Monochorionic twins are monozygotic (identical) twins that arise from a single fertilized ovum and share a single placenta, or chorion, which develops from the trophoblast layer shortly after fertilization.[1] This shared placental structure results in vascular anastomoses that interconnect the fetal circulations, distinguishing monochorionic twins from dichorionic twins, who have separate placentas.[2] They account for approximately 70% of monozygotic twin pregnancies and about 20% of all twin gestations, with an overall incidence of roughly 1 in 250 natural conceptions.[3]The majority of monochorionic twins—around 95%—are monochorionic-diamniotic (MCDA), meaning they share one placenta but develop in separate amniotic sacs, formed when the embryonic splitting occurs between days 4 and 8 post-fertilization.[1] The rarer monochorionic-monoamniotic (MCMA) subtype, comprising about 5% of monochorionic cases, involves both twins sharing a single amniotic sac due to later splitting (days 8–13), which heightens risks such as umbilical cord entanglement.[1] Diagnosis typically occurs via ultrasound at 11–14 weeks' gestation, identifying the thin dividing membrane or T-sign indicative of a shared chorion.[1]Due to the interconnected circulations, monochorionic twins face significantly elevated risks of complications compared to dichorionic twins, with up to 15% developing serious placental vascular issues.[1] Key complications include twin-twin transfusion syndrome (TTTS), affecting 10–15% of cases and involving unbalanced blood flow between twins, leading to oligohydramnios-polyhydramnios sequence and potential fetal demise if untreated; selective fetal growth restriction (sFGR), where one twin receives inadequate placental share; and twin anemia-polycythemia sequence (TAPS), characterized by chronic intertwin transfusion imbalances.[2][1] Additionally, monochorionic pregnancies have a 5% rate of structural anomalies (often discordant, affecting one twin) and a fourfold increased risk of low birth weight, contributing to higher perinatal mortality (up to 7.5% loss of both twins) and morbidity, including preterm delivery before 32 weeks in 10–15% of cases.[1][4]Management of monochorionic twins requires specialized prenatal care, including biweekly ultrasound surveillance starting at 16 weeks to monitor for complications, with interventions like fetoscopic laser ablation for TTTS achieving 65–70% survival of both twins.[2][1] Elective delivery is typically recommended at 36–37 weeks to balance maturity with risks of intrauterine demise, emphasizing the need for referral to high-risk fetal medicine centers.[1] Advances in understanding these intertwin hemodynamics have improved outcomes, though long-term neurodevelopmental risks persist in survivors of severe complications.[2]
Definition and Formation
Definition
Monochorionic twins are monozygotic twins that share a single chorion and placenta, arising from the splitting of a single fertilized egg after the chorion has formed.[5] This shared placental structure distinguishes them from dichorionic twins, which have separate placentas and chorions, as well as from the subset of monozygotic twins that develop separate chorions (known as diamniotic-dichorionic).[6] All monochorionic twins are monozygotic by definition, though not all monozygotic twins are monochorionic.[7]These twins occur in approximately 0.3% of all pregnancies, representing about 20% of twin gestations overall.[8] Among monozygotic twin pregnancies, which have an incidence of roughly 1 in 250 to 400 pregnancies, 70-75% are monochorionic.[9][4]The concept of monochorionic twinning was first explored in early 20th-century embryology studies, with foundational descriptions emerging from analyses of placental morphology in animal models.[10] Modern incidence data derive from large-scale cohort studies tracking placental types in twin births.[11]
Embryological Development
Monochorionic twins develop from a single fertilized zygote that undergoes splitting during early embryogenesis, specifically after the formation of the chorion but before the amnion. Fertilization occurs when a sperm penetrates the ovum, forming the zygote on day 0. This zygote then progresses through cleavage stages, dividing into a multicellular morula by approximately days 3-4 post-fertilization. By day 5, the structure cavitates to form the blastocyst, comprising an outer layer of trophoblast cells surrounding the inner cell mass (ICM), which will give rise to the embryo proper.