Advertisement

Hydrocephalus and Aqueductal Stenosis

  • Giuseppe Cinalli
  • Pietro Spennato
  • Emilio Cianciulli
  • Maria d’Armiento
Chapter

Abstract

The cerebral aqueduct was described by Vesalius in 1542 as a short tube “so that the animal spirit may flow continuously into the dorsal marrow” [32]. Actually, the aqueduct of Sylvius connects the third and fourth ventricle: it is a narrow, irregular channel, situated in the dorsal midbrain, with posterior commissure and lamina tecti behind, oculomotor and trochlear nerves nuclei, and medial longitudinal fasciculus and red nuclei in front. It forms a gentle concave curve to the base of the skull and is surrounded by the periaqueductal gray matter. In fixed brain specimens two areas of relative constriction have been found: one is at the level of the superior colliculus, the other at the level of the intercollicular sulcus; in cross section the lumen is highly changeable, probably owing to the influence of different nuclear masses or fiber tracts surrounding it at different levels. The lumen is usually triangular with the apex directed ventrally at the two constrictions and ovoid in the central dilated area, which has been referred to as the ampulla of the aqueduct or the ventricle of the midbrain [8].

Keywords

Fourth Ventricle Obstructive Hydrocephalus Aqueductal Stenosis Shunt Malfunction Congenital Hydrocephalus 
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.

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. 1.
    Alvord EC: The pathology of hydrocephalus. In: Fields WS, Desmond MM (ed) Disorders of the developing nervous system. Thomas, Springfield, pp 343–419, 1961Google Scholar
  2. 2.
    Anderson B: Relief of akinetic mutism from obstructive hydrocephalus using bromocriptine and ephedrine. J Neurosurg 76: 152–155, 1992PubMedCrossRefGoogle Scholar
  3. 3.
    Atlas SW, Mark AS, Fram EK: Aqueductal stenosis: evaluation with gradient-echo rapid MR imaging. Radiology 169: 449–453, 1988PubMedGoogle Scholar
  4. 4.
    Avman N, Gökalp HZ, Arasil E, et al: Symptomatology, evaluation and treatment of aqueductal stenosis. Neurol Res 6: 194–198, 1984PubMedGoogle Scholar
  5. 5.
    Azar-Kia B, Palacios E, Churchil R: Aqueductal stenosis and Parinaud’s syndrome. Illinois Med J 148: 532–533, 1975Google Scholar
  6. 6.
    Baloh RW, Furman JM, Yee RD: Dorsal midbrain syndrome: clinical and oculographic findings. Neurology 35: 54–60, 1985PubMedCrossRefGoogle Scholar
  7. 7.
    Barrer SJ, Schut L, Bruce DA: Global rostral midbrain dysfunction secondary to shunt malfunction and hydrocephalus. Neurosurgery 7: 322–325, 1980PubMedCrossRefGoogle Scholar
  8. 8.
    Beckett RS, Netsky MG, Zimmerman HM: Developmental stenosis of the aqueduct of Sylvius. Am J Pathol 26: 755–787, 1950PubMedGoogle Scholar
  9. 9.
    Berger L, Gauthier S, Leblanc R: Akinetic mutism and parkinsonism associated with obstructive hydrocephalus. Can J Neurol Sci 12: 255–258, 1958Google Scholar
  10. 10.
    Bering EA: Choroid plexus and arterial pulsation of the choroid plexus as a cerebrospinal fluid pump. Arch Neurol Psychiatr 73: 165–172, 1955CrossRefGoogle Scholar
  11. 11.
    Bickers DS, Adams RD: Hereditary stenosis of aqueduct of Sylvius as a cause of congenital hydrocephalus. Brain 72: 246–262, 1949PubMedCrossRefGoogle Scholar
  12. 12.
    Blackmore CC, Mamourian AC: Aqueduct compression from venous angioma: MR findings. AJNR Am J Neuroradiol 17: 458–460, 1996PubMedGoogle Scholar
  13. 13.
