From the Department of Radiology, University of Utah School of Medicine, 30 North 1900 East, Room 1A071, Salt Lake City, UT 84132-2140.
Radiographics. 2015 Mar-Apr;35(2):636-49. doi: 10.1148/rg.352140104.
Abdominal wall defects are a complex group of anomalies, and many are incorrectly diagnosed. Evaluation of the defect relative to the umbilical cord insertion site is fundamentally important in differentiating among the various malformations. The two most common abdominal wall defects are gastroschisis, in which the defect is on the right side of the normally inserting cord and free-floating bowel loops are present, and omphalocele, in which the cord inserts on a membrane-covered midline defect. Omphalocele may also form a portion of a more complex defect that may remain undiagnosed without thorough evaluation. In cloacal exstrophy, the defect extends inferiorly and the bowel loops extrude between the two bladder halves. In pentalogy of Cantrell, the defect extends superiorly and is typically associated with ectopia cordis. Bladder exstrophy is a lower abdominal defect in which the hallmark finding is absence of a fluid-filled bladder. The cord insertion site is normal to low but does not form part of the defect. Both body stalk anomaly and abdominoschisis due to amniotic bands cause severe malformations, often involving extrusion of solid organs and the bowel. Although these two entities have many overlapping features, body stalk anomaly may be recognized on the basis of absence of a free-floating umbilical cord. With use of an algorithmic approach beginning with discovery of the location of the defect, a more precise diagnosis can be determined that may directly affect pre- and postnatal management decisions.
腹壁缺陷是一组复杂的异常,许多缺陷都被误诊。评估缺陷相对于脐血管插入部位对于区分各种畸形至关重要。最常见的两种腹壁缺陷是腹裂,其中缺陷位于正常插入脐带的右侧,并且存在游离的肠袢;脐膨出,其中脐带插入覆盖中线的膜缺陷。脐膨出也可能形成更复杂缺陷的一部分,如果没有彻底评估,可能仍未被诊断。在直肠外翻中,缺陷向下延伸,肠袢从两个膀胱半部分之间突出。在 Cantrell 五联征中,缺陷向上延伸,通常与异位心有关。膀胱外翻是一种下腹部缺陷,其标志性发现是膀胱无积液。脐带插入部位正常或较低,但不构成缺陷的一部分。体蒂异常和羊膜带引起的腹壁裂都导致严重的畸形,常涉及实质器官和肠的突出。尽管这两种实体有许多重叠的特征,但体蒂异常可能由于缺乏游离的脐带而被识别。使用从发现缺陷位置开始的算法方法,可以确定更精确的诊断,这可能直接影响产前和产后管理决策。