ABSTRACT
Objectives: We report our experiences and characteristics of intracranial
arachnoids cysts (IAC) over a six year period at our clinic.
Methods: We used a
retrospective study design to review all IAC patients, managed in our
neurosurgical clinic between 2014 and 2019. Patient files, radiological images
computed tomography (CT) or magnetic resonance imaging (MRI) scans) were
reviewed for patient characteristics, presentation, cyst types, site and clinical
management.
Results: In total, 365
patients with IAC were included (spinal arachnoid cyst excluded). The mean age
was 16 years ±
13.61 years, and 65% were male. Our data showed that 59% of patients had
asymptomatic arachnoid cysts, with no further management required. However, 23%
required medication for seizure control, whereas surgical intervention was
required for 18% of patients.
Conclusions: Arachnoid cysts are common neurosurgical pathologies, with different
clinical presentations. Variable management modalities are available, and
should be guided by the clinical picture and radiological appearance. Our
cohort data are comparable with international datasets.
Keywords: Intracranial arachnoid cysts, cyst
management, surgical procedures, clinical outcomes.
RMS August
2022; 29 (2): 10.12816/0061166
INTRODUCTION
Intracranial arachnoid cysts (IACs), also
known as lepto-meningeal cysts, are intra-arachnoid cysts filled with a
Cerebrospinal Fluid-like fluid. The first description of an IAC was by Richard
Bright in 1831; he described the pathology as a “serious cyst forming in
connection with the arachnoid, and apparently lying within its layers” [1-3].
However, the exact aetiology of arachnoid cysts remains unclear [4]; they
may be congenital or acquired [5]. Studies have revealed that in most cases,
cysts are primary in nature (i.e. developmental), but in a minority, they are
secondary (i.e. traumatic, post-haemorrhagic or post-infection) [1, 5].
Histo-pathologically, the cyst wall is
formed of duplicated layers of superfine, translucent fibrous connective tissue
(believed to be lamellar collagen), lined by a single layer of meningothelial
cells i.e. epithelial lepto-meningeal cells, either in a diffuse or focal
pattern [6-9]. Secondary cysts may show signs of inflammatory changes e.g.
gliosis or hemosiderin within the walls [1,7,9].
Cysts occur at any age, but approximately
50-70% occur in children (according to our study 220 patients were below 20
years, which is equal to 60%)[10]. IAC prevalence is approximated at 1.7% [11],
with a higher percentage in children (2.6%), when compared with adults (1.4%)
[12].
Headache is the most common symptom, is experienced by up to 241
patients 66% of sufferers and may be caused by local mass effects, high
intracranial pressure (ICP), or hydrocephalus [1,13-15], however unilateral
headaches occurring in the supra orbital or temporal region may be exacerbated
by physical exertion [1]. Seizures are the next common symptom [1], occurring
in up to 110 patients 30% of individuals [1,13]. They can be of any type
(generalised, focal, or complex-partial) [1], with no clear correlative
evidence between seizure type and cyst location [1,13].
MATERIALS
AND METHODS
Patients
All patients diagnosed
at King Hussein Medical Centre (KHMC) for IACs between 2014 and 2019 were
included. We included patients who were symptomatic, or had incidental IAC
findings by CT or MRI. Ethical approval was granted by the research departmen
ethical committee at the Royal Medical Services.
We reviewed 365 eligible patients with intracranial arachnoid cyst
files and radiological images. Gathered patient characteristics included: age,
gender, clinical presentation, management and complications. Radiological
evaluations included cyst size and site identification (Galassi classification)
[1-3], any associated signs of mass effects and/or hydrocephalus, and also any
communications between the cyst &the subarachnoid space [16] (Figures 1–3).
We identified three
management modalities: conservative, medical (headache and seizure) and
surgical. Surgical management included: microscopic fenestration, endoscopic
fenestration, shunt insertion and evacuation of the haematoma, either alone or
in combination with cyst-peritoneal shunt. Descriptive statistics in the form of
percentages, mean and standard deviation were applied.
RESULTS
Patient population
365 patients were
recruited. Their ages ranged between 18 months to 78 years, with a mean age of
16 years. The male to female ratio was 2:1 (Table I). The paediatric age group
was < 10 years, and comprised the majority of patients (131, 36%) (Figure
4).
Total number
of patients
|
365
|
Age(mean±
SD)
|
16±13.61
|
Gender
Male
Female
|
238(65%)
127(35%)
|
Galassi grade (205 Sylvian locations)
Grade 1
Grade 2
Grade 3
|
96 (47%)
56 (27%)
53 (26%)
|
Treatment modality
Conservative
Medical
Surgical
Open
Endoscopic
Shunt
|
216(59%)
84(23%)
65(18%)
18(28%)
23 (35%)
24 (37%)
|
Surgical complications
Fenestration failure
Meningitis
Collection(mandate evacuation)
Cerebrospinal fluid leak
Pseudomeningocele
Hydrocephalus
|
17/65(26%)
7
3
3
2
1
1
|
Table I. Male-female ratio, Galassi grading ratio, modality of treatment,
and percentage of surgical complications.
