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[Acute and chronic fetofetal transfusion syndrome].

BACKGROUND: In the fetofetal transfusion syndrome we can discriminate between a chronic form with severe difference in weight and an acute form with a difference in haemoglobin between the twins. The aim of the retrospective study was to investigate the influence of both forms regarding infant morbidity and mortality. PATIENTS: Of the 135 twins treated from 1.1.1984 to 31.12.1993 in the Department of Pediatrics at the Christian-Albrechts-University Kiel, 27 (20%) showed evidence of fetofetal transfusion. RESULTS: Twelve (8.8%) fulfilled the criteria for an acute and 10 (7.4%) for a chronic transfusion. In 5 (3.9%) twins both forms were evident. Clear differences between twins with acute or chronic fetofetal transfusion syndrome could be seen in gestational age and birth weight. The twins with an acute fetofetal transfusion were significantly older (median: 31.5 vs. 29.0 weeks of gestation), and heavier (median: 1900 vs. 1020 g). Furthermore this group faired significantly better statistically when compared to the chronic form as regards the incidence of mortality (7 vs. 0), the Respiratory Distress Syndrome grades III and IV (7 vs. 2), persistent ductus arteriosus (13 vs 9), intraventricular haemorrhage (14 vs 6) and posthaemorrhagic hydrocephalus (4 vs 0). Similar significant differences could be seen as regards mortality, the Respiratory Distress Syndrome, and intraventricular haemorrhage when acute fetofetal transfusion was compared with the combined acute on chronic form. No significant difference regarding neonatal morbidity and mortality, however, could be shown between infants having undergone chronic fetofetal transfusion and infants with the combined acute on chronic form. CONCLUSIONS: It can be concluded that twins with chronic fetofetal transfusion show a higher rate of neonatal morbidity and mortality, whereas, the acute form does not significantly influence these factors. As both forms not only differ in onset of the disease, pathogenesis, and the actual course of the disease. It is important to always differentiate between the acute and chronic forms.

Birth Weight↗

Successful outcome after serial amnioreductions in triplet fetofetal transfusion syndrome.

BACKGROUND: Triplet fetofetal transfusion is an extremely rare complication with high perinatal mortality. Its rarity does not allow any prospective randomized study on various interventional methods to be conducted. CASE: We report one case of triplet fetofetal transfusion syndrome with survival of all three fetuses. Two were donor fetuses, and one was the recipient fetus. Serial amnioreductions were performed at 22, 24, and 26 weeks' gestation to relieve symptomatic polyhydramnios. Premature rupture of membranes occurred at 27 weeks and cesarean delivery was performed. All three babies were discharged home by 4 months of age, and all had normal neurological development when assessed at 6 months of age. CONCLUSION: The option of serial amnioreduction, with the anticipation and preparation for delivery at around 28 weeks, should be seriously considered when triplet fetofetal transfusion syndrome is encountered.

Adult↗

Monochorionic triplet pregnancy complicated by severe fetofetal transfusion.

We report two cases of fetofetal transfusion in monochorionic triamniotic triplet pregnancies. Case 1: At 23 weeks' gestation an amnioreduction was carried out. Three days later, the donor triplet died in utero. Immediately after that, a cardiotocogram of the triplet who had been thought to be unaffected by the fetofetal transfusion, showed a non-reassuring fetal status. Although cesarean section was carried out, none of the triplets survived. Case 2: At 24 weeks' gestation a woman was transferred to our center because of fetofetal transfusion in monochorionic triplets. Cesarean section was carried out. However, the recipient died on the 75th day after birth, and the others had neurological problems. Previous reports on fetofetal transfusion in triplets are very limited. The prognosis of this condition has been reported to be severe, irrespective of chorionicity, gestational age at delivery, maternal age, and parity. When managing a monochorionic or dichorionic triplet pregnancy, serial and careful ultrasound examination is mandatory to find early symptoms of this serious condition.

Adult↗

Neonatal cranial ultrasonographic findings in preterm twins complicated by severe fetofetal transfusion syndrome.

OBJECTIVE: To investigate cranial ultrasonographic findings in survivors of monochorionic pregnancies complicated by fetofetal transfusion syndrome. STUDY DESIGN: Case details of all monochorionic twin pregnancies complicated by fetofetal transfusion syndrome were obtained from the Centre for Fetal Care database for a 3-year period. Fetofetal transfusion syndrome was diagnosed according to ultrasonographic criteria. Eligible for entry were twin pregnancies resulting in live-born preterm infants and complicated by fetofetal transfusion syndrome severe enough to require amnioreduction. Cranial ultrasonographic scans performed within 48 hours of birth were reviewed for evidence of abnormality. RESULTS: Seventeen pregnancies were eligible for inclusion in the study. Median gestational age was 25 weeks (between 17 and 29 weeks) at diagnosis and 30 weeks (between 25 and 35 weeks) at delivery. Three infants died before ultrasonography could be performed. The remaining 31 twin infants received an early cranial ultrasonographic scan. One of the 31 had a major cerebral infarct; 10 others had evidence of other, more minor, antenatally acquired lesions. CONCLUSIONS: Both donor and recipient survivors from pregnancies complicated by fetofetal transfusion syndrome are at significant risk for antenatally acquired cerebral lesions. Long-term neurologic follow-up studies are indicated to determine the clinical significance of these lesions.

