Familial syndrome presenting with short small bowel, malrotation, and pyloric hypertrophy

Tanner, M. S., Smith, B., and Lloyd, J. K. (1976). Archives of Disease in Childhood, 51, 837. Functional Intestinal obstruction due to deficiency of argyrophil neurones in the myenteric plexus: famnlial syndrome presenting with short small bowel, malrotation, and pyloric hypertrophy. In 3 infants functional intestinal obstruction, associated with a short small intestine, malrotation, and pyloric hypertrophy, was shownto be due to failure of development oftheargyrophil myenteric plexus, with the absence of ongoing peristalsis. 4 infants with similar clinical features have been described previously, and there is evidence for an autosomal recessive mode of inheritance of this syndrome.