Current diagnosis, management and treatment of fetal myelomeningocele

Authors

DOI:

https://doi.org/10.29166/rfcmq.v47i2.3554

Keywords:

meningomyelocele, spinal dysraphism, prenatal diagnosis

Abstract

Introduction: Fetal myelomeningocele is spinal cord extrusion that occurs due to the caudal neuropore incomplete closure, which exposes the neural plate to mechanical and chemical trauma to the uterus wall and amniotic fluid respectively, with serious sequelae. Prenatal detection innovated the diagnosis and with this intra-uterine repair has improved treatment strategies, generating care options in those patients who comply the selection criteria for prenatal surgery.

Objective: Submit a bibliographic review on the diagnosis, management and treatment of fetal myelomeningocele and to offer the scientific community a consultation tool to improve knowledge of the subject and timely treatment alternatives for fetuses with this malformation.

Methodology: This is a systematic review study without meta-analysis, based on the PRISMA recommendations. The information search was structured under the PICO system. The searches were carried out in Pubmed, Tripdatabase and Pubmed Central; selecting articles published during the last ten years in English or Spanish, on diagnosis, management and treatment of fetal myelomeningocele.

Results: 120 articles met the search criteria, of which 42 were eligible, with relevant information for determining the current diagnosis, management and treatment of fetal myelomeningocele through innovative procedures.

Discussion: Neural tube defects appear as a consequence of an alteration of the neurulation process between days 21-28 after conception. MMCf is considered the most common non-fatal congenital defect of the CNS. It is characterized by protrusion of the meninges and spinal cord with permanent neurological damage. For this reason, the diagnosis and timely management of this pathology have allowed intra utero fetal surgery to be considered the optimal method, improving rhombencephalon hernia, reducing the need for a ventricular shunt and maintaining the

lower motor skills, as well as neuronal, bladder and gastrointestinal function, improving the quality of life of the patient affected by this pathology.

Downloads

Download data is not yet available.

Metrics

Metrics Loading ...

Author Biographies

Lizeth Cali-Tobar , 0000-0002-3514-6662

Posgradista de Ginecología y Obstetricia. Universidad Central del Ecuador. Quito-Ecuador.

Lautaro Chávez-Iza

Posgradista de Ginecología y Obstetricia. Universidad Central del Ecuador. Quito-Ecuado

Alberto López-Mayorga

Docente. Posgrado de Ginecolo- gía y Obstetricia. Universidad Central del Ecuador. -Gineco-obste-tra. Departamento de Ginecología. Hospital Gineco-Obstétrico Isidro Ayora. Quito-Ecuador

References

Moldenhauer J, Flake A. Open fetal surgery for neural tube defects.

Best Pract Res Clin Obstet Gynaecol. [Internet], 2019 Jul; [cited 2019 Mar 18], 58:121-132. doi: 10.1016/j.bpobgyn.2019.03.004. Available from: https://www.sciencedirect.com/science/article/abs/pii/S1521693419300227?via%3Dihub

Hii LY, Sung ChA, Shaw S. Fetal surgery and stem cell therapy for meningomyelocele. Curr Opin Obstet Gynecol. [Internet], 2020 Apr; 32(2):147-151.

doi: 10.1097/GCO.0000000000000614. Available from: https://journals.lww.com/co-obgyn/Abstract/2020/04000/Fetal_surgery_and_stem_cell_therapy_for.8.aspx

Reghunath A, Gupta Ghasi R, Aggarwal A. Unveiling the tale of the tail: an illustration of spinal dysraphisms. Neurosurg Rev. [Internet], 2021 Feb; [cited 2019 Dec 7], 44(1):97-114. doi: 10.1007/s10143-019-01215-z. Available from: https://link.springer.com/article/10.1007%2Fs10143-019-01215-z

Zamłyński M, Zamłyński J, Horzelska E, Maruniak-Chudek I, Bablok R, Szukiewicz D, et al. The Use of Indomethacin with Complete Amniotic Fluid Replacement and Classic Hysterotomy for the Reduction of Perinatal Complications of Intrauterine Myelomeningocele Repair. Fetal Diagn Ther. [Internet], 2019 Jan; [cited 2019 May 14], 46(6):415-424. doi: 10.1159/000496811. Available from: https://www.karger.com/Article/Abstract/496811

Meller C, Covini D, Aiello H, Izbizky G, Portillo S, Otaño L. Update on prenatal diagnosis and fetal surgery for myelomeningocele. Arch Argent Pediatr. [Internet], 2021 Jun; 119 (3):e215-e228. doi: 10.5546/aap.2021.eng.e215. Available from: https://www.sap.org.ar/docs/publicaciones/archivosarg/2021/v119n3a16e.pdf

Meuli M, Moehrlen U. Fetal Surgery for Myelomeningocele: A Critical Appraisal.

