Kirillova I A
Arkh Patol. 1976;38(2):84-7.
A rare case of congenital intestinal obstruction of hereditary nature, the so-called "pagoda" syndrome, is described. This syndrome consists in atresia of the initial department of the jejunum, considerable shortening of the intestine and in a peculiar deformation of the remaining part of the small intestine and its mesentery. The pattern of the blood supply of the intestine and its morphology support the suggestion that this syndrome is underlayed by agensia of the anterior knee of the middle intestine of the embryo, and its teratogenetic termination period does not exceed the first four weeks of embryogenesis.
本文描述了一例罕见的具有遗传性质的先天性肠梗阻,即所谓的“宝塔”综合征。该综合征表现为空肠起始部闭锁、肠管显著缩短以及小肠其余部分及其系膜的特殊变形。肠道的血液供应模式及其形态学支持了这样一种观点,即该综合征的基础是胚胎中肠前膝部发育不全,其致畸终止期不超过胚胎发育的前四周。