Brain↗

Timely diagnosis by cardiotocography of critical fetal reserve due to fetofetal transfusion syndrome.

In a review of 90 twin pregnancies monitored by antenatal cardiotocography there were 8 cases of subsequently proven fetofetal transfusion syndrome. In 2 of these evidence of severe fetal stress had indicated immediate delivery; the 4 babies survived in circumstances that were perilous. In 1 case there was no evidence of critical fetal reserve but the recipient twin died, this probably representing an acute fetofetal transfusion during labour. In the 5 milder cases with no evidence of stress on cardiotocography, all babies survived and they required minimal or no treatment for problems relating to the fetofetal transfusion. In multiple pregnancy the cardiotocograph offers the additional bonus of detection of fetal compromise due to fetofetal transfusion.

Adult↗

Fetofetal transfusion syndrome in triplet pregnancies: outcome after endoscopic laser surgery.

OBJECTIVE: The purpose of this study was to determine the outcome of fetofetal transfusion syndrome in triplet pregnancies after treatment with endoscopic laser ablation of communicating placental vessels. STUDY DESIGN: Cases of severe fetofetal transfusion syndrome that occur in triplet pregnancies and that are treated with endoscopic laser ablation of placental anastomosis were identified from a prospectively collected fetal medicine database. Chorionicity was determined by sonography and classified as dichorionic or monochorionic. Perinatal outcome was obtained in all cases, and long-term follow-up was obtained in all pregnancies that resulted in at least 1 survivor. RESULTS: During a 6-year period, 10 cases of severe fetofetal transfusion syndrome that were treated with endoscopic laser ablation were identified, of which 7 cases were dichorionic and 3 cases were monochorionic. At least 1 fetus survived in all 7 dichorionic pregnancies and in 2 of the 3 monochorionic pregnancies. In the dichorionic pregnancies, 14 of 21 fetuses (66.7%) survived, but in the monochorionic pregnancies only 2 of 9 fetuses (22.2%) survived. CONCLUSION: Endoscopic laser ablation is feasible in triplet pregnancies that are complicated by severe fetofetal transfusion syndrome. The treatment appears to be associated with improved perinatal outcome in dichorionic, but not in monochorionic, triplets, probably because of the technical inability in achieving ablation of all the communicating vessels in monochorionic triplets.

Databases, Factual↗

Fetofetal transfusion in triplets.

A case of fetofetal transfusion syndrome (FFTS) in a monochorionic triplet pregnancy, in which all three fetuses shared a common circulation, is reported. All babies were born alive, although two died within two days of delivery. This case highlights the problem of FFTS with accompanying high perinatal morbidity and mortality in naturally occurring monochorionic triplet gestations.

Adult↗

Incidence and impact of perioperative complications in 175 fetoscopy-guided laser coagulations of chorionic plate anastomoses in fetofetal transfusion syndrome before 26 weeks of gestation.

OBJECTIVE: The purpose of this study was to evaluate the perioperative complications in fetoscopy-guided laser therapy in fetofetal transfusion syndrome. STUDY DESIGN: Details of fetofetal transfusion syndrome cases who were delivered between 1999 and 2004 in a single center were reviewed retrospectively. RESULTS: One hundred seventy-five procedures were performed percutaneously with the use of local anesthesia. Survival at 6 months of at least 1, 1, and 2 babies was 73%, 38%, and 35%, respectively. Placental abruption and miscarriage was diagnosed in 3 and 12 cases, respectively. Premature rupture of membranes occurred in 49 cases (28%) and including 12, 29, and 46 cases that occurred before 24, 28, and 34 weeks of gestation, respectively. The entry of the trocar was transplacental in 48 cases (27%), but it was not associated with miscarriage (P = .26), premature rupture of membranes (P = .58), or placental abruption (P = .37). CONCLUSION: Fetoscopic laser treatment of fetofetal transfusion syndrome can be performed percutaneously with local anesthesia without significant maternal morbidity. Transplacental entry was not associated with premature rupture of membranes or miscarriage.

Chorion↗

Fetofetal transfusion syndrome: do the neonatal criteria apply in utero?

