Brain Imaging in Pediatric Pseudotumor Cerebri Syndrome

Abstract

Pseudotumor cerebri syndrome (PTCS) is a clinical entity of unclear etiology characterized by raised intracranial pressure but normal cerebrospinal fluid contents and no associated anomalies evident in the brain parenchyma. Idiopathic intracranial hypertension, which is the primary (idiopathic) form of PTCS, generally affects adult obese females of childbearing age. Pediatric PTCS has been reported in association with various potential comorbidities, including chronic medical illness, infections, endocrine disorders, and ingestion and/or withdrawal of some drugs. Clinical signs and symptoms of PTCS in children are heterogeneous, but they classically include headache, papilledema, and visual disturbances (e.g., diplopia and/or vision loss). An important criterion for diagnosing PTCS involves imaging. The role of computed tomography is useful to rule out some secondary causes of elevated increased intracranial pressure (e.g., hydrocephalus, hemorrhage, mass effect); magnetic resonance imaging and magnetic resonance venography are helpful in many diagnostic challenges and in the detection of subtle intracranial anomalies (e.g., isodense tumors, meningeal infiltrations, pathologies of posterior fossa, sinus venous thrombosis). In addition, magnetic resonance imaging is able to identify some secondary signs supportive of a diagnosis of PTCS, such as flattened posterior globe/sclera, empty sella, partially empty sella/decreased pituitary height, patulous optic nerve sheath, intraocular protrusion of optic nerve head, and enhanced and increased tortuosity of the optic nerve, though these are not specific. We briefly discuss the application of imaging in the assessment of pediatric PTCS patients.