You are in: eMedicine Specialties > Radiology > BRAIN/SPINE Arachnoid CystArticle Last Updated: Apr 3, 2007AUTHOR AND EDITOR INFORMATIONAuthor: Ali Nawaz Khan, MBBS, LRCP, FRCS, FRCP, FRCR, Chairman of Medical Imaging, Professor of Radiology, NGHA, King Fahad Hospital, King Abdulaziz Medical City, Riyadh, Saudi Arabia Ali Nawaz Khan is a member of the following medical societies: American Institute of Ultrasound in Medicine, Radiological Society of North America, Royal College of Physicians, Royal College of Physicians and Surgeons of the USA, Royal College of Radiologists, and Royal College of Surgeons of England Coauthor(s): Ian Turnbull, MD, Lecturer, Department of Radiology, University of Manchester; Consulting Neuroradiologist, Hope Hospital, Salford, Manchester and North Manchester Hospital; Riyadh Al-Okaili, MBBS, Interventional/Therapeutic and Diagnostic Neuro-Radiologist, King Abdulaziz Medical City; Sumaira Macdonald, MBChB, MRCP, FRCR, PhD, Lecturer, Sheffield University Medical School; Endovascular Fellow, Sheffield Vascular Institute; Khalid Mahmood, MBBS, FCPS, Locum Appointment Training Specialist Registrar, Department of Radiology - Paediatric, Royal Liverpool (Alder Hey) Children's Hospital Editors: Lucien M Levy, MD, PhD, Director of Neuroradiology, Professor of Radiology, Department of Radiology, George Washington University Medical Center; Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand; Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute; James G Smirniotopoulos, MD, Professor of Radiology, Neurology, and Biomedical Informatics, Chairman, Department of Radiology and Radiological Sciences, Uniformed Services University of the Health Sciences Author and Editor Disclosure Synonyms and related keywords: glioependymal cyst, meningeal cyst, intra-arachnoid cerebrospinal fluid–containing cysts, intracranial mass, leptomeningitis, arachnoid diverticula, spinal arachnoid cysts, intradural spinal arachnoid cysts, agenesis of corpus callosum, endocrinopathy, kyphoscoliosis, myelodysplasia, spinal dysraphism, intracystic hemorrhage, subdural hygroma, subdural hematoma, calvarial bulging, intracranial hypertension, craniomegaly, developmental delay, visual loss, precocious puberty, seizures, focal neurologic signs, temporal lobe agenesis, supratentorial cysts, chronic subdural hematomas, pneumosinus dilatans of sphenoid sinus, Cockayne syndrome, Menkes disease, spinal cord displacement, spinal cord compression, epidural arachnoid cysts INTRODUCTIONBackgroundArachnoid cysts represent benign cysts that occur in the cerebrospinal axis in relation to the arachnoid membrane and do not communicate with the ventricular system. They usually contain clear, colorless fluid that is most likely normal cerebrospinal fluid, but they rarely contain xanthochromic fluid. Most are developmental anomalies. A small number of arachnoid cysts are acquired, such as those in association with neoplasms or those that are due to adhesions following leptomeningitis, hemorrhage, or surgery. They constitute approximately 1% of intracranial masses, with 50-60% occurring in the middle cranial fossa. Cysts in the middle cranial fossa are found more frequently in males and on the left side. Most arise as developmental anomalies. A small number of arachnoid cysts are associated with neoplasms or occur as complications of adhesions following leptomeningitis, hemorrhage, or surgery. Arachnoid cysts also occur within the spinal canal, in which arachnoid cysts or arachnoid diverticula may be located subdurally or in the epidural space, respectively. Spinal arachnoid cysts are commonly located dorsal to the cord in the thoracic region. A cyst in this location is usually secondary to a congenital or acquired defect and is situated in an extradural location. Intradural spinal arachnoid cysts are secondary to a congenital deficiency within the arachnoidal trabecula, especially in the septum posticum, or are the result of adhesions resulting from previous infection or trauma. Microscopic examination shows that their walls are formed from a splitting of the arachnoid membrane, with an inner and outer leaflet surrounding the cyst cavity. Arachnoid cysts often are an incidental finding on imaging, and usually, patients are asymptomatic even if the cyst is quite large. The most commonly associated clinical features are headache, calvarial bulging, and seizures, with focal neurologic signs occurring less frequently. Controversy surrounds the treatment of arachnoid cysts. Some clinicians advocate treating only patients with symptomatic cysts, while others believe that even in asymptomatic patients, cysts should be decompressed to