    Bleasel AF, Ell JJ, Johnston I: Pretectal syndrome and ventricular shunt dysfunction. Neuro-Ophthalmol 12: 193–196, 1992CrossRefGoogle Scholar
  14. 14.
    Bourneville, Noir J: Hydrocephalie. Prog Med Paris 12: 17–23, 1900Google Scholar
  15. 15.
    Bradley WG, Cortman KE, Burgoine B: Flowing cerebrospinal fluid in normal and hydrocephalic states: appearance on MR images. Radiology 159: 601–616, 1986Google Scholar
  16. 16.
    Büttner-Ennever JA, Büttner U, Cohen B, et al: Vertical gaze paralysis and the rostral interstitial nucleus of the medial longitudinal fasciculus. Brain 105: 125–149, 1982PubMedCrossRefGoogle Scholar
  17. 17.
    Cabezudo JM, Vaquero J, Garcia-de-Sola R, et al: Direct communication between the lateral ventricle and the frontal sinus as a cause of CSF rhinorrhea in aqueductal stenosis. Acta Neurochir 57: 95–98, 1981CrossRefGoogle Scholar
  18. 18.
    Cairns H, Oldfield RC, Pennybacker JB, et al: Akinetic mutism with an epidermoid cyst of the third ventricle. Brain 64: 273–290, 1941CrossRefGoogle Scholar
  19. 19.
    Ceddia A, Di Rocco C, Iannelli A, et al: Idrocefalo neonatale ad eziologia non tumorale. Minerva Pediatr 44: 445–450, 1992PubMedGoogle Scholar
  20. 20.
    Chapman PH: Indolent gliomas of the midbrain tectum. Concepts Pediatr Neurosurg 10: 97–107, 1990Google Scholar
  21. 21.
    Chatta AS, Delong GR: Sylvian aqueduct syndrome as a sign of acute obstructive hydrocephalus in children. J Neurol Neurosurg Psychiatry 38: 288–296, 1975CrossRefGoogle Scholar
  22. 22.
    Cinalli G, Sainte-Rose C, Simon I, et al: Sylvian aqueduct syndrome and global rostral midbrain dysfunction associated to shunt malfunction. J Neurosurg 90: 227–236, 1999PubMedCrossRefGoogle Scholar
  23. 23.
    Citrin CM, Sherman JL, Gangarosa RE, et al: Physiology of the CSF flow-void sign: modification by cardiac gating. AJNR Am J Neuroradiol 7: 1021–1024, 1984Google Scholar
  24. 24.
    Conner ES, Foley L, Black PM: Experimental normal-pressure hydrocephalus is accompanied by increased trans-mantle pressure. J Neurosurg 61: 322–327, 1984PubMedCrossRefGoogle Scholar
  25. 25.
    Curran T, Lang AE. Parkinsonian syndromes associated with hydrocephalus: case reports, a review of the literature and pathophysiological hypotheses. Mov Disord 9: 508–520, 1994PubMedCrossRefGoogle Scholar
  26. 26.
    Dandy WE: Diagnosis and treatment of hydrocephalus resulting from strictures of the aqueduct of Sylvius. Surg Gynecol Obstet 31: 340–358, 1920Google Scholar
  27. 27.
    Dennis M, Fitz CR, Netley CT, et al: The intelligence of hydrocephalic children. Arch Neurol 38: 607–615: 1981PubMedCrossRefGoogle Scholar
  28. 28.
    DeVera Reyers JA: Parkinsonism-like syndrome caused by posterior fossa tumour. J Neurosurg 33: 599–601, 1970CrossRefGoogle Scholar
  29. 29.
    Di Rocco C, Di Trapani G, Pettorossi VE, et al: On the pathology of experimental hydrocephalus induced by artificial increase in endoventricular CSF pulse pressure. Childs Brain 5: 81–95, 1979PubMedGoogle Scholar
  30. 30.
    Di Rocco C, Iannelli A, Tamburrini G: Idrocefalo da stenosi dell’acquedotto ad insorgenza tardiva. Minerva Pediatr 47: 511–520, 1995PubMedGoogle Scholar
  31. 31.