Thirteen fetuses (five twin, one triplet) were compromised by fetofetal transfusion syndrome in six pregnancies, five in the mid trimester, and one in the third trimester. This diagnosis, which was suspected because of ultrasound findings of discordant growth, discordant amniotic fluid volumes, concordant external genitalia, and monochorial placentation, was confirmed postnatally in each. Nine fetuses underwent blood sampling to aid diagnosis and assessment of fetal wellbeing. In contrast to fetofetal transfusion syndrome investigated postnatally, a difference in haemoglobin concentration of 50 g/l or more in utero was found in only one pregnancy, which was near term, although all had fetal erythroblastaemia and a difference in weight of 20% or more. In vivo confirmation of shared circulation was achieved in two pregnancies by transfusing adult Rh negative red cells into the smaller fetus and then detecting them by Kleihauer testing in blood aspirated from the larger. Invasive procedures also yielded information on fetal blood gas measurements (acidaemia in four and hypoxaemia in six) and amniotic pressure (raised in two). We suggest that comparison of haemoglobin concentrations is inaccurate in fetofetal transfusion syndrome in utero, the diagnosis of which may necessitate detection of a shared circulation using a marker such as adult red cells.

Adult↗

Hydrostatic and osmotic pressure gradients produce manifestations of fetofetal transfusion syndrome in a computerized model of monochorial twin pregnancy.

OBJECTIVE: In spite of recent advances in the assessment and treatment of fetofetal transfusion syndrome, its underlying mechanism remains controversial. We aimed to determine whether the clinical features of fetofetal transfusion syndrome could be explained by unidirectional or bidirectional intertwin transfusion along placental vascular anastomoses. STUDY DESIGN: We constructed a dynamic computerized model of monochorial twin fetoplacental units on the basis of numerous interrelated hemodynamic, osmotic, and metabolic physiologic variables. The circulations were then linked by various combinations of direction and number of arteriovenous anastomoses. RESULTS: With unidirectional anastomoses disease severity, characterized by disparity in blood solids, depended on donor arterial pressure but not on the number of anastomoses. In the chronic state water movement resulting from raised osmotic pressure in the recipient and reduction in the donor produced hydroosmotic pressure equilibrium, reducing anastomotic flow to near zero. Atrial natriuretic peptide-driven urine production was markedly increased in the recipient because of the raised vascular hydrostatic pressure component. With bidirectional anastomoses recirculation between twins reduced discordancy in colloids and hematocrit, and the clinical picture was determined by the degree of asymmetry in the number of connections. CONCLUSIONS: Severe manifestations of fetofetal transfusion syndrome can be explained by unidirectional intertwin transfusion and lesser degrees by asymmetric bidirectional transfusion.

Arteriovenous Anastomosis↗

Placental angioarchitecture in monochorionic twin pregnancies: relationship to fetal growth, fetofetal transfusion syndrome, and pregnancy outcome.

OBJECTIVE: We sought to correlate placental vasculature with fetal growth and outcome in monochorionic twins. STUDY DESIGN: Eighty-two patients with consecutive monochorionic pregnancies underwent biweekly ultrasonography for determination of fetal growth and well-being. After delivery, blinded placental injection studies delineated vascular anastomoses and territory share. Degree of balance in arteriovenous anastomoses equaled the number of arteriovenous anastomoses in one direction minus the number in the other. RESULTS: Pregnancies affected by fetofetal transfusion syndrome (n = 21) had numbers of arteriovenous and venovenous anastomoses that were similar to those in pregnancies without fetofetal transfusion syndrome but fewer arterioarterial anastomoses (P <.0001). Fetofetal transfusion syndrome occurred in 78% of pregnancies with >/=1 arteriovenous and no arterioarterial anastomoses. Birth weight discordancy correlated with placental territory discordancy (P <.0001) and the degree of balance in arteriovenous anastomoses (P =.004). The larger placental share twin had a greater growth velocity than its smaller placental share co-twin (P =.008) for all but one anastomotic pattern. Where arteriovenous anastomoses were aligned with the net venous outflow to the fetus with the smaller territory, co-twins had similar birth weights and growth velocities irrespective of placental share. Fetal survival was higher in pregnancies with an arterioarterial anastomosis (P =.01) but lower with a venovenous anastomosis (P =. 01). Survival of both fetuses was inversely associated with birth weight discordancy (P <.0001). CONCLUSION: Although interrelationships among the various types of anastomoses are complex, our data suggest that the placental territory share and the pattern of arteriovenous anastomoses influence fetal growth, that arterioarterial anastomoses protect against fetofetal transfusion syndrome, and that venovenous anastomoses reduce perinatal survival.

Arteriovenous Anastomosis↗

[Acute fetofetal transfusion in dichorionic twins: a clinical case report].

