Impact of the volume of the myelomeningocele sac on imaging, prenatal neurosurgery and motor outcomes: a retrospective cohort study

Romain Corroenne, Amy R Mehollin-Ray, Rebecca M Johnson, William E Whitehead, Jimmy Espinoza, Jonathan Castillo, Heidi Castillo, Gunes Orman, Roopali Donepudi, Thierry A G M Huisman, Ahmed A Nassr, Michael A Belfort, Magdalena Sanz Cortes, Alireza A Shamshirsaz, Romain Corroenne, Amy R Mehollin-Ray, Rebecca M Johnson, William E Whitehead, Jimmy Espinoza, Jonathan Castillo, Heidi Castillo, Gunes Orman, Roopali Donepudi, Thierry A G M Huisman, Ahmed A Nassr, Michael A Belfort, Magdalena Sanz Cortes, Alireza A Shamshirsaz

Abstract

To investigate the association of the myelomeningocele (MMC) volume with prenatal and postnatal motor function (MF) in cases who underwent a prenatal repair. Retrospective cohort study (11/2011 to 03/2019) of 63 patients who underwent a prenatal MMC repair (37 fetoscopic, 26 open-hysterotomy). At referral, measurements of the volume of MMC was performed based on ultrasound scans. A large MMC was defined as greater than the optimal volume threshold (ROC analysis) for the prediction of intact MF at referral (2.7 cc). Prenatal or postnatal intact motor function (S1) was defined as the observation of plantar flexion of the ankle based on ultrasound scan or postnatal examination. 23/63 participants presented a large MMC. Large MMC lesions was associated with an increased risk of having clubfeet by 9.5 times (CI%95[2.1-41.8], p < 0.01), and reduces the chances of having an intact MF at referral by 0.19 times (CI%95[0.1-0.6], p < 0.01). At birth, a large MMC reduces the chance of having an intact MF by 0.09 times (CI%95[0.01-0.49], p < 0.01), and increases the risk of having clubfeet by 3.7 times (CI%95[0.8-18.3], p = 0.11). A lower proportion of intact MF and a higher proportion of clubfeet pre- or postnatally were observed in cases with a large MMC sac who underwent a prenatal repair.Trial registration: Clinicaltrials.gov NCT02230072 and NCT03794011 registered on September 3rd, 2014 and January 4th, 2019.

Conflict of interest statement

The authors declare no competing interests.

Figures

Figure 1
Figure 1
Volume of the myelomeningocele: ultrasound measurements. (A) Sagittal view of a myelomeningocele at 20 weeks of gestation: length. (B) Transversal view of a myelomeningocele at 20 weeks of gestation: width and depth. (C) Volume of the myelomeningocele: prolate ellipsoid volume: length × width × depth × 0.52.

