双胎输血综合征宫内诊断和治疗

双胎输血综合征(twin-to-twin transfusion syndrome,TTTS)是单绒毛膜双胎最严重的并发症之一,胎儿和新生儿死亡率很高。对单绒毛膜双胎患者应进行密切超声检查以及时发现TTTS并评价胎儿发育情况。对TTTS的治疗主要包括期待治疗、羊水减量、羊膜分隔造口术、选择性减胎术和胎儿镜下激光消融术。应根据患者具体情况选择个体化治疗。     

Intrauterine transfusion treatment of nonimmune hydrops fetalis secondary to human parvovirus B19 infection

Congenital human parvovirus B19 infection presented in this case as nonimmune hydrops fetalis that resulted from aplastic anemia at 20 weeks' gestation. Intravascular transfusion therapy led to resolution of the hydrops and a term delivery of an appropriately grown neonate.     

Intrauterine fetal demise following laser treatment in twin-to-twin transfusion syndrome

OBJECTIVE: To evaluate the incidence, risk factors and consequences of intrauterine fetal demise (IUFD) of at least one twin in twin-to-twin transfusion syndrome (TTTS) treated by laser. DESIGN: Retrospective analysis. SETTING: Experience of a single centre between 1999 and 2004. POPULATION: A subgroup of 45 cases with fetal demise of one or both twins from a series of 120 cases of TTTS treated by laser. METHODS: All cases were entered prospectively into a dedicated database and the results were analysed retrospectively. MAIN OUTCOME MEASURES: Fetal demise prognostic factors, survival, fetal anaemia, brain lesions, neonatal death and intact survival. RESULTS: IUFD of one twin occurred in 40 of 120 cases (19 donors and 21 recipients). IUFD of both twins occurred in another five cases. From these 40 cases, miscarriage occurred in two and pregnancy termination was requested in another two cases because of antenatal brain lesions. Two neonates died and two presented severe morbidity, survivors were therefore neurologically normal at 6-44 months of life in 89% (32/36) of the cases. Univariate analysis showed that preoperative abnormal umbilical artery Doppler in the donor before laser treatment and in the recipient following laser treatment was associated with their demise. Incomplete coagulation was suspected in cases where anaemia or cerebral lesions developed following the death of the first twin (10). CONCLUSIONS: IUFD of one or both twins occurred in 45 of 120 (38%) cases of severe TTTS treated by laser. In these, separation of the placental circulations was incomplete in at least 22% (10/45) of the cases. Umbilical artery Doppler abnormalities before laser were found to be risk factors for the donors' demise following the procedure.     

Rapid development of hydrops fetalis in the donor twin following death of the recipient twin in twin-twin transfusion syndrome.

Intrauterine death of one fetus in monochorionic twinning is associated with high rates of perinatal morbidity and mortality in the surviving fetus. Subsequent development of hydrops fetalis in the donor twin after fetal demise of the recipient twin has been described in only two case reports and pathophysiology remains unclear. We report on a monochorionic-diamniotic twin pregnancy complicated by severe twin-twin transfusion syndrome. Ultrasound examination at 20 weeks of gestation showed discrepant twins with oligohydramnios in the smaller twins' sac and polyhydramnios in that of the larger twin. Repeated amniocenteses permitted prolongation of the pregnancy. However, the recipient twin developed deteriorating hydrops fetalis and died at 28 weeks of gestation. After this event, subsequent development of hydrops fetalis in the surviving donor twin could be observed, as well as an increase of amniotic fluid. An elective cesarean section was performed at 29 weeks of gestation. Initial hypoxemia could be effectively treated by high frequency oscillatory ventilation, surfactant therapy and inotropic support. The infant was discharged in good condition at the age of 2 months. Although rare, antenatal demise of the recipient twin in a monochorionic pregnancy can be associated with the subsequent development of hydrops fetalis in the surviving donor twin. We speculate that this phenomenon is due to ischemia-reperfusion injury of the previously poorly perfused twin.     

Myocardial hypertrophy of the recipient twins in twin-to-twin transfusion syndrome and cerebral palsy.

OBJECTIVES: This study was performed to determine risk factors for cerebral palsy (CP) in monochorionic twins, especially with twin-to-twin transfusion syndrome (TTTS). METHODS: In 33 pathologically confirmed monochorionic pregnancies, we analyzed the incidence of cardiovascular and neurological complications. RESULTS: Seventeen cases were diagnosed as TTTS. Myocardial hypertrophy was detected in seven recipient twins of TTTS, but not found in the donor twins of TTTS or in non-TTTS. CP developed in six out of 29 in TTTS and one out of 32 in non-TTTS. In TTTS, all the cases with CP were seen in the recipient twins. The development of CP was significantly associated with cardiovascular complications such as myocardial hypertrophy and hydropic changes. CONCLUSIONS: Myocardial hypertrophy found in the recipient twins in TTTS seems to be a risk for developing CP later on, which may have implications in understanding the pathogenesis of CP.     

