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Mielomeningocele Diagnóstico
- 1. Mielomeningocele Mariana Nunes Pinheiro Rialto Especializanda em Medicina Materno-Fetal
- 2. Definição • É uma malformação que resulta da falha de fechamento do tubo neural. • Protusão de meninges e raízes nervosas por meio de arcos vertebrais abertos. Mielomeningocele Bianchi, DW; Crombleholme, TM; D´Alton, ME; Malone, FD. Fetology, Diagnosis and Management of the Fetal Patient. 2 ed. Mc Graw Hill, 2010. p. 151-165
- 3. Embriologia Mielomeningocele Bianchi, DW; Crombleholme, TM; D´Alton, ME; Malone, FD. Fetology, Diagnosis and Management of the Fetal Patient. 2 ed. Mc Graw Hill, 2010. p. 151-165
- 4. Incidência • Varia de acordo com etnia e localização geográfica. • Maior: Inglaterra, Índia, Egito, Irlanda e Paquistão • Menor: Finlândia, Japão, Israel • EUA – 0,5-1/1000 nascidos vivos (Shaer et al., 2007) Mielomeningocele Bianchi, DW; Crombleholme, TM; D´Alton, ME; Malone, FD. Fetology, Diagnosis and Management of the Fetal Patient. 2 ed. Mc Graw Hill, 2010. p. 151-165
- 5. Incidência • Fatores associados • Intervalo curto entre as gestações • Uso de medicações • Ácido valpróico, carbamazepina, diuréticos, anti- histamínicos, sulfonamidas Mielomeningocele Bianchi, DW; Crombleholme, TM; D´Alton, ME; Malone, FD. Fetology, Diagnosis and Management of the Fetal Patient. 2 ed. Mc Graw Hill, 2010. p. 151-165
- 6. Incidência • Fatores associados • Obesidade na gestação, DM, doenças cardíacas e pulmonares • Baixo nível socioeconômico • História familiar e pessoal • Gemelaridade • Mutações na enzimas MTHFR Mielomeningocele Bianchi, DW; Crombleholme, TM; D´Alton, ME; Malone, FD. Fetology, Diagnosis and Management of the Fetal Patient. 2 ed. Mc Graw Hill, 2010. p. 151-165
- 7. Etiologia • Multifatorial • O uso de ácido fólico pré-conceptual na dose de 0,4mg/dia diminui o risco em 70-80% Mielomeningocele Bianchi, DW; Crombleholme, TM; D´Alton, ME; Malone, FD. Fetology, Diagnosis and Management of the Fetal Patient. 2 ed. Mc Graw Hill, 2010. p. 151-165
- 8. Diagnóstico • Avaliação ultrassonográfica • Achados diretos e indiretos • Alfafetoproteína no sangue materno • Acetilcolinesterease no LA Mielomeningocele Bianchi, DW; Crombleholme, TM; D´Alton, ME; Malone, FD. Fetology, Diagnosis and Management of the Fetal Patient. 2 ed. Mc Graw Hill, 2010. p. 151-165
- 9. Achados Ultrassonográficos Mielomeningocele R Chaoui; KH Nicolaides. From nuchal translucency to intracranial translucency: towards the early detection of spina bífida. Ultrasound Obstet Gynecol 2010; 35: 133-138
- 10. Achados Ultrassonográficos Mielomeningocele • Intracranianos • Sinal do limão • Sinal da banana • Ventriculomegalia lateral • Diminuição DBP • Chiari II • Hidrocefalia Bianchi, DW; Crombleholme, TM; D´Alton, ME; Malone, FD. Fetology, Diagnosis and Management of the Fetal Patient. 2 ed. Mc Graw Hill, 2010. p. 151-165
- 11. Achados Ultrassonográficos Mielomeningocele • Intracranianos • Sinal do limão • Sinal da banana • Ventriculomegalia lateral • Diminuição DBP • Chiari II • Hidrocefalia Bianchi, DW; Crombleholme, TM; D´Alton, ME; Malone, FD. Fetology, Diagnosis and Management of the Fetal Patient. 2 ed. Mc Graw Hill, 2010. p. 151-165
- 12. Achados Ultrassonográficos • Espinhais • Espinha bífida • Escoliose/cifose Mielomeningocele Importante localizar o nível da lesão Bianchi, DW; Crombleholme, TM; D´Alton, ME; Malone, FD. Fetology, Diagnosis and Management of the Fetal Patient. 2 ed. Mc Graw Hill, 2010. p. 151-165
- 13. Achados Ultrassonográficos • Espinhais Mielomeningocele Bianchi, DW; Crombleholme, TM; D´Alton, ME; Malone, FD. Fetology, Diagnosis and Management of the Fetal Patient. 2 ed. Mc Graw Hill, 2010. p. 151-165
- 14. Achados Ultrassonográficos • Outros achados • Cisto em região dorsal • Raízes nervosas • Movimentação de membros Mielomeningocele Bianchi, DW; Crombleholme, TM; D´Alton, ME; Malone, FD. Fetology, Diagnosis and Management of the Fetal Patient. 2 ed. Mc Graw Hill, 2010. p. 151-165
- 15. Diagnóstico Diferencial Mielomeningocele Sinal do limão Craniossinostose Encefalocele Displasia tanatofórica Higroma cístico Massa sacral Teratoma sacrococcígeo Abertura dos arcos vertebrais Hemivértebra isolada Ossos sacrais não visualizados Sd. Regressão caudal Sirenomelia Bianchi, DW; Crombleholme, TM; D´Alton, ME; Malone, FD. Fetology, Diagnosis and Management of the Fetal Patient. 2 ed. Mc Graw Hill, 2010. p. 151-165
