What do we do with myelomeningocele? Multicentric survey
DOI:
https://doi.org/10.59156/revista.v0i0.687Keywords:
Chiari, Folic acid, Spina bifida, SurvivalAbstract
Background: myelomeningocele (MM) is a well-known pathology on which there are few studies at the national level.
Objectives: to survey the neurosurgical management of the treatment of myelomeningocele and associated entities.
Methods: retrospective and observational study of the medical records of 475 patients operated on in the last 5 years. The questionnaire was sent to 18 members of the Argentine Society of Pediatric Neurosurgery. Frequents complications, appearance and treatment of hydrocephalus, behavior in the face of Chiari malformation, syringomyelia and transition in adulthood were recorded. The calculation of the statistical median and percentages was applied.
Results: we received 15 questionnaires: 10 complete, 5 incomplete. The incidence of MM since 2003 decreased by 20-50% for 42.9% and more than 80% for 21.4% of respondents. Of 413 postpartum surgeries, 72.8% were performed between 12-48 hours. 62 by fetal surgery, around week 28.5. The main complication was cerebral spinal fluid leak: 42.7% A total of 314 ventricle peritoneal shunts (VPS) were implanted, 81.7% from birth to 30 days. Symptomatic Chiari treated by occipitocervical decompression (OD): 73 cases, with two peaks, in the first month of life (27.6%) and 3-6 months (24.1%). 57.1% of known patients survived 20 years and more.
Conclusion: flour fortification has decreased the incidence of MM. Closure during the 24 hours postpartum is the predominant behavior. Fetal surgery is not performed on a massive scale. Orthopedic surgery induces OD even in asymptomatic patients. Transitional medicine has been poorly implemented.
Downloads
References
Garcia RM, Ghotme KA, Arynchyna-Smith A, Priyanka M, Koning M, Boop F, et al. Global neurosurgery: progress and resolutions at the 75th World Health Assembly. Neurosurgery, 2023; 93(3): 496-501. doi:10.1227/neu.0000000000002472. DOI: https://doi.org/10.1227/neu.0000000000002472
Carrea R. Microcirugía del mielomeningocele lumbosacro. Act Neurol Latinoamer, 1971; 17: 238-41.
Tulipan N, Sutton LN, Bruner JP, Cohen BM, Adzick NS. The effect of intrauterine myelomeningocele repair on the incidence of shunt-dependent hydrocephalus. Pediatr Neurosurg, 2003; 38(1): 27-33. doi:10.1159/000067560. DOI: https://doi.org/10.1159/000067560
Adzick SN, Thom EA, Spong CY, Brock JW 3rd, Burrows PK, Johnson M, et al. A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med, 2011; 364(11): 993-1004. doi:10.1056/NEJMoa1014379. DOI: https://doi.org/10.1056/NEJMoa1014379
Palma F, et al. Cirugía fetal de mielomeningocele: evolución obstétrica y resultados perinatales a corto plazo de una cohorte de 21 casos. Rev Argent Neuroc, 2018; 32(2): 58-70. Disponible en: https://aanc.org.ar/ranc/items/show/1192.
Zuccaro G. Why fetal neurosurgery? Childs Nerv Syst, 2017; 33: 1081-2. DOI: https://doi.org/10.1007/s00381-017-3444-8
Silverkasten M. La construcción imaginaria de la discapacidad. 2da ed. Ciudad Autónoma de Buenos Aires: Topía Editorial; 2014.
Sosa F, Rodriguez F, Diaz A, Argañaraz R, Lambre J, Liñares JM. Neurocirugía transicional: ¿una nueva especialidad? Rev Argent Neurocir, 2020; 34. doi:10.59156/revista.v34i04.56. DOI: https://doi.org/10.59156/revista.v34i04.56
Honorable Congreso de la Nación Argentina. Ley 25630/2002. Agosto 2002. Disponible en: https://www.argentina.gob.ar.