Chronic twin to twin transfusion syndrome with non-immunologic hydrops fetalis "donor"]

We describe a case of twin to twin transfusion syndrome, with few distinct features that are not commonly reported. Not immunological hydrops fetalis (NIHF) was met in donor and there was no hydramnion around the recipient. In prenatal diagnosis we additionally used the ultrasound testing of the dividing membranes and the Doppler velocimetry in umbilical arteries.     

Intrauterine therapy of fetal tachyarrhythmias: intraperitoneal administration of antiarrhythmic drugs to the fetus in fetal tachyarrhythmias with severe hydrops fetalis

In cases of fetal tachyarrhythmia with congestive heart failure accompanied by signs of non-immune hydrops fetalis, the transplacental treatment of the fetus with antiarrhythmic agents by administration of drugs to the mother is only rarely successful. In the two cases reported, the cardioversion of a supraventricular tachycardia to a sinus rhythm or a constant 2:1 AV conduction block to a 1:1 AV conduction with atrial flutter could only be achieved after additional antiarrhythmic treatment directly administered to the fetus using ultrasound guidance. Drugs used include: beta-methyldigoxin, verapamil, propafenon, and they were administered according to the dosing amounts for intravascular injections. This was carried out 12 times in case 1 by the intraperitoneal route into the fetal ascites and twice in case 2. This led in both cases to varying durations of a sustained sinus rhythm after 5-15 minutes. This technically relatively simple procedure affords the option of rapidly achieving high concentrations, even when antiarrhythmic agents are administered which do not adequately cross the placenta. This direct treatment is indicated in cases of tachyarrhythmia with advanced signs of non-immune hydrops fetalis as a supplement to the high-dose transplacental therapy using antiarrhythmic agents.     

Intrauterine fetal transfusion in the treatment of erythroblastosis fetalis

Assay of serum oxytocinase levels by the chemical method, using L-cystine-di-β-naphthylamide as substrate and the dynamic oxytocin test with Syntocinon were made in 136 hospitalized pregnant women before delivery. A statistically significant relationship was found between prenatal levels of serum oxytocinase and the following clinical data: body surface of the fetus, state of neonate, placental weight, perinatal blood loss, and duration of labor. No differences were found in the level of the enzyme when the reactivity of the pregnant uterus to oxytocin was in the range of 10 to 60 mU. of Syntocinon injected intravenously. However, the serum oxytocinase level in women requiring administration of oxytocin for induction of labor was significantly lower than in other parturient women, regardless of whether delivery was spontaneous or operative. The observed facts were explained in the light of a biochemical theory concerning pregnancy and labor.     

Pulmonary stenosis and reactive right ventricular hypertrophy in the recipient fetus as a consequence of twin-to-twin transfusion.

The present study describes an association between adverse outcome in the twin-to-twin transfusion syndrome (TTTS) and pulmonary stenosis or reactive right ventricular hypertrophy. Six discordant monozygotic twin pregnancies with TTTS are described. Ventricular hypertrophy and atrioventricular valvular regurgitation occurred in all the recipient twins with pulmonary valvular stenosis in three cases and infundibular stenosis in one case. The recipient twin in one pair and both twins in another pregnancy died as a consequence of immaturity but the remaining twins all survived. Surgical intervention was required in one baby for valvular pulmonary stenosis. Our observations suggest that elevated blood pressure in the transfusion recipient may play an important role in pathogenesis. We hypothesise that both pulmonary stenosis and right chamber hypertrophy are secondary to hemodynamic changes. Although we have found valvular pulmonary stenosis in three recipients and infundibular stenosis in only one, this (obstruction to outflow) could be due to right chamber hypertrophy.     

Intrauterine hypercalcaemia and non-immune hydrops fetalis--relationship to the Williams syndrome

We describe a 28-week-old fetus with severe non-immune hydrops. Intrauterine cord blood sampling revealed hypercalcaemia of 3.4 mmol/l (n = 2.6 +/- 0.1). Subsequently, a postmortem examination revealed supravalvular aortic and pulmonary artery stenosis together with extensive arterial calcification. The maternal calcium, 25-hydroxyvitamin D3, 1,25-dihydroxyvitamin D3, and parathyroid hormone levels were normal at delivery. This is the first time that hypercalcaemia has been diagnosed in utero. We speculate on the fact that the disorder resulted as a consequence of abnormal vitamin D metabolism in the fetoplacental unit, and that it might be related to the Williams syndrome.     

