Skip to main content
Log in

Experimental models of congenital hydrocephalus and comparable clinical problems in the fetal and neonatal periods

  • Review Paper
  • Published:
Child's Nervous System Aims and scope Submit manuscript

Abstract

Morphological and developmental changes of the ventricular system are analyzed in three major experimental models of congenital hydrocephalus in the rat: 6-aminonicotinamide (6-AN)-induced and LEW/Jms and HTX mutant hydrocephalus. The clinically comparable forms of hydrocephalus and problems occurring during each period of intrauterine hydrocephalus are then discussed. Comparative morphological study revealed that 6-AN-induced hydrocephalus was comparable to the Dandy-Walker syndrome and that the critical period regarding this syndrome in fetal life was at the time of “legal termination”. The LEW/Jms and HTX mutant models were identical with regard to the form of progressive hydrocephalus in the postnatal period, but the condition underlying the hydrocephalus during the fetal period differed. The LEW/Jms model was comparable to primary congenital aqueductal stenosis (aqueductal agenesis), and the hydrocephalic state appeared in the period of “intrauterine preservation” before pulmonary maturation was completed. On the other hand, the HTX fetuses demonstrated secondary change of the aqueduct in the perinatal period, although the model was considered to be of congenital communicating hydrocephalus. Cerebrospinal fluid (CSF) dynamics studied in the fetuses with 6-AN-induced hydrocephalus disclosed considerable pathophysiology comparable to “hydromyelic hydrocephalus.” The historical trends of animal experimental models of congenital hydrocephalus are reviewed and comparable clinical problems suggested by those models discussed further.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  1. Adeloye A, Warkany J (1976) Experimental congenital hydrocephalus. A review with special consideration of hydrocephalus produced by zinc deficiency. Child's Brain 2:325–360

    Google Scholar 

  2. Aikawa H, Kobayashi S, Suzuki K (1986) Aqueductal stenosis in 6-aminonicotinamide-treated sucking mice. Acta Neuropathol (Berl) 71:243–250

    Google Scholar 

  3. Berry RJ (1961) The inheritance and pathogenesis of hydrocephalus-3 in the mouse. J Pathol Bacteriol 81:157–167

    Google Scholar 

  4. Brent RL, Averich E, Drapiewski VA (1961) Production of congenital malformations using tissue antibodies. I. Kidney antisera. Proc Soc Exp Biol Med 106:523–526

    Google Scholar 

  5. Broit A, Sidman RJ (1972) New mutant mouse with communicating hydrocephalus and secondary aqueductal stenosis. Acta Neuropathol (Berl) 21:316–331

    Google Scholar 

  6. Carton CA, Perry JH, Winter A, Tennyson V (1956) Studies of hydrocephalus in C57 blank mice. Trans Am Neurol Assoc 81:147–149

    Google Scholar 

  7. Chamberlain JG (1970) Early neurovascular abnormalities underlying 6-aminonicotinamide (6-AN) induced congenital hydrocephalus in rats. Teratology 3:377–388

    Google Scholar 

  8. Chamberlain JG, Nelson MM (1963) Multiple congenital abnormalities in the rat resulting from acute maternal niacin deficiency during pregnancy. Proc Soc Exp Biol Med 112:836–840

    Google Scholar 

  9. Christoferson LA, Leech RW, Hazen GA (1977) Bovine hydrocephalus in North Dakota: a survey and morphologic study. Surg Neurol 7:165–170

    Google Scholar 

  10. Clark FH (1932) Hydrocephalus: a hereditary character in the house mouse. Proc Natl Acad Sci USA 18:654–656

    Google Scholar 

  11. Clewell WH, Johnson ML, Meier PR Jr, Newkirk JB, Zide SL, Hendee RW, Bowes WA, Hecht F, O'Keeffe D, Henry GP, Shikes RH (1982) A surgical approach to the treatment of fetal hydrocephalus. N Engl J Med 306:1320–1325

    Google Scholar 

  12. Cohlam SQ, Stone SM (1955) Congenital malformation of the brain produced by exposure of the pregnant rat to rubella virus. Am J Dis Child 90:616–617

    Google Scholar 

  13. Cordon HW, Peer LA, Bernhard WG (1961) The relation of the teratogenic action of cortisone to liver transaminase activity. Biol Neonate 3:36–48

    Google Scholar 

  14. D'Agostino AN, Kernohan JW, Brown JR (1963) The Dandy-Walker syndrome. J Neuropathol Exp Neurol 22:450–470

    Google Scholar 

  15. Deol MS (1964) The origin of the abnormalities of inner ear in Dreher mice. J Embryol Exp Morphol 12:727–733

    Google Scholar 

  16. Dohrmann GJ (1972) Cervical spinal cord in experimental hydrocephalus. J Neurosurg 37:538–542

    Google Scholar 

  17. Faulhauer K, Donauer D (1985) Experimental hydrocephalus and hydrosyringomyelia in the cat — radiological findings. Acta Neurochir (Wien) 72:72–80

