectrum of CES. The histopathologic picture of the intrinsic type may show fibromuscular disease (FMD) or tracheobronchial remnants (TBR). CES can be an isolated lesion or associated with esophageal atresia and/or tracheoesophageal fistula (EA and/or TEF). The presentation can be early or late. Some may have a benign course whereas others may have a very stormy one. TBR needs to be differentiated by means of histological examination at the anastomotic site or using miniprobe endoscopic ultrasonography. Dilatation is usually the initial management. FMD responds better to dilatation, whereas the TBR usually needs resection.

The development of the esophagus and trachea is due to simple reduction of the size of the foregut, caused by a system of folds that approximates, but do not fuse. The imbalance in the growth of this set of folds would lead to different forms of malformation. Advances in genetics and molecular biology, lead to focusing on the process that occurs at a cellular level and gene patterns of expression and elucidating signaling pathways.

The congenital membranous disease (MD) has been considered to represent a missed form of EA. The Fibromuscular disease or stenosis (FMD) has no clear embryologic or pathogenic factors to explain these lesions. The Tracheobronchial Remnants (TBR form) occurs as a part of spectrum of anomalies, including EA/TEF, related to separation of the foregut from the respiratory tract around the 25th embryonic day.

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References

  1. Fékété CN, Backer AD, Jacob SL. Congenital esophageal stenosis, a review of 20 cases. Pediatr Surg Int. 1987;2:86–92. https://doi.org/10.1007/BF00174179.

    Article  Google Scholar 

  2. Olguner M, Ozdemir T, Akgur FM, Aktuğ T. Congenital esophageal stenosis owing to tracheobronchial remnants: a case report. J Pediatr Surg. 1997;32:1485–7. https://doi.org/10.1016/S0022-3468(97)90570-4.

    Article  CAS  PubMed  Google Scholar 

  3. Hokama A, Myers NA, Kent M, Campell PE, Chow CW. Esophageal atresia with tracheoesophageal fistula. A histopathologic study. Pediatr Surg Int. 1986;1:117–21. https://doi.org/10.1007/BF00166872.

    Article  Google Scholar 

  4. Kawahara H, Imura K, Yagi M, Kubota A. Clinical characteristic of congenital oesophageal stenosis distal to associated oesophageal atresia. Surgery. 2001;129:29–3.

    Article  CAS  Google Scholar 

  5. Yeung CK, Spitz L, Brereton RJ, Kiely EM, Leake J. Congenital esophageal stenosis due to tracheobronchial remnants: a rare but important association with esophageal atresia. J Pediatr Surg. 1992;27:852–5. https://doi.org/10.1016/0022-3468(92)90382-H.

    Article  CAS  PubMed  Google Scholar 

  6. Ibrahim AH, Al Malki TA, Hamza AF, Bahnasy AF. Congenital esophageal stenosis associated with esophageal atresia: new concepts. Pediatr Surg Int. 2007;23:533–7. https://doi.org/10.1007/00383-1927-5.

    Article  PubMed  Google Scholar 

  7. Al-Shraim MM, Ibrahim AHM, Al Malki TA, Morad N. Histopathologic profile of esophageal atresia and tracheoesophageal fistula. Ann Pediatr Surg. 2014;10:1–6. https://doi.org/10.1097/01.XPS.0000438124055523006.

    Article  Google Scholar 

  8. Ibrahim AHM, Bazeed MF, Jamil S, Hamad HA, Abdel Raheem IM, Ashraf I. Management of congenital esophageal stenosis associated with esophageal atresia and its impact on postoperative esophageal stricture. Ann Pediatr Surg. 2016;12:36–4. https://doi.org/10.1097/01.XPS.0000482656.06000.84.

    Article  Google Scholar 

  9. Teuri K, Saito T, Mitsunaga T, Nakata M, Yoshida H. Endoscopic management for congenital esophageal stenosis: a systematic review. World J Gastrointest Endosc. 2015;7(3):183–91. https://doi.org/10.4253/wige.v7.13.183.

    Article  Google Scholar 

  10. Michaud L, Coutenier F, Podevin G, Bonnard A, Becmeur F, Khen-Dunlop N, et al. Characteristics and management of congenital esophageal stenosis: findings from a multicenter study. Orphanet J Rare Dis. 2013;8:186–90. https://doi.org/10.1186/1750-1172-8-186.

    Article  PubMed  PubMed Central  Google Scholar 

  11. Boeckxstaens GE. The lower esophageal sphincter. Neurogastroenterol Motil. 2005;17(Suppl 1):13–21. https://doi.org/10.1111/j.1365-2982.20050066.

    Article  PubMed  Google Scholar 

  12. Kluth D, Steding G, Seidle W. The embryology of the foregut malformation. J Pediatr Surg. 1987;22:389–93. https://doi.org/10.1016/S0022-3468(87)80254-3.

    Article  CAS  PubMed  Google Scholar 

  13. Gray SW. The esophagus. In: Gray SW, Skandalakis JE, editors. Embryology for surgeons. 1st ed. Philadelphia, PA: Saunders; 1972. p. 63–99.

    Google Scholar 

  14. Zaw-Tun HA. The tracheoesophageal septum – fact or fantasy? Acta Anat. 1982;114:1–21.

    Article  CAS  Google Scholar 

  15. Diez-Pardo JA, QiB NC, Tovar JA. A new model of experimental model of esophageal atresia and tracheoesophageal fistula: preliminary report. J Pediatr Surg. 1996;31:498–502.

