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Objectives: To evaluate incidence of cerebral injury and outcome in a large series of monochorionic (MC) twin survivors after spontaneous single fetal demise. Methods: Retrospective analysis of all MC pregnancies with single fetal demise diagnosed at, or referred to, the Fetal Therapy Unit “U. Nicolini,” V. Buzzi Children’s Hospital, Milan, Italy, from 2004 to 2015. Survivors evaluation protocol included detailed ultrasound (US) of intracranial anatomy, Doppler investigation of peak systolic velocity in the middle cerebral artery (MCA-PSV), and magnetic resonance (MR). Data were collected on pregnancy characteristics, postnatal brain scan, and MR and neurological follow-up. Results: Seventy-eight consecutive MC pregnancies were analyzed. Median gestational age (GA) at single fetal demise was 22 weeks (range 15–36); median interval between single demise and live birth was 105 days (range 1–175), with a median GA at birth of 36 weeks (range 23–41). Prenatal MR was performed in 57 of 78 cases (73%). Cerebral injury affected 14/78 (18%) co-twins, 2 of whom were born immediately after single demise, with postnatal diagnosis of cerebral injury; of the other 12 fetuses that were studied before birth, 10 had a prenatal diagnosis of lesion both with US and MR, one only with MR, and in one case, a grade III intraventricular hemorrhage was reported only after delivery, which occurred at 25 weeks, 5 weeks after the single demise. Signs of fetal anemia (MCA-PSV value above 1.55 MoM) were related to a higher risk of prenatal cerebral injury; cases with postnatal diagnosis of lesion were delivered at lower GA. Conclusions: Cerebral injury affects 18% of co-twin survivors after single fetal demise in MC twin pregnancies, and evaluation and follow-up of these cases can improve detection rate of such damage.

Background/Purpose: We report a case study of jejunal atresia and the results of a systematic literature review of all reported cases of bowel complications occurring after fetoscopic laser ablation (FLA) for the treatment of twin-to-twin transfusion syndrome (TTTS). Methods: A systematic literature review was performed of bowel complications after FLA for TTTS according to PRISMA guidelines. Results: There are 11 published cases of small bowel atresia, 5 cases of necrotising enterocolitis (NEC), and 2 cases with foetal bowel perforations. Recipient twins were more likely to be affected by small bowel atresia (7 recipient and 4 donor cases) and NEC (3 recipient and 2 donor twins). Prenatal ultrasonographic abnormalities were demonstrated in 7 out of 9 cases with bowel atresia and in both cases of bowel perforation. The overall survival rate for neonates with bowel complications after FLA is 72%, but is much lower for co-twins at 22%. The survival rates for jejunoileal atresia and NEC are 91 and 40%, respectively. Conclusions: It is uncertain as to whether these bowel anomalies are due to bowel ischaemia associated with TTTS, the treatment with FLA, or a combination of both. Cases with prenatal abdominal ultrasonographic abnormalities after FLA should have close prenatal and postnatal assessment to detect bowel complications.

Introduction: Ultrasound-guided bipolar umbilical cord occlusion (UCO) is used in complicated monochorionic multiple pregnancies in Denmark. The aim of this study was to assess a learning curve in the procedure of UCO. Materials and Methods: One hundred and two monochorionic pregnancies treated with UCO at Rigshospitalet, Denmark between 2004 and 2015 were included. The procedures were divided into period 1 (2004-2009) and period 2 (2010-2015) to determine a learning curve. Primary outcome measure was survival rate. Secondary outcome measures were time from operation to fetal loss and gestational age (GA) at delivery. Results: Period 1 included 59 cases. The median GA at procedure was 19.9 weeks (range 16.7-25.9) and at delivery 34.7 weeks (range 24.3-40.3). Period 2 included 43 cases. The median GA at procedure was 20.7 weeks (range 16.7-27.6) and at delivery 37.3 weeks (range 29.1-40.3). Survival rate increased from 78% (period 1) to 95% (period 2) (p = 0.02). GA at delivery increased as well. Fetal death within 48 h after surgery decreased from 4 (period 1) to 0 (period 2). Discussion: Our results suggest a learning curve in the procedure of UCO with improved outcome on all measures.

Background: Multifetal pregnancies are considered high-risk pregnancies compared to singleton pregnancies. Monochorionic diamniotic (MCDA) twin pregnancies tend to have a more complicated intrauterine course than their dichorionic counterparts. The most common complications are twin-to-twin transfusion syndrome (TTTS), selective fetal growth restriction (sFGR), and unexplained intrauterine fetal demise (IUFD). Early recognition of pregnancies at risk of developing complications will be helpful for counselling the parents and preparing them for necessary interventions in case a complication arises. Objectives: The aim of our study was to assess the role of discordant crown-rump length (dCRL), discordant nuchal translucency (dNT), and discordant abdominal circumference (dAC) measurements in isolation and in combination at the 11–13+6 weeks’ scan in predicting the development of TTTS, sFGR, and IUFD. Methods: All MCDA twin pregnancies with structurally normal, both live fetuses at the 11–13+6 weeks’ scan were followed up by FMF (Fetal Medicine Foundation)-certified operators for development of TTTS, sFGR, and single/double IUFD until delivery. Discordance of CRL, NT, and AC of more than or equal to 10% was considered a “marker,” and its presence was correlated with the occurrence of specific antenatal complications, i.e., TTTS, sFGR, and single or double IUFD. Results: Combination of two markers predicted MCDA-related complications in the majority of cases (70.8% positive predictive value). Combination of dNT and dAC was the strongest predictor (80% positive predictive value). Conclusions: In our population of MC twin pregnancies, dCRL, dNT, and dAC at the 11+0–13+6 weeks’ scan, in isolation, had a low predictive value for antenatal complications, i.e., TTTS, sFGR, and single/double IUFD. However, the presence of at least two markers in the first trimester could predict complications in the majority of these pregnancies. The combination of dNT and dAC appears to be the best.

