Hydrocephalus — Basic Concepts and General Pathology

  • Reinhard L. Friede


The term hydrocephalus, in its broadest sense, means an increased amount of fluid in the CSF spaces, particularly in the cerebral ventricles, as opposed to local accumulations of fluid in subdural hygromas, arachnoid cysts, or within tissue defects. Enlarged cerebral ventricles may result from their distension by increased pressure, or from atrophy caused by the loss of cells. It is customary to distinguish “increased pressure hydrocephalus” from “hydrocephalus e vacuo”. It needs to be emphasized from the start that these two mechanisms of ventricular dilation are not mutually exclusive and that they may combine and interact. For example, long-standing distension of the cerebral ventricles because of increased pressure eventually causes tissue damage and hemispheric atrophy. Conversely, massive destruction of the hemispheres, such as in hydranencephaly (cf. Chap. 3), may become superimposed with secondary impairment of CSF circulation, causing pressure hydrocephalus.


General Pathology Primary Ciliary Dyskinesia Cerebral Ventricle Slit Ventricle Syndrome Hydrocephalic Brain 
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  1. Adam H (1953) Kugelförmige Pigmentzellen als Anzeiger der Liquorströmung in den Gehirnventrikeln von Krallenfroschlarven. Z Naturforsch (B) 8:250–258Google Scholar
  2. Adams RD, Fisher CM, Hakim S, Ojemann RG, Sweet WH (1965) Symptomatic occult hydrocephalus with “normal” cerebrospinal-fluid pressure. A treatable syndrome. N Engl J Med 273:117–126PubMedCrossRefGoogle Scholar
  3. Adolph RJ, Fukusumi H, Fowler NO (1967) Origin of cerebrospinal fluid pulsations. Am J Physiol 212:840–846PubMedGoogle Scholar
  4. Alvord EC Jr (1961) The pathology of hydrocephalus. In: Fields WS, Desmond MM (eds) Disorders of the developing nervous system, Chap XIV. Thomas, Springfield, Ill, pp 343–412Google Scholar
  5. Bachs A, Walker AE (1953) Experimental hydrocephalus. J Neuropathol Exp Neurol 12:283–292PubMedCrossRefGoogle Scholar
  6. Bakay L Jr (1947) Phylogenesis of the perivascular spaces of the brain. Nature 160:789–790PubMedCrossRefGoogle Scholar
  7. Bering EA Jr (1955) Choroid plexus and arterial pulsation of cerebrospinal fluid. Arch Neurol Psychiatry 73:165–172Google Scholar
  8. Bering EA Jr (1962) Circulation of the cerebrospinal fluid. Demonstration of the choroid plexuses as the generator of the force for flow of fluid and ventricular enlargement. J Neurosurg 19:405–413PubMedCrossRefGoogle Scholar
  9. Bering EA Jr, Sato O (1963) Hydrocephalus: changes in formation and absorption of cerebrospinal fluid within the cerebral ventricles. J Neurosurg 20:1050–1063PubMedCrossRefGoogle Scholar
  10. Boulay GH du (1966) Pulsatile movements in the CSF pathways. Br J Radiol 39:255–262PubMedCrossRefGoogle Scholar
  11. Brightman MW (1967) Movement within the brain of ferritin injected into the cerebrospinal fluid compartments. In: Klatzo I, Seitelberger F (eds) Brain edema. Springer, Wien New York, pp 271–284Google Scholar
  12. Brightman MW (1968) The intracerebral movement of proteins injected into blood and cerebrospinal fluid of mice. In: Lajtha A, Ford DH (eds) Brain barrier systems. Progress in Brain Research 29:19–37CrossRefGoogle Scholar
  13. Brightman MW, Reese TS (1969) Junctions between intimately apposed cell membranes in the vertebrate brain. J Cell Biol 40:648–677PubMedCrossRefGoogle Scholar
  14. Cathcart III RS, Worthington WC Jr (1964) Ciliary movement in the rat cerebral ventricles: clearing action and directions of currents. J Neuropathol Exp Neurol 23:609–618PubMedCrossRefGoogle Scholar
  15. Cutler RW, Deuel RK, Barlow CF (1967) Albumin exchange between plasma and cerebrospinal fluid. Arch Neurol 17:261–270PubMedGoogle Scholar
  16. Cutler RWP, Murray JE, Moody RA (1973) Overproduction of cerebrospinal fluid in communicating hydrocephalus. A case report. Neurology 23:1–5Google Scholar
