Abstract
The intrauterine repair of myelomeningocele presents certain advantages and has gained widespread acceptance. It significantly reduces the incidence of Chiari-2 anomalies and hydrocephalus, and it is thought to enhance the neurologic outcome. Nevertheless, several issues remain unsettled and there are no negligible disadvantages. After working with patients with myelomeningocele for 30 years, I thought about how we currently treat them. There are ethical, organizational, neurological, obstetrical, and postnatal aspects worth discussing.
Similar content being viewed by others
Data availability
No datasets were generated or analyzed during the current study.
References
Adzick NS, Thom EA, Spong CY, Brock JW, Burrows PK, Johnson MP, Howell LJ, Farrell JA, Dabrowiak ME, Sutton LN, Gupta N, Tulipan NB, D’Alton ME, Farmer DL (2011) A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med 364:993–1004. https://doi.org/10.1056/NEJMoa1014379
Danzer E, Adzick NS, Rintoul NE, Zarnow DM, Schwartz ES, Melchionni J, Ernst LM, Flake AW, Sutton LN, Johnson MP (2008) Intradural inclusion cysts following in utero closure of myelomeningocele: clinical implications and follow-up findings. J Neurosurg Pediatr 2:406–413. https://doi.org/10.3171/PED.2008.2.12.406
Dewan MC, Wellons JC (2019) Fetal surgery for spina bifida. J Neurosurg Pediatr 24:105–114. https://doi.org/10.3171/2019.4.PEDS18383
Houtrow AJ, MacPherson C, Jackson-Coty J, Rivera M, Flynn L, Burrows PK, Adzick NS, Fletcher J, Gupta N, Howell LJ, Brock JW III, Lee H, Walker WO, Thom EA (2021) Prenatal repair and physical functioning among children with myelomeningocele: a secondary analysis of a randomized clinical trial. JAMA Pediatr 175(4):e205674. https://doi.org/10.1001/jamapediatrics.2020.5674
Moldenhauer JS, Soni S, Rintoul NE, Spinner SS, Khalek N, Martinez-Poyer J, Flake AW, Hedrick HL, Peranteau WH, Rendon N, Koh J, Howell LJ, Heuer GG, Sutton LN, Johnson MP, Adzick NS (2015) Fetal myelomeningocele repair: the post-MOMS experience at the Children’s Hospital of Philadelphia. Fetal Diagn Ther 37:235–240. https://doi.org/10.1159/000365353
Pontell ME, Yengo-Kahn AM, Taylor E, Kane M, Newton M, Bennett KA, Wellons JC, Braun SA (2023) Intrauterine closure of myelomeningocele defects with primary linear repair versus bipedicle fasciocutaneous flaps: a post-MOMS cohort study with long-term follow-up. J Neurosurg Pediatr 31:143–150. https://doi.org/10.3171/2022.10.PEDS22357
Sacco A, Ushakov F, Thompson D, Peebles D, Pandya P, De Coppi P, Wimalasundera R, Attilakos G, David AL, Deprest J (2019) Fetal surgery for open spina bifida. Obstet Gynaecol 21:271–282. https://doi.org/10.1111/tog.12603
Sival DA, Patuszka A, Koszutski T, Heep A, Verbeek RJ (2023) Neurologic outcome comparison between fetal open-, endoscopic- and neonatal-intervention techniques in spina bifida aperta. Diagnostics (Basel) 13:251. https://doi.org/10.3390/diagnostics13020251
Deprest J, Toelen J, Debyser Z, Rodrigues C, Devlieger R, De Catte L, Lewi L, Van Mieghem T, Naulaers G, Vandevelde M, Claus F, Dierickx K (2011) The fetal patient – ethical aspects of fetal therapy. Facts Views Vis Obgyn 3:221–227. PMID: 24753868; PMCID: PMC3991449
Beuriat PA, Poirot I, Hameury F, Demede D, Sweeney KJ, Szathmari A, Di Rocco F, Mottolese C (2019) Low level myelomeningoceles: do they need prenatal surgery? Childs Nerv Syst 35:957–963. https://doi.org/10.1007/s00381-019-04123-1
Bowman RM, Lee JY, Yang J, Kim KH, Wang KC (2023) Myelomeningocele: the evolution of care over the last 50 years. Child Nerv Syst 39:2829–2845. https://doi.org/10.1007/s00381-023-06057-1
Stiefel D, Copp AJ, Meuli M (2007) Fetal spina bifida in a mouse model: loss of neural function in utero. J Neurosurg 106:213–221. https://doi.org/10.3171/ped.2007.106.3.213
Sanz Cortes M, Chmait RH, Lapa DA, Belfort MA, Carreras E, Miller JL, Brawura Biskupski Samaha R, Sepulveda Gonzalez G, Gielchinsky Y, Yamamoto M, Persico N, Santorum M, Otaño L, Nicolaou E, Yinon Y, Faig-Leite F, Brandt R, Whitehead W, Maiz N, Baschat A, Kosinski P, Nieto-Sanjuanero A, Chu J, Kershenovich A, Nicolaides KH (2021) Experience of 300 cases of prenatal fetoscopic open spina bifida repair: report of the International Fetoscopic Neural Tube Defect Repair Consortium. Am J Obstet Gynecol 225:678.e1-678.e11. https://doi.org/10.1016/j.ajog.2021.05.044
