Chapter 24: Fetal Head and Brain - pathology

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vein of galen aneurysm

CF: CHF SF: anechoic mass within midline of brain that contains turbulent arterial and venous flow when interrogated with pulsed and color doppler, fetal hydrops, cardiomegaly (caused by cardiac overload)

acrania (anencephaly)

CF: elevated MSAFP SF: absent cranial vault, some cerebral tissue, "froglike" facies or bulging eyes

arnold-chiari II malformation

CF: elevated MSAFP SF: lemon sign, banana sign*, obliterated cisterna magna, colpocephaly, enlarged massa intermedia, hydrocephalus, open spinal defect

cephalocele

CF: possible elevation of MSAFP SF: open cranial defect (typically posterior in location), small or obliterated cisterna magna, complex or simple appearing mass protruding from the cranium

occipital region

Most common location for cephalocele

choroid plexus cysts

SF: anechoic, round, smooth-walled cyst located within the choroid plexus of the lateral ventricle

aqueductal stenosis

SF: atrium of lateral ventricle > 10 mm, atrial measurement > 15 mm considered moderate to marked, dangling choroid sign (echogenic choroid plexus, hanging limp, surrounded by CSF), dilation of lateral ventricles and third ventricle (fourth ventricle is normal)

ventriculomegaly

SF: atrium of lateral ventricle > 10 mm, atrial measurement > 15 mm considered moderate to marked, dangling choroid sign* (echogenic choroid plexus, hanging limp, surrounded by CSF), dilation of any part of system

dandy-walker malformation (DWM)

SF: enlargement of cisterna magna > 10 mm in AP, communication of enlarged cisterna magna with dilated fourth ventricle, agenesis or hypoplasia of the cerebellar vermis, varying degrees of ventriculomegaly

mega cisterna magna

SF: enlargement of cisterna magna > 10 mm in AP, normal cerebellum and fourth ventricle

schizencephaly

SF: fluid-filled clefts within the cerebrum, agenesis of CSP and corpus callosum (50% of time), ventriculomegaly

hydraencephaly

SF: fluid-filled cranium, absent or partial absence of the falx cerebri, maintained brain stem, basal ganglia, and thalamus, no identifiable cerebral cortex

alobar holoprosencephaly

SF: horseshoe-shaped monoventricle, fused echogenic thalami; absence of CSP, interhemispheric fissure, falx cerebri, corpus callosum, third ventricle; normal cerebellum and brain stem

porencephaly

SF: image B (bottom), cystic mass that communicates with lateral ventricle, most often unilateral, confused with arachnoid cysts (image A) which will not communicate with lateral ventricle

lissencephaly

SF: lack of sulci and gyri within the cerebrum

agenesis of corpus callosum and CSP

SF: partial or complete absence of the corpus callosum and absence of CSP (after 18 weeks), "sunburst" sign* (radial arrangement of the sulci which produces a "spoke wheel" pattern), colpocephaly (small frontal horns and enlarged occipital horns - teardrop shaped lateral ventricles). elevated and dilated third ventricle

development of fluid-filled clefts within cerebrum

Schizencephaly

the cortex can have 3 shapes (pancake, cup, or ball), often results in neonatal death

alobar holoprosencephaly

most common cause of hydrocephalus in utero

aqueductal stenosis

banana sign

banana-shaped cerebellum

protrusions of intracranial contents through a defect in the skull

cephalocele

typically regress by end of 3rd trimester, associated with trisomy 18

choroid plexus cysts

found within choroid plexus, produce increased CSF, leading to ventriculomegaly

choroid plexus papillomas

obstruction lies outside the ventricular system

communicating hydrocephalus

apert syndrome, holoprosencephaly, DWM, aqueductal stenosis, trisomy 18, trisomy 8, trisomy 13

conditions linked with agenesis of corpus callosum and CSP

severe mental retardation, trisomy 13, patau syndrome

conditions linked with lobar holoprosencephaly

maternal diabetes, use of valproic acid (seizure medication), chromosomal anomalies (Edwards and Patau syndrome), triploidy

conditions linked with neural tube defects

hydraencephaly will not have cerebral mantle present, whereas the other two will have a rim of cerebral tissue maintained

difference between hydraencephaly and severe ventriculomegaly and holoprosencephaly

high impedance with continuous forward flow, effective at evaluating potential hypoxia in fetuses SFD, resistance pattern should be greater than umbilical artery

doppler of middle cerebral artery

omphalocele, gastroschisis, multiple gestations, fetal demise, incorrect gestational dating

elevated levels of AFP linked with

mass of brain tissue

encephalocele

cyclopia, proboscis, anopthalmia, hypotelorism, median cleft lip, cebocephaly

facial anomalies linked with holoprosencephaly

fatal condition which cerebrum is replaced by large sac containing CSF

hydraencephaly

dilation of ventricular system caused by an increased volume of CSF, resulting in increased intraventricular pressure

hydrocephalus

lemon sign

lemon-shaped cranium with flattened frontal bones

encephalomeningocele

mass of meninges and brain tissue

encephalomeningocystocele

mass of meninges, brain tissue, and lateral ventricle

mass of meninges

meningocele

teratoma

most common intracranial tumor found in utero

distal lumbosacral region

most common location of spina bifida

germinal matrix

most common origin of intracranial hemorrhage

the obstruction level is located within the ventricular system

noncommunicating hydrocephalus

> 1.0

normal MCA/umbilical artery ratio

starts in germinal matrix, may spread to lateral ventricle, often leading to a noncommunicating hydrocephalus (because clot obstructs flow of CSF), could lead to formation of cystic cavities within cerebral hemispheres

process of intracranial hemorrhage

maternal platelet disorders

risk for fetal intrauterine intracranial hemorrhage

maternal use of cocaine, trauma, history of amniocentesis

risks for fetal intracranial hemorrhage

mass (myelomeningocele or meningocele) that protrudes from the spine

spina bifida

cephaloceles, spinal dysraphisms, anencephaly, and spina bifida

types of neural tube defects

0.4 mg of folic acid

what supplement can reduce the likelihood of her fetus developing a neural tube defect?


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