[12]The chorion, derived from the trophoblast layer, begins to form around day 5 with blastocyst formation and further develops upon implantation around days 6-7, establishing the extraembryonic membrane that interfaces with the maternal endometrium. If splitting of the inner cell mass occurs between days 4-8 post-fertilization—after the trophoblast has formed a single layer but while the ICM is still unified—it results in monochorionic diamniotic (MoDi) twins. At this stage, the ICM divides within the shared blastocyst, leading to two distinct embryonic discs that develop separate amniotic cavities while enveloped by a common chorion. This timing prevents the formation of individual chorions, as the trophoblast has already coalesced into a single layer.[13][14][15]In monochorionic twins, the unified chorion promotes the early integration of placental structures following implantation around days 6-7. As the trophoblast invades the uterine wall, chorionic villi emerge from both embryonic contributions, merging into a single placenta. Vascular connections, or anastomoses, between the twins' circulations arise within this shared placenta shortly thereafter, typically by the second week post-fertilization, as fetal capillaries vascularize the villi and interconnect across the chorionic plate. These early vascular links are a hallmark of monochorionic placentation, facilitating nutrient sharing but also predisposing to complications if unbalanced.[13][12]
Classification
Amniotic Types
Monochorionic twins are classified into subtypes based on the number of amniotic sacs they share, which arises from the timing of embryonic division following fertilization of a single zygote. This classification includes monochorionic-diamniotic (MoDi) twins, who have separate amniotic sacs, and monochorionic-monoamniotic (MoMo) twins, who share a single amniotic sac. The distinction is determined by when the inner cell mass splits during early embryogenesis, influencing the membrane architecture while maintaining a shared chorion and placenta.[16][17][18]Monochorionic-diamniotic (MoDi) twins represent the most common subtype, accounting for approximately 95% of all monochorionic pregnancies and about 70% of monozygotic twin pregnancies overall. These twins develop when the zygote splits between days 4 and 8 post-fertilization, at the blastocyst stage, resulting in a single chorionic sac but two distinct amniotic sacs and yolk sacs. This configuration allows for physical separation within the shared placenta, though vascular connections between the fetuses remain possible. The incidence of MoDi twins is estimated at around 1 in 400 pregnancies.[19][16][18][11]In contrast, monochorionic-monoamniotic (MoMo) twins are a rare variant, comprising about 5% of monochorionic pregnancies and thus approximately 3-4% of monozygotic twin pregnancies. They form from a later split of the embryonic disc, typically between days 8 and 13 post-fertilization, or from incomplete division, leading to a single shared amniotic sac in addition to the common placenta and chorion. This shared space heightens the potential for physical interactions between the fetuses, such as cord entanglement. The overall incidence of MoMo twins is approximately 1 in 10,000 pregnancies.[19][17][18][20]
Zygosity and Genetic Aspects
Monochorionic twins are nearly always monozygotic, originating from a single fertilized egg that splits after implantation, resulting in the sharing of a single placenta and 100% of their nuclear DNA.[21] This monozygosity accounts for the high genetic similarity observed in the vast majority of such twins, with splitting occurring between days 4 and 8 post-fertilization to form a monochorionic structure.[7]Rare exceptions exist where monochorionic twins are dizygotic, typically arising from mechanisms such as blood chimerism or early embryonic fusion, with fewer than 15 documented spontaneous cases worldwide, representing an incidence well below 1%.[22] These dizygotic monochorionic twins are confirmed through DNA testing, such as short tandem repeat (STR) or microsatellite analysis, which reveals distinct genetic profiles despite the shared chorion.[23][22]Genetically, monozygotic monochorionic twins exhibit high concordance for inherited traits and diseases, making them valuable for heritability studies that estimate genetic contributions to conditions like schizophrenia (50% concordance) and autoimmune disorders.[24] However, post-zygotic mutations can introduce somatic mosaicism, leading to subtle genetic differences between co-twins and potential discordance for certain phenotypes.[25]
Diagnosis
Prenatal Imaging Techniques
