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Published in: Child's Nervous System 2/2018

01-02-2018 | Original Paper

Neurosurgical pathology of limited dorsal myeloschisis

Authors: Takato Morioka, Satoshi O. Suzuki, Nobuya Murakami, Takafumi Shimogawa, Nobutaka Mukae, Satoshi Inoha, Takakazu Sasaguri, Koji Iihara

Published in: Child's Nervous System | Issue 2/2018

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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.
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Metadata
Title
Neurosurgical pathology of limited dorsal myeloschisis
Authors
Takato Morioka
Satoshi O. Suzuki
Nobuya Murakami
Takafumi Shimogawa
Nobutaka Mukae
Satoshi Inoha
Takakazu Sasaguri
Koji Iihara
Publication date
01-02-2018
Publisher
Springer Berlin Heidelberg
Published in
Child's Nervous System / Issue 2/2018
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-017-3625-5

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