Research Article| Volume 17, ISSUE 5, P338-348, September 1995

Download started.


Classification of hydrocephalus and outcome of treatment

      This paper is only available as a PDF. To read, Please Download here.


      Purpose: Retrospective analysis in cooperative study of hydrocephalus at institutions of members of the Research Committee on Intractable Hydrocephalus sponsored by the Ministry of Health and Welfare of Japan was performed to determine the functional prognosis for all types of hydrocephalus and thus to clarify the outcome.
      Methods: In preparation of this study, we have proposed the definition, clinical classification and diagnostic criteria of hydrocephalus. We have classified non-tumoral hydrocephalus into eight types based on its etiology and the time of onset. To establish the diagnosis in each type of hydrocephalus, we have set up inclusion and exclusion criteria, as well as supplementary criteria which are useful for its diagnosis.
      Results: Analysis of the 1450 cases of hydrocephalus of various etiologies stored in the data base obtained from the study for each type of hydrocephalus revealed that following types and conditions were associated with a neurologic deficit: (1) early fetal hydrocephalus, (2) overt neonatal hydrocephalus, (3) hydrocephalus associated with such severe brain malformations as hydranencephaly, holoprosencephaly and lissencephaly, (4) hydrocephalus associated with severe brain damage, (5) hydrocephalus associated with epilepsy, (6) hydrocephalus shunted late after detection, and (7) hydrocephalus complicated by a shunting operation.
      Conclusion: The postnatal functional outcome was significantly poor in fetal hydrocephalus diagnosed in the early gestation. Childhood onset hydrocephalus showed a poorer outcome than adult hydrocephalus.


      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to Brain and Development
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


