CHAPTER 3 ARACHNOID CYSTS

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Thomas Westermaier,* Tilmann Schweitzer and Ralf-Ingo Ernestus

Department of Neurosurgery, University of Wuerzburg, Wuerzburg, Germany *Corresponding Author: Thomas WestermaierEmail: westermaier.t@nch.uni-wuerzburg.de

Abstract:

Arachnoid cysts are fluidfilled duplications or splittings of the arachnoid layer with a content which is similar but not equal to the cerebrospinal fluid. Arachnoid cysts are not actual neurodegenerative disorders, rather the underlying defect of the texture of the arachnoid layer is probably congenital in nature. They can occur sporadically or can be associated with other malformations or diseases. Arachnoid cysts may be discovered in early childhood. However, they can develop de novo, grow or decrease in size. They may be diagnosed by ultrasound screening in the fetal period or be discovered during childhood or adulthood. Many arachnoid cysts are asymptomatic. Treatment strategies are discussed controversially. If they are diagnosed incidentally or are correlated with only very mild symptoms, a conservative management with followup imaging may be favored. If they grow, they can cause headaches, seizures or other neurological symptoms and require neurosurgical treatment. This chapter addresses aspects of pathogenesis, clinical symptoms, indication for neurosurgical treatment and treatment options.

INTRODUCTION Cystic malformations of the arachnoid layer had first been reported about 180 years ago.1 In 1979, the English anatomist Cunningham published an autopsy report on a young acromegalic patient who had died from diabetes insipidus. He described the coincidence of a right hemispheric arachnoid cyst (AC) with a pituitary adenoma.2 During the opening of the cyst, a large quantity of serosanguilent fluid escaped. This autoptic finding and a previous episode of strong headache, suggested intracystic hemorrhage. In the first half of the 20th century, after Xray and cerebral angiography had been introduced, further reports on this pathological entity were published.3 Since this time, the nature and most favorable treatment of these spaceoccupying lesions has been discussed controversially.
Neurodegenerative Diseases, edited by Shamim I. Ahmad. 2012 Landes Bioscience and Springer Science+Business Media.
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Pathogenesis Cystic fluid retentions within the arachnoid layer, which may develop secondary to intracranial hemorrhage or meningitis, have to be differentiated from true, primary AC. In addition, they have to be distinguished from other cystic lesions which involve the outer part of the cerebral cortex and the meninges, like glioependymal cysts or cystic lesions of infectious or neoplastic origin.4,5 True AC are benign lesions and considered to be developmental or at least based on a congenital mistake of the arachnoid architecture. It has to be noticed that, particularly in the area of the skullbase and the Sylvian fissure, arachnoid septations of varying strength are woven through the subarachnoid space. A variety of inner arachnoid membranes can be defined which physiologically form the subarachnoid cisterns.6 Ultrastructurally, AC walls are similar to the normal arachnoid membrane. Analyzing operatively resected AC walls, by light and electron microscopy, Miyagami and Tsubokawa found that the inner surface of the AC wall consisted of one or several layers of arachnoid cells with slender processes and large extracellular spaces.7 Rengachary and Watanabe observed structural differences from the normal arachnoid: (1) the splitting of the arachnoid membrane at the margin of the cyst, (2) a thicker layer of collagen in the cyst wall, (3) the absence of traversing trabecular processes within the cyst and (4) the presence of hyperplastic arachnoid cells in the cyst wall, which presumably participate in collagen synthesis.8 The pathogenesis of AC is still not entirely known. Meningeal tissue is of mesectodermal origin and derives from the neural crest. AC, as well as meningeomas, may be located intraventricularly, which indicates that mesectodermal tissue may be dislocated into the neural tube during the folding of the latter, which begins around embryonic day 20. A network of mesenchymal cells develops between the brain and the epidermis and eventually differentiates into a dense outer layer and a reticular inner layer to form the meninges. The inner layer further differentiates to form the dura mater and the leptomeninges.9 The improvement of ultrasound technology and the practice of routine prenatal ultrasound diagnostic provided new insight regarding the time of appearance of AC, their in utero development and the coincidence with other brain malformations. Intracranial cysts now can be detected as early as in the first trimester of pregnancy.10 AC may occur together with anomalies of the corpus callosum,11 Chiari malformations,12 skeletal malformations,13 and complex vascular malformations.14 Furthermore, they may appear in the frame of syndromes and genetic disorders such as neurofibromatosis Type 1,15 PallisterHallSyndrome or Aicardis syndrome.4 Their frequent occurrence in early childhood, histological features with absent postinflammatory or posthemorrhagic changes and the occurrence together with genetic disorders strongly suggest a developmental nature. Therefore, it is the current opinion that AC are congenital malformations. However, this opinion has recently been challenged. In children and adults, temporal and Sylvian location is highly predominant16,17 while in a prenatal analysis most AC are located interhemispherically and temporal AC are extremely rare11 (Table 1). This observation raises some doubt on the hypothesis that the formation of AC is strictly congenital. This might be confirmed by the observations, that they may develop de novo in adults,18 may grow over time and, the cyst size on first diagnosis is larger with increasing age.19 In fetuses, the subarachnoid spaces covering the temporal lobes are very deep. Under normal circumstances, these spaces regress in size from the end of gestation until the first few months of life. It may be hypothesized that any peri or postnatal event, such

