羊膜带综合征相关畸形与胚胎发育异常关系的案例分析  

Relation between pathoembryogenesis and amniotic band syndrome

作　　者：韦玮[1] 武卫平[1] 顾芸[1] 

机构地区：[1]南京医科大学附属南京妇幼保健院病理科,南京210004

出　　处：《解剖学杂志》2010年第5期621-625,共5页Chinese Journal of Anatomy

摘　　要：目的：报告18例羊膜带综合征（ABs），对其伴有的复杂畸形进行病理解剖学观察，从胚胎发生学的角度探讨其与羊膜带粘连致畸的关系。方法：对18例ABS进行尸体解剖，并对13例羊膜粘连带及受累组织和胎盘进行组织学观察。结果：本组多为严重的不对称的复合性畸形，包括头面部、腹壁及肢体等异常，所有病例均找到明确的羊膜带粘连证据。胎盘显示羊膜与绒毛膜分离，绒毛膜裸露。15例脐带明显短于正常值或无真性脐带。粘连带为纤维结缔组织，局部有胚外中胚层特征，可见羊膜上皮。皮肤或头皮-羊膜粘连处为羊膜的中胚层面粘合于表皮下的结缔组织，腹壁缺损缘为羊膜逐渐向皮肤鳞状上皮移行（羊膜-外胚层过渡带）。结论：ABS主要表现为外表面组织器官缺陷；受累组织器官由于其在发生的关键时期，因羊膜囊破裂及羊膜带粘连受到干扰而致畸；畸形的严重程度及范围取决于羊膜囊破裂的时间和部位。Objective： To observe and study the features of 18 cases accompanied amniotic band syndrome （ABS） with complex malformations by pathoanatomy, and approach its relation with the amnion band adhering the fetus by the embryogenetic etiopathogeneisis. Methods： The autopsy was performed in 18 cases of ABS. Light microscopic examination was conducted in 13 cases of the amniotic bands, and the tissues involved and the placentas. Results： The majority of 18 cases were severe, asymmetrical and complex malformations, including head and facial, abdominal wall and extremity abnormality. While the evidence of amniotic band adhering to the different tissues of dead fetus could be found in them. In some of the placentas, the amnion detached from the chorion and the chorion was exposed. The umbilical cords of 15 cases were clearly shorter than the normal value or the definite umbilical cords could not be found at all. Histologically, the adhering band was constructed of fibrous connective tissue, which had the features of extraembryonic mesoderm locally and there was the amnion epithelium. The skin adhering to the amnion showed the mesoderm of amnion sticking to the connective tissue under the epidermis in histology. A transition from the amnion into the squamous epithelium was found in the skin-membranous zone of AWD （The amnio-ectodermal transition zone）. Conclusion： ABS mainly manifested the superficial external organs abnormity. During the critical embryogenetic time, the amniotic sac ruptured and the amnion band adhered to the fetus and then induced the deformity. The gestational age at which the amniotic sac ruptured and the position were believed to be the major determining factors in influencing the range and severity of deformities found in ABS.

关 键 词：羊膜带综合征 羊膜 粘连 畸形 胚胎发生 

分 类 号：R714.53[医药卫生—妇产科学]