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Closed neural tube defects in children with caudal regression

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Abstract

Introduction

Infants born with caudal regression (CR) may have serious multisystem abnormalities that require prompt attention in the neonatal period. The presence of a closed neural tube defect (NTD) that can lead to future neurological deterioration may be overlooked.

Materials and methods

An IRB-approved retrospective review was conducted among patients with CR and a closed NTD that underwent neurosurgical operative intervention between 1996 and 2012 at a single institution.

Results

Twenty-two patients who met the above criteria were identified. Of this group, 13 were identified and surgically addressed in the first year of life; however, nine additional children were diagnosed with a closed NTD after a year of age with progressive neurological deterioration. Of the entire group, none had any cutaneous markers that are often seen with a closed NTD.

Conclusion

The frequent finding of a closed NTD associated with major CR abnormalities, even in the absence of any cutaneous markers for dysraphism, recommends that infants with CR undergo a MRI screening in early infancy to exclude the presence of a closed NTD.

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References

  1. Alexander E (1956) Agenesis of the sacrococcygeal region. J Neurosurg 13:507–513

    Article  Google Scholar 

  2. Andrish J, Kalamchi A, MacEwen GD (1979) Sacral agenesis: a clinical evaluation of its management, heredity, and associated anomalies. Clin Orthop Relat Res 139:52–57

    PubMed  Google Scholar 

  3. Banta JV, Nichols O (1969) Sacral agenesis. J Bone Joint Surg Am 51(4):693–703

    PubMed  CAS  Google Scholar 

  4. Bauer SB (2007) Neuropathic dysfunction of the lower urinary tract. In: Wein A, Kavoussi LR, Novick AC, Partin AW, Peters CA (eds) Campbell-Walsh urology, vol 4, 9th edn. Saunders, Philadelphia, pp 3644–3647

    Google Scholar 

  5. Caird MS, Hall JM, Bloom DA, Park JM, Farley FA (2007) Outcome study of children, adolescents, and adults with sacral agenesis. J Pediatr Orthop 27(6):682–685. doi:10.1097/01.bpo.0000190841.69412.eb

    Article  PubMed  Google Scholar 

  6. Emami-Naeini P, Rahbar Z, Nejat F, Kajbafzadeh A, El Khashab M (2010) Neurological presentations, imaging, and associated anomalies in 50 patients with sacral agenesis. Neurosurgery 67(4):894–900. doi:10.1227/NEU.0b013e3181eb500d, discussion 900

    Article  PubMed  Google Scholar 

  7. Hall DE, Udvarhelyi GB, Altman J (1981) Lumbosacral skin lesions as markers of occult spinal dysraphism. JAMA 246(22):2606–2608

    Article  PubMed  CAS  Google Scholar 

  8. Harlow CL, Partington MD, Thieme GA (1995) Lumbosacral agenesis: clinical characteristics, imaging, and embryogenesis. Pediatr Neurosurg 23(3):140–147

    Article  PubMed  CAS  Google Scholar 

  9. Kriss VM, Desai NS (1998) Occult spinal dysraphism in neonates: assessment of high-risk cutaneous stigmata on sonography. AJR Am J Roentgenol 171(6):1687–1692. doi:10.2214/ajr.171.6.9843314

    Article  PubMed  CAS  Google Scholar 

  10. Landauer W (1945) Rumplessness of chicken embryos produced by the injection of insulin and other chemicals. J Exp Zool 98:65–77

    Article  CAS  Google Scholar 

  11. Lehmann F (1935) Die Entwicklung von Ruchenmark. Spinalganglien und Wirbeanlagen in Chordalosen Korperregionen von Tritonlarver. Rev Suisse Zool 42:405–415

    Google Scholar 

  12. Muller F, O'Rahilly R (1987) The development of the human brain, the closure of the caudal neuropore, and the beginning of secondary neurulation at stage 12. Anat Embryol 176(4):413–430

    Article  PubMed  CAS  Google Scholar 

  13. Muthukumar N (1996) Surgical treatment of nonprogressive neurological deficits in children with sacral agenesis. Neurosurgery 38(6):1133–1137, discussion 1137–1138

    PubMed  CAS  Google Scholar 

  14. O'Neill BR, Yu AK, Tyler-Kabara EC (2010) Prevalence of tethered spinal cord in infants with VACTERL. J Neurosurg Pediatr 6(2):177–182. doi:10.3171/2010.5.peds09428

    Article  PubMed  Google Scholar 

  15. O'Neill OR, Piatt JH Jr, Mitchell P, Roman-Goldstein S (1995) Agenesis and dysgenesis of the sacrum: neurosurgical implications. Pediatr Neurosurg 22(1):20–28

    Article  PubMed  Google Scholar 

  16. Pang D (1993) Sacral agenesis and caudal spinal cord malformations. Neurosurgery 32(5):755–778, discussion 778–759

    Article  PubMed  CAS  Google Scholar 

  17. Pang D (1995) Caudal agenesis and dysembryogenesis of the caudal spinal cord. In: Pang D (ed) Disorders of the pediatric spine. Raven, New York, pp 277–308

    Google Scholar 

  18. Sardana K, Gupta R, Garg VK, Mishra D, Mishra P, Grover C, Mendiratta V (2009) A prospective study of cutaneous manifestations of spinal dysraphism from India. Pediatr Dermatol 26(6):688–695. doi:10.1111/j.1525-1470.2009.01014.x

    Article  PubMed  Google Scholar 

  19. Schropp C, Sorensen N, Collmann H, Krauss J (2006) Cutaneous lesions in occult spinal dysraphism—correlation with intraspinal findings. Child's Nerv Syst: ChNS: Off J Int Soc Pediatr Neurosurg 22(2):125–131. doi:10.1007/s00381-005-1150-4

    Article  CAS  Google Scholar 

  20. George TM, Adamson DC (2008) Normal and abnormal development of the nervous system. In: Leland Albright A, David Adelson P (eds) Principles and practice of pediatric neurosurgery, 2nd edn. Thieme, New York, pp 25–26

    Google Scholar 

  21. White RI, Klauber GT (1976) Sacral agenesis. Analysis of 22 cases. Urology 8(6):521–525

    Article  PubMed  CAS  Google Scholar 

  22. Zaw W, Stone DG (2002) Caudal regression syndrome in twin pregnancy with type II diabetes. J Perinatol: Off J Calif Perinat Assoc 22(2):171–174. doi:10.1038/sj.jp.7210614

    Article  Google Scholar 

  23. Zilva SS, Golding J, Drummond JC, Coward KH (1921) The relation of the fat-soluble factor to rickets and growth in pigs. Biochem J 15(3):427–437

    PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Division of Neurosurgery, Children’s Hospital Los Angeles, 1300 N. Vermont Avenue, Suite #1006, Los Angeles, CA, 90027, USA

    Yasser Jeelani, Gina M. Mosich & J. Gordon McComb

  2. Department of Neurological Surgery, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA

    Yasser Jeelani

Authors
  1. Yasser Jeelani
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  2. Gina M. Mosich
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  3. J. Gordon McComb
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Correspondence to Yasser Jeelani.

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Jeelani, Y., Mosich, G.M. & McComb, J.G. Closed neural tube defects in children with caudal regression. Childs Nerv Syst 29, 1451–1457 (2013). https://doi.org/10.1007/s00381-013-2119-3

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  • DOI: https://doi.org/10.1007/s00381-013-2119-3

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