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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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.
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Morioka, T., Suzuki, S.O., Murakami, N. et al. Neurosurgical pathology of limited dorsal myeloschisis. Childs Nerv Syst 34, 293–303 (2018). https://doi.org/10.1007/s00381-017-3625-5
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DOI: https://doi.org/10.1007/s00381-017-3625-5