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Published in: Child's Nervous System 4/2007

01-04-2007 | Original Paper

Assessment of neurosurgical outcome in children prenatally diagnosed with myelomeningocele and development of a protocol for fetal surgery to prevent hydrocephalus

Authors: Helder Zambelli, Edmur Carelli, Donizeti Honorato, Sérgio Marba, Giselle Coelho, Aline Carnevalle, Alexandre Iscaife, Elton da Silva, Ricardo Barini, Lourenço Sbragia

Published in: Child's Nervous System | Issue 4/2007

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Abstract

Introduction

Prenatal diagnosis of myelomeningocele (MMC) has permitted a better planning for optimum management of the disease. More recently, it has allowed for a possible intrauterine repair of the spinal defect.

Objective

To describe neurosurgical outcome in children with myelomeningocele and follow-up at a referral center in Fetal Medicine. Patients were characterized for the development of a protocol suitable for fetal surgery, and fetuses who were possible candidates for intrauterine surgery were identified.

Materials and methods

A retrospective descriptive analysis was performed of 98 cases of fetal myelomeningocele, seen at CAISM-UNICAMP, from January 1994 to December 2002, identifying cases with a possible indication for fetal surgery.

Results

Mean gestational age at diagnosis was 29 weeks (17–39); level of lesion was above the sacral region in 92.84%; association with hydrocephalus occurred in 78.57%. During clinical course, 82.5% of patients had neurogenic bladder and 60% had neural and mental deficits. Potential intrauterine repair rate was 11.57%, using criteria from the protocol developed in our service.

