Disorders of CSF hydrodynamics

Abstract This article reviews the range of hydrodynamic disorders affecting the CSF circulation. Initially consideration is given to questions of definition and classification. A scheme for the practical, clinical analysis for the diagnosis of such disorders is then presented. The physiology and pathophysiology of the CSF circulation is reviewed, with particular emphasis on issues that remain unresolved. This provides a background to consideration of the adverse consequences of abnormal CSF hydrodynamics, again focusing on areas where further information is required. Methods of clinical investigation of CSF hydrodynamics are reviewed, followed by general considerations of treatment. Finally, each of the main, clinically important, forms of disordered CSF hydrodynamics is briefly considered, with particular emphasis, again, on areas where current knowledge is deficient. The conditions considered include hydrocephalus of various forms (childhood, adult, arrested, multi-compartment), infantile macrocephaly, arachnoid and glio-ependymal cysts, syringo- and hydromyelia, pseudotumour cerebri, impaired cranial venous outflow, altered CSF composition, shunt obstruction without ventricular enlargement and low-CSF-pressure states.

[1]  J. Montes,et al.  Radionuclide shuntogram: adjunct to manage hydrocephalic patients. , 1996, Journal of nuclear medicine : official publication, Society of Nuclear Medicine.

[2]  A. Jayatilaka An electron microscopic study of sheep arachnoid granulations. , 1965, Journal of anatomy.

[3]  Low-pressure hydrocephalic state and viscoelastic alterations in the brain. , 1994 .

[4]  A. Price,et al.  Early changes in experimental hydrocephalus. , 1986, Investigative radiology.

[5]  J Hermans,et al.  Dutch normal-pressure hydrocephalus study: prediction of outcome after shunting by resistance to outflow of cerebrospinal fluid. , 1997, Journal of neurosurgery.

[6]  M. Weiss,et al.  The effect of glucocorticoids on CSF flow in dogs. , 1970, Journal of neurosurgery.

[7]  T. Eller,et al.  Isolated ventricles following intraventricular hemorrhage. , 1985, Journal of neurosurgery.

[8]  F. Sklar,et al.  The pressure-volume function of brain elasticity. Physiological considerations and clinical applications. , 1977, Journal of neurosurgery.

[9]  S. Brusilow,et al.  Effects of indomethacin on cholera toxin-induced cerebrospinal fluid production , 1978, Brain Research.

[10]  S. Brusilow,et al.  Cerebrospinal fluid production: stimulation by cholera toxin. , 1977, Science.

[11]  B. Irthum,et al.  Benign intracranial hypertension and disorders of CSF absorption. , 1981, Surgical neurology.

[12]  K. Wester,et al.  Syringomyelia treated with a nonvalved syringoperitoneal shunt: a follow-up study. , 1997, Neurosurgery.

[13]  M. Pollay Pharmacological Manipulation of Cerebrospinal Fluid Secretion , 1992 .

[14]  E. Oldberg,et al.  CHANGES IN THE BRAIN IN PLEXECTOMIZED DOGS: WITH COMMENTS ON THE CEREBROSPINAL FLUID , 1937 .

[15]  A. Marmarou,et al.  Mechanism of pseudotumor in Guillain-Barré syndrome. , 1984, Archives of neurology.

[16]  M. Walker,et al.  Diagnosis and treatment of the slit ventricle syndrome. , 1993, Neurosurgery clinics of North America.

[17]  B. Drayer,et al.  Distinction Between Obstructive and Atrophic Dilatation of Ventricles in Children , 1980, Journal of computer assisted tomography.

[18]  N. Rosman,et al.  Hydrocephalus caused by increased intracranial venous pressure: A clinicopathological study , 1978, Annals of neurology.

[19]  R. Fishman,et al.  Spontaneous intracranial hypotension , 1992, Neurology.

[20]  J. McComb Recent research into the nature of cerebrospinal fluid formation and absorption. , 1983, Journal of neurosurgery.

[21]  E. Hendrick,et al.  Subtemporal decompression for the slit-ventricle syndrome after shunting in hydrocephalic children. , 1979, Child's brain.

