DOI QR코드

DOI QR Code

Spinal Dysraphism in the Last Two Decades : What I Have Seen during the Era of Dynamic Advancement

  • Wang, Kyu-Chang (Division of Pediatric Neurosurgery, Seoul National University Children's Hospital)
  • Received : 2020.03.09
  • Accepted : 2020.04.02
  • Published : 2020.05.01

Abstract

Compared to any other decade, the last two decades have been the most dynamic period in terms of advances in the knowledge on spinal dysraphism. Among the several factors of rapid advancement, such as embryology during secondary neurulation and intraoperative neurophysiological monitoring, there is no doubt that Professor Dachling Pang stood high amidst the period. I review here the last two decades from my personal point of view on what has been achieved in the field of spinal dysraphism, focusing on occult tethered cord syndrome, lumbosacral lipomatous malformation, terminal myelocystocele, retained medullary cord, limited dorsal myeloschisis and junctional neural tube defect. There are still many issues to revise, add and extend. Profound knowledge of basic science is critical, as well as refined clinical analysis. I expect that young scholars who follow the footsteps of precedent giants will shed bright light on this topic in the future.

Keywords

References

  1. Beardmore HE, Wiglesworth FW : Vertebral anomalies and alimentary duplications; clinical and embryological aspects. Pediatr Clin North Am 5 : 457-474, 1958 https://doi.org/10.1016/s0031-3955(16)30661-7
  2. Bremer JL : Dorsal intestinal fistula; accessory neurenteric canal; diastematomyelia. AMA Arch Pathol 54 : 132-138, 1952
  3. Caram PC, Carton CA, Scarcella G : Intradural lipomas of the spinal cord; with particular emphasis on the intramedullary lipomas. J Neurosurg 14 : 28-42, 1957 https://doi.org/10.3171/jns.1957.14.1.0028
  4. Chapman PH : Congenital intraspinal lipomas: anatomic considerations and surgical treatment. Childs Brain 9 : 37-47, 1982
  5. Dady A, Havis E, Escriou V, Catala M, Duband JL : Junctional neurulation: a unique developmental program shaping a discrete region of the spinal cord highly susceptible to neural tube defects. J Neurosci 34 : 13208-13221, 2014 https://doi.org/10.1523/JNEUROSCI.1850-14.2014
  6. Dias MS, Walker ML : The embryogenesis of complex dysraphic malformations: a disorder of gastrulation? Pediatr Neurosurg 18 : 229-253, 1992 https://doi.org/10.1159/000120670
  7. Eibach S, Moes G, Hou YJ, Zovickian J, Pang D : Unjoined primary and secondary neural tubes: junctional neural tube defect, a new form of spinal dysraphism caused by disturbance of junctional neurulation. Childs Nerv Syst 33 : 1633-1647, 2017 https://doi.org/10.1007/s00381-016-3288-7
  8. Garceau GJ : The filum terminale syndrome (the cord-traction syndrome). J Bone Joint Surg Am 35-A : 711-716, 1953 https://doi.org/10.2106/00004623-195335030-00020
  9. Hoffman HJ, Hendrick EB, Humphreys RP : The tethered spinal cord: its protean manifestations, diagnosis and surgical correction. Childs Brain 2 : 145-155, 1976
  10. Kang JK, Kim MC, Kim DS, Song JU : Effects of tethering on regional spinal cord blood flow and sensory-evoked potentials in growing cats. Childs Nerv Syst 3 : 35-39, 1987 https://doi.org/10.1007/BF00707191
  11. Kim JW, Wang KC, Chong S, Kim SK, Lee JY : Limited dorsal myeloschisis: reconsideration of its embryological origin. Neurosurgery 86 : 93-100, 2020 https://doi.org/10.1093/neuros/nyy632
  12. Lee JY, Kim SP, Kim SW, Park S-H, Choi JW, Phi JH, et al. : Pathoembryogenesis of terminal myelocystocele: terminal balloon in secondary neurulation of the chick embryo. Childs Nerv Syst 29 : 1683-1688, 2013 https://doi.org/10.1007/s00381-013-2196-3
  13. Lee JY, Park SH, Chong S, Phi JH, Kim SK, Cho BK, et al. : Congenital dermal sinus and limited dorsal myeloschisis: "spectrum disorders" of incomplete dysjuction between cutaneous and neural ectoderms. Neurosurgery 84 : 428-434, 2019 https://doi.org/10.1093/neuros/nyy058