References

    1. Parker SE, et al. Updated national birth prevalence estimates for selected birth defects in the United States, 2004–2006. Birth Defects Res. Part A Clin. Mol. Teratol. 2010;88:1008–1016. doi: 10.1002/bdra.20735.
    1. Bowman RM, McLone DG, Grant JA, Tomita T, Ito JA. Spina bifida outcome: A 25-year prospective. Pediatr. Neurosurg. 2001;34:114–120. doi: 10.1159/000056005.
    1. Shin M, et al. Improved survival among children with spina bifida in the United States. J. Pediatr. 2012;161:1132–1137.e3. doi: 10.1016/j.jpeds.2012.05.040.
    1. Heffez DS, Aryanpur J, Hutchins GM, Freeman JM. The paralysis associated with myelomeningocele: Clinical and experimental data implicating a preventable spinal cord injury. Neurosurgery. 1990;26:987–992. doi: 10.1227/00006123-199006000-00011.
    1. Wasserman RM, Holmbeck GN. Profiles of neuropsychological functioning in children and adolescents with spina bifida: Associations with biopsychosocial predictors and functional outcomes. J. Int. Neuropsychol. Soc. 2016;22:804–815. doi: 10.1017/S1355617716000680.
    1. Dias MS. Neurosurgical management of myelomeningocele (spina bifida) Pediatr. Rev. 2005;26:50–60. doi: 10.1542/pir.26-2-50.
    1. Al-Shanafey SN, et al. Reduction in neural injury with earlier delivery in a mouse model of congenital myelomeningocele: Laboratory investigation. J. Neurosurg. Pediatr. 2013;12:390–394. doi: 10.3171/2013.7.PEDS1351.
    1. Hunt GM, Oakeshott P. Outcome in people with open spina bifida at age 35: Prospective community based cohort study. BMJ. 2003;326:1365–1366. doi: 10.1136/bmj.326.7403.1365.
    1. Adzick NS, et al. A randomized trial of prenatal versus postnatal repair of myelomeningocele. N. Engl. J. Med. 2011;364:993–1004. doi: 10.1056/NEJMoa1014379.
    1. Oliver ER, et al. Myelomeningocele sac associated with worse lower extremity neurologic sequela: Evidence for prenatal neural stretch injury? Ultrasound Obstet. Gynecol. 2019 doi: 10.1002/uog.21891.
    1. Farmer DL, et al. The management of myelomeningocele study: Full cohort 30-month pediatric outcomes. Am. J. Obstet. Gynecol. 2018;218(256):e1–256.e13.
    1. Danzer E, et al. Lower extremity neuromotor function and short-term ambulatory potential following in utero myelomeningocele surgery. Fetal Diagn. Ther. 2009;25:47–53. doi: 10.1159/000197359.
    1. Wilson RD, et al. Does a myelomeningocele sac compared to no sac result in decreased postnatal leg function following maternal fetal surgery for spina bifida aperta? Fetal Diagn. Ther. 2007;22:348–351. doi: 10.1159/000103294.
    1. Nagaraj, U. D. et al. Myelomeningocele versus myelocele on fetal MR images: Are there differences in brain findings? In American Journal of Roentgenology, vol. 211, 1376–1380 (American Roentgen Ray Society, 2018).
    1. Sival DA, et al. Neonatal loss of motor function in human spina bifida aperta. Pediatrics. 2004;114:427–434. doi: 10.1542/peds.114.2.427.
    1. Sival DA, et al. Spinal hemorrhages are associated with early neonatal motor function loss in human spina bifida aperta. Early Hum. Dev. 2008;84:423–431. doi: 10.1016/j.earlhumdev.2007.11.003.
    1. Michejda M. Intrauterine treatment of spina bifida: Primate model. Z. Kinderchir. 1984;39:259–261.
    1. Heffez DS, Aryanpur J, Rotellini NA, Hutchins GM, Freeman JM. Intrauterine repair of experimental surgically created dysraphism. Neurosurgery. 1993;32:1005–1010. doi: 10.1227/00006123-199306000-00021.
    1. Fetoscopic Meningomyelocele Repair Study (fMMC). NCT02230072 (2014).
    1. Belfort MA, et al. Fetoscopic open neural tube defect repair: Development and refinement of a two-port, carbon dioxide insufflation technique. Obstet. Gynecol. 2017 doi: 10.1097/AOG.0000000000001941.
    1. Belfort MA, et al. Comparison of two fetoscopic open neural tube defect (ONTD) repair techniques: Single-layer vs three-layer closure. Ultrasound Obstet. Gynecol. 2019 doi: 10.1002/uog.21915.
    1. Sutton LN, et al. Improvement in hindbrain herniation demonstrated by serial fetal magnetic resonance imaging following fetal surgery for myelomeningocele. J. Am. Med. Assoc. 1999;282:1826–1831. doi: 10.1001/jama.282.19.1826.
    1. Danzer E, et al. Long-term neurofunctional outcome, executive functioning, and behavioral adaptive skills following fetal myelomeningocele surgery. Am. J. Obstet. Gynecol. 2016;214(269):e1–269.e8.

Source: PubMed

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