Neurosurgical pathology of limited dorsal myeloschisis

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

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10 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)293-303
Number of pages11
JournalChild's Nervous System
Volume34
Issue number2
DOIs
Publication statusPublished - Feb 1 2018

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All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

Cite this

Morioka, T., Suzuki, S. O., Murakami, N., Shimogawa, T., Mukae, N., Inoha, S., ... Iihara, K. (2018). Neurosurgical pathology of limited dorsal myeloschisis. Child's Nervous System, 34(2), 293-303. https://doi.org/10.1007/s00381-017-3625-5