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Congenital Spinal Cysts: An Update and Review of the Literature
Sarah E. McNutt1, Oliver D. Mrowczynski2, Jessica Lane2, Ryan Jafrani2, Pratik Rohatgi4, Charles Specht3,
R. Shane Tubbs5, T. Thomas Zacharia6, Elias B. Rizk2
Key words
Arachnoid cysts
- Cysts
- Dermoid cysts
- Enterogenous cysts
- Ependymal cysts
- Epidermoid cysts
- Neurenteric cysts
-
Abbreviations and Acronyms
CSF: Cerebrospinal fluid
CT: Computed tomography
GFAP: Glial fibrillary acidic protein
MRI: Magnetic resonance imaging
From the 1Pennsylvania State College of Medicine, Hershey,
Pennsylvania; Departments of 2Neurosurgery and 3Pathology,
Pennsylvania State Milton S. Hershey Medical Center,
Hershey, Pennsylvania; 4Department of Neurosurgery, Boston
University, Boston, Massachusetts; 5Department of
Neurosurgery, Tulane University, New Orleans, Louisiana;
and 6Seattle Science Foundation, Seattle, Washington, USA
To whom correspondence should be addressed:
Elias B. Rizk, M.D.
[E-mail: erizk@pennstatehealth.psu.edu]
Citation: World Neurosurg. (2020).
https://doi.org/10.1016/j.wneu.2020.08.092
Journal homepage: www.journals.elsevier.com/worldneurosurgery
Available online: www.sciencedirect.com
1878-8750/$ - see front matter ª 2020 Elsevier Inc. All
rights reserved.
INTRODUCTION
Congenital spinal cysts are relatively unusual and rare. These lesions encompass a
wide variety of diseases, including arachnoid cysts, enterogenous cysts, teratomatous cysts, neurenteric cysts, foregut
cysts, bronchogenic cysts, epithelial cysts,
ependymal cysts, dermoid cysts, and
epidermoid cysts. Although many of these
lesions are discovered incidentally on imaging, resulting symptoms include pain,
weakness, ataxia, and bladder incontinence. Patient presentation depends on
the rate of growth and location of
compression on the spinal cord and nerve
roots. Here, we review the epidemiology,
pathology, pathogenesis, and diagnostic
findings of the most common congenital
spinal cysts, followed by a discussion of
their presentation and treatment options.
Congenital spinal cysts are rare and encompass a wide variety of diseases
including arachnoid, enterogenous, teratomatous, neurenteric, foregut, bronchogenic, epithelial, ependymal, dermoid, and epidermoid cysts. Here, we
elucidate the epidemiology, pathology, pathogenesis, and diagnostic findings of
the most common congenital spinal cysts, followed by a discussion of their
presentation and treatment options. Differentiating the cause of each lesion is
crucial for targeted clinical and surgical management for the patient. Our review
describes how arachnoid cysts can be observed, fenestrated, percutaneously
drained, or shunted; however, the primary goal for neurenteric, dermoid, and
epidermoid cysts is removal. Further, we discuss how patient presentation is
dependent on the rate of growth and location of compression on the spinal cord
and nerve roots. However, although many of these lesions are discovered
incidentally on imaging, the spectrum of possible symptoms include pain,
weakness, ataxia, bladder incontinence, and progressive or acute neurologic
deficits. We present and review the histology and imaging of a variety of cysts
and discuss how although the goal of treatment is resection, the risks of surgery
must be considered against the benefits of complete resection in each case.
EPIDEMIOLOGY
Arachnoid Cysts
Spinal arachnoid cysts in pediatric patients
are rare. The first verified cases were reported by Collins and Marks in 1915, who
reported 2 patients, ages 14 and 17 years,
who presented with atypical symptoms.1
Because of the rarity of this condition, the
subsequent body of literature primarily
consists of case reports. The 2 largest case
series in the literature include 22 patients
(Nabors et al.2) and 31 patients (Bond
et al.3). Nabors et al. reported on 22
patients, classifying them into type I
extradural meningeal cysts without nerve
root fibers (n ¼ 9), type II extradural
meningeal cysts with nerve root fibers (n ¼
9), and type III intradural meningeal cysts/
intradural arachnoid cysts lesions (n ¼ 4).2
According to this classification system,
Tarlov perineural cysts or spinal nerve root
diverticula are the same as type II
meningeal cysts.2 Type I meningeal cysts
were further subdivided into IA (extradural
arachnoid
cysts)
and
IB
(sacral
meningoceles
or
occult
sacral
meningoceles). Of Nabors et al.‘s 22
patients, 14 had preoperative computed
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tomography (CT) myelogram, showing
communication between the subarachnoid
and the cyst in 12 patients.2 Of Bond
et al.‘s 31 patients, 58% were intradural
(n ¼ 18) (9 ventral to the spinal cord and 9
dorsal), whereas 36% were extradural (all
dorsal to the spinal cord [n ¼ 11]).3
Thoracic type I cysts are found more
frequently during adolescence, whereas
sacral type I cysts, as well as type II and III at
any location, often are not discovered until
adulthood.2,3 This variation in the onset of
symptoms is postulated to be caused by
the varying diameters of the spinal canal
at these 2 locations.2,3 Intradural cysts are
primarily located in the thoracic region,
whereas extradural cysts are spread
across the thoracic, lumbar, and sacral
spinal canal.3,4 Most arachnoid cysts
are extradural, solitary, and dorsal to
the spinal cord.2-5 However, anterior cysts
are
associated
with
concurrent
myelomeningocele or hydrosyringomyelia
in pediatric patients.2,5
Enterogenous Cysts
Enterogenous cysts are rare congenital
endodermal inclusion cysts of the spinal
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LITERATURE REVIEW
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CONGENITAL SPINAL CYSTS
Table 1. Summary of Congenital Spinal Cysts Cases in the Literature
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Type of Cyst
Cases (n)
Publications (n)
Largest Series (n)
Neurenteric
204
110
13
Epidermoid
177
97
17
Ependymal
17
15
2
Dermoid
343
127
19
Epidermoid
177
97
17
canal with an unknown cause.6 These cysts
have been reported in all age-groups6-9 but
occur more commonly in males than in
females.10,11 The nomenclature used in
the literature includes archenteric cysts,9
enteric cysts,12 enterogenic cysts,13
enterogenous cysts,10,12-28 endodermal
cysts,29 gastrogenic cysts,30 gastrogenous
cysts,31
gastrocytoma,32
neurenteric
cysts,8,11,33-39
teratogenic
cysts,
intestionoma, dorsal enteric fistula, and
neurenteric canal remnant.40-42 Because of
the rarity of these cysts, there are limited
studies in the literature. Brooks et al.
reported on 9 patients (6 male and 3
female) with enterogenous cysts.7 One
patient’s cyst was located exclusively
within the cerebellopontine angle, another
spanned from the cerebellopontine angle
to C2, and the remainder were entirely
intraspinal (4 cervical, 2 thoracic, and 1
lumbar).7 Eight of the 9 cysts were located
anteriorly.7 These cysts have been known
to occur not only in these locations but
also in the posterior mediastinum and
abdomen, with multiple cysts often
occurring within 1 patient.7
The thoracic spine is the most common
location of these lesions, which might reflect
their embryonic relationship to the primitive
Q7
Table 2. Summary Table of Ideal
Primary Diagnostic Tool for Each Cyst
Type of Cyst
Diagnostic Tool of
Choice
Neurenteric
MRI
Enterogenous
MRI
Ependymal
MRI
Dermoid and
epidermoid
Diffusion-weighted MRI
MRI, magnetic resonance imaging.
2
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lung bud from the foregut.24,33 Most
enterogenous cysts are extramedullary,
intradural, and anterior to the spinal
cord.7,8,10,12,23,43 Enterogenous cysts are
often in the lower cervical and upper
thoracic levels and are rarely located within
the cranium or intramedullary.10,14,15,22,23
Enterogenous cysts should be strongly
suspected in patients with anterior spina
bifida and have also been associated with
Klippel-Feil anomaly, diastematomyelia,
posterior spina bifida defects, and other
segmentation fusion anomalies.7,11,19
Ependymal Cysts
Ependymal cysts are also a rare entity, with
most of the literature on the subject being
case studies. Alternative names in the
literature include glioependymal, neuroepithelial, and choroidal epithelial
cysts.44,45 They are typically located
intramedullary44-64 and are anterior or
anterolateral to the spinal cord, off center,
and do not communicate with the central
canal.54,55,60 Ependymal cysts can be
located anywhere along the craniospinal
axis but commonly develop in the conus
medullaris region.44,48,50,60 Associated
spinal or intestinal malformations are not
common.47,53,63
Epidermoid and Dermoid Cysts
Cruveilhier57 first described a spinal
epidural cyst in 1835 as a pearly tumor,
with the appearance of “a pearl of the
finest water.” Epidermoid cysts represent
<1% of all intraspinal tumors.65,66 Most of
the literature on dermoid and epidermoid
cysts involves case reports.67 Mathew and
Todd68 reported the frequency of dermoid
(n ¼ 11) and epidermoid (n ¼ 9) cysts in
the spinal canal from 62 patients with
intradural tumors.
Bostroem proposed to differentiate between dermoid and epidermoid tumors in
1897. However, not until 1949, when Sachs
and Horrax proposed to differentiate dermoid from epidermoid tumors, did this
distinction take hold.69 The difference
between epidermoid and dermoid cysts
is histologic, embryologic, and imaging
based, with little clinical implication.67
However, dermoid cysts are slightly more
common than epidermoid cysts in the
spinal canal.67
The median age of presentation occurs at
35 years but ranges across the life span.65,70
No statistical difference in prevalence
between the sexes has been noted.70 These
cysts are frequently located in the subdural
extramedullary space of the lumbosacral
region66,67 and are less commonly found in
the thoracic and cervical spine or as
intramedullary lesions.65,71 Epidermoid
cysts have a strong association with
additional vertebral defects.65
Summary
All reported congenital spinal cyst cases in
the literature are summarized in Table 1.
References can be found in Appendices
A, B, C, and D.
PATHOGENESIS
Arachnoid Cyst
Extradural arachnoid cysts are usually
congenital but can also be acquired (e.g.,
trauma).72 Elsberg73 described extradural
arachnoid cyst as being composed of
spontaneous herniation of arachnoid
material into the epidural space, through a
defect in the dura mater.3,5 Intradural
arachnoid cysts arise on a congenital basis
via an unclear mechanism and can be
associated with vertebral anomalies, neural
tube defects,5 syringomyelia,4 infection,74
or trauma.75 The congenital theory is
supported by a proven familial association
and with congenital defects.3,5 In
Bright’s,76 and later Starkman et al.’s,77
descriptions of the pathogenesis of
arachnoid cysts, the cause of the cyst was
attributed to an anomalous splitting of the
arachnoid membrane during development,
similar to the prevailing theory for the
pathogenesis of cranial arachnoid cysts.
