Child's Nervous System

, Volume 34, Issue 2, pp 293–303 | Cite as

Neurosurgical pathology of limited dorsal myeloschisis

  • Takato MoriokaEmail author
  • Satoshi O. Suzuki
  • Nobuya Murakami
  • Takafumi Shimogawa
  • Nobutaka Mukae
  • Satoshi Inoha
  • Takakazu Sasaguri
  • Koji Iihara
Original Paper



The term limited dorsal myeloschisis (LDM) was used by Pang et al. (2010) to describe a distinct clinicopathological entity. LDMs are characterized by two invariable features: a focal-closed neural tube defect and a fibroneural stalk that links the skin lesion to the underlying spinal cord.


We retrospectively analyzed the neurosurgical pathologic findings of four LDM patients.


Case 1 had a saccular skin lesion with nonterminal abortive myelocystocele at T11–12. Cases 2, 3, and 4 had a non-saccular (flat) skin lesion in the lumbosacral region. The morphologic features of the lesion in case 2 were those of meningocele manque. Cases 3 and 4 had accompanying non-LDM anomalies, caudal-type lipoma and type II split-cord malformation with neurenteric cyst, respectively. At preoperative diagnosis of the LDM stalk, magnetic resonance imaging, including 3D heavily T2-weighted image was useful; however, minute findings were often missed in the complicated cases 3 and 4. All patients had a favorable outcome following untethering of the stalk from the cord. The central histopathological feature of the LDM stalk is neuroglial tissue in the fibrocollagenous band; however, the stalk in cases 2 and 4 did not have glial fibrillary acidic protein-immunopositive neuroglial tissues.


Therefore, the diagnosis of LDM should be made based on comprehensive evaluation of histologic and clinical findings.


Limited dorsal myeloschisis Tethered cord Occult spinal dysraphism Untethering 



This work was partly supported by Research Foundation of Fukuoka Children’s Hospital. We thank Denise Di Salvo, MS, from Edanz Group ( for editing a draft of this manuscript.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.


