Neurosurgical pathology of limited dorsal myeloschisis
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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.
We retrospectively analyzed the neurosurgical pathologic findings of four LDM patients.
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.
Therefore, the diagnosis of LDM should be made based on comprehensive evaluation of histologic and clinical findings.
KeywordsLimited dorsal myeloschisis Tethered cord Occult spinal dysraphism Untethering
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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Conflict of interest
The authors declare that they have no conflict of interest.
- 11.Murakami N, Morioka T, Hashiguchi K, Yoshiura T, Hiwatashi K, Suzuki SO, Nakamizo A, Amano T, Hata N, Sasaki T (2013) Usefulness of three-dimensional T1-weighted spoiled gradient-recalled echo and three-dimensional heavily T2-weighted images in preoperative evaluation of spinal dysraphism. Childs Nerv Syst 29:1905–1914CrossRefGoogle Scholar
- 13.Muthukumar N, Arunthathi J, Sundar V (2000) Split cord malformation and neurenteric cyst-case report and a theory of embryogenesis. Br J Neurosurg 8:586–587Google Scholar
- 15.Pang D (1993) Cervical myelomanigoceles. Neurosurgery 33:363–373Google Scholar