- 16. História Natural Antenatal Mielomeningocele • Mortalidade DTN – 8 semanas (Main and Mennuti, 1986) • ½ aborto espontâneo • ¼ OFIU • ¼ nascidos vivos • Herniação cerebelar • Hidrocefalia • Diminuição ou ausência de movimentação MMII Bianchi, DW; Crombleholme, TM; D´Alton, ME; Malone, FD. Fetology, Diagnosis and Management of the Fetal Patient. 2 ed. Mc Graw Hill, 2010. p. 151-165
- 17. História Natural Antenatal Mielomeningocele • Danos às raízes nervosas • Trauma • Líquido amniótico Perda de funções “Two-hit” hypotesis Bianchi, DW; Crombleholme, TM; D´Alton, ME; Malone, FD. Fetology, Diagnosis and Management of the Fetal Patient. 2 ed. Mc Graw Hill, 2010. p. 151-165
- 18. Manejo da Gestação Mielomeningocele • Cariótipo fetal • 2 – 16% associado à cromossomopatias e sd. gênicas • Definir prognóstico, manejo e intervenção. • Discutir prognóstico com a família – equipe multidisciplinar. • Discutir necessidade cirúrgica fetal/neonatal. Bianchi, DW; Crombleholme, TM; D´Alton, ME; Malone, FD. Fetology, Diagnosis and Management of the Fetal Patient. 2 ed. Mc Graw Hill, 2010. p. 151-165
- 19. Via de parto Mielomeningocele • Cesariana • Movimentação de joelhos e tornozelos • Euplóides Bianchi, DW; Crombleholme, TM; D´Alton, ME; Malone, FD. Fetology, Diagnosis and Management of the Fetal Patient. 2 ed. Mc Graw Hill, 2010. p. 151-165 Evitar trauma
- 20. Intervenção fetal Mielomeningocele • Cirurgia fetal para reparo da mielomeningocele. Bianchi, DW; Crombleholme, TM; D´Alton, ME; Malone, FD. Fetology, Diagnosis and Management of the Fetal Patient. 2 ed. Mc Graw Hill, 2010. p. 151-165
- 21. Tratamento do neonato Mielomeningocele • Manipulação o mais estéril possível • Curativo na lesão • Manutenção da temperatura • Avaliação por neurologista ou neurocirurgião • TC/RM crânio • Orientação dos pais – possível hidrocefalia pós-cirúrgica Bianchi, DW; Crombleholme, TM; D´Alton, ME; Malone, FD. Fetology, Diagnosis and Management of the Fetal Patient. 2 ed. Mc Graw Hill, 2010. p. 151-165
- 22. Cirurgia pós-natal Mielomeningocele • Entre 24-72h de vida • Mortalidade próxima de 0% • 80% desenvolve hidrocefalia • Necessidade de shunt Bianchi, DW; Crombleholme, TM; D´Alton, ME; Malone, FD. Fetology, Diagnosis and Management of the Fetal Patient. 2 ed. Mc Graw Hill, 2010. p. 151-165
- 23. Desfecho a longo prazo Mielomeningocele • Paraplegia • Hidrocefalia (Rintoul et al., 2002) • Disfunção pulmonar (Sherman et al., 1997) • Disfunção sexual • Deformidades esqueléticas e espinhais (Iborraetal.,1999) • Incontinências • Comprometimento cognitivo (Tsaietal.,2002) Bianchi, DW; Crombleholme, TM; D´Alton, ME; Malone, FD. Fetology, Diagnosis and Management of the Fetal Patient. 2 ed. Mc Graw Hill, 2010. p. 151-165
- 24. Desfecho a longo prazo Mielomeningocele • Nível funcional da lesão • Permanecer em pé – L3 • Andar – L4-L5 • Função sexual masculina – S2-S4 Bianchi, DW; Crombleholme, TM; D´Alton, ME; Malone, FD. Fetology, Diagnosis and Management of the Fetal Patient. 2 ed. Mc Graw Hill, 2010. p. 151-165 Quanto mais baixa a lesão melhor o prognóstico
- 25. Desfecho a longo prazo Mielomeningocele • Chiari II • Aumento da mortalidade • Dificuldade de deglutição • Apnéia • Estridor • Estrabismo (61%) • Ventriculomegalia – piora cognitiva Bianchi, DW; Crombleholme, TM; D´Alton, ME; Malone, FD. Fetology, Diagnosis and Management of the Fetal Patient. 2 ed. Mc Graw Hill, 2010. p. 151-165
- 26. Desfecho a longo prazo Mielomeningocele • Urológicos • Falência renal (Zawin and Lebowitz, 1992) • Bexiga hiperativa • Incontinência • Cateterização intermitente • Urostomia Bianchi, DW; Crombleholme, TM; D´Alton, ME; Malone, FD. Fetology, Diagnosis and Management of the Fetal Patient. 2 ed. Mc Graw Hill, 2010. p. 151-165
- 27. Desfecho a longo prazo Mielomeningocele • Deformidades musculoesqueléticas • Escoliose • Cifose • Pé-torto • Deslocamento de quadril Bianchi, DW; Crombleholme, TM; D´Alton, ME; Malone, FD. Fetology, Diagnosis and Management of the Fetal Patient. 2 ed. Mc Graw Hill, 2010. p. 151-165
- 28. Desfecho a longo prazo Mielomeningocele Bianchi, DW; Crombleholme, TM; D´Alton, ME; Malone, FD. Fetology, Diagnosis and Management of the Fetal Patient. 2 ed. Mc Graw Hill, 2010. p. 151-165 (Hunt, 1990; Hunt e Poulton ,1995)
