Abstract
This article describes a classification and imaging diagnosis of intracranial midline cystic malformations based on neuroembryologic analysis. Midline cystic malformations are classified into two categories from an embryologic point of view. In one category, the cyst represents expansion of the roof plate of the brain vesicle, and in the other the cyst consists of extraaxial structures such as an arachnoid membrane or migrating ependymal cells. Infratentorial cysts, such as the Dandy-Walker cyst or Blake's pouch cyst, and supratentorial cysts, such as a communicating interhemispheric cyst with callosal agenesis or a dorsal cyst with holoprosencephaly, are included in the first category. Infratentorial arachnoid cavities, such as the arachnoid cyst, arachnoid pouch, and mega cisterna magna, are in the second category. Noncommunicating interhemispheric cysts, such as interhemispheric arachnoid cyst or ependymal cyst, with callosal agenesis are also in the second category. A careful review of embryologic development is essential for understanding these midline cysts and for making a more accurate radiologic diagnosis.
Article PDF
Similar content being viewed by others
Avoid common mistakes on your manuscript.
References
WE Dandy KD Blackfan (1914) ArticleTitleInternal hydrocephalus: an experimental, clinical study Am J Dis Child 8 406–82
JK Taggart AE Walker (1942) ArticleTitleCongenital atresia of the foramen of Luschuka and Magendie Arch Neurol Psychiatry 48 538–612
CE Benda (1954) ArticleTitleThe Dandy-Walker syndrome of the so-called atresia of the foramen of Megendie J Neuropathol Exp Neurol 13 14–29 Occurrence Handle13118372 Occurrence Handle1:STN:280:DyaG2c%2FjvFegtg%3D%3D
A Brodal E Hauglie-Hanssenn (1959) ArticleTitleCongenital hydrocephalus with defective development of the cerebellar vermis (Dandy-Walker syndrome) J Neurol Neurosurg Psychiatry 22 99–108 Occurrence Handle13655097 Occurrence Handle1:STN:280:DyaG1M%2FosVOltg%3D%3D
AJ Barkovich BO Kjos D Norman MS Edwards (1989) ArticleTitleRevised classification of posterior fossa cysts and cystlike malformations based on the results of multiplanar MR imaging Am J Neuroradiol 10 977–88
JN Young WJ Oakes HP Hatten SuffixJr (1992) ArticleTitleDorsal third ventricular cyst: an entity distinct from holoprosencephaly J Neurosurg 77 556–61 Occurrence Handle1527614 Occurrence Handle1:STN:280:DyaK38vgslKmtg%3D%3D
GA De Leon MA Radkowski FA Gutierrez (1995) ArticleTitleSingle forebrain ventricle without prosencephaly: agenesis of the corpus callosum with dehiscent fornices Acta Neuropathol (Berl) 89 454–8 Occurrence Handle1:STN:280:DyaK2MzktlOhtQ%3D%3D
H Utsunomiya (2004) ArticleTitleMorphogenesis and MR findings in holoprosencephaly, with attention to definition of the holospheric brain (in Japanese) Jpn J Neurosurg 13 454–63
NR Altman TP Naidich BH Braffman (1992) ArticleTitlePosterior fossa malformations AJNR Am J Neuroradiol 13 691–724 Occurrence Handle1566724 Occurrence Handle1:STN:280:DyaK383jtVKnsg%3D%3D
SS Kollias WS Ball EC Prenger (1993) ArticleTitleCystic malformations of the posterior fossa: differential diagnosis clarified through embryologic analysis Radiographics 13 1211–31 Occurrence Handle8031352 Occurrence Handle1:STN:280:DyaK2c7itVWjtw%3D%3D
LB Arey (1946) Developmental anatomy: a text book and laboratory manual of embryology EditionNumber5th ed Saunders Philadelphia