[22]  D. Mccullough,et al.  Negative intracranial pressure hydrocephalus in adults with shunts and its relationship to the production of subdural hematoma. , 1974, Journal of neurosurgery.

[23]  J. Kloss Craniosynostosis Secondary to Ventriculoatrial Shunt , 1968 .

[24]  H F Cserr,et al.  Bulk flow of interstitial fluid after intracranial injection of blue dextran 2000. , 1974, Experimental neurology.

[25]  S. Hawke,et al.  The pseudotumor syndrome. Disorders of cerebrospinal fluid circulation causing intracranial hypertension without ventriculomegaly. , 1991, Archives of neurology.

[26]  J O Rowan,et al.  Raised intracranial pressure and cerebral blood flow , 1972, Journal of Neurology Neurosurgery & Psychiatry.

[27]  J. Drake,et al.  Tonsillar herniation: the rule rather than the exception after lumboperitoneal shunting in the pediatric population. , 1993, Journal of neurosurgery.

[28]  V. Logue,et al.  Syringomyelia and its surgical treatment--an analysis of 75 patients. , 1981, Journal of neurology, neurosurgery, and psychiatry.

[29]  A. Ommaya,et al.  The production of cerebrospinal fluid in man and its modification by acetazolamide. , 1966, Journal of neurosurgery.

[30]  D. Long,et al.  Subdural hematoma as a complication of shunting procedures for normal pressure hydrocephalus. , 1972, Journal of neurosurgery.

[31]  F. Plum,et al.  Papilledema in poliomyelitis. , 1951, A.M.A. archives of neurology and psychiatry.

[32]  G. Teasdale,et al.  Serial ventricular volume measurements: further insights into the aetiology and pathogenesis of benign intracranial hypertension. , 1981, Journal of neurology, neurosurgery, and psychiatry.

[33]  M. Pollay,et al.  Extracorporeal perfusion of choroid plexus. , 1972, Journal of applied physiology.

[34]  A. Hirano,et al.  Influence of the rate of ventricular enlargement on the ultrastructural morphology of the white matter in experimental hydrocephalus. , 1987, Neurosurgery.

[35]  G. B. Hassin NOTES ON THE NATURE AND ORIGIN OF THE CEREBROSPINAL FLUID , 1924 .

[36]  M. Hara,et al.  The effect of dexamethasone phosphate on the production rate of cerebrospinal fluid in the spinal subarachnoid space of dogs. , 1973, Journal of neurosurgery.

[37]  R. Bright Reports of Medical Cases, Selected with a View of Illustrating the Symptoms and Cure of Diseases by a Reference to Morbid Anatomy , 1828, Edinburgh Medical and Surgical Journal.

[38]  S. Rengachary Intracranial arachnoid and ependymal cysts. , 1985 .

[39]  W. Dandy,et al.  EXTIRPATION OF THE CHOROID PLEXUS OF THE LATERAL VENTRICLES IN COMMUNICATING HYDROCEPHALUS , 1918, Annals of surgery.

[40]  H. Hoffman,et al.  Isolated fourth ventricle as a complication of ventricular shunting. Report of three cases. , 1978, Journal of neurosurgery.

[41]  B. Williams Is aqueduct stenosis a result of hydrocephalus? , 1973, Brain : a journal of neurology.

[42]  W. Dandy INTRACRANIAL PRESSURE WITHOUT BRAIN TUMOR: DIAGNOSIS AND TREATMENT. , 1937, Annals of surgery.

[43]  C. Owman,et al.  Sympathetic Nervous Control of CSF Production in Normal and Hydrocephalic Animals, and Its Relationship to Cerebral Blood Volume and Intracranial Pressure , 1983 .

[44]  W. Dandy,et al.  INTERNAL HYDROCEPHALUS: An Experimental, Clinical and Pathological Study , 1964 .

[45]  C. Benesch,et al.  Overdrainage and shunt technology. A critical comparison of programmable, hydrostatic and variable-resistance valves and flow-reducing devices. , 1995, Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery.