Conclusion

Myelomeningocele is associated with severe and frequent sequelae. In virtually 12% of our cases, fetal surgery could have been offered as a therapeutic option.
Literature
1.
go back to reference Adzick NS, Sutton LN, Crombleholme TM, Flake AW (1998) Successful fetal surgery for spina bifida. Lancet 352:1675–1676PubMedCrossRef Adzick NS, Sutton LN, Crombleholme TM, Flake AW (1998) Successful fetal surgery for spina bifida. Lancet 352:1675–1676PubMedCrossRef
2.
go back to reference Bruner JP, Tulipan N, Paschall RL et al (1999) Fetal surgery for myelomeningocele and the incidence of shunt-dependent hydrocephalus. JAMA 282:1819–1825PubMedCrossRef Bruner JP, Tulipan N, Paschall RL et al (1999) Fetal surgery for myelomeningocele and the incidence of shunt-dependent hydrocephalus. JAMA 282:1819–1825PubMedCrossRef
3.
go back to reference Bruner JP, Tulipan NE, Richards WO (1997) Endoscopic coverage of fetal open myelomeningocele in utero. Am J Obstet Gynecol 176:256–257PubMedCrossRef Bruner JP, Tulipan NE, Richards WO (1997) Endoscopic coverage of fetal open myelomeningocele in utero. Am J Obstet Gynecol 176:256–257PubMedCrossRef
4.
go back to reference Caldarelli M, DiRocco C, LaMarca F (1996) Shunt complications in the first postoperative year in children with myelomeningocele. Childs Nerv Syst 12:748–754PubMed Caldarelli M, DiRocco C, LaMarca F (1996) Shunt complications in the first postoperative year in children with myelomeningocele. Childs Nerv Syst 12:748–754PubMed
5.
go back to reference Dias M, McLode DG (1993) Hydrocephalus in the child with dysraphism. Neurosurg Clin N Am 4:715–726PubMed Dias M, McLode DG (1993) Hydrocephalus in the child with dysraphism. Neurosurg Clin N Am 4:715–726PubMed
6.
go back to reference Dillon CM, Davis BE, Duguay S et al (2000) Longevity of patients born with myelomeningocele. Eur J Pediatr Surg 10:33–34PubMedCrossRef Dillon CM, Davis BE, Duguay S et al (2000) Longevity of patients born with myelomeningocele. Eur J Pediatr Surg 10:33–34PubMedCrossRef
7.
go back to reference Drewek MJ, Bruner JP, Whetsell WO, Tulipan N (1997) Quantitative analysis of the toxicity of human amniotic fluid to cultured rat spinal cord. Pediatr Neurosurg 27:190–193PubMedCrossRef Drewek MJ, Bruner JP, Whetsell WO, Tulipan N (1997) Quantitative analysis of the toxicity of human amniotic fluid to cultured rat spinal cord. Pediatr Neurosurg 27:190–193PubMedCrossRef
8.
go back to reference Heffez DS, Aryanpur J, Cuello-Rotellini NA, Hutchins GM, Freeman JM (1993) Intrauterine repair of experimental surgically created dysraphism. Neurosurgery 32:1005–1010PubMedCrossRef Heffez DS, Aryanpur J, Cuello-Rotellini NA, Hutchins GM, Freeman JM (1993) Intrauterine repair of experimental surgically created dysraphism. Neurosurgery 32:1005–1010PubMedCrossRef
9.
go back to reference Heffez DS, Aryanpur J, Hutchins GM, Freeman JM (1990) The paralysis associated with myelomeningocele: clinical and experimental data implicating a preventable spinal cord injury. Neurosurgery 26:987–992PubMedCrossRef Heffez DS, Aryanpur J, Hutchins GM, Freeman JM (1990) The paralysis associated with myelomeningocele: clinical and experimental data implicating a preventable spinal cord injury. Neurosurgery 26:987–992PubMedCrossRef
10.
go back to reference Hirose S, Farmer DL, Albanese CT (2001) Fetal surgery for myelomeningocele. Curr Opin Obstet Gynecol 13:215–222PubMedCrossRef Hirose S, Farmer DL, Albanese CT (2001) Fetal surgery for myelomeningocele. Curr Opin Obstet Gynecol 13:215–222PubMedCrossRef
11.
go back to reference Hogge WA, Dungan JS, Brooks MP et al (1990) Diagnosis and management of prenatally detected myelomeningocele: a preliminary report. Am J Obstet Gynecol 163:1061–1064PubMed Hogge WA, Dungan JS, Brooks MP et al (1990) Diagnosis and management of prenatally detected myelomeningocele: a preliminary report. Am J Obstet Gynecol 163:1061–1064PubMed
12.
go back to reference Lary JM, Edmonds LD (1996) Prevalence of spina bifida at birth—United States 1983–1990: a comparison of two surveillance systems. MMWR 45:15–26PubMed Lary JM, Edmonds LD (1996) Prevalence of spina bifida at birth—United States 1983–1990: a comparison of two surveillance systems. MMWR 45:15–26PubMed
13.
go back to reference Luthy DA, Wardinsky T, Shurtleff DB et al (1991) Caesarean section before the onset of labor and subsequent motor function in infants with meningomyelocele diagnosed antenatally. N Engl J Med 324:662–666PubMedCrossRef Luthy DA, Wardinsky T, Shurtleff DB et al (1991) Caesarean section before the onset of labor and subsequent motor function in infants with meningomyelocele diagnosed antenatally. N Engl J Med 324:662–666PubMedCrossRef