Figure 4 Arachnoid cyst group distribution by gender and age
Cyst location
and grading
Galassi and associates had classified Sylvian
fissure cysts, from a radiological perspective into (depending on CT scan ),
Type I: biconvex cyst, at the temporal lobe tip, they appear to communicate
freely with the adjacent CSF spaces,
producing a little mass effect & no skull deformities. Type II:
triangular or rectangular cyst, occupies the proximal & intermediate
segments of the fissure, less likely to communicate with adjacent subarachnoid
space. Type III: they comprise up to 30% of Sylvian fissure cysts, it occupies
the entire fissure, not communicating with the CSF cisterns, causing skull
deformities & marked mass effect [1,11].
Arachnoid cysts location: 86% in
the supra-tentorial space, and 14% in the posterior fossa, exhibited the
following distributions (Table II).
Ninety-six patients (47%)with Sylvian arachnoid cyst exhibited
Galassi grade 1 classification (Table I) The Sylvian
fissure is the most common site of arachnoid cyst, with 205(56%) patients
experiencing a left side dominance (70%).
Arachnoid cyst site
|
Number of
patients
|
Percentage
|
Sylvian fissure
|
205
|
56%
|
Parasellar i.e. intrasellar and suprasellar
|
45
|
12%
|
Convexity and interhemispheric
|
35
|
10%
|
Cerebellopontine angle
|
29
|
8%
|
Vermian
|
40
|
11%
|
Clival
|
11
|
3%
|
Table II.Arachnoid cyst
site distribution by patient number and percentage
Management
&complications
Two hundred and
sixteen patients were treated conservatively (59% of total patients) (Table I).Surgical
management with a cyst-peritoneal shunt accounted for 37% of procedures,
followed by endoscopic fenestration (35%) (Table I).Out of 65 procedures, 17
(26%) patients developed complications (Table I).
DISCUSSION
IACs are benign lesions
with excellent long-term outcomes [8]. Usually, they show gender variations:
the male to female ratio can range from
2:1 to 1:5 [1,6,12,14,17], but this preference is still unclear, however, it may be related to
the presence of progesterone receptors in cyst wall cells [9].These ratios were
similar to our data (2:1) in the pediatric age group, whereas the male to
female ratio at ages> 50 years were almost equal.
IAC distribution analyses showed that Sylvian locations
(56%) and the left side (70%) were the most common cyst locations, in agreement
with Magnus et al. and other
studies[1,6,13,18], and all arachnoid cysts were intra dural, except intra
sellar cysts [18].Our cohort also revealed that Sylvian cysts exhibited
Galassi grade 1dominancy.
Unfortunately, there is no class I guidance on IAC treatment
[19,20,21], thus there is controversy over best management approaches (surgical
versus conservative),and optimal surgical options [1.2.3]. The most critical
step in selecting surgical versus conservative treatment is identifying the
relationship between the cyst and the presenting symptoms/signs [1,22].
Surgical options include aspiration via a simple burr, shunting into the
abdomen or a natural Cerebrospinal Fluid space, endoscopic fenestration
(ventricular cystostomy or ventricular cyst-cisternostomy) and microsurgical
fenestration ± marsupialisation [12,13,19]. IACs in the supra sellar and
quadrigeminal regions are most amenable to neuro-endoscopy [15]. In our clinic,
we adopted classical guidelines to manage IACs.
Some shunting complications include shunt revision, with incidences
of up to 30% [7,12,23], shunt dependency [19], and slit-cyst syndrome
(resembles slit-ventricle syndrome),but should be considered if
neuro-deterioration occurs [24]. In contrast, most authors advocate
microsurgical fenestration ± marsupialisation as their first surgical
preference, to achieve long-term clinical success rates approaching 75% [1].
Surgical and endoscopic fenestration has major advantages over
shunting, including the avoidance of shunt insertion and associated
complications .The procedure also facilitates direct cyst inspection,
potentially confirming a diagnosis, avoiding or coagulating arachnoid blood
vessels, and treating the flocculated cyst [7,18,23,25,26].
Surgical IAC complications may occasionally occur, and include
hydrocephalous (secondary to disturbing(cerebrospinal fluid) dynamics), pseudo
meningocele, CSF leak, wound related complications, infection, subdural hygroma
, subdural haematoma, seizures, repeating surgery, cranial nerve palsy (due to
excessive dissection of arachnoid membranes covering the nerves), and the risk
of shunt dependency and its over drainage [13,25,27]. In our cohort, we
reported a complication rate of 26%, which was comparable [13,25,27]
Our study had several limitations: its observational retrospective
study design, limited analysed variables and confounders, and a lack of
advanced statistical methods .However, our sample size was robust, and our data
reporting could establish further, more comprehensive studies in our country.
Fig. 1 T1 weighted axial MRI Shunted left Sylvain arachnoid cyst
Fig. 2 .T2 weighted coronal MRI Fig. 3. Brain CT scan
Left Sylvain arachnoid cyst Left Sylvain arachnoid cyst
CONCLUSION
IACs are common benign lesions (spinal arachnoid cysts were excluded in
this study), with different clinical presentations and excellent outcomes.
Variable management modalities are available, and should be guided by the
clinical picture and radiological appearance. Observation is a valid management
option in most cases. Importantly, our cohort data were comparable with
international figures. In the future, we recommend further prospective studies
investigating clinical outcomes, using different management options.
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