Sentilhes L, Verspyck E, Patrier S, Eurin D, Lechevallier J, Marpeau L
Clinique Gynécologique et Obstétricale, Pavillon Mère-Enfant, CHU Charles Nicolle, 1, rue de Germont, 76031 Rouen.
J Gynecol Obstet Biol Reprod (Paris). 2003 Dec;32(8 Pt 1):693-704.
Amniotic band syndrome is a collection of fetal congenital malformations, affecting mainly the limbs, but also the craniofacial area and internal organs. Two mains pathogenic mechanisms are proposed: early amnion rupture (exogenous theory) leading to fibrous bands, which entrap the fetal body; the endogenous theory privileges vascular compromise, mesoblastic strings not being a causal agent. The outcome of the disease depends on the gravity of the malformation. Termination of the pregnancy is usually proposed at the time of the diagnostic of severe craniofacial and visceral abnormalities, whereas minor limb defects can be repaired with postnatal surgery. In case of an isolated amniotic band with a constricted limb, in utero lysis of the band can be considered to avoid a natural amputation. However, as the exact etiology of amniotic band syndrome remains unknown and its natural course unpredictable, prenatal surgery continues to be controversial. Doppler studies of the constricted limb could be of useful predictive value of in utero amputation, and therefore could be helpful to determine when in utero treatment should be considered.
羊膜带综合征是一组胎儿先天性畸形，主要影响四肢，但也累及颅面部和内脏器官。目前提出了两种主要的致病机制：早期羊膜破裂（外源性理论）导致纤维带形成，从而束缚胎儿身体；内源性理论则认为血管受损起主要作用，中胚层条索并非致病因素。该病的预后取决于畸形的严重程度。在诊断出严重的颅面部和内脏异常时，通常建议终止妊娠，而轻微的肢体缺陷可在出生后进行手术修复。对于仅有羊膜带且肢体受压的情况，可考虑在子宫内松解羊膜带以避免自然截肢。然而，由于羊膜带综合征的确切病因仍不清楚，其自然病程也无法预测，因此产前手术仍存在争议。对受压肢体进行多普勒研究可能对子宫内截肢具有有用的预测价值，从而有助于确定何时应考虑进行子宫内治疗。
