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Arachnoid Cysts

Important
It is possible that the main title of the report Arachnoid Cysts is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.
Synonyms

* Cysts, Arachnoid

Disorder Subdivisions

* Intracranial Arachnoid Cysts
* Spinal Arachnoid Cysts

Related Disorders List

Information on the following diseases can be found in the Related Disorders section of this report:

* Acoustic Neuroma
* Arachnoiditis
* Brain Tumors, General
* Dandy-Walker Syndrome
* Empty Sella Syndrome
* Epidermoids
* Midline Caves of the Brain
* Porencephaly
* Syringomyelia
* Bobble-Head Doll Syndrome
* Hyperprolactinemia
* Panhypopituitarism
* Arnold-Chiari Malformaion

General Discussion

Arachnoid cysts are fluid-filled sacs that occur on the arachnoid membrane that covers the brain (intracranial) and the spinal cord (spinal). There are three membranes covering these components of the central nervous system: dura mater, arachnoid, and pia mater. Arachnoid cysts appear on the arachnoid membrane, and they may also expand into the space between the pia mater and arachnoid membranes (subarachnoid space). The most common locations for intracranial arachnoid cysts are the middle fossa (near the temporal lobe), the suprasellar region (near the third ventricle) and the posterior fossa, which contains the cerebellum, pons, and medulla oblongata.

In many cases, arachnoid cysts do not cause symptoms (asymptomatic). In cases in which symptoms occur, headaches, seizures and abnormal accumulation of excessive cerebrospinal fluid in the brain (hydrocephalus) are common. The exact cause of arachnoid cysts is unknown.

Arachnoid cysts are classified according to location.
Symptoms

Arachnoid cysts are present at birth (congenital), but, in most cases, may not cause any symptoms (asymptomatic) throughout an individual's life. Whether symptoms occur depends upon the size and location of the cyst. Small cysts usually do not cause symptoms. However, cysts can increase in size causing symptoms to appear, especially if they press against a cranial nerve, the brain, or the spinal cord.

Most cases of arachnoid cysts that are associated with symptoms occur in childhood. The specific symptoms present vary from case to case. It is important to note that affected individuals will not have all of the symptoms listed below.

Suprasellar cysts usually present with headaches, vomiting, and hydrocephalus. Hydrocephalus in children presents with enlarged head due to the pressure of the fluids that fill the empty spaces in the head. In adults, hydrocephalus is noticeable, in extreme cases, in the form of incontinence, imbalance and dementia. Rarely, suprasellar cysts will be accompanied by endocrinological disorders as well.

Temporal fossa cysts frequently go unnoticed (asymptomatic) except for headaches. Since the walls of this form of cyst contain blood vessels, even minor injury may lead to bleeding in the membrane and/or the cyst.

Convexity cysts are usually accompanied by seizures.

Posterior fossa cysts are accompanied by headaches, vomiting, and disorders of the cerebellum (in the lower back of the brain). Hydro-cephalus, due to obstruction of the flow or drainage of the cerebral spinal fluid, is frequently found in these patients.

Pineal arachnoid cysts usually present with obstructive hydrocephalus and "failure of upward gaze."

Headaches, seizures, and the accumulation of excessive cerebrospinal fluid in the brain (hydrocephalus), resulting in increased intracranial pressure, are the most common findings. In children, malformation of certain cranial bones and an abnormally enlarged head (macrocephaly) may also occur.

A variety of additional symptoms occur in some individuals with arachnoid cysts depending upon the size and location of the cyst. Most cysts occur near the middle fossa region of the brain. Such symptoms include lethargy, nausea, vomiting, and hearing abnormalities. Neurological signs may be present because arachnoid cysts may cause increased pressure on structures of the brain. Such neurological findings may include developmental delays, behavioral changes, an inability to control voluntary movements (ataxia) and mental retardation.

In addition to hydrocephalus, cysts located in the suprasellar region may be associated with vision disturbances, continuous bobbing of the head, and abnormalities affecting certain hormone-producing glands that help to regulate the rate of growth, sexual development, and certain metabolic functions (endocrine system). Endocrine system abnormalities may result in a variety of symptoms such as premature sexual development (precocious puberty).

