Hydrocephalus—Special Pathology

  • Reinhard L. Friede


The recognition of hydrocephalus dates back to ancient times (Dandy, 1920, review), but no insights were obtained into the pathogenesis of the disease until the physiology of flow of CSF, reviewed in Chapter 21, was understood. Disturbances in the flow of CSF may result from its altered secretion, circulation or reabsorption. A comprehensive review of the morbid anatomy of hydrocephalus by Russell (1949) led her to conclude that at least 99 percent of her cases had resulted from impaired circulation of CSF caused by mechanical obstructions of the CSF spaces. Neoplasia is a common cause of obstruction, but description of these lesions is beyond the scope of the present text and they are mentioned only occasionally for reference. The most common nonneoplastic types of obstructions are shown in Table 3 from the statistics of Ford (1926) and Gilles and Shillito (1970). The description of lesions causing hydrocephalus in this chapter is arranged as to follow the normal flow of CSF: The problem of overproduction of CSF by the choroid plexus is considered first; next, the various obstructive lesions within and outside of the ventricles; and, last, impaired mechanisms of CSF reabsorption.


Subarachnoid Space Superior Sagittal Sinus Aqueductal Stenosis Choroid Plexus Papilloma Arachnoid Granulation 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. Alksne, J. F., Lovings, E. T.: The role of the arachnoid villus in the removal of red blood cells from the subarachnoid space. An electron microscope study in the dog. T. Neurosurg. 36: 192–200, 1972.CrossRefGoogle Scholar
  2. Alksne, J. F., Lovings, E. T.: Functional ultrastructure of the arachnoid villus. Arch. Neurol. (Chic.) 27: 371–377, 1972.CrossRefGoogle Scholar
  3. Anderson, H., Carlsson, C.-A., Gomes, S. P.: Unilateral hydrocephalus. Neurochirurgie 17: 63–65. 1974.Google Scholar
  4. Bailey, O. J., Woodward, J. S.: Small vascular malformations of the brain: Their relationship to unexpected death, hydrocephalus and mental deficiency. J. Neuropath. exp. Neurol. 18: 98–108, 1959.PubMedCrossRefGoogle Scholar
  5. Berling, E. A., Jr., Salibi, B.: Production of hydrocephalus by increased cephalic-venous pressure. Arch. Neurol. (Chic.) 81: 693–698, 1959.Google Scholar
  6. Bickers, D. S., Adams, R. D.: Hereditary stenosis of the aqueduct of Sylvius as a cause of congenital hydrocephalus. Brain 72: 246–262, 1949.PubMedCrossRefGoogle Scholar
  7. Borit, A., Sidman, R. L.: New mutant mouse with communicating hydrocephalus and secondary aqueductal stenosis. Acta Neuropath. 21: 316–331, 1972.PubMedCrossRefGoogle Scholar
  8. Carton, C. A., Pascal, R. R.: Hydrocephalus and vitamin-A-deficiency in the rabbit: General considerations. In: Disorders of the Developing Nervous System (Fields, W. S., Desmond, M. M., eds.), chapter XI, pp. 214–266. Springfield, Ill.: Ch. C Thomas 1961.Google Scholar
  9. Clark, F. H.: Anatomical basis of hereditary hydrocephalus in the house mouse. Anat. Rec. 58: 225–233, 1934.CrossRefGoogle Scholar
  10. Clark, W. E. L.: On the Pacchionian bodies. J. Anat. 55: 40–48, 1920.Google Scholar
  11. Cloward, R. B.: Atresia of the lateral ventricle. J. Neurol. Neurosurg. Psychiat. 32: 624–629, 1969.PubMedCrossRefGoogle Scholar
