Abstract
This chapter comprises three parts: (1) embryology of the central nervous system, (2) normal ultrasound appearance of the brain from the embryonic period to term of pregnancy, and (3) ultrasound prenatal diagnosis of primary and secondary ventriculomegaly/hydrocephalus. In particular, the first part of the chapter represents a thorough description of the early development of the brain in the human fetus, including an interesting sono-anatomic correlation since when the embryonic brain can be explored with ultrasound, i.e., from the 7th gestational week. The second part is meant to illustrate to non-experts in fetal neurology how ultrasound is able to follow the dramatic changes in the brain appearance and anatomy due to the various phases of cerebral development (from the normally lissencephalic brain of the 2nd trimester to the 3rd trimester process of neuronal proliferation and migration). The last part describes in details the diagnostic criteria to establish in the fetus a diagnosis of primary (aqueductal stenosis) versus secondary ventriculomegaly, the latter related to a wide range of genetic and developmental disorders.
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Almog B, Gamzu R, Achiron R et al (2003) Fetal lateral ventricular width: what should be its upper limit? A prospective cohort study and reanalysis of the current and previous data. J Ultrasound Med 22:39–43
Bahlmann F, Reinhard I, Schramm T et al (2015) Cranial and cerebral signs in the diagnosis of spina bifida between 18 and 22 weeks of gestation: a German multicentre study. Prenat Diagn 35(3):228–235. https://doi.org/10.1002/pd.4524. Epub 2015 Feb 4
Barkovich AJ, Kjos BO (1989) Revised classification of posterior fossa cysts and cystlike malformations based on the results of multiplanar MR imaging. AJR 153:1289–1300
Barkovich AJ, Millen KJ, Dobyns WB (2009) A developmental and genetic classification for midbrain-hindbrain malformations. Brain 132(Pt 12):3199–3230
Bayer SA, Altman J (2008) Atlas of human central nervous system development – 5 volume set. CRC Press/Taylor and Francis Group, Boca Raton
Blake JA (1900) The roof and lateral recesses of the 4th ventricle, considered morphologically and embryologically. J Comp Neurol 10:79–108
Brocklehurst G (1969) The development of the human cerebrospinal fluid pathway with particular reference to the roof of the fourth ventricle. J Anat 105(Pt 3):467–475
Bromley B, Frigoletto FD Jr, Benacerraf BR (1991) Mild fetal lateral cerebral ventriculomegaly: clinical course and outcome. Am J Obstet Gynecol 164:863–867
Bystron I, Blakemore C, Rakic P (2008) Development of the human cerebral cortex: Boulder Committee revisited. Nat Rev Neurosci 9(2):110–122
Cagneaux M, Vasiljevic A, Massoud M et al (2013) Severe second-trimester obstructive ventriculomegaly related to disorders of diencephalic, mesencephalic and rhombencephalic differentiation. Ultrasound Obstet Gynecol 42(5):596–602
Cardoza JD, Goldstein RB, Filly RA (1988) Exclusion of fetal ventriculomegaly with a single measurement: the width of lateral ventricular atrium. Radiology 169:711–714
Cohen-Sacher B, Lerman-Sagie T, Lev D et al (2006) Sonographic developmental milestones of the fetal cerebral cortex: a longitudinal study. Ultrasound Obstet Gynecol 27(5):494–502
De Keersmaecker B, Van Esch H, Van Schoubroeck D et al (2013) Prenatal diagnosis of MPPH syndrome. Prenat Diagn 33(3):292–295
Deloison B, Chalouhi GE, Sonigo P (2012) Hidden mortality of prenatally diagnosed vein of Galen aneurysmal malformation: retrospective study and review of the literature. Ultrasound Obstet Gynecol 40(6):652–658
Emery SP, Hogge WA, Hill LM (2005) Accuracy of prenatal diagnosis of isolated aqueductal stenosis. Prenat Diagn 35:319–324
Garel C (2004) Abnormalities of the fetal cerebral parenchyma: ischaemic and haemorrhagic lesions. In: Garel C (ed) MRI of the fetal brain. Springer-Verlag Berlin Heidelberg, pp 247–262