In addition, the mechanism of cyst
expansion is unknown but multiple theories have been documented. A 1-way-valve
mechanism has been described that allows
filling of the cyst followed by valve closure
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develop when these 2 layers fail to
separate.7,82-88 These cysts are usually
associated with a defect in the anterior
spinal
elements
and
vertebral
anomalies.7,43,89,90
For classification purposes, cysts anterior to the cord in the cervical spine,
without any associated vertebral anomaly,
have been deemed “developmental cysts,”
whereas those posterior to the cord at the
conus, often associated with a developmental vertebral anomaly, have been
called “teratomatous” cysts.8 In addition,
there is often a connection between the
enterogenous cyst and the vertebral
body.56
Figure 1. Masson trichrome, original magnification 500 of an arachnoid
cyst. The meningothelial lining cells of the arachnoid cyst stain red (arrow)
with Masson trichrome. The underlying collagen of the cyst wall is stained
blue.
during pressure surges.3 Alternatively, an
osmotic gradient generated by differences
in protein concentration between the cyst
and subarachnoid space has been
postulated to cause cyst expansion.3 The
cyst itself has been theorized to actively
secrete fluid.3
Enterogenous Cysts
These lesions result from an error during the
third and fourth weeks of neurogenesis.78,79
However, no single hypothesis explains the
development of this malformation.
Multiple explanations have been proposed,
including a persistent primitive neurenteric
canal,28,44,45,48-54,60 adhesions between
ectoderm and endoderm,7 aberrant
vascular supply to the neurenteric tube,80,81
or
notochord
abnormalities.7
The
neurenteric canal initially communicates
the neural and enteric tubes. This canal is
later separated by the notochord; cysts
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Figure 2. Hematoxylin-eosin, original magnification 400 of an
enterogenous cyst. This type of cyst is lined by pseudostratified columnar
epithelium that is often ciliated (arrows).This epithelial lining lies on a
delicate collagenous stroma.
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Ependymal Cysts
The origin of ependymal cyst is hypothesized to be an entrapment during the
invagination of the floor plate.48,55,56,60 This
entrapment becomes isolated, later forming
a cyst, with the location determined by
whether the isolated ependymal tissue is
extramedullary or intramedullary.44,48,54 In
addition, this theory explains how
ependymal cysts can occur along the entire
portion of the spinal canal.44 However,
some
investigators
postulate
that
extramedullary ependymal cyst formation
is caused by glioependymal ectopia.48,54
Cyst expansion is believed to be caused by
active secretion.45,52
Epidermoid and Dermoid Cysts
The genesis of the epidermoid cysts may be
caused by anomalous implantation of ectodermal cells, between the third and fifth
week of embryonic life.65,66,91 Several
hypotheses have been proposed to explain
the development of these cells into
dermoid or epidermoid cysts. It was
believed that the sooner the inclusion
occurs in the embryonic stage, the more
likely it will develop into a dermoid; and,
the later the inclusion occurs, the more
likely it will develop into an epidermoid.92-94
The strong association of epidermoid cyst
with spina bifida, meningomyelocele, syringomyelia, dermal sinus tracts, and other
vertebral anomalies suggests a congenital
origin.65 Since the improvement of lumbar
puncture tools and techniques, epidermoid
and dermoid cysts are rarely iatrogenic
secondary to intraspinal needle puncture or
following surgical procedures because of
epidermal cell implantation into the spinal
canal.65,66,71,95 The growth of the
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Figure 3. Hematoxylin-eosin, original magnification 500 of an
enterogenous cyst. Higher magnification to show cilia (arrow).
epidermoid cyst occurs linearly through the
division and accumulation of normally
dividing cells.65
PATHOLOGY
Arachnoid Cysts
The cyst wall is usually a thin, transparent,
delicate fibrous membrane similar in
appearance to normal arachnoid. With
hematoxylin-eosin staining (Figure 1), the
walls of spinal arachnoid cysts are
typically seen as made of fibrous
connective tissue and lined by arachnoid
meningothelial cells.74,95 Ultrastructural
examination often shows abnormalities
that may or may not otherwise be
apparent, including increased collagen,
hyperplastic meningothelial cells, a lack of
characteristic spiderlike trabeculations,
and
rarely,
small
inflammatory
infiltrates.96 The presence of hemosiderin
within the cyst wall is considered
unrelated to the cause and instead to
result from trauma.2
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SARAH E. MCNUTT ET AL.
Figure 4. Hematoxylin-eosin, original magnification 200 of an epidermoid
cyst. This cyst is lined by keratinizing squamous epithelium (arrows).The
cyst lumen is filled with flaky keratin (star).
4
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Enterogenous Cysts
Histologically, enterogenous cysts are
indicative of the tissue of origin from the
alimentary canal.7 The tissue can be well
differentiated and similar in structure to
stomach, esophagus, or small bowel or
less differentiated. In the welldifferentiated subtype, it presents as a
layer of columnar, pseudostratified, or
stratified cuboidal epithelium cells on a
basement membrane layer with a supporting connective tissue layer (Figures 2
and 3). In the less-differentiated subtype,
it presents as a basement membrane of
mucin-filled, basal-oriented cuboidal or
columnar cells.7,12,22,44 In addition,
enterogenous cysts with features of
respiratory
epithelium
have
been
described,7 as well as features of smooth
and striated muscle, fat, and cartilage.44
Wilkins and Odam12 offered a histologic
classification of enterogenous cysts. Type I
cysts have a simple, pseudostratified,
columnar or cuboidal epithelium with or
without cilia. Type II cysts include the
features of type I cysts, with the addition
of mucous glands, serous glands,
smooth muscle, fat, cartilage, bone,
elastic fibers, lymphoid tissues, or nerve
ganglia.12 Type III cysts include type II
features plus ependymal or glial tissue.12
Type I cysts occur more commonly in the
cervical cord, whereas type II and III
cysts are more prevalent in the lumbar
region.12,97
On immunohistologic staining, if present, mucin-secreting goblet cells can be
detected with periodic acid-Schiff staining
and mucicarmine staining.14,22,44 The
content of the cyst could include pepsin
or acid.18 In addition, enterogenous cysts
are typically positive for cytokeratin and
epithelial membrane antigen.49 Testing
for glial cell markers glial fibrillary acidic
protein (GFAP) and S-100 shows a
negative
result.50
Carcinoembryonic
antigen staining can be, but is not often,
positive.22
Ependymal Cyst
Microscopically, the ependymal cyst wall
consists of either simple cuboidal or
columnar cells, with or without cilia.44,54,60
The use of electron microscopy can be
beneficial
to
further
differentiate
ependymal cysts from endodermal
cysts.48,49 Electron microscopy shows
intercellular junctions, membrane-bound
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previous trauma and a possible inciting
incident of cyst enlargement rather than
a characteristic feature of the ependymal
cyst itself.56
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Epidermoid Cyst
Macroscopically, the lesion is well circumscribed.67 The capsule usually carries
a pearly sheen. The interior of the
capsule is filled with soft, white material
in concentric lamellar layers67; the
contents can be grayish-brown and can
contain calcium deposits.81,98,99
Microscopically, epidermoid cysts usually contain only squamous stratified
epithelium and desquamated epithelial
supported by an outer layer of collagenous
connective tissue (Figures 4 and 5).66
Figure 5. Hematoxylin-eosin, original magnification 400 of an epidermoid
cyst. Higher magnification of cyst wall. Note the granular cell layer (arrow);
these dark blue keratohyalin granules form the keratinous cyst contents
(star).
granules in nonciliated cells, and a lack of
coating on the luminal surfaces of the
cells.48,49,52 Pseudostratification can be
seen.55,60 Generally, lack of a basement
membrane and glycoproteins on light
microscopy are characteristic features of
ependymal cysts.44,48,54,55,64
On immunohistologic staining, the
ependymal cysts show positive uptake
with GFAP and S-100.49,50 The outer lining
cells around the cyst wall stain positive for
CAM5.2, AE1/AE3 keratin (low-molecularweight and high-molecular-weight keratins),
and
epithelial
membrane
antigen.49,50,60,64 Periodic acid-Schiff
stain, Alcain blue, and mucicarmine
staining are negative, because no mucous
or glycoproteins are typically present.44,52
In addition, ependymal cysts are positive
for glial markers GFAP and S-100.50
Similar to arachnoid cysts, the presence
of hemosiderin in the cyst indicates
Dermoid Cysts
Macroscopically, dermoid tumors are round,
smooth, and well defined67,98,99 (Figure 6).
Inside, the contents are usually thickened,
cheesy, and yellowish, with calcium
deposits and hair integuments.67,70,81,98,99
Contents can occasionally be yellow-brown,
mucoid, or even liquid because of the presence of keratin, desquamated epithelium,
and sebum.67,70
Similar to epidermoids, microscopically,
dermoid cysts are lined by stratified squamous epithelium supported by collagen.67
However, dermoids are distinguishable
from epidermoids by their epidermal
adnexa, including hair follicles, and
sebaceous and sweat glands in the cyst
wall.70,81,98,99 Blood vessels have been
noted surrounding the cyst, but never
penetrating the epithelial wall.70
DIAGNOSTIC STUDIES
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Figure 6. Hematoxylin-eosin, original magnification 400 of a dermoid
cyst. Like the epidermoid cyst, this lesion is lined by keratinizing
squamous epithelium. In addition, the walls of dermoid cysts contain
cutaneous adnexa such as a sebaceous gland (star).
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Arachnoid Cyst
Plain radiographs offer little benefit in the
diagnosis of arachnoid cysts, although
some changes can be detected in the
contour of the spinal elements. On CT,
arachnoid cysts appear hypodense and do
not take up contrast. Magnetic resonance
imaging (MRI) is the imaging modality of
choice, with signal characteristics similar
to cerebrospinal fluid (CSF). The cysts are
hypointense on T1 and hyperintense on T2
images, occasionally more so than adjacent CSF spaces because of less flow
within the cyst. They neither restrict on
diffusion-weighted images nor enhance
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Figure 7. Intraspinal arachnoid cyst: sagittal short tau inversion recovery (A),
sagittal T1 postcontrast (B), and axial T2 (C) images showing a dorsal
with contrast (Figure 7). To classify,
Nabors et al. recommend MRI initially to
locate the lesion, followed by CT
myelography to identify if a connection
between the cyst and subarachnoid space
exists.2
Various
other
modalities
including cinematic (cine)-MRI, phasecontrast MRI, and magnetic resonance
myelography can also be used to localize
the connection with the adjacent CSF
spaces and further characterize related
fluid dynamics. Although this strategy
likely plays a more important role in surgical management decisions of symptomatic intracranial arachnoid cysts, there
have been reports of treating extradural
spinal arachnoid cysts via selective closure
of this connection as identified on previously mentioned modalities.100
Enterogenous Cysts
Imaging modalities include plain radiographs (to visualize spinal dysraphism and
myelography), CT scans, and MRI. On CT
scan, enterogenous cysts appear as hypodense, isodense, or hyperdense lesions.12,33,42 The cyst often appears as an
intradural extramedullary multilobulated
lesion that can produce anterior cord
expansion7 or with a widening of the
canal.12 On MRI, enterogenous cysts may
appear as hypointense or hyperintense
lesions, on T1-weighted images, and may
appear hyperintense on T2-weighted
images7,8,20,33,34,54,101
(Figure
8).