  12. Cohlan, S. Q.: Congenital anomalies in the rat produced by excessive intake of vitamin A during pregnancy. Pediatrics 13: 556–567, 1954.PubMedGoogle Scholar
  13. Courville, C. B.: Obstructive internal hydrocephalus incident to small vascular anomaly of midbrain. Bull. Los Angeles neurol. Soc. 26: 41–45, 1961.PubMedGoogle Scholar
  14. Cronqvist, S., Granholm, L., Lundström, N.-R.: Hydrocephalus and congestive heart failure caused by intracranial arteriovenous malformations in infants. J. Neurosurg. 36: 249–254, 1972.PubMedCrossRefGoogle Scholar
  15. Cutler, R. W. P., Page, L., Galicich, J., Watters, G. V.: Formation and absorption of cerebrospinal fluid in man. Brain 91: 707–720, 1968.PubMedCrossRefGoogle Scholar
  16. Dandy, W. E.: Diagnosis and treatment of hydrocephalus resulting from stricture of the aqueduct of Sylvius. Surg. Gynec. Obstet. 31: 340–358, 1920.Google Scholar
  17. Dandy, W. E.: Hydrocephalus in chondrodystrophy. Bull. Johns Hopk. Hosp. 32: 5–10, 1921.Google Scholar
  18. Dandy, W. E., Blackfan, K. D.: Internal hydrocephalus. Amer. J. Dis. Child. 8: 406–482, 1914.Google Scholar
  19. Dooling, E. C., Barlow, J. F., Murphy, J. V., Richardson, E. P., Jr.: Cyst of the cavum septi pellucidi. Arch. Neurol. (Chic.) 27: 79–84, 1972.CrossRefGoogle Scholar
  20. Drachman, D. A., Richardson, E. P.: Aqueductal narrowing, congenital and acquired. A critical review of the histologic criteria. Arch. Neurol. (Chic.) 5: 552–559, 1961.CrossRefGoogle Scholar
  21. Edwards, J. H., Norman, R. M., Roberts, J. M.: Sex-linked hydrocephalus: report of a family with 15 affected members. Arch. Dis. Child. 36: 481–485, 1961.PubMedCrossRefGoogle Scholar
  22. Ekbom, K., Greitz, T., Kugelberg, E.: Hydrocephalus due to ectasia of the basilar artery. J. Neurol. Sci. 8: 465–477, 1968.CrossRefGoogle Scholar
  23. Ellington, E., Margolis, G.: Block of arachnoid villus by subarachnoid hemorrhage. J. Neurosurg. 30: 651–657, 1969.PubMedCrossRefGoogle Scholar
  24. Emery, J. L.: Intracranial effects of long-standing decompression of the brain in children with hydrocephalus and meningomyelocele. Develop. Med. Child. Neurol. 7: 302–309, 1965.PubMedCrossRefGoogle Scholar
  25. Emery, J. L., Staschak, M. C.: The size and form of the cerebral aqueduct in children. Brain 95: 591–598, 1972.PubMedCrossRefGoogle Scholar
  26. Emery, J. L., Zachary, R. B.: Hydrocephalus associated to obliteration of the longitudinal sinus. Arch. Dis. Child. 31: 288–292, 1956.PubMedCrossRefGoogle Scholar
  27. Fishman, M. A., Hogan, G. R., Dodge, P. R.: The concurrence of hydrocephalus and craniosynostosis. J. Neurosurg. 34: 621–629, 1971.PubMedCrossRefGoogle Scholar
  28. Ford, F. R.: Cerebral birth injuries and their results. Medicine 5: 121–194, 1926.CrossRefGoogle Scholar
  29. Friede, R. L.: Surface structures of the aqueduct and the ventricular walls: a morphologic, comparative and histochemical study. J. comp. Neurol. 116: 229–247, 1961.PubMedCrossRefGoogle Scholar
  30. Gardner, W. J., Spitler, D. K., Whitten, C.: Increased intracranial pressure caused by increased protein content in the cerebrospinal fluid. New Engl. J. Med. 250: 932–936, 1954.Google Scholar