Uterine contractions might increase heart preload in the recipient fetus in early-onset twin-twin transfusion syndrome: an ultrasound assessment

OBJECTIVES: Uterine contractions (UC) in twin pregnancy are often experienced, yet the effects of UC in twin-twin transfusion syndrome (TTTS) remain to be clarified. The recipient heart preload condition and the effects of UC were evaluated and the final objective was to clarify the effects of tocolysis. METHODS: Firstly, the preload indexes (PLIs) and cardiothoracic area ratios (CTARs) were analyzed on both fetuses of 10 TTTS cases, aged from 14 to 28 gestational weeks in stage III/IV cases for evaluating the preload condition. Then, the PLIs in the presence and absence of UC in the recipient fetuses were determined to evaluate the difference. RESULTS: The PLIs of the recipient and the donor fetuses were 0.78 +/- 0.34 (n = 163) and 0.35 +/- 0.13 (n = 71) respectively. The CTARs were 30.2 +/- 6.1 (n = 62) and 23.4 +/- 5.4 (n = 62) respectively. The PLIs in the absence and presence of UC in the recipient fetuses were 0.69 +/- 0.29 and 0.99 +/- 0.38 (n = 35). All above comparisons showed highly significant differences (p < 0.0001). CONCLUSION: The recipient fetuses have signs of cardiac dilatation and a high-preload condition. UC transiently further raises high-preload conditions of the recipient fetus. Thus, tocolysis may be necessary for management in cases of early-onset severe TTTS.     

Prenatal diagnosis of twin-twin transfusion syndrome complicated with hydrops fetalis at 14 weeks of gestation.

Twin-twin transfusion syndrome (TTTS) may complicate multiple pregnancy. Monochorionic discordant twins with oligohydramnios and polyhydramnios may be diagnostic. Hydrops fetalis is particularly ominous. All the signs can appear independently at any stage of gestation. However, TTTS with hydrops fetalis in early pregnancy is rare. We report here a case of TTTS complicated with hydrops fetalis diagnosed at 14 weeks of gestation. Our case may be one of the earliest cases of the prenatal diagnosis of TTTS complicated with hydrops fetalis in the literature.     

A reappraisal of the maternal syndrome associated with hydrops fetalis

A pregnancy complicated by severe rhesus isoimmunization can not only lead to fetal loss but may also be complicated by a rarely described maternal syndrome. The unusual clinical features of one such case and the probable underlying pathophysiology are described.     

Management of the twin-to-twin transfusion syndrome (TTTS)

Twin-to-twin transfusion syndrome (TTTS) is a severe complication of multiple pregnancy occurring in 10-12% of monochorionic twins. This complication is mainly due to the presence of unbalanced arterio-venous anastomoses between placental branches of the umbilical circulations. The natural history of TTTS is variable. It is often difficult to predict whether a patient will show stable rather than rapidly progressive disease. A formal staging system has been suggested based on the sonographic time sequence of cases with progressive deterioration and poorer outcome. The mortality of untreated TTTS has been quoted to be more than 80% with advances in neonatal care the mortality rate may have decreased. The three most commonly used therapies for mid-trimester TTTS are amnioreduction, fetoscopic laser coagulation of the vascular anastomoses and septostomy. Outcome for fetuses with TTTS treated with amnioreduction depends on several antenatal variables which may help in counseling patients. Fetoscopic laser coagulation of vascular anastomoses represents an important evolution in the surgical treatment of TTTS. The management of the TTTS according to stage should be corroborated with an appropriate clinical trial.     

The development of hydrops fetalis in the ovine fetus after lymphatic ligation or lymphatic excision

Sixteen ovine fetuses underwent either ligation or excision of the left thoracic, left cervical, and left brachiocephalic lymphatic ducts. Our purpose was to test the hypothesis that interruption of lymphatic flow would lead to hydropic changes in the ovine fetus. Of the 11 animals in the group that underwent ligation, hydrops developed in 1. All five of the fetuses that underwent excision of these major lymphatic ducts were hydropic at the time of autopsy (3 to 7 days), with 62 to 502 ml of free fluid collected from the thoracic and abdominal cavities. The mean edema fluid total protein concentration in the hydropic fetuses was 2.6 gm/dl. This value was 71% to 94% of that found in the plasma, suggesting that the fetus is capable of producing new plasma proteins at a high rate. The observation that lymphatic excision led to hydropic changes in the ovine fetus, whereas ligation did not consistently produce hydrops, suggests that fetal lymph vessels may be capable of very rapid regrowth over short distances. Thus lymphatic excision, but not ligation, produces an animal model for the study of hydrops fetalis.     

双胎输血综合征的判断和临床处理

双胎输血综合征（twin-twin transfusion syndrome，TTTS）是单合子单绒毛膜双胎妊娠的一种严重并发症，一胎儿（供血儿）的血液通过不平衡的胎盘血管吻合网输入另一胎儿（受血儿）而引起的一系列病理生理改变和临床症状，预后较差，围生儿病死率高达80％～100％。