Gato A, Desmond ME (2009) Why the embryo still matters: CSF and the neuroepithelium as interdependent regulators of embryonic brain growth, morphogenesis and histiogenesis. Dev Biol 327(2):263–272
Hertzberg BS, Kliewer MA, Freed KS et al (1997) Third ventricle: size and appearance in normal fetuses through gestation. Radiology 203(3):641–644
International Society of Ultrasound in Obstetrics & Gynecology Education Committee (2007) Sonographic examination of the fetal central nervous system: guidelines for performing the ‘basic examination’ and the ‘fetal neurosonogram’. Ultrasound Obstet Gynecol 29(1):109–116
Kennedy D, Chitayat D, Winsor EJT et al (1998) Prenatally diagnosed neural tube defects: ultrasound, chromosome, and autopsy or postnatal findings in 212 cases. Am J Med Genet 77:317–321
Kier EL, Truwit CL (1996) The normal and abnormal genu of the corpus callosum: an evolutionary, embryologic, anatomic, and MR analysis. AJNR Am J Neuroradiol 17(9):1631–1641
Lun MP, Monuki ES, Lehtinen MK (2015) Development and functions of the choroid plexus-cerebrospinal fluid system. Nat Rev Neurosci 16(8):445–457
Mack J, Squier W, Eastman JT (2009) Anatomy and development of the meninges: implications for subdural collections and CSF circulation. Pediatr Radiol 39(3):200–210
Mangione R, Fries N, Godard P et al (2011) Neurodevelopmental outcome following prenatal diagnosis of an isolated anomaly of the corpus callosum. Ultrasound Obstet Gynecol 37(3):290–295
Melchiorre K, Bhide A, GIka AD et al (2009) Counseling in isolated mild fetal ventriculomegaly. Ultrasound Obstet Gynecol 34:212–224
Monteagudo A, Timor-Tritsch IE (1997) Development of fetal gyri, sulci and fissures: a transvaginal sonographic study. Ultrasound Obstet Gynecol 9(4):222–228
Monteagudo A, Timor-Tritsch IE (2009) Normal sonographic development of the central nervous system from the second trimester onwards using 2D, 3D and transvaginal sonography. Prenat Diagn 29(4):326–339
Mortazavi MM, Griessenauer CJ, Adeeb N et al (2014) The choroid plexus: a comprehensive review of its history, anatomy, function, histology, embryology, and surgical considerations. Childs Nerv Syst 30(2):205–214
Müller F, O’Rahilly R (2003) Segmentation in staged human embryos: the occipitocervical region revisited. J Anat 203(3):297–315
Napolitano R, Maruotti GM, Quarantelli M (2009) Prenatal diagnosis of Seckel syndrome on three-dimensional sonography and magnetic resonance imaging. J Ultrasound Med 28:369–374
Nicolaides KH, Campbell S, Gabbe SG (1986) Ultrasound screening for spina bifida: cranial and cerebellar signs. Lancet 2(8498):72–74
O’Rahilly R, Muller F (1987) Developmental stages in human embryos. Carnegie institution of Washington, Washington, DC
O’Rahilly R, Muller F (1999) Minireview: Summary of the initial development of the human nervous system. Teratology 1999; 60(1):39–41
O’Rahilly R, Muller F (2005) The embryonic human brain: an atlas of developmental stages, 3rd edn. Wiley, New York
O’Rahilly R, Muller F (2010) Developmental stages in human embryos: revised and new measurements. Cells Tissues Organs 192(2):73–84
Ouahba J, Luton D, Garel C et al (2006) Prenatal isolated mild ventriculomegaly: outcome in 167 cases. BJOG 113:1072–1079
Paladini D (2008) Diagnosis of spina bifida and other dysraphic states in the fetus. In: Ozek MM, Cinalli G, Maixner WJ (ed). Spina Bifida. Management and outcome. Springer-Verlag Italia, Milan, p 75–102
Paladini D, Volpe P (2006) Posterior fossa and vermian morphometry in the characterization of fetal cerebellar abnormalities: a prospective three-dimensional ultrasound study. Ultrasound Obstet Gynecol 27(5):482–489
Paladini D, Sglavo G, Quarantelli M et al (2005) Large infratentorial subdural hemorrhage diagnosed by ultrasound and MRI in a second-trimester fetus. Letter to the Editor. Ultrasound Obstet Gynecol 26:789–796