[46]  Charles Tator,et al.  Favorable results with syringosubarachnoid shunts for treatment of syringomyelia. , 1982, Journal of neurosurgery.

[47]  A D Dayan,et al.  Pathogenesis of syringomyelia. , 1972, Lancet.

[48]  N. Zwetnow The influence of an increased intracranial pressure on the lactate, pyruvate, bicarbonate, phosphocreatine, ATP, ADP and AMP concentrations of the cerebral cortex of dogs. , 1970, Acta physiologica Scandinavica.

[49]  P. Newman,et al.  Some observations on the pathogenesis of syringomyelia. , 1981, Journal of neurology, neurosurgery, and psychiatry.

[50]  H. Rekate,et al.  Elevated intracranial venous pressure as a universal mechanism in pseudotumor cerebri of varying etiologies , 1996, Neurology.

[51]  G. Chiro,et al.  RISA‐ventriculography and RISA‐cisternography , 1964, Neurology.

[52]  T. Milhorat,et al.  The subarachnoid space in congenital obstructive hydrocephalus. 2. Microscopic findings. , 1971, Journal of neurosurgery.

[53]  E. Hitchcock,et al.  Midland Centre for Neurosurgery and Neurology, Smethwick, Warley, West Midlands, England Sino-Jugular Venous Graft in Otitic Hydrocephalus , 1981 .

[54]  R. Pudenz,et al.  Hydrocephalus: overdrainage by ventricular shunts. A review and recommendations. , 1991, Surgical neurology.

[55]  A. Key,et al.  Studien in der anatomie des Nervensystems und des Bindegewebes , 1875 .

[56]  J. Drake,et al.  The shunt book , 1995 .

[57]  J. E. Levine,et al.  The morphological correlates of primate cerebrospinal fluid absorption , 1982, Brain Research.

[58]  Gardner Wj,et al.  HYDRODYNAMIC MECHANISM OF SYRINGOMYELIA: ITS RELATIONSHIP TO MYELOCELE. , 1965 .

[59]  J. Pappenheimer,et al.  Bulk flow and diffusion in the cerebrospinal fluid system of the goat. , 1962, The American journal of physiology.

[60]  J. Selhorst,et al.  CSF infusion test in pseudotumor cerebri. , 1978, Transactions of the American Neurological Association.

[61]  D Wyper,et al.  USE OF MAGNETIC RESONANCE IMAGING TO MEASURE INTRACRANIAL CEREBROSPINAL FLUID VOLUME , 1986, The Lancet.

[62]  Hugh Davson,et al.  Physiology of the CSF and Blood-Brain Barriers , 1996 .

[63]  G. Krol,et al.  MRI changes in intracranial hypotension , 1993, Neurology.

[64]  R. Howman-Giles,et al.  Evaluation of CSF shunt patency by means of technetium-99m DTPA. , 1982, Journal of neurosurgery.

[65]  R. D. Adams,et al.  SYMPTOMATIC OCCULT HYDROCEPHALUS WITH "NORMAL" CEREBROSPINAL-FLUID PRESSURE.A TREATABLE SYNDROME. , 1965, The New England journal of medicine.

[66]  E. Foltz,et al.  Symptomatic low intracranial pressure in shunted hydrocephalus. , 1988, Journal of neurosurgery.

[67]  E. Bering,et al.  HYDROCEPHALUS: CHANGES IN FORMATION AND ABSORPTION OF CEREBROSPINAL FLUID WITHIN THE CEREBRAL VENTRICLES. , 1963, Journal of neurosurgery.

[68]  J. Galicich,et al.  Formation and absorption of cerebrospinal fluid in man. , 1968, Brain : a journal of neurology.

[69]  J L Keltner,et al.  Optic Nerve Sheath Decompression: How Does It Work? Has Its Time Come? , 1988 .

[70]  G. Chiro,et al.  The subarachnoid space in congenital obstructive hydrocephalus , 1971 .

[71]  M. Raichle,et al.  Cerebral hemodynamics and metabolism in pseudotumor cerebri , 1978, Annals of neurology.

