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机构地区:[1]广西省百色市妇幼保健院优生遗传科,广西百色533000
出 处:《中国优生与遗传杂志》2013年第1期82-83,37,F0003,共4页Chinese Journal of Birth Health & Heredity
摘 要: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),its associated with malformation complex analysis,explore the amniotic band syndrome associated malformations associated factors,diagnosis and treatment result analysis.Methods: 2 cases of ABS B ultrasound image analysis of results,which a case of amniotic adhesion bands and the involved tissues for histological observation.Results: In the 1 cases of serious asymmetric composite deformities,including the head and face,abdominal and limb abnormalities,the case can be found clear evidence of amniotic band adhesion,adhesion with fibrous connective tissue,visible amniotic epithelial,skin or scalp-amniotic adhesions as amniotic mesoderm surface bonded to the epidermis under connective tissues,abdominal wall defect edge for amniotic membrane gradually to cutaneous squamous epithelial migration(amniotic-ectoderm transition zone).Another example is a simple meningoencephalocele malformations,all cases of B ultrasound imaging in amniotic fluid can be seen floating band echo,the part attached to the fetus;amniotic band adhesions of fetal limb portion of visible deformity deformity,often to scan the amniotic band echo the adhesion.Conclusion: ABS mainly for outer surface of tissue and organ defects;involvement of tissue and organ because of its occurrence in the critical period,because of the amniotic sac and amniotic membrane rupture with adhesions interference and teratogenicity;the severity of the deformity and range depends on the amnion rupture time and site.ABS prevention,pregnancy trimester routine ultrasound screening,early diagnosis,early intervention.
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