CHOROID PLEXUS CYST

CHOROID PLEXUS CYST - keywords
choroid plexus cyst r e f e r e n c e s n a v i g a t o r choroid plexus cyst: references < Adeeb N, Deep A, Griessenauer CJ, Mortazavi MM, Watanabe K, Loukas M, Tubbs RS, Cohen-Gadol AA (2013) The intracranial arachnoid mater : a comprehensive review of its history, anatomy, imaging, and pathology. Childs Nerv Syst. Jan;29(1):17-33. Altman J, Bayer SA (2015) Development of the human neocortex: a review and interpretation of the histological record. A Free eBook from the Laboratory of Developmental Neurobiology, Inc. www.neurondevelopment.org © 2015, The Laboratory of Developmental Neurobiology, Inc. Ocala, FL 34481, USA Ariëns Kappers JA (1958) Structural and functional changes in the telencephalic choroid plexus during brain ontogenesis. In: Wolstenholme GEW, O’Connor CM (eds) The Cerebrospinal Fluid. Little, Brown, Boston, MA, pp 3–25. Bartelmez GW, Dekaban AS (1962) The early development of the human brain. Contrib Embryol Carnegie Instn 37:13–32 Boxill M, Becher N, Sunde L, Thelle T. Choroid plexus hyperplasia and chromosome 9p gains. Am J Med Genet A. 2018 Jun;176(6):1416-1422. Bronsteen R, Lee W, Vettraino I, Balasubramaniam M, Comstock C (2006) Isolated choroid plexus separation on second trimester sonography: natural history and postnatal importance. J Ultrasound Med 25(3): 343-347. Catala M (1998) Embryonic and fetal development of structures associated with the cerebrospinal fluid in man and other species. Part I: The ventricular system, meninges and choroid plexuses. Arch Anat Cytol Pathol 46(3):153–169. Chitkara U, Cogswell C, Norton K, Wilkins IA, Mehalek K, Berkowitz RL (1988) Choroid plexus cysts in the fetus: a benign anatomic variant or pathologic entity? Report of 41 cases and review of the literature. Obstetr Gynecol 72:185–189. Desmond ME, Jacobsen AG (1977) Embryonic brain enlargement requires cerebrospinal fluid pressure. Dev Biol 57:188–198. Dohrmann, G. J. 1970. The choroid plexus: A historical review. Brain Research, 18:197-218. Dziegielewska KM, Ek J, Habgood MD, Saunders NR (2001) Development, evolution and ageing: Development of the Choroid Plexus. Microscopy research and technique 52:5-20. Fakhry J, Schechter A, Tenner MS, Reale M (1985) Cysts of the choroid plexus in neonates: documentation and review of the literature. J Ultrasound Med 4:561–563. Fong K, Chong K, Toi A, Uster T, Blaser S, Chitayat D. Fetal ventriculomegaly secondary to isolated large choroid plexus cysts: prenatal findings and postnatal outcome. Prenat Diagn. 2011 Apr;31(4):395-400. Galarza M (2002) Evidence of the subcommissural organ in humans and its association with hydrocephalus. Neurosurg Rev 25:205–15. Gomez DG, DiBenedetto AT, Pavese AM et al. (1982) Development of arachnoid villi and granulations in man. Acta Anat (Basel) 111:247–58. Gupta JK, Cave M, Lilford RJ, Farrell TA, Irving HC, Mason G, Hau CM (1995) Clinical significance of fetal choroid plexus cysts. Lancet 346:724–729. Heibel M, Heber R, Bechinger D, Kornhuber HH (1993) Early diagnosis of perinatal cerebral lesions in apparently normal full-term newborns by ultrasound of the brain. Neuroradiology 35:85–91. Hertzberg BS, Kay HH, Bowie JD (1989) Fetal choroid plexus lesions. Relationship of antenatal sonographic appearance to clinical outcome. J Ultrasound Med 8:77–82. Hochstetter F (1939) Über die Entwicklung und Differenzierung der Hüllen des menschlichen Gehirns. Morphol Jahrb 83:359– 494. Iliff JJ, Wang M, Liao Y et al. (2012) A paravascular pathway facilitates CSF flow through the brain parenchyma and the clearance of interstitial solutes, including amyloid beta. Sci Transl Med 4:147ra111. Kraus I, Jirásek JE (2002) Some observations of the structure of the choroid plexus and its cysts. Prenat Diagn 22:1223–1228. Kurjak A, Schulman H, Predanic A, Predanic M, Kupesic S, Zalud I (1994) Fetal choroid plexus vascularisation assessed by color flow ultrasonography. J Ultrasound med 13(11): 841-844. Møllgård K, Malinowska DH, Saunders NR (1976) Lack of correlation between tight junction morphology and permeability properties in developing choroid plexus. Nature 264:293–294. Nakada T, Kwee IL (2018) Fluid Dynamics Inside the Brain Barrier: Current Concept of Interstitial Flow, Glymphatic Flow, and Cerebrospinal Fluid Circulation in the Brain. The Neuroscientist 1-12. Nakase H, Hisanaga M, Hashimoto S, Imanishi M, Utsmi S (1988) Intraventricular arachnoid cyst. Report of two cases. J Neurosurg 68:482–486. Netsky MG, Shuangshoti S (1975) The choroid plexus in health and disease. Bristol: Wright. O’Rahilly R, Müller F (1986) The meninges in human development. J Neuropathol Exp Neurol 45:588–608 O’Rahilly R, Müller F (1999) The Embryonic Human Brain. An atlas of developmental stages, 2nd ed. Wiley-Liss, New York Paladini D, Quarantelli