Spinal arachnoid cysts may be associated with progressive weakness of the legs, abnormal side-to-side curvature of the spine (scoliosis), back pain, and involuntary muscle spasms (spasticity) that result in slow, stiff movements of the legs. In rare cases, these cysts may cause paralysis of the legs (paraplegia). Urinary tract infections may also occur in individuals with spinal arachnoid cysts.
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Causes

The exact cause of arachnoid cysts is not known. Researchers believe that most cases of arachnoid cysts are developmental malformations that arise from the unexplained splitting or tearing of the arachnoid membrane. According to the medical literature, cases of arachnoid cysts have run in families (familial cases) suggesting that a genetic predisposition may play a role in the development of arachnoid cysts in some individuals.

In some cases, arachnoid cysts occurring in the middle fossa are accompanied by underdevelopment (hypoplasia) or compression of the temporal lobe. The exact role that temporal lobe abnormalities play in the development of middle fossa arachnoid cysts is unknown.

In a few rare cases, intracranial arachnoid cysts may be inherited as an autosomal recessive trait. In recessive disorders, the condition does not appear unless a person inherits the same defective gene for the same trait from each parent. If an individual receives one normal gene and one gene for the disease, the person will be a carrier for the disease, but usually will not show symptoms. The risk of transmitting the disease to the children of a couple, both of whom are carriers for a recessive disorder, is 25 percent. Fifty percent of their children risk being carriers of the disease, but generally will not show symptoms of the disorder. Twenty-five percent of their children may receive both normal genes, one from each parent, and will be genetically normal (for that particular trait). The risk is the same for each pregnancy.

In a few rare cases, spinal intradural arachnoid cysts may be inherited as an autosomal dominant trait.

Some complications of arachnoid cysts can occur when a cyst is damaged because of minor head trauma. Trauma can cause the fluid within a cyst to leak into other areas (e.g., subarachnoid space). Blood vessels on the surface of a cyst may tear and bleed into the cyst (intracystic hemorrhage), increasing its size. If a blood vessel bleeds on the outside of a cyst, a collection of blood (hematoma) may result. In the cases of intracystic hemorrhage and hematoma, the individual may have symptoms of increased pressure within the cranium and signs of compression of nearby nerve (neural) tissue.

Arachnoid cysts can also occur secondary to other disorders such as Marfan’s syndrome, arachnoiditis, or agenesis of the corpus callosum. (For more information on these disorders, choose the specific disorder name as your search term in the Rare Disease Database.)
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Affected Populations

These cysts may occur at any age and have been found in all races and geographic locations. Because many cases of arachnoid cysts have no symptoms, it is difficult to determine the true frequency of this disorder in the general population.
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Related Disorders

Symptoms of the following disorders can be similar to those of arachnoid cysts. Comparisons may be useful for a differential diagnosis:

Acoustic neuroma (cerebellopontine angle syndrome) is a benign (noncancerous) tumor of the 8th cranial nerve. The early symptoms of an acoustic neuroma include a ringing sound in the ear (tinnitus) and/or a hearing loss. These symptoms occur due to pressure from the tumor on the 8th cranial nerve. Some cases of arachnoid cysts may initially present with tinnitus and hearing loss and be confused with an acoustic neuroma. The exact cause of acoustic neuromas is unknown. (For more information on this disorder, choose "Acoustic Neuroma" as your search term in the Rare Disease Database.)

Brain tumors are growths in the brain that can be either cancerous (malignant) or noncancerous (benign). The symptoms of malignant and benign brain tumors are similar, and depend on the type of tumor and its location. Symptoms may include recurrent headache, seizures, emotional instability, nausea, and/or facial pain or numbness. There are many different types of brain tumors. (For more information on these disorders, choose "Brain Tumor" as your search term in the Rare Disease Database.)

Dandy-Walker malformation is a rare malformation of the brain that is present at birth (congenital). It is characterized by an abnormally enlarged space at the back of the brain (cystic 4th ventricle) that interferes with the normal flow of cerebrospinal fluid through the openings between the ventricle and other parts of the brain (foramina of Magendia and Luschka). Excessive amounts of fluid accumulate around the brain and cause abnormally high pressure within the skull, swelling of the head (congenital hydrocephalus), and neurological impairment. Motor delays and learning problems may also occur. Dandy-Walker malformation is a form of "obstructive" or "internal noncommunicating hydrocephalus," meaning that the normal flow of cerebrospinal fluid is blocked resulting in the widening of the ventricles. (For more information on this disorder, choose "Dandy-Walker" as your search term in the Rare Disease Database.)