    Edwards JH: The syndrome of sex-linked hydrocephalus. Arch Dis Child 36: 486–493, 1961PubMedCrossRefGoogle Scholar
  32. 32.
    Emery JL, Staschak MC: The size and form of cerebral aqueduct in children. Brain 95: 591–598, 1972PubMedCrossRefGoogle Scholar
  33. 33.
    Enzmann DR, Pelec NJ: Normal flow pattern in intracranial and spinal cerebrospinal fluid defined with phase-contrast cine-MR imaging. Radiol 178: 467–474, 1991Google Scholar
  34. 34.
    Fletcher JM, Bohan TP, Brandt ME, et al: Cerebral white matter and cognition in hydrocephalic children. Arch Neurol 49: 818–824: 1992PubMedCrossRefGoogle Scholar
  35. 35.
    Fram E, Hedlund L, Dimick R, et al: Parameters determining the signal of flowing fluid in gradient refocused imaging: flow velocity, TR and flip angle. Proc Intl Soc Mag Reson Med 1: 84–85, 1986Google Scholar
  36. 36.
    Fukuhara T, Luciano M: Clinical features of late-onset idiopathic aqueductal stenosis. Surg Neurol 55: 132–137, 2001PubMedCrossRefGoogle Scholar
  37. 37.
    Gatto M, Micheli F, Pardal MF: Blepharoclonus and parkinsonism associated with aqueductal stenosis. Mov Disord 5: 310–313, 1990PubMedCrossRefGoogle Scholar
  38. 38.
    Hanigan WC, Morgan A, Shaaban A, et al: Surgical treatment and neurodevelopment outcome for infants with idiopathic aqueductal stenosis. Child’s Nerv Syst 7: 386–390, 1991CrossRefGoogle Scholar
  39. 39.
    Harrison MJG, Robert CM, Uttley D: Benign aqueductal stenosis in adults. J Neurol Neurosurg Psychiatry 37: 1322–1328, 1974PubMedCrossRefGoogle Scholar
  40. 40.
    Hatcher MA, Klintworth GK: The sylvian aqueduct syn drome. A clinico-pathological study. Arch Neurol 15: 215–222, 1966PubMedCrossRefGoogle Scholar
  41. 41.
    Hirsch JF, Hirsch E, Sainte-Rose C, et al: Stenosis of the aqueduct of Sylvius (etiology and treatment). J Neurosurg Sci 30: 29–39, 1986PubMedGoogle Scholar
  42. 42.
    Jacobson EE, Fletcher DF, Morgan MK, et al: Computer modelling of the CSF flow dynamics of aqueductal stenosis. Med Biol Eng Comput 37: 59–63, 1999PubMedCrossRefGoogle Scholar
  43. 43.
    Jacobson EE, Fletcher DF, Morgan MK, et al: Fluid dynamics of the cerebral aqueduct. Pediatr Neurosurg 24: 229–236, 1996PubMedCrossRefGoogle Scholar
  44. 44.
    Jankovic J, Newmark M, Peter P: Parkinsonism and acquired hydrocephalus. Mov Disord 1: 59–64, 1986PubMedCrossRefGoogle Scholar
  45. 45.
    Jellinger G: Anatomopathology of nontumoral aqueductal stenosis. J Neurosurg Sci 30: 1–16, 1986PubMedGoogle Scholar
  46. 46.
    Johnson RT, Yates PO: Clinico-pathological aspects of pressure changes at tentorium. Acta Radiol 46: 242–249, 1956PubMedCrossRefGoogle Scholar
  47. 47.
    Johnston IH, Kowman-Giles R, Whittle IR: The arrest of treated hydrocephalus in children. A radionuclide study. J Neurosurg 61: 752–756, 1984PubMedCrossRefGoogle Scholar
  48. 48.
    Jones RFC, Stening WA, Brydon M: Endoscopic third ventriculostomy. Neurosurgery 26: 86–92, 1990PubMedCrossRefGoogle Scholar
  49. 49.
    Kadowaki C, Hara M, Numoto M, et al: Cine magnetic resonance imaging of aqueductal stenosis. Child’s Nerv Syst 11: 107–111, 1995CrossRefGoogle Scholar
  50. 50.
