Child's Nervous System

, Volume 35, Issue 1, pp 119–128 | Cite as

Surgical histopathology of limited dorsal myeloschisis with flat skin lesion

  • Takato MoriokaEmail author
  • Satoshi O. Suzuki
  • Nobuya Murakami
  • Nobutaka Mukae
  • Takafumi Shimogawa
  • Hironori Haruyama
  • Ryutaro Kira
  • Koji Iihara
Original Paper



Limited dorsal myeloschisis (LDM) is characterized by two invariable features: a focal closed neural tube defect and a fibroneural stalk linking the skin lesion to the underlying spinal cord. Although detailed histopathological findings of the LDM stalk were originally described by Pang et al., the precise relationship between the histopathological findings and clinical manifestations including intraoperative findings has not been fully determined.


We retrospectively analyzed the histopathological findings of the almost entire stalk and their relevance to the clinical manifestations in six Japanese LDM patients with flat skin lesions.


Glial fibrillary acidic protein (GFAP)-immunopositive neuroglial tissues were observed in three of the six patients. Unlike neuroglial tissues, peripheral nerve fibers were observed in every stalk. In four patients, dermal melanocytosis, “Mongolian spot,” was seen surrounding the cigarette-burn lesion. In three of these four patients, numerous melanocytes were distributed linearly along the long axis of the LDM stalk, which might represent migration of melanocytes from trunk neural crest cells during formation of the LDM stalk.


Immunopositivity for GFAP in the LDM stalk was observed in as few as 50% of our patients, despite the relatively extensive histopathological examination. We confirm that the clinical diagnosis of LDM should be made based on comprehensive histopathological examination as well as clinical manifestations. The profuse network of peripheral nerve fibers in every stalk and the high incidence of melanocyte accumulation associated with dermal melanocytosis might assist the histopathological diagnosis of LDM.


Glial fibrillary acidic protein Peripheral nerves Melanocyte Untethering 



We thank Jane Charbonneau, DVM, from Edanz Group ( for editing a draft of this manuscript.

Funding information

This work was partly supported by the Research Foundation of Fukuoka Children’s Hospital.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.


  1. 1.
    Cichorek M, Wachulska M, Stasiewicz A, Tyminska A (2013) Skin melanocytes: biology and development. Postepy Dermatol Alergol 1:30–41CrossRefGoogle 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.
    Franceschini D, Dinulos JG (2015) Dermal melanocytesis and associated disorders. Curr Opin Pediatr 27:480–485CrossRefGoogle Scholar
  4. 4.
    Gupta D, Thappa DM (2013) Mongolian spots: how important are they? World J Clin Cases 16:230–232CrossRefGoogle Scholar
  5. 5.
    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) 103:537–543CrossRefGoogle Scholar
  6. 6.
    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
  7. 7.
    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
  8. 8.
    Lee JY, Park S-H, Chong S, Phi JH, Kim S-K, Cho B-K, Wang K-C (2018) Congenital dermal sinus and limited dorsal myeloschisis: “spectrum disorders” of incomplete dysjunction between cutaneous and neural ectoderms. Neurosurgery (Epub ahead of print)Google Scholar
  9. 9.
    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
  10. 10.
    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
  11. 11.
    Morioka T, Suzuki SO, Murakami N, Shimogawa T, Mukae N, Inoha S, Sasaguri T, Iihara K (2018) Neurosurgical pathology of limited dorsal myeloschisis. Childs Nerv Syst 34:293–303CrossRefGoogle Scholar
  12. 12.
    Morota N, Ihara S, Ogiwara H (2017) New classification of spinal lipomas based on embryonic stage. J Neurosurg Pediatr 19:428–439CrossRefGoogle Scholar
  13. 13.
    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
  14. 14.
    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
  15. 15.
    Murakami N, Morioka T, Shimogawa T, Hashiguchi K, Mukae N, Uchihashi K, Suzuki SO, Iihara K (2018) Retained medullary cord extending to a sacral subcutaneous menigocele. Childs Nerv Syst 34(3):527–533CrossRefGoogle 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.
    Shirozu N, Morioka T, Inoha S, Imamoto N, Sasaguri T (2018) Enlargement of sacral subcutaneous meningocele associated with retained medullary cord. Childs Nerv Syst (Epub ahead of print)Google Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

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 Pediatric NeurologyFukuoka Children’s HospitalFukuokaJapan

Personalised recommendations