Jc. Larroche et al., ABNORMAL CORTICAL PLATE (POLYMICROGYRIA), HETEROTOPIAS AND GRAIN DAMAGE IN MONOZYGOTIC TWINS, Biology of the neonate, 65(6), 1994, pp. 343-352
We report 5 cases of abnormal cortical plate (polymicrogyria or microg
yric-like pattern) and heterotopias associated with hypoxic-ischemic b
rain injuries in monochorionic diamniotic twin fetuses of respectively
22, 26, 28, 31, 32 weeks gestation. These fetuses belonged to a serie
s of 5 pairs of patients (10 cases) presenting with the characteristic
features of the twin-to-twin transfusion syndrome. Three of them (2 d
onors and 1 recipient) were macerated and the brains were not availabl
e for study. Two (most likely recipient twins) survived. In the remain
ing 5 fetuses (3 donors and 2 recipients) with neuropathological study
there were cortical plate abnormalities. In 2 cases, the dorter was d
ysmorphic and consisted of focal nodular distribution or vertical stri
pes of neurons. True polymicrogyria was focal in 2 cases and involved
almost the entire surface of the hemispheres in another one. Heterotop
ias of immature cells were found in 4 cases, either in the white matte
r or in the cortex or in both sites. There was a focal laminar necrosi
s only in 2 cases. The morphological pattern of the anomalies depends
on the time of occurrence of the insult and on its severity. These abn
ormalities, although similar to those already described in singleton f
etuses, illustrate the variety of cortical dysmorphia which may be ass
ociated with fetal hypoxic-ischemic injuries and emphasize the particu
lar vulnerability of the brain in monozygotic twins, whether it belong
s to the donor or the recipient.