Myelography and CT show only an
intradural
extramedullary
mass
6
www.SCIENCEDIRECT.com
thoracic spine arachnoid cyst from T4 to T11. Note the mass effect on the
spinal cord being compressed anteriorly.
compressing the cord but are not
diagnostic.8,12,43,54
Without
MRI,
enterogenous cysts can be misdiagnosed as
syringomyelia.22 In about half of cases,
vertebral anomalies are identified.6-8,23
Ependymal Cyst
MRI is the modality of choice to evaluate
ependymal cysts.55,60,64 The borders of the
cysts are smooth and well defined.48,52,60
They usually present as isointense on T1
and T2 images, with no contrast
enhancement48,50,55,60,64
(Figure
9).
Ependymal cysts can also be detected on
CT as hypodense well-circumscribed lesions, distinct from the spinal cord.48,52
Myelograms are not diagnostically useful
for ependymal cysts.44,53 The off-center
location of the cyst on imaging can be
an important diagnostic factor for ependymal cysts.60
Epidermoid and Dermoid Cysts
Dermoid cysts and, less frequently,
epidermoid cysts can be associated with a
dermal sinus tract.102 The tract is associated
with spinal element defects, often visible on
plain radiographic imaging. CT can
visualize a heterogeneously dense
structure in the spinal canal.103 On MRI,
epidermoid cysts can show a variety of
signals on T1-weighted and T2-weighted
images103-115 (Figures 10 and 11), making
MRI nonspecific in the diagnosis of
epidermoid
and
dermoid
cysts.65
However, hyperintensity on T1 for an
epidermoid cyst is rare65 but common for
dermoid cysts.67 Still, it is difficult to
discern between an epidermoid and
dermoid cyst on MRI.67 Arachnoid cysts
could have similar characteristics to
epidermoid or dermoid cysts.113 Diffusion
imaging on MRI can differentiate between
arachnoid and epidermoid-dermoid cysts,
because arachnoid cysts do not
restrict.113,116-118
Signs and Symptoms
Congenital spinal cysts can present with a
wide range of symptoms related to
compression of neural or vertebral elements. These symptoms include back
pain, radicular pain, paresthesia, weakness, gait disturbance, and bowel or
bladder dysfunction. Additional considerations for the different entities are presented in the following sections.
Arachnoid Cysts
Most patients with arachnoid cysts are
asymptomatic and incidentally discovered.3,77 In addition, cyst size is not
correlated with signs and symptoms.119 In
a large series of symptomatic spinal
arachnoid cysts, Bond et al.3 identified 31
patients, 21 of whom initially presented
with radiculopathy or myelopathy (68%).
Additional symptoms included pain
(42%), lower limb weakness (39%), ataxia
(32%), and spasticity (19%). Only 10%
experienced sensory loss and 7%
experienced bladder dysfunction. In
addition to presenting with the cardinal
symptoms of spinal lesions, unusual and
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Figure 8. Intraspinal enterogenous cyst: sagittal T2 (A),
sagittal T1 postcontrast with fat saturation (B), axial T1
(C), and axial short tau inversion recovery images
Figure 9. Intraspinal ependymal cyst: sagittal short tau inversion recovery
(A), axial T2 (B), and axial T1 postcontrast with fat saturation (C) images
WORLD NEUROSURGERY -: ---, MONTH 2020
showing a sacral spine enterogenous cyst within the
sacral spinal canal at S3-S4 with the expansion of spinal
canal and osseous remodeling.
Q5
showing a dorsal thoracic spine intramedullary ependymal cyst at T11
measuring approximately 1 cm.
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Figure 10. Intraspinal epidermoid cyst: sagittal T2 (A), sagittal T1
postcontrast (B), and axial T2 (C) images showing a dorsal thoracic spine
insidious presentation of spinal arachnoid
cysts is well documented. Collins and
Mark1 reported 1 patient who experienced
gait instability at 17 years of age that
progressed over 4 months with right
weaker than left. No other symptoms were
present. These investigators’ other patient
reported daily precordia pain at the age of
11 years, which at age 14 years progressed
to bilateral foot drop, spasticity, and
occasional incontinence, resulting in the
inability to walk.1 The case report was the
beginning of a series of reports of patients
who did not present with the typical
radiculopathy and instead experienced
minor
sensory
disturbances.
Subsequently, Nabors et al.’s cohort of 22
patients found that type I extradural cysts
presented primarily with low back pain,
and type II and III often presented either
asymptomatically or with sciatica and
associated bowel or bladder problems.2
Type II cysts, which can occur anywhere,
are most frequently symptomatic in the
sacrum.2
Enterogenous Cysts
Enterogenous cysts, either developmental or
teratomatous, often present with signs and
symptoms of cord compression with associated relapsing and remitting pain.8,19 The
relapsing and remitting symptoms can
make an enterogenous cyst difficult to
distinguish from multiple sclerosis.11
Patients often present in their 20s with
symptoms mimicking more aggressive
tumors.14 Respiratory symptoms are the
most common for tumors in the
8
www.SCIENCEDIRECT.com
acquired epidermoid cyst at T2-T3 deep to laminectomy defect of previous
dermal sinus tract resection. Note mass effect.
mediastinum, including episodes of
cyanosis, dyspnea, cough, or hemoptysis.21
Hemorrhage into the alimentary canal,
postprandial pain, and failure to thrive can
also occur.21 An enterogenous cyst is rarely
discovered incidentally.21 Other uncommon
presentations include unexplained fever or
recurrent bacterial meningitis if a
connection between the enterogenous cyst
and the alimentary canal exists, aseptic
meningitis with cyst rupture, or acute
neurologic deterioration from cyst rupture
or hemorrhage.44
Ependymal Cyst
There is no standard age for presenting
symptoms of ependymal cysts to occur.56
However, trauma is often a precipitating
factor for signs and symptoms to
appear.54,56,63 A range of symptoms for
ependymal cysts have been reported,
including radicular pain, abdominal pain,
weakness, and lower back pain.44 Pain is
the most common presenting symptom.54
Comparably, ependymal cysts often
present with less severe symptoms
compared with the other spinal cysts.44,53
Dermoid/Epidermoid Cyst
Aseptic meningitis caused by cyst rupture
can be a presenting sign of dermoid and
epidermoid cysts.44 However, lower back
pain is the most common presenting
symptom.71 Because of the slow-growing
nature of these tumors, adult presentation is most common, but presenting
symptoms are otherwise similar to any
other lesion in the spinal column.66
TREATMENT
Arachnoid Cysts
Surgical treatment of arachnoid cysts is
generally recommended only for symptomatic lesions.5 Surgical options are
numerous
and
include
resection,
fenestration,
shunting
to
various
locations, and percutaneous drainage. In
general, extradural arachnoid cysts are
typically resected, whereas intradural or
anterior cysts are typically fenestrated. As
would be expected, percutaneous drainage
is rarely favored because of high
recurrence. Surgical removal or partial
resection typically results in a 45%e70%
reduction of symptoms and 20%e30%
complete
symptom
resolution.3
Recurrence even after total resection is
possible.2,3 Specific surgical goals are
dictated by cyst subtype and relationship
with adjacent neural structures. The
removal of communication between the
cyst and subarachnoid space is the
primary goal in type I extradural
arachnoid cysts, and additional dissection
and complete removal are often feasible.
Type II cysts (extradural with associated
nerve fibers) and intradural type III cysts
often dictate deciding between complete
resection and preservation of function2,3
frequently necessitating partial resection/
fenestration or shunting type procedures.
Enterogenous, Ependymal, Epidermoid,
and Dermoid Cysts
Surgical considerations of the other entities discussed share many similarities.
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Figure 11. Intraspinal dermoid cyst: sagittal T1
precontrast (A), sagittal short tau inversion recovery
(B), axial T1 (C), and axial computed tomography (D)
images showing a thoracic and lumbar spine dermoid
cyst from T11 to L3 with the expansion of the spinal
canal. Note high T1 signal within the cyst (A, C) relative
As for arachnoid cysts, surgery is the
appropriate treatment for symptomatic
cysts. Although the goal is complete
removal, it is not always possible to achieve this safely. However, partial treatment
to decompress neural elements has favorable results but may require repeat procedures in the future.
Surgical outcomes for enterogenous
cysts are generally good, with 70% of patients who undergo resection having resolution of preoperative neurologic
deficits.43 The resolution of even severe
deficits after removal of an enterogenous
cyst has been well documented, even
with incomplete resection.7,10 Surgery
to cerebrospinal fluid with suppression on short tau
inversion recovery images (B). Dense calcifications are
seen on computed tomography images (D). Mass
effect on spinal cord and evidence of previous
decompression (AeC).
can also prevent rarer sequelae of
recurrent bacterial meningitis, intracystic
hemorrhage, or rupture leading to
chemical meningitis or irreversible
neurologic deficits.10
Ependymal cysts resection often presents a challenge because of lack of a
plane between the cyst and the spinal
cord, rendering total removal not always
possible.44,52,53 In this case, fenestration
or marsupialization between the cyst
cavity and subarachnoid space is
performed, with or without biopsy of the
cyst wall; however, this is more likely to
require repeated procedures, as would be
expected.44
WORLD NEUROSURGERY -: ---, MONTH 2020
Similarly, dermoid and epidermoid
cysts are commonly adherent to medullary
structures, often making total resection
difficult.66,68 If complete removal is
deemed impossible, partial resection can
give the patient years of relief.65
Differentiating among these lesions is
crucial for clinical and surgical management. Symptomatic congenital cysts
should likely be operated on with a decision on type of surgery, approach, and
degree of resection tailored to lesion
cause, subtype, location, and adherence to
neural structures. Although a surgical cure
is the goal, complete excision should be
weighed against surgical morbidity in each
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case. Recurrence is possible, albeit rare,
even in complete removal of cysts. All
patients should be monitored closely after
surgical intervention.
8. Pierot L, Dormont D, Queslati S, Cornu P,
Rivierez M, Bories J. Gadolinium-DTPA
enhanced MR imaging of intradural neurenteric
cysts. J Comput Assist Tomogr. 1988;12:762-764.