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Table 1. Distribution of arachnoid cysts

Localization Supratentorial Middle fossa Interhemispheric Hemispheric Suprasellar Skull base Intraventricular Infratentorial CPA Hemispheric Other Tentorial incisure Prenatal (n = 54)11 34 1 16 1 5 7 5 12 1 1 10 8 Pediatric (n = 67)16 31 13 9 5 4 31 1 22 8 5 Elderly (n = 23)17 20 11 9

Distribution of arachnoid cysts discovered by (a) routine ultrasound screening in pregnancy, (b) in children and (c) in patients older than 65 years. Arachnoid cysts rarely become symptomatic in old patients. Published cases were collected and reviewed by Caruso etal.17 One of the infratentorial cysts was located within the fourth ventricle causing symptoms of normal pressure hydrocephalus. The differences in the distribution between pre and postnatally diagnosed arachnoid cysts is apparent. Arachnoid cysts in the middle cranial fossa do practically not appear in the prenatal period but make up the majority of lesions in the pediatric and adult age. On the contrary, the predominant localization in prenatal diagostics is interhemisheric. These lesions are less frequently found in infants and rare in adults.

as even minor hemorrhage or inflammatory processes, could lead to the formation of a cyst by inhibiting this process of regression.11 In summary, most reports suggest that there is at least a congenital mistake in the texture of the arachnoid layer. Minor irregularities of the developing CSF flow through the mesenchymal network can result in a splitting of the developing arachnoid in the fetus. The development or filling of these arachnoid pockets, however, seems to be dynamic and further changes can occur at any stage of life. NATURAL BEHAVIOUR OF ARACHNOID CYSTS The knowledge about the spontaneous behaviour of AC is not only essential for assessing the indication for surgical treatment but also, in case of prenatal diagnosis, in order to counsel parents on the further course of pregnancy. PierreKahn etal thoroughly analyzed the outcome of 56 fetuses in whom AC were diagnosed by routine ultrasound screening. In that study, 55% of the cysts were diagnosed between gestational week 20 and 30 and 45% after gestational week 30. Interestingly, the latter had normal ultrasound examinations between week 20 and 30, supporting that AC are developing dynamically. After their detection, serial followup ultrasound and MRI examinations were performed. In utero, the cysts grew in 20% of the cases, remained stable in 76% and decreased in 4%. After delivery, the cysts grew in 24%, remained stable in 52% and decreased in 24%.11 Focussing on AC of the middle cranial fossa and the Sylvian fissure, Galassi et al classified the lesions into three categories, class 1 and 2 representing small and mediumsized cyst with none or only little mass effect and a more or less open

