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目的报告2例羊膜带综合征(ABS),对其伴有的复杂畸形进行分析,探讨羊膜带综合征相关畸形的相关因素、诊断及处理结果分析。方法对2例ABS进行B超影像结果分析,其中1例进行羊膜粘连带及受累组织进行组织学观察。结果本文1例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,该病例能找到明确的羊膜带粘连证据,粘连带为纤维结缔组织,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)。另1例为单纯的脑膜脑膨出畸形,所有病例的B超影像均在羊水中可见到漂浮的带状回声,其部分贴附于胎儿;羊膜带粘连处的胎儿肢体部分可见畸形,畸形部位常可扫查到羊膜的带状回声与之粘连。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。ABS重在预防,妊娠中期常规进行超声检查,早期诊断,早期干预。
Objective To report 2 cases of amniotic band syndrome (ABS), to analyze the complicated deformities associated with amniotic band syndrome, to analyze the related factors of diagnosis and treatment of amniotic band syndrome. Methods Two cases of ABS were analyzed by B-mode ultrasonography. One case was involved in the histological observation of the amniotic adhesions and the affected tissues. Results In this paper, one case of severe asymmetric composite deformity, including head and face, abdominal wall and limb abnormalities, the case can find a clear evidence of amniotic band adhesion, adhesions with fibrous connective tissue, visible amniotic epithelium, skin or scalp - Amniotic adhesions at the amnodeal mesoderm surface adhesion to the connective tissue under the epidermis, abdomen defect edge of the amniotic membrane gradually shift to the skin squamous epithelium (amnion - ectodermal transition zone). Another case of meningoencephala simple deformity, all cases of B ultrasound images are seen floating amniotic fluid ribbon echo, part of the attached to the fetus; amniotic band adhesion at the fetal limb part of the deformity can be seen, deformity site Often can be scanned to the amniotic ribbon echo and adhesion. Conclusion The main manifestation of ABS is the defect of the surface tissue and organ. The involved tissues and organs are teratogenic due to the rupture of the amniotic sac and the amniotic membrane adhesions in the critical period. The severity and scope of the deformity depend on the time of amniotic sac rupture and Site. ABS focus on prevention, mid-pregnancy routine ultrasound examination, early diagnosis, early intervention.