Kim I, Hopson B, Aban I, Rizk E, Dias M, Bowman R, et al. Treated hydrocephalus in individuals with myelomeningocele in the National Spina Bifida Patient Registry. J Neurosurg Pediatr, 2018; 22(6): 646-51. DOI: https://doi.org/10.3171/2018.5.PEDS18161
Sharrad W. The segmental innervation of lower limb muscles in man. Ann R Coll Surg Engl, 1964; 27: 106-22.
Red Nacional de Anomalías Congénitas – RENAC. Ministerio de Salud, Argentina. Disponible en: www.argentina.gob.ar.
McCarthy M, Sheinberg D, Luther E, McCrea H. Myelomeningocele-associated hydrocephalus: nationwide analysis and systematic review. Neurosurg Focus, 2019; 47(4): E5. DOI: https://doi.org/10.3171/2019.7.FOCUS19469
Stein C, Schut L. Hydrocephalus in myelomeningocele. Childs Brain, 1979; 5(4): 413-9. doi:10.1159/000119836. DOI: https://doi.org/10.1159/000119836
Oakeshott P, Hunt G, Poulton A, Reid F. Expectation of life and unexpected death in open spina bifida: a 40-year complete, non-selective, longitudinal cohort study. Dev Med Child Neurol, 2010; 52(8): 749-53. doi:10.1111/j.1469-8749.2009.03543.x. DOI: https://doi.org/10.1111/j.1469-8749.2009.03543.x
Balthasar AJR, Kort H, Cornips EMJ, Beuls EAM, Weber JW, Vles JSH. Analysis of the success and failure of endoscopic third ventriculostomy in infants less than 1 year of age. Childs Nerv Syst, 2007; 23(2): 151-5. DOI: https://doi.org/10.1007/s00381-006-0219-z
Grisotto L, Colombo G, Tello Brogiolo N, Bustamante J, D’Agustini M. Revisión endoscópica ante el fallo de la tercer ventriculostomía en pacientes pediátricos. Rev Argent Neurocir, 2021. doi:10.59156/revista.v0i0.284. DOI: https://doi.org/10.59156/revista.v0i0.284
Mai TC, et al. Changes in spina bifida lesion level after folic acid fortification in the US. J Pediatr, 2022; 249: 59-66.e1. doi:10.1016/j.jpeds.2022.06.023. DOI: https://doi.org/10.1016/j.jpeds.2022.06.023
Cáceres A, Jimenez-Chaverri AL, Alpizar-Quiros PA, Wong-McClure R. Pre and postnatal care characteristics and management features of children born with myelomeningocele in the post-folate fortification era of staple foods in Costa Rica (2004–2022). Childs Nerv Syst, 2023; 39(7): 1755-64. doi:10.1007/s00381-023-05951-y. DOI: https://doi.org/10.1007/s00381-023-05951-y
Vencio RCC, Lino-Fliho A, Bonfim RCF, Pereira NM, Franco CL, Ribeiro PR, et al. Surgical management of Chiari II malformation: a systematic review of literature. Childs Nerv Syst, 2024; 40(7): 2093-100. doi:10.1007/s00381-024-06368-x. DOI: https://doi.org/10.1007/s00381-024-06368-x
Messing-Jünger M, Röhirig. Primary and secondary management of the Chiari II malformation in children with myelomeningocele. Childs Nerv Syst, 2013; 29(9): 1553-62. doi:10.1007/s00381-013-2134-4. DOI: https://doi.org/10.1007/s00381-013-2134-4
Bizz JWJ, Machado A. Mielomeningocele: conceitos básicos e avanços recentes. J Bras Neurosurg, 2012; 23(2): 138-51. doi:10.22290/jbcn.v8i3.32. DOI: https://doi.org/10.22290/jbnc.v23i2.1161
Lew SM, Kothbauer K. Tethered cord syndrome: an updated review. Pediatr Neurosurg, 2007; 43(3): 236-48. doi:10.1159/000098836. DOI: https://doi.org/10.1159/000098836