  17. Dandy WE, Blackfan KD (1914) Internal hydrocephalus. An experimental, clinical and pathological study. Am J Dis Child 8:406–482Google Scholar
  18. Davson H (1967) Physiology of the cerebrospinal fluid. Churchill, LondonGoogle Scholar
  19. Del Bigio MR, Bruni JE, Fewer HD (1985) Human neonatal hydrocephalus. An electron microscopic study of the periventricular tissue. J Neurosurg 63:56–63PubMedCrossRefGoogle Scholar
  20. Di Rocco C, Di Trapani G, Pettorossi VE, Caldarelli M (1979) On the pathology of experimental hydrocephalus induced by artificial increase in endoventricular CSF pulse pressure. Childs Brain 5:81–95PubMedGoogle Scholar
  21. Dohrmann GJ (1971) The choroid plexus in experimental hydrocephalus. Alight and electron microscopic study in normal, hydrocephalic, and shunted hydrocephalic dogs. J Neurosurg 34:56–69PubMedCrossRefGoogle Scholar
  22. Dyke CG, Davidoff LM (1939) An explanation for the ribbing seen in the walls of dilated cerebral ventricles. Yale J Biol Med 11:485–486PubMedGoogle Scholar
  23. Edvinsson L, West KA (1971) Relation between intracranial pressure and ventricular size at various stages of experimental hydrocephalus. Acta Neurol Scand 47:451–457PubMedCrossRefGoogle Scholar
  24. Emery JL (1965) Intracranial effects of long-standing decompression of the brain in children with hydrocephalus and meningomyelocele. Dev Med Child Neurol 7:302–309PubMedCrossRefGoogle Scholar
  25. Fishman RA, Greer M (1963) Experimental obstructive hydrocephalus. Changes in the cerebrum. Arch Neurol 8:156–161PubMedGoogle Scholar
  26. Friede R (1955) Untersuchungen an flimmerndem Ependym in Kultur. Arch Psychiatr Nervenkr 193:295–302CrossRefGoogle Scholar
  27. Friede RL (1962) A quantitative study of myelination in hydrocephalus. (Factors controlling glial proliferation in myelination.) J Neuropathol Exp Neurol 21:645–648PubMedCrossRefGoogle Scholar
  28. Friede RL, Hu KH (1971) A new approach for determining the volume of cerebral extracellular fluid and demonstration of its communication with CSF. J Physiol (Lond) 218:477–493Google Scholar
  29. Gonzales-Darder J, Barbera J, Cerda-Nicolas M, Segura D, Broseta J, Barcia-Salorio JL (1984) Sequential morphological and functional changes in kaolin-induced hydrocephalus. J Neurosurg 61:918–924CrossRefGoogle Scholar
  30. Granholm L (1966) Induced reversibility of ventricular dilatation in experimental hydrocephalus. Acta Neurol Scand 42:581–588PubMedCrossRefGoogle Scholar
  31. Greenstone MA, Jones RWA, Dewar A, Neville BGR, Cole PJ (1984) Hydrocephalus and primary ciliary dyskinesia. Arch Dis Child 59:481–482PubMedCrossRefGoogle Scholar
  32. Grundy HF (1962) Circulation of cerebrospinal fluid in the spinal region of the cat. J Physiol (Lond) 163:457–465Google Scholar
  33. Harris LS, Roessmann U, Friede RL (1968) Bursting of cerebral ventricular walls. J Pathol 96:33–38CrossRefGoogle Scholar
  34. Hayden P, Shurtleff DB, Foltz EL (1970) Ventricular fluid pressure recordings in hydrocephalic patients. Arch Neurol 23:147–154PubMedGoogle Scholar
  35. Hesdorffer MB, Scammon RE (1935) Growth of human nervous system. I. Growth of cerebral surface. Proc Soc Exp Biol 33:415–418Google Scholar
  36. Hild W (1957) Ependymal cells in tissue culture. Z Zellforsch 46:259–271PubMedCrossRefGoogle Scholar
  37. Hirayama A (1982) Slit ventricle. A reluctant goal of ventriculoperitoneal shunt. Monogr Neural Sci 8:108–111Google Scholar
  38. Hochwald GM, Sahar A, Sadik AR, Ransohoff J (1969) Cerebrospinal fluid production and histological observations in animals with experimental obstructive hydrocephalus. Exp Neurol 25:190–199PubMedCrossRefGoogle Scholar
  39. Hochwald GM, Lux WE Jr, Sahar A, Ransohoff J (1972) Experimental hydrocephalus. Changes in cerebrospinal fluid dynamics as a function of time. Arch Neurol 26:120–129PubMedGoogle Scholar
