A case of hydrocephalus confounded by suprasellar arachnoid cyst and concomitant reversible cerebral vasoconstriction syndrome

Abstract

Background: Obstructive hydrocephalus is a neurologic condition that has varied clinical and imaging presentations, as well as a multitude of congenital etiologies including aqueductal stenosis and less commonly arachnoid cysts. Aqueductal stenosis is a physical limitation to cerebrospinal fluid flow along the course of the aqueduct, which results in enlargement of the third and lateral ventricles. Arachnoid cysts are thin walled and fluid filled central nervous system lesions that can result in mass effect on adjacent structures. While arachnoid cysts are mostly asymptomatic, they may present with neurological symptoms that vary depending on the location of the lesion. Suprasellar cysts in particular may cause obstructive hydrocephalus as well as endocrine dysfunction. Reversible cerebral vasoconstriction syndrome (RCVS) is an unusual condition caused by cerebral arterial vasoconstriction that often presents initially with a thunderclap headache. Frequently, there is some environmental trigger associated with this condition. RCVS more commonly affects women and can induce stroke. Case Description: A 57-year-old female presented to the emergency department with progressive headache and visual changes. Initial workup suggested the patient’s symptoms where related to RCVS but subsequent surgical management of what was presumed to be long standing, compensated hydrocephalus resulted in resolution of the patient’s symptoms. Conclusion: We report, to the best of our knowledge, the first case of aquedutal stenosis and suprasellar arachnoid cyst with concomitant RCVS. The presence of multiple pathologies found on radiologic imaging illustrates the challenges presented by incidental findings and subsequent anchoring bias in medical diagnosis.