      1. 2nd edn. Annual report (1992) by research committee on intractable hydrocephalus. The Ministry of Health and Welfare of Japan, Tokyo1993 (Suppl) (in Japanese with English abstract)
        • Mori K.
        Hydrocephalus — revision of its definition and classification with special reference to ‘intractable infantile hydrocephalus’.
        Child's Nerv Syst. 1990; 6: 198-204
      2. Mori K. Proceeding of symposium on hydrocephalus: current concepts. The Ministry of Health and Welfare of Japan, Tokyo1993 (in Japanese with English abstract)
        • Johnson M.L.
        • Pretorius D.H.
        Ultrasonic diagnosis of fetal and neonatal hydrocephalus.
        Clin Neurosurg. 1985; 32: 574-592
        • Jeanty P.
        • Dramaix-Wilmet M.
        • Delbek D.
        • Rodesch F.
        • Struyven J.
        Ultrasonic evaluation of fetal ventricular growth.
        Neuroradiology. 1981; 21: 127-137
        • James Jr, A.E.
        • Flor W.J.
        • Merz T.
        • Strecker E.-P.
        • Burns B.
        A pathophysiologic mechanism for ventricular entry of radiopharmaceutical and possible relation to chronic communicating hydrocephalus.
        AJR. 1974; 122: 38-43
        • Katzman R.
        • Hussey F.
        A simple constant infusion manometric test for measurement of CSF absorption. 1. Rationale and method.
        Neurology. 1970; 20: 543-544
        • Shapiro K.
        Characterization of clinical CSF dynamics and neural compliance using the pressure-volume index. 1. The normal pressure-volume index.
        Ann Neurol. 1980; 7: 508-514
        • Lundberg N.
        Continuous recording and control of ventricular fluid pressure in neurosurgical practice.
        Acta Psychiatr Neurol Scand. 1960; 36: 1-193
        • Nellhaus G.
        Head circumference from birth to eighteen years. Practical composite international and interracial graphs.
        Pediatrics. 1968; 41: 106-114
        • Huckmann M.S.
        • Fox J.
        • Topel J.
        The validity of criteria for the evaluation of cerebral atrophy by computed tomography.
        Radiology. 1975; 116: 85-92
        • Barkovich A.J.
        • Edwards M.S.B.
        Applications of neuroimaging in hydrocephalus.
        Pediatr Neurosurg. 1992; 18: 65-83
        • Marmarou A.
        • Shulman K.
        • Lamorgese J.
        Compartmental analysis of compliance and outflow resistance of the cerebrospinal fluid system.
        J Neurosurg. 1975; 43: 523-534
        • Marmarou A.
        • Shulman K.
        • Rosende M.
        A nonlinear analysis of the cerebrospinal fluid system and intracranial pressure dynamics.
        J Neurosurg. 1978; 48: 332-343
        • Salibi N.A.
        • Lourie G.L.
        • Lourie H.
        A variant of normal-pressure hydrocephalus simulating Pick's disease on computerized tomography.
        J Neurosurg. 1983; 59: 902-904
        • Symon L.
        • Dorsch N.W.C.
        Use of long term intracranial pressure measurement to assess hydrocephalic patients prior to shunt surgery.
        J Neurosurg. 1978; 42: 258-273
        • Graff-Radford N.R.
        • Godersky J.C.
        Idiopathic normal pressure hydrocephalus and systemic hypertension.
        Neurology. 1987; 37: 868-871
        • Braffman B.H.
        • Zimmerman R.A.
        • Trojanowski J.Q.
        • Gonatas N.K.
        • Hickey W.F.
        • Schlaepfer W.W.
        Brain MR: pathologic correlation with gross and histopathology. 2. Hyperintense white-matter foci in the elderly.
        AJNR. 1988; 9: 629-636
        • Derouesne C.
        • Gray F.
        • Escourolle R.
        • Castaigne P.
        ‘Expanding cerebral lacunae’ in a hypertensive patient with normal pressure hydrocephalus.
        Neuropathol Appl Neurobiol. 1987; 13: 309-320
        • Bradley W.G.
        • Whittemore A.R.
        • Watanabe A.S.
        • Davis S.J.
        • Teresi L.M.
        • Homyak M.
        Association of deep white matter infarction with chronic communicating hydrocephalus; implications regarding the possible origin of normal-pressure hydrocephalus.
        AJNR. 1991; 12: 31-39
      3. 2nd edn. Annual report (1993) by research committee on intractable hydrocephalus. The Ministry of Health and Welfare of Japan, Tokyo1994 (in Japanese with English abstract)
      4. 2nd edn. Annual report (1992) by research committee on intractable hydrocephalus. The Ministry of Health and Welfare of Japan, Tokyo1993 (in Japanese with English abstract)
      5. 2nd edn. Annual report (1994) by research committee on intractable hydrocephalus. The Ministry of Health and Welfare of Japan, Tokyo1995 (in Japanese with English abstract)
        • Milhorat T.H.
        Hydrocephalus and the cerebrospinal fluid.
        in: Williams and Wilkins, Baltimore1972: 55-58
        • Aronyk K.E.
        The history and classification of hydrocephalus.
        Neurosurg Clin North Am. 1993; 4: 625-632
        • Oi S.
        • Sato O.
        • Matsumoto S.
        A new classification for congenital hydrocephalus: perspective classification of congenital hydrocephalus (PCCH) and postnatal prognosis. Part 1. A proposal of a new classification of fetal/neonatal/infantile hydrocephalus based on neuronal maturation and chronological changes (in Japanese with English abstract).