14.
go back to reference McLone DG (1983) Results of treatment of children born with a myelomeningocele. Clin Neurosurg 30:407–412PubMed McLone DG (1983) Results of treatment of children born with a myelomeningocele. Clin Neurosurg 30:407–412PubMed
15.
go back to reference Meuli M, Meuli-Simmen C, Yingling CD et al (1995) Creation of myelomeningocele in utero: a model of functional damage from spinal cord exposure in fetal sheep. J Pediatr Surg 30:1028–1033PubMedCrossRef Meuli M, Meuli-Simmen C, Yingling CD et al (1995) Creation of myelomeningocele in utero: a model of functional damage from spinal cord exposure in fetal sheep. J Pediatr Surg 30:1028–1033PubMedCrossRef
16.
go back to reference Michejda M (1984) Intrauterine treatment of spina bifida: primate model. Z Kinderchir 39:259–261PubMed Michejda M (1984) Intrauterine treatment of spina bifida: primate model. Z Kinderchir 39:259–261PubMed
17.
go back to reference Reigel DH (1982) Spina bifida. In: Pediatric neurosurgery, surgery of the developing nervous system. Section of Pediatric Neurosurgery of the American Association of Neurological Surgeons. Grune & Stratton, New York, chap 2, pp 23–47 Reigel DH (1982) Spina bifida. In: Pediatric neurosurgery, surgery of the developing nervous system. Section of Pediatric Neurosurgery of the American Association of Neurological Surgeons. Grune & Stratton, New York, chap 2, pp 23–47
18.
go back to reference Shurtleff DB, Luthy DA, Nyberg DA, Benedetti TJ, Mack LA (1994) Meningomyelocele: management in utero and post natum. Ciba Found Symp 181:270–286PubMed Shurtleff DB, Luthy DA, Nyberg DA, Benedetti TJ, Mack LA (1994) Meningomyelocele: management in utero and post natum. Ciba Found Symp 181:270–286PubMed
19.
go back to reference Stark GD, Baker GCW (1967) The neurological involvement of the lower limbs in meningomyelocele. Dev Med Child Neurol 9:732–744CrossRef Stark GD, Baker GCW (1967) The neurological involvement of the lower limbs in meningomyelocele. Dev Med Child Neurol 9:732–744CrossRef
20.
go back to reference Stark GD, Drummond M (1971)The spinal cord lesion in myelomeningocele. Dev Med Child Neurol 13:1–14CrossRef Stark GD, Drummond M (1971)The spinal cord lesion in myelomeningocele. Dev Med Child Neurol 13:1–14CrossRef
21.
go back to reference Sutton LN, Adzick NS, Bilaniuk LT, Johnson MP, Crombleholme TM, Flake AW (1999) Improvement in hindbrain herniation demonstrated by serial fetal magnetic resonance imaging following fetal surgery for myelomeningocele. JAMA 282:1826–1831PubMedCrossRef Sutton LN, Adzick NS, Bilaniuk LT, Johnson MP, Crombleholme TM, Flake AW (1999) Improvement in hindbrain herniation demonstrated by serial fetal magnetic resonance imaging following fetal surgery for myelomeningocele. JAMA 282:1826–1831PubMedCrossRef
22.
go back to reference Urich H (1976) Malformations of the nervous system. Perinatal damage and related conditions in early life. In: Blackwood W, Corsellis JAN (eds) Greenfield’s neuropathology. Edward Arnold, London, pp 361–469 Urich H (1976) Malformations of the nervous system. Perinatal damage and related conditions in early life. In: Blackwood W, Corsellis JAN (eds) Greenfield’s neuropathology. Edward Arnold, London, pp 361–469
23.
go back to reference Velie EM, Shaw GM (1996) Impact of prenatal diagnosis on the birth prevalence and the risk estimates of neural tube defects in California, 1989–1991. Am J Epidemiol 144(5):473–479PubMed Velie EM, Shaw GM (1996) Impact of prenatal diagnosis on the birth prevalence and the risk estimates of neural tube defects in California, 1989–1991. Am J Epidemiol 144(5):473–479PubMed
24.
go back to reference Walsh DS, Adzick NS, Sutton LN, Johnson MP (2001) The rationale for in utero repair of myelomeningocele. Fetal Diagn Ther 16:312–322PubMedCrossRef Walsh DS, Adzick NS, Sutton LN, Johnson MP (2001) The rationale for in utero repair of myelomeningocele. Fetal Diagn Ther 16:312–322PubMedCrossRef
Metadata
Title
Assessment of neurosurgical outcome in children prenatally diagnosed with myelomeningocele and development of a protocol for fetal surgery to prevent hydrocephalus
Authors
Helder Zambelli
Edmur Carelli
Donizeti Honorato
Sérgio Marba
Giselle Coelho
Aline Carnevalle
Alexandre Iscaife
Elton da Silva
Ricardo Barini
Lourenço Sbragia
Publication date
01-04-2007
Publisher
Springer-Verlag
Published in
Child's Nervous System / Issue 4/2007
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-006-0261-x

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