M, Pastore G, Sorrentina M, Sglavo G, Nappi C (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 in Obstetrics and Gynecology 39:279-287. Retzius G (1896) Das Menshenhirn: Studien in der Makroskopischen Morphologie. Sockholm: PA Norstedt 1-167. Riebel T, Nasir R, Weber K (1992) Choroid plexus cysts: a normal finding on ultrasound. Pediatr Radiol 22:410–412. Robinson AJ, Goldstein R (2007) The cisterna magna septa. J Ultrasound Med 26:83-95. Rodriguez EM, Oksche A, Montecinos H (2001) Human subcommissural organ, with particular emphasis on its secretory activity during the fetal life. Microsc Res Tech 52:573–90. Sakkaa L, Coll G, Chazala J (2011) Anatomy and physiology of cerebrospinal fluid. European Annals of Otorhinolaryngology, Head and Neck diseases 128, 309—316. Sepulveda W, Lopez-Tenoria J (2001) The value of minor ultrasound markers for fetal aneuploidy. Curr Opin Obstetr Gynecol 13:183-191. ten Donkelaar H, van der Vliet T (2014) Overview of the Development of the Human Brain and Spinal Cord. Ch 1 in: ten Donkelaar HJ, Lammens M, Hori A: Clinical Neuroembryology: Development and Developmental Disorders of the Human Central Nervous System. Timor-Tritsch I, Monteagudo A, Pilu G, Malinger G (2012) Ultrasonography of the prenatal brain. 3rd edition, McGraw–Hill Professional 60–63. Tsuboi K, Maki Y, Hori A, Ebihara R (1984) Accessory ventricles of the posterior horn. Prog Comp Tomogr 6:529–534 Voetmann E (1949) On the structure and surface area of the human choroid plexuses: a quantitative anatomical study. Acta Anat 8(Suppl 10):1–116. > plexus histology The choroid plexus sits between two barriers (blood and cerebrospinal fluid) with as primary goal the production and regulation of cerebrospinal fluid (Catala 1998). Plexus expansion may initially also just increase ventricular surface and thus spread the proliferation of neocortical precursors (Desmond and Jacobsen 1977, Altman and Bayer 2015). Differentiating neurons leave the neuroepithelium in larger numbers early during the 9th week after conception, associated in time with a rapid expansion of choroid plexus in equally expanding ventricles. The nuclei of precursors shuttle to the ventricular margin to undergo mitosis. The size of any neuronal population depends on the size of that wall space occupied by its precursors. An immense number of neurons in the neocortex can be achieved due to the volume of the ventricles, before they begin to shrink and in some regions disappear altogether. The expansion of that large space requires production of CSF with adequate pressure, and that appears to be initially dependent, before the vascular system develops (Padget, 1957), on the enlarged meninges and the hypertrophied choroid plexus. The cranial meninges originate from the prechordal plate, the parachordal mesoderm and the neural crest (O’Rahilly and Müller 1986). They induce proliferation of neuroblasts and axonal growth. The loose mesenchyme around the brain at 5 weeks of development (stage 15) forms a primary meninx. At 6 weeks (stage 17), the dural limiting layer is found basally and the skeletogenous layer of the head becomes visible. At 7 weeks (stage 19), a cranial pachy- and leptomeninx are distinguishable. plexus volume CUS plexus cysts CUS plexus anatomy choroid plexus cyst: development and function of choroid plexus On closure of the neuropores, the choroid plexus is not yet functional. However, CSF pressure in the neural tube and the volume of the cephalic cavities already increase, suggesting initial secretion of CSF outside choroid plexus. Some subarachnoid spaces appear on the 32nd day at the ventral rhombencephalon, then extend caudally and dorsally (Sakkaa et al. 2011). However, the fourth ventricle is not yet open and CSF circulation is only effective on the 41st day (postmenstrual week 16), weeks after onset of CSF production (Robinson and Goldstein 2007). Even later opening of the foramina of Luschka in the lateral recesses of the fourth ventricle hence leads to prolonged dilatation of the fourth ventricle, referred to as Blake’s pouch (Paladini et al. 2012). Formation of the subarachnoid spaces is therefore not exclusively due to CSF pressure, but capillaries elsewhere play an initial role in secretion of CSF. A microscopic flow of CSF has been described in brain interstitial spaces (Iliff et al. 2012), the significance of which is not yet known in the fetus or newborn. So fluid circulation in the cranium depends on maturation of arachnoid villi, development of glymphatics and of nasal lymphatic fluid drainage (Adeeb et al. 2013, Nakada and Twee 2018). Arachnoid villi mature near term, and change into arachnoid granulations is mainly a postnatal event (Gomez et al. 1982). The human subcommissural organ is differentiated ependyma caudal to the pineal gland at