Poeuf B, Samson P, Magalon G
Centre phocéen de la main, clinique Résidence-du-Parc, rue Gaston-Berger, 13010 Marseille, France.
Chir Main. 2008 Dec;27 Suppl 1:S136-47. doi: 10.1016/j.main.2008.07.016. Epub 2008 Aug 20.
Amniotic band syndrome is a well-described clinical entity, which includes several congenital deformities. Hand malformations and limb defects represent the most frequent clinical characteristics, gathering, with variable localization, constriction rings, acrosyndactylies and amniotic amputations. Other anomalies of skull, face, body wall and internal organs, sometimes complex and lethal, are significantly associated with this syndrome. The syndrome is then included in the larger entity of limb body wall complex (LBWC). Congenital ring constriction, amniotic band disruption complex, or congenital transverse defect are some of the numerous synonyms defining this malformative syndrome, showing either its clinical variability, or the uncertainties surrounding its etiology. Indeed, several pathogenic theories have been successively opposed, bringing about a certain degree of confusion. Recent experimental genetic studies could unify the different fetal malformations. The surgical treatment actually applies only to the aftereffects of the intrauterine phenomenon, until antenatal diagnosis followed by in utero surgery will be perfected.
羊膜带综合征是一种已被充分描述的临床病症,包括多种先天性畸形。手部畸形和肢体缺陷是最常见的临床特征,表现为不同部位的缩窄环、并指畸形和羊膜截断,其位置各异。颅骨、面部、体壁和内脏的其他异常,有时复杂且致命,与该综合征显著相关。该综合征随后被纳入肢体-体壁复合征(LBWC)这一更大的范畴。先天性环状缩窄、羊膜带破坏复合体或先天性横向缺损是定义这种畸形综合征的众多同义词中的一部分,这既显示了其临床变异性,也表明了围绕其病因的不确定性。事实上,几种致病理论相继受到反对,造成了一定程度的混乱。最近的实验性基因研究可能会统一不同的胎儿畸形。目前的手术治疗实际上仅适用于子宫内现象的后遗症,直到产前诊断并随后完善宫内手术。