The Pyloric Sphincteric Cylinder in Health and Disease

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Chapter 21 (page 97)

Congenital Double Pylorus

Congenital double pylorus is an extremely rare condition. In reviewing their cases of gastro-intestinal duplications encountered over an l8 year period, Grosfeld et al. (l970) did not mention a single example of this anomaly. A case was reported by Christien et al (l97l), but subsequently doubt was expressed as to the congenital nature of the lesion (Hansen et al. l972). Acquired double pylorus, due to pyloroduodenal fistula, is not nearly as rare and is a well documented condition (Chap. 31).

Sufian et al. (l977) described a case which appears to be of true congenital origin. It concerned a 30 year old female with a long history of epigastric pain and aspirin and ethanol abuse. The radiological examination revealed two pyloric apertures; gastroscopy failed to show any abnormality. Although no evidence of active or previous ulceration was found at operation, a partial gastrectomy was performed. The resection specimen revealed a second pyloric opening 2.0 cm in length and 1.0cm in diameter, adjacent to the normal pylorus. The mucosa and muscularis mucosae of both apertures were normal, but a nodule of ectopic pancreatic tissue was situated in the wall of the second opening.

Gupta and Hollander (l977) described a case of achalasia of the oesophagus occurring in a 56 year old female. During endoscopic examination a double pyloric aperture was an unexpected finding. There was no evidence of peptic ulcer disease and it was considered to be a case of double pylorus of congenital origin.

Williams et al. (l98l) mentioned the case of a 35 year old male who had had duodenal ulcer symptoms for 5 years. Endoscopy showed a duodenal ulcer and a double pylorus proximal to the ulcer. At operation a septum between the 2 apertures was excised. It was composed of normal mucosa with a complete ring of muscularis mucosae, and the case was considered to be a double pylorus of congenital origin.


Congenital double pylorus is extremely rare and cases have not been encountered by us. Acquired double pylorus due to pyloroduodenal fistula is much less rare and is well documented.


  1. Christien G, Branthomme J, Volny L, et al. Pylore double: malformation congénitale. Sem Hôp Paris l97l, 47, 1485-1488.
  2. Grosfeld JL, O'Neill JA, Clatworthy HW. Enteric duplications in infancy and childhood: an l8 year review. Ann Surg l970, l72, 83-90.
  3. Gupta A, Hollander D. Duplication of the pylorus found concomitantly with achalasia: congenital or peptic etiology? Amer J Dig Dis l977, 22, 829-830.
  4. Hansen HO, Kronborg O, Pedersen T. The double pylorus. Scand J Gastroenterol l972, 7, 695-696.
  5. Sufian S, Ominsky S, Matsumoto T. Congenital double pylorus: a case report and review of the literature. Gastroenterology l977, 73, 154-157.
  6. Williams RS, Gilmore IT, Johnson AG. Congenital double pylorus: a case report. Brit J Surg l98l, 68, 65.

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