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Published in: Child's Nervous System 4/2020

01-04-2020 | Syringomyelia | Original Article

Terminal syringomyelia associated with lumbar limited dorsal myeloschisis

Authors: Takato Morioka, Nobuya Murakami, Haruhisa Yanagida, Toru Yamaguchi, Yushi Noguchi, Yasushi Takahata, Ayumi Tsukamoto, Satoshi O. Suzuki

Published in: Child's Nervous System | Issue 4/2020

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Abstract

Purpose

Limited dorsal myeloschisis (LDM) is characterized by a fibroneural tethering stalk linking the skin lesion to the underlying spinal cord. Terminal syringomyelia, which is located at the lower third of the cord, is often associated with a tethered cord caused by various spinal dysraphisms; however, terminal syringomyelia has not been documented in LDM. The purpose of this study was to clarify the pathophysiological mechanisms of syringomyelia in LDM.

Methods

In our 16 patients with lumbar LDM, three patients had terminal syringomyelia. We retrospectively analyzed the clinical, neuroradiological, intraoperative, and histopathological findings for these patients, with particular attention to the clinical course of the syrinx.

Results

Patient 1 had a saccular skin lesion and patients 2 and 3 had flat lesions. In all patients, the syringomyelic cavity was located in the lower thoracolumbar cord, immediately rostral to the stalk–cord attachment at the lumbar level. The caudal pole of the syrinx extended to the thickened stalk at the attachment instead of at the caudal cord. Patient 3 had another syrinx in the stalk itself. The longitudinal axis of the syrinx and central canal coincided with the traveling angle of the LDM stalk at the stalk–cord attachment. In patient 1, histology revealed an ependyma-lined central canal in both the LDM stalk and meningocele sac. Patients 1 and 2 underwent syringostomy, but long-term effects were not obtained. Preoperative spontaneous resolution occurred in patient 3.

