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Published in: Child's Nervous System 3/2012

01-03-2012 | Original Paper

Intrauterine myelomeningocele repair Postnatal results and follow-up at 3.5 years of age — initial experience from a single reference service in Brazil

Authors: Wagner Jou Hisaba, Sérgio Cavalheiro, Carlos Gilberto Almodim, Carolina Peixoto Borges, Tereza Cristina Carbonari de Faria, Edward Araujo Júnior, Luciano Marcondes Machado Nardozza, Antonio Fernandes Moron

Published in: Child's Nervous System | Issue 3/2012

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Abstract

Purpose

Present the outcomes of six cases submitted to intrauterine myelomeningocele (MMC) repair.

Methods

Descriptive observational study of six children submitted to antenatal surgical repair of MMC between 26 and 27 weeks gestation. All deliveries were through cesarean section. The following neonatal variables were assessed: gestational age at delivery, birth weight, Apgar scores, need for intubation, duration of hospital stay and need for postnatal shunt procedures. After 3.5 years, the children were evaluated using the Columbia Mental Maturity Scale or Denver II tests and the Hoffer Ambulation Scale.

Results

All deliveries were preterm at a mean gestational age of 32 + 4 weeks and mean birth weight was 1,942 g. Two infants had Apgar scores <7 at 1 min and 1 at 5 min. Ventricular–peritoneal shunts were placed in two cases. All six children are alive: five have normal cognitive development and one has a neuropsychomotor developmental delay. Two children had normal leg movements, a sacral functional level and are community ambulators. Three children had upper lumbar anatomical level lesions and one had a lower thoracic level lesion at the time of fetal surgery. One child, with an L1–L2 anatomical level lesion, in noambulatory and fully dependent on a wheelchair for mobility.

