Vanishing Twin Syndrome 

Updated: May 10, 2016
Author: Ann L Anderson-Berry, MD, PhD; Chief Editor: Christine Isaacs, MD 

Overview

Background

Vanishing twin syndrome, first described by Stoeckel in 1945, is the identification of a multifetal gestation with subsequent disappearance of one or more fetuses. The rate of multifetal gestation at conception is higher than the incidence noted at birth.[1] Vanishing twin syndrome has been diagnosed more frequently since the use of ultrasonography in early pregnancy.[2, 3] In vitro fertilization techniques have improved the understanding of vanishing twin syndrome because these pregnancies are closely monitored, and the number of implanted fertilized eggs is known.[4]

In vanishing twin syndrome, there may be complete reabsorption of a fetus, formation of a fetus papyraceus (ie, a "mummified" or compressed fetus), or development of a subtle abnormality on the placenta such as a cyst, subchorionic fibrin, or amorphous material.[5]

The timing of this event significantly affects the outcome of the viable twin and the maternal complications. For example, if the event occurs during the second half of pregnancy, the fetus could develop cerebral palsy or cutis aplasia, and the mother could develop preterm labor, infection, puerperal hemorrhage, consumptive coagulopathy, or obstruction of labor.[6, 7]

Pathophysiology

Abnormalities that result in the disappearance of a fetus usually appear to be present from early in development rather than occurring from an acute insult. Placental or fetal analysis frequently reveals chromosome abnormalities. These abnormalities include diploidy, triploidy, and alternate sex chromosome results on placental pathology, skin biopsies, and chorionic villus sampling.[8, 9, 10, 11] Study findings of the viable twin chromosomes in these reports are normal. Therefore, it is thought that the vanished twin had a chromosomal abnormality resulting in disappearance.

Epidemiology

Frequency

United States

The frequency of multiple gestations is 3.3-5.4% at 8 weeks' gestation.[1] Vanishing twin syndrome occurs in 21-30% of multifetal gestation.[4]

Research from a European series of pregnancies associated with assisted reproductive technology (ART) show that 10-15% of singleton births were initially twin gestations.

International

International prevalence is similar to that of the United States.

Mortality/Morbidity

Maternal first-trimester morbidity

Morbidity when vanishing twin syndrome occurs during the first trimester is limited. The mother is most likely to develop mild vaginal bleeding and cramping. If the event occurs later in the first trimester, morbidity may be similar to that of the second and third trimesters.

Maternal morbidity in the second and third trimesters

Maternal complications include premature labor, infection from a retained fetus, severe puerperal hemorrhage, consumptive coagulopathy, and obstruction of labor by a low-lying fetus papyraceus causing dystocia and leading to a cesarean delivery.[6]

The diagnosis of vanishing twin in a pregnancy significantly increases both preterm (< 37 gestational weeks) and very preterm (< 32 gestational weeks) births.[6]

Fetal morbidity and mortality

In addition to loss of a twin, the surviving fetus has an increased risk of cerebral palsy, particularly if vanishing twin syndrome occurred during the second half of pregnancy.[12]

Other forms of morbidity reported in the surviving twin are aplasia cutis or areas of skin necrosis. In twins connected through vascular connection by placental anastomoses, temporary hypotension in the surviving twin at the time of fetal demise of the vanishing twin leads to poor perfusion and skin necrosis.[13]

Complications

Prematurity

Singleton pregnancies that were reduced to singleton because of vanishing twin syndrome are more likely to be delivered preterm (35.1 vs 38.2 wk, P =0001). These rates of preterm delivery are similar to twin IVF pregnancies (23%).[14] In one series, singletons remaining after a vanishing twin event were associated with very premature birth (< 32 wk estimated gestational age).[15]

Low birth weight/small for gestational age

Singleton pregnancies that were reduced to singleton because of vanishing twin syndrome are at higher risk for growth restriction (32.6% vs 16.3% of control IVF singleton pregnancies).[16] Risks associated with intrauterine growth restriction include increased morbidity and mortality in the neonatal period and increased rates of hospitalization in the neonatal intensive care unit.

Cerebral palsy

Researchers proposed that vanishing twin syndrome could result in spastic cerebral palsy in the remaining twin. (Cerebral palsy is the most common hypothesized pathological clinical sequela in the viable twin.)[12]

A possible mechanism is the transfusion of thromboplastic proteins from the vanishing twin to the surviving twin, leading to disseminated intravascular coagulation (DIC). Researchers hypothesize that DIC results from reverse blood flow from the macerated twin to the viable twin, thus carrying thromboplastins into the circulation. This large thromboplastin load is hypothesized to lead to a state of DIC in the viable twin, which then leads to intrauterine central nervous system damage.[17]

Another proposed mechanism for central nervous system damage involves large amounts of blood loss from the surviving twin to the low resistance system of the vanishing twin through placental anastomoses. This transfusion could cause wide fluctuation in intravascular pressures, leading to intraventricular hemorrhage that results in cerebral palsy.[18]

Cerebral impairment in the survivor has also been linked to confirmed cases of vanishing twin with impairment on the Griffiths Mental and Developmental Scales and Optimality score (relative risk 6.1 p=0.03).[18]

Associated congenital anomalies

A link in children with cerebral palsy and other congenital anomalies is possible. In one series, the relative risk for congenital malformations, including microcephaly, isolated hydrocephaly, eye, cleft lip/palate, and cardiac anomalies, increased over baseline from relative risk 3.1-116 (95% CI, 1.9-4.8 to 84 to 162.3; P< 0.01 to P< 0.0001) depending on the specific defect.[19] These anomalies are postulated to be due in part to perturbations in fetal flow in the surviving twin at the time of loss of the vanishing twin.[19]

Cutis aplasia

The mechanism of development of cutis aplasia is most likely vascular. There is thought to be decreased perfusion to the affected area at the time of demise of the vanished twin. Most case reports include a fetus papyraceous as the vanished twin.[13, 20]

Other complications

Preterm birth of the surviving twin has been described with a 2.3-fold increased risk and a mortality rate that is 3-fold increased.[21, 22]

The surviving singleton infant after a twin vanishes has been shown to have a lower birth weight (mean difference, 368 g) than singleton pregnancies.[23]

Race

No predilection for any race has been reported.