    Kaufmann GE, Clark K: Continuous simultaneous monitoring of intraventricular and cervical subarachnoid cerebrospinal fluid pressure to indicate development of cerebral or tonsillar herniation. J Neurosurg 33: 135–140, 1970Google Scholar
  51. 51.
    Kemp SS, Zimmerman RA, Bilaniuk LT, et al: Magnetic resonance imaging of the cerebral aqueduct. Neuroradiology 29: 430–436, 1987PubMedCrossRefGoogle Scholar
  52. 52.
    Landrieu O, Ninane J, Ferriere G, et al: Aqueductal stenosis in X-linked hydrocephalus: a secondary phenomenon? Dev Med Child Neurol 21: 637–652, 1979PubMedCrossRefGoogle Scholar
  53. 53.
    Lapras C, Bret P, Patet JD, Huppert J, et al: Hydrocephalus and aqueductal stenosis. Direct surgical treatment by interventriculostomy (aqueduct cannulation). J Neurosurg Sci 30: 47–53, 1986PubMedGoogle Scholar
  54. 54.
    Lapras C, Bret P, Tommasi M, et al: Les sténoses de l’aqueduc de Sylvius. Neurochirurgie 26(Suppl 1): 1–152, 1980PubMedGoogle Scholar
  55. 55.
    Lee BCP: Magnetic resonance imaging of peri-aqueductal lesions. Clin Radiol 38: 527–533, 1987PubMedCrossRefGoogle Scholar
  56. 56.
    Lim ST, Potts DG, Deonarine V, et al: Ventricular compliance in dogs with and without aqueductal obstruction. J Neurosurg 39: 463–473, 1973PubMedCrossRefGoogle Scholar
  57. 57.
    Little JR, Houser OW, MacCarty CS: Clinical manifestations of aqueductal stenosis in adults. J Neurosurg 43: 546–552, 1975PubMedCrossRefGoogle Scholar
  58. 58.
    MacFarlane WV, Falconer MA: Diverticulum of the lateral ventricle extending into posterior cranial fossa: report of case successively relieved by operation. J Neurol Neurosurg Psychiatry 10: 101–106, 1947CrossRefGoogle Scholar
  59. 59.
    Martins AN, Wiley JK, Myers PW: Dynamics of the cerebrospinal fluid and spinal dura mater. J Neurol Neurosurg Psychiatry 35: 468–473, 1972PubMedCrossRefGoogle Scholar
  60. 60.
    McCullough DC, Balzer-Martin LA: Current prognosis in overt neonatal hydrocephalus. J Neurosurg 57: 378–383, 1982PubMedCrossRefGoogle Scholar
  61. 61.
    McFarlane A, Maloney AFJ: The appearance of aqueduct and its relotionship with hydrocephalus in the Arnold-Chiari malformation. Brain 80: 479–491, 1957CrossRefGoogle Scholar
  62. 62.
    Messert B, Henke TK, Langheim W: Syndrome of akinetic mutism associated with obstructive hydrocephalus. Neurology 16: 635–649, 1966PubMedCrossRefGoogle Scholar
  63. 63.
    Mise B, Klarica M, Bulat M, et al: Experimental hydrocephalus and hydromyelia: a new insight in mechanism of their development. Acta Neurochir 138: 862–869, 1996CrossRefGoogle Scholar
  64. 64.
    Mott M, Cummins B: Hydrocephalus related to pulsion diverticulum of lateral ventricle. Arch Dis Child 49: 407–410, 1974PubMedCrossRefGoogle Scholar
  65. 65.
    Naidich TP, McLone DG, Hahn YS, et al: Atrial diverticula in severe hydrocephalus. AJNR Am J Neuroradiol 3: 257–266, 1982PubMedGoogle Scholar
  66. 66.
    Naidich TP, Schott LH, Baron RL: Computed tomography in evaluation of hydrocephalus. Radiol Clin North Am 20: 143–167, 1982PubMedGoogle Scholar
  67. 67.