- 29. Desfecho a longo prazo Mielomeningocele • Nível sensorial ao nascimento (Hunt, 1990; Hunt e Poulton ,1995) • Acima de T11 • 50% sobrevida até idade adulta • 50% QI normal • Incapazes de andar • Incontinentes • 10% vida independente Bianchi, DW; Crombleholme, TM; D´Alton, ME; Malone, FD. Fetology, Diagnosis and Management of the Fetal Patient. 2 ed. Mc Graw Hill, 2010. p. 151-165
- 30. Desfecho a longo prazo Mielomeningocele Bianchi, DW; Crombleholme, TM; D´Alton, ME; Malone, FD. Fetology, Diagnosis and Management of the Fetal Patient. 2 ed. Mc Graw Hill, 2010. p. 151-165 • Nível sensorial ao nascimento (Hunt, 1990; Hunt e Poulton ,1995) • Entre T11 e L3 • 55% sobrevida até idade adulta • 70% QI normal • 40% capazes de andar • 15% continentes • 45% vida independente
- 31. Desfecho a longo prazo Mielomeningocele Bianchi, DW; Crombleholme, TM; D´Alton, ME; Malone, FD. Fetology, Diagnosis and Management of the Fetal Patient. 2 ed. Mc Graw Hill, 2010. p. 151-165 • Nível sensorial ao nascimento (Hunt, 1990; Hunt e Poulton ,1995) • Abaixo de L3 • 70% sobrevida até idade adulta • 80% QI normal • 90% capazes de andar • 45% continentes • 85% vida independente
- 32. Desfecho a longo prazo Mielomeningocele Bianchi, DW; Crombleholme, TM; D´Alton, ME; Malone, FD. Fetology, Diagnosis and Management of the Fetal Patient. 2 ed. Mc Graw Hill, 2010. p. 151-165
- 33. Genética e Recorrência Mielomeningocele Bianchi, DW; Crombleholme, TM; D´Alton, ME; Malone, FD. Fetology, Diagnosis and Management of the Fetal Patient. 2 ed. Mc Graw Hill, 2010. p. 151-165 • Defeitos de tubo neural • Isolados e multifatoriais (Main and Mennuti, 1986) • Risco de recorrência • 1 caso – 1,5 - 3% • 2 casos – 5,7% (EUA); 12% (UK) • Irmãs de gestantes de fetos com MMC – risco aumentado
- 34. Genética e Recorrência Mielomeningocele Bianchi, DW; Crombleholme, TM; D´Alton, ME; Malone, FD. Fetology, Diagnosis and Management of the Fetal Patient. 2 ed. Mc Graw Hill, 2010. p. 151-165 Mulheres com história de MMC em gestações anteriores devem utilizar 4mg/dia de ácido fólico por 3 meses antes de engravidar
- 35. Mielomeningocele
- 36. Método Mielomeningocele – MOMS Trial • Trial conduzido em 3 centros de cirurgia materno-fetal • Children’s Hospital of Philadelphia, Vanderbilt University, e University of California, San Francisco • Comparar desfechos entre pacientes submetidos à cirurgia intra-útero X após o nascimento. • 2003 - 2010 Adzick NS, Thom EA, Spong CY, Brock III JW, Burrows PK, Johnson MP, Howell LJ, Farrell JA, Dabrowiak ME, Sutton LN, Gupta N, Tulipan NB, D’Alton ME, Farmer DL; the MOMS Investigators: A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med 2011; 364: 993–1004.
- 37. Método Mielomeningocele – MOMS Trial • Critérios de inclusão • Gestação única • Limite superior da mielomeningocele entre T1 e S1 • Herniação de fossa posterior • Idade gestacional entre 19.0 e 25.9 semanas • Cariótipo normal • Residentes EUA • Idade materna de pelo menos 18 anos Adzick NS, Thom EA, Spong CY, Brock III JW, Burrows PK, Johnson MP, Howell LJ, Farrell JA, Dabrowiak ME, Sutton LN, Gupta N, Tulipan NB, D’Alton ME, Farmer DL; the MOMS Investigators: A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med 2011; 364: 993–1004.
- 38. Método Mielomeningocele – MOMS Trial • Critérios maiores de exclusão • Outras malformações não relacionada à mielomeningocele • Cifose acentuada • Risco aumentado de TPP • Descolamento de placenta • IMC >35 • Contra-indicações cirúrgicas • Cicatriz uterina corporal Adzick NS, Thom EA, Spong CY, Brock III JW, Burrows PK, Johnson MP, Howell LJ, Farrell JA, Dabrowiak ME, Sutton LN, Gupta N, Tulipan NB, D’Alton ME, Farmer DL; the MOMS Investigators: A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med 2011; 364: 993–1004.
- 39. Método Mielomeningocele – MOMS Trial Gestantes interessadas entravam em contado Elegíveis? Encaminhadas para um dos 3 centros onde eram randomizadas Cirurgia fetal Cirurgia pós- natal Retorno com 37 semanas para CST e cirurgia na sequência Cirurgia e acompanhamento próximo CST com 37 semanas Adzick NS, Thom EA, Spong CY, Brock III JW, Burrows PK, Johnson MP, Howell LJ, Farrell JA, Dabrowiak ME, Sutton LN, Gupta N, Tulipan NB, D’Alton ME, Farmer DL; the MOMS Investigators: A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med 2011; 364: 993–1004.
- 40. Mielomeningocele – MOMS Trial Adzick NS, Thom EA, Spong CY, Brock III JW, Burrows PK, Johnson MP, Howell LJ, Farrell JA, Dabrowiak ME, Sutton LN, Gupta N, Tulipan NB, D’Alton ME, Farmer DL; the MOMS Investigators: A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med 2011; 364: 993–1004.