9. Hutchison J, Thomson J. Congenital archenteric
cysts. Br J Surg. 1953;41:15-20.
CONCLUSIONS
Our review describes the epidemiology,
pathogenesis, histology, imaging, signs
and symptoms, and treatment strategies
of congenital spinal cysts. In the pediatric
population, symptomatic congenital spinal cysts are rare. Patient presentation
could vary from an incidental finding to
progressive or acute neurologic findings.
A detailed history, physical and neurologic
examination, adequate imaging workup,
and, if necessary, urodynamic function
testing, are essential in the workup of
these entities. Pathology and imaging
studies are critical in the diagnosis of the
lesion. The goal of treatment is safe surgical resection. The alternative to total
resection is partial resection, decompression, or continued observation of the cyst
when the risk of surgical injury outweighs
the benefits of complete resection.
UNCITED TABLE
11. Geremia GK, Russell EJ, Clasen RA. MR imaging
characteristics of a neurenteric cyst. AJNR Am J
Neuroradiol. 1988;9:978-980.
12. Aoki S, Machida T, Sasaki Y, et al. Enterogenous
cyst of cervical spine: clinical and radiological
aspects (including CT and MRI). Neuroradiology.
1987;29:291-293.
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Conflict of interest statement: The authors declare that the
article content was composed in the absence of any
commercial or financial relationships that could be construed
as a potential conflict of interest.
Received 17 July 2020; accepted 13 August 2020
Citation: World Neurosurg. (2020).
https://doi.org/10.1016/j.wneu.2020.08.092
Journal homepage: www.journals.elsevier.com/worldneurosurgery
Available online: www.sciencedirect.com
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spinal cord compression in a child. J Spinal Cord
Med. 2008;31:306-308.
31. Dev R, Singh G, Singh SK, Mamgain A. Cystothecostomy: a new technique to treat long
segment spinal extradural arachnoid cyst. Br J
Neurosurg. 2008;22:585-587.
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39. Yabuki S, Kikuchi S. Multiple extradural arachnoid cysts: report of two operated cousin cases.
Spine. 2007;32:E585-E588.
40. de Oliveira RS, Amato MC, Santos MV,
Simão GN, Machado HR. Extradural arachnoid
cysts in children. Childs Nerv Syst. 2007;23:
1233-1238.
41. Ghannane H, Haddi M, Aniba K, Lmejjati M, Aït
Benali S. Symptomatic intramedullary arachnoid
cyst. Report of two cases and literature review.
Neurochirurgie. 2007;53:54-57.
42. Yabuki S, Kikuchi S, Ikegawa S. Spinal extradural
arachnoid cysts associated with distichiasis and
lymphedema. Am J Med Genet A. 2007;143A:
884-887.
43. Suryaningtyas W, Arifin M. Multiple spinal extradural arachnoid cysts occurring in a child.
J Neurosurg. 2007;106(2 suppl):158-161.
44. Guzel A, Tatli M, Yilmaz F, Bavbek M. Unusual
presentation of cervical spinal intramedullary
arachnoid cyst in childhood: case report and review of the literature. Pediatr Neurosurg. 2007;43:
50-53.
45. Maiuri F, Iaconetta G, Esposito M. Neurological
picture. Recurrent episodes of sudden tetraplegia
caused by an anterior cervical arachnoid cyst.
J Neurol Neurosurg Psychiatry. 2006;77:1185-1186.
46. Nejat F, Cigarchi SZ, Kazmi SS. Posterior spinal
cord herniation into an extradural thoracic
arachnoid cyst: surgical treatment. Case report
and review of the literature. J Neurosurg. 2006;104(3
suppl):210-211.
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CONGENITAL SPINAL CYSTS
47. Apel K, Sgouros S. Extradural spinal arachnoid
cysts associated with spina bifida occulta. Acta
Neurochir (Wien). 2006;148:221-226.
48. Liu JK, Cole CD, Sherr GT, Kestle JR, Walker ML.
Noncommunicating spinal extradural arachnoid
cyst causing spinal cord compression in a child.
J Neurosurg. 2005;103(3 suppl):266-269.
49. Takagaki T, Nomura T, Toh E, Watanabe M,
Mochida J. Multiple extradural arachnoid cysts at
the spinal cord and cauda equina levels in the
young. Spinal Cord. 2006;44:59-62.
50. Boueva A, Cochat P, Gabrovski S. Neurogenic
bladder in an infant due to spinal arachnoid cyst.
Pediatr Nephrol. 2005;20:1195-1197.
51. Sharma A, Karande S, Sayal P, Ranadive N,
Dwivedi N. Spinal intramedullary arachnoid cyst
in a 4-year-old girl: a rare cause of treatable acute
quadriparesis: case report. J Neurosurg. 2005;102(4
suppl):403-406.
52. Kumar R, Singh V. Benign intradural extramedullary masses in children of northern India.
Pediatr Neurosurg. 2005;41:22-28.
53. Nakagawa A, Kusaka Y, Jokura H, Shirane R,
Tominaga T. Usefulness of constructive interference in steady state (CISS) imaging for the diagnosis and treatment of a large extradural spinal
arachnoid cyst. Minim Invasive Neurosurg. 2004;47:
369-372.
54. Sharma A, Sayal P, Badhe P, Pandey A, Diyora B,
Ingale H. Spinal intramedullary arachnoid cyst.
Indian J Pediatr. 2004;71:e65-e67.
55. Chang IC. Surgical experience in symptomatic
congenital intraspinal cysts. Pediatr Neurosurg.
2004;40:165-170.
56. Muthukumar N. Anterior cervical arachnoid cyst
presenting with traumatic quadriplegia. Childs Nerv
Syst. 2004;20:757-760.
57. Brunk I, Stöver B, Ikonomidou C, Brinckmann J,
Neumann LM. Ehlers-Danlos syndrome type VI
with cystic malformations of the meninges in a 7year-old girl. Eur J Pediatr. 2004;163:214-217.
58. Takahashi S, Morikawa S, Egawa M, Saruhashi Y,
Matsusue Y. Magnetic resonance imaging-guided
percutaneous fenestration of a cervical intradural
cyst. J Neurosurg. 2003;99(3 suppl):313-315.
63. Gómez-Escalonilla Escobar CI, GiménezTorres MJ, García-Morales I, Galán-Dávila L,
Floriach M, Mateos-Beato F. Intradural spinal
arachnoid cyst associated with Noonan’s syndrome. Rev Neurol. 2001;32:833-835.
64. Gelabert-González M, Cutrín-Prieto JM, GarcíaAllut A. Spinal arachnoid cyst without neural tube
defect. Childs Nerv Syst. 2001;17:179-181.
65. Tsutsumi S, Wachi A, Uto A, Koike J, Arai H,
Sato K. Infantile arachnoid cyst compressing the
sacral nerve root associated with spina bifida and
lipomaecase report. Neurol Med Chir. 2000;40:
435-438.
66. Ziv T, Watemberg N, Constantini S, LermanSagie T. Cauda equina syndrome due to lumbosacral arachnoid cysts in children. Eur J Paediatr
Neurol. 1999;3:281-284.
67. Kazan S, Ozdemir O, Akyüz M, Tuncer R. Spinal
intradural arachnoid cysts located anterior to the
cervical spinal cord. Report of two cases and review of the literature. J Neurosurg. 1999;91(2 suppl):
211-215.
68. Mittler MA, McComb JG. Adjacent thoracic neuroenteric and arachnoid cysts. Pediatr Neurosurg.
1999;30:164-165.
69. Myles LM, Gupta N, Armstrong D, Rutka JT.
Multiple extradural arachnoid cysts as a cause of
spinal cord compression in a child. J Neurosurg.
1999;91(1 suppl):116-120.
70. Aithala GR, Sztriha L, Amirlak I, Devadas K,
Ohlsson I. Spinal arachnoid cyst with weakness in
the limbs and abdominal pain. Pediatr Neurol. 1999;
20:155-156.
71. Jean WC, Keene CD, Haines SJ. Cervical arachnoid
cysts after craniocervical decompression for Chiari
II malformations: report of three cases. Neurosurgery. 1998;43:941-944.
72. Adelson PD, Firlik KS, Firlik AD, Hamilton RL.
A meningeal cyst of the thoracic spine presenting
as prolonged paresis after ankle injury: case
report. Neuropediatrics. 1996;27:207-210.
73. Rabb CH, McComb JG, Raffel C, Kennedy JG.
Spinal arachnoid cysts in the pediatric age group:
an association with neural tube defects.
J Neurosurg. 1992;77:369-372.
59. Fujimura M, Kusaka Y, Shirane R. Spinal lipoma
associated with terminal syringohydromyelia and
a spinal arachnoid cyst in a patient with cloacal
exstrophy. Childs Nerv Syst. 2003;19:254-257.
74. Osenbach RK, Godersky JC, Traynelis VC,
Schelper RD. Intradural extramedullary cysts of
the spinal canal: clinical presentation, radiographic diagnosis, and surgical management.
Neurosurgery. 1992;30:35-42.
60. Miravet E, Sinisterra S, Birchansky S, et al. Cervicothoracic extradural arachnoid cyst: possible
association with obstetric brachial plexus palsy.
J Child Neurol. 2002;17:770-772.
75. Shih DY, Chen HJ, Lee TC, Chen L. Congenital
spinal arachnoid cysts: report of 2 cases with review of the literature. J Formos Med Assoc. 1990;89:
588-592.
61. Lee HJ, Cho DY. Symptomatic spinal intradural
arachnoid cysts in the pediatric age group:
description of three new cases and review of the
literature. Pediatr Neurosurg. 2001;35:181-187.
76. Jena A, Gupta RK, Sharma A, Prakesh VE,
Khushu S. Magnetic resonance diagnosis of spinal
arachnoid cyst. A report of two cases. Childs Nerv
Syst. 1990;6:107-109.
62. Baysefer A, Izci Y, Erdogan E. Lateral intrathoracic
meningocele associated with a spinal intradural
arachnoid cyst. Pediatr Neurosurg. 2001;35:107-110.
77. Gray L, Djang WT, Friedman AH. MR imaging of
thoracic extradural arachnoid cysts. J Comput Assist
Tomogr. 1988;12:646-648.
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78. Nabors MW, Pait TG, Byrd EB, et al. Updated
assessment and current classification of spinal
meningeal cysts. J Neurosurg. 1988;68:366-377.
79. Alvisi C, Cerisoli M, Giulioni M, Guerra L. Longterm results of surgically treated congenital
intradural spinal arachnoid cysts. J Neurosurg. 1987;
67:333-335.
80. Lesbros D, Guillaud R, Frerebeau P. Spinal
intradural arachnoid cyst. Arch Fr Pediatr. 1985;42:
309-311.
81. Schroeder KA, Venes JL. Multiple intradural
arachnoid diverticuli: the need for complete
myelography. Neurosurgery. 1984;15:863-867.