    Nashold BS, Gills JP: Ocular signs from brain stimulation and lesions. Arch Ophthalmol 77: 609–618, 1967PubMedCrossRefGoogle Scholar
  68. 68.
    Nugent GR, Al-Mefty O, Chou S: Communicating hydrocephalus as a cause of aqueductal stenosis. J Neurosurg 51: 812–818, 1979PubMedCrossRefGoogle Scholar
  69. 69.
    Oi S, Shimoda M, Shibata M, et al: Pathophysiology of long-standing overt vebtriculomegaly in adults. J Neurosurg 92: 933–940 2000PubMedCrossRefGoogle Scholar
  70. 70.
    Oi S, Yamada H, Sato O, et al: Experimental models of congenital hydrocephalus and comparable clinical problems on the fetal and neonatal period. Child’s Nerv Syst 12: 292–302, 1996CrossRefGoogle Scholar
  71. 71.
    Oliver R: Parkinsonism due to midbrain compression. Lancet 2: 817–819, 1959PubMedCrossRefGoogle Scholar
  72. 72.
    Pollack IF, Pang D Albright AL: The long-term outcome in children with late-onset aqueductal stenosis resulting from benign intrinsic tectal tumors. J Neurosurg 80: 681–688, 1994PubMedCrossRefGoogle Scholar
  73. 73.
    Quencer RM, Donovan Post MJ, Hinks RS: Cine MR in the evaluation of normal and abnormal CSF flow: intracranial and intraspinal studies. Neuroradiology 32: 371–391, 1990PubMedCrossRefGoogle Scholar
  74. 74.
    Raimondi AJ, Clark SJ, McLone DG: Pathogenesis of aqueductal occlusion in congenital murine hydrocephalus. J Neurosurg 45: 66–77, 1976PubMedCrossRefGoogle Scholar
  75. 75.
    Renier D, Saint-Rose C, Pierre-Kahn A, et al: Prenatal hydrocephalus: outcome and prognosis. Child’s Nerv Syst 4: 213–222, 1988CrossRefGoogle Scholar
  76. 76.
    Robertson JA, Leggate JRS, Miller JD, et al: Aqueductal stenosis-presentation and prognosis. Br J Neurosurg 4: 101–106, 1990PubMedCrossRefGoogle Scholar
  77. 77.
    Ross ED, Stewart RM: Akinetic mutism from hypothalamic damage: successful treatment with dopamine agonists. Neurology 31: 1435–1439, 1981PubMedCrossRefGoogle Scholar
  78. 78.
    Rotilio A, d’Avella D, de Blasi F, et al: Disendocrine manifestations during non tumoral aqueductal stenosis. J Neurosurg Sci 30: 71–76, 1986PubMedGoogle Scholar
  79. 79.
    Rovira A, Capellades J, Grive E, et al: Spontaneous ventriculostomy: report of three cases revealed by flow-sensitive phase-contrast cine MR imaging. AJNR Am J Neuroradiol 20: 1647–1652, 1999PubMedGoogle Scholar
  80. 80.
    Russell DS, Nevin S: Aneurysm of the great vein of Galen causing internal hydrocephalus: report of two cases. J Pathol Bacteriol 51: 447–448, 1940CrossRefGoogle Scholar
  81. 81.
    Russell DS: Observations on the pathology of hydrocephalus. Medical Res Council, special report series No. 265. His Majesty’s Stationery Office, London, 1949Google Scholar
  82. 82.
    Schroeder HWS, Gaab MR: Endoscopic aqueductoplasty: technique and results. Neurosurgery 45: 508–518, 1999PubMedCrossRefGoogle Scholar
  83. 83.
    Shallat RF, Pawl RP Jerva MJ: Significance of upward gaze palsy (Parinaud’s syndrome) in hydrocephalus due to shunt malfunction. J Neurosurg 38: 717–721, 1973PubMedCrossRefGoogle Scholar
  84. 84.
    Sherman JL, Citrin CM, Gangarosa RE, et al: The MR appearance of CSF flow in patients with ventriculomegaly. AJNR Am J Neuroradiol 7: 1025–1031, 1986Google Scholar
  85. 85.
