Abstract
We investigated the etiology of hydrocephalus present at birth, i.e. of fetal hydrocephalus. Both inherited and dysraphic major malformations are very rare. Intraventricular hemorrhages and viral infections during pregnancy are among the proposed etiologies; they are supported more by anatomical, physiopathological and experimental findings than by clinical evidence. Cases of fetal intraventricular hemorrhages cited in the literature are anecdotical, and the reports fail to identify maternal or fetal predisposing factors. The role of viruses in the etiology of connatal hydrocephalus has been postulated on the basis of epidemiological considerations in human pathology and of a considerable amount of experimental studies in animals. Investigations were generally focused on aqueduct ependyma, but research should also address other structures involved in the genesis of hydrocephalus (choroid plexus, extraventricular CSF pathways, including arachnoid villi). Furthermore, experimental evidence has emerged concerning a number of toxins and of drugs administered during pregnancy, which are thought to be involved in the genesis of hydrocephalus: once more, the conclusions reached in these experimental trials lend further credence to the human epidemiological data linking pregnancy disturbances with fetal hydrocephalus. Since most of these toxic agents are also thought to induce major malformations, we could assume the degree of their effect to depend on the embryonal stage affected: the earlier the action, the worse the malformation.
Similar content being viewed by others
References
Angerpointner TA, Pockrandt L, Schroer KL (1990) Catamnestic investigations in children with congenital hydrocaphalus. Z Kinderchir 45:151–155
Chambers SE, Johnstone FD, Laing IA (1988) Ultrasound in utero diagnosis of choroid plexus haemorrhage. Br J Obstet Gynaecol 95:1317–1320
Chervenak FA, Berkowitz RL, Romero R, Tortora M, Mayden K, Duncan C, Mahoney MJ, Hobbins JC (1983) The diagnosis of fetal hydrocephalus. Am J Obstet Gynecol 147:703–716
Craig Jackson J, Blumhagen JD (1983) Congenital hydrocephalus due to prenatal intracranial hemorrhage. Pediatrics 72:344–346
Desmonts G, Couvreur J (1974) Congenital toxoplasmosis. A prospective study of 378 pregnancies. N Engl J Med 290:1110–1116
Friede RL (1989) Hydrocephalus —special pathology. In: Friede RL (ed) Developmental neuropathology. Springer, New York Berlin Heidelberg, pp 231–246
Friedman HM, Gilden DH, Lief FS, Rorke LB, Santoli D, Koprowski H (1975) Hydrocephalus produced by the 6/94 virus. Arch Neurol 32:408–413
Gautier A, Metz C, Chabaud JJ, Le Guern H, Alix D, Boog G, Lyon G (1990) Hemorragie intraventriculaire in utero. Arch Fr Pediatr 47:429–431
Gustavson EE, Chen H (1985) Goldenhar syndrome, anterior encephalocele and aqueductal stenosis following fetal primidone exposure. Teratology 32:13–17
Hambleton G, Wigglesworth JS (1976) Origin of intraventricular haemorrhage in the preterm infant. Arch Dis Child 51:651–659
Hudgins RJ, Edwards MSB, Goldstein R, Callen PW, Harrison MR, Filly RA, Golbus MS (1988) Natural history of fetal ventriculomegaly. Pediatrics 82:692–697
Jacobson CB, Berlin CM (1972) Possible reproductive detriment in LSD users. JAMA 222:1367–1373
Jellinger G (1986) Anatomopathology of non-tumoral aqueductal stenosis. J Neurosurg Sci 30:1–16
Johnson RT (1975) Hydrocephalus and viral infections. Dev Med Child Neurol 17:807–816
Johnson RT (1982) Selective vulnerability of neural cells to viral infections. Adv Neurol 36:331–337