RJ Lemire JD Loesor RW Lecch CA Ellsworth (1975) Normal and abnormal development of the human nervous system Harper & Row New York
F Muller R O'Rahilly (1988) ArticleTitleThe first appearance of the future cerebral hemispheres in the human embryo at stage 14 Anat Embryol (Berl) 177 495–511 Occurrence Handle1:STN:280:DyaL1c3jvFSitg%3D%3D Occurrence Handle10.1007/BF00305137
C Raybaud (1982) ArticleTitleCystic malformations of the posterior fossa: abnormalities associated with the development of the roof of the fourth ventricle and adjacent meningeal structures J Neuroradiol 9 103–33 Occurrence Handle7108588 Occurrence Handle1:STN:280:DyaL383mvVWntg%3D%3D
LH Weed (1917) The development of the cerebro-spinal space in pig and in man Contributions in embryology Carnegie Institution of Washington Washington, DC
R O'Rahilly F Muller GM Hutchins GW Moor (1988) ArticleTitleComputer ranking of the sequence of appearance of 40 features of the brain and related structures in staged human embryos during the 7th week of development Am J Anat 182 295–317 Occurrence Handle3189193 Occurrence Handle10.1002/aja.1001820402
A Louvi P Alexandre C Metin W Wurst M Wassef (2003) ArticleTitleThe isthmic neuroepithelium is essential for cerebellar midline fusion Development 130 5319–30 Occurrence Handle14507778 Occurrence Handle1:CAS:528:DC%2BD3sXps12qtrg%3D Occurrence Handle10.1242/dev.00736
MD Nelson SuffixJr K Maher FH Gilles (2004) ArticleTitleA different approach to cysts of the posterior fossa Pediatr Radiol 34 720–32 Occurrence Handle15316692 Occurrence Handle10.1007/s00247-004-1253-1
JA Blake (1900) ArticleTitleThe roof and lateral recess of the forth ventricle, considered morphologically and embryologically J Comp Neurol 10 79–108 Occurrence Handle10.1002/cne.910100104
JT Wilson (1937) ArticleTitleOn the nature and mode of origin of the foramen of Magendie J Anat 71 423–8 Occurrence Handle17104654 Occurrence Handle1:STN:280:DC%2BD2svjtlSgtg%3D%3D
R O'Rahilly F Muller (1986) ArticleTitleThe meninges in human development J Neuropathol Exp Neural 45 588–608
MN Hart N Malamud WG Ellis (1972) ArticleTitleThe Dandy-Walker syndrome: a clinicopathological study based on 28 cases Neurology 22 771–80 Occurrence Handle4343429 Occurrence Handle1:STN:280:DyaE3s%2Fjt12qtg%3D%3D
DC Harwood-Nash CR Fitz (1976) Neuroradiology in infants and children Mosby St. Louis
FH Gilles FX Rockett (1971) ArticleTitleInfantile hydrocephalus: retrocerebellar “arachnoidal” cyst J Pediatr 79 436–43 Occurrence Handle5315078 Occurrence Handle1:STN:280:DyaE3M3ot1CqsQ%3D%3D Occurrence Handle10.1016/S0022-3476(71)80153-1
MJ Harrison (1971) ArticleTitleCerebral arachnoid cyst in children J Neurol Neurosurg Psychiatry 34 316–23 Occurrence Handle5315217 Occurrence Handle1:STN:280:DyaE3M3ptV2nsw%3D%3D
CM Shaw EC Alvord SuffixJr (1977) Congenital arachnoid cysts and thier differential diagnosis PJ Vinken GW Bruyn (Eds) Handbook of clinical neurology congenital malformations of the brain and skull. Part II North-Holland Amsterdam 75–135
A Yokota H Kajiwara S Matsuoka Y Matsukado M Choux (1987) ArticleTitleClassification of dorsal midline cystic cavities in the posterior fossa (in Japanese) Nerv Syst Child 12 167–77
ML Barr (1948) ArticleTitleObservations on the foramen of Magendie in a series of human brains Brain 71 281–9 Occurrence Handle18099551 Occurrence Handle1:STN:280:DyaH1M%2FgtFWntQ%3D%3D
