TY - JOUR
T1 - Clinicopathological findings of limited dorsal myeloschisis associated with spinal lipoma of dorsal-type
AU - Murakami, Nobuya
AU - Morioka, Takato
AU - Suzuki, Satoshi O.
AU - Mukae, Nobutaka
AU - Shimogawa, Takafumi
AU - Matsuo, Yoshihiro
AU - Sasaguri, Takakazu
AU - Mizoguchi, Masahiro
N1 - Funding Information:
This work was partly supported by the Research Foundation of Fukuoka Children’s Hospital.
Funding Information:
We thank Dr. Naoyuki Imamoto, Department of Neurosurgery, JCHO Kyushu Hospital and Dr. Nobuko Kawamura, Department of Radiology, Fukuoka Children's Hospital for supporting our study. We thank Edanz Group (https://en-author-services.edanzgroup.com/) for editing a draft of this manuscript. This work was partly supported by the Research Foundation of Fukuoka Children's Hospital. Murakami N, Morioka T and Mizoguchi M: Contributed to the study design and project outline. Murakami N, Suzuki SO, Mukae N, Shimogawa T, Matsuo Y, Sasaguri T: Contributed to the data acquisition, analysis, and manuscript drafting. Morioka T, Suzuki SO, Mizoguchi M: Contributed to critically revising the manuscript, figures, and table.
Publisher Copyright:
© 2020 The Authors
PY - 2020/9
Y1 - 2020/9
N2 - Purpose: Limited dorsal myeloschisis (LDM) is thought to arise from focal incomplete disjunction between the cutaneous and neural ectoderm during primary neurulation, while spinal lipoma of dorsal-type (dorsal lipoma) arises from premature disjunction. Thus, simultaneous occurrence of an LDM and dorsal lipoma are not surprising, and may represent slightly different perturbations of disjunction caused by the same insult in neighboring loci. However, the clinicopathological findings of the LDM with dorsal lipoma have not been fully determined. Methods: Of 21 patients with LDM, 3 (14.3%) had dorsal lipoma. We retrospectively analyzed the clinicopathological findings of these 3 patients, especially the histopathological distribution of the fibrocollagenous LDM tract and fibroadipose tissue of the lipoma. Results: Patients 1 and 2 had flat skin lesions, while patient 3 had a human tail-like cutaneous appendage. In the tethering stalks linking the skin lesion at the lumbosacral lesion to the low-lying conus medullaris of the three patients, fibrocollagenous tissues embedding adipose tissues at the subcutaneous site, and with abundant adipose tissues at the extradural site, were changed to fibroadipose tissue at the intradural site. While glial fibrillary acidic protein-immunopositive neuroglial tissues were observed in 2 (patients 1 and 2), peripheral nerve fibers were observed in every stalk. Smooth muscle fibers were noted in patient 1, while a large amount of striated muscle fibers were seen in patients 2 and 3. Conclusion: These cases showed various tissues with different origins in the stalk. There may also be a seamless continuation between fibrocollagenous LDM tissue at the distal site and lipomatous tissue at the proximal site. Peripheral nerve fibers and smooth muscle fibers of neural crest origin may be dragged into the stalk during incomplete disjunction, while the striated muscle fibers of mesodermal origin may enter the stalk along with the lipomatous tissues during premature disjunction.
AB - Purpose: Limited dorsal myeloschisis (LDM) is thought to arise from focal incomplete disjunction between the cutaneous and neural ectoderm during primary neurulation, while spinal lipoma of dorsal-type (dorsal lipoma) arises from premature disjunction. Thus, simultaneous occurrence of an LDM and dorsal lipoma are not surprising, and may represent slightly different perturbations of disjunction caused by the same insult in neighboring loci. However, the clinicopathological findings of the LDM with dorsal lipoma have not been fully determined. Methods: Of 21 patients with LDM, 3 (14.3%) had dorsal lipoma. We retrospectively analyzed the clinicopathological findings of these 3 patients, especially the histopathological distribution of the fibrocollagenous LDM tract and fibroadipose tissue of the lipoma. Results: Patients 1 and 2 had flat skin lesions, while patient 3 had a human tail-like cutaneous appendage. In the tethering stalks linking the skin lesion at the lumbosacral lesion to the low-lying conus medullaris of the three patients, fibrocollagenous tissues embedding adipose tissues at the subcutaneous site, and with abundant adipose tissues at the extradural site, were changed to fibroadipose tissue at the intradural site. While glial fibrillary acidic protein-immunopositive neuroglial tissues were observed in 2 (patients 1 and 2), peripheral nerve fibers were observed in every stalk. Smooth muscle fibers were noted in patient 1, while a large amount of striated muscle fibers were seen in patients 2 and 3. Conclusion: These cases showed various tissues with different origins in the stalk. There may also be a seamless continuation between fibrocollagenous LDM tissue at the distal site and lipomatous tissue at the proximal site. Peripheral nerve fibers and smooth muscle fibers of neural crest origin may be dragged into the stalk during incomplete disjunction, while the striated muscle fibers of mesodermal origin may enter the stalk along with the lipomatous tissues during premature disjunction.
UR - http://www.scopus.com/inward/record.url?scp=85085355636&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85085355636&partnerID=8YFLogxK
U2 - 10.1016/j.inat.2020.100781
DO - 10.1016/j.inat.2020.100781
M3 - Article
AN - SCOPUS:85085355636
SN - 2214-7519
VL - 21
JO - Interdisciplinary Neurosurgery: Advanced Techniques and Case Management
JF - Interdisciplinary Neurosurgery: Advanced Techniques and Case Management
M1 - 100781
ER -