82. Swamy KS, Reddy AK, Srivastava VK, Das BS,
Reddy GN. Intraspinal arachnoid cysts. Clin Neurol
Neurosurg. 1984;86:143-148.
83. Yoshioka H, Iino S, Ishimura K, et al. An arachnoid cyst in an 8-year-old boy with neurofibromatosis. Brain Dev. 1984;6:551-553.
84. Roski RA, Rekate HL, Kurczynski TW,
Kaufman B. Extradural meningeal cyst. Case
report and review of the literature. Childs Brain.
1984;11:270-279.
85. Fortuna A, Mercuri S. Intradural spinal cysts. Acta
Neurochir (Wien). 1983;68:289-314.
86. McCrum C, Williams B. Spinal extradural arachnoid pouches. Report of two cases. J Neurosurg.
1982;57:849-852.
87. Kendall BE, Valentine AR, Keis B. Spinal arachnoid cysts: clinical and radiological correlation
with prognosis. Neuroradiology. 1982;22:225-234.
88. Schwartz JF, O’Brien MS, Hoffman JC Jr. Hereditary spinal arachnoid cysts, distichiasis, and
lymphedema. Ann Neurol. 1980;7:340-343.
89. Lee MG, Cancina JE. Extradural spinal arachnoidal cyst. West Indian Med J. 1980;29:67-72.
90. Duncan AW, Hoare RD. Spinal arachnoid cysts in
children. Radiology. 1978;126:423-429.
91. Herskowitz J, Bielawski MA, Venna N, Sabin TD.
Anterior cervical arachnoid cyst simulating syringomyelia: a case with preceding posterior arachnoid cysts. Arch Neurol. 1978;35:57-58.
92. Palmer JJ. Spinal arachnoid cysts. Report of six
cases. J Neurosurg. 1974;41:728-735.
APPENDIX B: REPORTED CASES OF
ENTEROGENOUS CYSTS IN THE
LITERATURE
1. Vasani V, Konar S, Nandeesh BN, Praharaj SS.
Multiple neurenteric cysts along the spinal axis of
an infant: a rare entity. Pediatr Neurosurg. 2019;54:
121-124.
2. Ozalp H, Hamzaoglu V, Karatas D, Dagtekin A,
Yildız M, Avcı E. Rare cause of acute tetraplegia
and respiratory arrest: cervicomedullary neuroenteric cyst in a child. NMC Case Rep J. 2018;6:1-4.
3. El Ahmadieh TY, Sillero R, Kafka B, Aoun SG,
Price AV. Isolated dorsal thoracic neuroenteric cyst
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CONGENITAL SPINAL CYSTS
with spinal cord compression: case reports in pediatrics. World Neurosurg. 2018;118:296-300.
intramedullary neurenteric cysts. J Neurosurg Spine.
2015;23:99-110.
4. Zbair S, Adnane A, Chbani K, Salam S,
Ouzidane L. Forme rare du dysraphisme spinal
fermé: la diastématomyélie. [A rare form of closed
spinal dysraphism: diastematomyelia]. Pan Afr Med
J. 2017;28:317 [in French].
19. Srinivas H, Kumar A. Silent neurenteric cyst with
split cord malformation at conus medullaris: case
report and literature review. J Pediatr Neurosci. 2014;
9:246-248.
5. Lan ZG, Richard SA, Lei C, Huang S. Thoracolumbar spinal neurenteric cyst with tethered
cord syndrome and extreme cervical lordosis in a
child: a case report and literature review. Medicine.
2018;97:e0489.
6. Gundapaneni S, Jain V, Sharma S, Gupta DK.
Perforated neuroenteric cyst masquerading as
congenital pulmonary airway malformation. BMJ
Case Rep. 2017;2017. bcr2017222537.
7. Lai PMR, Zaazoue MA, Francois R, et al. Neurenteric cyst at the dorsal craniocervical junction in a
child: case report. J Clin Neurosci. 2018;48:86-89.
20. Dokumcu Z, Uygun O, Turhan T, Yalaz M,
Ozcan C, Erdener A. Two-stage approach in the
management of thoracic neuroenteric cyst with
spinal
extension:
thoracoscopic
excision
following dorsal laminectomy. Childs Nerv Syst.
2015;31:185-189.
21. Sarici D, Akin MA, Kurtoglu S, et al. Iodine deficiency: a probable cause of neural tube defect.
Childs Nerv Syst. 2013;29:1027-1030.
22. Okechi H, Albright AL, Nzioka A. Tethered cord
syndrome secondary to the unusual constellation
of a split cord malformation, lumbar myelomeningocele, and coexisting neurenteric cyst. Case
Rep Neurol Med. 2012;2012:635029.
8. Weng JC, Ma JP, Hao SY, et al. Intradural extramedullary bronchogenic cyst: clinical and radiologic characteristics, surgical outcomes, and
literature review. World Neurosurg. 2018;109:
e571-e580.
23. Kikkawa Y, Nakamizo A, Suzuki SO, et al. Spinal
endodermal cyst resembling an arachnoid cyst in
appearance: pitfalls in intraoperative diagnosis of
cystic lesions. Surg Neurol Int. 2012;3:78.
9. Morioka T, Suzuki SO, Murakami N, et al. Neurosurgical pathology of limited dorsal myeloschisis.
Childs Nerv Syst. 2018;34:293-303.
24. Jhawar SS, Mahore A, Goel A. Intramedullary
spinal neurenteric cyst with fluid-fluid level.
J Neurosurg Pediatr. 2012;9:542-545.
10. Esfahani DR, Burokas L, Brown HG, Hahn YS,
Nikas D. Management of an unusual, recurrent
neurenteric cyst in an infant: case report and review of the literature. Childs Nerv Syst. 2017;33:
1603-1607.
25. Zipkin R. A 3-month-old infant with upper extremity weakness. Pediatr Ann. 2012;41:e1-e4.
11. Joshi KC, Singh D, Suggala S, Mewada T. A rare
case of solid calcified intramedullary neurenteric
cyst: case report and technical note. Asian J Neurosurg. 2017;12:290-292.
12. Singh H, Patir R, Vaishya S, Gupta A, Miglani R.
Application of a far-lateral approach to the subaxial spine: application, technical difficulties, and
results. World Neurosurg. 2017;100:167-172.
13. Caro-Domínguez P, Bass J, Hurteau-Miller J.
Currarino syndrome in a fetus, infant, child, and
adolescent: spectrum of clinical presentations and
imaging findings. Can Assoc Radiol J. 2017;68:90-95.
14. Al Qahtani HM, Suliman Aljoqiman K, Arabi H, Al
Shaalan H, Singh S. Fatal meningitis in a 14month-old with Currarino triad. Case Rep Radiol.
2016;2016:1346895.
15. Kojima S, Yoshimura J, Takao T, et al. Mobile
spinal enterogenous cyst resulting in intermittent
paraplegia in a child: case report. J Neurosurg
Pediatr. 2016;18:448-451.
16. Sathe PA, Ghodke RK, Laxmilal VN,
Kandalkar BM, Vinod PS. An unusual presacral
cyst in an infant. J Clin Diagn Res. 2015;9:
ED07-ED08.
17. Tumturk A, Kaya Ozcora G, Kacar Bayram A, et al.
Torticollis in children: an alert symptom not to be
turned away. Childs Nerv Syst. 2015;31:1461-1470.
18. Yang T, Wu L, Fang J, Yang C, Deng X, Xu Y.
Clinical presentation and surgical outcomes of
26. Savardekar A, Salunke P, Rane S, Chhabra R.
Dorsally placed extradural infected neurenteric
cyst in a two-year old with paraspinal extension.
Neurol India. 2012;60:129131.
27. Ito K, Aoyama T, Kiuchi T, et al. Ventral intradural endodermal cyst in the cervical spine treated
with anterior corpectomyecase report. Neurol Med
Chir. 2011;51:863-866.
28. Zenmyo M, Ishido Y, Yamamoto T, et al. Intradural neurenteric cystetwo case reports of surgical treatment. Int J Neurosci. 2010;120:625-629.
29. Theret E, Litre CF, Lefebvre F, et al. Huge intramedullar neurenteric cyst with intrathoracic
development in a 1 month-old boy: excision
though the anterior approach. A case report and
review of the literature. Acta Neurochir (Wien). 2010;
152:481-483.
30. Cai C, Shen C, Yang W, Zhang Q, Hu X. Intraspinal neurenteric cysts in children. Can J Neurol
Sci. 2008;35:609-615.
31. Akil H, Mahon B, Wickremesekera A. Anterior
cervicothoracic approach to an upper thoracic
spinal endodermal cyst. J Clin Neurosci. 2009;16:
557-559.
32. Sheaufung S, Taufiq A, Nawawi O, Naicker MS,
Waran V. Neurenteric cyst of the cervicothoracic
junction: a rare cause of paraparesis in a paediatric patient. J Clin Neurosci. 2009;16:579-581.
33. Takahashi S, Morikawa S, Saruhashi Y,
Matsusue Y, Kawakami M. Percutaneous transthoracic fenestration of an intramedullary neurenteric cyst in the thoracic spine with
WORLD NEUROSURGERY -: ---, MONTH 2020
intraoperative magnetic resonance image navigation and thoracoscopy. J Neurosurg Spine. 2008;9:
488-492.
34. Garg N, Sampath S, Yasha TC, Chandramouli BA,
Devi BI, Kovoor JM. Is total excision of spinal
neurenteric cysts possible? Br J Neurosurg. 2008;22:
241-251.
35. Muzumdar D, Bhatt Y, Sheth J. Intramedullary
cervical neurenteric cyst mimicking an abscess.
Pediatr Neurosurg. 2008;44:55-61.
36. Daszkiewicz P, Roszkowski M, Grajkowska W.
Currarino syndrome in a teenage girlea case
report. Neurol Neurochir Pol. 2007;41:361-364.
37. Kumar R, Prakash M. Unusual split cord with
neurenteric cyst and cerebellar heterotopia over
spinal cord. Childs Nerv Syst. 2007;23:243-247.
38. Rauzzino MJ, Tubbs RS, Alexander E 3rd,
Grabb PA, Oakes WJ. Spinal neurenteric cysts and
their relation to more common aspects of occult
spinal dysraphism. Neurosurg Focus. 2001;10:e2.
39. Daszkiewicz P, Roszkowski M, Przasnek S,
Grajkowska W, Jurkiewicz E. Teratoma or enterogenous cyst? The histopathological and clinical
dilemma in co-existing occult neural tube dysraphism. Folia Neuropathol. 2006;44:24-33.
40. Rossi A, Piatelli G, Gandolfo C, et al. Spectrum of
nonterminal myelocystoceles. Neurosurgery. 2006;
58:509-515.