Conclusions

The histological findings in patient 1 supported the idea that segmental myelocystocele is involved in the development of saccular LDM. The hydromyelic central canal herniates and distends the stalk, resulting in the formation of the myelocystocele. It is possible that the hydromyelic central canal also distends the stalk of flat LDM lesions. The syrinx in patient 3 differed from that in patients 1 and 2, in that the syrinx resolved spontaneously. Further studies are needed to clarify the outcomes of syrinxes associated with LDM stalks.
Literature
1.
go back to reference Erkan K, Unal F, Kiris T, Karalar T (2000) Treatment of terminal syringe in association with tethered cord syndrome: clinical outcomes with and without syrinx drainage. Neurosurg Focus 8(3):E8CrossRef Erkan K, Unal F, Kiris T, Karalar T (2000) Treatment of terminal syringe in association with tethered cord syndrome: clinical outcomes with and without syrinx drainage. Neurosurg Focus 8(3):E8CrossRef
2.
go back to reference Hashiguchi K, Morioka T, Fukui K, Miyagi Y, Mihara F, Yoshiura T, Nagata S, Sasaki T (2005) Usefulness of constructive interference in steady-state (CISS) MR imaging in the presurgical examination for lumbosacral lipoma. J Neurosurg (6 Pediatrics) 103:537–543CrossRef Hashiguchi K, Morioka T, Fukui K, Miyagi Y, Mihara F, Yoshiura T, Nagata S, Sasaki T (2005) Usefulness of constructive interference in steady-state (CISS) MR imaging in the presurgical examination for lumbosacral lipoma. J Neurosurg (6 Pediatrics) 103:537–543CrossRef
3.
go back to reference Hashiguchi K, Morioka T, Yoshida F, Miyagi Y, Mihara F, Yoshiura T, Nagata S, Sasaki T (2007) Feasibility and limitation of constructive interference in steady-state (CISS) MR imaging in neonates with lumbosacral myeloschisis. Neuroradiology 49:579–585CrossRef Hashiguchi K, Morioka T, Yoshida F, Miyagi Y, Mihara F, Yoshiura T, Nagata S, Sasaki T (2007) Feasibility and limitation of constructive interference in steady-state (CISS) MR imaging in neonates with lumbosacral myeloschisis. Neuroradiology 49:579–585CrossRef
4.
go back to reference Hiraoka A, Morioka T, Murakami N, Suzuki SO, Mizoguchi M (2018) Limited dorsal myeloschisis with no extradural stalk linking to a flat skin lesion: a case report. Childs Nerv Syst 34:2497–2501CrossRef Hiraoka A, Morioka T, Murakami N, Suzuki SO, Mizoguchi M (2018) Limited dorsal myeloschisis with no extradural stalk linking to a flat skin lesion: a case report. Childs Nerv Syst 34:2497–2501CrossRef
5.
go back to reference Iskandar BJ, Oakes WJ, McLaughlin C, Osumi AK, Tien RD (1994) Terminal syringomyelia and occult spinal dysraphism. J Neurosurg 81:513–519CrossRef Iskandar BJ, Oakes WJ, McLaughlin C, Osumi AK, Tien RD (1994) Terminal syringomyelia and occult spinal dysraphism. J Neurosurg 81:513–519CrossRef
6.
go back to reference Lee JY, Phi JH, Cheon J-E, Kim S-K, Kim I-O, Cho B-K, Wang K-C (2012) Preuntethering and postuntethering courses of syringomyelia associated with tethered spinal cord. Neurosurgery 71:23–29CrossRef Lee JY, Phi JH, Cheon J-E, Kim S-K, Kim I-O, Cho B-K, Wang K-C (2012) Preuntethering and postuntethering courses of syringomyelia associated with tethered spinal cord. Neurosurgery 71:23–29CrossRef
7.
go back to reference McComb JG (2015) A practical clinical classification of spinal neural tube defects. Childs Nerv Syst 31:1641–1657CrossRef McComb JG (2015) A practical clinical classification of spinal neural tube defects. Childs Nerv Syst 31:1641–1657CrossRef
8.
go back to reference Morioka T, Suzuki SO, Murakami N, Shimogawa T, Mukae N, Inoha S, Sasaguri T, Iihara K (2018) Neurosurgical pathology of limited dorsal myeloschisis. Childs Nerv Syst 34:293–303CrossRef Morioka T, Suzuki SO, Murakami N, Shimogawa T, Mukae N, Inoha S, Sasaguri T, Iihara K (2018) Neurosurgical pathology of limited dorsal myeloschisis. Childs Nerv Syst 34:293–303CrossRef
9.
go back to reference Morioka T, Suzuki SO, Murakami N, Mukae N, Shimogawa T, Haruyama H, Kira R, Iihara K (2019) Surgical histopathology of limited dorsal myeloschisis with flat skin lesion. Childs Nerv Syst 35:119–128CrossRef Morioka T, Suzuki SO, Murakami N, Mukae N, Shimogawa T, Haruyama H, Kira R, Iihara K (2019) Surgical histopathology of limited dorsal myeloschisis with flat skin lesion. Childs Nerv Syst 35:119–128CrossRef
10.
go back to reference 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 29:1905–1914CrossRef 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 29:1905–1914CrossRef
11.
go back to reference Pang D, Zovickian J, Oviedo A, Moes GS (2010) Limited dorsal myeloschisis: a distinctive clinicopathological entity. Neurosurgery 67:1555–1580CrossRef Pang D, Zovickian J, Oviedo A, Moes GS (2010) Limited dorsal myeloschisis: a distinctive clinicopathological entity. Neurosurgery 67:1555–1580CrossRef
12.
go back to reference Pang D, Zovickian J, Wong S-T, Hou YJ, Moes GS (2013) Limited dorsal myeloschisis: a not-so-rare form of primary neurulation defect. Childs Nerv Syst 29:1459–1484CrossRef Pang D, Zovickian J, Wong S-T, Hou YJ, Moes GS (2013) Limited dorsal myeloschisis: a not-so-rare form of primary neurulation defect. Childs Nerv Syst 29:1459–1484CrossRef
13.
go back to reference Quon JL, Grant GA (2019) Commentary: limited dorsal myeloschisis: reconsideration of its embryological origin. Neurosurgery (Epub ahead of print) Quon JL, Grant GA (2019) Commentary: limited dorsal myeloschisis: reconsideration of its embryological origin. Neurosurgery (Epub ahead of print)
14.
go back to reference Rossi A, Piatelli G, Gandolfo C, Pavanello M, Hoffman C, Van Goethem JW, Cama A, Tortori-Donali P (2006) Spectrum of nonterminal myelocystoceles. Neurosurgery 58:509–515CrossRef Rossi A, Piatelli G, Gandolfo C, Pavanello M, Hoffman C, Van Goethem JW, Cama A, Tortori-Donali P (2006) Spectrum of nonterminal myelocystoceles. Neurosurgery 58:509–515CrossRef
15.
go back to reference Sarukawa M, Morioka T, Murakami N, Shimogawa T, Mukae N, Kuga N, Suzuki SO, Iihara K (2019) Human tail-like cutaneous appendage with contiguous stalk of limited dorsal myeloschisis. Childs Nerv Syst 35:973–978 Sarukawa M, Morioka T, Murakami N, Shimogawa T, Mukae N, Kuga N, Suzuki SO, Iihara K (2019) Human tail-like cutaneous appendage with contiguous stalk of limited dorsal myeloschisis. Childs Nerv Syst 35:973–978
16.
go back to reference Steinbok P, Cochrane DD (1995) Cervical meningoceles and myelocystoceles: a unifying hypothesis. Pediatr Neurosurg 23:317–322CrossRef Steinbok P, Cochrane DD (1995) Cervical meningoceles and myelocystoceles: a unifying hypothesis. Pediatr Neurosurg 23:317–322CrossRef
17.
go back to reference Tomita Y, Morioka T, Murakami N, Noguchi Y, Sato Y, Suzuki OS (2019) Slender stalk with combined features of saccular limited dorsal myeloschisis and congenital dermal sinus in a neonate. Pediatr Neurosurg 54:125–131CrossRef Tomita Y, Morioka T, Murakami N, Noguchi Y, Sato Y, Suzuki OS (2019) Slender stalk with combined features of saccular limited dorsal myeloschisis and congenital dermal sinus in a neonate. Pediatr Neurosurg 54:125–131CrossRef
18.
go back to reference Tsitouras V, Sgouros S (2013) Syringomyelia and tethered cord in children. Childs Nerv Syst 29:1625–1634CrossRef Tsitouras V, Sgouros S (2013) Syringomyelia and tethered cord in children. Childs Nerv Syst 29:1625–1634CrossRef
Metadata
Title
Terminal syringomyelia associated with lumbar limited dorsal myeloschisis
Authors
Takato Morioka
Nobuya Murakami
Haruhisa Yanagida
Toru Yamaguchi
Yushi Noguchi
Yasushi Takahata
Ayumi Tsukamoto
Satoshi O. Suzuki
Publication date
01-04-2020
Publisher
Springer Berlin Heidelberg
Keyword
Syringomyelia
Published in
Child's Nervous System / Issue 4/2020
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-019-04297-8

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