avoid future complications. The most effective surgical treatment appears to be excision of the outer cyst membrane and cystoperitoneal shunting. PathophysiologyCystic lesions localized within the arachnoid membrane may be classified according to the location along the neural axis or by the histologic composition of the cyst wall, which is either arachnoid connective tissue or glioependymal tissue. Distribution of these 2 types of cysts differs along the neural axis. The cysts located along the cerebral convexity and in the spinal cord are mostly arachnoid, while cysts found in the supracollicular or retrocerebellar region may be either arachnoid or glioependymal cysts. Microscopic examination of arachnoid cysts shows that the walls are formed from a splitting of the arachnoid membrane, with an inner and outer leaflet surrounding the cyst cavity. The cyst wall consists of fibrous connective tissue slightly denser than normal arachnoid tissue, with hyaline change at times. No epithelial lining is present. The outer wall of the cyst adheres loosely to the dura. The cyst wall is devoid of blood vessels, and changes of inflammation or hemorrhage seldom occur. Arachnoid cysts usually occur in association with normal arachnoid cisterns, and such cysts are congenital, arising from arachnoid clefts and arachnoid duplications. Glioependymal cysts are rare; only a few instances of interhemispheric glioependymal cysts are known. Glioependymal cysts may be associated with agenesis of the corpus callosum, heterotopia, and other dysplasias. Arachnoid cyst expansion most likely occurs because of intracranial pulsation pushing CSF through defects (which behave like valves) to become entrapped in arachnoid locations or, less likely, because of fluid secretion by the cyst wall perhaps along an osmotic gradient. The cysts may be unilocular or loculated by septations. The wall of the cyst is usually smooth. Most cysts are filled with clear, colorless fluid of low protein content that is comparable to that of CSF. A few cysts may contain elevated protein content. Gross appearances of glioependymal cysts usually are indistinguishable from arachnoid cysts; however, their microscopic appearances vary. Glioependymal cysts have epithelial lining and may bear cilia. When the epithelial lining is lacking, glial tissue is seen to line the lesion. Glioependymal cysts are believed to derive from displaced neuroectodermal tissue. Histologic classification is only of systematic interest and has little bearing on prognosis. Acquired arachnoid cysts may develop following surgery, trauma, subarachnoid hemorrhage, or neonatal infections and can occasionally occur in association with extra-axial neoplasm. Arachnoid cysts associated with tumors develop as a consequence of CSF loculation surrounded by arachnoid scarring, with expansion due to osmotic filtration or via a ball-valve mechanism. These acquired arachnoid cysts have been described variably as acquired, secondary, or leptomeningeal cysts. The reason arachnoid cysts grow and become space occupying is far from clear. No inner lining is present through which active transport can take place. Neurosurgeons have observed ostia with pulsating fluid in exposed cysts, suggesting a hydrodynamic flap-valve or ball-valve mechanism. Recently, cine MRI has shown abnormal fluid flow in intradural spinal arachnoid cysts presenting with cord compression. A common location is in the floor of the middle cranial fossa—in particular, anteriorly and the parasagittal location in the interhemispheric fissure. Most cysts are unilateral, smoothly rounded, and adhere loosely to the dura. Arachnoid cysts can indent deeply into the hemisphere or invaginate into major fissures, displacing and flattening the underlying cortex. Compression severe enough to cause tissue necrosis is exceptional. Other locations of arachnoid cysts include the suprasellar/chiasmatic site (which may produce endocrinopathy), the cerebellopontine angle (11%), the quadrigeminal plate cistern (10%) in relationship to the vermis (9%), and the prepontine/interpeduncular cistern (3%). Patients with spinal arachnoid cysts may become symptomatic because of local cord displacement/cord compression. Typically, this occurs at the midthoracic level and, less frequently, at the lumbosacral or sacral level. Epidural arachnoid cysts often are associated with kyphoscoliosis in juveniles. Spinal arachnoid cysts form elongated membranous sacs of CSF dorsal to the cord. Some cysts have no connection to the dura, while others may communicate with the subarachnoid space via a thin stalk penetrating the dura. Arachnoid cysts are associated with myelodysplasia in