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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

Abstract

Purpose

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.

Methods

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

Results

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.

Conclusions

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

Keywords

Limited dorsal myeloschisis Tethered cord Occult spinal dysraphism Untethering 

Notes

Acknowledgements

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

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

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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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