Arachnoid cysts are common incidentally found lesions in the brain when imaging is being performed.[1][2][3] The majority of arachnoid cysts are in the anterior/middle cranial fossa or in a retrocerebellar location. The etiology of most arachnoid cysts is unknown, but very likely the majority occur during development. These cysts occur as small or large fluid-filled lesions. Even large arachnoid cysts are often incidental and not associated with symptoms. However, many symptoms have been associated with the mass effect caused by an arachnoid cyst, and at times the arachnoid cyst does need to be treated secondary to the anatomical distortion and the neurological symptoms it is causing.
The etiology of formation is uncertain, but arachnoid cysts, in theory, are predominantly formed due to abnormal splitting during embryogenesis of the arachnoid. If histopathology shows the presence of inflammatory cells, excess collagen, or hemosiderin staining, then an inflammatory or traumatic etiology is suggested. Underlying gliosis is not usually found in the adjacent brain secondary to mass effect. Some syndromes have been associated with arachnoid cysts, and these syndromes include Aicardi syndrome, mucopolysaccharidosis, acrocallosal syndrome, Marfan syndrome, a missense mutation (c.2576C>T) in the arginine-glutamic acid dipeptide repeats gene (RERE), and Chudley-Mullough syndrome.[4][5][6][7][8][9][10]
The arachnoid cyst consists of a thin membrane. Histologically, the wall has been found to be variable but predominantly composed of arachnoid. There has also been found within arachnoid cysts fibrous tissue with a simple epithelial lining, and non-arachnoid luminal epithelia with plentiful microvilli and/or cilia.[11][12]
A wide range in the percentages of symptomatic versus asymptomatic cysts has been reported, and this reported rate will depend greatly on the population in which it is being calculated. The location of the arachnoid cyst is quite variable, with a clear dominance of lesions consistently found in the middle cranial fossa and the retrocerebellar locations. A smaller number of cysts are present at the convexity and in the suprasellar region. The lowest frequency of lesions is found in the cerebellopontine angle, intraventricular space, supracerebellar cistern, quadrigeminal cistern, ambient cistern, anterior fossa, and brainstem. A significant variation is reported in the incidence of the less frequent arachnoid cysts locations. For instance, Al-Holou et al. report very few intraventricular cysts in children (0.3%), no intraventricular cysts in adults, and Hall et al. reported 12% of cysts to be intraventricular in children.[1][2][3]
Most commonly, the arachnoid cyst will be detected incidentally unless a patient is sent to a referral center due to being symptomatic. Fortunately, the most common locations for cysts in the middle cranial fossa and retrocerebellar region are not associated with symptoms. Cysts in less common locations are found to be more likely symptomatic. In unusual cases, when arachnoid cysts cause symptoms, they present mainly by two mechanisms. One is secondary to mass effect, and the other is due to rupture. Arachnoid cysts are discovered related to many different presentations since they are usually incidental and not associated with the reason the patient is being imaged. Arachnoid cysts have been found during imaging work-up for headache, seizure, increased intracranial pressure, hydrocephalus, suspected stroke, hemiparesis, nausea/vomiting, dizziness, cranial nerve dysfunctions, tinnitus, vertigo, developmental delay/regression, behavior concerns, visual symptoms, and trauma.[1][2][3]
Even in patients who present with a headache, if an arachnoid cyst is detected, it most likely is not related to the headache and is an incidental finding. However, careful evaluation of each case is required. The arachnoid cyst can be very large and can be a definitive cause of headaches and seizures.[13] Larger cysts are more likely to be symptomatic than small cysts, and larger cysts are more likely to undergo an operation.
It is rare for the arachnoid cyst to be a cause of a significant neurological symptom such as hydrocephalus, ataxia, or cranial nerve impingement. However, given the relatively common incidence and the many locations that arachnoid cysts occur in, an extensive array of symptoms are caused by arachnoid cysts. These include vision loss, nausea/vomiting, macrocephaly, third nerve palsy, trochlear dysfunction, trigeminal neuropathy, hemifacial spasm, sensory neuronal hearing loss, facial palsy, vagus nerve palsy, vertigo, and eighth cranial neuropathy.[3][14][15][16][17][18][19][20][21] The symptoms depend on the cyst location. A quadrigeminal plate cistern arachnoid cyst could compress the trochlear nerves and quadrigeminal plate and result in trochlear nerve dysfunction.[22] An arachnoid cyst impinging the optic nerve could cause a deficit in the ipsilateral visual field in the eye of origin, a cyst in the suprasellar cistern could cause bitemporal hemianopsia, and a cyst affecting the occipital cortex would cause homonymous hemianopsia.