Preterm birth remains a major complication of fetal laser surgery (FLS) due to twin-to-twin transfusion syndrome (TTTS). Objectives: We tested the hypothesis that neonatal outcomes in fetuses born at >24 weeks are worse in TTTS survivors compared to uncomplicated monochorionic diamniotic (MCDA) twins. Methods: 196 patients with TTTS treated with laser therapy and 91 uncomplicated MCDA gestations were compared. Neonatal outcomes included respiratory distress syndrome (RDS), transient tachypnea of the newborn (TTN), bronchopulmonary dysplasia, intraventricular hemorrhage, necrotizing enterocolitis, and neonatal death. Risk factors assessed were TTTS, maternal age, maternal body mass index, race, premature prolonged rupture of membranes, stage of TTTS, parity, and gestational age (GA) at delivery. Results: GA at delivery was lower in the TTTS group (31.0 ± 4.6 vs. 33.5 ± 2.4 weeks, p < 0.001). RDS and TTN occurred at higher rates in the TTTS than in the uncomplicated MCDA group. After multivariate logistic regression, the only factor significantly associated with the composite adverse outcome was GA at delivery (OR 0.61; 95% CI: 0.58–0.7). Conclusion: TTTS twins treated with FLS are deliver 2.5 weeks earlier than uncomplicated MCDA twins. Respiratory complications were significantly higher in TTTS twins and were mainly the consequence of the early GA at delivery.

Pseudoamniotic band syndrome (PABS) is a rare iatrogenic complication that arises after invasive procedures in monochorionic twins. We report 3 cases of PABS, 2 after fetoscopic laser photocoagulation and 1 after bipolar cord coagulation. Two cases were detected antenatally by ultrasound; out of the two, one underwent successful fetoscopic release of amniotic band, which is the first report in twin pregnancy to our knowledge. In our centre, the incidence of PABS was found to be 2%. There were 25 cases of PABS reported previously, of which 12 cases with clinical details were reviewed together with our 3 cases. The fetal limbs were involved in all 15 cases, leading to constriction or amputation. The umbilical cord was involved in 2 cases, resulting in fetal death in one and pregnancy termination in the other. Antenatal detection of PABS is rare (27%; 4/15) as this requires a high index of suspicion. Serial postoperative targeted ultrasound surveillance of the fetal limbs and umbilical cord is necessary, particularly when features of septostomy or chorioamniotic membrane separation are found. Colour Doppler examination for the perfusion of the affected limb should be performed when PABS is detected. Fetoscopic release of amniotic band could salvage the fetal limb from amputation when impaired blood flow is detected.

Objective: To determine predictors of survival in monochorionic diamniotic twins with selective fetal growth restriction type II (sFGR-II), with or without twin-to-twin transfusion syndrome (TTTS), treated by endoscopic placental laser coagulation. Methods: Laser surgery was performed at 20 (15-27) weeks' gestation in 405 cases of sFGR-II with and 142 without coexisting TTTS. Multivariable logistic regression analysis was performed to determine significant predictors of survival to discharge from hospital. Results: There was survival of the small twin in 216 (39.5%) and of the large twin in 379 (69.3%) cases. Significant predictors of survival of both the small and larger twin were ductus venosus Doppler findings in the small twin, gestational age at laser and cervical length, but not the presence of TTTS or Doppler findings in the large twin. Conclusions: In sFGR-II, survival after laser surgery is primarily dependent on the condition of the small twin.

