arachnoid cyst

arachnoid cyst

description

a benign developmental cyst within the pia-arachnoid layers, typically containing cerebrospinal fluid. arachnoid cysts represent 1 per cent of all intracranial masses, and may be congenital or acquired. congenital arachnoid cysts are believed to be formed by maldevelopment of the leptomeninges. they are located between the two layers of the arachnoid and communicate originally with the subarachnoid space, and consequently have the potential to enlarge. fluid accumulation is the result of a ball-valve mechanism. in addition, the presence of choroid plexus-like tissue within the cyst wall has been described by several authors. this ectopic tissue secretes cerebrospinal fluid and will also cause progressive distension. acquired arachnoid cysts are formed as a result of hemorrhage, trauma, or infection.

diagnosis

arachnoid cysts will present as anechoic cystic masses in virtually any intracranial site. there may be a mass effect present, depending on size and location, so that hydrocephalus is the primary presentation. the cyst, as a space occupying lesion, can obstruct the ventricular foramina, displace the aqueduct posteriorly, or block the basal cisterns. they usually cause symptoms during infancy, and the majority have been reported in patients under ten years of age. early diagnosis and treatment of these benign cysts will prevent irreversible brain damage. posterior fossa arachnoid cysts may be considered when the cisterna magna is enlarged, but are not common. in one series of twenty infratentorial arachnoid cysts collected over a forty year period, only five were discovered before adulthood. while less common than its supratentorial counterpart, the arachnoid cyst of the posterior fossa is more likely to become symptomatic and may produce obstructive hydrocephalus. arachnoid cysts have been reported in association with other central nervous system abnormalities such as agenesis of the corpus callosum, defective cerebellar lobulation, chiari type 1 malformations, and absence of the cavum septi pellucidi. they have also been noted in the presence of fallotís tetralogy and sacrococcygeal teratoma. 

differential diagnosis

supratentorial arachnoid cysts need to be distinguished from other cystic malformations in the same region: where aqueductal stenosis is present, the dilated third ventricle appears oval with tapered edges posteriorly, and there will be corresponding ventriculomegaly. in agenesis of the corpus callosum, the enlarged third ventricle is high in location at the level of the lateral ventricles, which themselves will display the characteristic features of that condition. porencephalic cysts are typically asymmetric, but do not exhibit mass effect. intracranial tumours are usually heterogenous in appearance. a cyst of the cavum septi pellucidi may be discerned by its characteristic location. semi lobar holoprosencephaly, in which a dorsal cyst is present, can be determined by examination of the thalami (fused) and cavum septi pellucidi (absent). a vein of galen aneurysm, superior and posterior to the thalami, will have turbulence and flow on using pulsed doppler. infratentorial arachnoid cysts must always be considered when there is enlargement of the cisterna magna (usually >10 mm in the trans-cerebellar plane). the differentiation from the dandy-walker syndrome or variant can be difficult. posterior fossa cysts are less common and tend to be asymmetric. a definite separation of the cyst and fourth ventricle by normal cerebellar vermis will indicate an arachnoid cyst, just as deficiency of the vermis or splaying of the cerebellar hemispheres will prompt the diagnosis of dandy-walker. there may also be other cns malformations seen in association with dandy-walker syndrome which will help in the diagnosis. in cerebellar hypoplasia, the diagnosis may be determined by comparing cerebellar dimensions with gestational age. where communicating hydrocephalus is present, there will typically be generalised subarachnoid accumulation of fluid in addition to ventriculomegaly. in megacisterna magna, no other abnormal findings will be evident.

sonographic features

asymmetric or midline thin walled intracranial cyst.

location and shape suggest diagnosis but it may not be possible to differentiate from other cystic lesions.

no associated anomalies but may cause hydrocephalus by compression.

cyst does not communicate with the lateral ventricles.

may show midline shift due to mass effect

associated syndromes

  • chromosomal
  • distichiasis lymphedema
  • majewski:short ribbed polydactyly
  • maternal diabetes mellitus
  • mohr (ofd ii)

references

  1. hogge wa, schnatterly p, ferguson je early prenatal diagnosis of an infratentorial arachnoid cyst: association with an unbalanced translocation prenat diagn 15: 186-188
  2. langer b, haddad j, favre r, frigue v, schlaeder g fetal arachnoid cyst: report of two cases ultrasound obstet gynecol 4: 68-72
  3. floris r, pastore fs, silvestrini m, vagnozzi r, guazzaroni m, giuffre r, simonetti g supracerebellar arachnoid cyst and reversible tonsillar herniation: magnetic resonance imaging and pathophysiological considerations neuroradiol 34: 404-406
  4. kwon t-h, jeanty p supratentorial arachnoid cyst the fetus 1: 7429.1
  5. raman s, rachagan sp, lim ct prenatal diagnosis of a posterior fossa cyst j clin ultrasound 19: 434-437
  6. diakoumakis ee, weinberg b, mollin j prenatal sonographic diagnosis of a suprasellar arachnoid cyst j ultrasound med 5: 529-530
  7. roach es, laster dw, sumner te, volberg fm posterior fossa arachnoid cyst demonstrated by ultrasound j clin ultrasound 10: 88-90