    Article  CAS  Google Scholar 

  16. Possögel AK, Diez-Pardo JA, Morales C, Navarro C, Tovar JA. Embryology of esophageal atresia in the Adriamycin rat model. J Pediatr Surg. 1998;33:606–12.

    Article  Google Scholar 

  17. Merei J, Kotsios C, Hutson JM, Hasthorpe S. Histopathologic study of esophageal atresia and tracheoesophageal fistula in an animal model. J Pediatr Surg. 1997;32:12–4. https://doi.org/10.1016/S0022-3468(97)90081-6.

    Article  CAS  PubMed  Google Scholar 

  18. Crisera CA, Connelly PR, Marmureanu AR, Colen KL, Rose MI, Li M, et al. Esophageal atresia with tracheoesophageal fistula: suggested mechanism in faulty organogenesis. J Pediatr Surg. 1999;34(1):204–8. https://doi.org/10.1016/S0022-3468(99)90258-0.

    Article  CAS  PubMed  Google Scholar 

  19. Robertson SP, Beasley SW. The genetics and molecular biology of esophageal development. In: Till H, et al., editors. Esophageal and gastric disorders in infancy and children. Berlin: Springer; 2017. p. 9–27. https://doi.org/10.1007/978-3-642-11202-7_2.

    Chapter  Google Scholar 

  20. Williams A, Qi BQ, Beasley SW. Three dimensional imaging clarifies the process of tracheoesophageal separation in the rat. J Pediatr Surg. 2003;38:173–7. https://doi.org/10.1053/jpsu.2003.50037.

    Article  PubMed  Google Scholar 

  21. Kluth D. Atlas of esophageal atresia. J Pediatr Surg. 1976;11:901–19. https://doi.org/10.1016/S0022-3468(76)80066-8.

    Article  CAS  PubMed  Google Scholar 

  22. Paterson WG, Mayrand S, Mercer CD. The esophagus. In: Thomson ABR, Shaffer EA, editors. First principles of gastroenterology and hepatology. Mankato, MN: CAPstone; 2012. p. 45–78.

    Google Scholar 

  23. Geboes K, Desmet V. Histology of the esophagus. Front Gastrointest Res. 1978;3:1. https://doi.org/10.1159/000400844.

    Article  CAS  PubMed  Google Scholar 

  24. Bradbury S. The digestive system. In:Hewer’s text book of histology for medical students. 9th ed; 1976. p. 292.

    Google Scholar 

  25. Botha GSM. Mucosal folds at the cardia as a component of the gastro-esophageal junction closing mechanism. Br J Surg. 1958;45(194):569–80. https://doi.org/10.1002/bjs.18004519402.

    Article  CAS  PubMed  Google Scholar 

  26. Bombeck CT, Dillard DH, Nyhus LM. Muscular anatomy of the gastroesophageal junction and role of phrenoesophageal ligament. Ann Surg. 1966;164(4):643–54.

    Article  CAS  Google Scholar 

  27. Gupta R, Sharma P, Shukla A, Mehra S. Kluth type Iv3 membranous esophageal atresia at the middle one-third of esophagus: an extremely rare entity. J Indian Assoc Pediatr Surg. 2017;22:254–6. https://doi.org/10.4103/jiaps.JIAPS_263_16was.

    Article  PubMed  PubMed Central  Google Scholar 

  28. Margarit J, Castanon M, Ribo JM, Rodo J, Muntaner A, Lee KW, et al. Congenital esophageal stenosis associated with tracheoesophageal fistula. Pediatr Surg Int. 1994;9:577–8. https://doi.org/10.1007/BF00179686.

    Article  Google Scholar 

  29. Frey EK, Duschl L. Cardiospasms. Ergeb Chirur Orthop. 1936;29:637–716.

    Google Scholar 

  30. Dunbar JS. Congenital esophageal stenosis. Pediatr Clin N Am. 1958;5:433–55. https://doi.org/10.1016/S0031-3955(16)30660-5.

    Article  Google Scholar 

  31. Spitz L. Congenital esophageal stenosis distal to associated esophageal atresia. J Pediatr Surg. 1973;8:973–4. https://doi.org/10.1016/0022-3468(91)90999-A.

    Article  CAS  PubMed  Google Scholar 

  32. Deiraniya AK. Congenital esophageal stenosis due to tracheobronchial remnants. Thorax. 1973;29:720–5.

    Article  Google Scholar 

  33. Harrison CA, Katon RM. Familial multiple congenital esophageal rings: report of an affected father and son. Am J Gastroenterol. 1992;87:1813–5.

    CAS  PubMed  Google Scholar 

  34. Rangel R, Lizarzabel M. Familial multiple congenital esophageal rings. Dig Dis. 1998;16:325. https://doi.org/10.1159/000016882.

    Article  CAS  PubMed  Google Scholar 

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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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Ibrahim, A., Al-Malki, T. (2019). General. In: Congenital Esophageal Stenosis. Springer, Cham. https://doi.org/10.1007/978-3-030-10782-6_1

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  • DOI: https://doi.org/10.1007/978-3-030-10782-6_1

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