Introduction: Cytokine imbalance has been implicated in placental-related pathologies, i.e. recurrent miscarriage and pre-eclampsia. Such conditions are more prevalent in multiple pregnancies. Twin-to-twin transfusion syndrome (TTTS) is associated with asymmetric placental blood flow and intra-cardiac pressures. We hypothesised that cytokine expression may be aberrant in this condition and that fetoscopic laser ablation (FLA) may cause local cytokine release. Material and Methods: A prospective cohort of monochorionic, diamniotic twins with TTTS (n = 23) was studied. Circulating T helper cell type 1 (TH1)/TH2 maternal cytokines and cytokine-related and angiogenic factors were measured in plasma and amniotic fluid before and after FLA by human FASTQuant or ELISA. Basal comparisons were made with uncomplicated monochorionic and dichorionic (DC) twins. Results: Median maternal plasma platelet-derived growth factor-BB was highest in uncomplicated DC twins (p = 0.049), whereas tissue inhibitor of metalloproteinases (TIMP)-1 was highest in TTTS twins (p = 0.003). In TTTS amniotic fluid, interleukin (IL)-6, IL-1β, tumour necrosis factor-α, IL-10, IL-4, IL-8, interferon-γ, TIMP-1 and intercellular adhesion molecule-1 were significantly higher than maternal plasma concentrations. There were no significant differences in plasma or amniotic fluid cytokines after FLA, with the exception of amniotic fluid keratinocyte growth factor, which was significantly reduced. Discussion: TTTS is associated with minimal changes in cytokine levels when compared to uncomplicated twins, although the majority of cytokine levels were higher in amniotic fluid than maternal blood. It does not appear that FLA evokes a significant change in cytokines.

Aim: To report our experience in selective termination of monochorionic twin pregnancies with bipolar cord coagulation and to analyze the pregnancy outcomes and complications based on the indication of the procedure. Methods: This is a retrospective study of 71 complicated monochorionic pregnancies treated with bipolar cord coagulation between August 2006 and March 2013. Results: The rates of live birth and survival up to 28 days after birth were 73.2% (52/71) and 63.4% (45/71), respectively. The highest rates of survival up to 28 days after birth were in the procedures with an indication of selective intrauterine growth restriction, while the lowest rates of survival were recorded with the indication of twin reversed arterial perfusion sequence and discordant anomaly. However, there were no statistically significant differences in the live birth and perinatal survival rates among the four different groups of indications. Conclusion: The survival rate of bipolar cord coagulation in complicated monochorionic pregnancies such as twin-to-twin transfusion syndrome, twin reversed arterial perfusion sequence, selective intrauterine growth restriction and discordant anomaly was 63% in our series.

Aim: To review the perinatal outcome of twin-to-twin transfusion syndrome (TTTS) treated with fetoscopic laser coagulation in a developing country with detailed analysis according to the stage of the syndrome. Methods: This was a retrospective study of 85 TTTS cases treated with fetoscopic laser coagulation at the Fetal Diagnosis and Treatment Unit of Istanbul Faculty of Medicine between January 2006 and March 2013. Results: The surgical failure rate was 5.8% (5/85). Among all the cases of the total cohort, only 1 fetus survived in 27 pregnancies (31.8%), and both fetuses survived in 22 pregnancies (25.9%). In 49 pregnancies (57.6%) at least one fetus survived at the end of the neonatal period. The overall survival and live birth rates were 41.8% (71/170) and 56.4% (96/170), respectively, and they significantly decreased as the stage of disease increased. Delivery occurred before 32 weeks of gestation in 54 (63.5%) pregnancies. Logistic regression analysis showed that gestational age at delivery was the only independent factor, and the risk of nonsurvival significantly decreased with increasing age. Conclusion: Based on our experience, the outcome of fetoscopic laser coagulation of the placental anastomoses for TTTS became worse as the Quintero stage of the disease advanced.

Introduction: This study aimed to investigate the risk factors, incidence, and influence on the perinatal outcome of chorioamniotic membrane separation (CMS) after fetoscopic laser photocoagulation (FLP). Material and Methods: This retrospective study included 312 women who underwent FLP for twin-to-twin transfusion syndrome (TTTS). Clinical records were used to review obstetrical examinations, complications, operative data, ultrasonographic findings, and perinatal and neonatal follow-up data in all patients. Results: A total of 260 cases of TTTS were analyzed. The incidence of CMS was 12.7% (33 cases). The only independent risk factor of CMS was performing FLP before 20 weeks of gestational age (GA) (odds ratio = 3.38 [1.44-7.93], p = 0.005). Concerning perinatal outcome, CMS was only related with increased risk of premature rupture of membranes (PROM) before 32 weeks (33.3 vs. 13.9%, p = 0.005), with no differences in GA at delivery and survival rate. Discussion: In our cohort, the incidence of CMS after FLP was approximately 13%, with surgery before 20 weeks being the only risk factor identified. Although in our population CMS did not worsen perinatal outcome in terms of preterm delivery or survival, the preterm PROM rate was increased and this should be considered for the patients' management.

