Low level myelomeningoceles: do they need prenatal surgery?

  • Pierre-Aurelien Beuriat
  • Isabelle Poirot
  • Frederic Hameury
  • Delphine Demede
  • Kieron J. Sweeney
  • Alexandru Szathmari
  • Federico Di Rocco
  • Carmine MottoleseEmail author
Original Article



Postnatal closure of a myelomeningocele remains the standard of care in many countries. The prenatal closure has given hope for decreasing the damage to the neural placode and has challenged classic management. However, this technique presents potential sources of complications. Patients with MMC with an anatomical level of L4 and below have a better functional prognosis than higher level malformations. Are they still candidates for prenatal surgery?


To evaluate outcome of MMC with an anatomical level of L4 and below and discuss, with support of the literature, the indications to perform prenatal closure in this particular group of patients.

Materials and methods

Twenty-nine children were included in this observational study. The level of the vertebral malformation was sacral in 12 cases (41.4%) or lumbar (level ≤ L4) in 17 cases (58.6%). All the patients was operated postnatally for closure of the MMC with microsurgical technique as soon as possible after clinical evaluation (range 0–97 days).


Only 11 out of 29 patients (37.9%) needed of a CSF diversion. A Chiari II malformation was present before MMC closure in 17 patients (58.6%) and only in 5 (17%) after. Twenty-six patients (89.7%) were able to walk. Seven (23%) and 16 (55%) of our patients have a normal bladder and bowel control, respectively. All school-aged children attend school.


The functional outcome for low-level MMC is good when managed with modern microneurosurgical techniques with a low risk for the patient and the mother. Therefore, we do not suggest prenatal surgery for subgroup of infant with MM.


Spinal dysraphism Multidisciplinary management Pediatric Postnatal surgery Myelomeningocele Prenatal surgery 