  31. Gilles, F. H., Davidson, R. I.: Communicating hydrocephalus associated with deficient dysplastic parasagittal arachnoidal granulations. J. Neurosurg. 35: 421–426, 1971.PubMedCrossRefGoogle Scholar
  32. Gilles, F. H., Shillito, J.: Infantile hydrocephalus: retrocerebellar subdural hematoma. J. Pediat. 76: 529–537, 1970.PubMedCrossRefGoogle Scholar
  33. Gomez, D. G., Potts, G., Deonarine, V., Reilly, K.: Effect of pressure gradient changes on the morphology of arachnoid villi and granulations in the monkey. Lab. Invest. 28: 648–657, 1973.PubMedGoogle Scholar
  34. Green, M.: The developmental effects of congenital hydrocephalus (ch) in the mouse. Develop. Biol. 23: 585–608, 1970.PubMedCrossRefGoogle Scholar
  35. Grüneberg, H.: Genetic studies on the skeleton of the mouse. VII. Congenital hydrocephalus. J. Genet. 51: 327–358, 1953.CrossRefGoogle Scholar
  36. Guleke, N.: Über die Entstehung des Hydrocephalus internus. Arch. klin. Chirurgie 162: 533–550, 1930.Google Scholar
  37. Gutierrez, Y., Friede, R. L., Kaliney, W. J.: Agenesis of arachnoid granulations and its relationship to communicating hydrocephalus. J. Neurosurg., in press.Google Scholar
  38. Harris, P.: Chronic progressive communicating hydrocephalus due to protein transudates from brain and spinal tumors. Develop. Med. Child. Neurol. 4: 270–278, 1962.PubMedCrossRefGoogle Scholar
  39. Henschen, F.: Tumoren des Zentralnervensystems und seiner Hüllen. In: Handbuch der speziellen pathologischen Anatomie und Histologie (Lubarsch, Henke, and Rössle, eds.), 13/3, pp. 413–1083. Berlin-Göttingen-Heidelberg: Springer 1955.Google Scholar
  40. Hogan, G. R., Bauman, M. L.: Hydrocephalus in Alperts’ syndrome. J. Pediat. 79: 782–787, 1971.PubMedCrossRefGoogle Scholar
  41. Hollingsworth, G., Davson, H., Segal, M. B.: The mechanism of drainage of the cerebrospinal fluid. Brain 93: 665–678, 1970.PubMedCrossRefGoogle Scholar
  42. Holmes, L. B., Nash, A., Zu Rhein, G. M., Levin, M., Opitz, J. M.: X-linked aqueductal stenosis: clinical and neuropathological findings in two families. Pediatrics 51: 697–704, 1973.PubMedGoogle Scholar
  43. Howell, D. A.: Upper brain-stem compression and foraminal impaction with intracranial space-occupying lesions and brain swelling. Brain 82: 525–550, 1959.PubMedCrossRefGoogle Scholar
  44. Jellinger, K., Grunert, V., Sunder-Plassman, M.: Choroid-plexus papilloma associated with hydrocephalus in infancy. Report of two cases. Neuropädiatrie 1: 344–348, 1970.PubMedCrossRefGoogle Scholar
  45. Jezequel, Ch., Belloir, D., Jehan, P., et al.: Congenital hydrocephalus. Bilateral abnormality of the thumbs and eye changes. Semaines Hopitaux 49: 927–934, 1973.Google Scholar
  46. Johnson, R. T., Johnson, K. P.: Hydrocephalus as a sequela of experimental myxovirus infections. Exp. Molec. Path. 10: 68–80, 1969.PubMedCrossRefGoogle Scholar
  47. Kahn, E. A., Luros, J. T.: Hydrocephalus from overproduction of cerebrospinal fluid. J. Neurosurg. 9: 59–67, 1952.PubMedCrossRefGoogle Scholar
  48. Keene, M. F. L., Hewer, E. E.: The sub-commissural organ and mesocoelic recess in the human brain, together with a note on Reissner’s fibers. J. Anat. 69: 501–507, 1935.PubMedGoogle Scholar
  49. Kepes, J. J., Clough, C., Villanueva, A.: Congenital fusion of the thalami (atresia of the third ventricle) and associated anomalies in a 6 months old infant. Acta Neuropath. 13: 97–104, 1969.PubMedCrossRefGoogle Scholar
