The Diagnostic Burden of Spontaneous Intracranial Hypotension: Imaging Volume and Specialists Involvement Prior to Diagnosis.

Background and purpose: Spontaneous intracranial hypotension (SIH) is a disabling condition that is frequently underdiagnosed due to diagnostic challenges. Delays in diagnosis can be attributed to underrecognized MRI findings or lack of clinical suspicion, given the wide range of symptoms. This study aims to explore the diagnostic burden of SIH by examining the number of imaging exams and clinician visits prior to diagnosis.

Materials and methods: This retrospective single-institution study included 71 patients with spinal CSF leaks who had a confirmed diagnosis of CSF-venous fistula (CVF) on digital subtraction myelography (DSM). We reviewed each patient's clinical history, including the number of providers in different specialties and institutions, as well as the number and type of imaging studies performed prior to the positive DSM at our center. Brain MR images were scored using the Bern criteria, and clinical history along with imaging data were compared across Bern groups (low, intermediate, high probability).

Results: The mean patient age was 57.6 years with the majority being female (65%). The mean duration of symptoms was 3.8 years, and 14.1% of patients experienced symptoms for ≥10 years prior to the positive DSM. The mean number of specialists seen per patient was 2.6 (SD = 2.7; range, 0-13), and 28% of patients consulted 4 or more specialists prior to the positive DSM. On average, each patient underwent 6 imaging studies (SD: 3.8; range: 2-22) before the DSM, including 5.3 MRIs (SD: 3.7; range: 2-22), and 2.5 brain MRIs (SD: 2.1; range: 1-11). 40.8% of patients underwent ≥ 7 total imaging studies, and 53.5% had ≥ 3 Brain MRIs prior to diagnosis. Although no significant differences were found in the mean number of specialists or imaging exams between Bern score groups, a trend of increasing diagnostic burden was observed from the high to low Bern score groups.

Conclusions: Patients with spinal CSF leaks often undergo an extensive number of imaging and specialty consultations, contributing to delays in diagnosis and appropriate treatment. This study highlights the need to increase awareness among healthcare providers regarding the typical symptoms and imaging features of spinal CSF leaks.

Abbreviations: CVF= CSF-venous fistulas; CTM = CT myelography; DSM = digital subtraction myelography; OH = orthostatic headache; SIH = spontaneous intracranial hypotension.