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communication to the subarachnoid space. Class 3 represented voluminous cysts with a distinct mass effect and dislocation of midline structures and no communication to the subarachnoid space as assessed by intracisternal contrast application.20 (Fig. 1) Becker etal found that only in class 3 cysts there is a positive correlation between the age at which a patient becomes symptomatic and the size of the cyst. This observation suggests that there is an obvious growth of these large lesions. Smaller lesions, however, do not show a correlation of size and age at first admission.19 In the same study, a subgroup of

Figure 1. Coronar (T2 TSE) and transverse (T2 TIRM) MRI of a large left temporal/Sylvian anrachnoid cyst of an 8yearold boy. An asymmetric bulging of the skull was the only symptom of the otherwise normally developed child who was not surgically treated.

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patients (11 patients) was not operated on but followed by CTscans. In 3 of these, the cyst grew in followup CTscans, 6 remained unchanged while 2 decreased in size. There are several reports on a decreasing ACs volume without surgical intervention. This may occur during traumatic brain injury, allegedly by a rupture of the cyst wall with consecutive drainage into the subarachnoid space. In some cases, however, spontaneous reduction or even disappearance of AC has been observed without a history of head injury.21,22 In summary, the spontaneous course of an AC is uncertain. This has a direct implication for treatment decisions. If the finding is truly incidental or the symptoms are only mild, a conservative management might be preferred at first to observe the further development. MECHANISMS OF CYST-GROWTH

Active Fluid Secretion In an ultrastructural and cytochemical study, Go etal analyzed the resected walls of AC. They observed similarities between the neurothelial lining of arachnoid granulations such as intercellular clefts and sinusoid dilatations and desmosomal intercellular junctions, pinocytic vesicles, lysosomal structures and a basal lamina. On the luminal surface, the cyst wall is densely covered with microvilli, thus, suggesting active secretion.24 Enzyme cytochemistry demonstrated NaKATPase at the luminal plasma membrane and alkaline phosphatase at the opposite membrane. This architecture might indicate fluid transport towards the lumen. Different consistence compared to CSF may be explained by this theory. Osmotic Gradient The hypothesis that AC grow by osmotic gradients has been proposed due to the minor differences of substance concentrations compared to CSF. A thin arachnoid wall with loose connective tissue could allow a penetration of water. However, the formation of an osmotic gradient has to be triggered. In secondary cysts, hemorrhage with products of blood degradation or inflammatory reactions could act as osmotic triggers. In primary AC, there is no evidence that these mechanisms exist.25 Valve Mechanism Small and medium-sized cysts have been found to remain constant in size and to communicate readily with the normal subarachnoid space.19,20 In larger cysts or in growing cysts, a valvemechanism may develop with unidirectional fluid transport. Under

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In a number of patients, the therapeutical strategy changes with the growth of the cyst. If this occurs, it may produce neurological symptoms. In 1964, Robinson reported a different consistence of the content of the cyst and the normal cerebrospinal fluid.3 Berle and coworkers systematically analyzed the intracystic fluid and found different contents of phosphate, protein, ferritin and lactate dehydrogenase as compared to normal CSF. Possible mechanisms of cystgrowth, therefore, have to be consistent with these findings.23 A variety of mechanisms has been proposed. Active secretion by the cells of the cystwall, expansion following an osmotic gradient or a unidirectional valvemechanism may be possible mechanisms.

