- Email: [email protected]
A Rare Case of Pediatric Lumbar Spinal Ependymoma Mimicking Meningitis
Accepted Manuscript A Rare Case of Pediatric Lumbar Spinal Ependymoma Mimicking Meningitis Ezeali Mike Ekuma, MD, Kiyoshi Ito, MD, PhD, Akihiro Chiba, MD, Yosuke Hara, MD, Kohei Kanaya, MD, Tetsuyoshi Horiuchi, MD, PhD, Samuel Ohaegbulam, MD, FRCS, Kazuhiro Hongo, MD, PhD PII:
S1878-8750(17)30176-6
DOI:
10.1016/j.wneu.2017.02.016
Reference:
WNEU 5247
To appear in:
World Neurosurgery
Received Date: 29 October 2016 Revised Date:
31 January 2017
Accepted Date: 2 February 2017
Please cite this article as: Ekuma EM, Ito K, Chiba A, Hara Y, Kanaya K, Horiuchi T, Ohaegbulam S, Hongo K, A Rare Case of Pediatric Lumbar Spinal Ependymoma Mimicking Meningitis, World Neurosurgery (2017), doi: 10.1016/j.wneu.2017.02.016. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT
A Rare Case of Pediatric Lumbar Spinal Ependymoma
2
Mimicking Meningitis
3
Ezeali Mike Ekuma, MD1,2, *Kiyoshi Ito, MD, PhD1, Akihiro Chiba, MD1, Yosuke
4
Hara, MD1, Kohei Kanaya, MD1, Tetsuyoshi Horiuchi, MD, PhD1, Samuel
5
Ohaegbulam, MD, FRCS,2 Kazuhiro Hongo, MD, PhD1
6
1
Department of Neurosurgery, Shinshu University School of Medicine, Matsumoto, Japan
7
2
Memfys Hospital for Neurosurgery, Enugu Nigeria
*
Corresponding author: Kiyoshi Ito, MD, Ph.D. Department of Neurosurgery, Shinshu
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University School of Medicine, 3-1-1 Asahi, Matsumoto 390-8621, Japan. Telephone number:
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+81-263-37-2690, Fax number: +81-263-37-0480. e-mail: [email protected]
12
E-mail of co-authors: Ezeali Mike Ekuma- [email protected]; Akihiro Chiba -
13
[email protected];
14
[email protected]; Tetsuyoshi Horiuchi- [email protected]; Samuel Ohaegbulam-
15
[email protected]; Kazuhiro Hongo- [email protected]
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Yosuke
Hara-
[email protected];
Kohei
Kanaya-
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Key Words: acute subarachnoid hemorrhage, bacterial meningitis, children, lumbar spinal
2
ependymoma
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Abstract
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Spontaneous acute subarachnoid hemorrhage (SAH) from lumbar ependymoma in children is
3
rare. We report a case of a14-year-old boy who developed sudden radicular low back pain while
4
playing baseball. He was initially managed conservatively in a local hospital for suspected
5
lumbar disc herniation, but later developed meningeal symptoms and fever before being referred
6
to our hospital. There he underwent a diagnostic lumbar puncture in the emergency room; his
7
cerebrospinal fluid suggested an SAH. Physical examination showed meningeal signs and cauda
8
equina features. Cerebrospinal fluid analysis was negative for bacterial meningitis. Lumbar
9
magnetic resonance imaging revealed a mass characterized as a hemorrhagic lesion. The patient
10
had an emergent evacuation of the mass via the posterior approach. Postoperatively, his
11
symptoms resolved completely. The histological diagnosis was, surprisingly, an ependymoma
12
(WHO grade II). This case is particularly interesting because of its rarity in children, and its
13
pattern of presentation. Though bacterial or viral meningitis is the most frequent cause of
14
meningeal features in children, SAH from a hemorrhagic spinal tumor should be considered.
15
Ultimately, a high index of suspicion is needed for prompt diagnosis.
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Running title: Hemorrhagic lumbar ependymoma in a child presenting with meningeal features
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Sources of funding: This work was supported by JSPS KAKENHI Grant Number 15K10356.