Objective: To report the incidence of fetal and maternal complications after selective fetoscopic laser surgery for twin-to-twin transfusion syndrome (TTTS). Methods: A total of 150 cases of TTTS were treated from January 2004 to June 2009 (period 1, 2004–2006, 62 cases; period 2, 2007 to June 2009, 88 cases). Fetal complications (double and single intrauterine fetal death, recurrence of TTTS, twin anemia-polycythemia sequence (TAPS), reversal of TTTS, cerebral lesions in one twin) and maternal complications were recorded, and retrospectively analyzed. Results: Nineteen (12.6%), 58 (38.7%), 61 (40.7%) and 12 cases (8.0%) were classified preoperatively as Quintero stage I, II, III and IV, respectively. The anterior placenta was described in 73 cases (48.6%). Double and single fetal death occurred overall in 7.3 and 36.0% of cases, respectively. The rate of recurrence was 11.3%, of TAPS 3.3%, and of reversal of TTTS 1.3%. Cerebral lesions were diagnosed in 3 donors (2.0%). Eighteen cases (12.0%) of fetal complications had a second procedure (6 repeat laser, 4 serial amnioreduction, 8 bipolar cord coagulation). Pregnancies undergoing a second procedure delivered at a median gestational age of 30.2 weeks compared to 32.1 weeks for those not repeating (p = 0.04). Perinatal survival of at least one twin improved from 66.1 to 79.5% (p = 0.06) in the two consecutive periods. For every 10 laser surgeries performed, there was an average improvement of 1.5% in the predicted percentage of survival of at least one twin (OR 1.09, 95% CI 1.00–1.19). Major maternal complications occurred in 9 cases (6.0%), 3 of which required admission to intensive care unit. Conclusions: Fetal complications are common after fetoscopic laser surgery. In this experience, an increasing number of procedures improved the performance of a new fetoscopic laser center.

Objective: To analyze social and economic factors affecting the implementation of a fetal surgery program in a developing country. Materials and Methods: During an 18-month period, 30 women with complicated monochorionic diamniotic twin pregnancies were evaluated. 27 women had twin-to-twin transfusion syndrome (TTTS), 2 women had a selective intrauterine growth restriction, and 1 a discordant twin pregnancy with 1 fetus with a severe open neural tube defect. The TTTS cases were treated with fetoscopic laser ablation of the interplacental vascular communications and the remaining 3 cases with bipolar cord occlusion of the affected twin. The patients were evaluated using family income levels (very low, low, median, and high income). Demographic characteristics, gestational age at referral, the prevalence of infections, tocolysis care with hospital readmission, prolonged hospital stay until delivery, and gestational age at delivery were evaluated. Results: The overall survival rate of fetuses treated with laser ablation was 60% (32/54). Survival of at least 1 twin was achieved in 21/27 pregnancies (77%), and survival of both twins in 12/27 pregnancies (44%). The 3 pregnancies treated with cord occlusion had survival of the normal twin. Patients with low and very low family income showed a significantly higher prevalence of vaginal infections, increased periods of tocolysis requiring hospitalization, prolonged hospital stay until the end of pregnancy, and lower gestational age at delivery. Conclusion: When a fetal surgery program is implemented in a developing country, social and economic factors will influence the overall healthcare costs and treatment outcomes.

About 30% of monochorionic twin pregnancies are complicated by twin-to-twin transfusion syndrome (TTTS), isolated discordant growth, twin anemia-polycythemia sequence, congenital defects or intrauterine demise. About 15% will be eligible for invasive fetal therapy, either fetoscopic laser treatment for TTTS or fetoscopic or ultrasound-guided umbilical cord coagulation for a severe congenital defect in one twin or severe discordant growth with imminent demise of the growth-restricted twin. Ultrasound examination in the first and early second trimester can differentiate the monochorionic twins at high risk for adverse outcome from those likely to be uneventful, which may be useful for patient counselling and planning of care.

We report a case of severe twin-to-twin transfusion syndrome (TTTS) in which fetoscopic laser ablation was successfully performed at 21 weeks’ gestation. Despite successful resolution of signs on ultrasound examination after a period of 7 weeks, the donor developed polyhydramnios whereas the ‘initial’ recipient maintained normal liquor volume, features suggestive of ‘reversed TTTS’. Two live babies were delivered at 31 weeks’ gestation with good neonatal outcome. The karyotype of both babies was normal. No significant inter-twin difference in umbilical vein haemoglobin levels was present at delivery and placental injection studies did not identify persistent anastomoses. This case report supports the importance of close monitoring of the pregnancy following apparently successful laser therapy. Further research is needed to understand the pathophysiology of this condition.

Objective: Laser coagulationof anastomotic vessels on the placental surface is the treatment of choice in severe second trimester twin-to-twin transfusion syndrome (TTTS). This procedure is associated with technical difficulties when the placenta is located on the anterior side of the uterus. We describe a novel technique for fetoscopy in TTTS with completely anterior placenta where laparoscopy is used to guide safe percutaneous insertion of the fetoscope through the lateral abdominal wall and the dorsal side of the uterus. Methods: Prospective controlled series of 16 TTTS pregnancies with completely anterior placenta (study group) treated with this novel technique. Studied outcomes were technical result of the procedure and perinatal survival. Outcome in the study group was compared with outcome of 49 TTTS pregnancies treated with conventional percutaneous fetoscopic laser without laparoscopy, 9 of these with partially anterior placenta (control group A) and 40 with lateral or posterior placenta (control group B). Results: In the study group, the procedure-related complication rate was 25% (4/16). In 1 case, uterine entry of the fetoscope from the lateral abdominal wall was not possible due to complex bowel adhesions. In 3 patients, intra-amniotic haemorrhage occurred after fetoscopic entry, preventing complete laser coagulation of anastomoses. One of these patients required 2 units of blood transfusion. The procedure-related complication rate in control groups A and B was 22% (2/9) and 5% (2/40), respectively (intra-amniotic haemorrhage n = 3, severe leakage of amniotic fluid into the peritoneal cavity, n = 1). Perinatal survival in the study group, control group A and control group B was 63% (20/32), 78% (14/18) and 70% (56/80), respectively. Conclusion: Combined laparoscopy and fetoscopy is a novel technique that enables safe uterine entry and creates optimal visualisation for laser coagulation of inter-twin anastomoses in TTTS pregnancies with completely anterior placenta. The procedure-related complication rate and perinatal survival rate were similar compared to the conventional percutaneous technique. Procedure-related complications occur more often with partially or completely anterior placenta.