41. Menezes AH, Traynelis VC. Spinal neurenteric
cysts in the magnetic resonance imaging era.
Neurosurgery. 2006;58:97-105.
42. de Oliveira RS, Cinalli G, Roujeau T, SainteRose C, Pierre-Kahn A, Zerah M. Neurenteric
cysts in children: 16 consecutive cases and review
of the literature. J Neurosurg. 2005;103(6 suppl):
512-523.
43. Tuzun Y, Izci Y, Sengul G, Erdogan F, Suma S.
Neurenteric cyst of the upper cervical spine:
excision via posterior approach. Pediatr Neurosurg.
2006;42:54-56.
44. Kumar R, Singh V. Benign intradural extramedullary masses in children of northern India.
Pediatr Neurosurg. 2005;41:22-28.
45. Shenoy SN, Raja A. Spinal neurenteric cyst.
Report of 4 cases and review of the literature.
Pediatr Neurosurg. 2004;40:284-292.
46. Kumar R, Singh V. Intramedullary mass lesion of
the spinal cord in children of a developing milieu.
Pediatr Neurosurg. 2004;40:16-22.
47. Hicdonmez T, Steinbok P. Spontaneous hemorrhage into spinal neurenteric cyst. Childs Nerv Syst.
2004;20:438-442.
48. Trehan G, Soto-Ares G, Vinchon M, Pruvo JP.
[Neurenteric cyst: an unusual congenital malformation of the spinal canal]. J Radiol. 2003;84:
412-424 [in French].
49. Kumar R, Nayak SR. Unusual neuroenteric cysts:
diagnosis and management. Pediatr Neurosurg.
2002;37:321-330.
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CONGENITAL SPINAL CYSTS
50. ten Donkelaar HJ, Willemsen MA, van der
Heijden I, Beems T, Wesseling P. A spinal intradural enterogenous cyst with well-differentiated
muscularis propria. Acta Neuropathol. 2002;104:
538-542.
51. Agrawal D, Suri A, Mahapatra AK, Sharma MC.
Intramedullary neurenteric cyst presenting as infantile paraplegia: a case and review. Pediatr Neurosurg. 2002;37:93-96.
52. Agrillo A, Passacantilli E, Santoro A, Delfini R.
Spinal intradural endodermal cyst located anterior
to the cervical spinal cord. J Neurosurg Sci. 2001;45:
220-223.
53. Tsuji T, Matsuyama Y, Sato K, Iwata H. Infantile
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54. Rizk T, Lahoud GA, Maarrawi J, et al. Acute
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55. Kumar R, Jain R, Rao KM, Hussain N. Intraspinal
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56. Sharma RR, Ravi RR, Gurusinghe NT, et al.
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57. Shetty DS, Lakhkar BN. Cervico-dorsal spinal
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58. Ergün R, Akdemir G, Gezici AR, Kara C,
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59. Kadhim H, Proaño PG, Saint Martin C, et al.
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60. Kim CY, Wang KC, Choe G, et al. Neurenteric
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84. Chhang WH, Kak VK, Radotra BD, Jena A.
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69. Hamamoto O, Guerreiro NE, Nakano H,
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55:319-324.
85. Jeng MJ, Chang KP, Hwang B, Wong TT, Ho DM.
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70. Lee SC, Chun YS, Jung SE, Park KW, Kim WK.
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86. Brunberg JA, DiPietro MA, Venes JL, et al. Intramedullary lesions of the pediatric spinal cord:
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71. Zahos PA, Goodman LA, Onesti ST,
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72. Mam MK, Mathew S, Prabhakar BR, Paul R,
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73. Rao MB, Rout D, Misra BK, Radhakrishnan VV.
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74. Prasad VS, Reddy DR, Murty JM. Cervico-thoracic
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75. Lazareff JA, Hoil Parra JA. Intradural neurenteric
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76. Menezes AH, Ryken TC. Craniocervical intradural
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77. Devkota UP, Lam JM, Ng H, Poon WS. An anterior
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78. Mendel E, Lese GB, Gonzalez-Gomez I,
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63. Durham MM, Chahine AA, Ricketts RR. Presacral
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79. Mooney JF 3rd, Hall JE, Emans JB, Millis MB,
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64. Wagner HJ, Seidel A, Reusche E, Sepehrnia A,
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80. Jain AK, Sethi S, Arora A, Tuli SM, Singh RP.
Enterogenous intramedullary cyst. Indian Pediatr.
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65. Ellis AM, Taylor TK. Intravertebral spinal neurenteric cysts: a unique radiographic signe“the
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81. Pianetti Filho G, Fonseca LF. High medular
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66. Rebhandl W, Rami B, Barcik U, Perneczky G,
Horcher E. Neurenteric cyst mimicking
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82. Brooks BS, Duvall ER, el Gammal T, Garcia JH,
Gupta KL, Kapila A. Neuroimaging features of
neurenteric cysts: analysis of nine cases and
87. Gumerlock MK, Spollen LE, Nelson MJ,
Bishop RC, Cooperstock MS. Cervical neurenteric
fistula causing recurrent meningitis in KlippelFeil sequence: case report and literature review.
Pediatr Infect Dis J. 1991;10:532-535.
88. Miyagi K, Mukawa J, Mekaru S, Ishikawa Y,
Kinjo T, Nakasone S. Enterogenous cyst in the
cervical spinal canal. J Neurosurg. 1988;68:292-296.
89. Sen S, Bourne AJ, Morris LL, Furness ME,
Ford WD. Dorsal enteric cystsea study of eight
cases. Aust N Z J Surg. 1988;58:51-55.
90. Radek A, Piwowarski W, Sordyl E, Harat M,
Błoch P, Kozłowski W. An hourglass-shaped
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extra- and intramedullary location. Neurol Neurochir
Pol. 1987;21:429-433.
91. Itakura T, Kusumoto S, Uematsu Y, et al. Enterogenous cyst of the cervical spinal cord in a
childecase report. Neurol Med Chir. 1986;26:49-53.
92. Matsushima T, Fukui M, Egami H. Epithelial cells
in a so-called intraspinal neurenteric cyst: a light
and electron microscopic study. Surg Neurol. 1985;
24:656-660.
93. Lerma S, Roda JM, Villarejo F, Perez-Higueras A,
Gutierrez-Molina M, Blazques MG. Intradural
neurenteric cyst: review and discussion. Neurochirurgia. 1985;28:228-231.
94. Chavda SV, Davies AM, Cassar-Pullicino VN.
Enterogenous cysts of the central nervous system:
a report of eight cases. Clin Radiol. 1985;36:
245-2451.
95. McMaster MJ. Occult intraspinal anomalies and
congenital scoliosis. J Bone Joint Surg Am. 1984;66:
588-601.
96. Takemi K, Kubo S, Ibayashi N, Ikeda M, Ohta T,
Yonezawa T. A case of cervical intramedullary
neurenteric cyst. No Shinkei Geka. 1984;12:539-543.
97. Carachi R. The split notochord syndrome: a case
report on a mixed spinal enterogenous cyst in a
child with spina bifida cystica. Z Kinderchir. 1982;
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CONGENITAL SPINAL CYSTS
98. Schiffer J, Till K. Spinal dysraphism in the cervical
and dorsal regions in childhood. Childs Brain. 1982;
9:73-84.
6. Kumar R, Nayak SR, Krishnani N, Chhabra DK.
Spinal intramedullary ependymal cyst. Report of
two cases and review of the literature. Pediatr Neurosurg. 2001;35:29-34.
patients undergoing tethered cord surgery after
fetal myelomeningocele repair. J Neurosur Pediatr.
2014;13:355-361.
99. Holmes GL, Trader S, Ignatiadis P. Intraspinal
enterogenous cysts. A case report and review of
pediatric cases in the literature. Am J Dis Child.
1978;132:906-908.
7. Iwahashi H, Kawai S, Watabe Y, et al. Spinal
intramedullary ependymal cyst: a case report. Surg
Neurol. 1999;52:357-361.
100. Laha RK, Huestis WS. Intraspinal enterogenous
cyst: delayed appearance following mediastinal
cyst resection. Surg Neurol. 1975;3:67-70.
8. Matsuyama T, Morimoto T, Sakaki T. Intraspinal
ependymal cyst. Acta Neurochir (Wien). 1996;138:
1366-1367.
101. Voth D, Eckert HG, Höhn P. Intraspinal neurenteric cyst associated with dystopia of lung
tissue and myelocele. J Neurol. 1975;208:233-239.
9. Osenbach RK, Godersky JC, Traynelis VC,
Schelper RD. Intradural extramedullary cysts of the
spinal canal: clinical presentation, radiographic
diagnosis, and surgical management. Neurosurgery.
1992;30:35-42.
9. Bansal S, Suri A, Borkar SA, Kale SS, Singh M,
Mahapatra AK. Management of intramedullary tumors in children: analysis of 82 operated cases.
Childs Nerv Syst. 2012;28:2063-2069.
10. Sharma BS, Banerjee AK, Khosla VK, Kak VK.
Congenital intramedullary spinal ependymal cyst.
Surg Neurol. 1987;27:476-480.
10. Liu H, Zhang JN, Zhu T. Microsurgical treatment
of spinal epidermoid and dermoid cysts in the
lumbosacral region. J Clin Neurosci. 2012;19:
712-717.
102. Piramoon AN, Abbassioun K. Mediastinal
enterogenic cyst with spinal cord compression.
J Pediatr Surg. 1974;9:543-545.
103. Gillespie R, Faithfull DK, Roth A, Hall JE.
Intraspinal anomalies in congenital scoliosis. Clin
Orthop Relat Res. 1973;93:103-109.
104. Deshpande DH, Pandya SK, Dastur HM,
Bharucha EP. An intraspinal enterogenous cyst.
Neurol India. 1972;20:217-220.
105. Silvernail WI Jr, Brown RB. Intramedullary
enterogenous cyst. J Neurosurg. 1972;36:235-238.
106. Kahn AP, Hirsch JF, da Lage C, Lyon G,
Saporta L, Evrard P. Intraspinal enteric cysts. 3
cases. Neurochirurgie. 1971;17:33-44.
107. Howat JM, Grant JC. Non-vitelline accessory
enteric formations. Br J Surg. 1970;57:205-209.
108. Till K. Spinal dysraphism. A study of congenital
malformations of the lower back. J Bone Joint Surg
Br. 1969;51:415-422.
109. Arnould G, Lepoire J, Tridon P, Laxenaire M,
Montaut J. Secondary ulcero-mutilating acropathy and teratomatous cyst of the sacral canal. Rev
Neurol. 1965;112:373-377.
11. Findler G, Hadani M, Tadmor R, Bubis JJ,
Shaked I, Sahar A. Spinal intradural ependymal
cyst: a case report and review of the literature.
Neurosurgery. 1985;17:484-486.