spinal dysraphism. FrequencyUnited StatesArachnoid cysts constitute 1% of intracranial masses, with 50-60% occurring in the middle cranial fossa. Mortality/MorbidityMorbidity and mortality depend on the location of the arachnoid cyst and complications, such as acute mass effect by intracystic hemorrhage or the development of a subdural hygroma/hematoma. The exact incidence of mortality and morbidity from an arachnoid cyst is not known. SexThe male-to-female ratio is 4:1. AgePatients who are symptomatic for arachnoid cysts can present at any age. Clinical DetailsArachnoid cysts often are an incidental finding on imaging, and patients usually are asymptomatic even if the cyst is quite large. The most common associated clinical features include headache, calvarial bulging, intracranial hypertension, craniomegaly, developmental delay, visual loss, precocious puberty, and seizures, with focal neurologic signs occurring less frequently. Arachnoid cysts are known to rupture into the subdural space or undergo intracystic hemorrhage. Anderson and Landing1 and Aicardi and Bauman2 described the clinicopathologic features of supratentorial cysts in infancy. Neither a history of antecedent trauma nor the clinical features of preceding cranial trauma were noted in the infants. The infants had a tense fontanellle and widened cranial sutures. Craniomegaly was present either diffusely or with localized protrusion, with transillumination and thinning of the calvarium, as noted on skull radiographs. In symptomatic patients, clinical features depend on the location of the arachnoid cyst. Cysts of the middle cranial fossa (50%) may compress the tip of the temporal lobe, displacing it in the occipital direction. Middle cranial fossa cysts have been linked to ipsilateral chronic subdural hematomas. Rarely, they may communicate with the subdural space, forming a slitlike extension over the hemispheric surface. Arachnoid cysts are found in several syndromes, but data are not sufficient to indicate whether the association is typical or fortuitous. Arachnoid cysts can be associated with Cockayne syndrome and Menkes syndrome. Ependymal cysts are common in oral-facial-digital syndromes. Patients with spinal arachnoid cysts may become symptomatic as a result of local cord displacement or cord compression. Typically, spinal arachnoid cysts occur at the midthoracic level and, less frequently, at the lumbosacral or sacral level. Epidural arachnoid cysts often are associated with kyphoscoliosis in juveniles. Arachnoid cysts also are associated with myelodysplasia in spinal dysraphic lesions. Pain produced by intraspinal arachnoid cysts typically is aggravated by the Valsalva maneuver, which increases pressure within the cyst. Preferred ExaminationMRI is the diagnostic procedure of choice because of its ability to demonstrate the exact location, extent, and relationship of the arachnoid cyst to adjacent brain or spinal cord. Myelography and CT myelography remain of diagnostic value, especially for cases that are not definitive on MRI. Plain radiographic findings are nonspecific and have little to offer in the diagnosis of arachnoid cysts, although changes in skull contour may be detected on skull radiographs performed for other indications, such as trauma. Cranial ultrasonography is an important diagnostic tool during the first year of life, and although symptomatic arachnoid cysts are comparatively rare in infants, ultrasound provides a noninvasive imaging technique with high yield in the detection and characterization of cystic masses. Although angiography can show associated anomalies of venous drainage and the relationship of the lesion to normal vasculature if needed for surgical planning, in practice, angiography is rarely performed, because CT angiography or magnetic resonance angiography may provide the same information noninvasively. Limitations of TechniquesSkull radiographic findings cannot be relied upon because the signs are nonspecific. Arachnoid cysts may be confused with several intracranial cysts of various etiologies (see Differential Diagnosis, below). The confusion is much more likely to occur with CT. CT attenuation values and signal intensities parallel those of CSF, but difficulties may be encountered in cases of hemorrhagic cysts. The most important differentiation to make is between arachnoid and epidermoid cysts; MRI diffusion-weighted images (DWIs) make differentiating the 2 masses easier. Some arachnoid cysts may contain proteinaceous fluid or blood, and signal loss on DWIs may not be marked, which may pose diagnostic problems. Also, tissue contrast with fluid-attenuated inversion recovery (FLAIR) imaging is similar to that with a T2-weighted image, but FLAIR shows no signal arising from the CSF. Thus, unlike with epidermoid cysts, arachnoid cysts containing CSF demonstrate a suppressed (low) signal on FLAIR. DIFFERENTIALSEpidermoid, Brain