[72]  Thrombosis of the dural venous sinuses as a cause of pseudotumor cerebri. , 1951, Annals of surgery.

[73]  R. Katzman.,et al.  A simple constant‐infusion manometric test for measurement of CSF absorption , 1970, Neurology.

[74]  J. Campbell,et al.  Complications in ventricular cerebrospinal fluid shunting. , 1993, Neurosurgery clinics of North America.

[75]  M. Gaab,et al.  Testing the hydrocephalus shunt valve: long-term bench test results of various new and explanted valves. The need for model for testing valves under physiological conditions. , 1991, European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift fur Kinderchirurgie.

[76]  J. Hirsch,et al.  Intracranial venous sinus hypertension: cause or consequence of hydrocephalus in infants? , 1984, Journal of neurosurgery.

[77]  J J Corbett,et al.  The rational management of idiopathic intracranial hypertension. , 1989, Archives of neurology.

[78]  J. Fenstermacher,et al.  Cerebrospinal fluid formation in ventricles and spinal subarachnoid space of the rhesus monkey. , 1975, Journal of neurosurgery.

[79]  J J Corbett,et al.  Results of optic nerve sheath fenestration for pseudotumor cerebri. The lateral orbitotomy approach. , 1988, Archives of ophthalmology.

[80]  N. Lundström,et al.  Hydrocephalus and congestive heart failure caused by intracranial arteriovenous malformations in infants. , 1972, Journal of neurosurgery.

[81]  M. C. Calhoun,et al.  Rates of formation and absorption of cerebrospinal fluid in bovine hypovitaminosis A. , 1967, Journal of dairy science.

[82]  A. Paterson,et al.  Benign intracranial hypertension. II. CSF pressure and circulation. , 1974, Brain : a journal of neurology.

[83]  R. Tripathi,et al.  The functional morphology of the outflow systems of ocular and cerebrospinal fluids. , 1977, Experimental eye research.

[84]  T. Milhorat,et al.  Flow of cerebral interstitial fluid as indicated by the removal of extracellular markers from rat caudate nucleus. , 1977, Experimental eye research.

[85]  D. Rall,et al.  Cerebrospinal Fluid Production by the Choroid Plexus and Brain , 1971, Science.

[86]  J. Wisoff,et al.  'Slit-ventricle syndrome': etiology and treatment. , 1988, Pediatric neuroscience.

[87]  T. Milhorat,et al.  Normal rate of cerebrospinal fluid formation five years after bilateral choroid plexectomy. Case report. , 1976, Journal of neurosurgery.

[88]  A. Artru,et al.  Closed recirculatory spinal subarachnoid perfusion for determining CSF dynamics. , 1982, Journal of neurosurgery.

[89]  R. Joynt,et al.  Brain Swelling of Unknown Cause , 1956, Neurology.

[90]  A. Mclaughlin,et al.  A radionuclide method of evaluating shunt function and CSF circulation in hydrocephalus. Technical note. , 1984, Journal of neurosurgery.

[91]  W G Bradley,et al.  Flow dynamics of cerebrospinal fluid: assessment with phase-contrast velocity MR imaging performed with retrospective cardiac gating. , 1992, Radiology.

[92]  M. E. Carey,et al.  Further data on the acute effect of intravenous steroids on canine CSF secretion and absorption. , 1979, Journal of Neurosurgery.

[93]  F. Epstein,et al.  Subtemporal craniectomy for recurrent shunt obstruction secondary to small ventricles. , 1974, Journal of neurosurgery.

[94]  D. Nyberg,et al.  Fetal hydrocephalus: sonographic detection and clinical significance of associated anomalies. , 1987, Radiology.

[95]  A. Olivi,et al.  Slit-ventricle syndrome: review of 15 cases. , 1987, Pediatric neuroscience.

[96]  H. Portnoy,et al.  Megalencephaly in infants and children. The possible role of increased dural sinus pressure. , 1978, Archives of neurology.

[97]  L. H. Weed FORCES CONCERNED IN THE ABSORPTION OF THE CEREBROSPINAL FLUID , 1935 .

