Moerman P, Fryns J P, Vandenberghe K, Lauweryns J M
Department of Pathology I, Katholieke Universiteit, Leuven, Belgium.
Am J Med Genet. 1992 Feb 15;42(4):470-9. doi: 10.1002/ajmg.1320420412.
In a fetopathologic evaluation of 18 cases with amniotic bands, we discerned 3 types of lesions: (1) constrictive tissue bands, (2) amniotic adhesions, and (3) more complex anomaly patterns, designated as limb-body wall complex (LBWC). Constrictive bands are caused by primary amnion rupture with subsequent entanglement of fetal parts (mostly limbs) by shriveled amniotic strands. Adhesive bands are the result of a broad fusion between disrupted fetal parts (mostly cephalic) and an intact amniotic membrane. Most of the craniofacial defects (encephaloceles and/or facial clefts) occurring in these fetuses are not caused by constrictive amniotic bands, but are the result of a vascular disruption sequence with or without cephalo-amniotic adhesion. Our observations confirm the fact that amnion rupture is not a conditio sine qua non for the development of LBWC. However, LBWC is often complicated by rupture of the unsupported amnion with ensuing formation of constrictive bands. We think that the concept that considers the 3 lesions in question as a single pathogenetic entity is erroneous and will inevitably lead to a never-ending debate between followers of the 2 prevailing theories. In our view, the theories of Streeter and Torpin are not mutually exclusive but rather apply to different types of lesions. The recognition of constrictive amniotic bands, amniotic adhesions, and LBWC as discrete but often combined disruption sequences with important pathogenetic overlap may resolve many dilemmas in interpretation when a fetus exhibits classical constrictive bands beside more severe defects.
在对18例羊膜带病例的胎儿病理学评估中,我们识别出3种类型的病变:(1)紧缩性组织带,(2)羊膜粘连,以及(3)更复杂的异常模式,称为肢体-体壁复合体(LBWC)。紧缩性带是由原发性羊膜破裂导致胎儿部分(主要是肢体)随后被皱缩的羊膜束缠住引起的。粘连性带是胎儿部分(主要是头部)破裂与完整羊膜之间广泛融合的结果。这些胎儿中出现的大多数颅面缺陷(脑膨出和/或面部裂隙)并非由紧缩性羊膜带引起,而是血管破坏序列的结果,伴有或不伴有头-羊膜粘连。我们的观察证实了羊膜破裂并非LBWC发生的必要条件这一事实。然而,LBWC常常因无支撑的羊膜破裂并随后形成紧缩性带而复杂化。我们认为,将所讨论的这3种病变视为单一发病实体的概念是错误的,并且将不可避免地导致两种主流理论的支持者之间无休止的争论。我们认为,斯特里特和托平的理论并非相互排斥,而是适用于不同类型的病变。当胎儿除了有更严重的缺陷外还表现出典型的紧缩性带时,将紧缩性羊膜带、羊膜粘连和LBWC识别为离散但常合并的破坏序列且具有重要的发病机制重叠,可能会解决许多解释上的困境。