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| Media file 1: T2-weighted sagittal MRI image (see Image 2 for axial view) of the brain in a 28-year-old woman with an incidental finding of a superior cerebellar cistern arachnoid cyst (arrow). | |
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| Media file 2: T2-weighted axial MRI image (see Image 1 for sagittal view) of the brain in a 28-year-old woman with an incidental finding of a superior cerebellar cistern arachnoid cyst (arrow). | |
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| Media file 3: Unenhanced CT scan of the head in a 26-year-old man with a history of seizures since childhood (same patient as in Image 4). The scan shows a large left frontoparietal cyst with a mass effect. The cyst was resected, and histologic analysis confirmed an arachnoid cyst. | |
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| Media file 4: Unenhanced CT scan of the head in a 26-year-old man with a history of seizures since childhood (same patient as in Image 3). The scan shows a large left frontoparietal cyst with a mass effect. The cyst was resected, and histologic analysis confirmed an arachnoid cyst. | |
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| Media file 5: Axial T2-weighted MRI image through the midbrain, showing a right middle cranial fossa homogeneous lesion (same lesion as in Images 6-8) with CSF signal intensity and no perceptible wall or internal complexity. There is associated remodeling of the adjacent sphenoid bone and brain displacement. These imaging features are typical of an arachnoid cyst. | |
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| Media file 6: Axial T2-weighted MRI image through the body of the lateral ventricles, showing superior extension of a right middle cranial fossa lesion (same lesion as shown in Images 5, 7-8). The lesion is homogeneous, with no perceptible wall, no internal complexity, and CSF signal intensity. There is associated remodeling of the adjacent calvarium and brain displacement. These imaging features are typical of an arachnoid cyst. | |
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| Media file 7: Coronal T1-weighted MRI image through a brain lesion (same lesion as in Images 5-6, 8), showing homogeneity of the lesion, lack of a perceptible wall, lack of internal complexity, and CSF signal intensity. There is associated remodeling of the adjacent calvarium and brain displacement. These imaging features are typical of an arachnoid cyst. | |
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| Media file 8: Sagittal fluid-attenuated inversion recovery (FLAIR) weighted image through a brain lesion (same lesion as shown in Images 5-7), showing homogeneity of the lesion, lack of a perceptible wall, lack of internal complexity, and CSF signal intensity. There is associated remodeling of the adjacent sphenoid bone and brain displacement. These imaging features are typical of an arachnoid cyst. | |
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| Media file 9: Prenatal coronal T1-weighted MRI images through the middle cranial fossa, showing a left temporal fossa homogeneous lesion (postnatal images of same patient shown in Images 10-11) with CSF signal intensity and no perceptible wall or internal complexity. There is associated displacement of adjacent brain. These imaging features are typical of an arachnoid cyst. | |
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| Media file 10: Postnatal coronal T2-weighted MRI images through the middle cranial fossa, showing a left temporal fossa homogeneous lesion (prenatal images of same patient shown in Image 9) with CSF signal intensity and no perceptible wall or internal complexity. There is associated remodeling of adjacent calvarium, brain displacement, and a midline shift. These imaging features are typical of an arachnoid cyst. | |
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| Media file 11: Postnatal coronal T1-weighted MRI images through the middle cranial fossa, showing a left temporal fossa homogeneous lesion (prenatal images of same patient shown in Image 9) with CSF signal intensity and no perceptible wall or internal complexity. There is associated remodeling of adjacent calvarium, brain displacement, and a midline shift. These imaging features are typical of an arachnoid cyst. | |
![]() | View Full Size Image | Media type: MRI |
Article Last Updated: Apr 3, 2007