Paladini D, Quarantelli M, Pastore G (2012) Abnormal or delayed development of the posterior membranous area of the brain: anatomy, ultrasound diagnosis, natural history and outcome of Blake’s pouch cyst in the fetus. Ultrasound Obstet Gynecol 39(3):279–287
Paladini D, Pastore G, Cavallaro A et al (2013) Agenesis of the fetal corpus callosum: sonographic signs change with advancing gestational age. Ultrasound Obstet Gynecol 42(6):687–690
Paladini D, Quarantelli M, Sglavo G (2014) Accuracy of neurosonography and MRI in clinical management of fetuses referred with central nervous system abnormalities. Ultrasound Obstet Gynecol 44(2):188–196
Pinto J, Paladini D, Severino M (2016) Delayed rotation of the cerebellar vermis: a pitfall in early second-trimester fetal magnetic resonance imaging. Ultrasound Obstet Gynecol 48(1):121–124
Pugash D, Hendson G, Dunham CP et al (2012) Sonographic assessment of normal and abnormal patterns of fetal cerebral lamination. Ultrasound Obstet Gynecol 40(6):642–651
Rakic S, Zecevic N (2003) Emerging complexity of layer I in human cerebral cortex. Cereb Cortex 13(10):1072–1083
Rubenstein JL, Shimamura K, Martinez S, Puelles L (1998) Regionalization of the prosencephalic neural plate. Annu Rev Neurosci 21:445–477
Salomon LJ, Ouahba J, Delezoide AL, Vuillard E, Oury JF, Sebag G, Garel C et al (2006) Third-trimester fetal MRI in isolated 10–12 mm ventriculomegaly: is it worth it? BJOG 113:942–947
Sari A, Ahmetoglu A, Dinc H et al (2005) Fetal biometry: size and configuration of the third ventricle. Acta Radiol 46(6):631–635
Sepulveda W, Corral E, Ayala C et al (2004) Chromosomal abnormalities in fetuses with open neural tube defects: prenatal identification with ultrasound. Ultrasound Obstet Gynecol 23:352–356
Strazielle N, Ghersi-Egea JF (2000) Choroid plexus in the central nervous system: biology and physiopathology. J Neuropathol Exp Neurol 59(7):561–574
Streeter GL (1911) Die Entwicklung des Nervensystems. In: Keibel F, Mall FP (eds) Handbuch der Entwicklungsgeschichte des Menschen. Zweiter Band, Hirzel/Leipzig, pp.1–156
Timor-Tritsch IE, Monteagudo A (1996) Transvaginal fetal neurosonography: standardization of the planes and sections by anatomic landmarks. Ultrasound Obstet Gynecol 8(1):42–47
Toi A, Lister WS, Fong KW (2004) How early are fetal cerebral sulci visible at prenatal ultrasound and what is the normal pattern of early fetal sulcal development? Ultrasound Obstet Gynecol 24(7):706–715
Tortori-Donati P, Fondelli MP, Rossi A et al (1996) Cystic malformations of the posterior cranial fossa originating from a defect of the posterior membranous area. Mega cisterna magna and persisting Blake’s pouch: two separate entities. Childs Nerv Syst 12(6):303–308
Van den Hof MC, Wilson RD, Diagnostic Imaging Committee, Society of Obstetricians and Gynaecologists of Canada, Genetics Committee, Society of Obstetricians and Gynaecologists of Canada (2005) Fetal soft markers in obstetric ultrasound. J Obstet Gynaecol Can 27:592–636
Vassallo M, Maruotti GM, Quarantelli M et al (2012) Choroid plexus carcinoma: prenatal characterization by 3-dimensional sonography and magnetic resonance imaging, perinatal management, and natural history. J Ultrasound Med 31(2):337–339
Volpe P, Muto B, Passamonti U (2015) Abnormal sonographic appearance of posterior brain at 11–14 weeks and fetal outcome. Prenat Diagn 35(7):717–723
Wilson JT (1937) On the nature and mode of origin of the foramen of Magendie. J Anat 71(Pt 4):423–428
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Paladini, D., Birnbaum, R. (2019). Prenatal Hydrocephalus: Prenatal Diagnosis. In: Cinalli, G., Özek, M., Sainte-Rose, C. (eds) Pediatric Hydrocephalus. Springer, Cham. https://doi.org/10.1007/978-3-319-27250-4_47
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DOI: https://doi.org/10.1007/978-3-319-27250-4_47
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