the entrance to the aqueduct (Galarza 2002). It becomes an active “gland" at midgestation, to regress during the first year of postnatal life (Rodriguez et al. 2001). Changes in the subcommissural organ were described in all species developing congenital hydrocephalus and in human fetuses, but the precise role of their secreted glycoproteins is unknown in the human fetal context. plexus pseudocyst after Netsky and Shuangshoti 1975 choroid plexus ultrasound anatomy in viable preterms, at 24 weeks PMA, choroid plexus still dominates the coronal sections throuhg the atria of the lateral ventricle; at term this relative dominance has disappeared due to expansion of hemispheric white matter tracts thick temporal horn plexus thick tela a rare condition exists, with an abnormal gain of parts of chro 9, where choroid plexus is hyperplastic (Boxill et al. 2018); even at term the plexus dominates the atrial section and due to overproduction of CSF the ventricles progressively dilate until treatment by coagulation; vascular anatomy and flow indices are normal choroid plexus cysts preterm infant of 28w GA without other problems; chance finding of intermediate size plexus cyst temporal plexus cyst in an infant with genetic cobalamine deficiency with in vivo imaging it is not possible to differentiate a germinolytic cyst from an anterior choroid plexus cyst term infant, small for gestational age: first day 7.5 MHz sections (left, coronal; right, parasagittal) of an anomaly detected antenatally in the third trimester a large cyst can be seen in the glomus of the choroid plexus of the right lateral ventricle: this child had trisomy 18 cerebellar hypoplasia was an additional finding Choroid plexus first appears in the roof of the fourth ventricle at stage 18 (day 41), in the lateral ventricles at stage 19, and in the third ventricle at stage 21 (Ariëns Kappers 1958; Bartelmez and Dekaban 1962). The primordia appear as simple or club-shaped folds protruding into the ventricles, emanating from pial mesenchymal vascular extensions covered by single ependymal epithelium. The stroma of the plexus originates from extensions of the arachnoid into the interior of the brain forming the vela interposita. The contemporary enlargement of meningeal constituents is most pronounced in the middle part of the neocortex, where this leads to the mid-cephalic constriction of the cerebrum (the frontal-temporal dimple and the invaginating insula). As vascularization of the expanding neocortex starts, that dimple becomes the widening frontal-temporal cleavage, where arterial branches of the MCA expand over the convexity. As white matter begins to expand in 4-months-old fetuses, there is concurrent shrinkage of the lateral ventricles. The time at which choroid plexus starts to secrete CSF has not been clearly determined, but carbonic anhydrase (for bicarbonate excretion) is present two weeks after appearance of plexus. It may be that an early nutritional role for plexus is present, but this function is lost during development. In the eight week, spreading from the choroidal fissure, the lobular character of the plexus gradually changes into an arrangement of wavy folds; capillaries form single loops under the cuboidal epithelium (Kraus and Jirasek 2002). The mechanisms involved in formation of a blood-CSF barrier at choroid plexus combine diffusion restraint (tight junctions between the plexus epithelial cells) with specification of exchange. One unique barrier mechanism transfers specific proteins from blood to CSF, via tubulocisternal endoplasmic reticulum in plexus epithelial cells. This results in a high concentration of proteins in early CSF. Proteins may function as colloid osmotic agents for ventricle expansion but also as specific carriers and growth factors. Some proteins are not imported but are locally produced, like transthyretin and IGF II. During stage 21, the choroid plexuses become vascularized. The early choroid plexus is lobulated with vessels running in the mesenchymal stroma to form capillary nets under the single-layered ependyma. The embryonic plexus is converted into the fetal type during the ninth week of development as this net is replaced by elongated loops of wavy capillaries that lie under regular longitudinal epithelial folds (Kraus and Jirásek 2002). Bone morphogenetic proteins and tropomyosin are involved in specification of plexus development. Four histological stages of lateral ventricle plexus development have been defined (Dziegielewska et al. 2001): stage 1: 7-9 weeks after conception, no glycogen, ill defined blood islets, pseudostratified epithelium; stage 2: 9-17 weeks after conception, abundant glycogen, primari villi, low columnar epithelium, subepithelial capillaries, very large relative size in relation to ventricle; stage 3: fetal