Conclusion

Antenatal surgical repair of MMC reduced the need for postnatal shunt placements. Despite preterm delivery, the cognitive development of most children at 3.5 years was normal. Antenatal surgery seemed to improve lower limb motor function in these cases.
Literature
1.
go back to reference Centers for Disease Control and Preventions (2004) Spina bifida and anencephaly before and after folic acid mandate – United States, 1995–1996 and 1999–2000. MMWR Morb Mortal Wkly Rep 53:362–365 Centers for Disease Control and Preventions (2004) Spina bifida and anencephaly before and after folic acid mandate – United States, 1995–1996 and 1999–2000. MMWR Morb Mortal Wkly Rep 53:362–365
2.
go back to reference Centers for Disease Control and Preventions (CDC) (1995) Economic costs of birth defects and cerebral palsy – United States, 1992. MMWR Morb Mortal Wkly Rep 44:694–699 Centers for Disease Control and Preventions (CDC) (1995) Economic costs of birth defects and cerebral palsy – United States, 1992. MMWR Morb Mortal Wkly Rep 44:694–699
3.
go back to reference Vintzileos AM, Ananth CV, Smulian JC, Beazoglou T, Knuppel RA (2000) Routine second-trimester ultrasonography in the United States: a cost benefit analysis. Am J Obstet Gynecol 182:655–660PubMedCrossRef Vintzileos AM, Ananth CV, Smulian JC, Beazoglou T, Knuppel RA (2000) Routine second-trimester ultrasonography in the United States: a cost benefit analysis. Am J Obstet Gynecol 182:655–660PubMedCrossRef
4.
go back to reference Tulipan N, Bruner JP (1998) Myelomeningocele repair in utero: a report of three cases. Pediatr Neurosurg 28:177–180PubMedCrossRef Tulipan N, Bruner JP (1998) Myelomeningocele repair in utero: a report of three cases. Pediatr Neurosurg 28:177–180PubMedCrossRef
5.
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
6.
8.
go back to reference Adzick NS, Thom EA, Spong CY, Brock JW 3rd, Burrows PK, Johnson MP, Howell LJ, Farrell JA, Dabrowiak ME, Sutton LN, Gupta N, Tulipan NB, D’Alton ME, Farmer DL, Investigators MOMS (2011) A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med 364:993–1004PubMedCrossRef Adzick NS, Thom EA, Spong CY, Brock JW 3rd, Burrows PK, Johnson MP, Howell LJ, Farrell JA, Dabrowiak ME, Sutton LN, Gupta N, Tulipan NB, D’Alton ME, Farmer DL, Investigators MOMS (2011) A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med 364:993–1004PubMedCrossRef
9.
go back to reference Hoffer MM, Feiwell E, Perry R, Perry J, Bonnett C (1973) Functional ambulation in patients with myelomeningocele. J Bone Jt Surg Am 55:137–418 Hoffer MM, Feiwell E, Perry R, Perry J, Bonnett C (1973) Functional ambulation in patients with myelomeningocele. J Bone Jt Surg Am 55:137–418
10.
go back to reference Johnson MP, Gerdes M, Rintoul N, Pasquariello P, Melchioni J, Sutton LN, Adzick NS (2006) Maternal-fetal surgery for myelomeningocele: neurodevelopment outcomes at 2 years of age. Am J Obstet Gynecol 194:1145–1152PubMedCrossRef Johnson MP, Gerdes M, Rintoul N, Pasquariello P, Melchioni J, Sutton LN, Adzick NS (2006) Maternal-fetal surgery for myelomeningocele: neurodevelopment outcomes at 2 years of age. Am J Obstet Gynecol 194:1145–1152PubMedCrossRef
11.
go back to reference Walsh DS, Adzick NS (2003) Foetal surgery for spina bifida. Semin Neonat 8:197–205CrossRef Walsh DS, Adzick NS (2003) Foetal surgery for spina bifida. Semin Neonat 8:197–205CrossRef
12.
go back to reference Tulipan N, Hernanz-Shulman M, Bruner JP (1998) Reduced hindbrain herniation after intrauterine myelomeningocele repair: a report of four cases. Pediatr Neurosurg 29:274–278PubMedCrossRef Tulipan N, Hernanz-Shulman M, Bruner JP (1998) Reduced hindbrain herniation after intrauterine myelomeningocele repair: a report of four cases. Pediatr Neurosurg 29:274–278PubMedCrossRef
13.
go back to reference Tulipan N, Hermanz-Schulman M, Lowe LH, Bruner JP (1999) Intrauterine myelomeingocele repair reverses preexisting hindbrain herniation. Pediatr Neurosurg 31:137–142PubMedCrossRef Tulipan N, Hermanz-Schulman M, Lowe LH, Bruner JP (1999) Intrauterine myelomeingocele repair reverses preexisting hindbrain herniation. Pediatr Neurosurg 31:137–142PubMedCrossRef
14.
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
15.
go back to reference Bruner JP, Tulipan N, Paschall RL, Boehm FH, Walsh WF, Silva SR, Hernanz-Schulman M, Lowe LH, Reed GW (1999) Fetal surgery for myelomeingocele and the incidence of shunt-dependent hydrocephalus. JAMA 282:1819–1825PubMedCrossRef Bruner JP, Tulipan N, Paschall RL, Boehm FH, Walsh WF, Silva SR, Hernanz-Schulman M, Lowe LH, Reed GW (1999) Fetal surgery for myelomeingocele and the incidence of shunt-dependent hydrocephalus. JAMA 282:1819–1825PubMedCrossRef
16.
go back to reference Tulipan N, Sutton LN, Bruner JP, Cohen BM, Johnson M, Adzick NS (2003) The effect of intrauterine myelomeningocele repair on the incidence of shunt-dependent hydrocephalus. Pediatr Neurosurg 38:27–33PubMedCrossRef Tulipan N, Sutton LN, Bruner JP, Cohen BM, Johnson M, Adzick NS (2003) The effect of intrauterine myelomeningocele repair on the incidence of shunt-dependent hydrocephalus. Pediatr Neurosurg 38:27–33PubMedCrossRef
17.
go back to reference Tubbs RS, Chambers MR, Smyth MD, Bartolucci AA, Bruner JP, Tulipan N, Oakes WJ (2003) Late gestational intrauterine myelomeningocele repair does not improve lower extremity function. Pediatr Neurosurg 38:128–132PubMedCrossRef Tubbs RS, Chambers MR, Smyth MD, Bartolucci AA, Bruner JP, Tulipan N, Oakes WJ (2003) Late gestational intrauterine myelomeningocele repair does not improve lower extremity function. Pediatr Neurosurg 38:128–132PubMedCrossRef
18.
go back to reference Johnson MP, Sutton LN, Rintoul N, Crombleholme TM, Flake AW, Howell LJ, Hedrick HL, Wilson RD, Adzick NS (2003) Fetal myelomeningocele repair: short-term clinical outcomes. Am J Obstet Gynecol 189:482–487PubMedCrossRef Johnson MP, Sutton LN, Rintoul N, Crombleholme TM, Flake AW, Howell LJ, Hedrick HL, Wilson RD, Adzick NS (2003) Fetal myelomeningocele repair: short-term clinical outcomes. Am J Obstet Gynecol 189:482–487PubMedCrossRef
19.
go back to reference Danzer E, Gerdes M, Bebbington MW, Sutton LN, Melchionni J, Adzick NS, Wilson RD, Johnson MP (2009) Lower extremity neuromotor function and short-term ambulatory potential following in utero myelomeningocele surgery. Fetal Diagn Ther 25:47–53PubMedCrossRef Danzer E, Gerdes M, Bebbington MW, Sutton LN, Melchionni J, Adzick NS, Wilson RD, Johnson MP (2009) Lower extremity neuromotor function and short-term ambulatory potential following in utero myelomeningocele surgery. Fetal Diagn Ther 25:47–53PubMedCrossRef
20.
go back to reference Johnson MP, Gerdes M, Rintoul N, Pasquariello P, Melchionni J, Sutton LN, Adzick NS (2006) Maternal-fetal surgery for myelomeningocle: neurodevelopment outcomes at 2 years of age. Am J Obstet Gynecol 194:1145–1152PubMedCrossRef Johnson MP, Gerdes M, Rintoul N, Pasquariello P, Melchionni J, Sutton LN, Adzick NS (2006) Maternal-fetal surgery for myelomeningocle: neurodevelopment outcomes at 2 years of age. Am J Obstet Gynecol 194:1145–1152PubMedCrossRef
21.
go back to reference Holzbeierlein J, Pope JC IV, Adams MC, Bruner J, Tulipan N, Brock JW 3rd (2000) The urodynamic profile of myelodysplasia in childhood with spinal closure during gestation. J Urol 164:1336–1339PubMedCrossRef Holzbeierlein J, Pope JC IV, Adams MC, Bruner J, Tulipan N, Brock JW 3rd (2000) The urodynamic profile of myelodysplasia in childhood with spinal closure during gestation. J Urol 164:1336–1339PubMedCrossRef
Metadata
Title
Intrauterine myelomeningocele repair Postnatal results and follow-up at 3.5 years of age — initial experience from a single reference service in Brazil
Authors
Wagner Jou Hisaba
Sérgio Cavalheiro
Carlos Gilberto Almodim
Carolina Peixoto Borges
Tereza Cristina Carbonari de Faria
Edward Araujo Júnior
Luciano Marcondes Machado Nardozza
Antonio Fernandes Moron
Publication date
01-03-2012
Publisher
Springer-Verlag
Published in
Child's Nervous System / Issue 3/2012
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-011-1662-z

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