12. Leech RW, Olafson RA. Epithelial cysts of the
neuraxis: presentation of three cases and a review
of the origins and classification. Arch Pathol Lab
Med. 1977;101:196-202.
13. Jacobs P, McKinnell JS. An intraosseous ependymal cyst. Clin Radiol. 1967;18:137-139.
14. Moore MT, Book MH. Congenital cervical ependymal cyst. Report of a case with symptoms
precipitated by injury. J Neurosurg. 1966;24:558-561.
15. Hyman I, Hamby W, Sanes S. Ependymal cyst of
the cervicodorsal region of the spinal cord. Arch
Neur Psych. 1938;40:1005-1012.
APPENDIX D: REPORTED CASES OF
EPIDERMOID CYSTS IN THE LITERATURE
110. Bortolotti G. Intrathoracic gastrogenic cyst
associated with abnormalities of the spinal column. Ann Ital Chir. 1958;35:85-96.
1. Acharyya S, Chakravarty S. Congenital dermal sinus
with extensive intramedullary expansion and an
infected spinal epidermoid cyst in an infant.
J Family Med Prim Care. 2018;7:1103-1105.
APPENDIX C: REPORTED CASES OF
EPENDYMAL CYSTS IN THE LITERATURE
2. Lee SM, Cheon JE, Choi YH, et al. Limited dorsal
myeloschisis and congenital dermal sinus: comparison of clinical and MR imaging features. AJNR
Am J Neuroradiol. 2017;38:176-182.
1. Ranjan R, Tewari R, Kumar S. Cervical intradural
extramedullary ependymal cyst associated with
congenital dermal sinus: a case report. Childs Nerv
Syst. 2009;25:1121-1124.
2. Kuo MF, Tsai Y, Hsu WM, Chen RS, Tu YK,
Wang HS. Tethered spinal cord and VACTERL association. J Neurosurg. 2007;106(3 suppl):201-204.
3. Lalitha AV, Rout P, Souza D, Shailesh F, Rao S.
Spinal intramedullary neuroepithelial (ependymal)
cyst. A rare cause of treatable acute para paresis.
Indian J Pediatr. 2006;73:945-946.
4. Kumar R, Singh V. Intramedullary mass lesion of
the spinal cord in children of a developing milieu.
Pediatr Neurosurg. 2004;40:16-22.
5. Chhabra R, Bansal S, Radotra BD, Mathuriya SN.
Recurrent intramedullary cervical ependymal cyst.
Neurol India. 2003;51:111-113.
3. Karatay M, Koktekir E, Celik H, Erdem Y, Sertbas I,
Bayar MA. Spinal intradural abscess caused by hematogenous spread of Prevotella oralis in a 3-year-old
child with an asymptomatic congenital spinal abnormality. Spinal Cord. 2015;53(suppl 1):S13-S15.
4. Grobelny BT, Weiner HL, Harter DH. Intramedullary spinal epidermoid presenting after
thoracic meningocele repair: case report.
J Neurosurg Pediatr. 2015;15:641-643.
5. Ak H, Atalay T, Gülşen I. The association of the
epidermoid cyst of the filum terminale, intradural
spinal lipoma, tethered cord, dermal sinus tract,
and type I diastematomyelia in a child. World Neurosurg. 2014;82:e836-e837.
6. Jackson EM, Schwartz DM, Sestokas AK, et al.
Intraoperative neurophysiological monitoring in
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7. Thompson DN. Spinal inclusion cysts. Childs Nerv
Syst. 2013;29:1647-1655.
8. Cox EM, Knudson KE, Manjila S, Cohen AR. Unusual presentation of congenital dermal sinus:
tethered spinal cord with intradural epidermoid
and dual paramedian cutaneous ostia. Neurosurg
Focus. 2012;33:E5.
11. Morita M, Miyauchi A, Okuda S, Oda T, Aono H,
Iwasaki M. Intraspinal epidermoid tumor of the
cauda equina region: seven cases and a review of
the literature. J Spinal Disord Tech. 2012;25:292-298.
12. Issaivanan M, Cohen S, Mittler M, Johnson A,
Edelman M, Redner A. Iatrogenic spinal epidermoid cyst after lumbar puncture using needles
with stylet. Pediatr Hematol Oncol. 2011;28:600-603.
13. Avcu S, Köseoglu MN, Bulut MD, Ozen O, Unal O.
The association of tethered cord, syringomyelia,
diastometamyelia, spinal epidermoid, spinal lipoma and dermal sinus tract in a child. JBR-BTR.
2010;93:305-307.
14. Kumar A, Singh P, Jain P, Badole CM. Intramedullary spinal epidermoid cyst of the cervicodorsal region: a rare entity. J Pediatr Neurosci. 2010;
5:49-51.
15. Munshi A, Talapatra K, Ramadwar M, Jalali R.
Spinal epidermoid cyst with sudden onset of
paraplegia. J Cancer Res Ther. 2009;5:290-292.
16. van Aalst J, Hoekstra F, Beuls EA, et al. Intraspinal
dermoid and epidermoid tumors: report of 18
cases and reappraisal of the literature. Pediatr
Neurosurg. 2009;45:281-290.
17. Danzer E, Adzick NS, Rintoul NE, et al. Intradural
inclusion cysts following in utero closure of myelomeningocele: clinical implications and followup findings. J Neurosurg Pediatr. 2008;2:406-413.
18. Yen CP, Kung SS, Kwan AL, Howng SL, Wang CJ.
Epidermoid cysts associated with thoracic
meningocele. Acta Neurochir (Wien). 2008;150:
305-308.
19. Gerlach R, Zimmermann M, Hermann E,
Kieslich M, Weidauer S, Seifert V. Large intramedullary abscess of the spinal cord associated
with an epidermoid cyst without dermal sinus.
J Neurosurg Spine. 2007;7:357-361.
20. Wilson PE, Oleszek JL, Clayton GH. Pediatric
spinal cord tumors and masses. J Spinal Cord Med.
2007;30(suppl 1):S15-S20.
21. Bayar MA, Gokcek C, Erdem Y, et al. Intramedullary spinal epidermoid associated with an
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LITERATURE REVIEW
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CONGENITAL SPINAL CYSTS
intramedullary lipoma. Pediatr Neurosurg. 2007;43:
418-420.
39. Teksam M, Casey SO, Michel E, Benson M,
Truwit CL. Intraspinal epidermoid cyst: diffusionweighted MRI. Neuroradiology. 2001;43:572-574.
22. Hung PC, Wang HS, Wu CT, Lui TN, Wong AM.
Spinal intramedullary abscess with an epidermoid
secondary to a dermal sinus. Pediatr Neurol. 2007;
37:144-147.
40. Erşahin Y, Barçin E, Mutluer S. Is meningocele
really an isolated lesion? Childs Nerv Syst. 2001;17:
487-490.
23. Per H, Kumandaş S, Gümüş H, Yikilmaz A,
Kurtsoy A. Iatrogenic epidermoid tumor: late
complication of lumbar puncture. J Child Neurol.
2007;22:332-336.
41. Avellino AM, Mesiwala AB, Shaw DW, et al.
Diffusion-weighted magnetic resonance image of
a pediatric spinal epidermoid cyst. Pediatr Neurosurg. 2001;34:325-326.
24. Gao B, Yang J, Zhuang S, et al. Mollaret meningitis associated with an intraspinal epidermoid
cyst. Pediatrics. 2007;120:e220-e224.
42. Patankar T, Krishnan A, Patkar D, Armao D,
Mukherji SK. Diastematomyelia and epidermoid
cyst in the hemicord. AJR Am J Roentgenol. 2000;
174:1793-1794 [erratum AJR Am J Roentgenol.
2001;176:815].
25. Refai D, Perrin RJ, Smyth MD. Iatrogenic intradural epidermoid cyst after lumbar puncture.
J Neurosurg. 2007;106(4 suppl):322.
26. Kalkan E, Karabagli P, Karabagli H, Baysefer A.
Congenital cranial and spinal dermal sinuses: a
report of 3 cases. Adv Ther. 2006;23:543-548.
27. Jeong IH, Lee JK, Moon KS, et al. Iatrogenic
intraspinal epidermoid tumor: case report. Pediatr
Neurosurg. 2006;42:395-398.
28. Er U, Yigitkanli K, Kazanci A, Bavbek M. Primary
lumbar epidermoid tumor mimicking schwannoma. J Clin Neurosci. 2006;13:130-133.
29. Kumar R, Singh V. Benign intradural extramedullary masses in children of northern India.
Pediatr Neurosurg. 2005;41:22-28.
30. Tubbs RS, Kelly DR, Mroczek-Musulman EC,
et al. Dwarfism, occult spinal dysraphism, and
presacral myxopapillary ependymoma with an
epidermoid cyst in a child. Acta Neurochir (Wien).
2005;147:299-302.
31. Chang IC. Surgical experience in symptomatic
congenital intraspinal cysts. Pediatr Neurosurg.
2004;40:165-170.
32. Cataltepe O, Berker M, Akalan N. A giant intramedullary spinal epidermoid cyst of the cervicothoracic region. Pediatr Neurosurg. 2004;40:120-123.
33. Kumar R, Singh V. Intramedullary mass lesion of
the spinal cord in children of a developing milieu.
Pediatr Neurosurg. 2004;40:16-22.
34. Ziv ET, Gordon McComb J, Krieger MD,
Skaggs DL. Iatrogenic intraspinal epidermoid tumor: two cases and a review of the literature.
Spine. 2004;29:E15-E18.
35. Mongia S, Devi BI, Shaji KR, Hegde T. Spinal
subdural epidermoidsea separate entity: report of
3 cases. Neurol India. 2002;50:529-531.
36. Minegishi K, Kusaka Y, Shirane R, Yoshimoto T.
Congenital dermal sinus tract of recurrent pyrexia:
case report. No Shinkei Geka. 2002;30:967-971.
37. Avellino AM, Wang PP, Miller NH, Herskovits EH.
FLAIR magnetic resonance image of a pediatric
spinal epidermoid cyst. Pediatr Neurosurg. 2002;36:
220-222.
38. Raqbi F, Zérah M, Bodemer C, Lenoir G. Dermoid
cysts revealed by meningitis with medullary
compression. Arch Pediatr. 2001;8:499-503.
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43. Jadhav RN, Khan GM, Palande DA. Intramedullary
epidermoid cyst in cervicodorsal spinal cord.
J Neurosurg. 1999;90(1 suppl):161.
44. Shamoto H, Yoshida Y, Shirane R, Yoshimoto T.
Anterior sacral meningocele completely occupied
by an epidermoid tumor. Childs Nerv Syst. 1999;15:
209-211.
45. Morandi X, Mercier P, Fournier HD, Brassier G.
Dermal sinus and intramedullary spinal cord abscess. Report of two cases and review of the
literature. Childs Nerv Syst. 1999;15:202-206.
