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Limited Dorsal Spinal Nondisjunctional Disorders: Limited Dorsal Myeloschisis, Congenital Spinal Dermal Sinus Tract, and Mixed Lesions

  • Sui-To WongEmail author
  • Amanda Kan
  • Dachling PangEmail author
Living reference work entry

Abstract

Spinal dysraphic lesions due to incomplete fusion and consequent failed disjunction of a limited segment of the dorsal neural tube during primary neurulation are the subjects of this chapter. We use the term “limited dorsal spinal nondisjunctional disorders” (LDSNDs) to encompass several known varieties of such lesions, namely, limited dorsal myeloschisis (LDM), congenital spinal dermal sinus tract (CSDST), mixed lesions of the two, and other closely related complex lesions (Wang et al. 1993; Pang et al. 2013a; Eibach et al. 2017). All of these probably share a common embryogenetic mechanism and similar clinical manifestations.

References

  1. Ackerman LL, Menezes AH (2003) Spinal congenital dermal sinuses: a 30-year experience. Pediatrics 112(3 Pt 1):641–647PubMedGoogle Scholar
  2. Colas JF, Schoenwolf GC (2001) Towards a cellular and molecular understanding of neurulation. Dev Dyn 221(2):117–145PubMedGoogle Scholar
  3. Cornips EM, Weber JW, Vles JS, van Aalst J (2011) Pseudo-dermal sinus tract or spinal dermal-sinus-like stalk? Childs Nerv Syst 27(8):1189–1191PubMedPubMedCentralGoogle Scholar
  4. De Vloo P, Lagae L, Sciot R, Demaerel P, van Loon J, Van Calenbergh F (2013) Spinal dermal sinuses and dermal sinus-like stalks analysis of 14 cases with suggestions for embryologic mechanisms resulting in dermal sinus-like stalks. Eur J Paediatr Neurol 17(6):575–584PubMedGoogle Scholar
  5. Eibach S, Moes G, Zovickian J, Pang D (2017) Limited dorsal myeloschisis associated with dermoid elements. Childs Nerv Syst 33(1):55–67PubMedGoogle Scholar
  6. Elton S, Oakes WJ (2001) Dermal sinus tracts of the spine. Neurosurg Focus 10(1):e4PubMedGoogle Scholar
  7. Girishan S, Rajshekhar V (2016) Rapid-onset paraparesis and quadriparesis in patients with intramedullary spinal dermoid cysts: report of 10 cases. J Neurosurg Pediatr 17(1):86–93PubMedGoogle Scholar
  8. Hiraoka A, Morioka T, Murakami N, Suzuki SO, Mizoguchi M (2018) Limited dorsal myeloschisis with no extradural stalk linking to a flat skin lesion: a case report. Childs Nerv Syst 34(12):2497–2501PubMedGoogle Scholar
  9. Jindal A, Mahapatra AK (2001) Spinal congenital dermal sinus: an experience of 23 cases over 7 years. Neurol India 49(3):243–246PubMedGoogle Scholar
  10. Lee CS (2005) Congenital dermal sinuses: an clinical analysis of 20 cases. J Korean Neurosurg Soc 37(1):29–33Google Scholar
  11. Lee SM, Cheon JE, Choi YH, Kim IO, Kim WS, Cho HH, Lee JY, Wang KC (2017a) Limited dorsal myeloschisis and congenital dermal sinus: comparison of clinical and MR imaging features. AJNR Am J Neuroradiol 38(1):176–182PubMedGoogle Scholar
  12. Lee JY, Chong S, Choi YH, Phi JH, Cheon JE, Kim SK, Park SH, Kim IO, Wang KC (2017b) Modification of surgical procedure for “probable” limited dorsal myeloschisis. J Neurosurg Pediatr 19(5):616–619PubMedGoogle Scholar
  13. Lee JY, Park SH, Chong S, Phi JH, Kim SK, Cho BK, Wang KC (2018) Congenital dermal sinus and limited dorsal myeloschisis: “Spectrum disorders” of incomplete dysjuction between cutaneous and neural ectoderms. Neurosurgery.  https://doi.org/10.1093/neuros/nyx058. [Epub ahead of print]
