The Role of Spontaneous Venous Pulsations in the Diagnosis of Adult Chiari Malformation

Objective: To perform neuro-ophthalmologic evaluations in patients with symptomatic Adult Chiari Malformations (ACM).Methods: Observational case series of 56 patients with symptoms and neuro-imaging consistent with ACM. Comprehensive histories and complete neuro-ophthalmic examinations were performed. Findings: Sixteen men and forty women were evaluated. The mean age was 41 years old (range 8 - 73 years old). The most common visual complaint was transient visual obscurations lasting seconds (n= 34). Pain or fullness behind the eyes was present in 32 patients. Forty-eight patients had headaches. Other frequent symptoms included neck pain, dizziness, facial pain, and numbness. Fundus examination was remarkable for absent spontaneous venous pulsations in one or both eyes in 28 patients. Interpretation: Adult Chiari Malformation (ACM) presents with characteristic symptoms that may be overlooked or misdiagnosed. Neuro-ophthalmic evaluation is helpful in ruling out other entities and identifying characteristic absent venous pulsations.