Surgical histopathology of limited dorsal myeloschisis with flat skin lesion

Takato Morioka, Satoshi Suzuki, Nobuya Murakami, Nobutaka Mukae, Takafumi Shimogawa, Hironori Haruyama, Ryutaro Kira, Koji Iihara

研究成果: ジャーナルへの寄稿記事

3 引用 (Scopus)

抄録

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.

元の言語英語
ページ(範囲)119-128
ページ数10
ジャーナルChild's Nervous System
35
発行部数1
DOI
出版物ステータス出版済み - 1 29 2019

Fingerprint

Melanocytes
Skin
Glial Fibrillary Acidic Protein
Peripheral Nerves
Nerve Fibers
Mongolian Spot
Neural Tube Defects
Neural Crest
Burns
Tobacco Products
Spinal Cord
Incidence

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

これを引用

Surgical histopathology of limited dorsal myeloschisis with flat skin lesion. / Morioka, Takato; Suzuki, Satoshi; Murakami, Nobuya; Mukae, Nobutaka; Shimogawa, Takafumi; Haruyama, Hironori; Kira, Ryutaro; Iihara, Koji.

:: Child's Nervous System, 巻 35, 番号 1, 29.01.2019, p. 119-128.

研究成果: ジャーナルへの寄稿記事

Morioka, Takato ; Suzuki, Satoshi ; Murakami, Nobuya ; Mukae, Nobutaka ; Shimogawa, Takafumi ; Haruyama, Hironori ; Kira, Ryutaro ; Iihara, Koji. / Surgical histopathology of limited dorsal myeloschisis with flat skin lesion. :: Child's Nervous System. 2019 ; 巻 35, 番号 1. pp. 119-128.
@article{3af292ef1ec6472c9ca8450f10c256ba,
title = "Surgical histopathology of limited dorsal myeloschisis with flat skin lesion",
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.",
author = "Takato Morioka and Satoshi Suzuki and Nobuya Murakami and Nobutaka Mukae and Takafumi Shimogawa and Hironori Haruyama and Ryutaro Kira and Koji Iihara",
year = "2019",
month = "1",
day = "29",
doi = "10.1007/s00381-018-3870-2",
language = "English",
volume = "35",
pages = "119--128",
journal = "Child's Nervous System",
issn = "0256-7040",
publisher = "Springer Verlag",
number = "1",

}

TY - JOUR

T1 - Surgical histopathology of limited dorsal myeloschisis with flat skin lesion

AU - Morioka, Takato

AU - Suzuki, Satoshi

AU - Murakami, Nobuya

AU - Mukae, Nobutaka

AU - Shimogawa, Takafumi

AU - Haruyama, Hironori

AU - Kira, Ryutaro

AU - Iihara, Koji

PY - 2019/1/29

Y1 - 2019/1/29

N2 - 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.

AB - 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.

UR - http://www.scopus.com/inward/record.url?scp=85048863142&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85048863142&partnerID=8YFLogxK

U2 - 10.1007/s00381-018-3870-2

DO - 10.1007/s00381-018-3870-2

M3 - Article

C2 - 29934704

AN - SCOPUS:85048863142

VL - 35

SP - 119

EP - 128

JO - Child's Nervous System

JF - Child's Nervous System

SN - 0256-7040

IS - 1

ER -