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
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
- hogge wa, schnatterly p, ferguson je early prenatal diagnosis of an infratentorial arachnoid cyst: association with an unbalanced translocation prenat diagn 15: 186-188
- langer b, haddad j, favre r, frigue v, schlaeder g fetal arachnoid cyst: report of two cases ultrasound obstet gynecol 4: 68-72
- 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
- kwon t-h, jeanty p supratentorial arachnoid cyst the fetus 1: 7429.1
- raman s, rachagan sp, lim ct prenatal diagnosis of a posterior fossa cyst j clin ultrasound 19: 434-437
- diakoumakis ee, weinberg b, mollin j prenatal sonographic diagnosis of a suprasellar arachnoid cyst j ultrasound med 5: 529-530
- roach es, laster dw, sumner te, volberg fm posterior fossa arachnoid cyst demonstrated by ultrasound j clin ultrasound 10: 88-90