Spontaneous Cerebrospinal Fluid Leak Precipitating Catastrophic and Complicated Venous Sinus Thrombosis

Introduction: Spontaneous intracranial hypotension (SIH) is an uncommon headache etiology, typically attributable to an unprovoked cerebrospinal fluid (CSF) leak. Although frequently benign, serious complications have been documented, including cerebral venous thrombosis (CVT). We report a highly complicated case of CVT attributable to SIH, and review the literature on this rare and dangerous condition.

Methods: Case report and systematic review of the literature.

Results: A 43-year-old man presented with one week of non-postural headaches, dizziness, and nausea. While undergoing outpatient evaluation, he developed acute right-sided weakness, prompting presentation to our emergency department. Head CT identified CVT involving the superior sagittal sinus and adjacent cortical veins; IV heparin was initiated, and the patient was admitted to the NICU. Three hours later, he had a generalized tonic-clonic seizure, and repeat head CT demonstrated 3.1 × 3.8 × 3.1 cm left parietal intracerebral hemorrhage, prompting initiation of lacosamide and hyperosmolar therapy. The hemorrhage subsequently expanded, which was managed via cessation of anticoagulation and escalation of hyperosmolar therapy. Digital subtraction angiography demonstrated near-normal filling of the venous system, with a “spot sign” suggesting likely contrast extravasation. In spite of maximal medical therapy, the patient developed herniation syndrome, and was brought emergently to the OR for exploratory craniotomy, clot evacuation, and EVD placement. Upon elevating the bone flap, the dura was noted to be remarkably deflated despite clinical signs indicating mass effect and herniation, suggesting underlying CSF hypotension. Postoperatively, the patient was placed in 5° Trendelenburg with the EVD open at 15 mm Hg; positioning and the EVD were progressively weaned until he was stable for cervical MRI, which identified a large T2-hyperintense ventral epidural fluid collection. CT-guided myelogram confirmed high-flow CSF leak at T1 adjacent to a large osteophyte, and the leak was treated with two separate injections of autologous blood and synthetic fibrin glue. The patient discharged to inpatient rehabilitation with moderate right hemiplegia, hemisensory loss, and partial left VI palsy, after which he mounted an extraordinary neurologic recovery, returning for three-month follow-up with mild sensory symptoms only. Systematic review identified 29 publications describing 36 cases of CVT attributable to SIH. The majority of patients achieved a complete neurologic recovery (81%), and an underlying coagulopathy or hypercoagulability risk factors were identified in a significant minority (30%).

Conclusion: CVT is a rare and potentially lethal sequela of SIH, with an estimated prevalence of 2%. Awareness of the condition is poor, and diagnostic delays in identifying SIH may expose patients to highly morbid CVT complications. Evaluation and treatment should be directed toward identification of a spinal or skull base CSF leak, which most frequently occurs at the cervicothoracic junction, and is often well managed via epidural blood patch. Encouragingly, good neurologic outcomes can be achieved through vigilant critical care.

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