- 41. Método Mielomeningocele – MOMS Trial • Acompanhamento • Avaliação com 12 e 30 meses • Exame físico • Neurológico • Avaliação do desenvolvimento • 12 meses • + Rx coluna • + RM crânio e coluna Adzick NS, Thom EA, Spong CY, Brock III JW, Burrows PK, Johnson MP, Howell LJ, Farrell JA, Dabrowiak ME, Sutton LN, Gupta N, Tulipan NB, D’Alton ME, Farmer DL; the MOMS Investigators: A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med 2011; 364: 993–1004.
- 42. Método Mielomeningocele – MOMS Trial • Desfechos primários • 12 meses • Óbito fetal / Óbito neonatal / Necessidade de shunt • 30 meses • Índice de Desenvolvimento Mental de Bailey • Diferença entre os níveis funcional e anatômico. Adzick NS, Thom EA, Spong CY, Brock III JW, Burrows PK, Johnson MP, Howell LJ, Farrell JA, Dabrowiak ME, Sutton LN, Gupta N, Tulipan NB, D’Alton ME, Farmer DL; the MOMS Investigators: A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med 2011; 364: 993–1004.
- 43. Método Mielomeningocele – MOMS Trial • Desfechos secundários • Complicações cirúrgicas e gestacionais • Morbidade e mortalidade neonatal • Chiari II • Tempo até necessidade de shunt • Locomoção • Desenvolvimento psicomotor • Grau de capacidade funcional • Grau de incapacidade Adzick NS, Thom EA, Spong CY, Brock III JW, Burrows PK, Johnson MP, Howell LJ, Farrell JA, Dabrowiak ME, Sutton LN, Gupta N, Tulipan NB, D’Alton ME, Farmer DL; the MOMS Investigators: A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med 2011; 364: 993–1004.
- 44. Resultados Mielomeningocele – MOMS Trial • Pacientes (fev/2003 - dez/2010) • 183 elegíveis • 7 de dezembro 2010 – estudo interrompido • Comprovada superioridade da cirurgia fetal • Resultados baseados em: • Desfechos até 12 meses - 158 (julho/2009) • Desfechos 30 meses – 134 (dezembro/2007) Adzick NS, Thom EA, Spong CY, Brock III JW, Burrows PK, Johnson MP, Howell LJ, Farrell JA, Dabrowiak ME, Sutton LN, Gupta N, Tulipan NB, D’Alton ME, Farmer DL; the MOMS Investigators: A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med 2011; 364: 993–1004.
- 46. Resultados Mielomeningocele – MOMS Trial • Complicações gestacionais e pré-natais • Nenhuma morte materna • Grupo cirurgia pré-natal teve mais complicações • Oligodrâmnio, separação corio-amniotica, descolamento de placenta, RUPREME pré-termo. • 1/3 deiscência ou cicatriz uterina fina. • IG media ao nascer menor. • 2 mortes perinatais em cada grupo • Cirurgia pré-natal: OFIU/prematuridade • Cirurgia pós- natal: sintomas Chiari II Adzick NS, Thom EA, Spong CY, Brock III JW, Burrows PK, Johnson MP, Howell LJ, Farrell JA, Dabrowiak ME, Sutton LN, Gupta N, Tulipan NB, D’Alton ME, Farmer DL; the MOMS Investigators: A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med 2011; 364: 993–1004.
- 49. Resultados Mielomeningocele – MOMS Trial • Desfecho primário - 12 meses • Óbito fetal ou neonatal • Necessidade de shunt Adzick NS, Thom EA, Spong CY, Brock III JW, Burrows PK, Johnson MP, Howell LJ, Farrell JA, Dabrowiak ME, Sutton LN, Gupta N, Tulipan NB, D’Alton ME, Farmer DL; the MOMS Investigators: A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med 2011; 364: 993–1004. 68% Grupo Pré-natal X 98% Grupo Pós-Natal
- 51. Resultados Mielomeningocele – MOMS Trial • Desfecho primário - 30 meses • Índice de Desenvolvimento Mental de Bailey • Diferença entre os níveis funcional e anatômico. Adzick NS, Thom EA, Spong CY, Brock III JW, Burrows PK, Johnson MP, Howell LJ, Farrell JA, Dabrowiak ME, Sutton LN, Gupta N, Tulipan NB, D’Alton ME, Farmer DL; the MOMS Investigators: A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med 2011; 364: 993–1004. Significativamente melhor no grupo Pré-Natal
- 53. Resultados Mielomeningocele – MOMS Trial • Desfecho secundário • Cirurgia pré-natal foi superior: • Nível funcional 2 ou + acima do grupo pós-natal. • Capacidade de andar sem aparelhos (42% vs. 21%, P = 0.01) • Melhor função motora. • Melhor independência e mobilidade (relatado pelos pais). • Sem diferença no escore cognitivo Adzick NS, Thom EA, Spong CY, Brock III JW, Burrows PK, Johnson MP, Howell LJ, Farrell JA, Dabrowiak ME, Sutton LN, Gupta N, Tulipan NB, D’Alton ME, Farmer DL; the MOMS Investigators: A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med 2011; 364: 993–1004.
- 54. Discussão Mielomeningocele – MOMS Trial Adzick NS, Thom EA, Spong CY, Brock III JW, Burrows PK, Johnson MP, Howell LJ, Farrell JA, Dabrowiak ME, Sutton LN, Gupta N, Tulipan NB, D’Alton ME, Farmer DL; the MOMS Investigators: A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med 2011; 364: 993–1004. Menor necessidade de shunt e melhor desenvolvimento motor X Maior morbidade materna e fetal
- 55. Mielomeningocel e Adzick NS, Thom EA, Spong CY, Brock III JW, Burrows PK, Johnson MP, Howell LJ, Farrell JA, Dabrowiak ME, Sutton LN, Gupta N, Tulipan NB, D’Alton ME, Farmer DL; the MOMS Investigators: A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med 2011; 364: 993–1004.
