CNS Embryology 5th Menstrual Week (Dorsal View)
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1 Imaging of the Fetal Brain; Normal & Abnormal Alfred Abuhamad, M.D. Eastern Virginia Medical School CNS Embryology 5th Menstrual Week (Dorsal View) Day 20 from fertilization Neural plate formed in ectoderm Edges of plate elevate neural folds CNS Embryology 5th Menstrual Week (Dorsal View) Day 22 from fertilization Folds start to fuse in cervical region Cranial neuropore closes on day 25 Caudal neuropore closes on day 27 Embryology 6-7th Menstrual Week Brain Vesicles Represent Divide into Ventricle Prosencephalon Forebrain Telencephalon Diencephalon Lateral 3 rd Mesencephalon Midbrain Aqueduct Rhombencephalon Hindbrain Metencephalon 4th Myelencephalon 7-9 wks Rhombencephalic Vesicle 9-13 wks 3 rd V 4 th V Aqueduct
2 9-13 wks CNS Anatomy Ventricular System CNS Anomalies Prosencephalon / Mesencephalon: Ventriculomegaly Holoprosencephaly Septo-optic dysplasia Cortical lesions Agenesis of corpus callosum Rhombencephalon Posterior fossa anomalies Ventriculomegaly Atrial Width Mean = 6-7 mm, SD ~ 1 mm Width of 10 mm is ~ 3 SDs (2 nd trim) Width of 10 mm is ~ 2 SDs (term) Male fetuses have wider measurements Radiology 1988;169:711 JUM 1991;7:79 Radiology 1994;193:405 Ventriculomegaly Incidence ~ /1000 livebirths Lateral ventricular enlargement Internal atrial diameter > 10 mm Choroid plexus does not fill space Radiology 1988;169:711
3 Ventriculomegaly Ventriculomegaly All Ventriculomegaly 75 % associated abnormalities Ventriculomegaly < 13 mm 30% associated abnormalities Ventriculomegaly Obstructive Ventricular (Noncommunicating) Aqueductal stenosis NTD D-W malformation Skeletal dysplasia Subarachnoid (Communicating) Hemorrhage Inflammation Nonobstructive (Communicating) Porencephaly Agenesis of CC Aneuploidy Work-up Targeted ultrasound, focus on CNS Endovaginal US, coronal / sagittal views Genetic counseling TORCH titers (CMV avidity testing) Amniocentesis (Chromosomes / Cultures)? MRI Average incidence 1-2 / 1000 (Geographic variations) Most commonly in lumbo-sacral area Hydrocephalus in % (Arnold-Chiari type 2) Open defect (Aperta) in 80 % of cases
4 Spinal Views Cranial Views (< 24 weeks) Lemon sign % Banana sign % Ventriculomegaly % Obliteration of cisterna magna % Small BPD % AJOG 1990;162:322 Lemon sign Banana sign
5 Prognosis IQ > 80 in 2/3 of patients Bladder and bowel incontinence in 83 % Hindbrain dysfunction in 32 % Normal ambulation in 23 % Radiographics 1988;8:695 Prognosis Associated anomalies Location and size of defect Kyphoscoliosis Severity of ventriculomegaly (?) AJOG 1997;177:319 Location of Lesion Lumbar Thoracic S4, last ossified vertebra in 2 nd trim S5, last ossified vertebra in 3 rd trim T12, last rib L5 - S1, top of iliac wing NTD & Volume Sonography
6 Neurologic Outcome Correlates very well with the bony level of the defect Pediatrics 2002;109:409 Cranial Defects Cranial Defects Cephaloceles Encephaloceles (brain & meninges) 85 % Cranial meningocele (CSF) 15 % Anencephaly Acrania Craniosynostosis Cephaloceles Incidence 1/2000-1/10,000 births Mostly in occipital region (80%) Mostly midline Many associated anomalies Associated syndromes Meckel-Gruber Syndrome Amniotic Band Syndrome Ultras Obstet Gynecol 1995;5:77
7 Cephaloceles Acrania Craniosynostosis Craniosynostosis Premature fusion of sutures Abnormally shaped cranium Sagittal suture affected mostly If untreated: Mental retardation Conjunctival / corneal injury Optic atrophy Craniosynostosis Crouzon
8 Posterior Fossa Anomalies Terminology Dandy Walker malformation Vermian hypoplasia/agenesis Blake s pouch cyst Arachnoid cyst Mega cisterna magna & Dandy Walker variant should be abandoned Normal Anatomy Normal Anatomy Pediatr Radiol 2004;34:720 Pediatr Radiol 2004;34:720
9 Arachnoid Cyst Blake s Pouch Cyst Pediatr Radiol 2004;34:720 Pediatr Radiol 2004;34:720 Dandy Walker Blake s Pouch Cyst 1. Enlarged cisterna magna 2. Vermis rotated superiorly 3. Vermis intact Pediatr Radiol 2004;34:720 Vermian Hypoplasia Dandy Walker Complex 1. Enlarged cisterna magna 2. Vermis rotated superiorly 3. Vermis hypoplastic Dandy Walker Malformation Vermian Hypoplasia/Agenesis
10 Dandy - Walker Malformation Normal Anatomy Developmental arrest in posterior medullary velum Accounts for 5-10 % of all hydrocephalus Chromosomal abnormalities in 29 % Features: Partial / complete absence of cerebellar vermis Greatly distented & cystic 4th ventricle Elevation of tentorium Enlargement of posterior fossa Dandy Walker Malformation Dandy - Walker Malformation Dandy - Walker Malformation Partial Vermian Agenesis High mortality rate Many associated abnormalities Low intellectual development (40 % normal) Hydrocephalus (80%) worsens prognosis Less dilated 4th ventricle / CM Good outcome when isolated (?) Clin Genet 1985;28:272 J Ultrasound Med 1994;13:607
11 Partial Vermian Agenesis Vermian Development 13 wks * * Hydranencephaly Hydranencephaly Hydranencephaly Sporadic abnormality Absence of cerebral hemispheres (fluid) Pathogenesis, obstruction of IC arteries Falx cerebri, thalami, occipital lobes and cerebellum usually spared May be associated with primary HSV
12 Intracranial Cysts Intracranial Cysts Large Series (n = 70) Gestational Age Diagnosis < 20 weeks 0 % weeks 55 % > 30 weeks 45 % Childs Nerv Syst 2003;19:477 Intracranial Cysts Large Series (n = 70) Cyst Size % Change Stable 76 % Progressed 20 % Regressed 4 % Intracranial Cysts Porencephalic Others Childs Nerv Syst 2003;19:477 Intracranial Cysts Porencephalic Cyst Hyperechoic, thick lining Mass around the cyst Sulci absent around the cyst Childs Nerv Syst 2003;19:477
13 CNS Lesions
14
intracranial anomalies
Chapter 5: Fetal Central Nervous System 84 intracranial anomalies Hydrocephaly Dilatation of ventricular system secondary to an increase in the amount of CSF. Effects of hydrocephalus include flattening
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