[98]  K. Sudo,et al.  Changes in cerebral microvasculature in congenital hydrocephalus of the inbred rat LEW/Jms: light and electron microscopic examination. , 1987, Surgical neurology.

[99]  le Gros Clark We,et al.  On the Pacchionian Bodies. , 1920 .

[100]  R. Griggs Cerebrospinal Fluid in Diseases of the Nervous System , 1981, Neurology.

[101]  J Ekstedt,et al.  CSF hydrodynamic studies in man. 1. Method of constant pressure CSF infusion. , 1977, Journal of neurology, neurosurgery, and psychiatry.

[102]  E. Staab,et al.  CSF rhinorrhea: detection and localization using overpressure cisternography with Tc-99m-DTPA. , 1985, Radiology.

[103]  W. Maixner,et al.  Ventricular volume in infantile hydrocephalus and its relationship to intracranial pressure and cerebrospinal fluid clearance before and after treatment. A preliminary study. , 1990, Pediatric neurosurgery.

[104]  D. Gilday,et al.  111In-DTPA evaluation of CSF diversionary shunts in children. , 1973, Journal of nuclear medicine : official publication, Society of Nuclear Medicine.

[105]  J. Scarff The treatment of nonobstructive (communicating) hydrocephalus by endoscopic cauterization of the choroid plexuses. , 1970, Journal of neurosurgery.

[106]  K. Crone,et al.  Endoscopic treatment of loculated hydrocephalus. , 1995, Journal of neurosurgery.

[107]  K M LAURENCE,et al.  The Natural History of Hydrocephalus , 1962, Archives of disease in childhood.

[108]  K. Shulman,et al.  Infantile hydrocephalus: clinical, histological, and ultrastructural study of brain damage. , 1972, Journal of neurosurgery.

[109]  L. H. Weed An anatomical consideration of the cerebro‐spinal fluid , 1917 .

[110]  R. G. Robinson CONGENITAL CYSTS OF THE BRAIN: ARACHNOIDS MALFORMATION , 1971 .

[111]  J. Drake,et al.  Reduced Local Cerebral Blood Flow in Periventricular White Matter in Experimental Neonatal Hydrocephalus—Restoration with CSF Shunting , 1995, Journal of cerebral blood flow and metabolism : official journal of the International Society of Cerebral Blood Flow and Metabolism.

[112]  W. Gardner,et al.  Hydrodynamic mechanism of syringomyelia: its relationship to myelocele , 1965, Journal of neurology, neurosurgery, and psychiatry.

[113]  I. Mcquarrie,et al.  Treatment of normal pressure hydrocephalus with low versus medium pressure cerebrospinal fluid shunts. , 1984, Neurosurgery.

[114]  M. Hara,et al.  Formation of Cerebrospinal Fluid in Spinal Subarachnoid Space , 1971, Nature.

[115]  M. Sakanoue,et al.  NA, K, CA, MG, AND C1 CONCENTRATIONS IN CHOROID PLEXUS FLUID AND CISTERNAL FLUID COMPARED WITH PLASMA ULTRAFILTRATE. , 1964, Journal of neurophysiology.

[116]  K. Welch,et al.  SECRETION OF CEREBROSPINAL FLUID BY CHOROID PLEXUS OF THE RABBIT. , 1963, The American journal of physiology.

[117]  D. Maxwell,et al.  The Morphology of the Arachnoid Villi: A Light and Electron Microscopic Study in the Monkey , 1968 .

[118]  J. Pappenheimer,et al.  Perfusion of the cerebral ventricular system in unanesthetized goats , 1962 .

[119]  F. Sklar,et al.  Cerebrospinal fluid dynamics in patients with pseudotumor cerebri. , 1979, Neurosurgery.

[120]  J. Mealey,et al.  Failure of oral acetazolamide to avert hydrocephalus in infants with myelomeningocele. , 1968, The Journal of pediatrics.

[121]  C. Owman,et al.  Neuroendocrine regulatory mechanisms in the choroid plexus-cerebrospinal fluid system , 1992, Brain Research Reviews.