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1
Introduction
Ependymomas are neuroectodermal tumors that represent 3-6% of all central nervous
3
system (CNS) tumors.1 Spinal ependymomas are common in the conus medullaris and filum
4
terminale, with a high incidence in young adults.2 Spontaneous intratumoral and/ or
5
subarachnoid hemorrhage is rare, and in the literature is limited to isolated case reports.3 Clinical
6
features are usually non-specific and depend on the amount and rate of bleeding.4 We report a
7
rare case of a 14-year-old boy who initially presented with exercise-induced, sudden-onset
8
radicular low back pain (LBP), but subsequently developed meningeal features while being
9
managed conservatively for the LBP.
10
Case Report
12
Clinical Presentation and Investigations
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A 14-year-old boy developed a sudden onset LBP radiating down both lower extremities
14
while playing baseball. He was evaluated at a local hospital, where he was initially managed
15
conservatively for suspected lumbar disc herniation. He subsequently developed neck pain and
16
stiffness and low-grade, continuous fever a couple of days later. He was then referred to our
17
facility as a case of suspected meningitis. He underwent a diagnostic lumbar puncture in the
18
emergency room, but the cerebrospinal fluid (CSF) obtained was bloody, suggesting a
19
subarachnoid hemorrhage (SAH). Positive findings on neurological examination were neck
20
stiffness and a positive Kernig’s sign, decreased perianal sensation, lax anal sphincter, reduced
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deep tendon reflexes and sensation in the lower extremities, and a positive straight leg-raising
2
test. Motor function in both lower extremities could not be assessed objectively because of pain.
3
C-reactive protein and other hematological investigations were fairly normal; in addition, CSF
4
biochemistry was negative for bacterial meningitis. Usually, healthy CSF is free of red blood
5
cells, and at most contains only a few white blood cells. However, the patient’s CSF revealed
6
changes including elevated cell counts (724/µl, monocytes: 72, seg: 652). The identified cells
7
were almost all red blood cells. An urgent lumbar magnetic resonance imaging (MRI) was
8
obtained because of the SAH and worsening cauda equina features. The MRI revealed an L3/4
9
heterogenously enhancing mass (Figure 1C and D), with mixed intensity on the T2-weighted
10
image (T2-WI) (Figures 1A) and isointensity on the T1-weighted image (T1-WI) (Figure 1B).
11
Such a pattern is indicative of a hemorrhagic lesion. He was then immediately referred to the
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Neurosurgery department for an urgent surgical intervention.
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Operation and Postoperative Course
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The patient underwent an emergent, complete L2/L3 and partial L1/L4 laminectomy,
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with complete evacuation of the mass after midline splitting of the spinous processes.
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Intraoperative findings included a ribbon-thin dura and intradural hematoma encasing the nerve
17
roots (Figure 2A and B). No definite tumor or abnormal vessels were seen. A specimen was
18
harvested and sent for histopathological diagnosis. Intraoperative monitoring was done using
19
electromyography and the bulbocavernosus reflex; the recordings were normal throughout
20
surgery.
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The histopathological diagnosis was ependymoma, WHO grade II (Figure 3A-D). He did
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not receive adjuvant radiotherapy because the lesion was totally resected as a whole (Figure 1F 5
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and G), and craniospinal MRI taken after surgery showed no dissemination. Postoperatively, his
2
symptoms resolved completely, and he was later discharged with no neurological deficit.
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Discussion
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Spinal tumors can be intradural (45%) or extradural (55%), and the former can further be
5
sub-classified as intra- or extramedullary.5 Ependymomas are the most common spinal
6
intramedullary tumors, representing 3-6% of all CNS tumors.1 They are histologically benign,
7
slow-growing tumors.4 Spinal ependymomas are common in the conus medullaris or filum
8
terminale, with a high incidence in young adults.2 Spinal SAH accounts for less than 1% of all
9
patients with SAH. The more common causes include trauma and vascular abnormalities.6
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Spontaneous SAH from hemorrhagic spinal tumors is rare, with conus medullaris ependymomas
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accounting for a limited number6; they tend to occur between the 2nd and 4th decades of life.7
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Although there have been a few reports in the literature of spinal ependymomas
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presenting as spontaneous SAH, this is to our knowledge the first case of a child developing
14
exercise-induced SAH with features mimicking acute meningitis (Table). The usual presenting
15
symptoms of hemorrhagic spinal ependymomas depend on the amount and rate of bleeding, with
16
significant bleeding resulting in sudden onset LBP with or without neurological deficits.1,4
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Sudden-onset radicular LBP, with concomitant severe headache and a significant quantity of
18
bloody CSF on lumbar puncture (Fincher syndrome),8 should suggest spinal tumoral SAH.