Sex

No predilection for either sex has been reported in the vanishing twin.

Age

Researchers report more cases in women older than 30 years. Advanced maternal age is also a recognized risk factor for fetal and placental chromosome abnormalities.

 

Presentation

History

Problems usually develop during the first trimester of pregnancy. The most common presenting complaints include bleeding, uterine cramps, and pelvic pain.[24]

Physical

Vaginal bleeding may be observed on pelvic examination.[24]

Causes

The cause of vanishing twin syndrome is frequently unknown; however, this condition occurs more often in fetuses with genetic or chromosomal abnormalities.[5] Improper cord implantation may also play a role in some cases.

 

DDx

Diagnostic Considerations

Important considerations

Do not perform dilation and curettage until it is certain that no viable fetus remains.

If chorionic villus sampling is performed during a multifetal gestation, be aware that a mosaic placenta may be present. A viable fetus with normal chromosomes may be supported by the placenta of a vanished twin with abnormal chromosomes.[25]

The full implications of fertility treatments that involve implantation of multiple eggs with reabsorption of several during the course of the pregnancy are unknown.[26]

Special concerns

Parents who have experienced a loss from vanishing twin syndrome often must deal with the death of one child while caring for another who is preterm or may have serious medical complications. Little is written on the long-term effects on the family.[27] One study examined parental attitudes on the psychological vulnerability of remaining singleton children after vanishing of a cotwin. This study found that, while parents perceived that these children had more motor difficulties, they thought of them as less vulnerable than controls.[28]

Other problems to be considered

The following should also be considered in cases of suspected vanishing twin syndrome:

  • Threatened abortion

  • Decidual reaction on sonogram

  • Amniotic cavity observed on sonogram as a second fetus

  • Chorionic sac observed on sonogram as a second fetus

  • Yolk sac or extraembryonic coelom observed on sonogram as a second fetus

  • Subchorionic hemorrhage or hydropic change in chorionic villi observed on sonogram as a second fetus[5]

 

Workup

Laboratory Studies

Recent vanishing twin has been shown to increase pregnancy associated plasma protein-A (PAPP-A) and free beta-hCG. This may affect risk assessment for aneuploidy in the surviving fetus.[29]

Alpha-fetoprotein levels are elevated compared with values at similar junctures in both a singleton pregnancy and a normal twin pregnancy.[30]

The rate of rise of beta-human chorionic gonadotropin is slower than in a normal twin pregnancy.[31]

If vanishing twin syndrome is diagnosed at the time of nuchal translucency ultrasound, biochemical screening markers may not be as precise as in singleton-assisted reproductive technique pregnancies.[32]

The use of biochemical markers may be indicated if, at the time of nuchal translucency, a vanishing twin is confirmed to have an empty gestational sac; however, if a dead fetus remains, biochemical markers are not accurate for screening.[33]

Other tests

Amniocentesis after diagnosis of a vanishing twin by prior ultrasonography has been reported to detect an XY cell by both FISH and real-time PCR in the sustained XX 20 weeks' gestation pregnancy. Take caution about interpretation of amniocentesis results because a vanishing twin fetus could lead to false-positive results.[25]

Imaging Studies

Ultrasonography is used to confirm the diagnosis of early twin pregnancy. Follow-up ultrasonography reveals the pregnancy loss (vanishing twin).[12, 34] In addition, after diagnosing first-trimester bleeding, using ultrasonography before dilation and curettage is important. This ensures that bleeding does not signal the loss of just one fetus.

Procedures

Chorionic villus sampling may be helpful if the placenta has a mosaic makeup and there is a singleton at birth.

Histologic Findings

Obtain histological samples of the placenta at birth since they may be the only evidence of vanishing twin syndrome with a reabsorbed fetus.[3, 8, 11]

 

Treatment

Medical Care

Uncomplicated vanishing twin syndrome requires no special medical care.

If a fetus papyraceus remains, the pregnancy should be followed closely with serial ultrasonographic evaluation of the live fetus. Risks include premature labor, obstruction of labor, or death of the surviving fetus due to placental abruption or chorioamnionitis.[35] This fetus is also at risk for low birth weight and small for gestational age (SGA) with increasing risk in the surviving twin for vanishing twin occurring later in gestation.[36, 37]

The provider should watch carefully for infection and consumptive coagulopathy.

Instruct pregnant women to seek medical care for vaginal bleeding, cramping, and pelvic pain.

The viable twin should receive specialized medical care as indicated by initial physical examination and subsequent mental and physical development.

Anand et al reported in 2007 that surviving cotwins had poorer scores on the Griffiths Mental and Development Scales when compared with singleton pregnancies.[38]

Transfer

Evaluate pregnant women with vaginal bleeding at a site with adequate ultrasonographic capabilities.

Surgical Care

Only perform dilation and curettage after ultrasonographic confirmation that a viable embryo or fetus does not exist.[25]