Prenatal imaging for monochorionic twins primarily relies on ultrasound techniques performed in the first trimester, between 10 and 14 weeks of gestation, to determine chorionicity and identify key placental features.[26] The T-sign, characterized by the thin intertwin membrane inserting directly into the fused placental mass without a visible ridge, indicates monochorionic diamniotic (MoDi) twins due to the shared chorion and fused membranes.[27] In contrast, the lambda sign, or twin-peak sign, shows a thickened membrane insertion forming a triangular projection into the intertwin space, signifying separate chorionic sacs typical of dichorionic twins.[28] These signs are visualized using transabdominal or transvaginal two-dimensional ultrasound, with the optimal window at 11-13 weeks when embryonic structures are clearly discernible.[29]Color Doppler ultrasound enhances the assessment by mapping vascular anastomoses within the shared placenta, which are present in nearly all monochorionic pregnancies.01066-6/fulltext) This technique employs power or high-definition color flow to detect superficial arterio-arterial or veno-venous anastomoses as bidirectional flow patterns across the intertwin membrane, while deeper arteriovenous anastomoses may appear as unidirectional shunts.[30] Real-time color Doppler sweeps along the chorionic plate allow for antenatal velocimetry, confirming the presence and directionality of these connections, which is crucial for risk stratification.Advanced imaging modalities complement standard ultrasound for more detailed placental and fetal evaluation. Three-dimensional (3D) ultrasound, often combined with power Doppler or tomographic ultrasoundimaging (TUI), provides volumetric rendering of the intertwin membrane thickness—typically less than 2 mm in monochorionic cases—and highlights anastomotic networks with greater spatial resolution than two-dimensional views. Magnetic resonance imaging (MRI), including diffusion-weighted and multicompartment techniques, enables non-invasive placental mapping by delineating vascular territories and tissue microstructure, particularly useful when ultrasound is limited.[31] Fetal echocardiography, performed as a targeted cardiac scan, detects early hemodynamic imbalances through metrics such as cardiothoracic ratio discrepancies or abnormal Doppler flows in the ductus venosus, which may signal unequal blood sharing.[32]The accuracy of ultrasound-based chorionicity determination reaches 95-99% when conducted by expert sonographers in the first trimester, with the T-sign and lambda sign serving as highly reliable predictors.[26] However, visibility can be compromised by placental location; anterior placentas often obscure membrane insertion sites due to acoustic shadowing from the uterine wall, potentially reducing diagnostic confidence compared to posterior positions.[33]
Diagnostic Timing and Challenges
The diagnosis of monochorionicity in twin pregnancies is optimally performed during the first trimester, specifically between 11 and 14 weeks of gestation, as part of the nuchal translucency scan, where ultrasound features such as the T-sign or lambda sign provide high accuracy in confirming a shared placenta.[26][34] Earlier assessment around 8 weeks is possible for determining amnionicity once amniotic sacs become visible, but it is less reliable for chorionicity due to incomplete membrane development and potential visualization limitations.[26]Several challenges can complicate the accurate diagnosis of monochorionicity via ultrasound, including overlapping placental masses that may mimic a single placenta in dichorionic twins or present as separate in up to 3% of monochorionic cases, leading to misclassification.[26] Maternal obesity often reduces ultrasound image clarity, impairing the detection of key chorionic features, while early twin demise can mask diagnostic signs by altering membrane visibility or amniotic fluid dynamics.[26][35] Overall, ultrasound-based chorionicity determination before 20 weeks gestation yields an error rate of approximately 6-10%, with false positives or negatives more common if performed later in pregnancy.[36] In cases of diagnostic uncertainty, pregnancies should be managed as monochorionic to ensure appropriate surveillance.[34]Following initial ultrasound diagnosis of monochorionicity, zygosity can be confirmed noninvasively using cell-free DNA testing, which analyzes single nucleotide polymorphisms to distinguish monozygotic from dizygotic twins with high accuracy, or invasively via amniocentesis sampling both sacs if genetic anomalies are suspected.[37][34] These methods are particularly useful when ultrasound signs alone, such as membrane insertion, require genetic corroboration for comprehensive risk assessment.[26]