- 56. Método Mielomeningocele – Reparo via mini- histerotomia • Estudo descritivo de uma série de casos de reparo de MMC via mini-histerotomia. • Outubro/2014 – Abril/2016 Botelho, RD; Imada, V; Da Costa, KJR; Watanabe, LC; Rossi, RJ; De Sallae, AAF; Romano, E; Peralta, CFA. Fetal Myelomeningocele Repair through a Mini-Hysterotomy. Fetal Diagn Ther, 2016
- 57. Método • Critérios de inclusão • Gestação única • Limite superior da mielomeningocele entre T1 e S1 • Chiari II • Idade gestacional entre 18+0 e 26+6 semanas • Cariótipo normal • Ausência de outras malformações Mielomeningocele – Reparo via mini- histerotomia Botelho, RD; Imada, V; Da Costa, KJR; Watanabe, LC; Rossi, RJ; De Sallae, AAF; Romano, E; Peralta, CFA. Fetal Myelomeningocele Repair through a Mini-Hysterotomy. Fetal Diagn Ther, 2016
- 58. Método • Critérios de inclusão • Ausência de fatores de risco para TPP • Ausência de escoliose > 30º • Ausência de doença materna que aumente risco • Sorologia negativa para Hepatite B, C e HIV • Fácil acesso ao centro terciário Mielomeningocele – Reparo via mini- histerotomia Botelho, RD; Imada, V; Da Costa, KJR; Watanabe, LC; Rossi, RJ; De Sallae, AAF; Romano, E; Peralta, CFA. Fetal Myelomeningocele Repair through a Mini-Hysterotomy. Fetal Diagn Ther, 2016
- 59. Mielomeningocele – Reparo via mini- histerotomia Botelho, RD; Imada, V; Da Costa, KJR; Watanabe, LC; Rossi, RJ; De Sallae, AAF; Romano, E; Peralta, CFA. Fetal Myelomeningocele Repair through a Mini-Hysterotomy. Fetal Diagn Ther, 2016
- 60. Método • Variáveis avaliadas • Características maternas e fetais no momento da cirurgia Mielomeningocele – Reparo via mini- histerotomia • Idade materna e fetal • História prévia de defeito do tubo neural • Paridade • IMC • Nível superior da MMC • Deformidade e movimentos de MMII • Tamanho do ventrículo lateral • Posição da placenta Botelho, RD; Imada, V; Da Costa, KJR; Watanabe, LC; Rossi, RJ; De Sallae, AAF; Romano, E; Peralta, CFA. Fetal Myelomeningocele Repair through a Mini-Hysterotomy. Fetal Diagn Ther, 2016
- 61. Método • Variáveis avaliadas • Perioperatórias Mielomeningocele – Reparo via mini- histerotomia • Tamanho da histerotomia • Tempo de cirurgia total • Tempo de cirurgia fetal • Intercorrências intra e pós-operatórias • Tempo de internamento Botelho, RD; Imada, V; Da Costa, KJR; Watanabe, LC; Rossi, RJ; De Sallae, AAF; Romano, E; Peralta, CFA. Fetal Myelomeningocele Repair through a Mini-Hysterotomy. Fetal Diagn Ther, 2016
- 62. Método • Variáveis avaliadas • Da alta cirúrgica ao nascimento Mielomeningocele – Reparo via mini- histerotomia • Mudança no tamanho do ventrículo lateral • Reversão do Chiari II • Presença de deformidade e movimentação em MMII • Separação corio-amniótica Botelho, RD; Imada, V; Da Costa, KJR; Watanabe, LC; Rossi, RJ; De Sallae, AAF; Romano, E; Peralta, CFA. Fetal Myelomeningocele Repair through a Mini-Hysterotomy. Fetal Diagn Ther, 2016
- 63. Método • Variáveis avaliadas • Da alta cirúrgica ao nascimento Mielomeningocele – Reparo via mini- histerotomia • RUPREME pré-termo • IG na RUPREME • Intervalo procedimento X nascimento • Intervalo RUPREME X nascimento Botelho, RD; Imada, V; Da Costa, KJR; Watanabe, LC; Rossi, RJ; De Sallae, AAF; Romano, E; Peralta, CFA. Fetal Myelomeningocele Repair through a Mini-Hysterotomy. Fetal Diagn Ther, 2016
- 64. Método • Variáveis avaliadas • Peri-parto • IG ao nascimento • Complicações materna/fetal na cesariana • Condição da cicatriz de histerotomia • Apgar • Peso ao nascer Mielomeningocele – Reparo via mini- histerotomia Botelho, RD; Imada, V; Da Costa, KJR; Watanabe, LC; Rossi, RJ; De Sallae, AAF; Romano, E; Peralta, CFA. Fetal Myelomeningocele Repair through a Mini-Hysterotomy. Fetal Diagn Ther, 2016
- 65. Método • Variáveis avaliadas • Neonatais até a alta • Condição da cicatriz • Resultado US transfontanelar/RM • Necessidade de shunt • Condição motora e neurológica • Tempo de internamento • Ocorrência de morte Mielomeningocele – Reparo via mini- histerotomia Botelho, RD; Imada, V; Da Costa, KJR; Watanabe, LC; Rossi, RJ; De Sallae, AAF; Romano, E; Peralta, CFA. Fetal Myelomeningocele Repair through a Mini-Hysterotomy. Fetal Diagn Ther, 2016
- 66. Resultado • 45 pacientes submetidas a cirurgia. • 39 nascimentos até o momento da análise. • Correção completa em todos os casos. • Nenhuma complicação materna ou fetal durante ou após a cirurgia até a alta (1 caso de pneumonia). • 2 pacientes com história prévia de defeito de tubo neural (4,4%) Mielomeningocele – Reparo via mini- histerotomia Botelho, RD; Imada, V; Da Costa, KJR; Watanabe, LC; Rossi, RJ; De Sallae, AAF; Romano, E; Peralta, CFA. Fetal Myelomeningocele Repair through a Mini-Hysterotomy. Fetal Diagn Ther, 2016
- 67. Resultado Mielomeningocele – Reparo via mini- histerotomia • Variáveis avaliadas • Características maternas e fetais no momento da cirurgia Botelho, RD; Imada, V; Da Costa, KJR; Watanabe, LC; Rossi, RJ; De Sallae, AAF; Romano, E; Peralta, CFA. Fetal Myelomeningocele Repair through a Mini-Hysterotomy. Fetal Diagn Ther, 2016