Johnson RT, Johnson KP (1968) Hydrocephalus as a sequela of experimental mumps virus infection in the hamster. J Neuropathol Exp Neurol 27:136–137
Johnson RT, Johnson KP (1969) Hydrocephalus as a sequela of experimental myxovirus infections. Exp Mol Pathol 10:68–80
Kaplan LC (1985) Congenital Dandy-Walker malformation associated with first trimester warfarin: a case report and literature review. Teratology 32:333–337
Kim MS, Elyaderani MK (1982) Sonographic diagnosis of cerobroventricular hemorrhage in utero. Radiology 142:479–480
Leidig E, Dannecker G, Pfeiffer KH, Salinas R, Pfeiffer J (1988) Intrauterine development of posthaemorrhagic hydrocephalus. Eur J Pediatr 147:26–29
Marlin AE, Gaskill SJ (1990) The etiology and management of the hydrocephalus in the preterm infant. In: Scott RM (ed) Hydrocephalus. (Concepts in neurosurgery) Williams & Wilkins, Baltimore, pp 67–78
Masters C, Alpers M, Kakulas B (1977) Pathogenesis of reovirus type 1 hydrocephalus in mice. Significance of aqueductal changes. Arch Neurol 34:18–28
McCarthy JW, Coble LL (1973) Intracranial hemorrhage and subsequent communicating hydrocephalus in a neonate with classical hemophilia. Pediatrics 51:122–124
McGahan JP, Haesslein HC, Meyers M, Ford KB (1984) Sonographic recognition of in utero intraventricular hemorrhage. AJR 142:171–173
Michejda M, Hodgen GD (1982) Induction of neural tube defects in nonhuman primates. In: Marois M (ed) Prevention of physical and mental congenital defects, part B. Liss, New York, pp 243–247
Overholser MD, Whitley JR, O'Dell BL, Hogan AG (1954) The ventricular system in hydrocephalic rat brains produced by a deficiency of vitamin B12 or folic acid in the maternal diet. Anat Rec 120:917–933
Pretorius DH, Singh S, Manco-Johnson ML, Rumack CM (1986) In utero diagnosis of intracranial hemorrhage resulting in fetal hydrocephalus. A case report. J Reprod Med 31:136–138
Russell DS (1949) Observation on the pathology of hydrocephalus. His Majesty's Stationery Office, London
Serlo W, Kirkinen P, Jouppila P, Herva R (1986) Prognostic signs in fetal hydrocephalus. Child's Nerv Syst 2:93–97
Sever LE (1982) Zinc deficiency and neural tube defects. J Pediatr 100:670–671
Sheinbergas MM (1976) Hydrocephalus due to prenatal infection with the lymphocytic choriomeningitis virus. Infection 4:185–191
Stempak JG (1964) Etiology of trypan blue induced antenatal hydrocephalus in the albino rat. Anat Rec 148:561–571
Stirling HF, Hendry M, Brown JK (1989) Prenatal intracranial haemorrhage. Dev Med Child Neurol 31:797–815
Warkany J (1970) Trends in teratologic research. Epilogue to the Third International Conference on Congenital Malformations. Teratology 3:89–91
Whitelaw A, Haines ME, Bolsover W, Harris E (1984) Factor V deficiency and antenatal intraventricular hemorrhage. Arch Dis Child 59:997–999
Woodard JC Newberne PM (1967) The pathogenesis of hydrocephalus in newborn rats deficient in vitamin B12. J Embryol Exp Morphol 17:177–187
Wunsch M, Kuenzig HJ (1989) Intrauterine Hirnblutung als Ursache eines Hydrocephalus. Geburtshilfe Frauenheilkd 49:394–395
Zalneraitis EL, Young RSK, Krishnamoorthy K (1979) Intracranial hemorrhage in utero as a complication of isoimmune thrombocytopenia. J Pediatrics 95:611–614
Zorzi C, Angonese I, Nardelli GB, Cantarutti F (1988) Spontaneous intraventricular haemorrhage in utero. Eur J Pediatr 148:83–85
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Giuffrè, R., Pastore, F.S. & De Santis, S. Connatal (fetal) hydrocephalus: an acquired pathology?. Child's Nerv Syst 11, 97–101 (1995). https://doi.org/10.1007/BF00303813
Issue Date:
DOI: https://doi.org/10.1007/BF00303813