Chiari type II malformation


Choroid plexus coagulation




Cerebro spinal fluid


Endoscopic third ventriculostomy


Gross motor function classification system






Management of myelomeningocele study


Magnetic resonance imaging


scan ultra-sound scan


Shunt ventriculo-peritoneal Shunt


Week of gestation



  1. 1.
    Beuriat P-A, Szathmari A, Hameury F, Poirot I, Massoud M, Massardier J, Mottolese C, di Rocco F (2017) Changes in the epidemiology of spina bifida in France in the last 30 years. Neurochirurgie 63:109–111. CrossRefGoogle Scholar
  2. 2.
    Beuriat P-A, Poirot I, Hameury F, Szathmari A, Rousselle C, Sabatier I, di Rocco F, Mottolese C (2018) Postnatal management of myelomeningocele: outcome with a multidisciplinary team experience. World Neurosurg 110:e24–e31. CrossRefGoogle Scholar
  3. 3.
    Adzick NS, Thom EA, Spong CY, Brock JW 3rd, Burrows PK, Johnson MP, Howell LJ, Farrell JA, Dabrowiak ME, Sutton LN, Gupta N, Tulipan NB, D'Alton ME, Farmer DL, MOMS Investigators (2011) A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med 364:993–1004. CrossRefGoogle Scholar
  4. 4.
    Beuriat P-A, Szathmari A, Grassiot B, Plaisant F, Rousselle C, Mottolese C (2016) Role of endoscopic third ventriculostomy in the management of myelomeningocele-related hydrocephalus: a retrospective study in a single French institution. World Neurosurg 87:484–493. CrossRefGoogle Scholar
  5. 5.
    Kabagambe SK, Jensen GW, Chen YJ, Vanover MA, Farmer DL (2018) Fetal surgery for myelomeningocele: a systematic review and meta-analysis of outcomes in fetoscopic versus open repair. Fetal Diagn Ther 43:161–174. CrossRefGoogle Scholar
  6. 6.
    Werner EF, Han CS, Burd I, Lipkind HS, Copel JA, Bahtiyar MO, Thung SF (2012) Evaluating the cost-effectiveness of prenatal surgery for myelomeningocele: a decision analysis. Ultrasound Obstet Gynecol 40:158–164. CrossRefGoogle Scholar
  7. 7.
    Johnson MP, Bennett KA, Rand L, Burrows PK, Thom EA, Howell LJ, Farrell JA, Dabrowiak ME, Brock JW 3rd, Farmer DL, Adzick NS, Management of Myelomeningocele Study Investigators (2016) The management of myelomeningocele study: obstetrical outcomes and risk factors for obstetrical complications following prenatal surgery. Am J Obstet Gynecol 215:778.e1–778.e9. CrossRefGoogle Scholar
  8. 8.
    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. CrossRefGoogle Scholar
  9. 9.
    Belfort MA, Whitehead WE, Shamshirsaz AA, Bateni ZH, Olutoye OO, Olutoye OA, Mann DG, Espinoza J, Williams E, Lee TC, Keswani SG, Ayres N, Cassady CI, Mehollin-Ray AR, Sanz Cortes M, Carreras E, Peiro JL, Ruano R, Cass DL (2017) Fetoscopic open neural tube defect repair: development and refinement of a two-port, carbon dioxide insufflation technique. Obstet Gynecol 129:734–743. CrossRefGoogle Scholar
  10. 10.
    Greene S, Lee PS, Deibert CP, Tempel ZJ, Zwagerman NT, Florio K, Bonfield CM, Emery SP (2016) The impact of mode of delivery on infant neurologic outcomes in myelomeningocele. Am J Obstet Gynecol 215:495.e1–495.e11. CrossRefGoogle Scholar
  11. 11.
    Pedreira DAL, Zanon N, Nishikuni K, Moreira de Sá RA, Acacio GL, Chmait RH, Kontopoulos EV, Quintero RA (2016) Endoscopic surgery for the antenatal treatment of myelomeningocele: the CECAM trial. Am J Obstet Gynecol 214:111.e1–111.e11. CrossRefGoogle Scholar
  12. 12.
    Graf K, Kohl T, Neubauer BA, Dey F, Faas D, Wanis FA, Reinges MHT, Uhl E, Kolodziej MA (2016) Percutaneous minimally invasive fetoscopic surgery for spina bifida aperta. Part III: neurosurgical intervention in the first postnatal year. Ultrasound Obstet Gynecol 47:158–161. CrossRefGoogle Scholar
  13. 13.
    Zamłyński J, Olejek A, Koszutski T, Ziomek G, Horzelska E, Gajewska-Kucharek A, Maruniak-Chudek I, Herman-Sucharska I, Kluczewska E, Horak S, Bodzek P, Zamłyński M, Kowalik J, Horzelski T, Bohosiewicz J (2014) Comparison of prenatal and postnatal treatments of spina bifida in Poland--a non-randomized, single-center study. J Matern Fetal Neonatal Med 27:1409–1417. CrossRefGoogle Scholar
  14. 14.
    Adzick NS (2013) Prospects for fetal surgery. Early Hum Dev 89:881–886. CrossRefGoogle Scholar
  15. 15.
    Araujo E, Tonni G, Martins WP (2016) Outcomes of infants followed-up at least 12 months after fetal open and endoscopic surgery for meningomyelocele: a systematic review and meta-analysis. J Evid-Based Med 9:125–135. CrossRefGoogle Scholar
  16. 16.
    Danzer E, Finkel RS, Rintoul NE, Bebbington MW, Schwartz ES, Zarnow DM, Adzick NS, Johnson MP (2008) Reversal of hindbrain herniation after maternal-fetal surgery for myelomeningocele subsequently impacts on brain stem function. Neuropediatrics 39:359–362. CrossRefGoogle Scholar
  17. 17.
