There is no consensus in the evaluation and management of pediatric cerebral arachnoid cysts. We reviewed the literature and suggest an algorithm based clinical approach.

Primary Intracranial arachnoid cysts (IACs) are often incidentally found in imaging studies, occurring in 2.6% of children and 1.4 % of adults. Only 6.8% of the children are symptomatic. Clinical features include focal neurological deficits, headaches and seizures from elevated intracranial pressure and local mass effect resulting in bony remodeling¹. About 50% of IACs are located in the middle cranial fossa. Most cases are of sporadic inheritance; some have a genetic predisposition and are associated with autosomal dominant polycystic kidney disease, Acrocallosal, Aicardi and Marfan’s syndromes and Neurofibromatosis Type 1².

12m old male presented with stereotypic, focal seizures, involving body and limb shudder, staring, lip smacking with perioral cyanosis. EEG showed seizures localized to left temporal lobe along with interictal focal slowing and epileptiform discharges. Patient had mild motor and significant social, language, and communication delays. Dilated fundus exam was without papilledema.  MRI revealed a large left temporal cyst (4.2x 5.3 x4.7cm) extending to the suprasellar cistern, encasement of internal carotid artery and elevation of anterior and middle cerebral arteries.  Patient underwent endoscopic fenestration of the cyst and was seizure free at 1 year follow up on monotherapy with improved developmental skills.

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Management of IACs depend on their location, radiological classification and clinical presentation.³ Large cysts in those under 4 years of age with symptoms and a type 3 Galassi radiological classification probably need surgical intervention.  There is no consensus on the procedure (cyst shunting vs cyst wall fenestration). Serial EEGs and additional genetic evaluation may add value.

For a systematic clinical approach to the neurological evaluation and management of IAC, we recommend an algorithm based on age, size, radiological classification and symptoms.