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circumstances with a positive extra/intracystic pressure, the cyst may increase in volume. In fact, valvelike foldings of the cyst wall or intracystic membranes have actually been observed during endoscopic or open operative procedures.26,27 Other Mechanisms Leading to Clinical Symptoms Apart from symptoms due to an enlargement of the cyst, several other mechanisms like spontaneous intracystic hemorrhage due to aneurysm rupture or traumatic brain injury have been reported to cause clinical symptoms.2830 Patients with AC appear to be prone to develop chronic subdural hematomas even after minor head trauma which can make the previously asymptomatic AC clinically apparent.31,32 Clinical symptoms due to a spontaneous or traumatic rupture of the cyst and consecutive formation of a subdural hygroma have also been described.33 CLINICAL SYMPTOMS The clinical symptoms are determined by the location of the cyst. AC can occur at any location in the cranium and spine where an arachnoid layer is present. In addition, they can be found in the ventricular system. In this case, it has to be noted, that tight adherance to the choroid plexus has been observed intraoperatively. It has been proposed that intraventricular AC arise from arachnoid tissue which has been displaced into the ventricles by ingrowing vascular mesenchyma at the time of formation of the choroid plexus.3437 The prevalence of AC in a group of healthy adults has been found to be 1.11.5%.38,39 Familial cases of AC have been described with40,41 or without association to other hereditary diseases.42-44 A predominance of the male gender for temporal AC is highly significant while cerebellopontine angle AC more often occur in females.45 To date, there is no firm explanation for this sex distribution. Typical symptoms due to AC are described below. Temporal and Sylvian Arachnoid Cysts The middle fossa is the most common location of AC. In larger series of pediatric and adult patients, approximately two third of them sustain AC in the middle fossa or in the Sylvian fissure. Rarely, temporal AC are found bilaterally; these patients may suffer from glutaric aciduria.46 In general, however, there is a predilection for the left hemisphere.45 Symptoms either result from a mass effect causing increased intracranial pressure or from an irritation of the temporal or frontal cortex. Most often, these patients present with headache and seizures as well as contralateral motor deficits.47,48 Developmental delay and retardation are more common in patients with AC.48 An asymmetry of the skull may develop as well as a facial asymmetry or exophthalmus due to a misdevelopment of the sphenoid ridge.3,49,50 There has been an ongoing debate about the association of temporal AC with psychiatric disorders and neuropsychiological deficits. Various cases of psychiatric disorders have been reported to be associated with AC. These include attention deficit hyperactivity syndrome, depressions, hallucinations, dementia, schizophrenia, anorexia nervosa, insomnia and other psychotic disorders. Some of those patients were surgically treated for their AC and an improvement after surgery was reported in some cases.51 Systematic pre and postoperative evaluation of cognitive function has been performed by Wester etal. They found that many patients with left temporal AC show

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significant deficits in various cognitive functions such as verbal perception, memory function, complex verbal tasks, visuospatial functions and visual attention. These deficits were, in fact, ameliorated in followup neuropsychological testing after surgical treatment. In many of these patients, however, these cysts did not cause complaints and neuropsychololgical deficits had not been clinically apparent until specific testing had been performed. These cysts can be interpreted as true incidental findings. Intraventricular Arachnoid Cysts Intraventricularly located AC are rare. They have been found in the lateral, third and fourth ventricles (Fig. 2). Classically, these cysts become clinically apparent by development of an acute or chronic hydrocephalus in case of increasing cyst size. Symptoms due to a compression of the surrounding structures have also been reported. AC in the fourth ventricle, for example, may cause cerebellar dysfunction27 or by depressionlike symptoms.52 Cerebellopontine Angle and Retrocerebellar Arachnoid Cysts Infratentorial location accounts for approximately 10% of all AC. The majority of these are located in the cerebellopontine angle or in retrocerebellar location.53,54 Symptoms of retrocerebellar cysts include hydrocephalus and compression of cerebellar or brainstem structures. Patients predominantly present with headaches or gait disturbances.

Figure 2. Intraoperative view after median suboccipital craniotomy and retraction of the cerebellar tonsils. The inferior vermis was slightly incised to allow sufficient overview of the fourth ventricle. A complex network of intracystic septations was found intraoperatively allowing free CSF flow after complete resection of the cyst.

