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American Academy of Pediatrics
Article

What—and Why—the Neonatologist Should Know About Twin-To-Twin Transfusion Syndrome

Joseph M. Bliss, Stephen R. Carr, Monique E. De Paepe and Francois I. Luks
NeoReviews January 2017, 18 (1) e22-e32; DOI: https://doi.org/10.1542/neo.18-1-e22
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Joseph M. Bliss
*The Fetal Treatment Program of New England and
the Divisions of †Neonatology,
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Stephen R. Carr
*The Fetal Treatment Program of New England and
‡Maternal-Fetal Medicine,
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Monique E. De Paepe
*The Fetal Treatment Program of New England and
§Pediatric and Developmental Pathology, and
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Francois I. Luks
*The Fetal Treatment Program of New England and
¶Pediatric Surgery, Alpert Medical School of Brown University, Providence, RI
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  • Abbreviations:
    AA,
    artery-to-artery
    AV,
    artery-to-vein
    BPD,
    bronchopulmonary dysplasia
    FLOC,
    fetoscopic laser occlusion of chorioangiopagous vessels
    MDA-PSV,
    middle cerebral artery peak systolic velocity
    MoM,
    multiples of the median
    PPROM,
    preterm premature rupture of membranes
    TAPS,
    twin anemia polycythemia sequence
    TTTS,
    twin-to-twin transfusion syndrome
    VV,
    vein-to-vein
  • Abstract

    Twin-to-twin transfusion syndrome results from unbalanced vascular anastomoses in monochorionic twin gestations. This condition, affecting 2,500 pregnancies each year in the United States, is most commonly identified with ultrasonography on the basis of unequal amniotic fluid volumes in a monochorionic, diamniotic pregnancy. Hemodynamic alterations in the syndrome lead to oligohydramnios, intrauterine growth restriction, and frequently, anemia in the “donor” twin while the “recipient” has polyhydramnios and polycythemia. In severe cases, both twins are at risk of developing hydrops fetalis and death. The Quintero staging system is widely used to characterize the features and severity of the disease in a given pregnancy and to guide decisions regarding therapy. The advent of endoscopic fetoplacental surgery, which affords the possibility of laser photocoagulation of connecting placental vessels and thereby separation of the twins’ circulation, has revolutionized the management of this condition and improved outcomes. The main risk of intervention is preterm premature rupture of membranes and subsequent preterm delivery of the twins. The outcomes for survivors of the syndrome are generally comparable to those of monochorionic, diamniotic twins in general and relate primarily to the degree of prematurity.

    • Copyright © 2017 by the American Academy of Pediatrics

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    What—and Why—the Neonatologist Should Know About Twin-To-Twin Transfusion Syndrome
    Joseph M. Bliss, Stephen R. Carr, Monique E. De Paepe, Francois I. Luks
    NeoReviews Jan 2017, 18 (1) e22-e32; DOI: 10.1542/neo.18-1-e22

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    What—and Why—the Neonatologist Should Know About Twin-To-Twin Transfusion Syndrome
    Joseph M. Bliss, Stephen R. Carr, Monique E. De Paepe, Francois I. Luks
    NeoReviews Jan 2017, 18 (1) e22-e32; DOI: 10.1542/neo.18-1-e22
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    • Article
      • Abstract
      • Education Gaps
      • Objectives
      • Introduction
      • Diagnosis Of Twin-To-Twin Transfusion Syndrome
      • Pathophysiology Of Twin-To-Twin Transfusion Syndrome
      • Staging Of Twin-To-Twin Transfusion Syndrome
      • Treatment Of Twin-To-Twin Transfusion Syndrome
      • Postoperative Results
      • Neonatal Outcomes
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