    Spadaro A, Ambrosio D, Moraci A, et al: Aqueductal stenosis as isolated localization involving the central nervous system in children affected by von Recklinghausen disease. J Neurosurg Sci 30: 87–93, 1989Google Scholar
  86. 86.
    Stellman GR, Bannister CM, Hillier V: The incidence of seizures disorders in children with congenital and acquired hydrocephalus. Z Kinderchir 41[Suppl 1]: 38–41, 1986PubMedGoogle Scholar
  87. 87.
    Stephensen H, Tisell M, Wikkelso C: There is no transmantle pressure gradient in communicating or noncommunicating hydrocephalus. Neurosurgery 50: 763–771, 2002PubMedCrossRefGoogle Scholar
  88. 88.
    Suh DY, Gaskill-Shipley M, Nemann MW, et al: Corpus callosal changes associated with hydrocephalus: a report of two cases. Neurosurgery 41: 488–494, 1997PubMedCrossRefGoogle Scholar
  89. 89.
    Tisell M, Edsbagge M, Stephenson H, et al: Elastance correlates with outcome after endoscopic third ventriculostomy in adults with hydrocephalus caused by primary aqueductal stenosis. Neurosurgery 50: 70–77, 2002PubMedGoogle Scholar
  90. 90.
    Turnbull IM, Drake CG: Membranous occlusion of the aqueduct of Sylvius. J Neurosurg 24: 24–33, 1966CrossRefGoogle Scholar
  91. 91.
    Vanneste J, Hyman R: Non-tumoral aqueductal stenosis and normal pressure hydrocephalus in the elderly. J Neurol Neurosurg Psychiatry 49: 529–535, 1986PubMedCrossRefGoogle Scholar
  92. 92.
    Villani R, Tomei G, Gaini SM, et al: Long-term outcome in aqueductal stenosis. Child’s Nerv Syst 11: 180–185, 1995CrossRefGoogle Scholar
  93. 93.
    Vindigni G, Del Fabro P, Facchin P, et al: On the neurological complications of internal and external shunt in patients with non-neoplastic stenosis of the aqueduct. J Neurosurg Sci 30: 83–86, 1986PubMedGoogle Scholar
  94. 94.
    Wakai S, Narita J, Hashimoto K, et al: Diverticulum of the lateral ventricle causing cerebellar ataxia. Case report. J Neurosurg 59: 895–898, 1983PubMedCrossRefGoogle Scholar
  95. 95.
    Williams B: Cerebrospinal fluid pressure-gradients in spina bifida cystica, with special reference to Arnold-Chiari malformation and aqueductal stenosis. Dev Med Child Neurol Suppl 35: 138–150, 1975PubMedGoogle Scholar
  96. 96.
    Woollam DH, Milien JW: Anatomical considerations in the pathology of stenosis of the cerebral aqueduct. Brain 76: 104–112, 1953PubMedCrossRefGoogle Scholar
  97. 97.
    Yamada H, Oi S, Tamaki N, et al: Prenatal aqueductal stenosis as a cause of congenital hydrocephalus in the inbred rat LEW/Jms. Child’s Nerv Syst 7: 218–222, 1991CrossRefGoogle Scholar
  98. 98.
    Yamasaki M, Arita N, Hiraga S, et al: A clinical and neuro-radiological study of X-linked hydrocephalus in Japan. J Neurosurg 83: 50–55, 1995PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Italia 2005

Authors and Affiliations

  • Giuseppe Cinalli
    • 1
  • Pietro Spennato
    • 2
  • Emilio Cianciulli
    • 3
  • Maria d’Armiento
    • 4
  1. 1.Department of Pediatric NeurosurgerySantobono-Pausilipon Children’s HospitalNaplesItaly
  2. 2.Department of NeurosurgerySecond University of NaplesNaplesItaly
  3. 3.Department of Pediatric NeuroradiologySantobono-Pausilipon Children’s HospitalNaplesItaly
  4. 4.Department of Biomorphological and Functional Sciences, Section of Pathology, Federico IIUniversity of NaplesItaly

Personalised recommendations