Complications
Vascular and Placental Issues
Monochorionic twins share a single placenta, which features vascular anastomoses that interconnect the circulatory systems of both fetuses. These anastomoses are present in nearly all monochorionic placentas, with arteriovenous (AV) connections found in approximately 95% of uncomplicated cases, arterio-arterial (AA) in about 80%, and veno-venous (VV) in around 20%.[38] The AV anastomoses are deep and unidirectional, allowing blood to flow from the artery of one twin directly into the vein of the other, while AA and VV are superficial and bidirectional, facilitating potential equalization of blood pressure or volume.[39] Although these connections enable inter-twin blood exchange, imbalances in flow—often due to unequal placental sharing or dominant anastomoses—can lead to hemodynamic instability and complications.[40]One major consequence of unbalanced vascular flow is twin-to-twin transfusion syndrome (TTTS), which affects 8-15% of monochorionic diamniotic (MoDi) twin pregnancies.[41] In TTTS, net chronic transfusion occurs from a donor twin to a recipient twin via predominantly AV anastomoses, resulting in oligohydramnios and growth restriction in the donor alongside polyhydramnios and cardiac overload in the recipient.[42] The severity is classified using the Quintero staging system (stages I-V), which progresses from stage I (oligohydramnios/polyhydramnios with visible donor bladder) to stage V (demise of one or both twins), incorporating ultrasound findings such as absent donor bladder filling, abnormal Doppler flows, hydrops fetalis, and fetal death.[43]Another key issue is selective intrauterine growth restriction (sIUGR), occurring in 10-15% of monochorionic twin pregnancies due to unequal placental territory allocation despite shared vascular connections.[44] sIUGR is subclassified into types I-III based on umbilical artery Doppler patterns in the smaller twin: type I features positive end-diastolic flow and generally better prognosis; type II shows persistent absent or reversed end-diastolic flow, indicating high placental resistance; and type III exhibits intermittent absent or reversed flow, often linked to large AA anastomoses causing periodic flow fluctuations.[45] These Doppler-based distinctions highlight how vascular dynamics exacerbate growth discordance beyond mere placental share inequality.[46]
Type-Specific Risks
Monochorionic diamniotic (MoDi) twins face distinct risks primarily stemming from imbalances in placental vascular sharing, including twin-to-twin transfusion syndrome (TTTS), which affects 10-15% of such pregnancies and involves unidirectional blood flow leading to volume discordance between the twins.[41][47] Selective intrauterine growth restriction (sIUGR) occurs in 10-15% of MoDi twins, characterized by one twin's estimated fetal weight below the 10th percentile due to unequal placental territory allocation.[48][49] Additionally, preterm birth before 37 weeks complicates approximately 80% of MoDi pregnancies, often linked to these vascular discrepancies.[50] Twin anemia-polycythemia sequence (TAPS) arises in 3-5% of spontaneous MoDi cases and up to 16% following interventions for TTTS, resulting from slow chronic transfusion via tiny residual anastomoses.[51][42]In contrast, monochorionic monoamniotic (MoMo) twins, sharing both placenta and amniotic sac, exhibit heightened risks related to physical proximity and cord interactions, with cord entanglement observed in up to 70% of cases, potentially compressing vessels and compromising blood flow.[52] This entanglement contributes to approximately 50% of perinatal deaths in MoMo twins, often through acute hypoxia or ischemia.[52] Overall perinatal loss rates in MoMo pregnancies range from 20-50%, significantly exceeding the 10-15% seen in MoDi twins, due to these mechanical complications alongside shared vascular anastomoses.[53][54]Both MoDi and MoMo twins share the risk of acute intertwin transfusion or exsanguination following the demise of one twin, particularly in the second trimester, where co-twin loss occurs in 15-20% of cases due to sudden hemodynamic shifts across placental connections.[55][56]
Management and Treatment
Routine Monitoring Protocols
