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.
Similar content being viewed by others
References
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
Aikawa H, Kobayashi S, Suzuki K (1986) Aqueductal stenosis in 6-aminonicotinamide-treated sucking mice. Acta Neuropathol (Berl) 71:243–250
Berry RJ (1961) The inheritance and pathogenesis of hydrocephalus-3 in the mouse. J Pathol Bacteriol 81:157–167
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
Broit A, Sidman RJ (1972) New mutant mouse with communicating hydrocephalus and secondary aqueductal stenosis. Acta Neuropathol (Berl) 21:316–331
Carton CA, Perry JH, Winter A, Tennyson V (1956) Studies of hydrocephalus in C57 blank mice. Trans Am Neurol Assoc 81:147–149
Chamberlain JG (1970) Early neurovascular abnormalities underlying 6-aminonicotinamide (6-AN) induced congenital hydrocephalus in rats. Teratology 3:377–388
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
Christoferson LA, Leech RW, Hazen GA (1977) Bovine hydrocephalus in North Dakota: a survey and morphologic study. Surg Neurol 7:165–170
Clark FH (1932) Hydrocephalus: a hereditary character in the house mouse. Proc Natl Acad Sci USA 18:654–656
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
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
Cordon HW, Peer LA, Bernhard WG (1961) The relation of the teratogenic action of cortisone to liver transaminase activity. Biol Neonate 3:36–48
D'Agostino AN, Kernohan JW, Brown JR (1963) The Dandy-Walker syndrome. J Neuropathol Exp Neurol 22:450–470
Deol MS (1964) The origin of the abnormalities of inner ear in Dreher mice. J Embryol Exp Morphol 12:727–733
Dohrmann GJ (1972) Cervical spinal cord in experimental hydrocephalus. J Neurosurg 37:538–542
Faulhauer K, Donauer D (1985) Experimental hydrocephalus and hydrosyringomyelia in the cat — radiological findings. Acta Neurochir (Wien) 72:72–80
Gardner WJ (1965) Hydrodynamic mechanism of syringomyelia: its relationship to myelocele. J Neurosurg Psychiatry 28:247–259
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
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
Granholm L, Radberg C (1963) Congenital communicating hydrocephalus. J Neurosurg 20:338–343
Green MC (1970) The developmental effects of congenital communicating hydrocephalus (ch) in the mouse. Dev Biol 23:585–608
Gruneberg H (1943) Two new mutant genes in the house mouse. J Genet 45:22–28
Gruneberg H (1943) Congenital hydrocephalus in the mouse: a case of spurious pleiotropism. J Genet 45:1–21
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
Haring OM (1960) Cardiac malformations in rats induced by exposure of the mother to carbon dioxide during pregnancy. Circ Res 8:1218–1227
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
Higashi K, Noda Y, Mifune H (1987) Pathological studies on the brain of congenital hydrocephalic rats. Shoni No Noshinkei 12:1–9
Ingraham FD, Alexander E Jr, Maston DD (1947) Experimental hydrocephalus. J Neurosurg 4:164–170
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
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
Johnson RT, Johnson KP (1969) Hydrocephalus as a sequela of experimental myxovirus infections. Exp Mol Pathol 10:68–80
Kalter H (1963) Experimental mammalian teratogenesis, a study of galactoflavin-induced hydrocephalus in mice. J Morphol 112:303–317
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
Kameyama Y, Hoshino K (1972) Postnatal manifestation of hydrocephalus in mice caused by prenatal X-radiation. Congen Anomal 12:1–9
Kohn DF, Chinookoswong N, Chou SM (1981) A new model of congenital hydrocephalus in the rat. Acta Neuropathol (Berl) 54:211–218
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
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
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
Masters C, Alpers M, Kakulas B (1977) Pathogenesis of Reovirus type 1 hydrocephalus in mice — significance of aqueductal stenosis. Arch Neurol 34:18–28
Millen JW, Woolam DH, Lamming GE (1953) Hydrocephalus associated with deficiency of vitamin A. Lancet 2:1234–1236
Mori T (1985) A study of the tellurium-induced experimental hydrocephalus. Neuropathology 6:355–365
Murphy ML (1962) Teratogenic effects in rats growth inhibiting chemicals including studies on thalidomide. Clin Proc Child Hosp 18:307–322
Ohba N (1958) Formation of embryonic abnormalities of the mouse by a viral infection of mother animals. Acta Pathol Jpn 8:127–138
Oi S (1992) Is the hydrocephalic state progressive to become irreversible during fetal life? Surg Neurol 37:66–68
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
Oi S, Katayama K, Mochizuki M, Matsumoto S (1990) Pathophysiology and postnatal outcome of fetal hydrocephalus. Child's Nerv Syst 6:338–345
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
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
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
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
Raimondi AJ, Clark SJ, McLone DG (1976) Pathogenesis of aqueductal occlusion in congenital murine hydrocephalus. J Neurosurg 45:66–77
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
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
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
Van Dyke JH, Ritchey MG (1962) Colchicine influence during embryonic development in rats. Anat Rec 97:375–376
Vickers TH (1961) Die experimentelle Erzeugung der Arnold-Chiari Missbildung durch Trypan-blue. Beitr Pathol Anat 124:295–310
Warkany J, Wilson JG, Geiger JF (1958) Myeloschisis and myelomeningocele produced experimentally in the rat. J Comp Neurol 109:35–64
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
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
Yoon GM, Slaney J (1972) Hydrocephalus: a new mutation in the Syrian golden hamster. J Hered 36:344–346
Zimmermann K (1933) Eine neue Mutation der Hausmaus: “hydrocephalus.” Z Indukt Abstamm Vererbungsl 64:176–180
Author information
Authors and Affiliations
Rights 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
Received:
Issue Date:
DOI: https://doi.org/10.1007/BF00301016