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Prognosis for fetuses with antenatally detected myelomeningocele

Published online by Cambridge University Press:  10 October 2008

Stephen Sturgiss*
Affiliation:
Department of Obstetrics and Gynaecology, University of Newcastle upon Tyne
Stephen Robson
Affiliation:
Department of Obstetrics and Gynaecology, University of Newcastle upon Tyne
*
Dr SN Sturgiss, Department of Fetal Medicine, Leazes Wing, Royal Victoria Infirmary, Newcastle upon Tyne NE1 4LP, UK

Extract

The ultrasound diagnosis of myelomeningocele has been greatly improved by the recognition of associated abnormalities in the fetal skull and brain, potentially leading to an increased number of cases detected during pregnancy. Unfortunately, the counselling of potential parents about the natural history and prognosis of myelomeningocele is frequently based on extrapolations from data in which the diagnosis was made following delivery. Moreover, much of this information predates recent advances in paediatric care. In 1981, Leonard stated “as most children (with myelomeningocele) will grow to be productive, participating adults, then perhaps we should consider myelomeningocele a new disease”1. It is, therefore, appropriate to review recent outcome data for children with myelomeningocele, emphasising those studies where the diagnosis was made before delivery. This review will concentrate on issues of particular concern to the perinatal obstetrician, such as the reliability of sonographic diagnosis, the frequency of karyotypic anomalies, the likely longterm infant outcome and the most appropriate mode of delivery.