  40. James AE Jr, Burns B, Flor WF, Strecker E-P, Merz T, Bush M, Price DL (1975) Pathophysiology of chronic communicating hydrocephalus in dogs (canis familiaris). J Neurol Sci 24:151–178PubMedCrossRefGoogle Scholar
  41. James AE, McComb JG, Christian J, Davson H (1976) The effect of cerebrospinal fluid pressure on the size of drainage pathways. Neurology 26:659–663PubMedGoogle Scholar
  42. James AE Jr, Novak GR, Strecker E-P, Flor WJ (1977) The central canal of the spinal cord in experimental hydrocephalus: preliminary results. Radiology 125:417–420PubMedGoogle Scholar
  43. Jones HC (1978) Continuity between the ventricular and subarachnoid cerebrospinal fluid in an amphibian, Rana pipiens. Cell Tissue Res 195:153–167PubMedCrossRefGoogle Scholar
  44. Kaufman B, Sandstrom PH, Young HF (1970) Alteration in size and configuration of the sella turcica as the result of prolonged cerebrospinal fluid shunting. Radiology 97:537–542PubMedGoogle Scholar
  45. Kaufman B, Weiss MH, Young HF, Nulsen FE (1973) Effects of prolonged cerebrospinal fluid shunting on the skull and brain. J Neurosurg 38:288–297PubMedCrossRefGoogle Scholar
  46. Laurence KM (1969) Neurological and intellectual sequelae of hydrocephalus. Arch Neurol 20:73–81PubMedGoogle Scholar
  47. Lindberg L-A, Vasenius L,Talanti S (1977) The surface fine structure of the ependymal lining of the lateral ventricle in rats with hereditary hydrocephalus. Cell Tissue Res 179:121–129PubMedGoogle Scholar
  48. Lindvall M, Owman C (1984) Sympathetic nervous control of cerebrospinal fluid production in experimental obstructive hydrocephalus. Exp Neurol 84:606–615PubMedCrossRefGoogle Scholar
  49. Lorenzo AV, Page LK, Watters GV (1970) Relationship between cerebrospinal fluid formation, absorption and pressure in human hydrocephalus. Brain 93:679–692PubMedCrossRefGoogle Scholar
  50. Lux WE Jr, Hochwald GM, Sahar A, Ransohoff J (1970) Periventricular water content. Effect of pressure in experimental chronic hydrocephalus. Arch Neurol 23:475–479PubMedGoogle Scholar
  51. Marlin AE, Wald A, Hochwald GM, Malhan C (1978) Kaolin-induced hydrocephalus impairs CSF secretion by the choroid plexus. Neurology 28:945–949PubMedGoogle Scholar
  52. McAllister II JP, Maugans TA, Shah MV, Truex RC Jr (1985) Neuronal effects of experimentally induced hydrocephalus in newborn rats. J Neurosurg 63:776–783PubMedCrossRefGoogle Scholar
  53. Mortensen OA, Weed LH (1934) Absorption of isotonic fluids from the subarachnoid space. Am J Physiol 108:458–468Google Scholar
  54. Muller J (1983) Congenital malformations of the brain. In: Rosenberg RN (ed)The clinical neurosciences, vol 3: Neuropathology. Churchill Livingstone, New York Edinburgh London Melbourne, pp III:1–III:33Google Scholar
  55. Nakagawa Y, Cervos-Navarro J, Artigas J (1984) A possible paracellular route for the resolution of hydrocephalic edema. Acta Neuropathol (Berl) 64:122–128CrossRefGoogle Scholar
  56. Nulsen FE, Spitz EB (1952) Treatment of hydrocephalus by direct shunt from ventricle to jugular vein. Surg Forum 2:399–403Google Scholar
  57. Ogata J, Hochwald GM, Cravioto H, Ransohoff J (1972) Distribution of intraventricular horseradish peroxidase in normal and hydrocephalic cat brains. J Neuropathol Exp Neurol 31:454–463PubMedCrossRefGoogle Scholar
  58. Oi S, Matsumoto S (1985) Slit ventricles as a cause of isolated ventricles after shunting. Childs Nerv Syst 1:189–193PubMedCrossRefGoogle Scholar
  59. O’Rahilly R, Müller F (1986) The meninges in human development. J Neuropathol Exp Neurol 45:588–608PubMedCrossRefGoogle Scholar
  60. Paisley WJ, Ouvrier RA, Johnston I, Jones RFC, Sofer-Schreiber M, Silva M de (1982) Chronic spinal arachnoiditis in childhood. Dev Med Child Neurol 24:798–807PubMedCrossRefGoogle Scholar
