Congenital Pyloric Stenosis, Webs and Strictures

Chapter
First Online:

Abstract

Congenital gastric outlet anomalies (CGOA) are extremely rare and represent only about 1 % of all gastrointestinal atresias. The term describes several different entities like congenital pyloric atresia (CPA), a congenital pyloric stenosis (not to be confused with a hypertrophic pyloric stenosis) and intraluminal mucosal webs with or without a central hole (windsock anomaly). Prompt endoscopic confirmation and surgical treatment are recommended. Cases with isolated anomalies usually have an excellent prognosis, whereas associations of HMIA (hereditary multiple intestinal atresias) or EHB (epidermolysis hereditaria bullosa) imply a high mortality due to sepsis and severe immunodeficiencies.

Keywords

Congenital gastric outlet obstructions Congenital pyloric stenosis Webs Strictures 
This is a preview of subscription content, log in to check access.

References

  1. 1.
    Al-Salem AH. Congenital pyloric atresia and associated anomalies. Pediatr Surg Int. 2007;23(6):559–63.CrossRefPubMedGoogle Scholar
  2. 2.
    Gahukamble DB, Adnan AR, Al Gadi M. Distal foregut atresias in consecutive siblings and twins in the same family. Pediatr Surg Int. 2003;19(4):288–92.CrossRefPubMedGoogle Scholar
  3. 3.
    Lin HH, Lee HC, Yeung CY, Chan WT, Jiang CB, Sheu JC, Wang NL. Congenital webs of the gastrointestinal tract: 20 years of experience from a pediatric care teaching hospital in Taiwan. Pediatr Neonatol. 2012;53(1):12–7.CrossRefPubMedGoogle Scholar
  4. 4.
    Cole C, Freitas A, Clifton MS, Durham MM. Hereditary multiple intestinal atresias: 2 new cases and review of the literature. J Pediatr Surg. 2010;45(4):E21–4.CrossRefPubMedGoogle Scholar
  5. 5.
    Bass J. Pyloric atresia associated with multiple intestinal atresias and immune deficiency. J Pediatr Surg. 2002;37(6):941–2.CrossRefPubMedGoogle Scholar
  6. 6.
    Moreno LA, Gottrand F, Turck D, Manouvrier-Hanu S, Mazingue F, Morisot C, Le Deist F, Ricour C, Nihoul-Feketé C, Debeugny P. Severe combined immunodeficiency syndrome associated with autosomal recessive familial multiple gastrointestinal atresias: study of a family. Am J Med Genet. 1990;37(1):143–6.CrossRefPubMedGoogle Scholar
  7. 7.
    Dank JP, Kim S, Parisi MA, Brown T, Smith LT, Waldhausen J, Sybert VP. Outcome after surgical repair of junctional epidermolysis bullosa-pyloric atresia syndrome: a report of 3 cases and review of the literature. Arch Dermatol. 1999;135(10):1243–7.CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2017

Authors and Affiliations

  1. 1.Department of Paediatric and Adolescent SurgeryGrazAustria

Personalised recommendations