Meningomyelocele

Mitali Sahni, Mahdi Alsaleem, Abhinav Ohri, Mitali Sahni, Mahdi Alsaleem, Abhinav Ohri

Excerpt

Meningomyelocele or myelomeningocele, commonly known as open spina bifida is a devastating congenital malformation of the central nervous system and is associated with significant morbidity and mortality. Neural tube defects are of two types:

  1. Open neural tube defect means that the defect is either not covered at all or just covered by a membrane. It comprises about 80% of all neural tube defects.

  2. Closed neural tube defect: No apparent defect externally, usually result from a defect either in the fat, bone, or membranes covering the spinal cord.

Myelomeningocele is the most common open neural tube defect. It is characterized by failure of the neural tube to close in the lumbosacral region during embryonic development (fourth-week post-fertilization), leading to the herniation of the meninges and spinal cord through a vertebral defect. The neural tube fusion starts at the level of the hindbrain (medulla and pons) and progresses rostrally and caudally. Incomplete fusion caudally leads to the formation of meningomyelocele around day 26 of gestation.

Copyright © 2023, StatPearls Publishing LLC.

References

    1. Copp AJ, Adzick NS, Chitty LS, Fletcher JM, Holmbeck GN, Shaw GM. Spina bifida. Nat Rev Dis Primers. 2015 Apr 30;1:15007.
    1. Brody BA, Kinney HC, Kloman AS, Gilles FH. Sequence of central nervous system myelination in human infancy. I. An autopsy study of myelination. J Neuropathol Exp Neurol. 1987 May;46(3):283-301.
    1. Sepulveda W, Corral E, Ayala C, Be C, Gutierrez J, Vasquez P. Chromosomal abnormalities in fetuses with open neural tube defects: prenatal identification with ultrasound. Ultrasound Obstet Gynecol. 2004 Apr;23(4):352-6.
    1. Au KS, Ashley-Koch A, Northrup H. Epidemiologic and genetic aspects of spina bifida and other neural tube defects. Dev Disabil Res Rev. 2010;16(1):6-15.
    1. Shimoji K, Kimura T, Kondo A, Tange Y, Miyajima M, Arai H. Genetic studies of myelomeningocele. Childs Nerv Syst. 2013 Sep;29(9):1417-25.
    1. Zaganjor I, Sekkarie A, Tsang BL, Williams J, Razzaghi H, Mulinare J, Sniezek JE, Cannon MJ, Rosenthal J. Describing the Prevalence of Neural Tube Defects Worldwide: A Systematic Literature Review. PLoS One. 2016;11(4):e0151586.
    1. Papp C, Adám Z, Tóth-Pál E, Török O, Váradi V, Papp Z. Risk of recurrence of craniospinal anomalies. J Matern Fetal Med. 1997 Jan-Feb;6(1):53-7.
    1. Greene ND, Massa V, Copp AJ. Understanding the causes and prevention of neural tube defects: Insights from the splotch mouse model. Birth Defects Res A Clin Mol Teratol. 2009 Apr;85(4):322-30.
    1. Avagliano L, Massa V, George TM, Qureshy S, Bulfamante GP, Finnell RH. Overview on neural tube defects: From development to physical characteristics. Birth Defects Res. 2019 Nov 15;111(19):1455-1467.
    1. Williams EN, Broughton NS, Menelaus MB. Age-related walking in children with spina bifida. Dev Med Child Neurol. 1999 Jul;41(7):446-9.
    1. Naidich TP, McLone DG, Fulling KH. The Chiari II malformation: Part IV. The hindbrain deformity. Neuroradiology. 1983;25(4):179-97.
    1. Nagler J, Levy JA, Bachur RG. Stridor in an infant with myelomeningocele. Pediatr Emerg Care. 2007 Jul;23(7):478-81.
    1. Holinger PC, Holinger LD, Reichert TJ, Holinger PH. Respiratory obstruction and apnea in infants with bilateral abductor vocal cord paralysis, meningomyelocele, hydrocephalus, and Arnold-Chiari malformation. J Pediatr. 1978 Mar;92(3):368-73.
    1. Shaer CM, Chescheir N, Schulkin J. Myelomeningocele: a review of the epidemiology, genetics, risk factors for conception, prenatal diagnosis, and prognosis for affected individuals. Obstet Gynecol Surv. 2007 Jul;62(7):471-9.
    1. Danzer E, Flake AW. In utero Repair of Myelomeningocele: Rationale, Initial Clinical Experience and a Randomized Controlled Prospective Clinical Trial. Neuroembryology Aging. 2008 Mar;4(4):165-174.
    1. 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, MOMS Investigators A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med. 2011 Mar 17;364(11):993-1004.
    1. Rendeli C, Nucera E, Ausili E, Tabacco F, Roncallo C, Pollastrini E, Scorzoni M, Schiavino D, Caldarelli M, Pietrini D, Patriarca G. Latex sensitisation and allergy in children with myelomeningocele. Childs Nerv Syst. 2006 Jan;22(1):28-32.
    1. McLone DG. Care of the neonate with a myelomeningocele. Neurosurg Clin N Am. 1998 Jan;9(1):111-20.
    1. Messing-Jünger M, Röhrig A. Primary and secondary management of the Chiari II malformation in children with myelomeningocele. Childs Nerv Syst. 2013 Sep;29(9):1553-62.
    1. Hopson B, Rocque BG, Joseph DB, Powell D, McLain ABJ, Davis RD, Wilson TS, Conklin MJ, Blount JP. The development of a lifetime care model in comprehensive spina bifida care. J Pediatr Rehabil Med. 2018;11(4):323-334.
    1. Wilson RD, Lemerand K, Johnson MP, Flake AW, Bebbington M, Hedrick HL, Adzick NS. Reproductive outcomes in subsequent pregnancies after a pregnancy complicated by open maternal-fetal surgery (1996-2007). Am J Obstet Gynecol. 2010 Sep;203(3):209.e1-6.
    1. Chen CP. Prenatal diagnosis, fetal surgery, recurrence risk and differential diagnosis of neural tube defects. Taiwan J Obstet Gynecol. 2008 Sep;47(3):283-90.
    1. Mummareddy N, Dewan MC, Mercier MR, Naftel RP, Wellons JC, Bonfield CM. Scoliosis in myelomeningocele: epidemiology, management, and functional outcome. J Neurosurg Pediatr. 2017 Jul;20(1):99-108.
    1. Beuriat PA, Poirot I, Hameury F, Szathmari A, Rousselle C, Sabatier I, di Rocco F, Mottolese C. Postnatal Management of Myelomeningocele: Outcome with a Multidisciplinary Team Experience. World Neurosurg. 2018 Feb;110:e24-e31.

Source: PubMed

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