- 68. Resultado Mielomeningocele – Reparo via mini- histerotomia • Variáveis avaliadas • Da alta cirúrgica ao nascimento Botelho, RD; Imada, V; Da Costa, KJR; Watanabe, LC; Rossi, RJ; De Sallae, AAF; Romano, E; Peralta, CFA. Fetal Myelomeningocele Repair through a Mini-Hysterotomy. Fetal Diagn Ther, 2016
- 69. Resultado Mielomeningocele – Reparo via mini- histerotomia • Variáveis avaliadas • Peri-parto Botelho, RD; Imada, V; Da Costa, KJR; Watanabe, LC; Rossi, RJ; De Sallae, AAF; Romano, E; Peralta, CFA. Fetal Myelomeningocele Repair through a Mini-Hysterotomy. Fetal Diagn Ther, 2016
- 70. Resultado Mielomeningocele – Reparo via mini- histerotomia • Variáveis avaliadas • Neonatais Botelho, RD; Imada, V; Da Costa, KJR; Watanabe, LC; Rossi, RJ; De Sallae, AAF; Romano, E; Peralta, CFA. Fetal Myelomeningocele Repair through a Mini-Hysterotomy. Fetal Diagn Ther, 2016
Notas do Editor
- Maternal polymorphisms or mutations in various enzymesinthehomocysteineremethylationpathwayconferan increasedriskforfetalneuraltubedefects.Theenzyme5,10methylenetetrahydrofolatereductase(MTHFR)isimportant intheproductionofthecirculatingformoffolicacid.Specific polymorphismsinthegeneforMTHFR,suchasmaternalhomozygosity for the 677T allele, or compound heterozygosity fortheC677T/A1298Calleles,carryanincreasedrisk(Peadar et al., 2004). Maternal variation in the gene for another enzyme,methioninesynthase(MTRR)alsocarriesanincreased riskforaffectedfetuseswhenpresentincombinationwithan MTHFR polymorphism or low B12 levels (van der Linden et al., 2006).
- the rates of open neural tube defects, both anencephaly and myelomeningocele, have fallen by 26% in the United States, compared to before 1998 when mandatory folate fortification of cereal grain products began The mechanism by which folic acid mediates neural tube closure is unknown, although the preventive effect has been shown even in lowrisk women (Milunsky, 1996).
- More than 80% of children with a neural tube defect can be detected by maternal serum AFP screening before birth (Brock and Sutcliffe, 1972). Although the determination of amniotic fluid acetylcholinesterase can be helpful, ultrasound examination is the method of choice for the diagnosis of neural tube defects.
- Tronco cerebral Translucencia intracraniana = IV ventrículo Cisterna Magna
- O sinal do limão é achado mais frequentemente antes de 24 semanas. Ele é causado pois a pressão intracraniana diminui devido ao escoamento do liquido cefalo raquidiano. Após 24 semanas tende a desaparecer devido à calcificação da calota craniana.
- the banana sign was more typical at <24 weeks, and apparent cerebellar absence was typical of fetuses at >24 weeks. A ventriculomegalia lateral é avaliada pela medida do átrio do ventriculo lateral >1cm. A hidrocefalia normalmente é progressiva e surge a partir do fim do Segundo trimestre. O sinal do limão aparece em 1% dos fetos normais. O sinal da banana não aprece em fetos normais.
- Taxa de mortalidade de 10% entre os nascidos vivos, A maioria dos sobreviventes terá paralisia e/ou disfunções intestinais e urinárias.
- O tecido que recobre a lesão não é pele normal e permite o contato das raízes nervosas com o líquido amniótico. Como a pele é não funcionante por mais que apenas a meninge esteja herniada, haverá lesão nervosa. Os danos e, consequentemente as sequelas, são progressivos. Com o passar da gestação a qtidade de LA vai diminuindo e os traumas tornam-se mais frequentes. These data suggest a “two-hit” hypothesis in which the final neurologic deficit results from a combination of failure of neural-tube formation and spinal cord injury resulting from prolonged exposure of neural elements to the intrauterine environment.
- A cromossomopatia mais frequentemente associada – T18 Em geral, quanto mais baixo o defeito melhor o prognóstico.
- With additional observations, the most recent recommendations for delivery are the following (Shurtleff and Lemire,1995):electivecesareansectionisindicatedwhenthe fetus demonstrates movement of the knees and ankles and a MMC sac is observed protruding dorsally beyond the plane of the infant’s back; cesarean section is contraindicated for fetuses with a known chromosomal abnormality, other congenitalanomaliesthatsignificantlyinterferewithsurvival,or theabsenceoffetalkneeoranklemovement;cesareansection has not been shown to be beneficial in primiparous women with a fetus already engaged in the breech position, fetuses withgibbousdeformities,andfetuseswithhypoplasticspinal cords.
- A técnica vem sendo desenvolvida desde a década de 90. Pacientes apresentam melhores desfechos se comparados com cx pós-natal.
- Determinar o nível funcional e a extensão do déficit neurológico
- 100% nível torácico necessita de shunt 88% lombar 68% sacral
- To stand erect, motor function is needed to at least the third lumbar level. To walk, the child must exhibit motor function from thefourthtothefifthlumbarlevel.Tofunctionsexuallyasan adult,amalemusthavemotorfunctiontoatleastthesecond to fourth sacral level.
