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Diagnostic ultrasound ( PDFDrive )

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CHAPTER 45 Neonatal and Infant Brain Imaging 1537

A

B

C

D

FIG. 45.31 Schizencephaly. (A) Coronal sonogram and (B) coronal magnetic resonance image of open-lip schizencephaly show bilateral clefts

(c) with wide openings to the ventricular (v) system. (C) Sonogram and (D) computed tomography image show closed-lip schizencephaly with

calciication from in utero infection.

have blindness, thought to be related to absence of the septum

pellucidum and associated optic nerve hypoplasia. his is thought

to be an acquired septo-optic dysplasia. here are genetic cases

from the EMX2 homeobox gene 31 and other patients with possible

in utero injury during the second trimester. Cytomegalovirus

(CMV) has been reported to cause schizencephaly as would be

expected for those maternal infections that occur in the irst

trimester at the time of initial brain development. 68,69 In patients

with unilateral involvement, recent reports show reorganization

of the motor area so that the unafected hemisphere takes over

function. 70

Lissencephaly

Complete lack of sulcal formation caused by lissencephaly

can be recognized on ultrasound when the sulcal pattern

does not match that expected for gestational age. Hourglass

or “igure 8” has been described as typical of the cortical

surface. he sylvian issures are oten open owing to failure

of the opercularization process. 30-33 he arrested myelination

results in only four layers of cortex and a smooth brain.

Because these patients typically have microcephaly, it is

important to study these patients very carefully. Although

lissencephaly is associated with many genetic syndromes,

CMV and other infections have also been reported to cause

lissencephaly.

DESTRUCTIVE LESIONS

Porencephalic Cyst

Before 26 weeks’ gestation, focal brain destruction will typically

heal with dysplastic gray matter and result in schizencephaly,

usually associated with migrational defects. 42 Ater 26 weeks’

gestation, a porencephalic cyst will develop in an area of normally

developed brain that has been damaged and heals with scarring

because of a lining of gliotic white matter. By deinition, porencephalic

cysts always connect with the ventricular system but

do not extend to the surface cortex. hey usually occur ater

birth, secondary to intraparenchymal hemorrhage (IPH), infection

(focal vasculitis, abscess), or trauma.

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