Management Of Twin-twin Transfusion Syndrome Research Articles

OP26.02: A systematic review of diagnosis, management and outcomes for Twin–twin transfusion syndrome (TTTS) diagnosed before 18 weeks' gestation

Current management protocols for TTTS are optimised for twins at 18-24 weeks gestational age (GA), however, up to 25% of cases of TTTS may be diagnosed <18 weeks GA. Methods which could be used prior to fusion of amnion and chorion are needed. We systematically reviewed the published literature on diagnosis, management and outcomes of TTTS diagnosed at <18 weeks GA. PubMed and Scopus databases were searched for articles regarding early onset TTTS, from inception to April 2016. All studies types with primary data on TTTS <18 weeks GA in MCDA pregnancies were included. Exclusion criteria included studies not in English, higher order pregnancies, and diagnosis >18 weeks. Studies were independently assessed for inclusion by two reviewers. Authors of those with incomplete data were contacted. Data was extracted for study characteristics, interventions and outcomes and entered into an Excel spreadsheet and outcomes analysed. 16 studies included for analysis assessed 171 pregnancies. Earliest gestation at diagnosis was 14 weeks. 6 papers used DVP discrepancy for diagnosis, 6 used Quintero staging and the remaining used custom methods, TAPS diagnosis or unspecified criteria. The majority had treatment with laser in 88.9% of pregnancies; median gestational age at laser was 17+0 (range 16+0-21+6). The next commonest intervention (6.4%) was selective reduction. 2.3% underwent amniodrainage and 2.3% of pregnancies were terminated. Median gestational age at delivery was 31+3 (range 23+0-39+5). The rate of double twin survival at birth was 59.8%; at least one twin survived in 80.4% cases and 18.5% pregnancies had no survivors. Heterogeneity in outcomes was high. Outcomes of early gestation TTTS are better than expected. Management of TTTS at <18 weeks GA requires standardisation to standard management protocols. Adjustments of diagnostic criteria and the use and timing of existing invasive and emerging non-invasive treatment options that can be used at earlier gestations should be explored.

842: Management of twin-twin transfusion syndrome with an extremely short cervix

842: Management of twin-twin transfusion syndrome with an extremely short cervix

Histologic Chorioamnionitis and Funisitis After Laser Surgery for Twin-Twin Transfusion Syndrome.

To estimate the incidence of histologic chorioamnionitis and funisitis after fetoscopic laser surgery for the management of twin-twin transfusion syndrome. A case-control study was performed at the Leiden University Medical Center from 2013 to 2014. All patients with twin-twin transfusion syndrome managed with laser surgery during the study period were included and compared with a control group of all monochorionic twins not treated with laser surgery. We excluded patients with fetal demise or higher order pregnancies. Placentas were reviewed for the presence and degree of chorioamnionitis and presence or absence of fetal inflammatory response. The primary outcome was the incidence of histologic chorioamnionitis and funisitis after laser surgery. Odds ratios (ORs) and 95% confidence intervals (CIs) for primary outcomes were calculated. A P value of <.05 was considered as statistical significance. Sixty-two patients treated with laser surgery were included in the study group and compared with 64 patients in the control group. The incidence of histologic chorioamnionitis was 13% (8/62) in the laser group compared with 5% (3/64) in the control group (OR 3.0, 95% CI 0.8-11.9, P=.12). Funisitis occurred in 8% (10/124) in the laser group compared with 0% in the control group (OR 11.1, 95% CI 1.3-96.9, P=.03). Histologic chorioamnionitis with or without funisitis after laser surgery was associated with a shorter laser-to-delivery interval (median 6.6 [range 3.4-14.1] compared with 13.6 [4.4-20.1] weeks, P<.01) and lower gestational age at birth (median 28.1 [range 23.1-32.6] compared with 32.7 [24.4-37.0] weeks, P<.01). These findings suggest that laser surgery for twin-twin transfusion syndrome is associated with an increased risk of funisitis.

Diagnosis and Management of Twin-Twin Transfusion Syndrome

Twin-twin transfusion syndrome (TTTS) affects 10% to 15% of monochorionic pregnancies. In the absences of timely diagnosis and intervention perinatal loss or long term developmental delay can be expected in over 90% of cases. Establishing chorionicity in the first trimester followed by serial ultrasounds beginning at 16 weeks of gestation and intervention with placental laser ablation before the development of advance disease overall survival rates can be expected in 70% to 80% of cases.

Donor Death After Selective Fetoscopic Laser Surgery for Twin-Twin Transfusion Syndrome.

To assess the incidence, timing, and risk factors for death of the donor fetus after fetoscopic laser surgery, we evaluated our cohort of patients who underwent the procedure for twin-twin transfusion syndrome. This was a prospective cohort study of 166 consecutive patients with twin-twin transfusion syndrome at a single center. Fetal death was diagnosed by ultrasonography after surgery and before onset of labor. Risk factors for death of the donor twin were identified on univariate analysis and then subjected to multivariate, stepwise, logistic regression analysis. Donor demise occurred in 20 (13%) cases and recipient twin death occurred in four (2.6%). The median procedure to death interval was 4 days (range 1-89 days). Risk factors for donor death were fetal growth discordance greater than 30% (odds ratio [OR] 6.7, 95% confidence interval [CI] 2-23), reverse end-diastolic velocity in the donor umbilical artery (OR 25.0, 95% CI 2-290), a marginal and velamentous cord insertion (OR 4.4, 95% CI 1-19), and an increased number of anastomoses (OR 1.2, 95% CI 1.1-1.5). All four donors with both fetal growth discordance greater than 30% and reverse end-diastolic velocity in the donor umbilical artery resulted in a demise. Four risk factors significantly affecting acute and delayed donor demise after fetoscopic laser surgery were identified. The presence of both fetal growth discordance greater than 30% and reverse end-diastolic velocity in the donor umbilical artery was highly predictive of donor demise in our cohort. Knowledge of these risk factors can aid in counseling and assist patients in choosing the most appropriate intervention in the management of twin-twin transfusion syndrome. III.

Ventricular strain changes in monochorionic twins with and without twin-to-twin transfusion syndrome

The objective of the study was to investigate whether vector velocity imaging (VVI), a non-Doppler speckle tracking ultrasound technology, is feasible in twin pregnancies and can aid management of twin-twin transfusion syndrome (TTTS). Twenty-seven women pregnant with monochorionic diamniotic twins affected by TTTS and 28 monochorionic pregnancies that did not develop TTTS were included in a prospective case-control study at a fetal medicine center. Fetal echocardiograms were recorded with dummy electrocardiography to retain original frame rates when exported for offline speckle tracking analysis using Syngo-VVI software (Siemens Corp, Munich, Germany). Right and left ventricular (LV) free wall Lagrangian strain was measured from the original coordinates. Within-twin pair ventricular strain differences including relationship to Quintero staging and response to laser therapy for TTTS were analyzed by Wilcoxon signed-rank test. The VVI strain measurements could be analyzed in 182 of 200 TTTS and 96 of 112 non-TTTS control ventricles. Within-pair strain was concordant in non-TTTS controls. Recipient LV strain was reduced at all Quintero stages compared with donors (P < .01). Recipient right ventricular strain was reduced only in stages 3 and 4 (P< .01). Strain improved at a median of 2 weeks following successful laser therapy. Intertwin differences in strain were independent of weight discordance. Recipient LV strain is reduced in stages 1 and 2 TTTS. Within-pair strain discordance may distinguish early TTTS from growthdiscordance and guide timing of and management following treatment.

Diagnosis and Management of Twin-Twin Transfusion Syndrome

ABSTRACT Twin-twin transfusion syndrome (TTTS) is a complication unique to monochorionic twin pregnancies, in which the two twins share a common placenta and unequal blood exchange from one twin (donor) to the cotwin occurs through placental arterovenous anastomoses. The diagnosis is posed with the sonographic detection of oligohydramnios in the donor's sac, as a consequence of hypovolemia, and polyhydramnios in the recipient's sac, resulting from hypervolemia. The severity of TTTS is assessed according to Quintero staging system, which is based on five stages that range from visualization of the donor's bladder to intrauterine demise of one or both twins. Because laser therapy has revealed the optimal treatment for TTTS, management of twins affected with TTTS consists in preoperative and postoperative assessment of the twins by ultrasound examination. The aim of this review was to describe the diagnosis and management of TTTS and discuss controversies and limitations of current literature. How to cite this article Rossi AC, D'Addario V. Diagnosis and Management of Twin-Twin Transfusion Syndrome. Donald School J Ultrasound Obstet Gynecol 2012;6(3):286-289.

Reduced left ventricular strain in the recipient heart is an early sign of twin-twin transfusion syndrome

Optimal management of twin-twin transfusion syndrome (TTTS) in monochorionic diamniotic (MCDA) twins remains challenging. Our aim was to investigate whether Vector Velocity Imaging (VVI), a non-Doppler speckle tracking ultrasound technology,...

A Rare Case of Antepartum Spontaneous Septostomy in a Monochorionic Diamniotic Twin Pregnancy

Spontaneous septostomy in a monochorionic diamniotic twin pregnancy is a rare phenomenon. We present a case of monochorionic diamniotic twin pregnancy with an intact dividing membrane seen in the 1st half of the pregnancy. At 26 weeks, when she was assessed for preterm contractions, the dividing membrane was not documented, which suggested spontaneous septostomy. There had been no invasive procedures during the pregnancy. She subsequently delivered at 29 weeks, secondary to preterm labor. No dividing membrane was noticed at the time of caesarian section. Spontaneous septostomy can complicate the management of monochorionic diamniotic twins by creating a pseudomonoamniotic environment resulting in cord entanglement, and difficulty in the diagnosis and management of twin-twin transfusion syndrome. We believe that such a case should be managed as monochorionic monoamniotic twin gestation.

Prevalence and progression of recipient‐twin cardiomyopathy in early‐stage twin–twin transfusion syndrome

The management of twin-twin transfusion syndrome (TTTS) in its early stages (Quintero Stages I and II) is controversial. We describe the prevalence, severity, incidence and rate of progression of recipient-twin cardiomyopathy in Stages I and II TTTS. Among 451 cases of TTTS evaluated between 2004 and 2009, 123 (27.3%) cases of Stages I and II were reviewed. Echocardiography was used to 'upstage' cases based on the presence or absence of mild (IIIA), moderate (IIIB), or severe (IIIC) recipient cardiomyopathy. Progression was defined by worsening in the degree of recipient-twin cardiomyopathy from initial presentation or failure to respond to amnioreduction. Outcome data included progression of recipient-twin cardiomyopathy, treatment and survival to birth. Data were compared by the chi-square, Fisher's exact test or t-test as appropriate. Seventy-seven of 123 (62.6%) cases were Quintero Stage I and 46/123 (37.4%) Quintero Stage II. Eighty (65.0%) were upstaged to Cincinnati Stage IIIA (n = 25), IIIB (n = 23) or IIIC (n = 32). Management included observation in 11 (8.9%), amnioreduction in 26 (21.1%), amnioreduction followed by selective fetoscopic laser photocoagulation (SFLP) in 43 (35.0%) and primary SFLP in 43 (35.0%). Of 80 cases managed by observation or amnioreduction initially, 43 (53.8%) progressed within a mean duration of 1.4 ± 1.5 weeks. The incidence of progression increased significantly as degree of recipient-twin cardiomyopathy at presentation worsened: Stage I, 9/27 (33.3%); Stage II, 8/15 (53.3%); Stage IIIA, 8/16 (50.0%); Stage IIIB, 10/10 (100%); and Stage IIIC, 8/12 (66.7%) (χ(2) = 14, P < 0.01). Overall fetal survival was 205 out of 244 (84.0%). Fetal survival with observation only was 81.8% (18/22), with amnioreduction only it was 92.3% (48/52), with initial observation or amnioreduction followed by SFLP it was 86.9% (73/84) and with primary SFLP it was 76.7% (66/86). Echocardiography demonstrates a high incidence of recipient-twin cardiomyopathy in early-stage TTTS. The more advanced the recipient-twin cardiomyopathy is, the more likely is progression to occur during observation or following amnioreduction.

Amnioreduction in management of twin-twin transfusion syndrome

Objective To find out the clinical outcomes of twin-twin transfusion syndrome (TTTS) after aminioreduction. Methods Twenty-nine pregnancies with TTTS delivered in Beijing Gynecological and Obstetric Hospital from Nov. 1, 2002 to Sept. 30, 2007 were recruited. The effect of gestational age at the onset, the degree of TTTS and amnioreduction therapy on the fetal outcomes were compared. Results All of the 8 cases with the onset of TTTS before 26 weeks without treat- ment were lost, but 4 out of the 6 with the onset after 26 weeks survived (P=0. 015). Among the 9 cases received amnioreduction, 4 out of the 5 with onset before 26 weeks survived, and significant difference was found compared to those untreated ones (P= 0. 007), but no difference was shown when compared with those developed after 26 weeks(4/4)(P= 1. 000). The average delivery weeks in the untreated ones was earlier than those received treatment E(28.7±4.7)weeks vs (33.2±4.9) weeks, P=0. 001]. Amnioreduction improved the umbilical cord blood flow. For severe TTTS(stage Ⅲ～Ⅳ) with the onset before 26 weeks, the survival rate after amnioreduction was significantly high- er than those untreated ones[4/5 vs 0/5, P=0. 048)]. Conclusions Aminioreduetion is effective in improving the cord blood flow, prolonging the pregnancies, and achieving a higher fetal survival rate. Key words: Amniotic fluid; Fetofetal transfusion; Treatment outcome

Analysis of the perinatal outcomes and management of twin-twin transfusion syndrome

To investigate the perinatal outcomes of twin-twin transfusion syndrome (TTTS) and the management. During Nov 1, 2002 to Sep 30, 2005, 24 cases of TTTS in Beijing Obstetrics and Gynecology Hospital were analyzed. The outcomes of them were compared with the pregnancy without TTTS in all twins and in monozygotic twins. The outcomes of the blood-supplying fetus and the blood-recepter were compared. 6.8% cases had TTTS in all twins. The group of TTTS had more maternal, fetal and neonatal complications than twins pregnancy without TTTS: polyhydramnios [37.5% (9/24) vs 2.1% (7/328), P < 0.01], gestational hypertension [20.8% (5/24) vs 7.0% (23/328), P = 0.043], premature labor [66.7% (16/24) vs 36.3% (119/328), P = 0.003], perinatal dead fetus in uterus [18.8% (6/32) vs 1.1% (7/640), P < 0.01], neonatal asphyxia [73.1% (19/26) vs 3.0% (19/632), P < 0.01], the proportion of NICU [88.5% (23/26) vs 23.4% (148/632), P < 0.01], neonatal death [15.4% (4/26) vs 1.7% (11/632), P = 0.002] and the rate of perinatal mortality [31.2% (0/32) vs 2.8% (18/632)]. Compared with the monozygotic twins without TTTS, in TTTS group there were more complications of the mother, the fetus and the neonates: gestational hypertension [20.8% (5/24) vs 9.9% (14/142), P = 0.224], premature labor [66.7% (16/24) vs 49.3% (70/142), P = 0.115], perinatal dead fetus in uterus [18.8% (6/32) vs 0.7% (2/282), P < 0.01], neonatal asphyxia [73.1% (19/26) vs 3.9% (11/280), P < 0.01], the proportion of NICU [88.5% (23/26) vs 29.3% (82/280), P < 0.01], neonatal death [15.4% (4/26) vs 2.1% (6/280), P = 0.006] and the rate of perinatal mortality [31.3% (10/32) vs 3.2% (8/282)]. The perinatal outcomes were better in those cases that the grades of TTTS were below 3 in the first diagnosis. We should try to diagnose and treat TTTS as early as possible because the outcome is poor.

Laser ablation for the management of Twin-Twin transfusion syndrome (TTTS)

Laser ablation for the management of Twin-Twin transfusion syndrome (TTTS)

Intrauterine surgery in monochorionic twins: management of twin-twin transfusion syndrome

Intrauterine surgery in monochorionic twins: management of twin-twin transfusion syndrome

Management of Twin–Twin Transfusion Syndrome: Laying the Foundation for Future Interventional Studies

Twin-twin transfusion syndrome is the most common complication of monochorionic pregnancies affecting between 5 and 15% of such pregnancies and accounts for 15-77% of perinatal mortality in twins. The management of twin-twin transfusion syndrome is complex and various treatment modalities have been tried. This review critically evaluates the different management options available for the syndrome and recommends some guidelines for future interventional studies.

Validation of the Quintero staging system for twin-twin transfusion syndrome

Objective To validate an established staging system for twin-twin transfusion syndrome. Methods Prospective observational study in a tertiary referral fetal medicine center of 52 consecutive cases of twin-twin transfusion syndrome. Each pregnancy was assessed longitudinally for a variety of prognostic factors including fetal biometry, amniotic fluid volume, arterial and venous Doppler sonogram abnormalities, and the presence of hydrops. Data were used to determine stage at diagnosis and first treatment, and worst stage throughout pregnancy. Perinatal outcome was assessed by stage. Management comprised serial amnioreduction, septostomy, selective reduction, or delivery, alone or in combination. Results Median gestation at presentation and first treatment were both 21 weeks (range 14–34 and 15–34), and at delivery it was 29 weeks (range 16–40). Sixty-three percent of pregnancies (33 of 52) were at least stage III at presentation. Forty-five percent of pregnancies (22 of 49) progressed to a more advanced stage. Overall survival was 47% (47 of 100), with no difference between donor and recipient fetuses (40% [20 of 50] versus 54% [27 of 50] [χ 2 P = .5]). Survival rates were 58% (15 of 26), 60% (six of ten), 42% (20 of 48), 43% (six of 14), and 0% (none of two) for stages I–V, respectively, with no significant influence of stage at presentation on survival. Survival was poorer where stage increased, versus decreased (27% [12 of 44] versus 94% [17 of 18] χ 2 P < .001). Kaplan-Meier survival curves indicated that staging at presentation identified pregnancies at greater risk of earlier rather than later gestational perinatal loss. Conclusion The Quintero staging system did not distinguish good from bad outcome at presentation, and thus should be used with caution in guiding initial management of twin-twin transfusion syndrome. However, prognosis was influenced by a change in stage, and pregnancies progressing to higher stage disease were at increased risk of earlier perinatal loss. Staging may thus be more useful in monitoring disease progression.

Validation of the Quintero Staging System for Twin-Twin Transfusion Syndrome

In Brief OBJECTIVE To validate an established staging system for twin-twin transfusion syndrome. METHODS Prospective observational study in a tertiary referral fetal medicine center of 52 consecutive cases of twin-twin transfusion syndrome. Each pregnancy was assessed longitudinally for a variety of prognostic factors including fetal biometry, amniotic fluid volume, arterial and venous Doppler sonogram abnormalities, and the presence of hydrops. Data were used to determine stage at diagnosis and first treatment, and worst stage throughout pregnancy. Perinatal outcome was assessed by stage. Management comprised serial amnioreduction, septostomy, selective reduction, or delivery, alone or in combination. RESULTS Median gestation at presentation and first treatment were both 21 weeks (range 14–34 and 15–34), and at delivery it was 29 weeks (range 16–40). Sixty-three percent of pregnancies (33 of 52) were at least stage III at presentation. Forty-five percent of pregnancies (22 of 49) progressed to a more advanced stage. Overall survival was 47% (47 of 100), with no difference between donor and recipient fetuses (40% [20 of 50] versus 54% [27 of 50] [χ2P < .5]). Survival rates were 58% (15 of 26), 60% (six of ten), 42% (20 of 48), 43% (six of 14), and 0% (none of two) for stages I–V, respectively, with no significant influence of stage at presentation on survival. Survival was poorer where stage increased, versus decreased (27% [12 of 44] versus 94% [17 of 18] χ2P < .001). Kaplan-Meier survival curves indicated that staging at presentation identified pregnancies at greater risk of earlier rather than later gestational perinatal loss. CONCLUSION The Quintero staging system did not distinguish good from bad outcome at presentation, and thus should be used with caution in guiding initial management of twin-twin transfusion syndrome. However, prognosis was influenced by a change in stage, and pregnancies progressing to higher stage disease were at increased risk of earlier perinatal loss. Staging may thus be more useful in monitoring disease progression. Quintero staging at presentation did not predict prognosis, although a change in stage did.

The role of Doppler blood flow velocimetry in multiple pregnancy

This article focuses on Doppler velocimetry in the assessment of fetal growth, placental insufficiency, management of intrauterine growth restriction, discordant growth and twin-twin transfusion syndrome. Problems specific to multiple pregnancy such as twin reversed arterial perfusion, cord entanglement and visualization of anastomoses are also reviewed. Doppler sonography seems to be superior to other tests for early detection of placental insufficiency during surveillance of multiple pregnancies. It is suggested that Doppler sonography helps to select multiple fetuses who are at higher than normal risk, and might reduce perinatal mortality and morbidity. Fetal echocardiography may provide an accurate assessment of cardiovascular adaptation to intertwin transfusion, early recognition of deterioration and evaluation of antenatal management. In the management of the twin-twin transfusion syndrome, Doppler echocardiography and Doppler ultrasound examination of the venous circulation appear to be useful tools for improved perinatal management. Doppler sonography is an important adjunct in monitoring high-risk multiple pregnancies.

The role of Doppler blood flow velocimetry in multiple pregnancy

ABSTRACTThis article focuses on Doppler velocimetry in the assessment of fetal growth, placental insufficiency, management of intrauterine growth restriction, discordant growth and twin-twin transfusion syndrome. Problems specific to multiple pregnancy such as twin reversed arterial perfusion, cord entanglement and visualization of anastomoses are also reviewed. Doppler sonography seems to be superior to other tests for early detection of placental insufficiency during surveillance of multiple pregnancies. It is suggested that Doppler sonography helps to select multiple fetuses who are at higher than normal risk, and might reduce perinatal mortality and morbidity. Fetal echocardiography may provide an accurate assessment of cardiovascular adaptation to intertwin transfusion, early recognition of deterioration and evaluation of antenatal management. In the management of the twin-twin transfusion syndrome, Doppler echocardiography and Doppler ultrasound examination of the venous circulation appear to be use...

Long term outcome of twin-twin transfusion syndrome

AIMSTo compare the perinatal mortality and morbidity of infants with twin-twin transfusion syndrome (TTTS) with those of gestation matched twin controls and to assess the neurodevelopmental outcome of surviving twins...