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Congenital agastria.

S F Dorney·A W Middleton·K Kozlowski·B N Benjamin·A E Kan·K R Kamath
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Abstract

Congenital agastria has never before been reported. Congenital microgastria is rare but is well documented. We have cared for a Melanesian child in whom there was no radiological, anatomical, or physiological evidence of either a rudimentary stomach or a pylorus. The esophagus joined the first part of the duodenum directly. At this esophago-duodenal junction there was microscopic evidence of fundic-type gastric mucosa. The intestine was normally rotated and there was a functioning spleen. The child also had a severe micrognathia and a cleft soft palate and required a tracheostomy to relieve upper airway obstruction. The combination of micrognathia and agastria presented a difficult nutritional delivery problem. A jejunal pouch was constructed and placed between the esophagus and duodenum to provide some storage capacity. The child grew and developed well for almost 3 years. He suffered three episodes of severe acute enteritis, probably related to lack of the protective acid-pepsin barrier against ingested microorganisms. Unfortunately, the third episode of acute enteritis caused his death 3 months after returning home to his own country.

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Child, PreschoolEnteritisHumansInfantJejunumMaleMicrognathismRadiographyStomachCase ReportsJournal Article

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S F Dorney, A W Middleton, K Kozlowski, B N Benjamin, A E Kan, K R Kamath. Congenital agastria.. https://doi.org/10.1097/00005176-198703000-00026

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