Abnormal cortical plate (polymicrogyria), heterotopias and brain damage in monozygous twins. 1994

J C Larroche, and N Girard, and F Narcy, and C Fallet
INSERM U 29, Hôpital Port-Royal, Paris, France.

We report 5 cases of abnormal cortical plate (polymicrogyria or microgyric-like pattern) and heterotopias associated with hypoxic-ischemic brain injuries in monochorionic diamniotic twin fetuses of respectively 22, 26, 28, 31, 32 weeks gestation. These fetuses belonged to a series of 5 pairs of patients (10 cases) presenting with the characteristic features of the twin-to-twin transfusion syndrome. Three of them (2 donors and 1 recipient) were macerated and the brains were not available for study. Two (most likely recipient twins) survived. In the remaining 5 fetuses (3 donors and 2 recipients) with neuropathological study there were cortical plate abnormalities. In 2 cases, the cortex was dysmorphic and consisted of focal nodular distribution or vertical stripes of neurons. True polymicrogyria was focal in 2 cases and involved almost the entire surface of the hemispheres in another one. Heterotopias of immature cells were found in 4 cases, either in the white matter or in the cortex or in both sites. There was a focal laminar necrosis only in 2 cases. The morphological pattern of the anomalies depends on the time of occurrence of the insult and on its severity. These abnormalities, although similar to those already described in singleton fetuses, illustrate the variety of cortical dysmorphia which may be associated with fetal hypoxic-ischemic injuries and emphasize the particular vulnerability of the brain in monozygotic twins, whether it belongs to the donor or the recipient.

UI MeSH Term Description Entries
D007231 Infant, Newborn An infant during the first 28 days after birth. Neonate,Newborns,Infants, Newborn,Neonates,Newborn,Newborn Infant,Newborn Infants
D007234 Infant, Premature A human infant born before 37 weeks of GESTATION. Neonatal Prematurity,Premature Infants,Preterm Infants,Infant, Preterm,Infants, Premature,Infants, Preterm,Premature Infant,Prematurity, Neonatal,Preterm Infant
D011247 Pregnancy The status during which female mammals carry their developing young (EMBRYOS or FETUSES) in utero before birth, beginning from FERTILIZATION to BIRTH. Gestation,Pregnancies
D001921 Brain The part of CENTRAL NERVOUS SYSTEM that is contained within the skull (CRANIUM). Arising from the NEURAL TUBE, the embryonic brain is comprised of three major parts including PROSENCEPHALON (the forebrain); MESENCEPHALON (the midbrain); and RHOMBENCEPHALON (the hindbrain). The developed brain consists of CEREBRUM; CEREBELLUM; and other structures in the BRAIN STEM. Encephalon
D005260 Female Females
D005311 Fetal Hypoxia Deficient oxygenation of FETAL BLOOD. Anoxia, Fetal,Fetal Anoxia,Hypoxia, Fetal
D005330 Fetofetal Transfusion Passage of blood from one fetus to another via an arteriovenous communication or other shunt, in a monozygotic twin pregnancy. It results in anemia in one twin and polycythemia in the other. (Lee et al., Wintrobe's Clinical Hematology, 9th ed, p737-8) Cross-Transfusion, Intrauterine,Intrauterine Cross-Transfusion,Twin Transfusion,Twin Transfusion Syndrome,Twin-to-Twin Transfusion,Fetal Transfusion Syndrome,Fetofetal Transfusion Syndrome,Twin Twin Transfusion Syndrome,Twin-to-Twin Transfusion Syndrome,Cross Transfusion, Intrauterine,Fetal Transfusion Syndromes,Fetofetal Transfusion Syndromes,Fetofetal Transfusions,Intrauterine Cross Transfusion,Intrauterine Cross-Transfusions,Syndrome, Fetal Transfusion,Syndrome, Fetofetal Transfusion,Syndrome, Twin Transfusion,Syndrome, Twin-to-Twin Transfusion,Transfusion Syndrome, Fetal,Transfusion Syndrome, Fetofetal,Transfusion Syndrome, Twin,Transfusion Syndrome, Twin-to-Twin,Transfusion, Fetofetal,Transfusion, Twin,Transfusion, Twin-to-Twin,Twin Transfusion Syndromes,Twin Transfusions,Twin to Twin Transfusion,Twin to Twin Transfusion Syndrome,Twin-to-Twin Transfusion Syndromes,Twin-to-Twin Transfusions
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000015 Abnormalities, Multiple Congenital abnormalities that affect more than one organ or body structure. Multiple Abnormalities
D014430 Twins, Monozygotic Two off-spring from the same PREGNANCY. They are from a single fertilized OVUM that split into two EMBRYOS. Such twins are usually genetically identical and of the same sex. Twins, Identical,Identical Twin,Identical Twins,Monozygotic Twin,Monozygotic Twins,Twin, Identical,Twin, Monozygotic

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