Very unusual symptomatology has resulted from arachnoid cysts. Bobble-head doll syndrome, a rhythmic movement of the head, has been reported related to third ventricular dilation and potentially cerebellar involvement.[23][24] Depression that resolves with marsupialization of an arachnoid has occurred. The presentation of an arachnoid cyst causing depression is quite rare but plausible given the location and marked mass effect the cyst had on the brain.[25]
The rupture of an arachnoid cyst to cause a subdural hygroma is rare.[26][27][28] The rupture can be due to trauma, or the arachnoid cyst can rupture spontaneously. Usually, the resulting subdural hygroma can be followed without treatment, but surgical intervention might be needed.[26][28] Also, the traumatic rupture of an arachnoid cyst rarely results in hemorrhage in the arachnoid cyst or a subdural hematoma.
The spinal arachnoid cyst is rare.[29] Arachnoid cysts are found throughout the spinal column but most commonly in the thoracic region. The most common symptoms relate to backache and fluctuating leg weakness. There have shown correlations with monoparesis, radicular pain, spastic quadriparesis, neurogenic claudication, sensory disturbance, monoplegia, incontinence, pain, paresthesia, and neurogenic bladder reported. More commonly, the cysts will be located posteriorly but can be located anteriorly.[29][30]
Computed tomogram (CT) imaging is often enough to make a diagnosis of an arachnoid cyst when the lesion is in an expected location with the appearance of a fluid-filled thin-walled cyst. When additional information is needed, an MRI is the imaging modality of choice for evaluating the anatomical location, size, and structures involved by an arachnoid cyst. MRI also can help refine the differential diagnosis when needed. The arachnoid cyst follows cerebrospinal fluid (CSF) on all pulse sequences unless it has experienced traumatic injury and blood products are present. Hemorrhage within an arachnoid cyst is rare.
The wall of the cyst is thin, and special pulse sequences can be employed to visualize the wall. The differential diagnosis for an arachnoid cyst includes epidermoid cyst, dermoid cyst, abscess, and other cysts such as cystic neurocysticercosis. On magnetic resonance imaging (MRI), the arachnoid cyst follows the signal intensity of CSF on T1 (dark signal), T2 fast spin-echo (FSE), or T2 spin-echo (SE) (high signal), T2-FLAIR (dark signal), and diffusion (dark signal). The apparent diffusion coefficient (ADC) maps created from the diffusion data will demonstrate the cyst with similar high ADC values as the CSF. Due to CSF pulsations, there can be mild signal differences between the fluid in the arachnoid cyst and the normal CSF on MRI sequences. Arachnoid cysts do not enhance.
Epidermoid cysts will be similar to CSF on T2 FSE and T2 SE, but on other sequences, will have variations in the signal, will not enhance, and have markedly elevated signal on diffusion-weighted imaging. This is a useful characteristic. Also, epidermoid cysts tend to be located anteriorly and laterally from the brainstem. A dermoid/lipoma can be differentiated on CT or MRI with signal intensities that follow fat. A T1 sequence with and without fat saturation to demonstrate the presence of fat on MRI is very useful to confirm a dermoid cyst/lipoma.
An abscess can be cystic in appearance, but due to inflammatory and cellular debris, the internal signal will not be similar to CSF. Very likely, the central fluid of an abscess will have a markedly elevated diffusion-weighted image (DWI) signal, similar to an epidermoid, and rim enhancement that is most often thin. Although thick rim enhancement is seen with some bacterial and fungal infections.
Neurocysticercosis cysts in the CSF (racemose neurocysticercosis) can have individual cysts that are very similar in appearance to an arachnoid cyst, but these cysts are very likely multiloculated. In cases of neurocysticercosis, there are very likely other findings indicating neurocysticercosis on brain imaging, in history, and by immunology workup. Usually, with neurocysticercosis, there are brain lesions with calcifications.