  50. Laurence, V. M.: Discussion. In: Ciba Foundation Symposium on Cerebrospinal Fluid (Wolstenholm, G. E., O’Conner, C. M., eds.), pp. 310–326. Boston, Mass.: Little, Brown and Co. 1958.Google Scholar
  51. McDonald, J. V.: Persistent hydrocephalus following the removal of papillomas of the choroid plexus of the lateral ventricles. Report of two cases. J. Neurosurg. 30: 736–740, 1969.PubMedCrossRefGoogle Scholar
  52. MacFarlane, A., Maloney, A. F. J.: The appearance of the aqueduct and its relationship to hydrocephalus in the Arnold-Chiari malformation. Brain 80: 479–491, 1957.PubMedCrossRefGoogle Scholar
  53. Marie, J., See, G.: Hydrocéphalie aigue bénigne du nourrisson après ingestion d’une dose massive unique de vitamine A. Ann. Pediat. (Paris) 180: 308–314, 1953.Google Scholar
  54. Martins, A. N., Wiley, J. K., Myers, P. W.: Dynamics of the cerebrospinal fluid and the spinal dura mater. J. Neurol. Neurosurg. Psychiat. 35: 468–473, 1972.PubMedCrossRefGoogle Scholar
  55. Matson, D. D., Crofton, F. D. L.: Papilloma of the choroid-plexus in childhood. J. Neurosurg. 17: 1002–1027, 1960.PubMedCrossRefGoogle Scholar
  56. Milhorat, T. H., Hammock, M. K., Chandra, R. S.: The subarachnoid space in congenital obstructive hydrocephalus. Part 2. Microscopic findings. J. Neurosurg. 35: 7–15, 1971.PubMedCrossRefGoogle Scholar
  57. Millen, J. W., Woollam, D. H. M.: Vitamins and the cerebrospinal fluid. In: Ciba Foundation Symposium on the Cerebrospinal Fluid, pp. 168–188. Boston, Mass.: Little, Brown and Co. 1958.Google Scholar
  58. Morley, J. B., Reynolds, E. H.: Papilloedema and the Landry-Uuillain-Barré syndrome. Brain 89: 205–222, 1966.PubMedCrossRefGoogle Scholar
  59. Morrice, G., Havenes, W. H., Kapelansky, F.: Vitamin A intoxication as a cause of pseudotumor cerebri. J.A.M.A. 173: 1802–1805, 1960.PubMedCrossRefGoogle Scholar
  60. Needleman, H. L., Root, A. W.: Sex-linked hydrocephalus. Pediatrics 31: 396–399, 1963.PubMedGoogle Scholar
  61. Overholser, M. D., Whitley, J. R., O’Dell, B. L., Hogan, A. G.: The ventricular system in hydrocephalic rat brains produced by a deficiency of vitamin B12 or of folic acid in the maternal diet. Anat. Rec. 120: 917–933, 1954.PubMedCrossRefGoogle Scholar
  62. Pudenz, R. H., Russell, F. E., Agnew, W. F.: Experimental hydrocephalus: a historical summary. Bull. Los Angeles neurol. Soc. 25: 118–129, 1960.Google Scholar
  63. Ray, B. S., Peck, F. C.: Papilloma of the choroid plexus in the lateral ventricles causing hydrocephalus in an infant. J. Neurosurg. 13: 405–410, 1956.Google Scholar
  64. Rowbotham, G. F.: Small aneurysm completely obstructing lower end of aqueduct of Sylvius. Arch. Neurol. Psychiat. (Chic.) 40: 1241–1243, 1938.Google Scholar
  65. Russell, D. S.: Observations on the Pathology of Hydrocephalus. Medical Res. Council, Special Report Series No. 265. London: HMSO 1949.Google Scholar
  66. Russell, D. S., Donald, C.: Mechanism of internal hydrocephalus in spina bifida. Brain 58: 203–215, 1935.CrossRefGoogle Scholar
  67. Shabo, A. L., Maxwell, D. S.: Electron microscopic observations on the fate of particulate matter in the cerebrospinal fluid. J. Neurosurg. 29: 464–474, 1968.CrossRefGoogle Scholar