  14. Li YC, Shin SH, Cho BK, Lee MS, Lee YJ, Hong SK, et al. : Pathogenesis of lumbosacral lipoma: a test of the "premature dysjunction" theory. Pediatr Neurosurg 34 : 124-130, 2001 https://doi.org/10.1159/000056007
  15. McLone DG, Naidich TP : Spinal dysraphism : Experimental and clinical in Holtzman RN, Stein BM (eds) : The tethered spinal cord. New York : Thieme-Stratton, 1985, pp14-28
  16. McLone DG, Naidich TP : Terminal myelocystocele. Neurosurgery 16 : 36-43, 1985 https://doi.org/10.1227/00006123-198501000-00008
  17. Naidich TP, McLone DG, Mutluer S : A new understanding of dorsal dysraphism with lipoma (lipomyeloschisis): radiologic evaluation and surgical correction. AJR Am J Roentgenol 140 : 1065-1078, 1983 https://doi.org/10.2214/ajr.140.6.1065
  18. Pang D : Split cord malformation: part II: clinical syndrome. Neurosurgery 31 : 481-500, 1992 https://doi.org/10.1227/00006123-199209000-00011
  19. Pang D : Sacral agenesis and caudal spinal cord malformations. Neurosurgery 32 : 755-778; discussion 778-779, 1993 https://doi.org/10.1227/00006123-199305000-00009
  20. Pang D, Dias MS, Ahab-Barmada M : Split cord malformation: part I: a unified theory of embryogenesis for double spinal cord malformations. Neurosurgery 31 : 451-480, 1992 https://doi.org/10.1227/00006123-199209000-00010
  21. Pang D, Zovickian J, Lee JY, Moes GS, Wang KC : Terminal myelocystocele: surgical observations and theory of embryogenesis. Neurosurgery 70 : 1383-1404; discussion 1404-1405, 2012 https://doi.org/10.1227/NEU.0b013e31824c02c0
  22. Pang D, Zovickian J, Moes GS : Retained medullary cord in humans: late arrest of secondary neurulation. Neurosurgery 68 : 1500-1519; discussion 1519, 2011 https://doi.org/10.1227/NEU.0b013e31820ee282
  23. Pang D, Zovickian J, Oviedo A : Long-term outcome of total and neartotal resection of spinal cord lipomas and radical reconstruction of the neural placode: part I-surgical technique. Neurosurgery 65 : 511-528; discussion 528-529, 2009 https://doi.org/10.1227/01.NEU.0000350879.02128.80
  24. Pang D, Zovickian J, Oviedo A : Long-term outcome of total and neartotal resection of spinal cord lipomas and radical reconstruction of the neural placode, part II: outcome analysis and preoperative profiling. Neurosurgery 66 : 253-272; discussion 272-273, 2010 https://doi.org/10.1227/01.NEU.0000363598.81101.7B
  25. Pang D, Zovickian J, Oviedo A, Moes GS : Limited dorsal myeloschisis: a distinctive clinicopathological entity. Neurosurgery 67 : 1555-1579; discussion 1579-1580, 2010 https://doi.org/10.1227/NEU.0b013e3181f93e5a
  26. Pierre-Kahn A, Zerah M, Renier D, Cinalli G, Sainte-Rose C, Lellouch-Tubiana A, et al. : Congenital lumbosacral lipomas. Childs Nerv Syst 13 : 298-334; discussion 335, 1997 https://doi.org/10.1007/s003810050090
  27. Selcuki M, Coskun K : Management of tight filum terminale syndrome with special emphasis on normal level conus medullaris (NLCM). Surg Neurol 50 : 318-322; discussion 322, 1998 https://doi.org/10.1016/S0090-3019(97)00377-7
  28. Wang KC, Lee JS, Kim K, Im YJ, Park K, Kim KH, et al. : Do junctional neural tube defect and segmental spinal dysgenesis have the same pathoembryological background? Childs Nerv Syst 36 : 241-250, 2020 https://doi.org/10.1007/s00381-019-04425-4
  29. Yamada S, Zinke DE, Sanders D : Pathophysiology of "tethered cord syndrome". J Neurosurg 54 : 494-503, 1981 https://doi.org/10.3171/jns.1981.54.4.0494
  30. Yang HJ, Lee DH, Lee YJ, Chi JG, Lee JY, Phi JH, et al. : Secondary neurulation of human embryos: morphological changes and the expression of neuronal antigens. Childs Nerv Syst 30 : 73-82, 2014 https://doi.org/10.1007/s00381-013-2192-7
  31. Yang HJ, Wang KC, Chi JG, Lee MS, Lee YJ, Kim SK, et al. : Neural differentiation of caudal cell mass (secondary neurulation) in chick embryos: Hamburger and Hamilton stages 16-45. Brain Res Dev Brain Res 142 : 31-36, 2003 https://doi.org/10.1016/S0165-3806(03)00009-9

Cited by

  1. Disorders of Secondary Neurulation : Mainly Focused on Pathoembryogenesis vol.64, pp.3, 2021, https://doi.org/10.3340/jkns.2021.0023