Verheijen R H, van Rijen-de Rooij H J, van Zundert A A, de Jong P A
Department of Obstetrics & Gynecology, Catharina Hospital, Eindhoven, The Netherlands.
Eur J Obstet Gynecol Reprod Biol. 1989 Oct;33(1):89-94. doi: 10.1016/0028-2243(89)90083-x.
A rare case of pregnancy in a patient with the syndrome of Klippel-Trenaunay-Weber is described. The arterio-venous anomalies in this patient originally occurred in her right leg, but in the course of her first pregnancy she also developed circumscript angiomatosis at the left and right side of the uterus. Her pregnancy was uneventful. However, because of prominent vascular changes in the cervix and lower uterine segment, there appeared to be a cephalo-pelvic discongruency. For this reason a Cesarean section was performed at term. Postoperatively, during heparinization, she had signs of abdominal bleeding, which could be controlled conservatively. There were no signs of intravascular coagulation or cardiac decompensation. A review of the scarce literature on diffuse uterine angiomatosis and angiomatosis occurring as a result of the syndrome of Klippel-Trenaunay-Weber is given. The clinical course and the risks of a pregnancy with this condition are discussed. It is concluded that the angiomatosis based on the syndrome of Klippel-Trenaunay-Weber is less hazardous than a diffuse angiomatosis of the uterus without this disease, the main risk being diffuse intravascular coagulation at or after delivery.
本文描述了一例患有克-特-韦综合征（Klippel-Trenaunay-Weber syndrome）患者的罕见妊娠病例。该患者的动静脉畸形最初出现在右腿，但在首次妊娠期间，其子宫左右两侧也出现了局限性血管瘤病。她的妊娠过程顺利。然而，由于宫颈和子宫下段明显的血管变化，似乎存在头盆不称。因此，足月时进行了剖宫产。术后，在肝素化期间，她出现了腹部出血迹象，可通过保守治疗控制。没有血管内凝血或心脏代偿失调的迹象。本文对关于弥漫性子宫血管瘤病以及由克-特-韦综合征导致的血管瘤病的稀少文献进行了综述。讨论了患有这种疾病时的临床过程和妊娠风险。得出的结论是，基于克-特-韦综合征的血管瘤病比无此疾病的弥漫性子宫血管瘤病危害小，主要风险是分娩时或分娩后发生弥漫性血管内凝血。