CT cisternography and MRI cisternography can be performed to confirm a diagnosis and to further evaluate an arachnoid cyst. In CT cisternography, a neuro safe non-ionic iodinated contrast is injected intrathecally, and then dynamic CT images of the arachnoid cyst are obtained. The amount of filling and the time it takes to fill the cyst can be utilized to guide management. Cysts that fill completely and early are considered freely communicating and may not need to be treated.[31]
MRI cisternography can be performed without contrast or with contrast. Intrathecal contrast-enhanced MRI cisternography has classically been thought to offer the advantage of demonstrating the connection of the arachnoid cyst with the surrounding CSF space. The MRI non-contrast techniques - constructive interference in steady-state (CISS), fast imaging employing steady-state acquisition (FIESTA), and 3D T2-weighted sampling perfection with application-optimized contrast with different flip-angle evolutions (3D SPACE) - demonstrate the arachnoid cyst wall and demonstrate surrounding structures including cranial nerves and vessels. Clear demonstration of the surrounding structures can help decrease surgical morbidities. The 3D SPACE technique is useful for demonstrating a connection to the CSF space. The 3D SPACE demonstrates signal loss in areas of CSF flow, and these areas of signal loss from flow highlight the connection of the cyst and the CSF space.
In a similar fashion, phase-contrast MRI can be utilized to evaluate for a CSF connection to an arachnoid cyst. The flowing CSF is specifically demonstrated as a signal change on phase contrast. However, secondary to false positives, a connection needs to be confirmed with contrast MR cisternography.[32]
The radiologist needs to evaluate the brain structures for mass effect secondary to the arachnoid cyst. This might be for a subtle mass effect on a cranial nerve or a prominent mass effect resulting in brain herniation. The final analysis of the arachnoid cyst should only be made after obtaining an accurate history from the clinician. An accurate history will assure that the radiology report properly reflects information about pertinent cranial nerve or vascular structures that are being clinically affected by the arachnoid cyst. The radiologist also needs to be proactive when there is a cyst in a critical location that is causing hydrocephalus or if a large cyst is present that is resulting in mass effect and brain herniation. In these situations, the ordering clinician needs to be called, or a neurosurgeon needs to be called directly.
The vast majority of cases of arachnoid cysts need no treatment. In cases when it has been determined that an arachnoid cyst is causing or is very likely causing symptoms, numerous surgical treatments exist. The surgical approach is often dictated by the location of the cyst and the involvement or lack of involvement of surrounding neurological structures. Surgical excision of the wall is performed with the creation of communication with the subarachnoid space when the lesion is readily amendable.[21][31] Also, surgical fenestration or endoscopic fenestration (opening of the cyst cavity into the closest part of the ventricular system) is utilized.[23][24][29][30]
Rarely, the placement of a cystoperitoneal shunt is done.[24][33] Shunting is avoided when possible due to the complications that can arise with long-term shunt placement. Unusual treatments are undertaken with unusual locations of the cyst, such as percutaneous aspiration through the foramen ovale.[16] Surgery is almost always curative, but recurrences have been reported.[34] When significant hydrocephalus or mass effect is present, emergency treatment could be needed.
The differential diagnosis for an arachnoid cyst includes epidermoid cyst, dermoid cyst, abscess, and other cysts such as cystic neurocysticercosis. The differential is defined predominantly on the basis of the radiological evaluation.
In the vast majority of cases, no treatment is necessary. When needed, surgery is almost always curative, but recurrences have been reported.[34]
There are usually no complications of an arachnoid cyst. If there is a rare complication from the arachnoid cyst, it is dependent on the location and what anatomical structure is distorted by the arachnoid cyst. A ruptured arachnoid cyst can also cause subdural hygromas or subdural hematomas that need to be treated.
Patients need to be informed that the vast majority of arachnoid cysts are incidental and no treatment is necessary. Only in rare situations does the arachnoid cyst result in the need for treatment.