[122]  H. Wiśniewski,et al.  EXPERIMENTAL HYDROCEPHALUS IN YOUNG DOGS: HISTOLOGICL AND ULTRASTRUCTURAL STUDY OF THE BRAIN TISSUE DAMAGE , 1971, Journal of neuropathology and experimental neurology.

[123]  S. Rengachary,et al.  Ultrastructure and pathogenesis of intracranial arachnoid cysts. , 1981, Journal of neuropathology and experimental neurology.

[124]  N. Lundberg,et al.  Continuous recording and control of ventricular fluid pressure in neurosurgical practice. , 1962, Acta psychiatrica Scandinavica. Supplementum.

[125]  K. Welch,et al.  Perfusion of particles through arachnoid villi of the monkey. , 1961, The American journal of physiology.

[126]  G. Maira,et al.  The study of cerebrospinal fluid dynamics in apparently 'arrested' hydrocephalus in children. , 1977, Child's brain.

[127]  STUDIES ON THE CEREBROSPINAL FLUID , 1920 .

[128]  I. Whittle,et al.  The arrest of treated hydrocephalus in children. A radionuclide study. , 1984, Journal of neurosurgery.

[129]  A. Paterson,et al.  The treatment of benign intracranial hypertension: A review of 134 cases , 1981 .

[130]  H. Portnoy,et al.  Anti-siphon and reversible occlusion valves for shunting in hydrocephalus and preventing post-shunt subdural hematomas. , 1973, Journal of neurosurgery.

[131]  D. Greitz,et al.  MR Imaging of Cerebrospinal Fluid Dynamics in Health and Disease , 1994, Acta radiologica.

[132]  G. Chiro Movement of the Cerebrospinal Fluid in Human Beings , 1964, Nature.

[133]  L. Edvinsson,et al.  Sympathetic nervous control of cerebrospinal fluid production from the choroid plexus. , 1978, Science.

[134]  T. Milhorat Hydrocephalus and the Cerebrospinal Fluid , 1972 .

[135]  E. Jacobson,et al.  The Acquired Chiari Malformation and Syringomyelia Following Spinal CSF Drainage: A Study of Incidence and Management , 1998, Acta Neurochirurgica.

[136]  H. Rekate Classification of slit-ventricle syndromes using intracranial pressure monitoring. , 1993, Pediatric neurosurgery.

[137]  G. Harsh,et al.  Intracranial Arachnoid Cysts in Children , 1986, Journal of neurosurgery.

[138]  G. Chiro Observations on the circulation of the cerebrospinal fluid. , 1966 .

[139]  I. Johnston Reduced C.S.F. absorption syndrome. Reappraisal of benign intracranial hypertension and related conditions. , 1973, Lancet.

[140]  C. Teo Third Ventriculostomy in the Treatment of Hydrocephalus: Experience with More than 120 Cases , 1998 .

[141]  J. Foley Benign forms of intracranial hypertension; toxic and otitic hydrocephalus. , 1955, Brain : a journal of neurology.

[142]  F. Gjerris,et al.  Persistent disturbances of cognitive functions in patients with pseudotumor cerebri , 1986, Acta neurologica Scandinavica.

[143]  E. Bering,et al.  Extra-ventricular formation of cerebrospinal fluid. , 1967, No to shinkei = Brain and nerve.

[144]  A. Lorenzo,et al.  Relationship between cerebrospinal fluid formation, absorption and pressure in human hydrocephalus. , 1970, Brain : a journal of neurology.

[145]  Juan Sahuquillo,et al.  Cognitive changes after cerebrospinal fluid shunting in young adults with spina bifida and assumed arrested hydrocephalus , 2000, Journal of neurology, neurosurgery, and psychiatry.

[146]  G. B. Hassin HYDROCEPHALUS: STUDIES OF THE PATHOLOGY AND PATHOGENESIS, WITH REMARKS ON THE CEREBROSPINAL FLUID , 1930 .

[147]  K. Welch,et al.  The cerebrospinal fluid valves. , 1960, Brain : a journal of neurology.