feto-fetal transfusion syndrome

胎儿输血综合征
  • 文章类型: Journal Article
    背景:双胎输血综合征(TTTS)激光手术期间双胎之间的术中输血可能因手术技术而异,并已被提议解释供体双胎存活率的差异。
    目的:本试验比较了两种激光技术:序贯技术,其中从容量耗尽的供体到容量超负荷的受体的动静脉通信在从受体到供体之前被激光阻断;和选择性技术,其中血管通信的闭塞不按特定顺序进行。
    方法:单中心,开放标签,我们进行了随机对照试验,其中TTTS患者被随机分为序贯和选择性激光手术.嵌套在审判中,第二项试验将浅表吻合(动脉和静脉)患者随机分为先消融这些连接(在消融动静脉吻合之前)和后消融.主要结果指标是出生时供体双胞胎的存活率。
    结果:总共642例患者被随机分组。两组供体双胞胎的总体存活率相似(85.6%[274/320]对84.2%[271/322],或1.12[0.73-1.73],P=.605)。在27.6%(177/642)的病例中发生了浅吻合术。与仅有动静脉通信的患者相比,浅表吻合组的供体存活率较低(70.6%[125/177]对90.3%[420/465],OR0.33[0.20-0.54],P<.001)。在浅层吻合的情况下,供者存活率与消融时机或手术技术无关.序贯组与选择性组术后平均大脑中动脉(MCA)收缩期峰值速度(PSV)较低(1.00±0.30对1.06±0.30MoM,P=.003)。事后分析显示,有2个因素与供体双胞胎总体生存率较差相关:供体双胞胎术前关键异常多普勒(CAD)参数的存在/不存在以及动脉动脉吻合(AA)的存在/不存在。根据这些因素,导致4类患者:(1)第1类(54%,347/642),无供体双胞胎CAD无AA:顺序组中供体双胞胎存活率为91.2%,选择性组中为93.8%;(2)类别2(22%,143/642),CAD存在+无AA:供体存活率为89.9%,而非75.7%;(3)类别3(11%,73/642),无CAD+AA存在:供体生存率为94.7%,而非74.3%;(4)第4类(12%,79/642),CAD存在+AA存在:供体存活率为47.6%对64.9%。
    结论:序贯激光技术与选择性激光技术的供体双胞胎存活率没有差异,如果首先消融浅层吻合与最后消融,则没有差异。序贯方法与选择性方法相比,供体双胞胎的术后MCAPSV得到了改善。事后分析表明,根据高风险因素,供体双胞胎的存活可能与激光技术的选择有关。需要进一步的研究来了解使用这些类别来指导手术技术的选择是否会改善结果。
    背景:没有外部资金的NCT02122328。
    BACKGROUND: Intraoperative blood transfer between twins during laser surgery for twin-twin transfusion syndrome can vary by surgical technique and has been proposed to explain differences in donor twin survival.
    OBJECTIVE: This trial compared donor twin survival with 2 laser techniques: the sequential technique, in which the arteriovenous communications from the volume-depleted donor to the volume-overloaded recipient are laser-occluded before those from recipient to donor, and the selective technique, in which the occlusion of the vascular communications is performed in no particular order.
    METHODS: A single-center, open-label, randomized controlled trial was conducted in which twin-twin transfusion syndrome patients were randomized to sequential vs selective laser surgery. Nested within the trial, a second trial randomized patients with superficial anastomoses (arterioarterial and venovenous) to ablation of these connections first (before ablating the arteriovenous anastomoses) vs last. The primary outcome measure was donor twin survival at birth.
