Multiple Congenital Septal Atresias of the Intestine: Histomorphologic and Pathogenetic Implications

Abstract

Congenital atresias of the gastrointestinal tract are usually single and divided into three forms on a morphologic basis. The septal or diaphragmatic type (type I) is the least common. We report an infant with multiple type I atresias involving both the small and large intestine and describe the unique histologic features of the septa: (1) fragmentation of muscularis mucosa, (2) multiple septal cysts lined by columnar epithelium, (3) circular and longitudinal muscular layers, and (4) absence of inflammation. This infant had a sibling who died with multiple intestinal atresias of the septal type.

Key Words:

• congenital atresia
• type I atresia
• septal atresia
• recanalization