R Gonsette R Potvliege G Andre-Balisaux J Stenuit (1968) ArticleTitleMega-cisterna magna: clinical, radiographic anatomopathologic study Acta Neurol Psychiatr Belg 68 559–70 Occurrence Handle4976720 Occurrence Handle1:STN:280:DyaF1M7ot1Oisw%3D%3D
EG Robertson (1949) ArticleTitleDevelopmental defects of the cisterna magna and dura mater J Neurol Neurosurg Psychiatry 12 39–51 Occurrence Handle18111229 Occurrence Handle1:STN:280:DyaH1M%2Fht1yhtw%3D%3D
R Adam JO Greenberg (1978) ArticleTitleThe mega cisterna magna J Neurosurge 48 190–2 Occurrence Handle1:STN:280:DyaE1c7htVymsQ%3D%3D Occurrence Handle10.3171/jns.1978.48.2.0190
PI Yakovlev (1962) ArticleTitleMorphological criteria of growth and maturation of the nervous system in man Res Publ Assoc Nerv Ment Dis 39 3–46 Occurrence Handle1:STN:280:DyaF383gtVKgtA%3D%3D
Yakovlev PI. Telencephalon (1968) ArticleTitle“impar,” “semipar” and “totopar” (morphogenetic, tectogenetic and architectonic deffinitions) Int J Neurol 6 245–65
W DeMyer (1988) Neuroanatomy: national medical series for independent study EditionNumber2nd ed Williams & Willkins Baltimore
FP Probst (1979) The prosencephalies Springer Verlag Berlin
P Rakic PI Yakovlev (1968) ArticleTitleDevelopment of the corpus callosum and cavum septi in man J Comp Neurol 132 45–72 Occurrence Handle5293999 Occurrence Handle1:STN:280:DyaL1c3ksVSlsA%3D%3D Occurrence Handle10.1002/cne.901320103
RL Sidman P Rakic (1982) Development of the human central nervous system W Haymaker RD Adams (Eds) Histology and histopathology of the nervous system Charles C Thomas Springfield, IL 3–145
R O'Rahilly F Muller (1990) ArticleTitleVentricular system and choroid plexuses of the human brain during the embryonic period proper Am J Anat 189 285–302 Occurrence Handle2285038 Occurrence Handle10.1002/aja.1001890402
FP Probst (1973) ArticleTitleCongenital defect of the corpus callosum morphology and encephalographic appearances Acta Radiol [Diagn] (Suppl) 331 1–152 Occurrence Handle1:STN:280:DyaE2c%2Flt1OqsA%3D%3D
AJ Barkovich EM Simon CA Walsh (2001) ArticleTitleCallosal agenesis with cyst: a better understanding and new classification Neurology 56 220–7 Occurrence Handle11160959 Occurrence Handle1:STN:280:DC%2BD3M7pt1yhsw%3D%3D
PI Yakovlev (1959) ArticleTitlePathoarchitectonic studies of cerebral malformation. III. Arhinencephalies (holotelencephalies) J Neuropathol Exp Neurol 18 22–55 Occurrence Handle13621243 Occurrence Handle1:STN:280:DyaG1M%2FltVOrsA%3D%3D
A Yokota T Oota Y Matsukado (1984) ArticleTitleDorsal cyst malformation. Part I. Clinical study and critical review on definition of holoprosencephaly Child's Brain 11 320–41 Occurrence Handle6389035 Occurrence Handle1:STN:280:DyaL2M%2FltVCqsQ%3D%3D Occurrence Handle10.1159/000120194
W DeMyer (1963) ArticleTitleAlobar holoprosencephaly (arhinencephaly) with median cleft lip and palate: clinical, electroencephalographic and nosologic considerations Confin Neurol 23 1–39 Occurrence Handle14026941 Occurrence Handle1:STN:280:DyaF383hvV2hsA%3D%3D
Author information
Authors and Affiliations
Corresponding author
Additional information
This work was presented as an educational lecture at the 64th Japan Radiological Society meeting (April 2005) in Yokohama.
About this article
Cite this article
Utsunomiya, H., Yamashita, S., Takano, K. et al. Midline cystic malformations of the brain: imaging diagnosis and classification based on embryologic analysis. Radiat Med 24, 471–481 (2006). https://doi.org/10.1007/s11604-006-0049-7
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/s11604-006-0049-7