Objective: To present an ethically justified decision-making pathway to use in the evaluation and treatment of pregnant women whose fetuses have twin-to-twin transfusion syndrome (TTTS). Methods: The ethical principles of beneficence, autonomy and professional integrity were applied to the diagnostic and treatment options for TTTS. Results: An algorithm was developed that includes the use of directive and non-directive counseling which can be used as a guide for informed consent of women whose pregnancies have TTTS. Conclusion: Ethics is an essential component of decision making for both pregnant women and their obstetricians in pregnancies complicated by TTTS.

Objective: This study aimed to assess the cardiac function of healthy and pathological fetuses by measuring radial velocity using phased tracking (PT). Based on phase differences, PT allows the displacement of a specified point to be detected with improved spatial and temporal resolution. Methods: PT was used to assess cardiac radial velocity in the basal free wall of the left and right ventricles in 134 healthy fetuses, 10 second-trimester intrauterine growth-restricted (IUGR) fetuses, and 10 recipient twins with twin-to-twin transfusion syndrome (TTTS). Maximum velocities were measured in systole and early diastole. Results: Maximum radial velocity was successfully measured in 126 healthy fetuses (94%) at gestational ages of 16-40 weeks. Systolic and early diastolic maximum velocities increased with gestational age in both ventricles. As compared with controls, IUGR fetuses had significantly lower early diastolic maximum velocities in the right ventricle, and recipient twins with TTTS had significantly lower systolic and early diastolic maximum velocities in both ventricles. Conclusions: PT demonstrated right ventricular diastolic dysfunction in second-trimester IUGR fetuses as well as systolic and diastolic dysfunctions in both ventricles in recipient twins with TTTS. PT could be useful for evaluating fetal cardiac radial function.

Objective: To clarify the long-term neurodevelopmental outcome of twin-to-twin transfusion syndrome (TTTS) survivors. Methods: From January 1994 to December 2003, 33 newborns with TTTS who were derived from 18 monochorionic twin gestations were enrolled for the study. The development and growth at 3 or 6 years of age were compared between TTTS survivors and weight-matched singleton controls. Results: The mortality in TTTS (8/33: 24%) was significantly higher than that of the controls (7/80: 9%). Of all 12 TTTS patients who were diagnosed before 25 weeks and born before 28 weeks of gestation, in 10 (83%) it led to neonatal or intrauterine death. The morbidity of cerebral palsy, epilepsy, and mental retardation did not differ between TTTS and control patients. The morbidity and severity of neurological deficits at school age were similar in both groups. No growth parameters at 3 or 6 years of age differed between TTTS and controls. Conclusions: Morbidity, growth and development of TTTS survivors attained comparable levels of weight-matched controls, despite the higher neonatal mortality. These results suggest that the early management of TTTS is critical for refining the total outcome of the affected twins.

Objectives: In twin pregnancies, it has been suggested that fluctuations of the two fetal heart rates should be considered as two variates that affect each other. We therefore investigated whether the relative power contribution (RPC) of heart rate fluctuation between twins reflects the clinical severity of twin-to-twin transfusion syndrome. Study Design: Sixty-three cases of twin pregnancy including 43 monochorionic twins and 20 dichorionic twins were studied. Thirteen monochorionic twins with polyhydramnios in one twin were regarded as twin-to-twin transfusion syndrome (TTTS). Of the 13 TTTS cases, 8 cases with polyhydramnios in one twin and oligohydramnios in the other were deemed to be a ‘stuck’ twin. The RPC of the very low frequency domain (VL; 0.0125–0.0625 Hz) of fetal heart rate fluctuation in the twin fetuses of monochorionic and dichorionic pregnancies was obtained within a week of delivery. The relationship between the value of the RPC and the outcome of these twins was examined. Results: For both monochorionic and dichorionic twins the RPC of twin fetuses was significantly higher in TTTS twins than in twins without TTTS. In particular, in pregnancies that resulted in fetal death, early neonatal death, or hydrops of one of the twins, this twin had a higher RPC than the other twin. No significant difference was observed in the RPC value between twins of either monochorionic or dichorionic pregnancies that did not develop TTTS. Serial changes in RPC values were followed in 7 cases of TTTS. The RPC value rose rapidly just before delivery in three cases with resultant poor outcome. Conclusion: A rapid change in the RPC of twin fetuses measured using the VL frequency domain of fetal heart rate fluctuations may predict poor outcome in twin pregnancies.