  14. Martínez-Lage JF, Almagro MJ, Ferri-Ñiguez B, Izura Azanza V, Serrano C, Domenech E (2011) Spinal dermal sinus and pseudo-dermal sinus tracts: two different entities. Childs Nerv Syst 27(4):609–616PubMedGoogle Scholar
  15. Martins-Green M (1988) Origin of the dorsal surface of the neural tube by progressive delamination of epidermal ectoderm and neuroepithelium: implications for neurulation and neural tube defects. Development 103:687–706PubMedGoogle Scholar
  16. Martins-Green M, Erickson CA (1987) Basal lamina is not a barrier to neural crest cell emigration: documentation by TEM and by immunofluorescent and immunogold labelling. Development 101:517–533PubMedGoogle Scholar
  17. Matson DD (1959) Neurosurgery of infancy and childhood. Charles Thomas, SpringfieldGoogle Scholar
  18. Mete M, Umur AS, Duransoy YK, Barutçuoğlu M, Umur N, Gurgen SG, Selçuki M (2014) Congenital dermal sinus tract of the spine: experience of 16 patients. J Child Neurol 29(10):1277–1282PubMedGoogle Scholar
  19. Morioka T, Suzuki SO, Murakami N, Mukae N, Shimogawa T, Haruyama H, Kira R, Iihara K (2018a) Surgical histopathology of limited dorsal myeloschisis with flat skin lesion. Childs Nerv Syst 35:119.  https://doi.org/10.1007/s00381-018-3870-2. [Epub ahead of print]PubMedGoogle Scholar
  20. Morioka T, Suzuki SO, Murakami N, Shimogawa T, Mukae N, Inoha S, Sasaguri T, Iihara K (2018b) Neurosurgical pathology of limited dorsal myeloschisis. Childs Nerv Syst 34(2):293–303PubMedGoogle Scholar
  21. Pang D, Dias M (1993) Cervical myelomeningoceles. Neurosurgery 33(3):363–373PubMedGoogle Scholar
  22. Pang D (2006) Surgical management of spinal dysraphism. In: Fessler R, Sekhar L (eds) Atlas of neurosurgical techniques. Thieme Medical and Scientific Publishers, New York, pp 729–758Google Scholar
  23. Pang D, Dias M, Ahdab-Barmada M (1992) Split cord malformation part I: a unified theory of embryogenesis for double spinal cord malformation. Neurosurgery 31:451–480PubMedGoogle Scholar
  24. Pang D, Zovickian J, Oviedo A, Moes GS (2010) Limited dorsal myeloschisis: a distinctive clinicopathological entity. Neurosurgery 67:1555–1580PubMedGoogle Scholar
  25. Pang D, Zovickian J, Wong ST, Hou YJ, Moes GS (2013a) Limited dorsal myeloschisis: a not-so-rare form of primary neurulation defect. Childs Nerv Syst 29(9):1459–1484PubMedGoogle Scholar
  26. Pang D, Zovickian J, Wong ST, Hou YJ, Moes GS (2013b) Surgical treatment of complex spinal cord lipomas. Childs Nerv Syst 29(9):1485–1513PubMedGoogle Scholar
  27. Radmanesh F, Nejat F, El Khashab M (2010) Dermal sinus tract of the spine. Childs Nerv Syst 26(3):349–357PubMedGoogle Scholar
  28. Rajpal S, Salamat MS, Tubbs RS, Kelly DR, Oakes WJ, Iskandar BJ (2007) Tethering tracts in spina bifida occulta: revisiting an established nomenclature. J Neurosurg Spine 7(3):315–322PubMedGoogle Scholar
  29. Rossi A, Piatelli G, Gandofolo C, Pavanello M, Hoffmann C, Van Goethem JW, Cama A, Tortori-Donati P (2006) Spectrum of nonterminal myelocystoceles. Neurosurgery 58:509–515PubMedGoogle Scholar
  30. Schoenwolf GC (1979) Observations on closure of the neuropores in the chick embryo. Am J Anat 155:445–466PubMedGoogle Scholar
  31. Schoenwolf GC (1982) On the morphogenesis of the early rudiments of the developing central nervous system. Scan Electron Microsc 1:289–308Google Scholar
  32. Schoenwolf GC, Smith JL (1990) Mechanisms of neurulation: traditional viewpoint and recent advances. Development 109(2):243–270PubMedGoogle Scholar
