Acta Radiologica

ISSN: 0284-1851 (Print) 1600-0455 (Online) Journal homepage: http://www.tandfonline.com/loi/iard20

Arachnoid Cyst of the Craniospinal Junction: a

Case Report and Review of the Literature

S. Cakirer

To cite this article: S. Cakirer (2004) Arachnoid Cyst of the Craniospinal Junction: a Case Report
and Review of the Literature, Acta Radiologica, 45:4, 460-463

To link to this article: http://dx.doi.org/10.1080/02841850410005453

Published online: 09 Jul 2009.

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 CASE REPORT ACTA RADIOLOGICA

Arachnoid Cyst of the Craniospinal Junction: a Case Report and

Review of the Literature
S. CAKIRER
Istanbul Sisli Etfal Hospital, Department of Radiology, Neuroradiology Section, Atasehir-Istanbul, Turkey

Cakirer S. Arachnoid cyst of the craniospinal junction: a case report and review of the
literature. Acta Radiol 2004;45:460463.
Arachnoid cysts are benign intra-arachnoid collections of cerebrospinal fluid and
comprise around 1% of the intracranial masses. Unless complicated with hemorrhage,
they are similar to cerebrospinal fluid in signal intensity in most cases. Diffusion-
weighted magnetic resonance imaging (MRI) reveals that they have no water restriction
and distinguishes them from epidermoid cysts, which show water restriction. Arachnoid
cysts of the craniospinal junction are rare lesions, with only seven cases reported in the
literature. Imaging findings of all craniospinal arachnoid cysts reveal a large posterior
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fossa arachnoid cyst extending through the foramen magnum to the level of the upper
spine. We present MRI findings of a 27-year-old female patient with a craniospinal
arachnoid cyst.
Key words: Arachnoid cyst; craniospinal junction; magnetic resonance imaging, brain
Sinan Cakirer, 67 Ada, Kardelen 4/2, Daire 37, 81120 Atasehir-Istanbul, Turkey (fax. z9
0216 455 3522, e-mail. scakirer@yahoo.com)
Accepted for publication 14 February 2004

Arachnoid cysts are benign intra-arachnoid collec-
tions of cerebrospinal fluid (CSF) and comprise
around 1% of the intracranial masses (2, 13). They
are found along the craniospinal CSF spaces, and
are rarely detected in the craniospinal junction,
where only seven cases have been reported in the
literature (1, 3, 6, 912). We review the literature
and present a rare case of craniospinal arachnoid
cyst.
Case Report
A 27-year-old female patient presented with head-
ache, neck pain, severe left arm pain associated with
weakness and dysesthesias, all of which had steadily
increased over a period of one year. Her complaints
were particularly severe during the daytime, and
decreasing slightly while sleeping. Neurological
examination of the patient confirmed motor weak-
ness of the left upper extremity, with a degree of
4/5.
A craniocervical magnetic resonance imaging
(MRI) study was performed on a 1.5T MR scanner
(1.5T Magnetom Vision; Siemens, Erlangen,
Germany) with spin-echo (SE) T1 (TR: 528 ms,
TE: 17 ms), fast spin-echo (FSE) T2 (TR: 3840 ms,
TE: 99 ms), diffusion-weighted imaging (b:1000),
and FLAIR (TR: 6000 ms, TE: 100 ms, TI: 1800 ms)
sequences on three orthogonal planes.
MRI of the patient revealed a craniocervical
cystic structure located along the left side of the
medulla oblongata and upper cervical spinal cord
(left perimedullary cistern), from the level of
medulla oblongata to the level of C2 (Fig. 1). The
mass was insinuated between, and mildly dislocated,
the distal segments of the left and right vertebral
arteries (Fig. 2A). The cystic mass caused a
prominent compression on the left side of the
medulla oblongata and upper cervical spinal cord
(Figs. 13); these neural structures did not show any
pathological signal change, however. The cystic
structure was almost isointense to CSF on T1-
weighted (Fig. 3A) and FLAIR (Fig. 3C) sequences,
and mildly hyperintense to CSF on T2-weighted
(Figs. 1, 2A, 3B) sequences. Diffusion-weighted
imaging revealed a hypointense signal of the cyst
(no water restriction) (Fig. 2B). The MRI findings
were consistent with arachnoid cyst.
The patient underwent surgery during which a
midline occipital craniectomy and C1 laminectomy

DOI 10.1080/02841850410005453 # 2004 Taylor & Francis

 Arachnoid Cyst of the Craniospinal Junction 461

Fig. 1. Coronal FSE T2-weighted image (TR: 3840 ms, TE: 99 ms)
reveals a cystic mass of craniospinal junction causing prominent
compression on the left side of the medulla oblongata and upper
cervical spinal cord (black arrow).
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were performed to partially excise the cyst and to

fenestrate the residual cyst into the subarachnoid
space.