We report the prenatal diagnosis of twin-to-twin transfusion syndrome (TTS) at 11 weeks’ gestation. The diagnosis was made in a trichorionic quadruplet pregnancy which was conceived after in vitro fertilization and intracytoplasmic sperm injection for male subfertility and transfer of 3 embryos. Growth discordance, oligo/polyhydramnios and abnormal arterial and venous Doppler flows were demonstrated in 2 monochorionic fetuses, while the remaining 2 dichorionic fetuses were unremarkable. Selective fetocide of the donor by intracardiac injection of potassium chloride was followed by the spontaneous demise of the recipient. The pregnancy course remained uneventful until 32 weeks of gestation when the patient developed preterm labor. Two healthy preterm babies were delivered by cesarean section. The diagnostic problems of this early manifestation of TTS are discussed.

Objective: To report our results of the measurement of the amniotic fluid pressure in twin-to-twin transfusion syndrome before and after amniodrainage and the separate measurement in the cavity of the stuck twin and the recipient, respectively, in 2 cases. Method: Six patients with severe TTS underwent therapeutic amniodrainage. Amniotic fluid pressure was obtained before and after reduction of the fluid volume. In 2 of these cases we were able to calculate the pressure separately in both amniotic cavities, the donors (stuck twin) and the recipients. Results: In all cases of TTS, amniotic pressure was high before and reduced after amniodrainage. The pressure was equal in the donors and in the recipients cavity. Conclusion: Amniotic pressure seems to be high in TTS, but equal in both cavities. Therefore, amniotic septostomy is probably of low value, especially if a high-risk monoamniotic situation is artificially created.

Limb ischemia is an extremely rare event occuring in monochorionic twin pregnancy complicated by twin-to-twin transfusion syndrome (TTTS) and twin anemia polycythemia sequence (TAPS). The authors describe a case of TTTS and TAPS treated successfully using amnioreduction and laser ablation. However, severe ischemia of both lower extremities in the recipient twin developed after the fetal treatment. This serious complication was diagnosed on MRI in utero and confirmed postnatally. Elective amputation of the affected limbs was performed. The etiology of the disease remains unclear despite profound clinical and histopathological examinations; although the role of thromboembolism in monochorionic pregnancy seems to be most likely, this unique case of multiple limb ischemia with distinct macroscopic findings has not yet been described.

Objective: The management of a case of severe twin-to-twin transfusion syndrome at 24 weeks of gestation is presented, using a combined surgical and medical approach. Methods: Operative fetoscopy was used to coagulate placental vessels crossing the intertwin membranes. Repeated intrauterine transfusions were used to correct recurrent anemia in the donor twin. Selective three-dimensional placental angiography and directed histological analysis were used to study the placenta after delivery. Results: Resolution of twin-to-twin transfusion syndrome was obtained by coagulation of placental anastomoses, but was followed by recurrent anemia in the donor twin. This was successfully treated by serial intrauterine blood transfusions, and 2 healthy twins were delivered at 31 weeks of gestation. One artery-to-vein anastomosis was demonstrated to be the only communication left between the two circulations. Conclusions: This case illustrates the limitations of placental surgery in twin-to-twin transfusion syndrome and highlights the need for ultrasound and Doppler follow-up of these high-risk fetuses to indicate further treatment. Placental anastomoses can be overlooked by macroscopic examination and injection technique, but can be demonstrated by placental angiography.

A case of twin-to-twin transfusion syndrome (TTTS) was associated with ischemic injury of the left leg of the recipient twin. Five reduction amniocenteses were performed between 17 6/7 and 23 3/7 weeks followed by fetoscopic laser ablation of chorioangiopagus vessels at 23 6/7 weeks of gestation. Anatomic assessment of the affected limb indicated timing of the injury at 19 4/7 weeks gestational age. The published literature suggests that ischemic fetal injuries in severe TTTS are associated with blood hyperviscosity in the affected twin.

To improve the outcome of severe twin-to-twin transfusion syndrome with 1 hydropic fetus and to prevent ischemic sequelae in the survivor, we developed a technique of selective feticide by vascular embolization of the most severely damaged twin. Acute second trimester polyhydramnios occurred in 4 biamniotic monochorial twin pregnancies, with 1 fetus normal on ultrasound but the other severely damaged by hydrops and hypertrophic hypokinetic cardiomyopathy. The hydropic fetus underwent embolization using a bolus of histoacryl injected into the umbilical vein and fetal heart under ultrasound guidance. In 1 triplet pregnancy with a set of monochorial fetuses, premature labor occurred at 26 weeks, 2 weeks after embolization, and there were 2 neonatal deaths. The 3 other cases resulted in the birth of a normal infant at 31-37 weeks of gestation. This suggests that in twin-to-twin transfusion syndrome with severe polyhydramnios and hydrops of 1 fetus, embolization may salvage the other twin.