The vast majority of arachnoid cysts are incidental, and no special communication needs to take place outside of the radiology report. In very rare cases when the arachnoid cyst is causing significant mass effect, hydrocephalus, or has ruptured and resulted in a significant subdural hygroma or hematoma, the radiologist will need to emergently call the referring clinician or a neurosurgeon. The patient may need to go to an emergency department and be evaluated. [Level 5]
[1] | Al-Holou WN,Terman S,Kilburg C,Garton HJ,Muraszko KM,Maher CO, Prevalence and natural history of arachnoid cysts in adults. Journal of neurosurgery. 2013 Feb [PubMed PMID: 23140149] |
[2] | Al-Holou WN,O'Lynnger TM,Pandey AS,Gemmete JJ,Thompson BG,Muraszko KM,Garton HJ,Maher CO, Natural history and imaging prevalence of cavernous malformations in children and young adults. Journal of neurosurgery. Pediatrics. 2012 Feb [PubMed PMID: 22295927] |
[3] | Hall S,Smedley A,Sparrow O,Mathad N,Waters R,Chakraborty A,Tsitouras V, Natural History of Intracranial Arachnoid Cysts. World neurosurgery. 2019 Jun [PubMed PMID: 30898748] |
[4] | Reichert R,Campos LG,Vairo F,de Souza CF,Pérez JA,Duarte JÁ,Leiria FA,Anés M,Vedolin LM, Neuroimaging Findings in Patients with Mucopolysaccharidosis: What You Really Need to Know. Radiographics : a review publication of the Radiological Society of North America, Inc. 2016 Sep-Oct [PubMed PMID: 27618324] |
[5] | Koenig R,Bach A,Woelki U,Grzeschik KH,Fuchs S, Spectrum of the acrocallosal syndrome. American journal of medical genetics. 2002 Feb 15 [PubMed PMID: 11857542] |
[6] | Arnold PM,Teuber J, Marfan syndrome and symptomatic sacral cyst: report of two cases. The journal of spinal cord medicine. 2013 Sep [PubMed PMID: 23941798] |
[7] | Wang Y,Cui J,Qin X,Hong X, Familial intracranial arachnoid cysts with a missense mutation (c.2576C > T) in RERE: A case report. Medicine. 2018 Dec [PubMed PMID: 30558068] |
[8] | Zafeiriou DI,Batzios SP, Brain and spinal MR imaging findings in mucopolysaccharidoses: a review. AJNR. American journal of neuroradiology. 2013 Jan [PubMed PMID: 22790241] |
[9] | Yüksel D,Yilmaz D,Usak E,Senbil N,Gürer Y, Arachnoid cyst and costovertebral defects in Aicardi syndrome. Journal of paediatrics and child health. 2009 Jun [PubMed PMID: 22530764] |
[10] | Doherty D,Chudley AE,Coghlan G,Ishak GE,Innes AM,Lemire EG,Rogers RC,Mhanni AA,Phelps IG,Jones SJ,Zhan SH,Fejes AP,Shahin H,Kanaan M,Akay H,Tekin M,Triggs-Raine B,Zelinski T, GPSM2 mutations cause the brain malformations and hearing loss in Chudley-McCullough syndrome. American journal of human genetics. 2012 Jun 8 [PubMed PMID: 22578326] |
[11] | Balsubramaniam C,Laurent J,Rouah E,Armstrong D,Feldstein N,Schneider S,Cheek W, Congenital arachnoid cysts in children. Pediatric neuroscience. 1989 [PubMed PMID: 2488949] |
[12] | Rabiei K,Tisell M,Wikkelsø C,Johansson BR, Diverse arachnoid cyst morphology indicates different pathophysiological origins. Fluids and barriers of the CNS. 2014 Mar 3 [PubMed PMID: 24581284] |
[13] | de Longpre J, Large Arachnoid Cyst. The New England journal of medicine. 2017 Jun 8 [PubMed PMID: 28591531] |
[14] | Shin CJ,Rho M,Won YS,Kim SO, Rapid Visual Deterioration Caused by Posterior Fossa Arachnoid Cyst. Journal of Korean Neurosurgical Society. 2016 May [PubMed PMID: 27226868] |
[15] | Brewington D,Petrov D,Whitmore R,Liu G,Wolf R,Zager EL, De Novo Intraneural Arachnoid Cyst Presenting with Complete Third Nerve Palsy: Case Report and Literature Review. World neurosurgery. 2017 Feb [PubMed PMID: 27923759] |
[16] | Bigder MG,Helmi A,Kaufmann AM, Trigeminal neuropathy associated with an enlarging arachnoid cyst in Meckel's cave: case report, management strategy and review of the literature. Acta neurochirurgica. 2017 Dec [PubMed PMID: 28762108] |
[17] | Gönül E,Izci Y,Onguru O, Arachnoid cyst of the cerebellopontine angle associated with gliosis of the eighth cranial nerve. Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia. 2007 Jul [PubMed PMID: 17475499] |
