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Congenital Abnormal Rotation of the Intestines

AMA Arch Surg. 1957;75(1):146-149. doi:10.1001/archsurg.1957.01280130152028.
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Anatomists recognized and described congenital abnormal intestinal rotations more than 100 years ago.1 The significance of this maldevelopment from a clinical viewpoint was not fully appreciated until a little more than 30 years ago.1-3

In the past two decades increasing experience with congenital abnormal rotation of the intestines and related incomplete peritoneal fixation has resulted in well-established and precisely defined clinical syndromes.2-9

The embryology of this abnormality has been well described.2,3,6,8,10 Some difference in opinion exists, referable to the exact mechanics of midgut rotation. There is, however, complete agreement concerning the pathology, diagnosis, and management of the resultant clinical entities.

Failure of proper rotation and related peritoneal fixation is believed to occur during the first 5 to 12 weeks of gestation. The midgut, growing faster than the surrounding celomic cavity, projects into the umbilical cord. As development continues, the peritoneal cavity increases in size, with the


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