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子宫内纤维带的意义——羊膜带序列合并肢体-体壁复合体:一例罕见的胎儿尸检病例

Significance of fibrotic bands in utero--Amniotic band sequence with limb body wall complex: A rare case of fetal autopsy.

作者信息

Devi P Lekshmi, Cicy P J, Thambi Renu, Poothiode Usha

机构信息

Department of Pathology, Government Medical College, Kottayam, Kerala, India.

出版信息

Indian J Pathol Microbiol. 2015 Oct-Dec;58(4):528-30. doi: 10.4103/0377-4929.168876.

Abstract

Amniotic band sequence (ABS) includes a wide spectrum of abnormalities resulting from entrapment of various fetal parts from a disrupted amnion, ranging from a mere constriction ring affecting a finger to a fatal form called limb body wall complex (LBWC). Reported cases of ABS with LBWC are very few. The spectrum of anomalies depends on which part gets entrapped and at what point of gestation. Hence, the clinical presentation can be extremely variable. Early detection of such cases using sonology is really challenging due to the small size of the fibrotic bands. Here, we present a case of amniotic band syndrome with LBWC in a fetus at 24 weeks of gestation, which was referred for an autopsy. The fetus also showed scoliosis, gastroschisis, lumbosacral meningocele, congenital talipes equinovarus, and cleft palate, thus having features of placenta cranial and placenta abdominal phenotype which is very rare.

摘要

羊膜带序列(ABS)包括一系列因羊膜破裂导致各种胎儿部位受困而产生的异常情况,范围从仅影响一根手指的收缩环到一种致命形式即肢体 - 体壁复合体(LBWC)。报道的伴有LBWC的ABS病例非常少。异常的范围取决于哪些部位受困以及在妊娠的哪个阶段。因此,临床表现可能极具变异性。由于纤维化带体积小,利用超声早期检测此类病例极具挑战性。在此,我们报告一例妊娠24周胎儿的伴有LBWC的羊膜带综合征病例,该病例被转诊进行尸检。该胎儿还表现出脊柱侧弯、腹裂、腰骶部脑脊膜膨出、先天性马蹄内翻足和腭裂,因此具有非常罕见的头颅胎盘和腹部胎盘表型特征。

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