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Cerebellopontine angle AC may occlude the CSF circulation in the posterior fossa and cause obstructive hydrocephalus. Cerebrllopontine angle structures may be directly compressed or affected in terms of a neurovascular compression. Cranial nerve symptoms may evolve, including hearing loss, trigeminal neuralgia, hemifacial spasm, diplopia, hoarseness, dysphagia or diplopia.5561 Supra- and Intrasellar Arachnoid Cysts Intra and suprasellar AC arise in the basal subarachnoid space in close vicinity to the optic nerve, pituitary gland and stalk, hypothalamus and mesencephalon. In case of massive suprasellar extension, they may obstruct the physiological drainage of cerebrospinal fluid at the level of the third ventricle or Foramen of Monroi, thus causing obstructive hydrocephalus. Visual impairment may be caused by compression of the optic nerve and endocrine disturbances by a compression of the pituitary gland or stalk. A compression of hypothalamic structures may cause developmental abnormalities like precocious puberty.62 A bobblehead doll syndrome with an involuntary back and forward movement of the head at a frequency of 23 per second may be due to a compression of third ventricle structures or thalamic nuclei. It is a clinical feature highly suggestive for suprasellar AC. However, this typical feature can only be found in a minority of these patients. Spinal Arachnoid Cysts Similar to cranial AC, spinal AC typically cause symptoms due to a compression of the spinal cord and nerve roots. Myelopathic symptoms like ataxia, reappearance of primitive reflexes or progressive paralysis may occur due to cord compression. Radicular symptoms due to involvement of exiting nerve roots may include pain, sensory deficits or weakness of the effector muscles (Fig. 3). DIAGNOSTIC PROCEDURES In many patients with intracranial and spinal AC, it is a challenging to evaluate the indication for surgical treatment. The success of the treatment strategy is based on the correlation of the lesion with the clinical symptoms. A detailed interview of the patient or the patients parents and a thorough neurological examination are indispensable in order to correlate signs and symptoms with the radiological finding. This particularly counts for presumably incidental cysts. If headache is the only symptom, its course should be documented and be brought in line with the results of serial imaging studies, if a conservative and observing strategy with followup imaging studies is chosen. The studies of Wester etal have demonstrated that patients with AC might have distinct cognitive deficits. In adult patients or older children, a neuropsychological testing should be recommended in order to detect more obscure clinical symptoms. Laboratory investigations for screening of glutaric aciduria must be recommended in patients with bilateral AC.46 In fetuses with AC detected by routine ultrasound screening, karyotype evaluation and fetal MRI should be performed to rule out a possible association with other malformations.11 Electroencephalography is essential in patients with supratentorial AC in order to determine the necessity for antiepileptic medication. In spinal AC a cord compression may

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Figure 3. T2weighted sagittal (A) and axial (Th 8) (B) magnetic resonance images of a large spinal arachnoid cyst in a 20yearold female who presented with a progressive paraparesis. The spinal cord is thinned and shifted anteriorly by the space occupying cyst. After hemilaminectomy, fenestration and excision of intracystic membranes, the patient recovered completely.

be further evaluated by somatosensory and motor evoked potentials. Magnetic resonance imaging (MRI) is the mainstay of radiological diagnostics. Due to their worse imaging quality and because of Xray exposure, computed tomography scan (CTS) is, especially in children, not acceptable as a diagnostic tool. Modern phase contrast MRI and functional MRI are not only capable of demonstrating structural abnormalities but also able to depict CSFflow patterns and functional organization of the brain respectively. Cisternography with contrast filling of the subarachnoid space followed by CTS, sometimes at more than one timepoint to evaluate the timecourse of contrastfilling of CSFcompartments, is reserved for very few cases of diagnostic uncertainty.