Routine monitoring protocols for monochorionic twin pregnancies emphasize intensive ultrasound surveillance to detect early signs of complications such as twin-to-twin transfusion syndrome (TTTS) and selective fetal growth restriction (sFGR). Following diagnosis, typically confirmed via prenatal imaging in the first trimester, standard care involves biweekly ultrasound examinations starting at 16 weeks of gestation. These scans assess fetal biometry for estimated fetal weight (EFW), amniotic fluid volume using the deepest vertical pocket (DVP) measurement, and Doppler velocimetry of the umbilical artery to evaluate blood flow. Monitoring may intensify to weekly if abnormalities such as growth discordance or abnormal Dopplers are detected.[26][57]Biweekly ultrasounds continue with focus on growth parameters, amniotic fluid levels, and Doppler studies, particularly middle cerebral artery peak systolic velocity (MCA-PSV) to screen for twin anemia-polycythemia sequence (TAPS). A weight discordance exceeding 20% between twins prompts heightened vigilance, often warranting referral to a maternal-fetal medicine specialist for further evaluation. The International Society of Ultrasound in Obstetrics and Gynecology (ISUOG) and the American College of Obstetricians and Gynecologists (ACOG) endorse this schedule to enable timely intervention while minimizing unnecessary procedures in uncomplicated cases.[26][57]Additional components include cervical length screening via transvaginal ultrasound at the mid-trimester anatomy scan (around 20 weeks) and repeated if risk factors for preterm birth are present, with a length below 25 mm indicating potential need for progesterone supplementation or cerclage. Non-stress tests (NSTs) are initiated weekly from 32 weeks to monitor fetal heart rate reactivity and wellbeing. Care is delivered through a multidisciplinary approach involving maternal-fetal medicine experts, per ACOG and ISUOG guidelines, to coordinate surveillance and manage high-risk features like abnormal Doppler findings. Hospital admission is considered for cases with significant discordance, abnormal fluid levels, or reversed end-diastolic flow on Doppler, transitioning to inpatientmonitoring with daily assessments.[26][57]
Interventions for Complications
Monochorionic twins are particularly susceptible to complications such as twin-to-twin transfusion syndrome (TTTS), selective intrauterine growth restriction (sIUGR), and twin anemia-polycythemia sequence (TAPS), necessitating targeted interventions to mitigate risks like fetal demise. For TTTS, the primary therapeutic approach is fetoscopic laser ablation of placental vascular anastomoses, often using the Solomon technique (SOLID procedure), which coagulates interfetal connections to equalize blood flow. This intervention, recommended for stages II-IV before 26 weeks' gestation, achieves dual twin survival rates of 50-70% and at least one survivor in 80-90% of cases, significantly outperforming historical untreated outcomes. Amnioreduction, involving serial removal of excess amniotic fluid from the recipient twin's sac, serves as an adjunct for maternal symptom relief in early or symptomatic cases but yields lower survival rates (around 60% for at least one twin) compared to laser therapy and is not first-line. Selective fetocide, typically via cord occlusion, is reserved for rare scenarios involving severe anomalies or discordant growth in one twin, with co-twin survival exceeding 80% when performed after 16 weeks.[58][59][60]In cases of sIUGR or TAPS, management is stratified by severity and gestational age to balance risks of progression against intervention complications. For sIUGR, classified into types I-III based on umbilical artery Doppler findings, expectant management with intensive surveillance is preferred for milder type I cases, while severe type II or III (with absent or reversed end-diastolic flow) may warrant cord occlusion techniques such as bipolar coagulation or radiofrequency ablation to protect the healthier twin, achieving co-twin survival rates of 70-85%. For TAPS, diagnosed via middle cerebral artery peak systolic velocity discrepancies, stage I cases before 32 weeks are managed expectantly with weekly monitoring, whereas stages II-V often require intrauterine blood transfusion for the anemic donor twin, partial exchange transfusion for the polycythemic recipient, or laser coagulation of tiny anastomoses, with perinatal survival around 85-90% post-intervention. These approaches, including cord occlusion methods, are individualized based on staging and fetal viability, prioritizing referral to specialized fetal centers.[58][61][62]Monochorionic-monoamniotic (MoMo) twins face unique cord entanglement risks, prompting specialized interventions focused on timely delivery and supportive measures. Elective cesarean section at 32-34 weeks is standard to preempt cord compression or prolapse, following inpatient monitoring from 24-26 weeks, which has contributed to overall perinatal survival rates improving to 70-90% in uncomplicated cases.[63] Amnioinfusion, the infusion of saline into the amniotic sac, is rarely employed for acute cord compression in MoMo pregnancies to alleviate oligohydramnios-related issues but carries risks of membrane rupture. Across monochorionic complications, these targeted interventions have collectively reduced mortality by approximately 50% compared to pre-fetal surgery eras, underscoring the value of multidisciplinary care.[64]
