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
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
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
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
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
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
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
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
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
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
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
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
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
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
Adzick NS (2013) Prospects for fetal surgery. Early Hum Dev 89:881–886. https://doi.org/10.1016/j.earlhumdev.2013.09.010
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
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
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
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
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
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
Hoffer MM, Feiwell E, Perry R et al (1973) Functional ambulation in patients with myelomeningocele. J Bone Joint Surg Am 55:137–148
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
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
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
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
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
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
Holmes NM, Nguyen HT, Harrison MR et al (2001) Fetal intervention for myelomeningocele: effect on postnatal bladder function. J Urol 166:2383–2386
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
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
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
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
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
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
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
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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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DOI: https://doi.org/10.1007/s00381-019-04123-1