CC BY-NC-ND 4.0 · Arquivos Brasileiros de Neurocirurgia: Brazilian Neurosurgery 2024; 43(01): e7-e16
DOI: 10.1055/s-0042-1749349
Original Article

Micro-Surgical Treatment of Fetal Myelomeningocele

Tratamento microcirúrgico da mielomeningocele fetal
1   Instituto Estadual do Cérebro Paulo Niemeyer, Rio de Janeiro, RJ, Brazil
,
1   Instituto Estadual do Cérebro Paulo Niemeyer, Rio de Janeiro, RJ, Brazil
,
1   Instituto Estadual do Cérebro Paulo Niemeyer, Rio de Janeiro, RJ, Brazil
,
1   Instituto Estadual do Cérebro Paulo Niemeyer, Rio de Janeiro, RJ, Brazil
,
2   Maternity School, Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, RJ, Brazil
,
1   Instituto Estadual do Cérebro Paulo Niemeyer, Rio de Janeiro, RJ, Brazil
› Author Affiliations

Abstract

Introduction Myelomeningocele (MMC) is the most common malformation of the central nervous system compatible with life. We will report the results obtained with the prenatal closure of MMC at the Instituto Estadual do Cérebro Paulo Niemeyer (IECPN).

Objectives Clinical outcome of fetuses undergoing intrauterine MMC repair by the Peralta mini-hysterotomy. Monitor the reduction of Arnold-Chiari II secondary to MMC, reduction of hydrocephalus and also motor development in these children.

Methods Descriptive study of 26 cases with intrauterine MMC repair by mini-hysterotomy, or Peralta technique, performed at the IECPN from December 2017 to February 2020.

Results Between December 2017 and February 2020, 26 pregnant women with children with MMC were operated on using Peralta technique. Fetuses were born at an average gestational age of 34.2 weeks and 8% were born before 30 weeks of gestation. The average birth weight was 2096g. It was possible to observe a significant reduction in the occurrence of Arnold-Chiari II in these patients, as well as an evident improvement in motor function in the neurological examination of these babies at the end of the first month of life, where 20 of 23 babies had active movement in the lower limbs.

Discussion This study demonstrates the correction of fetal MMC through a mini-hysterotomy of 2.5 to 3.5 cm, developed in order to reduce maternal and fetal mortality. This mini-hysterotomy technique is not a minimally invasive procedure, as it is based on open surgery for the treatment of fetal MMC, as recommended by the Management of Myelomeningocele Study (MOMS).

Resumo

Introdução Mielomeningocele (MMC) é a malformação mais comum do sistema nervoso central compatível com a vida. Nós relataremos os resultados obtidos com o fechamento pré-natal da MMC no Instituto Estadual do Cérebro Paulo Niemeyer (IECPN).

Objetivos Desfecho clínico dos fetos submetidos a correção intrauterina de MMC por mini-histerotomia de Peralta. Monitorar a redução de Arnold-Chiari II secundária à MMC, redução da hidrocefalia e também o desenvolvimento motor nessas crianças.

Métodos Estudo descritivo de 26 casos com correção intrauterina de MMC por mini-histerotomia ou técnica de Peralta realizadas no IECPN no período entre dezembro de 2017 a fevereiro de 2020.

Resultados Entre dezembro de 2017 a fevereiro de 2020, 26 gestantes com filhos portadores de MMC, foram operadas utilizando-se a técnica de Peralta. Os fetos nasceram com uma idade gestacional média de 34,2 semanas e 8% nasceram antes das 30 semanas de gestação. O peso médio ao nascer foi de 2096 gramas. Foi possível observarmos uma significativa redução na ocorrência de Arnold-Chiari II nestes pacientes, bem como uma evidente melhora da função motora no exame neurológico destes bebês ao final do primeiro mês de vida, aonde 20 de 23 bebês apresentavam movimentação ativa nos membros inferiores.

Discussão Este estudo demonstra a correção da MMC fetal através de uma mini-histerotomia de 2,5 à 3,5cm, desenvolvida com o intuito de reduzir a mortalidade materna e fetal. Esta técnica de mini-histerotomia não é um procedimento minimamente invasivo, pois é baseada na cirurgia aberta para o tratamento da MMC fetal, como preconiza o estudo Management of Myelomeningocele Study (MOMS).