Prognosis and Outcomes
Short-Term Perinatal Results
Monochorionic twins exhibit higher rates of adverse short-term perinatal outcomes compared to dichorionic twins due to shared placental vascular anastomoses and associated complications. The overall perinatal mortality rate for monochorionic diamniotic (MoDi) twins ranges from 4% to 12%, reflecting variability across studies influenced by factors such as timely diagnosis and intervention.[65] In contrast, monochorionic monoamniotic (MoMo) twins face a markedly elevated perinatal mortality rate of 30% to 50%, primarily attributable to cord entanglement and other structural risks.[66]Prematurity, defined as delivery before 37 weeks gestation, occurs in approximately 80% of monochorionic twin pregnancies, contributing significantly to neonatal morbidity. Low birth weight, under 2500 grams, affects about 60% of these neonates, often linked to placental insufficiency and growth discordance. With specialized antenatal monitoring protocols, overall survival rates for monochorionic twins improve to 85% to 90%. Following the intrauterine demise of one twin, the surviving co-twin faces approximately a 15% risk of death overall, with lower risk if the loss occurs in the first trimester and higher risk later in gestation due to more developed intertwin vascular connections.[67][68][55]Delivery management in monochorionic twin pregnancies frequently involves cesarean section in 70% to 80% of cases, driven by complications such as twin-to-twin transfusion syndrome or growth restriction. For uncomplicated monochorionic pregnancies, the optimal timing for delivery is between 36 and 37 weeks to balance risks of stillbirth and neonatal respiratory issues.[69]
Long-Term Health Implications
Monochorionic twins face elevated risks of neurodevelopmental impairments extending beyond the perinatal period, primarily due to complications such as twin-to-twin transfusion syndrome (TTTS) and prematurity. Cerebral palsy occurs in approximately 5-10% of monochorionic twin survivors, with rates reaching up to 20% among those affected by TTTS prior to modern interventions, though recent advancements have lowered severe neurodevelopmental impairment (NDI) to 3-6%.[70][71][72] Cognitive delays are frequently linked to preterm birth, a common outcome in these pregnancies, affecting attention, learning, and executive function in affected children.[73] Follow-up studies indicate that 80-90% of survivors achieve normal development with early interventions like physical therapy and educational support, particularly in uncomplicated cases.[74]Cardiovascular and growth-related issues persist into childhood and adolescence for a subset of monochorionic twin survivors. Persistent growth discordance affects 10-15% of these twins, where the smaller twin at birth often maintains lower height, weight, and head circumference compared to the co-twin into childhood, stemming from selective fetal growth restriction.[75] This discordance is associated with an increased risk of hypertension in adulthood due to intrauterine programming from unequal placental sharing. Additionally, the genetic concordance in monozygotic monochorionic twins contributes to shared risks for neurodevelopmental conditions like autism spectrum disorder, with twin registry studies reporting concordance rates of 77-90% in affected pairs, underscoring a strong heritable component.[76][77]Post-2010 cohort studies demonstrate improved long-term outcomes following fetoscopic laser therapy for TTTS, which has reduced overall morbidity by approximately 30% through better equalization of fetal circulation and decreased preterm delivery rates.[78][79] This intervention lowers the incidence of severe NDI from historical highs of 13-20% to current levels of 5-8%, enabling more twins to reach developmental milestones without significant deficits.[80] Rare cases of blood chimerism, occurring in monochorionic dizygotic twins due to stem cell exchange, generally have benign long-term effects, with no established links to increased morbidity beyond potential immune modulation, though monitoring for autoimmune risks is recommended.[81][82]