    RESULTS: A total of 642 patients were randomized. Overall donor twin survival was similar between the 2 groups (274 of 320 [85.6%] vs 271 of 322 [84.2%]; odds ratio, 1.12 [95% confidence interval, 0.73-1.73]; P=.605). Superficial anastomoses occurred in 177 of 642 cases (27.6%). Donor survival was lower in the superficial anastomosis group vs those with only arteriovenous communications (125 of 177 [70.6%] vs 420 of 465 [90.3%]; adjusted odds ratio, 0.33 [95% confidence interval, 0.20-0.54]; P<.001). In cases with superficial anastomoses, donor survival was independent of the timing of ablation or surgical technique. The postoperative mean middle cerebral artery peak systolic velocity was lower in the sequential vs selective group (1.00±0.30 vs 1.06±0.30 multiples of the median; P=.003). Post hoc analyses showed 2 factors that were associated with poor overall donor twin survival: the presence or absence of donor twin preoperative critical abnormal Doppler parameters and the presence or absence of arterioarterial anastomoses. Depending on these factors, 4 categories of patients resulted: (1) Category 1 (347 of 642 [54%]), no donor twin critical abnormal Doppler + no arterioarterial anastomoses: donor twin survival was 91.2% in the sequential and 93.8% in the selective groups; (2) Category 2 (143 of 642 [22%]), critical abnormal Doppler present + no arterioarterial anastomoses: donor survival was 89.9% vs 75.7%; (3) Category 3 (73 of 642 [11%]), no critical abnormal Doppler + arterioarterial anastomoses present: donor survival was 94.7% vs 74.3%; and (4) Category 4 (79 of 642 [12%]), critical abnormal Doppler present + arterioarterial anastomoses present: donor survival was 47.6% vs 64.9%.
    CONCLUSIONS: Donor twin survival did not differ between the sequential vs selective laser techniques and did not differ if superficial anastomoses were ablated first vs last. The donor twin\'s postoperative middle cerebral artery peak systolic velocity was improved with the sequential vs the selective approach. Post hoc analyses suggest that donor twin survival may be associated with the choice of laser technique according to high-risk factors. Further study is needed to determine whether using these categories to guide the choice of surgical technique will improve outcomes.
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  • 文章类型: Case Reports
    一名40岁的primigravida妇女,患有单绒毛膜-三胎羊膜(MT)三胎妊娠,由于在16孕周的先兆流产而住院。两个胎儿的羊水过多和第三个胎儿的羊水过少支持在妊娠23周和3天诊断为胎儿输血综合征(FRTS)。严重的呼吸困难和肝功能障碍需要入住重症监护病房和机械通气支持,临床诊断为羊水过多引起的腹室综合征(ACS)。当她的一般情况没有改善,无论重症监护,患者在妊娠23周和5天时通过剖宫产分娩了三个胎儿。分娩时实现了腹部减压,术后13天出院,无发病。这是在MT三胎妊娠中由FFTS引起的ACS导致极度早产的第一例报告。
    A 40-year-old primigravida woman with a monochorionic-triamniotic (MT) triplet pregnancy was hospitalized due to threatened abortion at 16 gestational weeks. Polyhydramnios in two fetuses and oligohydramnios in the third supported a diagnosis of feto-fetal transfusion syndrome (FFTS) at 23 weeks and 3 days of gestation. Severe dyspnea and liver dysfunction required intensive care unit admission and mechanical ventilation support, and abdominal compartment syndrome (ACS) caused by polyhydramnios was clinically diagnosed. When her general condition was not improved regardless of intensive care, the patient delivered the three fetuses by cesarean section at 23 weeks and 5 days gestation. Abdominal decompression was achieved with delivery, and the patient was discharged 13 days after operation without morbidity. This is the first case report of ACS caused by FFTS in a MT triplet pregnancy resulting in extremely preterm birth.
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  • 文章类型: Case Reports
    Feto-fetal transfusion syndrome (FFTS) severely affects monochorionic (MC) multiple pregnancies and affects 1 in 1600 pregnancies overall. The number of increasing disputed obstetrics cases in China is related to unavailability of prompt diagnosis of FFTS. We present here a woman with a MC triplet pregnancy with intrauterine fetal death at 33 weeks of gestation due to FFTS. Subsequent pathological anatomy showed that the MC placenta contained vascular anastomoses, including arterio-arterial anastomosis and arterio-venous anastomosis. These anastomoses led to unidirectional blood flow with the absence of adequate compensatory counter-transfusion and bi-directional flow. When encountering such challenging conditions, medical practitioners should discreetly compare the fetuses\' characteristics with features of placental blood vessels and consult morphological and pathological findings. Furthermore, they should perform ultrasound examinations, particularly focussing on fetal size differences and the maximum vertical pocket in the diagnosis of FFTS, especially in MC multiple pregnancies with abdominal symptoms.
