Congenital agastria.
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.
Dorney
S F
SF
Department of Gastroenterology, Royal Alexandra Hospital for Children, Camperdown, New South Wales, Australia.
Middleton
A W
AW
Kozlowski
K
K
Benjamin
B N
BN
Kan
A E
AE
Kamath
K R
KR
D002675:Child, Preschool; D004751:Enteritis / Q000209:etiology; D006801:Humans; D007223:Infant; D007583:Jejunum / Q000637:transplantation; D008297:Male; D008844:Micrognathism; D011859:Radiography; D013270:Stomach / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery