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Diagnosis, Treatment and Outcome in Complicated Monochorionic Twins

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A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Obstetrics & Gynecology".

Deadline for manuscript submissions: closed (20 May 2019) | Viewed by 38951

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Special Issue Editors

Prof. Dr. Enrico Lopriore

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Department of Pediatrics, Division of Neonatology, Leiden University Medical Center, Leiden, The Netherlands
Interests: monochorionic twins; twin–twin transfusion syndrome; twin anaemia polycythaemia sequence; neonatal haematology; anaemia; thrombocytopenia
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Prof. Dr. Asma Khalil

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Fetal Medicine Unit, Department of Obstetrics & Gynaecology, St George's University Hospitals NHS Foundation Trust, University of London, Blackshaw Road, London SW17 0QT, UK
Interests: twin pregnancy; prenatal diagnosis; fetal therapy; twin-to-twin transfusion syndrome; stillbirth; hypertension in pregnancy; fetal growth restriction
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Prof. Liesbeth Lewi

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Guest Editor

Department of Gynaecology and Obstetrics, University of Leuven, Leuven, Belgium
Interests: twin pregnancy; monochorionic twin pregnancy; twin-to-twin transfusion syndrome; twin reversed arterial perfusion; monoamniotic twins; twin anaemia polycythaemia sequence
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Twin gestations occur in 2% of all pregnancies. Two-thirds of these twin gestations are dizygotic and one-third are monozygotic, thus leading to identical twins. Dizygotic twin gestations are always dichorionic and have two separate placentas. In contrast, the majority of monozygotic twin gestations are monochorionic in which both twins share their placenta. Monochorionic twin placentas are characterized by the invariable presence of vascular anastomoses connecting the two fetal circulations. Unbalanced blood transfusion through the vascular anastomoses can lead to severe fetal complications such as twin-twin transfusion syndrome (TTS), twin anemia-polycythemia sequence (TAPS) of twin reversed arterial perfusion (TRAP). In case of unequal placental sharing, selective fetal growth restriction (sFGR) can occur. Accurate and timely detection of these potentially devastating complications is of utmost importance, as well as optimal antenatal and postnatal management. Management options include the use of minimally invasive surgical techniques such as fetoscopic laser coagulation of the vascular anastomoses or selective feticide using cord coagulation with bipolar forceps or radiofrequency ablation. Significant technological improvement in the field of fetal therapy has led to an increase in perinatal survival in the past decades. Nevertheless, the management of the various forms of complications in monochorionic twins remains a challenge in perinatal medicine. Surviving twins are at risk of developing severe cerebral injury and long-term neurodevelopment sequelae. Continuing close collaboration between fetal medicine specialists and neonatologists is crucial to improve the care and outcome of complicated monochorionic twins.

Prof. Dr. Enrico Lopriore
Prof. Dr. Asma Khalil
Prof. Dr. Liesbeth Lewi
Guest Editor

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Keywords

Monochorionic twins
Twin-twin Transfusion Syndrome (TTS)
Twin Anemia-Polycythemia Sequence (TAPS)
Twin Reversed Arterial Perfusion (TRAP)
Selective Fetal Growth Restriction (sFGR)
Discordant anomaly
Fetal therapy
Clinical management
Long-term neurodevelopment outcome

Published Papers (9 papers)

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Editorial

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3 pages, 174 KiB

Open AccessEditorial

Monochorionic Twins: A Delicate Balance

by Enrico Lopriore, Liesbeth Lewi and Asma Khalil

J. Clin. Med. 2019, 8(10), 1711; https://doi.org/10.3390/jcm8101711 - 17 Oct 2019

Cited by 10 | Viewed by 2634

Abstract

Monochorionic (MC) twins are identical twins who share one placenta, with vascular anastomoses connecting the circulations of both fetuses [...] Full article

(This article belongs to the Special Issue Diagnosis, Treatment and Outcome in Complicated Monochorionic Twins)

Research

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10 pages, 433 KiB

Open AccessArticle

Long-Term Neurodevelopmental Outcome in Twin-to-Twin Transfusion Syndrome: Is there still Room for Improvement?

by Marjolijn S. Spruijt, Enrico Lopriore, Ratna N.G.B. Tan, Femke Slaghekke, Frans J.C.M. Klumper, Johanna M. Middeldorp, Monique C. Haak, Dick Oepkes, Monique Rijken and Jeanine M.M. van Klink

J. Clin. Med. 2019, 8(8), 1226; https://doi.org/10.3390/jcm8081226 - 15 Aug 2019

Cited by 21 | Viewed by 3377

Abstract

Despite many developments in its management, twin-to-twin transfusion syndrome (TTTS) remains an important risk factor for long-term neurodevelopmental impairment (NDI). Our objective was to compare the incidence of severe NDI in a recent cohort of TTTS survivors, treated with laser surgery from 2011 to 2014, with a previous cohort treated from 2008 to 2010. Neurological, cognitive, and motor development were assessed at two years of age. We determined risk factors associated with Bayley-III scores. Severe NDI occurred in 7/241 (3%) survivors in the new cohort compared to 10/169 (6%) in the previous cohort (p = 0.189). Disease-free survival (survival without severe impairment) did not significantly differ. Low birth weight and being small for gestational age (SGA) were independently associated with lower cognitive scores (both p < 0.01). Severe cerebral injury was related to decreased motor scores (B = −14.10; 95% CI −3.16, −25.04; p = 0.012). Children with severe NDI were born ≥32 weeks’ gestation in 53% of cases and had no evidence of cerebral injury on cranial ultrasound in 59% of cases. Our results suggest that improvement in outcome of TTTS has reached a plateau. Low birth weight, SGA, and cerebral injury are risk factors for poor neurodevelopmental outcome. Neither gestational age above 32 weeks nor the absence of cerebral injury preclude severe NDI. Full article

(This article belongs to the Special Issue Diagnosis, Treatment and Outcome in Complicated Monochorionic Twins)

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9 pages, 272 KiB

Open AccessArticle

Maternal Satisfaction with Healthcare after Perinatal Loss in Monochorionic Twin Pregnancy

by Mònica Druguet, Laura Nuño, Carlota Rodó, Silvia Arévalo, Elena Carreras and Juana Gómez-Benito

J. Clin. Med. 2019, 8(8), 1213; https://doi.org/10.3390/jcm8081213 - 14 Aug 2019

Cited by 4 | Viewed by 2338

Abstract

Introduction: The analysis of patients’ satisfaction with healthcare is recognised as being useful in the evaluation of health outcomes and perceived quality of care. Little is known, however, about how the psychological status of women who experience perinatal complications may affect their perceived satisfaction with care. Methods: We assessed healthcare satisfaction in 52 women who had undergone intrauterine surgery during a complicated monochorionic twin pregnancy and examined the influence that fetal loss and sociodemographic, clinical, and psychological factors had on the degree of satisfaction. Data were gathered in an individual interview and through the administration of the Medical Patient Satisfaction Questionnaire, Beck Depression Inventory, and State–Trait Anxiety Inventory. Relationships between variables were analysed using a chi-square test, Spearman’s rho, Student’s t test, and the Mann–Whitney U test, in accordance with the metric nature of the variables and the assumptions fulfilled. Results: Age and level of education were not associated with the degree of healthcare satisfaction. Negative but non-significant correlations were observed between the level of satisfaction and symptoms of anxiety and depression. Satisfaction with healthcare was high in the sample as a whole, although it was significantly higher among women who had not experienced fetal loss. There were no differences in satisfaction with services involving direct contact with medical staff, whereas satisfaction with indirect services was lower among women who had experienced perinatal loss. Conclusions: Due to the unique characteristics of this population, specialised care teams of both professional healthcare and indirect services are needed. Although administrative aspects of healthcare are regarded as being of secondary importance, this may not be the case with more vulnerable populations. Full article

(This article belongs to the Special Issue Diagnosis, Treatment and Outcome in Complicated Monochorionic Twins)

8 pages, 503 KiB

Open AccessArticle

Twin-Twin Transfusion Syndrome with Anemia-Polycythemia: Prevalence, Characteristics, and Outcome

by Lisanne S. A. Tollenaar, Femke Slaghekke, Jeanine M. M. van Klink, Sophie G. Groene, Johanna M. Middeldorp, Monique C. Haak, Frans J. C. M. Klumper, Dick Oepkes and Enrico Lopriore

J. Clin. Med. 2019, 8(8), 1129; https://doi.org/10.3390/jcm8081129 - 30 Jul 2019

Cited by 16 | Viewed by 4965

Abstract

The aim of this study was to estimate the prevalence of co-existing anemia-polycythemia (AP) in twin pregnancies with twin-twin transfusion syndrome (TTTS) prior to laser surgery, and to evaluate the characteristics and outcomes in TTTS twins with and without AP. All TTTS cases treated with laser between 2001 and 2019 were retrospectively reviewed for the presence of AP before surgery. AP was defined as delta middle cerebral artery–peak systolic velocity > 0.5 multiples of the median. The primary outcome was a composite of perinatal survival and severe neurodevelopmental impairment (NDI). Secondary outcomes included procedure-related characteristics, severe neonatal morbidity, and disease-free survival. In total, 66% (461/696) of TTTS twin pregnancies were eligible for analysis. AP was detected in 15% (70/461) of the TTTS twins prior to laser surgery. Gestational age at laser was higher in the TTTS+AP group compared to the TTTS-only group—21.0 weeks (interquartile rage (IQR): 18.8–24.0) versus 19.3 weeks (IQR: 17.3–21.9), respectively (p < 0.0001). Fewer placental anastomoses were detected in the TTTS+AP group than in the TTTS-only group—five (IQR: 4–6) versus six (IQR: 5–8), respectively (p < 0.0001). Perinatal survival was 77% (599/782) in the TTTS-only group and 83% (118/142) in the TTTS+AP group (p = 0.130). Severe NDI was 8% (28/370) in TTTS-only and 3% (2/74) in TTTS+AP. TTTS-only twins showed more severe neonatal morbidity than twins with TTTS+AP—23% (132/575) versus 11% (13/115), respectively (p = 0.005). Disease-free survival was lower in the TTTS-only group compared to the TTTS+AP group—62% (341/548) versus 73% (72/98), respectively (p = 0.046). Thus, AP complicates 15% of TTTS twins prior to laser. TTTS+AP twins show a different placental angioarchitecture, a later time of onset of the disease, and a more favorable outcome. Full article

(This article belongs to the Special Issue Diagnosis, Treatment and Outcome in Complicated Monochorionic Twins)

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12 pages, 1742 KiB

Open AccessArticle

Twin-Twin Transfusion Syndrome with and without Selective Fetal Growth Restriction Prior to Fetoscopic Laser Surgery: Short and Long-Term Outcome

by Sophie G. Groene, Lisanne S. A. Tollenaar, Jeanine M. M. van Klink, Monique C. Haak, Frans J. C. M. Klumper, Johanna M. Middeldorp, Dick Oepkes, Femke Slaghekke and Enrico Lopriore

J. Clin. Med. 2019, 8(7), 969; https://doi.org/10.3390/jcm8070969 - 03 Jul 2019

Cited by 29 | Viewed by 3763

Abstract

As twin-twin transfusion syndrome (TTTS) and selective fetal growth restriction (sFGR) are both prevalent complications of monochorionic (MC) twin pregnancies, its coexistence is not uncommon. The aim of this study is to evaluate the short and long-term outcome in TTTS with and without sFGR prior to fetoscopic laser coagulation. All TTTS cases treated with laser surgery at our center between 2001–2019 were retrospectively reviewed for the presence of sFGR, defined as an estimated fetal weight (EFW) <10th centile. We compared two groups: TTTS-only and TTTS + sFGR. Primary outcomes were perinatal survival and long-term severe neurodevelopmental impairment (NDI). Of the 527 pregnancies eligible for analysis, 40.8% (n = 215) were categorized as TTTS-only and 59.2% (n = 312) as TTTS + sFGR. Quintero stage at presentation was higher in the TTTS + sFGR group compared to the TTTS-only group (57% compared to 44% stage III). Separate analysis of donors showed significantly lower perinatal survival for donors in the TTTS + sFGR group (72% (224/311) compared to 81% (173/215), p = 0.027). Severe NDI at follow-up in long-term survivors in the TTTS-only and TTTS + sFGR group was present in 7% (13/198) and 9% (27/299), respectively (p = 0.385). Both sFGR (OR 1.5;95% CI 1.1–2.0, p = 0.013) and lower gestational age at laser (OR 1.1;95% CI 1.0–1.1, p = 0.001) were independently associated with decreased perinatal survival. Thus, sFGR prior to laser surgery is associated with a more severe initial presentation and decreased donor perinatal survival. The long-term outcome was not affected. Full article

(This article belongs to the Special Issue Diagnosis, Treatment and Outcome in Complicated Monochorionic Twins)

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12 pages, 1150 KiB

Open AccessArticle

Maternal Complications and Hemodynamic Changes Following Intrauterine Interventions for Twin-to-Twin Transfusion Syndrome in Monochorionic Diamniotic Twin Pregnancies

by Patrick Greimel, Angela Zenz, Bence Csapó, Martin Haeusler, Uwe Lang and Philipp Klaritsch

J. Clin. Med. 2019, 8(5), 605; https://doi.org/10.3390/jcm8050605 - 02 May 2019

Cited by 6 | Viewed by 5170

Abstract

Twin-to-twin transfusion syndrome (TTTS) is a challenging complication in monochorionic diamniotic (MCDA) twins. Intrauterine interventions, such as fetoscopic laser ablation and cord occlusion followed by amniodrainage, are established treatments. Little is known about maternal complications and hemodynamics following these interventions. We performed a retrospective analysis of maternal procedure-related complications and the impact of such procedures on maternal hemodynamics and blood characteristics. Within the study period, 100 women with severe TTTS treated by fetoscopic laser ablation (FLA) or cord occlusion (CO) were identified. Clinically relevant maternal complications were reported in four (4%) cases. There was a significant decrease in hemoglobin, hematocrit, and albumin between admission and postoperative measurements (all p < 0.001). Systolic and diastolic blood pressure, as well as maternal heart rate, decreased from time of skin suture to postoperative measurements (all p < 0.001). Within a 24 h interval, there was a positive correlation between hematocrit (Spearman’s rho 0.325; p = 0.003), hemoglobin (Spearman’s rho 0.379; p < 0.001), and albumin (Spearman’s rho 0.360; p = 0.027), and the amount of amniodrainage during the intervention. Maternal procedure-related complications are relatively rare. Significant hemodynamic alterations and maternal hemodilution are common clinical findings following intrauterine interventions. Full article

(This article belongs to the Special Issue Diagnosis, Treatment and Outcome in Complicated Monochorionic Twins)

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Review

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16 pages, 485 KiB

Open AccessReview

Dispelling Myths about Antenatal TAPS: A Call for Action for Routine MCA-PSV Doppler Screening in the United States

by Lauren Nicholas, Rebecca Fischbein, Julie Aultman and Stephanie Ernst-Milner

J. Clin. Med. 2019, 8(7), 977; https://doi.org/10.3390/jcm8070977 - 04 Jul 2019

Cited by 7 | Viewed by 8759

Abstract

In the United States, routine middle cerebral artery peak systolic velocity (MCA-PSV) Doppler screening for the detection of antenatal twin anemia-polycythemia sequence (TAPS) is not recommended. The current and only national clinical guideline from the highly-influential Society for Maternal-Fetal Medicine states that, “There is no evidence that monitoring for TAPS with MCA PSV Doppler at any time, including > 26 weeks, improves outcomes, so that this additional screening cannot be recommended at this time.” We argue this recommendation has disproportionate influence on patients and the care they are offered and receive. We use current evidence to highlight and dispel pervasive myths surrounding antenatal TAPS and the value of routine MCA-PSV screening. An ethical framework that illustrates the importance of giving patients the opportunity for routine screening is presented. Findings demonstrate that: (1) both spontaneous and post-laser TAPS is a serious, potentially life-threatening complication, (2) treatment for TAPS is effective and includes expectant management, intrauterine transfusion (IUT), or surgery, (3) and routine MCA-PSV, which has satisfactory diagnostic accuracy, is currently the only way to provide early detection of TAPS. We conclude that routine TAPS screening is a medically proven valuable resource that should be offered to patients in need and to the clinicians who are trying to act toward their benefit. Full article

(This article belongs to the Special Issue Diagnosis, Treatment and Outcome in Complicated Monochorionic Twins)

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10 pages, 369 KiB

Open AccessReview

The Impact of Selective Fetal Growth Restriction or Birth Weight Discordance on Long-Term Neurodevelopment in Monochorionic Twins: A Systematic Literature Review

by Sophie G. Groene, Lisanne S.A. Tollenaar, Dick Oepkes, Enrico Lopriore and Jeanine M.M. van Klink

J. Clin. Med. 2019, 8(7), 944; https://doi.org/10.3390/jcm8070944 - 28 Jun 2019

Cited by 29 | Viewed by 3227

Abstract

The aim of this review was to assess the impact of selective fetal growth restriction (sFGR) and/or birth weight discordance (BWD) on long-term neurodevelopment in monochorionic (MC) twins. Five out of 28 articles assessed for eligibility were included. One article concluded that the incidence of long-term neurodevelopmental impairment (NDI) was higher in BWD MC twins (11/26, 42%) than in BWD dichorionic (DC) (5/38, 13%) and concordant MC twins (6/71, 8%). BWD MC twins had a 6-fold higher risk of cerebral palsy compared to DC twins (5/26, 19% vs. 1/40, 3%, p < 0.05). Another article described a linear relationship between birth weight and verbal IQ scores, demonstrating a 13-point difference for a 1000 gram BWD between the twins, with a disadvantage for the smaller twin (p < 0.0001). Three articles analyzing within-pair differences showed that the smaller twin more frequently demonstrated mild NDI (6/80, 8% vs. 1/111, 1%) and lower developmental test scores (up to 5.3 points) as opposed to its larger co-twin. Although these results suggest that MC twins with sFGR/BWD are at increased risk of long-term NDI as compared to BWD DC or concordant MC twins, with a within-pair disadvantage for the smaller twin, the overall level of evidence is of moderate quality. As only five articles with a high degree of heterogeneity were available, our review mainly demonstrates the current lack of knowledge of the long-term outcomes of MC twins with sFGR/BWD. Insight into long-term outcomes will lead to improved prognostics, which are essential in parent counseling and crucial in the process of forming a management protocol specifically for twins with sFGR to optimally monitor and support their development. Full article

(This article belongs to the Special Issue Diagnosis, Treatment and Outcome in Complicated Monochorionic Twins)

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10 pages, 1123 KiB

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Congenital Heart Defects in Monochorionic Twins: A Systematic Review and Meta-Analysis

by Manon Gijtenbeek, Maryam R. Shirzada, Arend D. J. Ten Harkel, Dick Oepkes and Monique C. Haak

J. Clin. Med. 2019, 8(6), 902; https://doi.org/10.3390/jcm8060902 - 24 Jun 2019

Cited by 28 | Viewed by 3941

Abstract

Monochorionic (MC) twins are at an increased risk of developing congenital heart defects (CHDs) compared to singletons and dichorionic twins. The development of acquired CHDs in this specific group of twins is associated with twin–twin transfusion syndrome (TTTS). We performed a systematic review and meta-analysis to provide an overview of the reported birth prevalence of CHDs in liveborn MC twins with and without TTTS. Twelve studies were included in this review. Compared to the reference population, MC twins were 6.3 times more likely to be born with a CHD (59.3 per 1000 liveborn twins; relative risk (RR) 6.3; 95% confidence interval (CI): 4.4–9.1), and TTTS twins had a 12-fold increased risk of having a CHD at birth (87.3 per 1000 live births; RR 12.4, 95% CI: 8.6–17.8). The increased incidence of CHDs can mainly be attributed to the risk of right ventricular outflow tract obstruction (35/1000 TTTS twin live births vs. 0.5/1000 singleton live births). We recommend an expert fetal echocardiogram in all MC twins, follow-up scans in the event of TTTS, and a postnatal cardiac evaluation in all TTTS survivors. Full article

(This article belongs to the Special Issue Diagnosis, Treatment and Outcome in Complicated Monochorionic Twins)

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