Larroche J C, Droullé P, Delezoide A L, Narcy F, Nessmann C
INSERM U29, Hôpital de Port-Royal, Paris, France.
Biol Neonate. 1990;57(5):261-78. doi: 10.1159/000243201.
A series of 15 monochorionic twins with a great variety of cerebral lesions is reported. Seven cases illustrate the classical situation: the recipient twin was affected and his co-twin, the donor was macerated. In 5 cases, the lesions were described in the donor twin as well and once, as early as 22 weeks. The lesions were usually hypoxic-ischemic, in 2 they were hemorrhagic. In 1 case there was a malformation. Fetal US were performed in 11 cases and the diagnosis of either IUGR, death of a fetus and/or brain lesions in the survivor could be made in 10 cases and once as early as 21 weeks. In fetuses born alive, transfontanellar US or CT scan have confirmed the diagnosis made on fetal US. The pathogenesis of the lesions is not fully understood. Lesions in the recipient twin may result from emboli or thromboplastic material originating from the macerated co-twin. We suggest that blood pressure instability or episodes of severe hypotension might as well lead to brain and/or visceral lesions in the recipient twin. In the donor, the lesions result from hypotension and/or anemia. With improvement and generalization of imaging techniques, the vitality of the fetuses as well as biometric parameters and anatomical structures will be better controlled. However, in case of a fetal death, occurrence of lesions in the survivor is unpredictable and no uniform policy has been proposed yet. Studies with Doppler and continuous monitoring of funicular circulation should improve our knowledge on feto-fetal transfusion and permit to detect hemodynamic fluctuation or impairment.
报告了15例具有多种脑损伤的单绒毛膜双胎病例。7例说明了典型情况:受血儿双胎患病,其供血儿双胎已浸软。在5例中,供血儿双胎也有损伤描述,其中1例早在孕22周时就有损伤。损伤通常为缺氧缺血性,2例为出血性。1例存在畸形。11例进行了胎儿超声检查,10例可诊断为宫内生长受限、一胎死亡和/或存活儿脑损伤,其中1例早在孕21周时就做出了诊断。对于存活出生的胎儿,经囟门超声或CT扫描证实了胎儿超声做出的诊断。损伤的发病机制尚未完全明了。受血儿双胎的损伤可能源于浸软的供血儿双胎产生的栓子或血栓形成物质。我们认为血压不稳定或严重低血压发作也可能导致受血儿双胎出现脑和/或内脏损伤。对于供血儿,损伤是由低血压和/或贫血导致的。随着成像技术的改进和普及,胎儿的活力以及生物测量参数和解剖结构将得到更好的控制。然而,在一胎死亡的情况下,存活儿出现损伤是不可预测的,目前尚未提出统一的处理策略。采用多普勒和持续监测脐血流循环的研究应能增进我们对胎儿-胎儿输血的认识,并有助于检测血流动力学波动或损害。