[18] | Clifton W,Rahmathulla G,Tavanaiepour K,Alcindor D,Jakubek G,Tavanaiepour D, Surgically Treated de Novo Cervicomedullary Arachnoid Cyst in Symptomatic Adult Patient. World neurosurgery. 2018 Aug [PubMed PMID: 29777892] |
[19] | Hayden MG,Tornabene SV,Nguyen A,Thekdi A,Alksne JF, Cerebellopontine angle cyst compressing the vagus nerve: case report. Neurosurgery. 2007 Jun [PubMed PMID: 17538363] |
[20] | Olaya JE,Ghostine M,Rowe M,Zouros A, Endoscopic fenestration of a cerebellopontine angle arachnoid cyst resulting in complete recovery from sensorineural hearing loss and facial nerve palsy. Journal of neurosurgery. Pediatrics. 2011 Feb [PubMed PMID: 21284461] |
[21] | Mastronardi L,Taniguchi R,Caroli M,Crispo F,Ferrante L,Fukushima T, Cerebellopontine angle arachnoid cyst: a case of hemifacial spasm caused by an organic lesion other than neurovascular compression: case report. Neurosurgery. 2009 Dec [PubMed PMID: 19934941] |
[22] | Pagni CA,Canavero S,Vinci V, Left trochlear nerve palsy, unique symptom of an arachnoid cyst of the quadrigeminal plate. Case report. Acta neurochirurgica. 1990 [PubMed PMID: 2275426] |
[23] | Ishihara M,Nonaka M,Oshida N,Hamada Y,Nakajima S,Yamasaki M, "No-no" type bobble-head doll syndrome in an infant with an arachnoid cyst of the posterior fossa: a case report. Pediatric neurology. 2013 Dec [PubMed PMID: 24075844] |
[24] | Olvera-Castro JO,Morales-Briceño H,Sandoval-Bonilla B,Gallardo-Ceja D,Venegas-Cruz MA,Estrada-Estrada EM,Contreras-Mota M,Guinto-Balanzar G,Garcia-Lopez R, Bobble-head doll syndrome in an 80-year-old man, associated with a giant arachnoid cyst of the lamina quadrigemina, treated with endoscopic ventriculocystocisternotomy and cystoperitoneal shunt. Acta neurochirurgica. 2017 Aug [PubMed PMID: 28488069] |
[25] | Shettar M,Karkal R,Misra R,Kakunje A,Mohan Chandran VV,Mendonsa RD, Arachnoid Cyst Causing Depression and Neuropsychiatric Symptoms: a Case Report. East Asian archives of psychiatry : official journal of the Hong Kong College of Psychiatrists = Dong Ya jing shen ke xue zhi : Xianggang jing shen ke yi xue yuan qi kan. 2018 Jun [PubMed PMID: 29921743] |
[26] | García Romero JC,Ortega Martínez R,Zabalo San Juan G,de Frutos Marcos D,Zazpe Cenoz I, Subdural hygroma secondary to rupture of an intracranial arachnoid cyst: description of 2cases and review of the literature. Neurocirugia (Asturias, Spain). 2018 Sep - Oct [PubMed PMID: 29627291] |
[27] | Donaldson JW,Edwards-Brown M,Luerssen TG, Arachnoid cyst rupture with concurrent subdural hygroma. Pediatric neurosurgery. 2000 Mar [PubMed PMID: 10867560] |
[28] | Gelabert-González M,Fernández-Villa J,Cutrín-Prieto J,Garcìa Allut A,Martínez-Rumbo R, Arachnoid cyst rupture with subdural hygroma: report of three cases and literature review. Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery. 2002 Nov [PubMed PMID: 12420120] |
[29] | Kumar A,Sakia R,Singh K,Sharma V, Spinal arachnoid cyst. Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia. 2011 Sep [PubMed PMID: 21724400] |
[30] | Garg K,Borkar SA,Kale SS,Sharma BS, Spinal arachnoid cysts - our experience and review of literature. British journal of neurosurgery. 2017 Apr [PubMed PMID: 28287894] |
[31] | Wang X,Chen JX,You C,Jiang S, CT cisternography in intracranial symptomatic arachnoid cysts: classification and treatment. Journal of the neurological sciences. 2012 Jul 15 [PubMed PMID: 22520095] |
[32] | Algin O, Evaluation of the Communication Between Arachnoid Cysts and Neighboring Cerebrospinal Fluid Spaces by T2W 3D-SPACE With Variant Flip-Angle Technique at 3 T. Journal of computer assisted tomography. 2018 Sep/Oct [PubMed PMID: 29787500] |
[33] | Jafrani R,Raskin JS,Kaufman A,Lam S, Intracranial arachnoid cysts: Pediatric neurosurgery update. Surgical neurology international. 2019 [PubMed PMID: 30815323] |
[34] | Nakahashi M,Uei H,Tokuhashi Y, Recurrence of a symptomatic spinal intradural arachnoid cyst 29 years after fenestration. The Journal of international medical research. 2019 Sep [PubMed PMID: 31448656] |