Empty Sella syndrome is a rare brain disorder in which the depression across the bone that contains the pituitary gland (sella turcica) appears as an extension of the subarachnoid space and is filled with cerebrospinal fluid. The syndrome may be primary or may be secondary to a pituitary tumor, irradiation, or surgery on the pituitary gland. Symptoms may include headaches, impaired vision, and obesity. Sex-specific differences may be seen, such as excessive hair growth (hirsutism) in women and reduced sex drive (libido) in men. The primary form of Empty Sella syndrome is inherited as an autosomal dominant trait. (For more information on this disorder, choose "Empty Sella" as your search term in the Rare Disease Database.)

Porencephaly is a major congenital malformation of the brain that is characterized by cysts or the formation of cavities (cavitations) within the brain. The symptoms of porencephaly usually include mental retardation, spastic paralysis, mild paralysis of cranial nerves (ophthalmoplegia), and/or epilepsy. Hydrocephalus, low muscle tone (hypotonia), and macrocephaly may also be present in some cases.

The following disorders may be associated with arachnoid cysts as secondary characteristics. They are not necessary for a differential diagnosis:

Hyperprolactinemia occurs when there is an abnormally high amount of prolactin in the blood. Hyperprolactinemia has been associated with the extension of arachnoid cysts into the suprasellar cistern. Symptoms in women may include absence or abnormal cessation of menstruation (amenorrhea) and/or spontaneous milk flow from the breasts (galactorrhea). Symptoms in men have been reported to include impotence and/or abnormally decreased function of the testes.

Panhypopituitarism is a rare disorder characterized by a generalized reduction or cessation of pituitary gland function. This can result when an expanding intrasellar arachnoid cyst causes the pituitary gland to become flattened. Such a cyst can be mistaken for a pituitary tumor.
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Standard Therapies

Diagnosis
A diagnosis of arachnoid cysts is often made incidentally, often during examination of an individual with seizures. A diagnosis may be suspected based upon a detailed patient history, a thorough clinical examination, and a variety of specialized tests, especially advanced imaging studies such as computed tomography (CT scan) and magnetic resonance imaging (MRI). CT scans and MRIs can reveal or confirm the presence of arachnoid cysts. During CT scanning, a computer and x-rays are used to create a film showing cross-sectional images of the brain's tissue structure. During MRI, a magnetic field and radio waves are used to create cross-sectional images of the brain.

DO NOT CONSENT TO INJECTING CAUSTIC DYE INTO YOUR SPINE BY DOING A TRADITIONAL MYLOGRAM. You can have a MRI mylogram done with a machine with a tesla rating over 1.5. Please make sure that your machine is accurate.

Treatment
Treatment is warrented depending on location and if they are symptomatic arachnoid cysts. In cases where treatment is necessary, therapy usually consists of #1 of laser done at Cedars or scapel removal done by Dr. Caton in Pasadena (this is the preferred method as Arachnoid Cysts are known for their complicated natures and how they often "come back" You do not want to put your child through countless surgeries so please educate yourself on your surgeons and the kind of surgeries available) #2 Fenestrations are temporary surgeries that often require doctors to go back in and remove a portion of the cell walls from the cyst several times. It is popular to use metal to hold these fenestrations open however not very popular among patients as it causes pain. #3 Aspirations is a simple draining of the cyst which will do nothing but take pressure off for a short amont of time. #4 The last surgery tried should be drainage of the cyst by surgically inserting a device (shunt) to provide a passageway for fluid from the cyst to one of the reservoirs for bodily fluids. This reduces pressure on the cysts and may prevent a recurrence of fluid build-up. In some cases, a more invasive surgical procedure may be necessary to remove an arachnoid cyst.

If the subarachnoid space has become blocked by an arachnoid cyst, it may also be necessary to place a shunt between a ventricle and the space between the layers of the membrane that lines the cavities of the body (the peritoneal cavity). This will bypass the blockage and provide an adequate passageway for cerebrospinal fluid to circulate.

Other treatment is symptomatic and supportive.
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Investigational Therapies

In one study, three individuals who had bilateral hearing loss as a result of arachnoid cysts were treated with diuretics. This therapy resulted in improvement in symptoms, with no evidence of enlargement of the cysts during several years of follow-up. More research is needed to determine whether diuretic drugs may help other people with this type of arachnoid cyst.

