Unique Gadolinium Enhancement Pattern in Spinal Dural Arteriovenous Fistulas

Importance: Spinal dural arteriovenous fistula (sDAVF) is often misdiagnosed as an inflammatory or a neoplastic myelopathy, often because of intraparenchymal gadolinium enhancement on magnetic resonance imaging (MRI); proper early diagnosis is important because deficits are reversible and a delay in treatment is associated with permanent morbidity. Tortuous flow voids on MRI are not universally present; thus, recognition of a unique gadolinium enhancement pattern may also aid in the early recognition and treatment of sDAVF. Objective: To describe a unique pattern of spinal cord gadolinium enhancement on MRI in sDAVF. Design, Setting, and Participants: This retrospective evaluation included pretreatment MRIs from 80 patients referred to the Mayo Clinic, Rochester, Minnesota, from January 1, 1997, through December 31, 2017, with a confirmed diagnosis of sDAVF and a control group of 144 patients with alternative confirmed myelopathy diagnoses. All participants underwent a neurologic evaluation at the Mayo Clinic. Main Outcomes and Measures: Evidence of at least 1 focal geographic nonenhancing area within a long segment of intense holocord gadolinium enhancement (termed the missing-piece sign) on MRI. Results: Of 51 patients with an sDAVF and a pretreatment MRI with gadolinium enhancement, 44 (86%) had intraparenchymal contrast enhancement, and 19 of these patients (43%) displayed the characteristic missing-piece sign. Of these 19 patients, symptom onset occurred at a median age of 67 years (range, 27-80 years); 15 patients were men. Progressive myelopathy features affecting the lower extremities occurred during a median of 33 months (range, 1-84 months). Eleven patients (58%) received an alternative diagnosis before confirmation of sDAVF. Tortuous flow voids were present on T2-weighted MRI in 13 of 19 patients. More than 1 digital subtraction angiogram was required for 5 patients to confirm the diagnosis. The missing-piece sign was not seen in any patients from the control group. Conclusions and Relevance: This unique gadolinium enhancement pattern in sDAVF was not found in a large control group of patients with other myelopathy. Identifying the missing-piece sign on MRI could potentially result in earlier time to angiography with improved outcomes for patients with an sDAVF..