- To stand erect, motor function is needed to at least the third lumbar level. To walk, the child must exhibit motor function from thefourthtothefifthlumbarlevel.Tofunctionsexuallyasan adult,amalemusthavemotorfunctiontoatleastthesecond to fourth sacral level.
- To stand erect, motor function is needed to at least the third lumbar level. To walk, the child must exhibit motor function from thefourthtothefifthlumbarlevel.Tofunctionsexuallyasan adult,amalemusthavemotorfunctiontoatleastthesecond to fourth sacral level.
- Necessário o uso de antibióticos evitar ITU. Anticolinérgicos bexiga hiperativa
- Pé torto mais rígido do que na forma idiopática. Normalmente necessita correção cirúrgica.
- Hunt e Poulton(1995) acompanharam 117 nascido com MMC e operados na sequencia. 25 morreram antes de 1 ano 15 entre 1-5 anos /// 8 entre 5-16 anos /// 8 entre 16-25 anos 16/56 mortes – insuficiência renal
- To stand erect, motor function is needed to at least the third lumbar level. To walk, the child must exhibit motor function from thefourthtothefifthlumbarlevel.Tofunctionsexuallyasan adult,amalemusthavemotorfunctiontoatleastthesecond to fourth sacral level.
- To stand erect, motor function is needed to at least the third lumbar level. To walk, the child must exhibit motor function from thefourthtothefifthlumbarlevel.Tofunctionsexuallyasan adult,amalemusthavemotorfunctiontoatleastthesecond to fourth sacral level.
- To stand erect, motor function is needed to at least the third lumbar level. To walk, the child must exhibit motor function from thefourthtothefifthlumbarlevel.Tofunctionsexuallyasan adult,amalemusthavemotorfunctiontoatleastthesecond to fourth sacral level.
- To stand erect, motor function is needed to at least the third lumbar level. To walk, the child must exhibit motor function from thefourthtothefifthlumbarlevel.Tofunctionsexuallyasan adult,amalemusthavemotorfunctiontoatleastthesecond to fourth sacral level.
- A cromossomopatia mais frequentemente associada – T18 Em geral, quanto mais baixo o defeito melhor o prognóstico.
- Abertura transversa da parede abdominal (longitudinal se IMC>35) Feto e placenta localizado. Posicionamento do feto Placoide neural identificado e dissecado dos tecidos subjacentes Fechamento da duramater sobre o placoide Fechamento da pele Prenatal Surgery Procedure: Cephazolin (1000 mg IV) and indomethacin (50 mg PR or PO) were given preoperatively. A combination of general and epidural anesthesia was used. The indwelling epidural catheter enabled administration of continuous postoperative analgesics. The gravid uterus was exposed via a low transverse laparotomy incision and exteriorized. A vertical skin incision was used in patients with a BMI>30 or those with a previous vertical skin scar. The fetus and placenta were then located by ultrasound and the hysterotomy location chosen by the primary surgeon. The fetus was visualized by ultrasound and manually positioned within the uterus such that the myelomeningocele sac was in the center of the hysterotomy. In the case of an anterior placenta, hysterotomy was either fundal or posterior. In the case of a posterior placenta, uterine entry was anterior. Under sonographic guidance, the surgeon placed two monofilament traction sutures through the full thickness uterine wall, initial uterine entry was accomplished sharply between the uterine traction sutures, then the uterine stapling device loaded with absorbable polyglycolic acid staples (Covidien Auto Suture, Norwalk CT) was passed into the uterine cavity. The stapler was palpated manually and ultrasonography was used to exclude the presence of fetal tissue, then the stapler was used to create a 6-8 cm uterine incision large enough to expose the fetal myelomeningocele. The fetus was given an intramuscular injection of fentanyl (20 mcg/kg) and vecuronium (0.2mg/kg). During the procedure, the fetal cardiac function was monitored with continuous echocardiography by an individual not involved in the actual prenatal surgery. The myelomeningocele was closed in a standardized manner under magnification. The neural placode was sharply dissected from surrounding tissue and allowed to drop into the spinal canal. The dura was then identified, reflected over the placode and then closed using a fine running suture. If there was insufficient dura for closure, Duragen (Integra Life Sciences Corporation, Plainsboro, NJ) was substituted. If it was not possible to obtain skin closure, relaxing incisions were made or Alloderm (Life Cell, Branchburg, NJ) was used. Finally, the skin was mobilized and closed using a fine running monofilament suture. The uterus was closed in two layers. The first layer incorporated the absorbable staples and uterine membranes. As the last stitches of this layer were placed, warmed Ringer’s lactate, mixed with 500 mg of Nafcillin or vancomycin, was added to the uterus until the amniotic fluid index was normal. A second imbricating layer of suture was tied. The abdominal fascial layer and dermis were closed in routine fashion.
- The second primary outcome, at 30 months, was a composite score of the Mental Development Index of the Bayley Scales of Infant Development II and the child’s motor function, with adjustment for lesion level.
- The second primary outcome, at 30 months, was a composite score of the Mental Development Index of the Bayley Scales of Infant Development II and the child’s motor function, with adjustment for lesion level.
- From February 2003 through December 2010, a total of 183 eligible women underwent randomization (Figure 2FIGURE 2Enrollment and Outcomes.). In the prenatal-surgery group, one woman chose postnatal surgery after randomization, and two women returned home for delivery. In the postnatal-surgery group, seven women chose not to return to the clinical center for delivery, and four were unable to return because of preterm labor or other complications. The data and safety monitoring committee met on December 7, 2010, and recommended termination of the trial on the basis of efficacy of prenatal surgery. Of the 183 women who underwent randomization, this report is based on the findings in 158 women who underwent randomization before July 1, 2009 (i.e., the outcome cohort reviewed by the committee). For outcomes up to 30 months, the report is based on the findings in 134 women who underwent randomization before December 1, 2007.
