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Abstract
It has been suggested that multiple intestinal atresias result from multiple ischemic
infarctions of the intestinal tract. We have studied surgical material from 59 neonates
with intestinal atresias seen at our hospital between 1975 and 1986. Forty (68%) patients
had single intestinal atresias and 19 (32%) had multiple atresias. There were seven
cases of hereditary multiple atresias seen in three families and 12 cases of nonhereditary
multiple atresias. All hereditary cases had numerous type I or type II gastrointestinal
atresias but none had type IIIa atresia. Six of the seven hereditary cases had multiple
atresias in the small as well as large bowel. The 12 patients with nonhereditary atresias
had various types of atresias but mesenteric or intestinal interruption was observed
in only two patients. All patients with hereditary multiple intestinal atresias showed
identical microscopic appearances in the small and large intestine, consisting of
sieve-like multiple lumina, each surrounded by its own mucosa and muscularis mucosae
but sharing a common muscle coat. There was no evidence of lanugo, bile pigments,
or squames within the lumen distal to atretic segments in any of these patients. Six
nonhereditary cases who had multiple septal atresias affecting only the small bowel
demonstrated essentially similar lesions on microscopic examination as seen in hereditary
cases. There was no evidence of arterial occlusion in the mesentery and lanugo, bile
pigments, and squames could not be found distally in the intestinal contents in any
of these cases. These pathologic findings suggest that all cases of hereditary multiple
intestinal atresias and some cases of nonhereditary multiple intestinal atresias are
a consequence of a malformative process of the gastrointestinal tract rather than
an ischemic process.