Type
Articles
Copyright
Copyright © Cambridge University Press 1995

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References

1Leonard, CO, Freeman, JM. Spina bifida: a new disease. Pediatr 1981; 68: 136–37.CrossRefGoogle ScholarPubMed
2Robinson, HP, Hood, VD, Adam, AH, Gibson, AAM, Ferguson-Smith, MA. Diagnostic ultrasound: early detection of fetal neural tube defects. Obstet Gynecol 1980; 56: 705–10.Google ScholarPubMed
3Hobbins, J.Diagnosis and management of NTDs today. N Engl J Med 1991; 324: 690–91.CrossRefGoogle ScholarPubMed
4Nicolaides, KH, Gabbe, SG, Campbell, S, Guidetti, R. Ultrasound screening for spina bifida: cranial and cerebellar signs. Lancet 1986 (ii): 7274.CrossRefGoogle Scholar
5Campbell, J, Gilbert, WM, Nicolaides, KH, Campbell, S. Ultrasound screening for spina bifida: cranial and cerebellar signs in a high-risk population. Obstet Gynecol 1987; 70: 247–50.Google ScholarPubMed
6Nyberg, DA, Mack, LA, Hirsch, J, Mahony, BS. Abnormalities of fetal cranial contour in sonographic detection of spina bifida: evaluation of the “lemon sign”. Radiology 1988; 167: 387–92.CrossRefGoogle ScholarPubMed
7Chambers, SE, Muir, BB, Bell, JE. “Bullet”-shaped head in fetuses with spina bifida: a pointer to the spinal lesion. J Clin Ultrasound 1988; 16: 2528.CrossRefGoogle Scholar
8Thiagarajah, S, Henke, J, Hogge, WA, Abbitt, PL, Breeden, N, Ferguson, JE. Early diagnosis of spina bifida: the value of cranial ultrasound markers. Obstet Gynecol 1990; 76: 5457.Google ScholarPubMed
9Van den Hof, MC, Nicolaides, KH, Campbell, J, Campbell, S. Evaluation of the lemon and banana signs in one hundred thirty fetuses with open spina bifida. Am J Obstet Gynecol 1990; 162 322–27.CrossRefGoogle ScholarPubMed
10Pilu, G, Romero, R, Reece, EA, Goldstein, I, Hobbins, JC, Bovicelli, L. Subnormal cerebellum in fetuses with spina bifida. Am J Obstet Gynecol 1988; 158: 1052–56.CrossRefGoogle ScholarPubMed
11Ball, RH, Filly, RA, Goldstein, RB, Callen, PW. The lemon sign: not a specific indicator of meningomyelocele. J Ultrasound Med 1993; 12: 131–34.CrossRefGoogle Scholar
12Bell, J, Gordon, A, Maloney, A. The association of hydrocephalus and Arnold-Chiari malformation with spina bifida in the fetus. Neuropathol Appl Neurobiol 1980; 6: 2939.CrossRefGoogle ScholarPubMed
13Blumenfeld, Z, Siegler, E, Bronshtein, M. The early diagnosis of neural tube defects. Prenat Diagn 1993; 13: 863–71.CrossRefGoogle ScholarPubMed
14Benacerraf, BR, Stryker, J, Frigoletto, FD Jr. Abnormal US appearance of the cerebellum (banana sign): indirect sign of spina bifida. Radiology 1989; 171: 151–53.CrossRefGoogle ScholarPubMed
15Hogge, WA, Dungan, JS, Brooks, MP, Dilks, SA, Abbitt, PL, Thiagarajah, S, Ferguson, JE. Diagnosis and management of prenatally detected myelomeningocele: a preliminary report. Am J Obstet Gynecol 1990; 163: 1061–65.CrossRefGoogle ScholarPubMed
16Babcook, CJ, Goldstein, RB, Barth, RA, Damato, NM, Callen, PW, Filly, RA. Prevalence of ventriculomegaly in association with myelomeningocele: correlation with gestational age and severity of posterior fossa deformity. Radiology 1994; 190: 703707.CrossRefGoogle ScholarPubMed
17Drugan, A, Zador, IE, Syner, FN, Sokol, RJ, Sacks, AJ, Evans, MI. A normal ultrasound does not obviate the need for amniocentesis in patients with elevated serum AFP. Obstet Gynecol 1988; 72: 627–30.Google ScholarPubMed
18Wald, N, Cuckle, HS, Haddow, JE, Doherty, RA, Knight, GJ, Palomaki, GE. Sensitivity of ultrasound in detecting spina bifida. N Engl J Med 1991; 324: 769–72.Google Scholar
19Platt, LD, Feuchtbaum, L, Filly, R, Lustig, L, Simon, M, Cunningham, GC. The California Maternal Serum alpha-Fetoprotein Screening Program: the role of ultrasonography in the detection of spina bifida. Am J Obstet Gynecol 1992; 166: 1328–29.CrossRefGoogle ScholarPubMed
20Nyberg, DA, Mack, LA. The spine and neural tube defects. In: Nyberg, DA, Mahony, BSPretorius, DH eds, Diagnostic ultrasound of fetal anomalies St Louis: Mosby Year Book, 1990: 146202.Google Scholar
21Babcook, CJ, Goldstein, RB, Filly, RA. Prenatally detected fetal myelomeningocele: is karyotype analysis warranted? Radiology 1995; 194: 491–94.CrossRefGoogle ScholarPubMed
22Hunt, GM, Whitaker, RH. The pattern of congenital renal anomalies associated with neural tube defects. Dev Med Child Neurol 1987; 29: 9195.CrossRefGoogle ScholarPubMed