Ten sets of diamniotic twins with discordant fetal growth (ultrasonographic estimated fetal weight difference > 25%) and abnormal amniotic fluid volume were assessed for fetal serum erythropoietin con

The discovery of a 'stuck twin' during an ultrasound examination has often been equated with twin-to-twin transfusion based to a great extent on postnatal confirmation of discordant fetal size and hemoglobin concentration. However, the diagnosis of twin-to-twin transfusion cannot be made with certainty after birth since virtually all monochorionic gestations have placental anastomoses and there are many causes of growth deficiency and abnormal hemoglobin concentration. The purpose of this study is to investigate the pathophysiology of chronic twin-to-twin transfusion syndrome and apply the findings to the development of a management algorithm. In 42 twin gestations with stuck twin associated with acute hydramnios, we performed targeted ultrasound cordocentesis in each fetus and therapeutic amniocentesis. The diagnosis of chronic twin-to-twin transfusion syndrome required: sonographic evidence of monochorionicity; rapid reaccumulation of fluid after amniocentesis; discordant fetal size, and divergent fetal hematocrit measurements with at least one above or below the 95% confidence interval for gestational age. These criteria were met in 20 of 42 (48%) pregnancies. The mean gestation was 23.8 +/- 2 weeks (range 21-27 weeks). In 4 pregnancies, the transfer of adult RBCs from the donor to the recipient was documented. Monochorionicity was confirmed in all postnatally. All recipients had polycythemia and hyperproteinemia. Hydrops developed only in the recipient twin (6 of 20) and was associated with an elevated umbilical venous pressure. All pregnancies were treated with aggressive serial therapeutic amniocenteses. There was no objective evidence that amniocentesis altered the magnitude of the shunt.(ABSTRACT TRUNCATED AT 250 WORDS)

We performed percutaneous umbilical blood sampling, under ultrasound guidance, in both twins of 11 twin pregnancies in whom twin-to-twin transfusion syndrome was suspected. Chorionicity and the presence of placental vascular anastomoses were assessed postnatally. The hemoglobin concentration, hematocrit and other biochemical variables in fetal blood were evaluated in both twins. The mean hemoglobin difference between the large and small twins was 4.8 g/dl (range 1.8-8.1 g/dl) in 5 monochorionic discordant twin pairs, and 1.2 g/dl in a monochorionic twin pair without discordancy. Hemoglobin values did not differ between dichorionic twins. The mean hematocrit difference between monochorionic discordant twins was 18.3%. In dichorionic discordant twins, the inter-twin hematocrit difference was very small. Total protein and albumin were normal in all twins.

We describe a monochorionic twin gestation with a severe twin-to-twin transfusion syndrome associated with a preterm premature rupture of membranes at 20 weeks of gestation in the polyhydramniotic sac. The pregnancy was managed expectantly and outcome was favourable for the 2 neonates. We discuss how these two severe pathologies seem to counteract each other by compensations of their symptoms.

Pseudoamniotic band syndrome (PABS) is an iatrogenic complication that causes entanglement of fetal parts in a constrictive sheet of detached or ruptured amniotic membrane after an invasive procedure, namely amniocentesis, amnioreduction or septostomy in twins. The incidence and risk factors for PABS after fetoscopy-guided laser have not been documented [Winer et al.: Am J Obstet Gynecol 2008;198:393.e1–393.e5]. We report a case of monochorionic biamniotic twin pregnancy submitted to selective fetoscopic laser photocoagulation for twin-to-twin transfusion syndrome at 16 weeks of gestation. The procedure was complicated by the death of one of the fetuses at 24 weeks of gestation. Moreover, the surviving twin was diagnosed postnatally with pseudoamniotic band syndrome, presenting with affected limbs. The newborn was submitted to surgical correction of these lesions with a successful outcome and was discharged on day 15.

Objective: To compare the outcomes of twin-to-twin transfusion syndrome (TTTS) cases treated with fetoscopic laser coagulation of vascular anastomoses before 25 + 6 weeks of gestation and between 26 and 28 weeks of gestation. Material and Methods: 28 consecutive cases of TTTS at Quintero stages II-IV treated with laser therapy between 26 + 0 and 28 + 6 weeks of gestation were compared with 324 cases treated between 15 + 0 and 25 + 6 weeks during a 3-year period in two centers. The following data were recorded and compared: duration of the fetoscopy, rate of complications (preterm labor before 28 weeks and before 32 weeks, chorioamnionitis, twin anemia-polycythemia syndrome and recurrent TTTS), gestational age at delivery and neonatal survival rate. Results: The study groups were similar as regards Quintero staging and the frequency of anterior placental location (50.0 vs. 47.8%, p = 0.85 in late and conventional laser, respectively). There were no significant differences in the duration of surgery (29 vs. 30, p = 0.27, respectively) and in the rates of any of the complications evaluated. Gestational age at delivery (33 vs. 33.3 weeks, p = 0.69) and neonatal survival of at least one fetus (92.3 vs. 88.6%, p = 0.24) were also similar. Conclusion: Fetoscopic laser coagulation for TTTS performed between 26 + 0 and 28 + 6 weeks of gestation was associated with similar outcomes as those observed in cases treated before 26 weeks.