19
Headache was not a prominent feature in this patient. The symptoms were instead neck pain and
20
stiffness, with a low-grade fever, which developed a couple of days after the exercise-induced
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radicular LBP. Our lumbar puncture yielded little bloody CSF, which was suggestive of SAH.
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This, coupled with worsening cauda equina features, necessitated the urgent MRI and subsequent
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referral to the neurosurgical team. It is believed that the patient had acute SAH following a tumor
2
bleed, which then irritated the nerve roots and meninges, leading to LBP and subsequent
3
meningeal symptoms. Different theories have been put forward to explain conus medullaris
4
ependymoma bleeding, with the mechanical and histopathological theories being the most
5
commonly accepted. The former states that the conus medullaris/filum terminale area is a highly
6
mobile segment of the spine, and traction forces which can occur during exercise (such as
7
baseball in this case) may cause disruption of blood vessels on the surface of the tumor. The
8
histopathological theory instead relates the bleeding to the numerous small, thin-walled blood
9
vessels and loss of connective tissue within the tumor.1 Acute bacterial meningitis is not
10
uncommon in children, and is an important cause of morbidity and mortality. On the other hand,
11
acute SAH, which can be easily confused with meningitis because of their similar clinical
12
features, is relatively rare in children compared to adults. However, the pattern of evolution of
13
clinical features, laboratory investigations, and neuroimaging may be able to differentiate
14
between these two clinical entities. Because of the high morbidity and mortality associated with
15
bacterial meningitis in children, a rapid and reliable diagnosis, as well as prompt initiation of
16
potent antibiotics, is required to mitigate its potentially disastrous consequences.9
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The best diagnostic investigation for conus medullaris/filum terminale lesions is MRI,
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either with or without contrast enhancement.10 MRI helped in clinching the diagnosis in this case,
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a fact that underlines the need for thorough clinical evaluation and imaging in cases of acute
20
onset LBP. Spinal ependymomas can disseminate through the subarachnoid space, which
21
necessitates screening of the entire neuraxis; such a procedure was done in the index case, but no
22
dissemination of the tumor was found. The primary treatment for conus medullaris/filum
23
terminale ependymomas is gross total resection (i.e., removal of the tumor as a single, whole
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unit).11,12 Generally, the effect of emergent surgery on outcome is unclear, with a variety of
2
postoperative results seen in reported cases; such cases report the postoperative results following
3
emergency surgery to range from complete neurological recovery12-14 or partial recovery3 to no
4
recovery.15,16 Nevertheless, in the presence of acute compression, the prognosis may be better if
5
surgical decompression is performed emergently, as occurred in this patient. In fact, our patient
6
achieved good neurological recovery.
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Conclusion
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Spontaneous acute SAH from lumbar ependymomas, rather than acute bacterial
9
meningitis, is rare in children. This can present a diagnostic challenge to the unsuspecting doctor
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because both have similar clinical features. The pattern of evolution of clinical features and a
11
high index of suspicion may help develop a prompt diagnosis and treatment.
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Acknowledgements
In writing this report, we express our gratitude to our colleagues in the Department of
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Neurosurgery, Shinshu University School of Medicine.
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Funding
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Japan Society for the Promotion of Science (JSPS) provided financial support in the form
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of Grants-in Aid for Scientific Research: KAKENHI. This work was supported by JSPS
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KAKENHI grant no. 15 K10356.
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Conflicts of interest: None
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References
1.
Kucia EJ, Maughan PH, Kakarla UK, Bambkidis NC, Spetzler RF. Surgical technique and outcomes in the treatment of spinal cord ependymomas: part II; Myxopapillary
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ependymoma. Neurosurgery 2011;68:90-94, discussion 94. 2.
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Meneses MS, Leal AG, Periotto LB, Milano JB, Coel-Neto M, Sobral AC et al.
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Primary filum terminale ependymoma. Arq Neuropsiquiatr 2008;66:529-33. 3.
Ozdemir O, Calisaneller T, Coven I, Altinors N. Posttraumatic intratumoral hemorrhage: an unusual presentation of spinal ependymoma. Eur Spine J
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2007;16:S293-5.
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4.
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Martinez-Perez R, Hernandez-Lain A, Paredes I, Munarriz PM, Castano-Leon AM, Lagares A. Acute neurological deterioration as a result of two synchronous
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hemorrhagic spinal ependymomas. Surg Neurol Int 2012;3:33. 5.