  33. Selleck MA, Bronner-Fraser M (1995) Origins of the avian neural crest: the role of neural plate-epidermal interactions. Development 121(2):525–538PubMedPubMedCentralGoogle Scholar
  34. Silver MH, Kerns JM (1978) Ultrastructure of neural fold fusion in chick embryos. Scan Electron Microsc II:209–215Google Scholar
  35. Singh I, Rohilla S, Kumar P, Sharma S (2015) Spinal dorsal dermal sinus tract: an experience of 21 cases. Surg Neurol Int 6(Suppl 17):S429–S434PubMedPubMedCentralGoogle Scholar
  36. Steinbok P (1995) Dysraphic lesions of the cervical spinal cord. In: Pang D (ed) Neurosurg Clin N Am. W.B. Saunders, Philadelphia, pp 367–376PubMedGoogle Scholar
  37. Steinbok P, Cochrane DD (1991) The nature of congenital posterior cervical or cervicothoracic midline cutaneous mass lesions. Report of eight cases. J Neurosurg 75:206–212PubMedGoogle Scholar
  38. Suneson A, Kalimo H (1979) Myelocystocele with cerebellar heterotopia. J Neurosurg 51:392–396PubMedGoogle Scholar
  39. Theveneau E, Mayor R (2012) Neural crest delamination and migration: from epithelium-to-mesenchyme transition to collective cell migration. Dev Biol 366(1):34–54PubMedPubMedCentralGoogle Scholar
  40. Tisdall MM, Hayward RD, Thompson DN (2015) Congenital spinal dermal tract: how accurate is clinical and radiological evaluation? J Neurosurg Pediatr 15(6):651–656PubMedGoogle Scholar
  41. van Aalst J, Beuls EA, Cornips EM, Vanormelingen L, Vandersteen M, Weber JW, Vles JS (2006) Anatomy and surgery of the infected dermal sinus of the lower spine. Childs Nerv Syst 22(10):1307–1315PubMedGoogle Scholar
  42. van Aalst J, Beuls EA, Cornips EM, van Straaten HW, Boselie AF, Rijkers K, Weber JW, Vles JS (2009) The spinal dermal-sinus-like stalk. Childs Nerv Syst 25(2):191–197PubMedGoogle Scholar
  43. van Straaten HW, Jaskoll T, Rousseau AM, Terwindt-Rouwenhorst EA, Greenberg G, Shankar K, Melnick M (1993) Raphe of the posterior neural tube in the chick embryo: its closure and reopening as studied in living embryos with a high definition light microscope. Dev Dyn 198(1):65–76PubMedGoogle Scholar
  44. Van Straaten HW, Janssen HC, Peeters MC, Copp AJ, Hekking JW (1996) Neural tube closure in the chick embryo is multiphasic. Dev Dyn 207(3):309–318PubMedGoogle Scholar
  45. Venkatesh R, Rajasekar G, Jeevarajan S (2018) Spectrum of spinal dermal sinus: analysis and outcome evaluation. J Spinal Surg 5:4–9Google Scholar
  46. Wang KC, Yang HJ, Oh CW, Kim HJ, Cho BK (1993) Spinal congenital dermal sinus – experience of 5 cases over a period of 10 years. J Korean Med Sci 8(5):341–347PubMedPubMedCentralGoogle Scholar
  47. Weprin BE, Oakes WJ (2000) Coccygeal pits. Pediatrics 105:e69PubMedGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Department of NeurosurgeryTuen Mun HospitalHong KongChina
  2. 2.Department of Clinical PathologyTuen Mun HospitalHong KongChina
  3. 3.University of CaliforniaDavisUSA
  4. 4.Great Ormond Street Hospital For Children, NHS TrustLondonUK

Section editors and affiliations

  • Dachling Pang
    • 1
  • Kyu-Chang Wang
    • 2
    • 3
  • Dominic N. P. Thompson
    • 4
  1. 1.LondonUK
  2. 2.Division of Pediatric NeurosurgerySeoul National University Children’s HospitalSeoulSouth Korea
  3. 3.Division of Pediatric NeurosurgerySeoul National University Children’s Hospital & Seoul National University College of MedicineSeoulSouth Korea
  4. 4.Department of NeurosurgeryGreat Ormond Street HospitalLondonUK

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