Most real people on arachnoidcyst.net have not found assistance using diuretics so we the peole have found this study to be bunk.
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References

McKusick VA., ed. Online Mendelian Inheritance in Man (OMIM). Baltimore. MD: The Johns Hopkins University; Entry No:207790; Last Update:12/10/98. Entry No:182990; Last Update:3/25/95.

TEXTBOOKS
Bennett JC, Plum F., eds. Cecil Textbook of Medicine. 20th ed. Philadelphia, PA: W.B. Saunders Co; 1996:2131.

Menkes JH., au., Pine JW, et al., eds. Textbook of Child Neurology, 5th ed. Baltimore, MD: Williams & Wilkins; 1995:308.

Wilson JD, et al. Textbook of Endocrinology. 8th ed. Philadelphia, PA: W. B. Saunders Co; 1992: 533, 539, 542, 545.

Buyce ML., ed. Birth Defects Encyclopedia. Dover, MA: Blackwell Scientific Publications; For: The Center for Birth Defects Information Services Inc; 1990:236-9.

REVIEW ARTICLES
Huang PP. Roller coaster headaches revisited. Surg Neurol. 2003;60:398-401.

Bhattacharyya KB, Senapati A, Basu S, et al. Bobble-head doll syndrome: some atypical features with a new lesion and review of the literature. Acta Neurol Scand. 2003;108:216-20.

Starzyk J, Kwiatkowski S, Urbanowicz W, et al. Suprasellar arachnoid cyst as a cause of precocious puberty:report of three patients and literature overview. J Pediatr Endocrinol Metab. 2003;16:447-55.

JOURNAL ARTICLES
Weber F, Knopf H. Cranial MRI as a screening tool: findings in 1,772 military pilot applicants. Aviat Space Environ Med. 2004;75:158-61.

Caroli E, Rocchi G, Salvati M, et al. Duraplasty: our current experience. Surg Neurol. 2004;61:55-59.

Levy ML, Wang M, Aryan HE, et al. Microsurgical keyhole approach for middle fossa arachnoid cyst fenestration. Neurosurgery. 2003;53:1138-44; discussion 1144-45.

Boltshauser E, et al. Outcome in children with space-occupying posterior fossa arachnoid cyst. Neuropediatrics. 2002;33:118-21.

Dodd RL, et al. Spontaneous resolution of prepontine arachnoid cyst. Case report and review of the literature. Pediatr Neurosurg. 2002;37:152-7.

Ottaviani F, et al. Arachnoid cyst of the cranial posterior fossa causing sensorineural hearing loss and tinnitus: a case report. Eur Arch Otorhinolaryngol. 2002;259:306-8.

De K, et al. Haemorrhage into an arachnoid cyst: a serous complication of minor head trauma. Emerg Med J. 2002;19:365-6.

Mazurkiewicz-Beldzinska M, Dilling-Ostrowska E. Presentation of intracranial arachnoid cysts in children: correlation between localization and clinical symptoms. Med Sci Monit. 2002;8:462-5.

Abou-Fakhr FS, et al. Thoracic spinal intradural arachnoid cyst: report of two cases and review of literature. Eur Radiol. 2002;12:877-82.

Muthukumar N. Sacral extradural arachnoid cyst: a rare cause of low back pain and perineal pain. Eur Spine J. 2002;11:162-6.

Zaatreh MM, et al. Morphometric and neuropsychologic studies in children with arachnoid cysts. Pediatr Neurol. 2002;26:134-8.

Gosalakkal JA. Intracranial arachnoid cysts in children: a review of pathogenesis, clinical features, and management. Pediatr Neurol. 2002;26:93-8.

Lee HJ, Cho DY. Symptomatic spinal intradural arachnoid cysts in the pediatric age group: description of three new cases and review of the literature. Pediatr Neurosurg. 2001;35:181-87.

Gomez Escalonilla CI, et al. Intracranial arachnoid cysts. A study of a series of 35 cases. Rev Neurol. 2001;33:305-11.

Motoyama Y, et al. Surgical treatment for symptomatic arachnoid cysts. No Shinkei Geka. 2001;29:217-26.
Resources

NIH/NINDS Brain Resources and Information Network
PO Box 5801
Bethesda, MD 20824
Tel: (301)496-5751
Fax: (301)402-2186
Tel: (800)352-9424
Internet: http://www.ninds.nih.gov/

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report

This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

Last Updated: 7/16/2004
Copyright 1994, 2002, 2004 National Organization for Rare Disorders, Inc.

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