period, moderate glycogen, many primary villi, cuboidal epithelium, mature capillaries in villous core; stage 4: after 29 weeks: villi with multiple fronds, disappearing glycogen, cuboidal or squamous epithelium, relatively small plexus in relation to ventricle size. plexus is absent at GW 7, CR 15 mm fetus; the stalk of the choroid plexus is beginning to produce the rudiment of the choroid plexus in GW 8; the blooming of the choroid plexus in the expanded lateral ventricle is in GW 10 from Altman and Bayer 2015 source and staging of development of choroid plexus single plexus cyst in a term infant with early onset infection without meningitis <---- choroid plexus pseudocysts Human fetal choroid plexus sometimes contains a large central mass of amorphous material with a few poorly distinguishable blood vessels, surrounded by a single layer of epithelial cells (Voetmann 1949, Netsky and Shuangshoti 1975). Its significance is uncertain, but the resemblance to Wharton’s jelly suggests it may have a mechanical function. In many fetuses plexus cysts appear (and often regress) in which fluid accumulates in a mesenchymal space without epithelial lining (unlike colloid cysts). The cysts have irregular angiomatous capillaries in there walls, unlike the normal capillary loops (Kraus and Jirasek 2002). As an isolated finding this is within normal, often an incidental sonographic finding. Already at 9 weeks, with transvaginal ultrasound, the plexus can be seen at the level of both lateral ventricles. Between 9 and 11 weeks, plexus covers most of the surface of the standard transventricular fetal ultrasound axial section. As pregnancy progresses, the volume ratio between choroid plexus and the ventricles decreases. As the ventricles develop, the choroid plexus grows to occupy the atrium, covering posterior thalamus (Timor-Tritsch et al. 2012). The finding of isolated choroid plexus separation by more than 3 mm from the medial ventricle wall is usually temporary in the second trimester, resolving in most cases within 4 weeks of the initial diagnosis in the second trimester. Most infants with this finding have no abnormalities (Bronsteen et al. 2006). During stage 21, the choroid plexuses become vascularized. Choroid plexus vessels are first seen with doppler ultrasound at 10 to 11 weeks GA. It has been estimated that in the course of the second trimester 0.3–0.6 % of all fetuses develop echographically detectable cysts in the plexus of one or both lateral ventricles. Microcysts are thus frequent. In fact plexus cyst formation is normal between the 17th and 28th gestational weeks. One should follow cysts when their diameter exceeds 3 mm (incidence rate < 0.5 per 1000 healthy live births). The pseudocysts consist of CSF and cell debris surrounded by a connective tissue membrane and an angiomatous capillary plexus (Kraus and Jirasek 2002). As a rule they do not follow an antecedent plexus haemorrhage and they are not bordered by ependyma unlike neuroepithelial (colloid) cysts. They naturally evolve towards regression before the end of the second trimester. Some cysts are big enough to bulge into the ventricle where they may rarely cause obstructive hydrocephalus. In the tela choroidea of the third ventricle similar cysts may be observed. Plexus cysts have been described in association with cysts of the germinal matrix at the foramen of Monro. Differentiation between those two is in some cases echographically impossible. Cysts identified in the perinatal period, even if their diameter is less than 7 mm, may remain visible for more than a year. If a plexus cyst persists after 22–24 gestational weeks, or if it is exceptionally big (> 1cm), and above all if in combination with high maternal age or another anomaly, an underlying condition such as a chromosomal disorder should be excluded. Plexus cysts are not more common than usual in trisomy 21, but they are in trisomy 18. Other associations are with Aicardi syndrome (cysts in half of cases, uni- or bilateral, small or large), nevoid basal cell carcinoma and chondrodysplasia punctata. MECP2 duplication is associated with ventriculomegaly, hydrocephalus, agenesis of the corpus callosum, choroid plexus cysts, foetal growth restriction and hydronephrosis. Midline interhemispheric cyst walls (see callosal agenesis) may contain choroid tissue. The existence of smaller cysts, however, does not exclude an anomaly of the karyotype. Isolated plexus cysts predict karyotype disorders in less than 1 %, and their isolated recognition therefore does not warrant amniocentesis. choroid plexus histology in orange, separation of pial and dural vessels in the meninx primitiva (from Altman and Bayer 2015) Mac OS X 2ÖATTRÜ,Ücom.apple.TextEncodingëcom.apple.provenanceöcom.apple.quarantineutf-8;134217984Â.Im0ÖWq/0081;00000000;;

Cerebrus