    Google Scholar 

  18. Gardner WJ (1965) Hydrodynamic mechanism of syringomyelia: its relationship to myelocele. J Neurosurg Psychiatry 28:247–259

    Google Scholar 

  19. Garro F, Pentschew A (1964) Neonatal hydrocephalus in the offspring of rats fed during pregnancy non-toxic amounts of tellurium. Arch Psychiatr Nervenkr 206:272–280

    Google Scholar 

  20. Gillman J, Gilbert C, Gillman T, Spence I (1948) A preliminary report on hydrocephalus, spina bifida, and other congenital anomalies in the rat produced by trypan blue. S Afr J Med Sci 13:47–90

    Google Scholar 

  21. Granholm L, Radberg C (1963) Congenital communicating hydrocephalus. J Neurosurg 20:338–343

    Google Scholar 

  22. Green MC (1970) The developmental effects of congenital communicating hydrocephalus (ch) in the mouse. Dev Biol 23:585–608

    Google Scholar 

  23. Gruneberg H (1943) Two new mutant genes in the house mouse. J Genet 45:22–28

    Google Scholar 

  24. Gruneberg H (1943) Congenital hydrocephalus in the mouse: a case of spurious pleiotropism. J Genet 45:1–21

    Google Scholar 

  25. Gruneberg H, Wickramaratne GA (1974) A re-examination of two skeletal mutants of the mouse, vestigital-tail (vt) and congenital hydrocephalus (ch). J Embryol Exp Morphol 31:207–222

    Google Scholar 

  26. Haring OM (1960) Cardiac malformations in rats induced by exposure of the mother to carbon dioxide during pregnancy. Circ Res 8:1218–1227

    Google Scholar 

  27. Harvey M, Friedman MD, Donald H, Gilden MD, et al (1975) Hydrocephalus produced by the 6/94 virus: a parainfluenza type 1 isolated from multiple sclerosis brain tissue. Arch Neurol 32:408–415

    Google Scholar 

  28. Higashi K, Noda Y, Mifune H (1987) Pathological studies on the brain of congenital hydrocephalic rats. Shoni No Noshinkei 12:1–9

    Google Scholar 

  29. Ingraham FD, Alexander E Jr, Maston DD (1947) Experimental hydrocephalus. J Neurosurg 4:164–170

    Google Scholar 

  30. Jensh RP, Magalhaes H (1962) The effect of whole body X-irradiation on the central nervous system of golden hamster embryos. Proc Penn Acad Sci 36:194–199

    Google Scholar 

  31. Johnson RT, Johnson KP (1968) Hydrocephalus following viral infection: the pathology of aqueductal stenosis developing after experimental mumps virus infection. J Neuropathol Exp Neurol 27:591–606

    Google Scholar 

  32. Johnson RT, Johnson KP (1969) Hydrocephalus as a sequela of experimental myxovirus infections. Exp Mol Pathol 10:68–80

    Google Scholar 

  33. Kalter H (1963) Experimental mammalian teratogenesis, a study of galactoflavin-induced hydrocephalus in mice. J Morphol 112:303–317

    Google Scholar 

  34. Kalter H, Walkany J (1957) Congenital malformations in inbred strains of mice induced by riboflavin-deficient galactoflavin-containing diets. J Exp Zool 136:531–566

    Google Scholar 

  35. Kameyama Y, Hoshino K (1972) Postnatal manifestation of hydrocephalus in mice caused by prenatal X-radiation. Congen Anomal 12:1–9

    Google Scholar 

  36. Kohn DF, Chinookoswong N, Chou SM (1981) A new model of congenital hydrocephalus in the rat. Acta Neuropathol (Berl) 54:211–218

    Google Scholar 

  37. Koyama T (1970) Erzeugung von Missbildungen im Gehirn durch Methyl-Nitroso-Harnstoff und Äthyl-Nitrose-Harnstoff an SD-JCL Ratten. Arch Jpn Chir 39:233–254

    Google Scholar 

  38. Lecyk M (1965) The effect of hypothermia applied in the given stages of pregnancy on the number and form of vertebrae in the offspring of white mice. Experientia 21:452–453

    Google Scholar 

  39. Lindauer MA, Griffith JQ Jr (1938) Cerebrospinal pressure, hydrocephalus and blood pressure in the cat following intracisternal injection of colloidal kaolin. Proc Soc Exp Biol 39:547–549

    Google Scholar 

  40. Masters C, Alpers M, Kakulas B (1977) Pathogenesis of Reovirus type 1 hydrocephalus in mice — significance of aqueductal stenosis. Arch Neurol 34:18–28

    Google Scholar 

  41. Millen JW, Woolam DH, Lamming GE (1953) Hydrocephalus associated with deficiency of vitamin A. Lancet 2:1234–1236

    Google Scholar 

  42. Mori T (1985) A study of the tellurium-induced experimental hydrocephalus. Neuropathology 6:355–365

    Google Scholar 

  43. Murphy ML (1962) Teratogenic effects in rats growth inhibiting chemicals including studies on thalidomide. Clin Proc Child Hosp 18:307–322