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  • 文章类型: Case Reports
    动脉导管未闭涉及维持位于肺动脉和降主动脉之间的血管导管的通透性,由于从胎儿型循环过渡到成人型循环的失败。这种畸形是出生体重极低的早产儿的特征。在该病理学中确定的主要病理生理因素是平滑肌的不成熟,血管舒张介质和持续性低氧血症的存在。导管依赖性心脏畸形需要药物治疗以保持动脉导管的通透性,直到矫正手术为止。
    我们介绍了一个极低出生体重的早产儿,来自双胎妊娠,怀疑有特定的病理,分别为胎儿输血综合征,被送往新生儿重症监护病房区域中心。胎龄31-32周的早产儿,极低的出生体重,源于双胎妊娠,有足够的产前随访,因怀疑胎儿输血综合征继发严重宫内生长受限而剖腹产出生。新生儿由于表面活性剂缺乏而发展为呼吸窘迫综合征,脑室出血I/II级,和严重的视网膜病变.在生命的第二周检测到收缩期杂音,经fontanellar超声显示的舒张盗窃以及舒张压值降低,增加了对动脉导管未闭的怀疑,因此用布洛芬进行了特异性治疗,环氧合酶抑制剂,已启动。新生儿病情的逐渐改变和主动脉缩窄的证据导致前列腺素E1治疗和随后的手术矫正。
    虽然有益,持续性动脉导管的预防性或治疗性闭合可能会使患有“沉默”心脏畸形和相关病理的新生儿的进化恶化。
    UNASSIGNED: Patent ductus arteriosus involves maintaining the permeability of the vascular ductus located between the pulmonary artery and the descending aorta, due to the failure of transition from foetal to adult type circulation. This malformation is characteristic to premature newborns with extremely low birth weight. The main pathophysiological factors identified in this pathology are immaturity of the smooth muscles, presence of vasodilator mediators and persistent hypoxaemia. Ductal-dependent cardiac malformations require drug therapy for keeping the permeability of the ductus arteriosus until the time of corrective surgery.
    UNASSIGNED: We present the case of an extremely low birth weight premature newborn, derived from twin pregnancy with suspected specific pathology, respectively feto-fetal transfusion syndrome, admitted to the Regional Centre of Neonatal Intensive Care Unit Tîrgu-Mureş.Premature newborn with gestational age 31-32 weeks, extremely low birth weight, derived from twin pregnancy, with adequate prenatal follow up, was born by caesarean section for severe intrauterine growth restriction secondary to feto-fetal transfusion syndrome suspicion. The newborn developed respiratory distress syndrome by surfactant deficiency, intraventricular-haemorrhage grade I/II, and severe retinopathy. The detection of a systolic murmur in the second week of life, the diastolic theft revealed by trans-fontanellar ultrasound as well as lowered diastolic blood pressure values raised the suspicion of a patent ductus arteriosus and therefore specific treatment with ibuprofen, a cyclooxygenase inhibitor, was initiated. Progressive alteration of the newborn\'s condition and the evidence of a coarctation of the aorta imposed the initiation of Prostaglandin E1 therapy and subsequent surgical correction.
    UNASSIGNED: Although beneficial, prophylactic or therapeutic closure of persistent ductus arteriosus may worsen the evolution of a newborn with a \"silent\" cardiac malformation and associated pathology.
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    文章类型: Case Reports
    Feto-fetal transfusion syndrome is a pathological process unique to diamniotic monochorionic pregnancies. It is the consequence of an unbalanced fetal blood flow through communicating vessels within a shared placenta. When it occurs, a polyuric, hypervolemic recipient twin co-exists with a hypovolemic oliguric donor. The presence of polyhydramnios or oligohydramnios is considered a poor prognostic indicator, whereas normal amniotic fluid volumes indicate a lack of clinically significant twintwin transfusion. In addition, the spontaneous normalization of amniotic fluid volume is usually seen as a favorable prognostic sign. Here, however, we present a case of feto-fetal transfusion in a 31 year-old primigravida at 19 week, in which the spontaneous normalization of amniotic fluid volume in the recipient twin preceded the death of the donor.
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