Fetofetal transfusion is due to the twin to twin blood shunt caused by vascular anastomoses. It has been suggested that two types of FFT exist: a chronic form existing during pregnancy, and an acute form occurring only during parturition. Cross-circulation may be demonstrated in monochorionic as well as dichorionic placentas. However, such communications were found in 85-100% of monochorionic placentas, compared with one of 68 dichorionic fused placentas. Authors describe one case of acute feto-fetal transfusion in dichorionic twins.

Acute Disease↗

Does amniotic fluid volume affect fetofetal transfusion in monochorionic twin pregnancies? Modelling two possible mechanisms.

Clinical evidence suggests that increased amniotic fluid volume due to polyhydramnios increases placental vascular resistance. We have sought to model the possible effects of an increased amniotic fluid volume on the net fetofetal transfusion in monochorionic twin pregnancies. We wanted to compare these effects with the results of previous simulations, which aimed to explain why the twin-twin transfusion syndrome (TTTS) placentas more often include bidirectional arteriovenous (AV) rather than AV plus arterioarterial (AA) anastomoses. We extended our mathematical model of TTTS by simulating two different mechanisms that increase the placental vascular resistance as a consequence of polyhydramnios. First, there is an increase in the placental capillary resistance and hence in deep AV and opposite AV (denoted as VA) resistances due to polyhydramnios. Second, there is an increase in the resistance of chorionic veins due to polyhydramnios, assuming that these veins act as Starling resistors. We then simulated the effects of polyhydramnios on different placental anastomotic patterns. The results were as follows. In the first mechanism (polyhydramnios affects AV-VA resistances), an increased amniotic fluid volume hardly affected bidirectional AV, but slightly decreased fetofetal transfusion in AV plus AA anastomoses. However, for these effects to change the natural development of the pregnancy, polyhydramnios needed to persist for approximately 4 weeks, and by comparing the effects of polyhydramnios with the effects of amnioreduction, amnioreduction was more beneficial for normalizing the donor amniotic fluid volume. Therefore, these beneficial effects due to polyhydramnios have no practical clinical significance. In the second mechanism (Starling resistor for chorionic veins), polyhydramnios slightly increased fetofetal transfusion and hence slightly increased TTTS severity in bidirectional AV and AV plus VV, but did not affect AV plus AA anastomoses. In conclusion, we hypothesize that the simulated effects of polyhydramnios are not the primary cause of the fact that TTTS placentas more often include bidirectional AV than AV plus AA anastomoses. Rather, the more likely explanation is the previously identified larger range of AA than VA anastomotic diameters that adequately compensate for the effects of the AV.

Amniotic Fluid↗

[Fetofetal transfusion syndrome. A case report].

Report about a twin pregnancy with an urgent necessary caesarean section because of a silent curve in the antenatal cardiotocography. The diagnosis of the fetofetal transfusion syndrome was made postnatally. The anastomosis between the two vessel areas of the placenta with respect to the donator--and acceptor--placenta has been visualized by x-ray. Discussion of all clinical problems and of the prognosis of the donator twin.

Adult↗

Multiple thromboembolic events in fetofetal transfusion syndrome in triplets contributing to the understanding of pathogenesis of hydranencephaly in combination with polymicrogyria.

Over the last 180 years, several theories concerning the origin of hydranencephaly have been proposed with an emphasis on infectious, aplastic, and vascular etiologies. In this report, we present a case of triplets with fetofetal transfusion syndrome of which 2 fetuses (1 and 2) developed almost similar hydranencephaly, whereas the third exhibited the features of a fetus papyraceus (3). In the monochorial triamniotic placenta, multiple arteriovenous anastomoses were detected, representing a probable route for the transmission of thrombi originating from fetus 3 causing visceral lesions in fetus 2. Hydranencephaly was histologically characterized by necrosis, macrophage invasion, and endothelial proliferation. In addition, polymicrogyria was seen in fetuses 1 and 2. The combination of multiple visceral thromboembolic events and the death of fetus 3 approximately in the 11th week of gestation suggested a vascular thrombotic pathogenesis of hydranencephaly. Polymicrogyria can be considered as postmigratory laminar necrosis. Our findings contribute to the pathogenetic understanding of combined hydranencephaly and polymicrogyria.

Abnormalities, Multiple↗

[Fetofetal transfusion syndrome in twin pregnancy with prune belly syndrome].

The authors report on a twin pregnancy with one phenotypically normal twin and one with Prune-Belly-syndrome. Since a severe urethral obstruction-malformation complex was apparent, a premature vaginal delivery was aimed for. A pathologic CTG of the healthy twin, with silent and sinusoidal graph pattern, rendered a primary cesarean section necessary in the 36th + 0 week of pregnancy. The cause was established as a fetofetal transfusion syndrome with severe anemia of the phenotypically normal newborn. Following appropriate blood transfusion the subsequent development of this child was free of complications. The child with Prune-Belly-syndrome died on the second day of life.

Adult↗