Discussion

Arachnoid cysts are uncommon non-neoplastic

extra-axial lesions filled with CSF. They are
classified as primary or secondary (2, 13). Primary
arachnoid cysts derive from the meninx primitiva,
which forms a perimedullary mesh around the
developing central nervous system at the 7th to
10th gestational week embryologically. With
the accumulation of subarachnoid CSF, the
meninx primitiva cavitates and resorbs, leaving
only the subarachnoid space, arachnoid membrane,
and delicate subarachnoid trabeculae normally
found throughout the subarachnoid space. The
developing arachnoid membrane splits during this
process. Arachnoid cells secrete fluid into the
resultant cleft, and, depending on the degree of
communication with the true subarachnoid space,
the cleft enlarges to become a cavity and ultimately
a cyst (5, 10). Secondary arachnoid cysts are
not as common as the primary cysts, and they
develop secondary to a variety of cerebral insults,
including head injury, subarachnoid hemorrhage,
chemical meningitis, infectious meningitis, and
tumors (2, 7).
Arachnoid cysts comprise approximately 1% of
the intracranial masses, with 3550% of cases
occurring in the middle cranial fossa. Although
the posterior fossa is the second most common site
for arachnoid cysts, with a rate of less than 20% of
Fig. 2. (A) Axial FSE T2-weighted (TR:3840 ms, TE:99 ms) and
(B) axial diffusion-weighted (b: 1000) images reveal the cystic
mass (A) insinuating between the mildly dislocated distal segments
of the left and right vertebral arteries, and causing a prominent
compression on the left side of the medulla oblongata. Note that
diffusion-weighted imaging gives a hypointense appearance of the
cyst secondary to non-water restriction.
cases, arachnoid cysts at the craniospinal junction
are rare (2, 7, 13). The seven cases that have been
reported in the literature at that location are briefly
summarized in the Table (1, 3, 6, 912).
The presenting clinical findings of the cranio-
spinal arachnoid cysts are either secondary to
compression of neighboring neural structures (cere-
bellum, brainstem, and spinal cord) or to the
obstruction of CSF circulation (hydrocephalus) (2,
911).
Arachnoid cysts are fluid-filled cavities that lie
entirely within the arachnoid membrane in gross
pathological specimens. They consist of a thin but
distinct transparent wall separated from the inner

Acta Radiol 2004 (4)

 Acta Radiol 2004 (4)

upper cervical spinal cord and left nerve root.

cyst (A) causing a prominent compression on the left side of the
FLAIR (TR: 6000 ms, TE: 100 ms, TI: 1800 ms) images show the
axial FSE T2-weighted (TR: 3840 ms, TE: 99 ms), and (C) axial
Fig. 3. (A) Axial SE T1-weighted (TR: 528 ms, TE: 17 ms), (B)

462
S. Cakirer
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Table. Review of the craniospinal arachnoid cysts reported in the literature

Case (reference no.) Age/sex Clinical findings Imaging findings

Kuroiwa, 1991 59, male Progressive gait disturbance Cyst extending from posterior fossa down to the C2 level
Bhatia, 1992 22, male Urinary hesitancy, spastic paraparesis Cyst extending from posterior fossa down to the C4 level
Takanashi, 1995 36, male Truncal ataxia, dysesthesia of right arm Large cyst in craniospinal junction
Fukushima, 1996 54, female Head and neck pain, vomiting, drowsiness Cyst extending from the level of left cerebellar hemisphere
to the C2 level
Shukla, 1998 16, male Head and neck pain, progressive quadriparesis Posterior fossa cyst extending to the C1 level
Pego-Reigosa, 2000 48, male Gait disorder, spastic paraparesis Large cyst in craniospinal junction and a cervical syrinx
Price, 2001 37, female Head and neck pain, papilledema Cisterna magna cyst extending down to the C2 level
Present case, 2004 27, female Head and neck pain, weakness and dysesthesia Left perimedullary cyst extending down to the C2 level
of left arm

dural layer and the underlying pia-arachnoid (13).
Imaging studies (computed tomography (CT) and
MRI) show the smoothly demarcated extra-axial
cystic mass that does not enhance following IV
contrast injection. Pressure erosion of the overlying
inner table may be seen in cases where the cyst
is adjacent to the calvarium. In most cases
arachnoid cysts are similar to CSF in signal
intensity unless complicated by hemorrhage.
Diffusion-weighted MRI reveals that they have no
water restriction and distinguishes them from
epidermoid cysts, which show water restriction (3,
4, 8, 10). Imaging findings of all craniospinal
arachnoid cysts reveal a large posterior fossa
arachnoid cyst extending through the foramen
magnum to the level of the upper spine. This was
found in our case, too (1, 3, 6, 912). Although
previous cases have been studied using MRI
(BHATIA et al. studied their case with CT myelo-
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graphy), none were studied using a diffusion-
weighted sequence to differentiate the non-water
restricting arachnoid cyst from epidermoid cyst,
which shows restricted diffusion (bright appear-
ance). Diffusion-weighted imaging of craniospinal
arachnoid cyst in our patient revealed a typical,
non-water restricting (low signal) appearance.
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Acta Radiol 2004 (4)