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intradural-extraparenchymal spinal tumors: current best practices and strategies. J
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Neurooncol 2004;69:291-31.
6.
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Parsa AT, Lee J, Parney IF, Weinstein P, McCormick PC, Ames C. Spinal cord and
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Cummings TM, Johnson MH. Neurofibroma manifested by spinal subarachnoid hemorrhage. Am J Roentgenol 1994;162:959-960.
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Bruni P, Esposito S, Oddi G, Hernandez R, Martines F, Patricolo A. Subarachnoid
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hemorrhage from multiple neurofibromas of the cauda equina: case report.
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Neurosurgery 1991;28:910-913. 9
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8.
Fincher EF. Spontaneous subarachnoid hemorrhage in intradural tumors of the lumbar
2 3
sac. A clinical syndrome. J Neurosurg 1951;8:576-584. 9.
Shrestha RG, Tandukar S, Ansari S, Subeditor A, Shrestha A, Poudel R, et al. Bacterial
4 5
meningitis in children under 15 years of age in Nepal. BMC Pediatr 2015;15:94. 10.
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Becco de Souza R, Brasiliero de Aguiar G, Saade N, Veiga JCE. Cauda equina syndrome caused by spontaneous bleeding in the filum terminale myxopapillary
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ependymoma: a rare pediatric case. Pediatr Neurosurg 2012;48:385-8. 11.
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Myxopapillary ependymoma of the conus medullaris with subarachnoid hemorrhage:
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Argyropoulou PI, Argyropoulou MI, Tsampoulas C, Goos P, Manavis I, Efremidis SC.
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MRI in two cases. Neuroradiology 2001;43:489-491. 12.
Malbrain ML, Kamper AM, Lambrecht GL, Hermans P, Baeck E, Verhoeven F, et al. Filum terminale ependymoma revealed by acute cauda compression syndrome
13
following intratumoral and spinal subarachnoid in a patient on oral anticoagulants. Acta
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Neurol Belg 1994;94:35-43.
15
13.
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Rivierez M, Ouestati S, Philippon J, Pradat P, Foncin JF, Muckensturm B, et al. Ependymoma of the intradural filum terminale in adults, 20 cases. Neurochirurgie
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1990;36:96-107.
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14.
Schweitzer JS, Batzdorf U. Ependymoma of the cauda equina region: diagnosis,
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treatment, and outcome in 15 patients. Neurosurgery 1992:30:202-7.
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Heuer GG, Stiefel MF, Bailey RL, Schuster JM. Acute paraparesis from hemorrhagic
spinal ependymoma: diagnostic dilemma and surgical management. Report of two cases and review of literature. J Neurosurg Spine 2007;7:652-5.
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1
16.
Khalatbari MR, Moharamzad Y. Myxopapillary ependymoma of the conus medullaris presenting with intratumoral hemorrhage during weight lifting in a teenager. Childs
3
Nerv Syst 2014;30:181-3.
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Figure Legends
5
Figure1: Preoperative lumbosacral MRI showing an L3/4 intradural mass measuring 21.9 x 10.9
6
x 7.4 mm with compression of the cauda equinae. The mass shows mixed intensity on sagittal
7
(A) T2-W1 and is isointense on sagittal T1-WI (B). It shows heterogenous enhancement on
8
sagittal T1 post-contrast sagittal (C) and axial (D) images. Three months postoperative images
9
(E-G) showed no residual tumor.
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Figure 2: Intraoperative images showing a hemorrhagic intradural mass (arrow) with
11
compression of cauda equinae (A) and complete evacuation of the mass (B).
12
Figure 3:
13
background on hematoxylin and eosin (HE) stain x 4 (A);
14
perivascular pseudorosettes (arrow) and ependymal tubules (B); positive S-100 immunostaining
15
(C); and positive glial fibrillary acid protein immunostaining (D).
showing pockets of hematoma (arrow) on a cellular
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Histopathological images
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HE stain x10 showing few
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Table : Review of the previous two cases of pediatric hemorrhagic lumbar spinal ependymoma Age
Pathology
(years)/Sex
Type of
PF
Clinical
bleeding
Clinical Features
Course
13/F
Mp
Souza et
Intra-
None
tumoral
sensory
USD
surgery
LBP,
UR
Emergent
PR
UR
Emergent
CR
UI
Emergent
CR
Paraplegia
SC Mp
Intra-
Weight
tumoral
lifting
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15/M
et al.,
Acute
Ep
SAH +
(WHO II)
intra-
Acute
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tumoral
Exercise
Absent Present
LBP, sensory level at L1-2
NA
Radicular LBP
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14/M
Absent Absent Paraparesis
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2014[7]
case
motor
numbness
2012[2]
Present
KS
Chronic Absent Absent
al.,
Khalatbari
Outcome
of HA
Becco de
Timing
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Authors
CR: complete recovery; Ep: Ependymoma; F: female; HA: headache; KS: Kernig’s sign; L1-2: 1st to 2nd lumbar vertebrae; LBP: low back pain; M: male; Mp: myxopapillary; NA: not applicable; PF: predisposing factor; PR: partial recovery; SAH: subarachnoid hemorrhage; UI: urinary incontinence; UR: urinary retention; USD: urinary sphincter dysfunction.
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HIGHLIGHTS
A 14-year old boy developed sudden low back pain, then meningeal
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•
symptoms and fever •
MRI showed a lumbar mass causing spontaneous acute subarachnoid
Histopathology showed the mass to be a rare hemorrhagic WHO grade II
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hemorrhage
ependymoma •
Despite meningeal signs, an emergency posterior evacuation produced a full
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recovery
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Abbreviations List: CNS, central nervous system; CSF, cerebrospinal fluid; LBP, lower back pain; MRI, magnetic resonance image; SAH, subarachnoid hemorrhage; WHO, World Health Organization
S1878-8750(17)30176-6
DOI:
10.1016/j.wneu.2017.02.016
Reference:
WNEU 5247
To appear in:
World Neurosurgery
Received Date: 29 October 2016 Revised Date:
31 January 2017
Accepted Date: 2 February 2017
Please cite this article as: Ekuma EM, Ito K, Chiba A, Hara Y, Kanaya K, Horiuchi T, Ohaegbulam S, Hongo K, A Rare Case of Pediatric Lumbar Spinal Ependymoma Mimicking Meningitis, World Neurosurgery (2017), doi: 10.1016/j.wneu.2017.02.016. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT
A Rare Case of Pediatric Lumbar Spinal Ependymoma
2
Mimicking Meningitis
3
Ezeali Mike Ekuma, MD1,2, *Kiyoshi Ito, MD, PhD1, Akihiro Chiba, MD1, Yosuke
4
Hara, MD1, Kohei Kanaya, MD1, Tetsuyoshi Horiuchi, MD, PhD1, Samuel
5
Ohaegbulam, MD, FRCS,2 Kazuhiro Hongo, MD, PhD1
6
1
Department of Neurosurgery, Shinshu University School of Medicine, Matsumoto, Japan
7
2
Memfys Hospital for Neurosurgery, Enugu Nigeria
*
Corresponding author: Kiyoshi Ito, MD, Ph.D. Department of Neurosurgery, Shinshu
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University School of Medicine, 3-1-1 Asahi, Matsumoto 390-8621, Japan. Telephone number:
11
+81-263-37-2690, Fax number: +81-263-37-0480. e-mail: [email protected]
12
E-mail of co-authors: Ezeali Mike Ekuma- [email protected]; Akihiro Chiba -
13
[email protected];
14
[email protected]; Tetsuyoshi Horiuchi- [email protected]; Samuel Ohaegbulam-
15
[email protected]; Kazuhiro Hongo- [email protected]
16
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Yosuke
Hara-
[email protected];
Kohei
Kanaya-
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Key Words: acute subarachnoid hemorrhage, bacterial meningitis, children, lumbar spinal
2
ependymoma
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Abstract
2
Spontaneous acute subarachnoid hemorrhage (SAH) from lumbar ependymoma in children is
3
rare. We report a case of a14-year-old boy who developed sudden radicular low back pain while
4
playing baseball. He was initially managed conservatively in a local hospital for suspected
5
lumbar disc herniation, but later developed meningeal symptoms and fever before being referred
6
to our hospital. There he underwent a diagnostic lumbar puncture in the emergency room; his
7
cerebrospinal fluid suggested an SAH. Physical examination showed meningeal signs and cauda
8
equina features. Cerebrospinal fluid analysis was negative for bacterial meningitis. Lumbar
9
magnetic resonance imaging revealed a mass characterized as a hemorrhagic lesion. The patient
10
had an emergent evacuation of the mass via the posterior approach. Postoperatively, his
11
symptoms resolved completely. The histological diagnosis was, surprisingly, an ependymoma
12
(WHO grade II). This case is particularly interesting because of its rarity in children, and its
13
pattern of presentation. Though bacterial or viral meningitis is the most frequent cause of
14
meningeal features in children, SAH from a hemorrhagic spinal tumor should be considered.
15
Ultimately, a high index of suspicion is needed for prompt diagnosis.
SC
M AN U
TE D
EP AC C
16
RI PT
1
17
Running title: Hemorrhagic lumbar ependymoma in a child presenting with meningeal features
18
Sources of funding: This work was supported by JSPS KAKENHI Grant Number 15K10356.
19
3
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1
Introduction
Ependymomas are neuroectodermal tumors that represent 3-6% of all central nervous
3
system (CNS) tumors.1 Spinal ependymomas are common in the conus medullaris and filum
4
terminale, with a high incidence in young adults.2 Spontaneous intratumoral and/ or
5
subarachnoid hemorrhage is rare, and in the literature is limited to isolated case reports.3 Clinical
6
features are usually non-specific and depend on the amount and rate of bleeding.4 We report a
7
rare case of a 14-year-old boy who initially presented with exercise-induced, sudden-onset
8
radicular low back pain (LBP), but subsequently developed meningeal features while being
9
managed conservatively for the LBP.
10
Case Report
12
Clinical Presentation and Investigations
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11
M AN U
SC
RI PT
2
A 14-year-old boy developed a sudden onset LBP radiating down both lower extremities
14
while playing baseball. He was evaluated at a local hospital, where he was initially managed
15
conservatively for suspected lumbar disc herniation. He subsequently developed neck pain and
16
stiffness and low-grade, continuous fever a couple of days later. He was then referred to our
17
facility as a case of suspected meningitis. He underwent a diagnostic lumbar puncture in the
18
emergency room, but the cerebrospinal fluid (CSF) obtained was bloody, suggesting a
19
subarachnoid hemorrhage (SAH). Positive findings on neurological examination were neck
20
stiffness and a positive Kernig’s sign, decreased perianal sensation, lax anal sphincter, reduced
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EP
13
4
ACCEPTED MANUSCRIPT
deep tendon reflexes and sensation in the lower extremities, and a positive straight leg-raising
2
test. Motor function in both lower extremities could not be assessed objectively because of pain.
3
C-reactive protein and other hematological investigations were fairly normal; in addition, CSF
4
biochemistry was negative for bacterial meningitis. Usually, healthy CSF is free of red blood
5
cells, and at most contains only a few white blood cells. However, the patient’s CSF revealed
6
changes including elevated cell counts (724/µl, monocytes: 72, seg: 652). The identified cells
7
were almost all red blood cells. An urgent lumbar magnetic resonance imaging (MRI) was
8
obtained because of the SAH and worsening cauda equina features. The MRI revealed an L3/4
9
heterogenously enhancing mass (Figure 1C and D), with mixed intensity on the T2-weighted
10
image (T2-WI) (Figures 1A) and isointensity on the T1-weighted image (T1-WI) (Figure 1B).
11
Such a pattern is indicative of a hemorrhagic lesion. He was then immediately referred to the
12
Neurosurgery department for an urgent surgical intervention.
13
Operation and Postoperative Course
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The patient underwent an emergent, complete L2/L3 and partial L1/L4 laminectomy,
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with complete evacuation of the mass after midline splitting of the spinous processes.
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Intraoperative findings included a ribbon-thin dura and intradural hematoma encasing the nerve
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roots (Figure 2A and B). No definite tumor or abnormal vessels were seen. A specimen was
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harvested and sent for histopathological diagnosis. Intraoperative monitoring was done using
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electromyography and the bulbocavernosus reflex; the recordings were normal throughout
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surgery.
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The histopathological diagnosis was ependymoma, WHO grade II (Figure 3A-D). He did
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not receive adjuvant radiotherapy because the lesion was totally resected as a whole (Figure 1F 5
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and G), and craniospinal MRI taken after surgery showed no dissemination. Postoperatively, his
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symptoms resolved completely, and he was later discharged with no neurological deficit.
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Discussion
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Spinal tumors can be intradural (45%) or extradural (55%), and the former can further be
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sub-classified as intra- or extramedullary.5 Ependymomas are the most common spinal
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intramedullary tumors, representing 3-6% of all CNS tumors.1 They are histologically benign,
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slow-growing tumors.4 Spinal ependymomas are common in the conus medullaris or filum
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terminale, with a high incidence in young adults.2 Spinal SAH accounts for less than 1% of all
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patients with SAH. The more common causes include trauma and vascular abnormalities.6
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Spontaneous SAH from hemorrhagic spinal tumors is rare, with conus medullaris ependymomas
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accounting for a limited number6; they tend to occur between the 2nd and 4th decades of life.7
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Although there have been a few reports in the literature of spinal ependymomas
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presenting as spontaneous SAH, this is to our knowledge the first case of a child developing
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exercise-induced SAH with features mimicking acute meningitis (Table). The usual presenting
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symptoms of hemorrhagic spinal ependymomas depend on the amount and rate of bleeding, with
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significant bleeding resulting in sudden onset LBP with or without neurological deficits.1,4
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Sudden-onset radicular LBP, with concomitant severe headache and a significant quantity of
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bloody CSF on lumbar puncture (Fincher syndrome),8 should suggest spinal tumoral SAH.
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Headache was not a prominent feature in this patient. The symptoms were instead neck pain and
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stiffness, with a low-grade fever, which developed a couple of days after the exercise-induced
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radicular LBP. Our lumbar puncture yielded little bloody CSF, which was suggestive of SAH.
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This, coupled with worsening cauda equina features, necessitated the urgent MRI and subsequent
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referral to the neurosurgical team. It is believed that the patient had acute SAH following a tumor
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bleed, which then irritated the nerve roots and meninges, leading to LBP and subsequent
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meningeal symptoms. Different theories have been put forward to explain conus medullaris
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ependymoma bleeding, with the mechanical and histopathological theories being the most
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commonly accepted. The former states that the conus medullaris/filum terminale area is a highly
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mobile segment of the spine, and traction forces which can occur during exercise (such as
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baseball in this case) may cause disruption of blood vessels on the surface of the tumor. The
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histopathological theory instead relates the bleeding to the numerous small, thin-walled blood
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vessels and loss of connective tissue within the tumor.1 Acute bacterial meningitis is not
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uncommon in children, and is an important cause of morbidity and mortality. On the other hand,
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acute SAH, which can be easily confused with meningitis because of their similar clinical
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features, is relatively rare in children compared to adults. However, the pattern of evolution of
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clinical features, laboratory investigations, and neuroimaging may be able to differentiate
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between these two clinical entities. Because of the high morbidity and mortality associated with
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bacterial meningitis in children, a rapid and reliable diagnosis, as well as prompt initiation of
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potent antibiotics, is required to mitigate its potentially disastrous consequences.9
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The best diagnostic investigation for conus medullaris/filum terminale lesions is MRI,
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either with or without contrast enhancement.10 MRI helped in clinching the diagnosis in this case,
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a fact that underlines the need for thorough clinical evaluation and imaging in cases of acute
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onset LBP. Spinal ependymomas can disseminate through the subarachnoid space, which
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necessitates screening of the entire neuraxis; such a procedure was done in the index case, but no
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dissemination of the tumor was found. The primary treatment for conus medullaris/filum
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terminale ependymomas is gross total resection (i.e., removal of the tumor as a single, whole
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unit).11,12 Generally, the effect of emergent surgery on outcome is unclear, with a variety of
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postoperative results seen in reported cases; such cases report the postoperative results following
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emergency surgery to range from complete neurological recovery12-14 or partial recovery3 to no
4
recovery.15,16 Nevertheless, in the presence of acute compression, the prognosis may be better if
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surgical decompression is performed emergently, as occurred in this patient. In fact, our patient
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achieved good neurological recovery.
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Conclusion
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Spontaneous acute SAH from lumbar ependymomas, rather than acute bacterial
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meningitis, is rare in children. This can present a diagnostic challenge to the unsuspecting doctor
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because both have similar clinical features. The pattern of evolution of clinical features and a
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high index of suspicion may help develop a prompt diagnosis and treatment.
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Acknowledgements
In writing this report, we express our gratitude to our colleagues in the Department of
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Neurosurgery, Shinshu University School of Medicine.
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Funding
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Japan Society for the Promotion of Science (JSPS) provided financial support in the form
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of Grants-in Aid for Scientific Research: KAKENHI. This work was supported by JSPS
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KAKENHI grant no. 15 K10356.
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Conflicts of interest: None
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References
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Meneses MS, Leal AG, Periotto LB, Milano JB, Coel-Neto M, Sobral AC et al.