Publication History

Received: 10 June 2020

Accepted: 06 April 2022

Article published online:
10 October 2023

© 2023. Sociedade Brasileira de Neurocirurgia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commecial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

Thieme Revinter Publicações Ltda.
Rua do Matoso 170, Rio de Janeiro, RJ, CEP 20270-135, Brazil

 
  • References

  • 1 Moldenhauer JS, Adzick NS. Fetal surgery for myelomeningocele: After the Management of Myelomeningocele Study (MOMS). Semin Fetal Neonatal Med 2017; 22 (06) 360-366 DOI: 10.1016/j.siny.2017.08.004. [Internet]
  • 2 Danzer E, Adzick NS. Fetal surgery for myelomeningocele: patient selection, perioperative management and outcomes. Fetal Diagn Ther 2011; 30 (03) 163-173
  • 3 Adzick NS, Thom EA, Spong CY. et al; MOMS Investigators. A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med 2011; 364 (11) 993-1004
  • 4 Hilton SA, Hodges MM, Dewberry LC. et al. MOMS Plus: Single-Institution Review of Outcomes for Extended BMI Criteria for Open Fetal Repair of Myelomeningocele. Fetal Diagn Ther 2019; 46 (06) 411-414 https://www.karger.com/Article/FullText/499484 [Internet]
  • 5 Blumenfeld YJ, Belfort MA. Updates in fetal spina bifida repair. Curr Opin Obstet Gynecol 2018; 30 (02) 123-129
  • 6 Gardner WJ. Hydrodynamic mechanism of syringomyelia: its relationship to myelocele. J Neurol Neurosurg Psychiatry 1965; 28: 247-259
  • 7 Elbabaa SK, Gildehaus AM, Pierson MJ, Albers JA, Vlastos EJ. First 60 fetal in-utero myelomeningocele repairs at Saint Louis Fetal Care Institute in the post-MOMS trial era: hydrocephalus treatment outcomes (endoscopic third ventriculostomy versus ventriculo-peritoneal shunt). Childs Nerv Syst 2017; 33 (07) 1157-1168
  • 8 Yamashiro KJ, Galganski LA, Hirose S. Fetal myelomeningocele repair. Semin Pediatr Surg 2019; 28 (04) 150823 DOI: 10.1053/j.sempedsurg.2019.07.006. [Internet]
  • 9 Gupta N. Surgical techniques for open fetal repair of myelomeningocele. Childs Nerv Syst 2017; 33 (07) 1143-1148 http://link.springer.com/10.1007/s00381-017-3439-5 [Internet]
  • 10 Kabagambe SK, Jensen GW, Chen YJ, Vanover MA, Farmer DL. Fetal Surgery for Myelomeningocele: A Systematic Review and Meta-Analysis of Outcomes in Fetoscopic versus Open Repair. Fetal Diagn Ther 2018; 43 (03) 161-174 https://www.karger.com/Article/FullText/479505 [Internet]
  • 11 Kahr MK, Winder FM, Vonzun L. et al. Open Intrauterine Fetal Myelomeningocele Repair: Changes in the Surgical Procedure and Perinatal Complications during the First 8 Years of Experience at a Single Center. Fetal Diagn Ther. 2020; 47 (06) 485-490 . [Epub]
  • 12 Danzer E, Johnson MP. Fetal surgery for neural tube defects. Semin Fetal Neonatal Med 2014; 19 (01) 2-8
  • 13 Pedreira DAL, Zanon N, Nishikuni K. et al. Endoscopic surgery for the antenatal treatment of myelomeningocele: the CECAM trial. Am J Obstet Gynecol 2016; 214 (01) 111.e1-111.e11 DOI: 10.1016/j.ajog.2015.09.065. [Internet]
  • 14 Horst M, Mazzone L, Schraner T. et al. Prenatal myelomeningocele repair: Do bladders better?. Neurourol Urodyn 2017; 36 (06) 1651-1658
  • 15 AlRefai A, Drake J, Kulkarni AV. et al. Fetal myelomeningocele surgery: Only treating the tip of the iceberg. Prenat Diagn 2019; 39 (01) 10-15
  • 16 Moron AF, Barbosa MM, Milani H. et al. Perinatal outcomes after open fetal surgery for myelomeningocele repair: a retrospective cohort study. BJOG 2018; 125 (10) 1280-1286
  • 17 Tulipan N, Sutton LN, Bruner JP, Cohen BM, Johnson M, Adzick NS. The effect of intrauterine myelomeningocele repair on the incidence of shunt-dependent hydrocephalus. Pediatr Neurosurg 2003; 38 (01) 27-33
  • 18 Mazzone LMM. Fetoscopic repair of spina bifida: safer and better?. Ultrasound Obs Gynecol 2016;48(06):
  • 19 Sival DA, Begeer JH, Staal-Schreinemachers AL, Vos-Niël JM, Beekhuis Jr JR, Prechtl HF. Perinatal motor behaviour and neurological outcome in spina bifida aperta. Early Hum Dev 1997; 50 (01) 27-37
  • 20 Botelho RD, Imada V, Rodrigues da Costa KJ. et al. Fetal Myelomeningocele Repair through a Mini-Hysterotomy. Fetal Diagn Ther 2017; 42 (01) 28-34