In the first trimester the ductus venosus can be easily identified with color Doppler and a ductus venosus waveform can be obtained by pulsed Doppler. At 11–13 weeks the prevalence of abnormal a-wave in the ductus venosus is inversely related to fetal crown-rump length and maternal serum pregnancy-associated plasma protein-A (PAPP-A), increases with fetal nuchal translucency (NT) thickness and is more common in women of Black racial origin and in fetuses with abnormal karyotype or cardiac defects. Ductus venosus flow provides an independent contribution in the prediction of chromosomal abnormalities when combined with NT and the maternal serum markers of PAPP-A and free β-hCG, increasing the detection rate to 96% at a false-positive rate of 2.6%. Abnormal ductus venosus flow increases the risk of cardiac defects in fetuses with NT above the 95th centile, and it may increase the risk in fetuses with normal NT. In twin pregnancies, abnormal ductus venosus flow is associated with chromosomal abnormalities and cardiac defects. In monochorionic twins, abnormal flow in the ductus venosus in at least 1 of the fetuses increases the risk of developing twin-to-twin transfusion syndrome.

We report a case of severe polycythemia in the recipient twin of a pregnancy with twin-to-twin transfusion syndrome (TTTS). Stage I TTTS was suspected at initial presentation (24 weeks) due to discordant growth associated with a twin oligo-polyhydramnios sequence. Increasing peak systolic velocity (PSV) in the middle cerebral artery (MCA) of the donor twin indicated fetal anemia. In addition, we observed a decreasing MCA-PSV of the recipient twin, indicating polyglobulia. Close follow-up without intervention showed a stable course of TTTS without progression to higher stages.Following caesarean section at 31 2/7 weeks due to preterm premature rupture of membranes of the leading donor twin, the donor twin showed anemia (hematocrit 28%) whereas the recipient twin demonstrated severe polycythemia (hematocrit 88%). Inter-twin difference of reticulocyte counts was high (38% for the donor and 5% for the recipient twin). Transfusion and partial exchange transfusion (PET) were performed in the donor and in the recipient twin, respectively. Normal hematocrit levels were achieved without complication. Perinatal outcome of both twins was good. Conclusion: Even in a stable course of pregnancy with stage I TTTS, severe polycythemia of the recipient twin can occur and can require careful PET in a specialized center.

Objective: Our aim was to evaluate the consequences of polyhydramnios on maternal plasma volume and maternal serum aldosterone and atrial natriuretic peptide (ANP) concentrations in monochorionic twin pregnancies complicated by twin-to-twin transfusion syndrome (TTTS). Methods: The maternal plasma volume, ionograms as well as plasma ANP and aldosterone concentrations were compared in normal monochorionic diamniotic twin pregnancies and in TTTS. Results: The maternal plasma volume was normal in TTTS despite severe polyhydramnios. The plasma aldosterone concentration was higher in pregnancies complicated by TTTS. Conclusion: The high plasma aldosterone concentration is likely to maintain normal plasma volume despite progressive extravascular volume enhancement. This suggests that TTTS is present early in pregnancy as a chronic phenomenon starting before its clinical expression and may trigger compensatory maternal hyperaldosteronism.

In a stage III twin-to-twin transfusion syndrome, death of the donor at 31 weeks was followed by unusual umbilical artery (UA) doppler findings in the recipient, with a high diastolic peak velocity and a low S/D ratio. Besides, middle cerebral artery waveforms could not be obtained in the recipient. The recipient was delivered by emergency cesarean section, weighed 1,230 g, was strikingly pale and had a cord hemoglobin concentration of 15 g/dl. Neonatal blood average pressure was of 29 mm Hg, immediately after delivery, and ranged from 33 to 43 mm Hg in the first 12 h of life. Neonatal heart function returned to normal on day 2. We hypothesize that death of the donor twin induced an acute decrease in placental resistance. This phenomenon, combined with decreased systolic blood flow due to the recipient’s systolic function impairment, could account for the umbilical artery flow pattern we observed. The acute decrease in volemia and the presence of systemic hypertension and cardiomyopathy may explain the inability to obtain middle cerebral artery doppler. This complex hemodynamic disturbance in the recipient may be responsible for morbidity unrelated to anemia in case of donor’s demise, as acute fetal distress.

Objective: In this prospective cohort study, we evaluated the initial results of fetoscopic laser surgery for severe second trimester twin-to-twin transfusion syndrome (TTTS) treated at our centre. Method: A total of 100 consecutive pregnancies with severe second trimester TTTS treated at our centre with selective fetoscopic laser coagulation of vascular anastomoses on the placental surface between August 2000 and November 2004 were included in the study. Perinatal survival was analysed in relation to Quintero stage. Results: Median gestational age was 20 weeks at fetoscopy (range: 16–26) and 33 weeks at delivery (range: 18–40). Perinatal survival rate was 70% (139/200). The treatment resulted in at least one survivor at the age of 4 weeks in 81% of pregnancies. Perinatal survival was significantly higher when treatment was performed in the early Quintero stages (95% in stage 1, 76% in stage 2, 70% in stage 3, 50% in stage 4) (p = 0.02). Conclusion: Results of fetoscopic laser surgery for TTTS in our centre are similar to those in specialised centres in other countries. Diagnosis and treatment in the early Quintero stages resulted in significantly higher perinatal survival.