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INDICATION FOR TREATMENT As discussed above, the successful treatment of AC depends on a thorough patient evaluation and determination of the appropriate treatment strategy. Rarely, patients present with signs of an acute obstructive hydrocephalus or compression of vital structures caused by an expansion of an AC requiring emergent decompression.52,63 Hemorrhage into an AC, e.g., by rupture of an intracranial aneurysm, might be another emergent situation, requiring rapid surgical intervention.28 The obvious association of AC with chronic subdural hematomas necessitates evacuation of the hematoma but not primarily a treatment of the asymptomatic AC.31 In contrast, subdural hygroma associated with an AC has been reported to disappear by a therapy with acetazolamind, avoiding surgery.64,65 In cases of progressive chronic hydrocephalus and focal neurological deficits like aphasia, motor deficits, cranial nerve deficits, spinal cord compression, surgery is to be recommended if the AC is the obvious cause of the respective deficit. Concerning epilepsy, the benefit of surgery is not clear. Most patients seem to improve as the frequency of seizures decreases. However, medical therapy might be as effective hence is the first choice of treatment.47 Headaches are the most frequent complaint of patients with an AC. However, the origin of headaches is manifold and in many patients with headaches, AC must be regarded as an incidental finding. The results of surgical treatment are discussed controversially. Some authors are restrictive to operate on patients with headaches as the sole symptom,47 others report very good results of surgery for headaches as the leading symptom.66,67 Headaches might be a sign of an intermittent elevation of intracranial pressure. The diagnostic challenge is the diagnosis of elevated ICP. In some patients, clear signs are visible on imaging studies. In cases of doubt, invasive ICP monitoring might be performed as reported by di Rocco etal.68 For patients with only moderate and infrequent headaches, responding to medication, a nonoperative strategy is adequate since the fate of AC is uncertain and a decrease in size is possible.19 Continuous followup examinations with assessment of the course of complaints, neurological symptoms and intake of analgetics are necessary in these cases. In systematic assessment of cognitive functions, patients with AC have been found to have reproducible deficits. Moreover, these deficits have been reported to improve after surgical therapy.69 However, many of the patients assessed did not report specific subjective complaints. A too aggressive surgical strategy bears the danger to impose operative risks on the patient without improving the quality of life. In addition, the cysts may spontaneously decrease in size. Similar to patients presenting with headaches, a followup strategy may be chosen including imaging studies, neurological and neuropsychological examinations. TREATMENT There are several options for treatment of AC. Resection of the cystwalls requires open surgery. Fenestration of the cyst with creation of an opening to the subarachnoid cisterns can be performed endoscopically or in an open microsurgical technique. Various shunting techniques have been reported including cystoperitoneal and cystosubdural shunts. There is an ongoing debate on the optimal surgical strategy. The form of treatment which is individually chosen, depends on various factors: The patients symptoms, the location and shape of the cyst, the extent of intracystic septations, the vicinity to relevant neural and vascular structures and the surgeons experience with different techniques. For patients without relevant symptoms and without related neurological deficits, observation

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may be recommended. If there is an indication for treatment, endoscopic fenestration can be recommended as the treatment of first choice for many AC, in particular if there are no relevant intracystic septations. For suprasellar arachnoid cysts, very good results have been reported. Ventriculocystostomy and ventriculocystocisternostomy seem to be the most successful longterm treatment. Temporal and temporobasal AC appear to be more difficult to be treated by endoscopic techniques.70 If there are intracystic septations or multiple fine neural structures like in the cerebellopontine angle, the recurrence rate might be higher if treatment only consists of cystfenestration. Complete or nearcomplete excision of the cyst wall or adherences supplies the most successful treatment at this locations (Fig. 4).53 For small or mediumsized temporal cysts, excision or fenestration supplies a good chance of complete disappearance of the cyst and complete reexpansion of the adjacent temporal lobe. For large cysts, an only incomplete expansion of the adjacent brain has been

Figure 4. Preoperative (A) T2weighted sagittal MRI depicting a large septated arachnoid cyst in the fourth ventricle containing multiple septations. Those were confirmed intraoperatively (see Fig. 4). Brainstem structures are displaced anteriorly. The lateral ventricles appear slightly enlarged as compared to postoperative imaging (B). Postoperative MRI shows a regular flowvoid in the aquaeductus mesencephali.