  • 21 Peranteau WH, Adzick NS. Prenatal surgery for myelomeningocele. Curr Opin Obstet Gynecol 2016; 28 (02) 111-118
  • 22 Cavalheiro S, da Costa MDS, Moron AF, Leonard J. Comparison of Prenatal and Postnatal Management of Patients with Myelomeningocele. Neurosurg Clin N Am 2017; 28 (03) 439-448
  • 23 Coleman BG, Langer JEHS, Horii SC. The diagnostic features of spina bifida: the role of ultrasound. Fetal Diagn Ther 2015; 37 (03) 179-196
  • 24 McComb JG. A practical clinical classification of spinal neural tube defects. Childs Nerv Syst 2015; 31 (10) 1641-1657
  • 25 Hashiguchi K, Morioka T, Murakami N. et al. Clinical Significance of Prenatal and Postnatal Heavily T2-Weighted Magnetic Resonance Images in Patients with Myelomeningocele. Pediatr Neurosurg 2015; 50 (06) 310-320
  • 26 Melvin EC, George TMWG, Worley G. et al; NTD Collaborative Group. Genetic studies in neural tube defects. Pediatr Neurosurg 2000; 32 (01) 1-9
  • 27 Patel TR, Bannister CMTJ, Thorne J. A study of prenatal ultrasound and postnatal magnetic imaging in the diagnosis of central nervous system abnormalities. Eur J Pediatr Surg 2003; 13 (05, Suppl 1): S18-S22
  • 28 Bowman RM, McLone DG, Grant JA, Tomita T, Ito JA. Spina bifida outcome: a 25-year prospective. Pediatr Neurosurg 2001; 34 (03) 114-120
  • 29 Farmer DL, Thom EA, Brock III JW. et al; Management of Myelomeningocele Study Investigators. The Management of Myelomeningocele Study: full cohort 30-month pediatric outcomes. Am J Obstet Gynecol 2018; 218 (02) 256.e1-256.e13 DOI: 10.1016/j.ajog.2017.12.001. [Internet]
  • 30 Moldenhauer JS, Soni S, Rintoul NE. et al. Fetal myelomeningocele repair: the post-MOMS experience at the Children's Hospital of Philadelphia. Fetal Diagn Ther 2015; 37 (03) 235-240
  • 31 Adzick NS, Sutton LN, Crombleholme TMFA, Flake AW. Successful fetal surgery for spina bifida. Lancet 1998; 352 (9141): 1675-1676
  • 32 Johnson MP, Sutton LN, Rintoul N. et al. Fetal myelomeningocele repair: short-term clinical outcomes. Am J Obstet Gynecol 2003; 189 (02) 482-487
  • 33 Bruner JP, Tulipan N, Paschall RL. et al. Fetal surgery for myelomeningocele and the incidence of shunt-dependent hydrocephalus. JAMA 1999; 282: 1819-1825
  • 34 Korenromp MJ, van Gool JD, Bruinese HWKR, Kriek R. Early fetal leg movements in myelomeningocele. Lancet 1986; 1 (8486): 917-918
  • 35 Licci M, Guzman R, Soleman J. Maternal and obstetric complications in fetal surgery for prenatal myelomeningocele repair: a systematic review. Neurosurg Focus 2019; 47 (04) E11
  • 36 Braga AdeF, Rousselet MS, Zambelli H, Sbragia L, Barini R. Anestesia para correção intra-útero de mielomeningocele: relato de caso. Rev Bras Anestesiol 2005; 55 (03) 329-335
  • 37 Almodin CG, Moron AF, Cavaliero S, Yamashita A, Hisaba W, Piassi J. The Almodin-Moron trocar for uterine entry during fetal surgery. Fetal Diagn Ther 2006; 21 (05) 414-417
  • 38 Lunet N, Rodrigues T, Correia S, Barros H. Adequacy of prenatal care as a major determinant of folic acid, iron, and vitamin intake during pregnancy. Cad Saude Publica 2008; 24 (05) 1151-1157
  • 39 World Health Organization. Department of Making Pregnancy Safer. Integrated Management of Pregnancy and Childbirth. Pregnancy, childbirth, postpartum and newborn care: a guide for essential practice. Geneva: WHO; 2006
  • 40 Lorena Barbosa, Davianne de Queiroz Ribeiro, Flávio Cunha de Faria, Luciana Neri Nobre, Angelina do Carmo Lessa. Fatores associados ao uso de suplemento de ácido fólico durante a gestação. Rev. Bras. Ginecol. Obstet. vol.33 No.9 Rio de Janeiro Sept. 2011
  • 41 McLone DG. Results of treatment of children born with a myelomeningocele. Clin Neurosurg 1983; 30: 407-412
  • 42 McLone DG. Continuing concepts in the management of spina bifida. Pediatr Neurosurg 1992; 18 (5-6): 254-256
  • 43 Mark S. Greenberg. Manual de neurocirurgia. Oitava edicao. Pag 240.
  • 44 Stevenson KL. Chiari Type II malformation: past, present, and future. Neurosurg Focus 2004; 16 (02) E5