46. Potgieter S, Dimin S, Lagae L, et al. Epidermoid
tumours associated with lumbar punctures performed in early neonatal life. Dev Med Child Neurol.
1998;40:266-269.
47. Chang PF, Wang PJ, Tu YK. Intradural extramedullary epidermoid cyst of the spinal canal:
report of one case. Zhonghua Min Guo Xiao Er Ke Yi
Xue Hui Za Zhi. 1996;37:222-224.
48. Kriss TC, Kriss VM, Warf BC. Recurrent meningitis: the search for the dermoid or epidermoid
tumor. Pediatr Infect Dis J. 1995;14:697-700.
49. Demaerel P, Casaer P, van Calenbergh F, et al.
Iatrogenic spinal epidermoid tumour associated
with tuberous sclerosis. A diagnostic pitfall.
J Neuroradiol. 1994;21:270-273.
50. Bollar A, Prieto A, Allut AG, Gelabert M, Cid E.
Spinal epidermoid cysts without congenital
anomalies associated. Report of two cases.
J Neurosurg Sci. 1994;38:171-175.
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73. Kumar S, Parkash B, Lodha PS, Malik R. Intramedullary epidermoid. Indian Pediatr. 1981;18:
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90. Teng P, Papatheodorou CA. Dermal sinus and
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92. Cantu RC, Wright RL. Aseptic meningitic syndrome with cauda equina epidermoid tumor.
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95. de Rougemont, Francois R, Levy, Hermier M,
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82. Harris HW, Miller OF. Midline cutaneous and
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8. Akhtar S, Azeem A, Shamim MS, Tahir MZ.
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LITERATURE REVIEW
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CONGENITAL SPINAL CYSTS
72. Peter JC, Sinclair-Smith C, de Villiers JC. Midline
dermal sinuses and cysts and their relationship to
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1:73-79.
73. Barkovich AJ, Edwards Ms, Cogen PH. MR evaluation of spinal dermal sinus tracts in children.
AJNR Am J Neuroradiol. 1991;12:123-129.
74. O’Neill P, Stack JP. Magnetic resonance imaging
in the pre-operative assessment of closed spinal
dysraphism in children. Pediatr Neurosurg. 19901991;16:240-246.
75. Lunardi P, Missori P, Gagliardi FM, Fortuna A.
Long-term results of the surgical treatment of
spinal dermoid and epidermoid tumors. Neurosurgery. 1989;25:860-864.
76. Weissert M, Gysler R, Sörensen N. The clinical
problem of the tethered cord syndromeea report
of 3 personal cases. Z Kinderchir. 1989;44:275-279.
77. Shikata J, Yamamuro T, Mikawa Y, Kotoura Y.
Intraspinal epidermoid and dermoid cysts. Surgical results of seven cases. Arch Orthop Trauma Surg.
1988;107:105-109.
78. Yasui T, Hakuba A, Katsuyama J, Nishimura S.
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cord tumours: report of 22 cases. Acta Neurochir
Suppl. 1988;43:9-12.
79. Schubert A, Todd MM, Luerssen TG, Hicks GE.
Loss of intraoperative evoked responses during
dorsal column surgery associated with isolated
postoperative sensory deficit. J Clin Monit. 1987;3:
277-281.
80. Scott RM, Wolpert SM, Bartoshesky LE,
Zimbler S, Klauber GT. Dermoid tumors occurring at the site of previous myelomeningocele
repair. J Neurosurg. 1986;65:779-783.
81. Verdu A, de la Cruz M, Pascual-Castroviejo I,
Villarejo F. Intramedullary dermoid of the cervical
spinal cord in a child. J Neurol Neurosurg Psychiatry.
1986;49:462-463.
82. Naidich TP, Radkowski MA, Britton J. Real-time
sonographic display of caudal spinal anomalies.
Neuroradiology. 1986;28:512-527.
83. Citron ND, Paterson FW, Jackson AM. Neuropathic osteonecrosis of the lateral femoral condyle
in childhood. A report on four cases. J Bone Joint
Surg Br. 1986;68:96-99.
84. Maroun FB, Jacob JC, Heneghan WD, Cooper AR,
Kennedy RF, Lewis K. Recurrent meningitis in a
child. Indian J Pediatr. 1984;51:355-357.
85. McMaster MJ. Occult intraspinal anomalies and
congenital scoliosis. J Bone Joint Surg Am. 1984;66:
588-601.
86. Dikshteĭn EA, Kurennaia SS, Gandera VF. Isolated
actinomycosis of the spinal cord. Arkh Patol. 1984;
46:67-70.
spinal dysraphia. Dermoid cyst of the cauda
equina associated with a pilonidal sinus: case
report. Minerva Pediatr. 1983;35:785-788.
89. Naidich TP, McLone DG, Shkolnik A,
Fernbach SK. Sonographic evaluation of caudal
spine anomalies in children. AJNR Am J Neuroradiol.
1983;4:661-664.
90. Graf R. Etiology of fixed lumbar lordosis: the
lumbar dermoid. Z Orthop Ihre Grenzgeb. 1982;120:
770-773.
91. Carachi R. The split notochord syndrome: a case
report on a mixed spinal enterogenous cyst in a
child with spina bifida cystica. Z Kinderchir. 1982;
35:32-34.
92. Schiffer J, Till K. Spinal dysraphism in the cervical
and dorsal regions in childhood. Childs Brain. 1982;
9:73-84.
93. Oi S, Raimondi AJ. Hydrocephalus associated
with intraspinal neoplasms in childhood. Am J Dis
Child. 1981;135:1122-1124.
94. Stein BM. Surgery of intramedullary spinal cord
tumors. Clin Neurosurg. 1979;26:529-542.
95. Barry JF, Harwood-Nash DC, Fitz CR, Byrd SE,
Boldt DW. Metrizamide in pediatric myelography.
Radiology. 1977;124:409-418.
96. Arseni C, Danaila L, Constantinescu A, Carp N.
Spinal dermoid tumours. Neurochirurgia. 1977;20:
108-116.
97. Kumar S, Gulati DR, Mann KS. Intraspinal dermoids. Neurochirurgia. 1977;20:105-108.
98. Garrido E, Stein BM. Microsurgical removal of
intramedullary spinal cord tumors. Surg Neurol.
1977;7:215-219.
99. Kordás M, Paraiez E, Szénásy J. Spinal tumors in
infancy and childhood. Zentralbl Neurochir. 1977;38:
331-337.
100. Harris HW, Miller OF. Midline cutaneous and
spinal defects. Midline cutaneous abnormalities
associated with occult spinal disorders. Arch
Dermatol. 1976;112:1724-1728.
101. Rueda Franco F, Monson de Souza MB, Takenaga
Mesquida R. Intraspinal tumors in children.
Review of 24 cases. Bol Med Hosp Infant Mex. 1975;
32:1073-1094.
102. Ekelund L, Cronqvist S. Roentgenological
changes in spinal malformations and spinal tumours in children. Radiologe. 1973;13:541-546.
103. Gillespie R, Faithfull DK, Roth A, Hall JE.
Intraspinal anomalies in congenital scoliosis. Clin
Orthop Relat Res. 1973;93:103-109.
104. Roth M, Gotfrýd O, Morávek V. Intraspinal dermoid and spinal extradural cyst: pneumomyelographic findings. Neuroradiology. 1973;5:127-128.
87. Bale PM. Sacrococcygeal developmental abnormalities and tumors in children. Perspect Pediatr
Pathol. 1984;8:9-56.
105. Nikiforov BM, Smirnov GI, Didenko EV. Diastematomyelia of the spine combined with dermoid tumor of equine cauda. Pediatriia. 1972;51:
81-82.
88. Bonioli E, Cama A, Bellini C, Pantaleo A,
Andreussi L. Importance of early diagnosis of
106. Kushnick T. Recurrent meningitis. Clin Pediatr.
1972;11:308-309.
WORLD NEUROSURGERY -: ---, MONTH 2020
107. Reddy DR, Prabhakar V, Rao BD. Intraspinal
dermoid tumours. Indian Pediatr. 1972;9:149-151.
108. Hirt HR, Zdrojewski B, Weber G. The manifestations and complications of intraspinal
congenital dermal sinuses and dermoid cysts.
Neuropadiatrie. 1972;3:231-247.
109. Dinakar I, Balaparameswara S. Intramedullary
tumours of spinal cord. Indian J Cancer. 1971;8:
292-296.
110. Intrau H, Usbeck W. Compressing processes in
the spinal canal in children. Zentralbl Chir. 1971;
96:1225-1230.
111. Fontenelle Filho O, Duarte F. Intraspinal dermoid
and epidermoid tumors. Arq Neuropsiquiatr. 1971;
29:26-48.
112. Maeda K, Yamashita H, Asai O. Case of diastematomylia. Seikei Geka. 1971;22:656-660.
113. Bailey IC. Dermoid tumors of the spinal cord.
J Neurosurg. 1970;33:676-681.
114. Teng P, Papatheodorou CA. Dermal sinus and
intraspinal dermoid and epidermoid cyst in
children. Bull Los Angeles Neurol Soc. 1970;35:
153-163.
115. Rao SB, Dinakar I. Intraspinal dermoids. Neurol
India. 1970;18:185-188.
116. Feldmann H, Wendt F. Epidermoid and dermoid
tumors of the central nervous system. Zentralbl
Chir. 1970;95:696-704.
117. Carrascosa RG, Lamas E, Nader R. Dermal sinuses associated with intraspinal tumors. Rev Clin
Esp. 1970;117:397-406.
118. Till K. Spinal dysraphism. A study of congenital
malformations of the lower back. J Bone Joint Surg
Br. 1969;51:415-422.
119. Ritchie GW, Flanagan MN. Diastematomyelia.
Can Med Assoc J. 1969;100:428-433.
120. Decker RE, Gross SW. Intraspinal dermoid tumor presenting as chemical meningitis. Report
of a case without dermal sinus. J Neurosurg. 1967;
27:60-62.
121. Brengola GM. Tumors of the sacrum. An
anatomo-clinical study. Minerva Ginecol. 1966;18:
846-853.
122. Swiatkowska I. Brain and spinal cord tumors of
children. Tumori. 1966;52:303-311.
123. Eerland LD. Presacral cysts and tumours. Arch
Chir Neerl. 1966;18:5-31.
124. Daroza AC. Primary intraspinal tumors: their
clinical presentation and diagnosis. An analysis
of ninety-five cases. J Bone Joint Surg Br. 1964;46:
8-15.
125. Geisler E, Schuck W. Spinal tumors and processes involving the spinal cord in children. Arch
Kinderheilkd. 1963;169:254-266.
126. Pettersson G, Werkmaster K. Intraspinal dermoid cysts in children. Survey of literature and
own cases. Acta Paediatr. 1963;52:187-189.
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