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demonstrated. Additional shunt placement may be required.71 Shunting procedures are only little invasive, supply good control of the cyst volumes by pressurecontrolled drainage, but may have a relatively high recurrence and revisionrate.72 Fenestration or excision of the cyst may bear a higher operative risk, but leaves the patient shuntindependent. A longterm successrate of 7690% has been reported for these ways of treatment.70,72 The necessity to treat and the choice of the optimal way of treatment have to be chosen deliberately. All forms of treatment have more or less operative risks and recurrence rates. Retreatment, again, imposes a recurrent treatmentrisk on the patient and patients may eventually become discontented. Conclusion

REFERENCES
1.Bright R. Serous cysts in the arachnoid diseases of the brain and nervous system, part I: reports of medical cases selected with a view of illustrating the symptoms and cure of diseases by a reference to morbid anatomy. 1831; 2:437439. 2.Cunningham DJ. A Large subarachnoid cyst involving the greater part of the parietal lobe of the brain. J Anat Physiol 1879; 13:508517. 3.Robinson RG. The temporal lobe agenesis syndrome. Brain 1964; 87:87106. 4.Bannister CM, Russell SA, Rimmer S etal. Fetal arachnoid cysts: their site, progress, prognosis and differential diagnosis. Eur J Pediatr Surg 1999; 9 Suppl 1:2728. 5.ShuklaDave A, Gupta RK, Roy R et al. Prospective evaluation of in vivo proton MR spectroscopy in differentiation of similar appearing intracranial cystic lesions. Magn Reson Imaging 2001; 19:103110. 6.Inoue K, Seker A, Osawa S etal. Microsurgical and endoscopic anatomy of the supratentorial arachnoidal membranes and cisterns. Neurosurgery 2009; 65:644664. 7.Miyagami M, Tsubokawa T. Histological and ultrastructural findings of benign intracranial cysts. Noshuyo Byori 1993; 10:151160. 8.Rengachary SS, Watanabe I. Ultrastructure and pathogenesis of intracranial arachnoid cysts. J Neuropathol Exp Neurol 1981; 40:6183. 9.Angelov DN, Vasilev VA. Morphogenesis of rat cranial meninges. A light and electronmicroscopic study. Cell Tissue Res 1989; 257:207216. 10.Bretelle F, Senat MV, Bernard JP etal. Firsttrimester diagnosis of fetal arachnoid cyst: prenatal implication. Ultrasound Obstet Gynecol 2002; 20:400402. 11.PierreKahn A, Hanlo P, Sonigo P etal. The contribution of prenatal diagnosis to the understanding of malformative intracranial cysts: state of the art. Childs Nerv Syst 2000; 16:619626. 12.Piatt JHJ, DAgostino A. The Chiari II malformation: lesions discovered within the fourth ventricle. Pediatr Neurosurg 1999; 30:7985. 13.Holmes LB, Redline RW, Brown DL et al. Absence/hypoplasia of tibia, polydactyly, retrocerebellar arachnoid cyst, and other anomalies: an autosomal recessive disorder. J Med Genet 1995; 32:896900. 14.Ito J, Yamazaki Y, Honda H etal. Angiographic appearance of a huge retrocerebellar arachnoid cyst in an infant. Neuroradiology 1977; 13:115119.

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Arachnoid cysts are benign malformations of the arachnoid texture. They can occur in any location in the cranium or spinal canal with an arachnoid layer. They can become symptomatic by growth causing a mass effect or by intracystic hemorrhage. The clinical symptoms mainly depend on the location of the cyst. With increasing availability of diagnostic facilities, in particular MRI, many arachnoid cysts are discovered incidentally. Therapeutically, endoscopic fenestration of the cyst, open resection of the cystwalls or permanent drainage of the intracystic fluid via a cystosubarachnoid or cystoperitoneal shunt are possible. The most challenging task is to evaluate the indication and the appropriate way of surgical treatment.

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