Neurosurgical pathology of limited dorsal myeloschisis

Childs Nerv Syst. 2018 Feb;34(2):293-303. doi: 10.1007/s00381-017-3625-5. Epub 2017 Oct 23.


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; Occult spinal dysraphism; Tethered cord; Untethering.

Publication types

  • Case Reports

MeSH terms

  • Child, Preschool
  • Female
  • Humans
  • Infant
  • Infant, Newborn
  • Male
  • Neural Tube Defects / pathology*
  • Neural Tube Defects / surgery*
  • Neurosurgical Procedures* / methods
  • Retrospective Studies
  • Skin Abnormalities / pathology*
  • Skin Abnormalities / surgery*