        Noshinkei-geka Journal. 1994; 3: 122-127
        • Cochran D.D.
        • Myles S.T.
        Management of intrauterine hydrocephalus.
        J Neurosurg. 1982; 57: 590-596
        • Renier D.
        • Saint-Rose C.
        • Pierre-Kahn A.
        • Hirsch J.-F.
        Prenatal hydrocephalus: outcome and prognosis.
        Child's Nerv Syst. 1988; 4: 213-222
        • Fernell E.
        • Hagberg B.
        • Hagberg G.
        • von Went L.
        Epidemiology of infantile hydrocephalus in Sweden. II. Origin in infants born at term.
        Acta Paediatr Scand. 1987; 76: 411-417
        • Oi S.
        • Matsumoto S.
        • Katayama K.
        • Mochizuki M.
        Pathophysiology and postnatal outcome of fetal hydrocephalus.
        Child's Nerv Syst. 1990; 6: 338-345
        • Fernell E.
        • Hagberg G.
        • Hagberg B.
        Infantile hydrocephalus — the impact of enhanced preterm survival.
        Acta Paediatr Scand. 1990; 79: 1080-1086
        • Fernell E.
        • Hagberg B.
        • Hagberg G.
        • von Went L.
        Epidemiology of infantile hydrocephalus in Sweden. I. Birth prevalence and general data.
        Acta Paediatr Scand. 1986; 75: 975-981
        • Fernell E.
        • Hagberg B.
        • Hagberg G.
        • von Went L.
        Epidemiology of infantile hydrocephalus in Sweden. III. Origin in preterm infants.
        Acta Paediatr Scand. 1987; 76: 418-423
        • Hale P.M.
        • McAllister II, J.P.
        • Katz S.D.
        • et al.
        Improvement of cortical morphology in infantile hydrocephalus animals after ventriculoperitoneal shunt placement.
        Neurosurgery. 1992; 31: 1085-1096
        • Harris N.G.
        • Jones H.C.
        • Patel S.
        Ventricle shunting in young H-Tx rats with inherited congenital hydrocephalus: a quantitative histological study of cortical grey matter.
        Child's Nerv Syst. 1994; 10: 293-301
        • Raimondi A.J.
        • Soare P.
        Intellectual development in shunted hydrocephalic children.
        Am J Dis Child. 1974; 127: 664-671
        • Miyazawa T.
        • Sato K.
        Learning disability and impairment of synaptogenesis in HTX rats with arrested shunt-dependent hydrocephalus.
        Child's Nerv Syst. 1991; 7: 121-128
        • Mealey J.
        • Gilmor R.L.
        • Bubb M.P.
        The prognosis of hydrocephalus overt at birth.
        J Neurosurg. 1973; 39: 348-355
      6. 2nd edn. Annual report (1991) by research committee on intractable hydrocephalus. The Ministry of Health and Welfare of Japan, Tokyo1992 (in Japanese with English abstract)
        • Kaiser G.
        The value of multiple shunt systems in the treatment of nontumoral infantile hydrocephalus.
        Child's Nerv Syst. 1986; 2: 200-205
      7. Mori K. CSF shunt system. Excerpta Medica, Amsterdam1986 (partly in English and partly in Japanese)
        • Choux M.
        • Genitori L.
        • Lang D.
        • Lena G.
        Shunt implantation: reducing the incidence of shunt infection.
        J Neurosurg. 1992; 77: 875-880
        • McCullough D.C.
        • Balzer-Martin L.A.
        Current prognosis in overt neonatal hydrocephalus.
        J Neurosurg. 1982; 57: 378-383
        • Habib Z.
        Genetics and genetic counseling in neonatal hydrocephalus.
        Obstet Gynecol Surg. 1981; 36: 529-534
        • Garver K.L.
        • Marchese S.G.
        Inheritance of hydrocephalus.
        in: Garver K.L. Marchese S.G. Genetic counseling for clinicians. Year Book Medical, Chicago1986: 71-72
        • Burton B.K.
        Recurrence risks for congenital hydrocephalus.
        Clin Genet. 1979; 16: 47-53
        • Willems P.J.
        • Brouwer O.F.
        • Dijkstra I.
        • Wilmink J.
        X-linked hydrocephalus.
        Am J Med Genet. 1987; 27: 921-928
        • Strain L.
        • Gosden C.M.
        • Brock D.J.
        • Bonthron D.T.
        Genetic heterogeneity in X-linked hydrocephalus: linkage to markers within Xq27.3.
        Am J Hum Genet. 1994; 54: 236-243
        • Rosenthal A.
        • Jouet M.
        • Kenwrick S.
        Aberrant splicing of neural cell adhesion molecule L1 mRNA in a family with X-linked hydrocephalus.
        Nature Genet. 1992; 2: 107-112
        • Van Camp G.
        • Vits L.
        • Coucke P.
        • et al.
        A duplication in the L1CAM gene associated with X-linked hydrocephalus.
        Nature Genet. 1993; 4: 421-425
        • Jouet M.
        • Rosenthal A.
        • MacFarlane J.
        • Kenwrick S.
        • Donnai D.
        A missense mutation confirms the L1 defect in X-linked hydrocephalus (HSAS).
        Nature Genet. 1993; 4: 331
        • Czeizel A.E.
        • Dudas I.
        Prevention of the first occurrence of neural-tube defects by periconceptional vitamin supplementation.
        New Engl J Med. 1992; 327: 1832-1835
        • Werler M.M.
        • Shapiro S.
        • Mitchell A.A.
        Periconceptional folic acid exposure and risk of occurrent neural tube defects.
        JAMA. 1993; 269: 1257-1261