  68. Siegel, N. J., Spackman, T. J.: Chronic hypervitaminosis A with intracranial hypertension and low cerebrospinal fluid concentration of protein. Two illustrative cases. Clin. Pediat. (Phila.) 11: 580–584, 1972.CrossRefGoogle Scholar
  69. Simmonds, J. W.: The subarachnoid space: some experimental approaches to its pathology. Med. J. Aust. 19: 452–456, 1953.Google Scholar
  70. Stempak, J. G.: Etiology of trypan blue induced antenatal hydrocephalus in the albino rat. Anat. Rec. 148: 561–571, 1964.PubMedCrossRefGoogle Scholar
  71. Symonds, C. P.: Otitic hydrocephalus. Brain 54: 55–71, 1931.CrossRefGoogle Scholar
  72. Turkewitsch, N.: Die Entwicklung des Aquaeductus cerebri des Menschen. P. f. Morphologie u. mikroskop. Anat. 76: 421–447, 1935.Google Scholar
  73. Turnbull, I. M., Drake, C. G.: Membranous occlusion of the aqueduct of Sylvius. J. Neurosurg. 24: 24–36, 1966.CrossRefGoogle Scholar
  74. Turner, L.: The structure of arachnoid granulations with observations on their physiological and pathological significance. Ann. roy. Coll. Surg. Eng. 29: 237–264, 1961.PubMedGoogle Scholar
  75. Vigouroux, A.: Ecoulement de liquide céphalo-rachidien. Hydrocéphalie papillome de plexus choroids du IVe ventricle. Rev. Neurol. 16: 281–285, 1908.Google Scholar
  76. Warren, M. C., Lu, A. T., Ziering, W. H.: Sex-linked hydrocephalus with aqueductal stenosis. J. Pediat. 63: 1104–1110, 1963.PubMedCrossRefGoogle Scholar
  77. Weed, L. H.: Studies on the cerebrospinal fluid III. The pathways of escape from the subarachnoid spaces with particular reference to the arachnoid villi. J. Med. Res. 31: 51–91, 1914.PubMedGoogle Scholar
  78. Weed, L. H.: Development of the cerebrospinal spaces in man. In: Contributions to Embryology, Vol. V, No. 14. Carnegie Institute of Washington, Pub. 1917.Google Scholar
  79. Weed, L. H.: An anatomical consideration of the cerebrospinal fluid. Anat. Rec. 12: 461–496, 1917.CrossRefGoogle Scholar
  80. Welch, K., Friedman, V.: The cerebrospinal fluid valves. Brain 83: 454–469, 1960.PubMedCrossRefGoogle Scholar
  81. Pollay, M.: Perfusion of particles through arachnoid villi of the monkey. Amer. J. Physiol. 201: 651–654, 1961.PubMedGoogle Scholar
  82. Pollay, M.: The spinal arachnoid villi of the monkeys Cercopithecus aethiops sabaeus and Macaca irus. Anat. Rec. 145: 43–48, 1963.PubMedCrossRefGoogle Scholar
  83. Wise, B. L., Sondheimer, F., Kaufman, S.: Achondroplasia and hydrocephalus. Neuropädiatrie 3: 106–113, 1971.PubMedCrossRefGoogle Scholar
  84. Wisniewski, H., Weller, R. O., Terry, R. D.: Experimental hydrocephalus produced by the subarachnoid infusion of silicone oil. J. Neurosurg. 31: 10–14, 1969.PubMedCrossRefGoogle Scholar
  85. Woodard, J. C., Newberne, P. M.: The pathogenesis of hydrocephalus in newborn rats deficient in vitamin B12. J. Embryol. exp. Morph. 17: 117–187, 1967.Google Scholar
  86. Woollam, D. H. M., Millen, J. W.: Anatomical considerations in the pathology of stenosis of the cerebral aqueduct. Brain 76: 104–112, 1953.PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Wien 1975

Authors and Affiliations

  • Reinhard L. Friede
    • 1
    • 2
  1. 1.Case Western Reserve UniversityClevelandUSA
  2. 2.University of ZurichSwitzerland

Personalised recommendations