Eur J Pediatr Surg. [Internet], 2013 Apr; [cited 2013 Apr 9], 23(2):103-9. doi: 10.1055/s-0033-1343082. Available from: https://www.thieme-connect.com/products/ejournals/abstract/10.1055/s-0033-1343082

Elbabaa S, Gildehaus A, Pierson M, Albers J, Vlastos E. First 60 fetal in-utero myelomeningocele repairs at Saint LouisFetal Care Institute in the post-MOMS trial era: hydrocephalustreatment outcomes (endoscopic third ventriculostomy versusventriculo-peritoneal shunt). Childs Nerv Syst. [Internet], 2017 Jul; [cited 2017 May 3], 33(7):1157-1168. doi: 10.1007/s00381-017-3428-8. Available from: https://link.springer.com/article/10.1007%2Fs00381-017-3428-8

Clayton D, Thomas J, Brock J. Fetal repair of myelomeningocele: current status and urologic implications. J Pediatr Urol. [Internet], 2020 Feb; [cited 2019 Dec 6], 16(1):3-9. doi: 10.1016/j.jpurol.2019.11.019. Available from: https://www.jpurol.com/article/S1477-5131(19)30424-3/fulltext

Van Calenbergh F, Joyeux L, Deprest J. Maternal-fetal surgery for myelomeningocele: some thoughts on ethical, legal, and psychological issues in a Western European situation. Childs Nerv Syst. [Internet], 2017 Aug; [cited 2017 May 23], 33(8):1247-1252. doi: 10.1007/s00381-017-3446-6. Available from: https://link.springer.com/article/10.1007%2Fs00381-017-3446-6

Kowitzke B, Cohrs G, Leuschner I, Koch A, Synowitz M, et al. Cellular Profiles and Molecular Mediators of Lesion Cascades in the Placode in Human Open Spinal Neural Tube Defects. J Neuropathol Exp Neurol. [Internet], 2016 Sep; [cited 2016 Jun 28], 75(9):827-42. doi: 10.1093/jnen/nlw057. Available from: https://academic.oup.com/jnen/article-lookup/doi/10.1093/jnen/nlw057

Kim A , Danzer E, Moldenhauer J, Khalek N, McClain L, Waqar L, Hedrick H, et al. Amniotic Fluid Concentrations of Glial Fibrillary Acidic Protein Do Not Correlate with Prenatal Metrics in Fetuses with Myelomeningocele. Fetal Diagn Ther. [Internet], 2018; [cited 2017 Jul 29], 43(4):297-303. doi: 10.1159/000478258.Available from: https://www.karger.com/Article/Abstract/478258

Al Refai A, Drake J, Kulkarni A, Connor K, Shannon P, Toi A. Fetal myelomeningocele surgery: Only treating the tip of the iceberg. Prenat Diagn. [Internet], 2019 Jan; [cited 2018 Dec 10], 39(1):10-15. doi: 10.1002/pd.5390.

Available from: https://obgyn.onlinelibrary.wiley.com/doi/10.1002/pd.5390

Cavalheiro S , Silva da Costa M, Fernandes A, Leonard J. Comparison of Prenatal and Postnatal Management of Patients with Myelomeningocele.

Neurosurg Clin N Am. [Internet], 2017 Jul; [cited 2017 Mar 24], 28(3):439-448. doi: 10.1016/j.nec.2017.02.005. Available from: https://www.sciencedirect.com/science/article/pii/S1042368017300177?via%3Dihub

Le H, Cardona-Grau D, Chiang G. Evaluation and Long-term Management of Neurogenic Bladder in Spinal Dysraphism.

Neoreviews. [Internet], 2019 Dec; 20(12):e711-e724. doi: 10.1542/neo.20-12-e711. Available from: https://neoreviews.aappublications.org/content/20/12/e711.long

Cass D. Impact of prenatal diagnosis and therapy on neonatal surgery.

Semin Fetal Neonatal Med. [Internet], 2011 Jun; [cited 2011 May 6], 16(3):130-8. doi: 10.1016/j.siny.2011.03.007.

Available from: https://www.sfnmjournal.com/article/S1744-165X(11)00024-2/fulltext

Nagaraj U, Kline-Fath B. Imaging of open spinal dysraphisms in the era of prenatal surgery. Pediatr Radiol. [Internet], 2020 Dec; [cited 2020 Nov 30], 50(13):1988-1998. doi: 10.1007/s00247-020-04734-w.

Sacco A, Simpson L, Deprest J, David AL. A study to assess global availability of fetal surgery for myelomeningocele. Prenat Diagn. [Internet], 2018 Dec; [cited 2018 Nov 20], 38(13):1020-1027. doi: 10.1002/pd.5383. Available from: https://obgyn.onlinelibrary.wiley.com/doi/10.1002/pd.5383

Tulipan N, Wellons J, Thom E, Gupta N, Sutton L, Burrows P. Prenatal surgery for myelomeningocele and the need for cerebrospinal fluid shunt placement. J Neurosurg Pediatr. [Internet], 2015 Dec; [cited 2015 Sep 15], 16(6):613-20. doi: 10.3171/2015.7.PEDS15336.

Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5206797/

Flanders T, Madsen P, Pisapia J, Hudgins E, Mackell C, Alexander E, et al. Improved Postoperative Metrics with Modified Myofascial Closure in Fetal Myelomeningocele Repair. Oper Neurosurg (Hagerstown). [Internet], 2020 Feb 1; 18(2):158-165. doi: 10.1093/ons/opz115. Available from: https://academic.oup.com/ons/article-abstract/18/2/158/5521236?redirectedFrom=fulltext

Joyeux L, De Bie F, Danzer E, Russo F , Javaux A, Peralta c, et al. Learning curves of open and endoscopic fetal spina bifida closure: systematic review and meta-analysis. Ultrasound Obstet Gynecol. [Internet], 2020 Jun; 55(6):730-739. doi: 10.1002/uog.20389. Available from: https://obgyn.onlinelibrary.wiley.com/doi/10.1002/uog.20389

Cavalheiro S, da Costa M, Mendonça J, Dastoli P, Suriano I, Barbosa M. Antenatal management of fetal neurosurgical diseases. Childs Nerv Syst. [Internet], 2017 Jul; [cited 2027 May 29], 33(7):1125-1141.doi: 10.1007/s00381-017-3442-x. Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5496971/

Trigubo D, Negri M, Salvatico R, Leguizamón G. The role of intrauterine magnetic resonance in the management of myelomeningocele.

Childs Nerv Syst. [Internet], 2017 Jul; [cited 2017 May 17], 33(7):1107-1111. doi: 10.1007/s00381-017-3418-x.

Available from: https://link.springer.com/article/10.1007%2Fs00381-017-3418-x

Sherrod B, Ho W, Hedlund A, Kennedy A, Ostrander B, Bollo R. A comparison of the accuracy of fetal MRI and prenatal ultrasonography at predicting lesion level and perinatal motor outcome in patients with myelomeningocele. Neurosurg Focus. [Internet], 2019 Oct 1; 47(4):E4. doi: 10.3171/2019.7.FOCUS19450.

Available from: https://thejns.org/focus/view/journals/neurosurg-focus/47/4/article-pE4.xml

Heuer G, Moldenhauer J, Scott Adzick N. Prenatal surgery for myelomeningocele: review of the literature and future directions. Childs Nerv Syst. [Internet], 2017 Jul; [cited 2017 May 17], 33(7):1149-1155. doi: 10.1007/s00381-017-3440-z.

Available from: https://link.springer.com/article/10.1007%2Fs00381-017-3440-z

Dewan M, Wellons J. Fetal surgery for spina bífida.

J Neurosurg Pediatr. [Internet], 2019 Aug 1; [cited 2019 Aug 1], 24(2):105-114. doi: 10.3171/2019.4.PEDS18383.

Available from: https://thejns.org/pediatrics/view/journals/j-neurosurg-pediatr/24/2/article-p105.xml

Farmer D, Thom E, Brock J, Burrows P, Johnson M, Howell L, et al. The Management of Myelomeningocele Study: Full cohort 30 month pediatric outcomes.

Am J Obstet Gynecol. [Internet], 2018 Feb; [cited 2017 Dec 12], 218(2):256.e1-256.e13. doi: 10.1016/j.ajog.2017.12.001. Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7737375/

Laskay N, Arynchyna A, McClugage S, Hopson B, Shannon C, Ditty B, et al. A comparison of the MOMS trial results to a contemporaneous, single-institution, post-natal closure cohort. Childs Nerv Syst. [Internet], 2017 Apr; [cited 2016 Dec 27], 33(4):639-646. doi: 10.1007/s00381-016-3328-3. Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5383528/

Pan E, Pallapati J, Krueger A, Yepez M, VanLoh S, Nassr A. Evaluation and Disposition of Fetal Myelomeningocele Repair Candidates: A Large Referral Center Experience. Fetal Diagn Ther. [Internet], 2020; [cited 2019 Jun 18], 47(2):115-122. doi: 10.1159/000500451. Available from: https://www.karger.com/Article/Abstract/500451

Moldenhauer J, Scott Adzick N. Fetal surgery for myelomeningocele: After the Management of Myelomeningocele Study (MOMS).

Semin Fetal Neonatal Med. [Internet], 2017 Dec; [cited 2017 Oct 12], 22(6):360-366. doi: 10.1016/j.siny.2017.08.004.

Available from: https://www.sfnmjournal.com/article/S1744-165X(17)30093-8/fulltext

Pedreira D, Zanon N, Nishikuni K, Moreira de Sá R, Acacio G, Chmait R. Endoscopic surgery for the antenatal treatmentof myelomeningocele: the CECAM trial.

Am J Obstet Gynecol. [Internet], 2016 Jan; [cited 2015 Sep 18], 214(1):111.e1-111.e11. doi: 10.1016/j.ajog.2015.09.065. Available from: https://www.ajog.org/article/S0002-9378(15)01104-7/fulltext

Published

2022-01-01

How to Cite

1.
Cali-Tobar L, Chávez-Iza L, López-Mayorga A. Current diagnosis, management and treatment of fetal myelomeningocele. Rev Fac Cien Med (Quito) [Internet]. 2022 Jan. 1 [cited 2024 Jul. 3];47(2):39-50. Available from: https://revistadigital.uce.edu.ec/index.php/CIENCIAS_MEDICAS/article/view/3554