    Google Scholar 

  44. Ohba N (1958) Formation of embryonic abnormalities of the mouse by a viral infection of mother animals. Acta Pathol Jpn 8:127–138

    Google Scholar 

  45. Oi S (1992) Is the hydrocephalic state progressive to become irreversible during fetal life? Surg Neurol 37:66–68

    Google Scholar 

  46. Oi S, Yamada H, Matsumoto S (1989) A prenatal CSF shunt procedure for fetal hydrocephalus, animal experimental model — pressure dynamics of intrauterine hydrocephalus and fetal ventriculo-mater peritoneal (FV-MP) shunt (in Japanese). Shoni No Noshinkei (Nerv Syst Child) 14:215–221

    Google Scholar 

  47. Oi S, Katayama K, Mochizuki M, Matsumoto S (1990) Pathophysiology and postnatal outcome of fetal hydrocephalus. Child's Nerv Syst 6:338–345

    Google Scholar 

  48. Oi S, Katayama K, Mochizuki M, Matsumoto S (1990) Factors affecting prognosis of intrauterine hydrocephalus diagnosed in the third trimester: computerized data analysis of controversies in fetal surgery. Neurol Med Chir (Tokyo) 30:456–461

    Google Scholar 

  49. Oi S, Kudo S, Urui S, et al (1991) Hydromyelic hydrocephalus — correlation of hydromyelia with various stages of hydrocephalus in postshunt isolated compartments. J Neurosurg 74:371–379

    Google Scholar 

  50. Oi S, Sato O, Matsumoto S (1994) A new classification of congenital hydrocephalus: prospective 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 process and chronological changes (in Japanese). Jpn J Neurosurg (Tokyo) 3:122–127

    Google Scholar 

  51. Raimondi AJ, Bailey OT, McLone DG, Lawson RF, Echeverry A (1973) The pathophysiology and morphology of murine hydrocephalus in hydrocephalus 3 and Ch mutants. Surg Neurol 1:50–55

    Google Scholar 

  52. Raimondi AJ, Clark SJ, McLone DG (1976) Pathogenesis of aqueductal occlusion in congenital murine hydrocephalus. J Neurosurg 45:66–77

    Google Scholar 

  53. Sasaki S, Goto H, Nagano H, Furuya K, Omata Y, et al (1983) Congenital hydrocephalus revealed in the inbred rat, LEW/Jms. Neurosurgery 13:548–554

    Google Scholar 

  54. Sato K, Naomi N, Akira S, Shozo I (1985) Experimental production of myeloschisis, Chiari malformation type II, posterior fossa hydrocephalus and other malformations related to craniospinal dysraphism in rat fetuses by single intragastric administration of ethylenethiourea. child's Nerv Syst 1:1–6

    Google Scholar 

  55. Thiersch JB (1957) Effects of 2–6 diamicropurine, 6 chlorpurine and thioguanine on rat litter in utero. Proc Soc Exp Biol Med 94:40–43

    Google Scholar 

  56. Van Dyke JH, Ritchey MG (1962) Colchicine influence during embryonic development in rats. Anat Rec 97:375–376

    Google Scholar 

  57. Vickers TH (1961) Die experimentelle Erzeugung der Arnold-Chiari Missbildung durch Trypan-blue. Beitr Pathol Anat 124:295–310

    Google Scholar 

  58. Warkany J, Wilson JG, Geiger JF (1958) Myeloschisis and myelomeningocele produced experimentally in the rat. J Comp Neurol 109:35–64

    Google Scholar 

  59. Yamada H, Oi S, Tamaki N, Matsumoto S, Taomoto K (1991) Congenital hydrocephalus mimicking Dandy-Walker syndrome induced by 6-aminonicotinamide injection in pregnant rat. Neurol Med Chir (Tokyo) 31:326–329

    Google Scholar 

  60. Yamada H, Oi S, Tamaki N, Matsumoto S, Sudo K (1992) Prenatal aqueductal stenosis as a cause of congenital hydrocephalus in the inbred rat LEW/Jms. Child's Nerv Syst 8:394–398

    Google Scholar 

  61. Yoon GM, Slaney J (1972) Hydrocephalus: a new mutation in the Syrian golden hamster. J Hered 36:344–346

    Google Scholar 

  62. Zimmermann K (1933) Eine neue Mutation der Hausmaus: “hydrocephalus.” Z Indukt Abstamm Vererbungsl 64:176–180

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Oi, S., Sato, O., Yamada, H. et al. Experimental models of congenital hydrocephalus and comparable clinical problems in the fetal and neonatal periods. Child's Nerv Syst 12, 292–302 (1996). https://doi.org/10.1007/BF00301016

Download citation

  • Received:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00301016

Key words

Navigation