The Pyloric Sphincteric Cylinder in Health and Disease



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Chapter 21 (page 95)


Chapter 21

Congenital Anomalies

Congenital anomalies of the pyloric region of the stomach are rare (Thompson et al. l968; Lamesch and Schneider l973; Korber and Glasson l977). The cause is usually thought to be localized vascular impairment occurring during foetal life (Bremer l944; Louw l959).

Pyloric Atresia

Almost all cases of gastric atresia occur in the pyloric region (Franken l982). The condition may present as a membrane occluding the lumen, as a gap in continuity, or a fibrous cord intervening between patent portions at the gastroduodenal junction. Some cases have been associated with epidermolysis bullosa (Korber and Glasson l977; Franken l982). Clinically the condition presents as upper abdominal distension in a newborn infant, with bile-free vomiting from birth (Davis and Douglas l961; Thompson et al. l968; Bronsther et al. l97l). Maternal hydramnios occurs in approximately 50 percent of cases (Thompson et al. l968; Bronsther et al. l97l).

Radiographic examination shows the stomach to be distended, with complete absence of gas in the remainder of the gastrointestinal tract. In the erect abdominal film this gives rise to a single air-fluid level in the stomach, topped by a gas bubble (the "single bubble" sign) (Davis & Douglas l961; Becker et al. l963; Bronsther et al. l97l). According to Franken (l982) neonatal gastric hypotonia, which is a much more frequent condition, may give a similar appearance. (In hypotonia gastric inactivity with lack of peristalsis and cyclical contraction of the pyloric sphincteric cylinder may delay gastric emptying as described in Chapter l9).

Discussion

Gastric atresia usually affects the region of the pyloric sphincteric cylinder; whether it is limited to the cylinder is not known.

References

  1. Becker JM, Schneider KM, Fischer AE. Pyloric atresia. Arch Surg l963, 87, 413-416.
  2. Bremer JL. Diverticula and duplications of the intestinal tract. Arch Path l944, 38, 132-140.
  3. Bronsther B, Nadeau MR, Abrams MW. Congenital pyloric atresia: a report of three cases and a review of the literature. Surgery l97l, 69, 130-136.
  4. Davis DA, Douglas KR. Congenital pyloric atresia, a rare anomaly. Ann Surg l96l, 153, 418-422.
  5. Franken EA. Gastrointestinal Imaging in Pediatrics. Harper and Row, Philadelphia l982, 2nd ed, p 138.
  6. Korber JS, Glasson MJ. Pyloric atresia associated with epidermolysis bullosa. J Pediatr l977, 90, 600-601.
  7. Lamesch A, Schneider H. Atrésie et sténose congenitales du pylore. Acta Paed Belgica l973, 27, 169-183.
  8. Louw JH. Congenital intestinal atresia and stenosis in the newborn; observations on its pathogenesis and treatment. Ann Roy Coll Surg l959, 25, 209- 234.
  9. Thompson NW, Parker W, Schwartz S, et al. Congenital pyloric atresia. Arch Surg l968, 97, 792-796.



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