  1. 1.
    Chatterjee S, Rao KSM (2015) Missed limited dorsal myeloschisis: an unfortunate cause for recurrent tethered cord syndrome. Childs Nerv Syst 31:1553–1557CrossRefGoogle Scholar
  2. 2.
    Eibach S, Moes G, Hou YJ, Zovickian J, Pang D (2017) 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 33:1633–1647CrossRefGoogle Scholar
  3. 3.
    Hashiguchi K, Morioka T, Fukui K, Miyagi Y, Mihara F, Yoshiura T, Nagata S, Sasaki T (2005) Usefulness of constructive interference in steady-state (CISS) MR imaging in the presurgical examination for lumbosacral lipoma. J Neurosurg (6 Pediatrics) 103:537–543PubMedPubMedCentralGoogle Scholar
  4. 4.
    Hashiguchi K, Morioka T, Yoshida F, Miyagi Y, Mihara F, Yoshiura T, Nagata S, Sasaki T (2007) Feasibility and limitation of constructive interference in steady-state (CISS) MR imaging in neonates with lumbosacral myeloschisis. Neuroradiology 49:579–585CrossRefGoogle Scholar
  5. 5.
    Lassman LP, James CC (1977) Menigocele manque. Child’s Brain 3:1–11PubMedPubMedCentralGoogle Scholar
  6. 6.
    Lee JY, Chong S, Choi YH, Phi JH, Cheon J-E, Kim S-K, Park SH, Kim I-O, Wang K-C (2017) Modification of surgical procedure for “probable” limited dorsal myeloschisis. J Neurosurg Pediatr 19:616–619CrossRefGoogle Scholar
  7. 7.
    Lee SM, Cheon J-E, Choi YH, Kim I-O, Kim WS, Cho H-H, Lee JY, Wang K-C (2017) Limited dorsal myeloschisis and congenitall dermal sinus: comparison of clinical and MR imaging features. AJNR Am J Neuradiol 38:176–182CrossRefGoogle Scholar
  8. 8.
    Morioka T, Hashiguchi K, Yoshida F, Nagata S, Miyagi Y, Mihara F, Sasaki T (2007) Dynamic morphological changes in lumbosacral lipoma during the first months of life revealed by constructive interference in steady-state (CISS) MR imaging. Childs Nerv Syst 23:415–420CrossRefGoogle Scholar
  9. 9.
    Morioka T, Murakami N, Shimogawa T, Mukae N, Hashiguchi K, Suzuki SO, Iihara K (2017) Neurosurgical management and pathology of lumbosacral lipomas with tethered cord. Neuropathology 37:385–392CrossRefGoogle Scholar
  10. 10.
    Morota N, Ihara S, Ogiwara H (2017) New classification of spinal lipomas based on embryonic stage. J Neurosurg Pediatr 19:428–439CrossRefGoogle Scholar
  11. 11.
    Murakami N, Morioka T, Hashiguchi K, Yoshiura T, Hiwatashi K, Suzuki SO, Nakamizo A, Amano T, Hata N, Sasaki T (2013) Usefulness of three-dimensional T1-weighted spoiled gradient-recalled echo and three-dimensional heavily T2-weighted images in preoperative evaluation of spinal dysraphism. Childs Nerv Syst 29:1905–1914CrossRefGoogle Scholar
  12. 12.
    Murakami N, Morioka T, Ichiyama M, Nakamura R, Kawamura N (2017) Lateral lipomyelomenigocele of the hemicord with split cord malformation type I revealed by 3D heavily T2-weighted MR imaging. Childs Nerv Syst 33:993–997CrossRefGoogle Scholar
  13. 13.
    Muthukumar N, Arunthathi J, Sundar V (2000) Split cord malformation and neurenteric cyst-case report and a theory of embryogenesis. Br J Neurosurg 8:586–587Google Scholar
  14. 14.
    Pang D, Mark DS, Mamdouha A-B (1992) Split cord malformation: part 1: a unified theory of embryonesis for double spinal cord malformation. Neurosurgery 31:451–458CrossRefGoogle Scholar
  15. 15.
    Pang D (1993) Cervical myelomanigoceles. Neurosurgery 33:363–373Google Scholar
  16. 16.
    Pang D, Zovickian J, Oviedo A, Moes GS (2010) Limited dorsal myeloschisis: a distinctive clinicopathological entity. Neurosurgery 67:1555–1580CrossRefGoogle Scholar
  17. 17.
    Pang D, Zovickian J, Wong S-T, Hou YJ, Moes GS (2013) Limited dorsal myeloschisis: a not-so-rare form of primary neurulation defect. Childs Nerv Syst 29:1459–1484CrossRefGoogle Scholar
  18. 18.
    Rossi A, Piatelli G, Gandolfo C, Pavenello M, Hoffmann C, van Goethem JW, Cama A, Tortori-Donati P (2006) Spectrum of nonterminal myelocystoceles. Neurosurgery 58:509–515CrossRefGoogle Scholar
  19. 19.
    Schmidt C, Bryant E, Iwanaga J, Oskouian RJ, Oakes WJ, Tubbs RS (2017) Menigocele manque: a comprehensive review of this enigmatic finding in occult spinal dysraphism. Childs Nerv Syst 33:1065–1071CrossRefGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany 2017

Authors and Affiliations

  1. 1.Department of NeurosurgeryFukuoka Children’s HospitalFukuokaJapan
  2. 2.Department of Neuropathology, Graduate School of Medical SciencesKyushu UniversityFukuokaJapan
  3. 3.Department of Neurosurgery, Graduate School of Medical SciencesKyushu UniversityFukuokaJapan
  4. 4.Department of Neurosurgery, Japan Community Health Care Organization (JCHO)Kyushu HospitalKitakyushuJapan
  5. 5.Department of Pathology, Japan Community Health Care Organization (JCHO)Kyushu HospitalKitakyushuJapan

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