    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. CrossRefGoogle Scholar
  18. 18.
    Beuriat PA, Szathmari A, Rousselle C, Sabatier I, di Rocco F, Mottolese C (2017) Complete reversibility of the Chiari TYPE II malformation following post natal repair of myelomeningocele. World Neurosurg 108:62–68. CrossRefGoogle Scholar
  19. 19.
    Danzer E, Thomas NH, Thomas A, Friedman KB, Gerdes M, Koh J, Adzick NS, Johnson MP (2016) Long-term neurofunctional outcome, executive functioning, and behavioral adaptive skills following fetal myelomeningocele surgery. Am J Obstet Gynecol 214:269.e1–269.e8. CrossRefGoogle Scholar
  20. 20.
    Mazur JM, Shurtleff D, Menelaus M, Colliver J (1989) Orthopaedic management of high-level spina bifida. Early walking compared with early use of a wheelchair. J Bone Joint Surg Am 71:56–61CrossRefGoogle Scholar
  21. 21.
    Hoffer MM, Feiwell E, Perry R et al (1973) Functional ambulation in patients with myelomeningocele. J Bone Joint Surg Am 55:137–148CrossRefGoogle Scholar
  22. 22.
    Brinker MR, Rosenfeld SR, Feiwell E, Granger SP, Mitchell DC, Rice JC (1994) Myelomeningocele at the sacral level. Long-term outcomes in adults. J Bone Joint Surg Am 76:1293–1300CrossRefGoogle Scholar
  23. 23.
    Dicianno BE, Karmarkar A, Houtrow A, Crytzer TM, Cushanick KM, McCoy A, Wilson P, Chinarian J, Neufeld J, Smith K, Collins DM (2015) Factors associated with mobility outcomes in a National Spina Bifida Patient Registry. Am J Phys Med Rehabil 94:1015–1025. CrossRefGoogle Scholar
  24. 24.
    Verbeek RJ, Heep A, Maurits NM et al (2012) Fetal endoscopic myelomeningocele closure preserves segmental neurological function. Dev Med Child Neurol 54:15–22. CrossRefGoogle Scholar
  25. 25.
    de Jong TPVM, Chrzan R, Klijn AJ, Dik P (2008) Treatment of the neurogenic bladder in spina bifida. Pediatr Nephrol Berl Ger 23:889–896. CrossRefGoogle Scholar
  26. 26.
    Brock JW, Carr MC, Adzick NS, Burrows PK, Thomas JC, Thom EA, Howell LJ, Farrell JA, Dabrowiak ME, Farmer DL, Cheng EY, Kropp BP, Caldamone AA, Bulas DI, Tolivaisa S, Baskin LS, for the MOMS Investigators (2015) Bladder function after fetal surgery for myelomeningocele. Pediatrics 136:e906–e913. CrossRefGoogle Scholar
  27. 27.
    Holzbeierlein J, Pope JC IV, Adams MC et al (2000) The urodynamic profile of myelodysplasia in childhood with spinal closure during gestation. J Urol 164:1336–1339CrossRefGoogle Scholar
  28. 28.
    Holmes NM, Nguyen HT, Harrison MR et al (2001) Fetal intervention for myelomeningocele: effect on postnatal bladder function. J Urol 166:2383–2386CrossRefGoogle Scholar
  29. 29.
    Koh CJ, DeFilippo RE, Borer JG et al (2006) Bladder and external urethral sphincter function after prenatal closure of myelomeningocele. J Urol 176:2232–2236. CrossRefGoogle Scholar
  30. 30.
    Clayton DB, Tanaka ST, Trusler L, Thomas JC, Pope JC, Adams MC, Brock JW (2011) Long-term urological impact of fetal myelomeningocele closure. J Urol 186:1581–1585. CrossRefGoogle Scholar
  31. 31.
    Lee NG, Gomez P, Uberoi V, Kokorowski PJ, Khoshbin S, Bauer SB, Estrada CR (2012) In utero closure of myelomeningocele does not improve lower urinary tract function. J Urol 188:1567–1571. CrossRefGoogle Scholar
  32. 32.
    Macedo A, Leal M, Rondon A et al (2015) Urological evaluation of patients that had undergone in utero myelomeningocele closure: a prospective assessment at first presentation and early follow-up. Do their bladder benefit from it? Neurourol Urodyn 34:461–464. CrossRefGoogle Scholar
  33. 33.
    Leal da Cruz M, Liguori R, Garrone G, Leslie B, Ottoni SL, Carvalheiro S, Moron AF, Ortiz V, Macedo A (2015) Categorization of bladder dynamics and treatment after fetal myelomeningocele repair: first 50 cases prospectively assessed. J Urol 193:1808–1811. CrossRefGoogle Scholar
  34. 34.
    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. CrossRefGoogle Scholar
  35. 35.
    Huang GO, Belfort MA, Whitehead WE, Olutoye OO, Castillo J, Castillo H, Ostermaier KK, Koh CJ, Tu DD (2017) Early postnatal bladder function in fetoscopic myelomeningocele repair patients. J Pediatr Rehabil Med 10:327–333. CrossRefGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  • Pierre-Aurelien Beuriat
    • 1
  • Isabelle Poirot
    • 2
  • Frederic Hameury
    • 3
  • Delphine Demede
    • 3
  • Kieron J. Sweeney
    • 1
  • Alexandru Szathmari
    • 1
  • Federico Di Rocco
    • 1
  • Carmine Mottolese
    • 1
    Email author
  1. 1.Department of Pediatric NeurosurgeryHôpital Femme Mère EnfantLyon CedexFrance
  2. 2.Department of Pediatric Rehabilitation l’ESCALEHôpital Femme Mère EnfantLyon CedexFrance
  3. 3.Department of Urologic SurgeryHôpital Femme Mère EnfantLyon CedexFrance

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