Abstract
Two cases of prenatal diagnosis of lissencephaly are presented in the context of a series of 118 cases of prenatally diagnosed hydrocephalus. Within this series there was one case of Walker-Warburg syndrome and another of Miller-Dieker syndrome. It is stressed that the cases reported here of ventriculomegaly diagnosed in utero show a very different outcome from those in published studies of fetal hydrocephalus which only deal with patients in whom the diagnosis was determined after birth. In those postnatal series there is a considerable selection bias, and the fate of the fetuses reported here was much worse than in postnatal series. Of the 118 fetuses 6 had fetal infections, 6 had chromosomal abnormalities, 26 had associated spina bifida, 64 fetuses had associated other anomalies, and only 28 had isolated hydrocephalus. Although it is difficult to determine the prognosis individually after prenatal diagnosis of ventriculomegaly, the data presented here may be helpful in counseling parents prenatally. The counseling should be performed with the collaboration of obstetricians, pediatricians, surgeons, and geneticists.
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References
Birnholz JC, Frigoletto FD (1981) Antenatal treatment of hydrocephalus. N Engl J Med 303:1021–1023
Bromley B, Frigoletto FD, Benacerraf BR (1991) Mild fetal lateral cerebral ventriculomegaly: clinical course and outcome. Am J Obstet Gynecol 164:863–867
Chervenak FA, Duncan C, Ment LR, Hobbins JC, McClure M, Scott D, Berkowitz RL (1984) Outcome of fetal ventriculomegaly. Lancet I:179–181
Chervenak FA, Isaacson G, Mahoney MJ, Tortora M, Mesologites T, Hobbins JC (1984) The obstetric significance of holoprosencephaly. Obstet Gynecol 63:115–121
Clewell WH, Johnson ML, Meier PR, 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
Clewell WH, Meier PR, Manchester DK, Manco-Johnson ML, Pretorius DH, Hendee RW (1985) Ventriculomegaly: evaluation and management. Semin Perinatol 9:98–102
Crowe C, Jassani M, Dickermann L (1985) The prenatal diagnosis of Warburg syndrome (abstract). Am J Hum Genet 37:A214
Dennis M, Fitz CR, Netley CT, Sugar J, Harwood-Nash DCF, Hendrick EB, Hoffman HF, Humphreys RP (1981) The intelligence of hydrocephalic children. Arch Neurol 38:607–615
Dobyns WB (1987) Developmental aspects of lissencephaly and the lissencephaly syndromes. In: Gilbert EF, Opitz JM (eds) Genetic aspects of developmental pathology. Liss, New York, pp 225–241
Habib Z (1981) Genetics and genetic conselling in neonatal hydrocephalus. Obstet Gynecol Surv 36:529–534
Hanefeld F, Stephani U, Christen HJ, Mortazavi-Ravari MS (1990) Migrationsstörungen im Zentralnervensystem. In: Scheffner D (ed) Hirnentwicklung und Epilepsie. (Epilepsie 90) Einhorn-Presse, Berlin, p 39–46
Hogge WA, Dungan JS, Brooks MP, Dilks SA, Abbitt PL, Thiagarajah S, Ferguson JE (1990) Diagnosis and management of prenatally detected myelomeningocele. A preliminary report. Am J Obstet Gynecol 163:1061–1065
Laurence KM, Coates S (1962) The natural history of hydrocephalus. Detailed analysis of 182 unoperated cases. Arch Dis Child 37:345–362
Lorber J (1972) Spina bifida cystica. Results of treatment of 270 consecutive cases with criteria for selection for the future. Arch Dis Child 47:854–873
Lorber J, Zachary RB (1968) Primary congenital hydrocephalus: long-term results of controlled therapeutic trial. Arch Dis Child 43:516–527
McCullough DC, Balzer-Martin LA (1982) Current prognosis in overt neonatal hydrocephalus. J Neurosurg 57:378–383
Miller G, Ladda RL, Towfighi J (1991) Cerebro-ocular dysplasia-muscular dystrophy (Walker Warburg) syndrome. Findings in 20-week-old fetus. Acta Neuropathol 82:234–238
Nakayama DK, Harrison MR, Berger MS, Chinn DH, Halks-Miller M, Edwards MS (1983) Correction of congenital hydrocephalus in utero. I. The model: intracisternal kaolin produces hydrocephalus in fetal lambs and rhesus monkeys. J Pediatr Surg 18:331–337
Nicolaides KH, Campbell S, Gabbe SG, Guidetti R (1986) Ultrasound screening for spina bifida: cranial and cerebellar signs. Lancet II;72–74
Oi S (1992) In utero diagnosis and management of fetal CNS malformations: advances in 1991. Crit Rev Neurosurg 2:171–176
Raimondi AF, Soare P (1974) Intellectual development in shunted hydrocephalic children. Am J Dis Child 127:664–671
Saltzman DH, Krauss CM, Goldman JM, Benacerraf BR (1991) Prenatal diagnosis of lissencephaly. Prenat Diagn 11: 139–143
Shurtleff DB, Kronmal R, Foltz EL (1975) Follow-up comparison of hydrocephalus with and without myelomeningocele. J Neurosurg 42:61–68
Wald NJ, Cuckle HS (1987) Recent advances in screening for neural tube defects and Down's syndrome. In: Rodeck C (ed) International practive and research. Fetal diagnosis of genetic defects. (Ballière's clinical obstetrics and gynaecology, vol 1, no 3) Baillière Tindall, London, pp 649–676
Wilson RD, Hitchman D, Wittman BK (1989) Clinical follow-up prenatally diagnosed isolated ventriculomegaly, microcephaly and encephalocele. Fetal Ther 4:49–57
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Holzgreve, W., Feil, R., Louwen, F. et al. Prenatal diagnosis and management of fetal hydrocephaly and lissencephaly. Child's Nerv Syst 9, 408–412 (1993). https://doi.org/10.1007/BF00306194
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DOI: https://doi.org/10.1007/BF00306194