Guilbaud L, Maurice P, Lallemant P, De Saint-Denis T, Maisonneuve E, Dhombres F, Friszer S, Di Rocco F, Garel C, Moutard ML, Lachtar MA, Rigouzzo A, Forin V, Zérah M, Jouannic JM (2021) Open fetal surgery for myelomeningocele repair in France. J Gynecol Obstet Hum Reprod 50:102155. https://doi.org/10.1016/j.jogoh.2021.102155
North T, Cheong A, Steinbok P, Radic JA (2018) Trends in incidence and long-term outcomes of myelomeningocele in British Columbia. Childs Nerv Syst 34:717–724. https://doi.org/10.1007/s00381-017-3685-6
Talamonti G, D’Aliberti G, Collice M (2007) Myelomeningocele: long-term neurosurgical treatment and follow-up in 202 patients. J Neurosurg 107:368–386. https://doi.org/10.3171/PED-07/11/368
Jackson EM, Schwartz DM, Sestokas AK, Zarnow DM, Adzick NS, Johnson MP, Heuer GG, Sutton LN (2014) Intraoperative neurophysiological monitoring in patients undergoing tethered cord surgery after fetal myelomeningocele repair. J Neurosurg Pediatrics 13:355–361. https://doi.org/10.3171/2014.1.PEDS11336
Weaver KJ, McDowell MM, White MD, Tempel ZJ, Zwagerman NT, Deibert CP, Bonfield CM, Johnson S, Greene S (2021) Comparison of follow-up length-matched single-center myelomeningocele postnatal closure cohort to the Management of Myelomeningocele Study (MOMS) trial results. Pediatr Neurosurg 56:229–238. https://doi.org/10.1159/000515038
Micu R, Chicea AL, Bratu DG, Nita P, Nemeti G, Chicea R (2018) Ultrasound and magnetic resonance imaging in the pre-natal diagnosis of open spina bifida. Med Ultrason 20:221–227. https://doi.org/10.11152/mu-1325
Brock JW, Thomas JC, Baskin LS, Zderic SA, Thom EA, Burrows PK, Lee H, Houtrow AJ, MacPherson C, Adzick NS et al (2019) Effect of prenatal repair of myelomeningocele on urological outcomes at school age. J Urol 202:812–818. https://doi.org/10.1097/JU.0000000000000334
Horst M, Mazzone L, Schraner T, Bodmer C, Möhrlen U, Meuli M, Gobet R (2017) Prenatal myelomeningocele repair: do bladders better? Neurourol Urodyn 36:1651–1658. https://doi.org/10.1002/nau.23174
Chakraborty A, Crimmins D, Hayward R, Thompson D (2008) Toward reducing shunt placement rates in patients with myelomeningocele. J Neurosurg Pediatr 1:361–365. https://doi.org/10.3171/PED/2008/1/5/361
Hudgins RJ, Gilreath CL (2004) Tethered spinal cord following repair of myelomeningocele. Neurosurg Focus 16(2):E7. https://doi.org/10.3171/foc.2004.16.2.8
Flanders TM, Franco AJ, Lincul KL, Pierce SR, Oliver ER, Moldenhauer JS, Adzick NS, Heuer GG (2023) Tethered cord release in patients after open fetal myelomeningocele closure: intraoperative neuromonitoring data and patient outcomes. Childs Nerv Syst 39:663–670. https://doi.org/10.1007/s00381-022-05756-5
Talamonti G, Marcati E, Mastino L, Meccariello G, Picano M, D’Aliberti G (2020) Surgical management of Chiari malformation type II. Childs Nerv Syst 36:1621–1634. https://doi.org/10.1007/s00381-020-04675-7
Tennant PW, Pearce MS, Bythell M, Rankin J (2010) 20-year survival of children born with congenital anomalies: a population-based study. Lancet 375:649–656. https://doi.org/10.1016/S0140-6736(09)61922-X
Bennett KA, Carroll MA, Shannon CN, Braun SA, Dabrowiak ME, Crum AK, Paschall RL, Kavanaugh-McHugh AL, Wellons JC, Tulipan NB (2014) Reducing perinatal complications and preterm delivery for patients undergoing in utero closure of fetal myelomeningocele: further modifications to the multidisciplinary surgical technique. J Neurosurg Pediatr 14:108–114. https://doi.org/10.3171/2014.3.PEDS13266
Tulipan N, Bruner JP (1998) Myelomeningocele repair in utero: a report of three cases. Pediatr Neurosurg 28:177–180. https://doi.org/10.1159/000028645
Author information
Authors and Affiliations
Contributions
G.T. is the only author of this paper.
Corresponding author
Ethics declarations
Ethical approval
This paper does not require the approval of the ethics committee.
Conflict of interest
I, as the author, hereby declare that I have no financial or non-financial vested interests that are directly or indirectly connected with the present paper. No funds, grants, or other support was received. I am a member of the Editorial Board.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.
About this article
Cite this article
Talamonti, G. Reflections upon the intrauterine repair of myelomeningocele. Childs Nerv Syst 40, 1571–1575 (2024). https://doi.org/10.1007/s00381-024-06365-0
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00381-024-06365-0