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Child's Nervous System

, Volume 35, Issue 1, pp 119–128 | Cite as

Surgical histopathology of limited dorsal myeloschisis with flat skin lesion

  • Takato MoriokaEmail author
  • Satoshi O. Suzuki
  • Nobuya Murakami
  • Nobutaka Mukae
  • Takafumi Shimogawa
  • Hironori Haruyama
  • Ryutaro Kira
  • Koji Iihara
Original Paper

Abstract

Purpose

Limited dorsal myeloschisis (LDM) is characterized by two invariable features: a focal closed neural tube defect and a fibroneural stalk linking the skin lesion to the underlying spinal cord. Although detailed histopathological findings of the LDM stalk were originally described by Pang et al., the precise relationship between the histopathological findings and clinical manifestations including intraoperative findings has not been fully determined.

Methods

We retrospectively analyzed the histopathological findings of the almost entire stalk and their relevance to the clinical manifestations in six Japanese LDM patients with flat skin lesions.

Results

Glial fibrillary acidic protein (GFAP)-immunopositive neuroglial tissues were observed in three of the six patients. Unlike neuroglial tissues, peripheral nerve fibers were observed in every stalk. In four patients, dermal melanocytosis, “Mongolian spot,” was seen surrounding the cigarette-burn lesion. In three of these four patients, numerous melanocytes were distributed linearly along the long axis of the LDM stalk, which might represent migration of melanocytes from trunk neural crest cells during formation of the LDM stalk.

Conclusion

Immunopositivity for GFAP in the LDM stalk was observed in as few as 50% of our patients, despite the relatively extensive histopathological examination. We confirm that the clinical diagnosis of LDM should be made based on comprehensive histopathological examination as well as clinical manifestations. The profuse network of peripheral nerve fibers in every stalk and the high incidence of melanocyte accumulation associated with dermal melanocytosis might assist the histopathological diagnosis of LDM.

Keywords

Glial fibrillary acidic protein Peripheral nerves Melanocyte Untethering 

Notes

Acknowledgments

We thank Jane Charbonneau, DVM, from Edanz Group (www.edanzediting.com/ac) for editing a draft of this manuscript.

Funding information

This work was partly supported by the Research Foundation of Fukuoka Children’s Hospital.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of NeurosurgeryFukuoka Children’s HospitalFukuokaJapan
  2. 2.Department of Neuropathology, Graduate School of Medical SciencesKyushu UniversityFukuokaJapan
  3. 3.Department of Neurosurgery, Graduate School of Medical SciencesKyushu UniversityFukuokaJapan
  4. 4.Department of Pediatric NeurologyFukuoka Children’s HospitalFukuokaJapan

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