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Low level myelomeningoceles: do they need prenatal surgery?

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Abstract

Background

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?

Objective

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).

Results

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.

Conclusions

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.

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Abbreviations

CM:

centimeter

CM-II:

Chiari type II malformation

CPC:

Choroid plexus coagulation

CS:

Cesarean-section

CSF:

Cerebro spinal fluid

ETV:

Endoscopic third ventriculostomy

GMF-CS:

Gross motor function classification system

Gr:

grams

MMC:

Myelomeningocele

MOMS:

Management of myelomeningocele study

MRI:

Magnetic resonance imaging

US:

scan ultra-sound scan

VP:

Shunt ventriculo-peritoneal Shunt

WG:

Week of gestation

References

  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. https://doi.org/10.1016/j.neuchi.2017.01.003

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1016/j.wneu.2017.09.169

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1056/NEJMoa1014379

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  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. https://doi.org/10.1016/j.wneu.2015.07.071

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1159/000479505

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1002/uog.11176

    Article  CAS  PubMed  Google Scholar 

  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. https://doi.org/10.1016/j.ajog.2016.07.052

    Article  Google Scholar 

  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. https://doi.org/10.3171/2014.3.PEDS13266

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1097/AOG.0000000000001941

    Article  CAS  PubMed  Google Scholar 

  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. https://doi.org/10.1016/j.ajog.2016.05.028

    Article  Google Scholar 

  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. https://doi.org/10.1016/j.ajog.2015.09.065

    Article  Google Scholar 

  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. https://doi.org/10.1002/uog.14937

    Article  CAS  PubMed  Google Scholar 

  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. https://doi.org/10.3109/14767058.2013.858689

    Article  PubMed  Google Scholar 

  14. Adzick NS (2013) Prospects for fetal surgery. Early Hum Dev 89:881–886. https://doi.org/10.1016/j.earlhumdev.2013.09.010

    Article  PubMed  PubMed Central  Google Scholar 

  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. https://doi.org/10.1111/jebm.12207

    Article  Google Scholar 

  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. https://doi.org/10.1055/s-0029-1202835

    Article  CAS  PubMed  Google Scholar 

  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. https://doi.org/10.1159/000365353

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1016/j.wneu.2017.08.152

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1016/j.ajog.2015.09.094

    Article  Google Scholar 

  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 71:56–61

    Article  CAS  PubMed  Google Scholar 

  21. Hoffer MM, Feiwell E, Perry R et al (1973) Functional ambulation in patients with myelomeningocele. J Bone Joint Surg Am 55:137–148

    Article  CAS  PubMed  Google Scholar 

  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 76:1293–1300

    Article  CAS  PubMed  Google Scholar 

  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. https://doi.org/10.1097/PHM.0000000000000404

    Article  PubMed  PubMed Central  Google Scholar 

  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. https://doi.org/10.1111/j.1469-8749.2011.04148.x

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1007/s00467-008-0780-7

    Article  Google Scholar 

  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. https://doi.org/10.1542/peds.2015-2114

    Article  PubMed  PubMed Central  Google Scholar 

  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 164:1336–1339

    Article  CAS  PubMed  Google Scholar 

  28. Holmes NM, Nguyen HT, Harrison MR et al (2001) Fetal intervention for myelomeningocele: effect on postnatal bladder function. J Urol 166:2383–2386

    Article  CAS  PubMed  Google Scholar 

  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. https://doi.org/10.1016/j.juro.2006.07.077

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1016/j.juro.2011.04.005

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1016/j.juro.2012.06.034

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1002/nau.22576

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1016/j.juro.2014.10.118

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1002/nau.23174

    Article  CAS  PubMed  Google Scholar 

  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. https://doi.org/10.3233/PRM-170465

    Article  Google Scholar 

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Authors and Affiliations

  1. Department of Pediatric Neurosurgery, Hôpital Femme Mère Enfant, 32 Avenue du Doyen Jean Lépine, 69677, Lyon Cedex, France

    Pierre-Aurelien Beuriat, Kieron J. Sweeney, Alexandru Szathmari, Federico Di Rocco & Carmine Mottolese

  2. Department of Pediatric Rehabilitation l’ESCALE, Hôpital Femme Mère Enfant, 32 Avenue du Doyen Jean Lépine, 69677, Lyon Cedex, France

    Isabelle Poirot

  3. Department of Urologic Surgery, Hôpital Femme Mère Enfant, 32 Avenue du Doyen Jean Lépine, 69677, Lyon Cedex, France

    Frederic Hameury & Delphine Demede

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  1. Pierre-Aurelien Beuriat
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Beuriat, PA., Poirot, I., Hameury, F. et al. Low level myelomeningoceles: do they need prenatal surgery?. Childs Nerv Syst 35, 957–963 (2019). https://doi.org/10.1007/s00381-019-04123-1

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