  61. Penfield WL, Elvidge AR (1932) Hydrocephalus and the atrophy of cerebral compression. In: Penfield W (ed) Cytology and cellular pathology of the nervous system, vol 3, section 28. Hafner, New York, pp 1203–1217 (Fascimile of 1932 edition, reprinted 1965)Google Scholar
  62. Portnoy HD, Branch C, Chopp M (1985) The CSF pulse wave in hydrocephalus. Childs Nerv Syst 1:248–254PubMedCrossRefGoogle Scholar
  63. Rall DP, Oppelt WW, Patlak CS (1962) Extracellular space of brain as determined by diffusion of inulin from the ventricular system. Life Sci 1:43–48CrossRefGoogle Scholar
  64. Rijssel TG van (1946) Circulation of cerebrospinal fluid in carassius gibelio. Arch Neurol Psychiatry 56:522–543Google Scholar
  65. Rosenberg GA, Saland L, Kyner WT (1983) Pathophysiology of periventricular tissue changes with raised CSF pressure in cats. J Neurosurg 59:606–611PubMedCrossRefGoogle Scholar
  66. Rubin RC, Hochwald GM, Liwnicz B,Tiell M, Mizutani H, Shulman K (1972) The effect of severe hydrocephalus on size and number of brain cells. Dev Med Child Neurol 14, Suppl 27:117–120Google Scholar
  67. Russell DS (1949) Observations on the pathology of hydrocephalus. Medical Research Council Special Report Series No. 265. Her Majesty’s Stationery Office, London, 4th impression 1968Google Scholar
  68. Sahar A, Hochwald GM, Ransohoff J (1969) Alternate pathway for cerebrospinal fluid absorption in animals with experimental obstructive hydrocephalus. Exp Neurol 25:200–206PubMedCrossRefGoogle Scholar
  69. Scammon RE, Hesdorffer MB (1935) Growth of human nervous system. II. Indices of relation of cerebral volume to surface in developmental period. Proc Soc Exp Biol 33:418–421Google Scholar
  70. Scarff JE (1952) Nonobstructive hydrocephalus. Treatment by endoscopic cauterization of choroid plexus. Long term results. J Neurosurg 9:164–176PubMedCrossRefGoogle Scholar
  71. Serlo W, Heikkinen E, Saukkonen A-L, Wendt L v (1985) Classification and management of the slit ventricle syndrome. Childs Nerv Syst 1:194–199PubMedCrossRefGoogle Scholar
  72. Shenkin HA, Perryman CR (1946) Reversibility of cerebral ventricular dilatation. J Neurosurg 3:234–238PubMedCrossRefGoogle Scholar
  73. Torvik A, Stenwig AE (1977) The pathology of experimental obstructive hydrocephalus. Electron microscopic observations. Acta Neuropathol (Berl) 38:21–26PubMedCrossRefGoogle Scholar
  74. Van Harreveld A (1966) Brain tissue electrolytes. Butterworth, WashingtonGoogle Scholar
  75. Wakai S, Nagai M (1984) Ventricular diverticulum. J Neurol Neurosurg Psychiatry 47:514–517PubMedCrossRefGoogle Scholar
  76. Weed LH (1917) The development of the cerebro-spinal spaces in pig and in man. Contributions to Embryology 5, No. 14. Carnegie Institute, WashingtonGoogle Scholar
  77. Weiler RO, Williams BN (1975) Cerebral biopsy and assessment of brain damage in hydrocephalus. Arch Dis Child 50:763–768CrossRefGoogle Scholar
  78. Weiler RO, Wisniewski H (1969) Histological and ultrastructural changes with experimental hydrocephalus in adult rabbits. Brain 92:819–828CrossRefGoogle Scholar
  79. Weiler RO, Wisniewski H, Shulman K, Terry RD (1971) Experimental hydrocephalus in young dogs: histological and ultrastructural study of the brain tissue damage. J Neuropathol Exp Neurol 30:613–626CrossRefGoogle Scholar
  80. White DN, Wilson KC, Curry GR, Stevenson RJ (1979) The limitation of pulsatile flow through the aqueduct of Sylvius as a cause of hydrocephalus. J Neurol Sci 42:11–51PubMedCrossRefGoogle Scholar
  81. Worthington WC Jr, Cathcart III RS (1963) Ependymal cilia: distribution and activity in the adult human brain. Science 139:221–222PubMedCrossRefGoogle Scholar
  82. Yakovlev PI (1947) Paraplegias in hydrocephalics. Am J Ment Defic 51:561–576PubMedGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 1989

Authors and Affiliations

  • Reinhard L. Friede
    • 1
  1. 1.Zentrum Neurologische Medizin, Abteilung NeuropathologieGeorg-August-Universität GöttingenGöttingenGermany

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