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Martinez-Perez R, Hernandez-Lain A, Paredes I, Munarriz PM, Castano-Leon AM, Lagares A. Acute neurological deterioration as a result of two synchronous
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Neurooncol 2004;69:291-31.
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Shrestha RG, Tandukar S, Ansari S, Subeditor A, Shrestha A, Poudel R, et al. Bacterial
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Becco de Souza R, Brasiliero de Aguiar G, Saade N, Veiga JCE. Cauda equina syndrome caused by spontaneous bleeding in the filum terminale myxopapillary
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Myxopapillary ependymoma of the conus medullaris with subarachnoid hemorrhage:
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Argyropoulou PI, Argyropoulou MI, Tsampoulas C, Goos P, Manavis I, Efremidis SC.
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Malbrain ML, Kamper AM, Lambrecht GL, Hermans P, Baeck E, Verhoeven F, et al. Filum terminale ependymoma revealed by acute cauda compression syndrome
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following intratumoral and spinal subarachnoid in a patient on oral anticoagulants. Acta
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Neurol Belg 1994;94:35-43.
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Rivierez M, Ouestati S, Philippon J, Pradat P, Foncin JF, Muckensturm B, et al. Ependymoma of the intradural filum terminale in adults, 20 cases. Neurochirurgie
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Khalatbari MR, Moharamzad Y. Myxopapillary ependymoma of the conus medullaris presenting with intratumoral hemorrhage during weight lifting in a teenager. Childs
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Nerv Syst 2014;30:181-3.
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Figure Legends
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Figure1: Preoperative lumbosacral MRI showing an L3/4 intradural mass measuring 21.9 x 10.9
6
x 7.4 mm with compression of the cauda equinae. The mass shows mixed intensity on sagittal
7
(A) T2-W1 and is isointense on sagittal T1-WI (B). It shows heterogenous enhancement on
8
sagittal T1 post-contrast sagittal (C) and axial (D) images. Three months postoperative images
9
(E-G) showed no residual tumor.
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Figure 2: Intraoperative images showing a hemorrhagic intradural mass (arrow) with
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compression of cauda equinae (A) and complete evacuation of the mass (B).
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Figure 3:
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background on hematoxylin and eosin (HE) stain x 4 (A);
14
perivascular pseudorosettes (arrow) and ependymal tubules (B); positive S-100 immunostaining
15
(C); and positive glial fibrillary acid protein immunostaining (D).
showing pockets of hematoma (arrow) on a cellular
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HE stain x10 showing few
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Table : Review of the previous two cases of pediatric hemorrhagic lumbar spinal ependymoma Age
Pathology
(years)/Sex
Type of
PF
Clinical
bleeding
Clinical Features
Course
13/F
Mp
Souza et
Intra-
None
tumoral
sensory
USD
surgery
LBP,
UR
Emergent
PR
UR
Emergent
CR
UI
Emergent
CR
Paraplegia
SC Mp
Intra-
Weight
tumoral
lifting
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et al.,
Acute
Ep
SAH +
(WHO II)
intra-
Acute
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tumoral
Exercise
Absent Present
LBP, sensory level at L1-2
NA
Radicular LBP
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Absent Absent Paraparesis
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case
motor
numbness
2012[2]
Present
KS
Chronic Absent Absent
al.,
Khalatbari
Outcome
of HA
Becco de
Timing
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CR: complete recovery; Ep: Ependymoma; F: female; HA: headache; KS: Kernig’s sign; L1-2: 1st to 2nd lumbar vertebrae; LBP: low back pain; M: male; Mp: myxopapillary; NA: not applicable; PF: predisposing factor; PR: partial recovery; SAH: subarachnoid hemorrhage; UI: urinary incontinence; UR: urinary retention; USD: urinary sphincter dysfunction.
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HIGHLIGHTS
A 14-year old boy developed sudden low back pain, then meningeal
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symptoms and fever •
MRI showed a lumbar mass causing spontaneous acute subarachnoid
Histopathology showed the mass to be a rare hemorrhagic WHO grade II
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hemorrhage
ependymoma •
Despite meningeal signs, an emergency posterior evacuation produced a full
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Abbreviations List: CNS, central nervous system; CSF, cerebrospinal fluid; LBP, lower back pain; MRI, magnetic resonance image; SAH, subarachnoid hemorrhage; WHO, World Health Organization