Berghella V, Kaufmann M
Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, Jefferson Medical College of Thomas Jefferson University, Philadelphia, Pennsylvania, USA.
J Reprod Med. 2001 May;46(5):480-4.
To determine the natural history of pregnancies with twin-twin transfusion syndrome (TTTS).
All cases of TTTS at our institution since 1991 and in a MED-LINE search since 1966 were retrospectively reviewed. The prenatal diagnosis of TTTS required the presence of monochorionic/diamniotic placentation (absence of twin-peak sign, thin membrane, single placenta, same sex) and of polyhydramnios (largest pocket > 8 cm) in one sac and oligohydramnios (largest pocket < 2 cm or stuck twin) in the other. Only cases diagnosed at < or = 28 weeks were included. Upon diagnosis, all patients were counseled as to the availability, risks and benefits of serial amniocentesis, laser therapy, septostomy, umbilical cord ligation, and other medical and surgical interventions.
Of 29 pregnancies identified at our institution with the above strict criteria for TTTS, 5 (17%) declined in utero therapy and were managed expectantly. The mean gestational age at presentation was 19.2 weeks (range, 16-28) and at delivery, 33.8 weeks (range, 22-41). Four (40%) of the twins survived past the neonatal period, and of the three with follow-up, all are free of neurologic sequelae despite the death in utero of their cotwin. From the literature, 136 fetuses with TTTS as defined above and managed expectantly were identified; 37 (27%) survived; 75% (9/12) of survivors with follow-up are neurologically normal. The most recent four studies have reported survival of untreated cases of 50% (17/34) when diagnosed at < or = 26 weeks and of 63% (20/32) when diagnosed at < or = 28 weeks.
The perinatal survival of TTTS pregnancies managed without in utero procedures is approximately 30% overall and 63% in the four most recent series when diagnosed at < or = 28 weeks. The success of in utero therapeutic intervention should be assessed by randomized studies or at least compared to that in similar cases managed without such intervention.
确定双胎输血综合征（TTTS）妊娠的自然病程。
对自1991年以来我院收治的所有TTTS病例以及自1966年以来MED-LINE检索到的病例进行回顾性分析。TTTS的产前诊断需要具备单绒毛膜/双羊膜胎盘（无双峰征、胎膜薄、单胎盘、同性），且一个羊膜腔内羊水过多（最大羊水池>8cm），另一个羊膜腔内羊水过少（最大羊水池<2cm或联体双胎）。仅纳入诊断孕周≤28周的病例。确诊后，向所有患者咨询了系列羊膜腔穿刺术、激光治疗、隔膜造口术、脐带结扎及其他医疗和外科干预措施的可行性、风险和益处。
按照上述严格的TTTS标准，我院确诊的29例妊娠中，5例（17%）拒绝宫内治疗，采取期待治疗。就诊时的平均孕周为19.2周（范围16 - 28周），分娩时的平均孕周为33.8周（范围22 - 41周）。4例（40%）双胎存活至新生儿期以后，在有随访的3例中，尽管其同卵双胎死于宫内，但所有存活者均无神经系统后遗症。从文献中，共识别出136例符合上述定义且采取期待治疗的TTTS胎儿；37例（27%）存活；在有随访的存活者中，75%（9/12）神经系统正常。最近的四项研究报告显示，诊断孕周≤26周时，未经治疗病例的存活率为50%（17/34）；诊断孕周≤28周时，存活率为63%（20/32）。
未经宫内治疗的TTTS妊娠围产期总体存活率约为30%，在最近的四个系列研究中，诊断孕周≤28周时的存活率为63%。宫内治疗干预的成功率应通过随机研究进行评估，或至少与未进行此类干预的类似病例进行比较。
