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Twin-to-Twin Transfusion Syndrome

  • Author: Terence Zach, MD; Chief Editor: Ronald M Ramus, MD  more...
 
Updated: Dec 30, 2015
 

Background

Twin-to-twin transfusion syndrome (TTTS) is the result of an intrauterine blood transfusion from one twin (donor) to another twin (recipient). TTTS only occurs in monozygotic (identical) twins with a monochorionic placenta. The donor twin is often smaller with a birth weight 20% less than the recipient's birth weight. The donor twin is often anemic and the recipient twin is often plethoric with hemoglobin differences greater than 5 g/dL.

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Pathophysiology

TTTS is the result of transfusion of blood from one fetal twin to another twin. The blood transfusion from the donor twin to the recipient twin occurs through placental vascular anastomoses. The most common vascular anastomosis is a deep, artery-to-vein anastomosis through a shared placental cotyledon.[1]

TTTS is a specific complication of monozygotic twins with monochorionic placentation. Monozygotic twins that have a dichorionic placentation are not at risk for TTTS. Monozygotic twins with monochorionic, diamniotic placentation or monochorionic, monoamniotic placentation are at risk for TTTS (see images below).[2]

Monozygotic twins with monochorionic, diamniotic p Monozygotic twins with monochorionic, diamniotic placentation.
Monozygotic twins with monochorionic, monoamniotic Monozygotic twins with monochorionic, monoamniotic placentation.

The clinical features of TTTS are the result of hypoperfusion of the donor twin and hyperperfusion of the recipient twin.

The donor twin becomes hypovolemic and oliguric or anuric. Oligohydramnios develops in the amniotic sac of the donor twin. Profound oligohydramnios can result in the stuck twin phenomenon in which the twin appears in a fixed position against the uterine wall. Ultrasonography typically fails to visualize the fetal bladder because of absent urine.

The recipient twin becomes hypervolemic and polyuric. Polyhydramnios develops in the amniotic sac of the recipient twin.

Either twin can develop hydrops fetalis. The donor twin can become hydropic because of anemia and high-output heart failure. The recipient twin can become hydropic because of hypervolemia. The recipient twin can also develop hypertension, hypertrophic cardiomegaly, disseminated intravascular coagulation, and hyperbilirubinemia after birth.

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Epidemiology

Frequency

United States

Monozygotic twins occur in 3-5 per 1000 pregnancies. Monozygotic twins can be monochorionic or dichorionic. Approximately 75% of monozygotic twins are monochorionic. Only monochorionic twins are at risk for TTTS. TTTS occurs in 5-38% of monochorionic twins.

Thus, TTTS only occurs in same sex, monozygotic twins with monochorionic placentation.

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Prognosis

Outcome is dependent upon gestational age at birth and whether intrauterine fetal brain ischemia occurred. The lower the gestational age at birth the greater the risk for long-standing neurologic or pulmonary sequelae. Catch-up growth occurs postnatally in most of the smaller donor twins.

Morbidity/mortality

Severe TTTS has a 60-100% fetal or neonatal mortality rate. Mild-to-moderate TTTS is frequently associated with premature delivery. Fetal demise of one twin is associated with neurologic sequelae in 25% of surviving twins. Fetal blood pressure instability can lead to brain ischemia in either the donor or recipient twin. Ischemia of the fetal brain can result in periventricular leukomalacia, porencephaly, microcephaly and cerebral palsy. The more premature the twins are at birth, the higher the incidence of postnatal morbidity and mortality.

In a review of 135 monochorionic twin pregnancies with single intrauterine death (sIUD), whether spontaneous or procedure related, O'Donoghue et al found that death of the co-twin followed in 22.9% of cases. In the pregnancies that continued after sIUD, the frequency of antenatally acquired brain injury in the co-twin was significantly lower after procedure-related than spontaneous sIUD: 2.6% versus 22.2% (P = 0.003). The investigators conclude that the risk of brain injury is reduced but not negated by procedures that restrict inter-twin transfusion.[3]

Complications

Neurologic sequelae

Intrauterine demise of one twin can result in neurologic sequelae in the surviving twin. Acute exsanguination of the surviving twin into the relaxed circulation of the deceased twin can result in intrauterine CNS ischemia.

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Contributor Information and Disclosures
Author

Terence Zach, MD Department Chair, Professor, Department of Pediatrics, Section of Newborn Medicine, Creighton University School of Medicine

Terence Zach, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

Coauthor(s)

Michael J Barsoom, MD, FACOG Director, Division of Maternal-Fetal Medicine, Alegent Health at Bergan Mercy Medical Center

Michael J Barsoom, MD, FACOG is a member of the following medical societies: American College of Obstetricians and Gynecologists, American Institute of Ultrasound in Medicine, Society for Maternal-Fetal Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Ronald M Ramus, MD Professor of Obstetrics and Gynecology, Director, Division of Maternal-Fetal Medicine, Virginia Commonwealth University School of Medicine

Ronald M Ramus, MD is a member of the following medical societies: American College of Obstetricians and Gynecologists, American Institute of Ultrasound in Medicine, Medical Society of Virginia, Society for Maternal-Fetal Medicine

Disclosure: Nothing to disclose.

Additional Contributors

Robert K Zurawin, MD Associate Professor, Chief, Section of Minimally Invasive Gynecologic Surgery, Department of Obstetrics and Gynecology, Baylor College of Medicine

Robert K Zurawin, MD is a member of the following medical societies: American College of Obstetricians and Gynecologists, American Society for Reproductive Medicine, Association of Professors of Gynecology and Obstetrics, Central Association of Obstetricians and Gynecologists, Society of Laparoendoscopic Surgeons, Texas Medical Association, AAGL, Harris County Medical Society, North American Society for Pediatric and Adolescent Gynecology

Disclosure: Received consulting fee from Ethicon for consulting; Received consulting fee from Bayer for consulting; Received consulting fee from Hologic for consulting.

References
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Monozygotic twins with monochorionic, diamniotic placentation.
Monozygotic twins with monochorionic, monoamniotic placentation.
Table. TTTS Staging System
Stage Oligohydramnios/



Polyhydramnios



Absent Urine in Donor Bladder Abnormal Doppler Blood Flows Hydrops Fetalis Fetal Demise
I + - - - -
II + + - - -
III + + + - -
IV + + + + -
V + + + + +
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