- The first primary outcome, fetal or neonatal death or the need for a cerebrospinal fluid shunt by the age of 12 months, occurred in 68% of infants in the prenatal-surgery group and in 98% in the postnatal-surgery group (relative risk, 0.70; 97.7% confidence interval [CI], 0.58 to 0.84; P<0.001) (Table 3). Rates of actual shunt placement were 40% in the prenatal-surgery group and 82% in the postnatal-surgery group (P<0.001). (Additional details about the primary outcome are provided in the figure in the Supplementary Appendix.) At 12 months of age, the proportion of infants who had no evidence of hindbrain herniation was higher in the prenatal-surgery group (36%) than in the postnatal-surgery group (4%). Similarly, at 12 months, the prenatal-surgery group had a lower rate of moderate or severe hindbrain herniation (25%) than the postnatal-surgery group (67%), as well as lower rates of brain-stem kinking, abnormal fourth-ventricle location, and syringomyelia. There were no significant betweengroup differences in the rates of identification of epidermoid cysts. Infants in the prenatal-surgery group underwent more procedures for delayed spinal cord tethering.
- Duas mortes, uma em cada grupo entre 12-30 meses: grupo pré-natal (sepse por coxsackie), pós natal (complicações de QT por carcinoma de plexo coróide)
- Duas mortes, uma em cada grupo entre 12-30 meses: grupo pré-natal (sepse por coxsackie), pós natal (complicações de QT por carcinoma de plexo coróide)
- Duas mortes, uma em cada grupo entre 12-30 meses: grupo pré-natal (sepse por coxsackie), pós natal (complicações de QT por carcinoma de plexo coróide)
- The inclusion criteria for fetal surgery were as follows: (1) singleton pregnancy; (2) gestational age (GA) from 18 +0 to 26 +6 weeks; (3) MMC with an upper anatomical level from T1 to S1 and the presence of a Chiari II malformation; (4) no chromosomal abnormality or fetal anatomical defect other than the MMC and associated alterations; (5) no previous history of prematurity or a short cervix of less than 25 mm in size during the current pregnancy; (6) no fetal scoliosis of more than 30°; (7) easy access to the tertiary referring center for follow-up and/or emergency assistance after fetal surgery; (8) no serious maternal disease that could significantly increase the surgical risk, and (9) no positive maternal serology for HIV or hepatitis B and C.
- The inclusion criteria for fetal surgery were as follows: (1) singleton pregnancy; (2) gestational age (GA) from 18 +0 to 26 +6 weeks; (3) MMC with an upper anatomical level from T1 to S1 and the presence of a Chiari II malformation; (4) no chromosomal abnormality or fetal anatomical defect other than the MMC and associated alterations; (5) no previous history of prematurity or a short cervix of less than 25 mm in size during the current pregnancy; (6) no fetal scoliosis of more than 30°; (7) easy access to the tertiary referring center for follow-up and/or emergency assistance after fetal surgery; (8) no serious maternal disease that could significantly increase the surgical risk, and (9) no positive maternal serology for HIV or hepatitis B and C.
- Abertura da parede – pfannestil Feto movido externamente para o local de abertura Histerotomia de 2,5-3,5cm à pelo menos 2cm da placenta Membranas suturadas no miométrio Colocado um retractor Neurocirurgiões operando, especilista em MF segura e manipula o feto cuidadosamente Fechamento do placóide neural Fechamento do útero em duas camadas
- The inclusion criteria for fetal surgery were as follows: (1) singleton pregnancy; (2) gestational age (GA) from 18 +0 to 26 +6 weeks; (3) MMC with an upper anatomical level from T1 to S1 and the presence of a Chiari II malformation; (4) no chromosomal abnormality or fetal anatomical defect other than the MMC and associated alterations; (5) no previous history of prematurity or a short cervix of less than 25 mm in size during the current pregnancy; (6) no fetal scoliosis of more than 30°; (7) easy access to the tertiary referring center for follow-up and/or emergency assistance after fetal surgery; (8) no serious maternal disease that could significantly increase the surgical risk, and (9) no positive maternal serology for HIV or hepatitis B and C.
- The inclusion criteria for fetal surgery were as follows: (1) singleton pregnancy; (2) gestational age (GA) from 18 +0 to 26 +6 weeks; (3) MMC with an upper anatomical level from T1 to S1 and the presence of a Chiari II malformation; (4) no chromosomal abnormality or fetal anatomical defect other than the MMC and associated alterations; (5) no previous history of prematurity or a short cervix of less than 25 mm in size during the current pregnancy; (6) no fetal scoliosis of more than 30°; (7) easy access to the tertiary referring center for follow-up and/or emergency assistance after fetal surgery; (8) no serious maternal disease that could significantly increase the surgical risk, and (9) no positive maternal serology for HIV or hepatitis B and C.
- The inclusion criteria for fetal surgery were as follows: (1) singleton pregnancy; (2) gestational age (GA) from 18 +0 to 26 +6 weeks; (3) MMC with an upper anatomical level from T1 to S1 and the presence of a Chiari II malformation; (4) no chromosomal abnormality or fetal anatomical defect other than the MMC and associated alterations; (5) no previous history of prematurity or a short cervix of less than 25 mm in size during the current pregnancy; (6) no fetal scoliosis of more than 30°; (7) easy access to the tertiary referring center for follow-up and/or emergency assistance after fetal surgery; (8) no serious maternal disease that could significantly increase the surgical risk, and (9) no positive maternal serology for HIV or hepatitis B and C.