23Nyberg, DA, Shepard, T, Mack, LA, Hirsch, J, Luthy, D, Fitzsimmons, J. Significance of a single umbilical artery in fetuses with central nervous system malformations. J Ultrasound Med 1988; 7: 265–73.CrossRefGoogle ScholarPubMed
24Luthy, DA, Wardinsky, T, Shurtleff, DB, Hollenbach, KA, Hickok, DE, Nyberg, DA, Benedetti, TJ. Cesarean section before the onset of labor and subsequent motor function in infants with meningomyelocele diagnosed antenatally. New Engl J Med 1991; 324: 662–66.CrossRefGoogle ScholarPubMed
25Lorber, J. Results of treatment of myelomeningocele. Dev Med Child Neurol 1971; 13: 279303.CrossRefGoogle ScholarPubMed
26Hunt, GM. Open spina bifida: outcome for a complete cohort treated unselectively and followed into adulthood. Dev Med Child Neurol 1990; 32: 108–18.CrossRefGoogle ScholarPubMed
27Laishram, H, Kennedy, R, Maroun, F, Price, D. The impact of spina bifida on the medical services of Newfoundland and Labrador. J Pediatr Surg 1993; 28: 1098–101.CrossRefGoogle ScholarPubMed
28Bensen, JT, Dillard, RG, Burton, BK. Open spina bifida: does Cesarean Section delivery improve prognosis? Obstet Gynecol 1988; 71: 532–34.Google ScholarPubMed
29Nelson, MD, Bracchi, M, Naidich, TP, McLone, DG. The natural history of repaired myelomeningocele. Radiographics 1988; 8: 695706.CrossRefGoogle ScholarPubMed
30McLaughlin, JF, Shurtleff, DB, Lamers, JY, Stuntz, JT, Hayden, PW, Kropp, RL. Influence of prognosis on decisions regarding the care of newborns with myelodysplasia. New Engl J Med 1985; 312: 1589–91.CrossRefGoogle ScholarPubMed
31Adams, MM, Greenberg, F, Khoury, MJ, Marks, JS, Oakley, GP Jr. Survival of infants with spina bifida-Atlanta, 1972–1979. Am J Dis Child 1985; 139: 518–23.Google ScholarPubMed
32Adams, MM, Greenberg, F, Khoury, MJ, Marks, JS, Oakley, GP. Trends in clinical characteristics of infants with spina bifida - Atlanta, 1972–1979. Am J Dis Child 1985; 139: 514–17.Google ScholarPubMed
33McLone, DG, Czyzewski, D, Raimondi, AJ, Sommers, RC. Central nervous system infections as a limiting factor in the intelligence of children with myelomeningocele. Pediatrics 1982; 70: 338–42.CrossRefGoogle Scholar
34Mapstone, TB, Rekate, HL, Nulsen, FE, Dixon, MS, Glaser, N, Jaffe, M. Relationship of CSF shunting and IQ in children with myelomeningocele: a retrospective analysis. Childs Brain 1984; 11: 112–18.Google ScholarPubMed
35Cass, AS, Bloom, BA, Luxenberg, M. Sexual function in adults with myelomeningocele. J Urology 1986; 136: 425–26.CrossRefGoogle ScholarPubMed
36Malone, PS; Wheeler, RA; Williams, JE. Continence in patients with spina bifida: long term results. Arch Dis Child 1994; 70: 107–10.CrossRefGoogle ScholarPubMed
37Park, TS, Hoffman, HJ, Hendrick, B, Humphreys, RP. Experience with surgical decompression of the Arnold-Chiari malformation in young infants with myelomeningocele. Neurosurgery 1983; 13: 147–52.CrossRefGoogle ScholarPubMed
38Smith, GK, Smith, ED. Selection for treatment in spina bifida cystica Br Med J 1973; (4): 189–97.CrossRefGoogle ScholarPubMed
39Vintzileos, AM, Campbell, WA, Nochimson, DJ, Weinbaum, PJ. Antenatal evaluation and management of ultrasonically detected fetal anomalies. Obstet Gynecol 1987; 69: 640–60.Google ScholarPubMed
40Seller, MJ. Is antenatal selection for spina bifida possible?. Br Med J 1990; 301: 251–52.CrossRefGoogle ScholarPubMed
41Kollias, SS, Goldstein, RB, Cogen, PH, Filly, RA. Prenatally detected myelomeningoceles: sonographic accuracy in estimation of the spinal level. Radiology 1992; 185: 109–12.CrossRefGoogle ScholarPubMed
42Hunt, GM, Lewin, W, Gleave, J, Gairdner, D. Predictive factors in open myelomeningocele with special reference to sensory level. Br Med J 1973; (4): 197201.CrossRefGoogle ScholarPubMed
43Stark, G, Drummond, M. Spina bifida as an obstetric problem. Dev Med Child Neurol [Suppl] 1970; 12: 157–60.CrossRefGoogle Scholar
44Cochrane, D, Aronyk, K, Sawatzky, B, Wilson, D, Steinbok, P. The effects of labor and delivery on spinal cord function and ambulation in patients with meningomyelocele. Childs Nerv Syst 1991; 7: 312–15.CrossRefGoogle ScholarPubMed
45Shurtleff, DB, Luthy, DA, Nyberg, DA, Benedetti, TJ, Mack, LA. Meningomyelocele: management in utero and post natum. Ciba Foundation Symposium 1994; 181: 270–86.Google ScholarPubMed