RESUMEN
BACKGROUND: Craniocervical junction arteriovenous fistulas (CCJAVFs) are known to be rare, but variations and clinical behaviors remain controversial. METHODS: A total of 11 CCJAVF patients (M: F=9:2, age 54-77 years) were investigated. Based on the radiological and intraoperative findings, they were categorized into three types: dural AVF (DAVF), radicular AVF (RAVF), and epidural AVF (EDAVF). RESULTS: There were four symptomatic patients (subarachnoid hemorrhage in two, myelopathy in one, and tinnitus in one) and seven asymptomatic patients in whom coincidental CCJAVFs were discovered on imaging studies for other vascular diseases (arteriovenous malformation in one, intracranial DAVF in two, ruptured cerebral aneurysm in two, and carotid artery stenosis in two). Of these 11 patients, 2 (18.2%) had multiple CCJAVFs. Of 14 lesions, the diagnoses were DAVF in 5, RAVF in 3, and EDAVF in 6 (C1-C2 level ratio =5:0, 2:1, 3:3). Patients with DAVF/RAVF in four lesions with intradural venous reflux underwent surgery, although an RAVF remained in one lesion after embolization/radiation. Since all six EDAVFs, two DAVFs, and one RAVF had neither feeder aneurysms nor significant symptoms, no treatment was provided; of these nine lesions, one DAVF and one RAVF remained unchanged, whereas six EDAVFs showed spontaneous obliteration within a year. Unfortunately, however, one DAVF bled before elective surgery. CONCLUSION: CCJAVFs have many variations of shunting site, angioarchitecture, and multiplicity, and they were frequently associated with coincidental vascular lesions. For symptomatic DAVF/RAVF lesions with intradural drainage, surgery is preferred, whereas asymptomatic EDAVFs without dangerous drainage may obliterate during their natural course.
RESUMEN
BACKGROUND: In patients with secondary empty sella syndrome (ESS), optic nerve herniation into the sella turcica is caused by shrinkage of the mass lesion at the sella turcica, resulting in visual disturbance. ESS is often surgically treated using chiasmapexy. Here, we report the first case of spontaneous improvement in a patient with ESS. CASE DESCRIPTION: A 69-year-old woman presented with a month-long history of visual disturbance in the right eye, poor visual acuity, and quadrantanopia in her upper temporal visual field. Magnetic resonance (MR) imaging showed herniation of her right optic nerve and gyrus rectus into the sella turcica. The visual disturbance gradually improved, and the patient's vision became almost normal after a month without any treatment. On repeated MR imaging, it was observed that the herniation of the right optic nerve and gyrus rectus disappeared due to an intrasellar cyst re-expansion. The secondary ESS caused by the shrinkage of the intrasellar cyst resulted in the visual disturbance and re-expansion of the cyst resulted in spontaneous improvement of symptoms. The visual disturbance did not recur for a year. CONCLUSION: Patients with secondary ESS without severe symptoms may be followed up conservatively. However, surgical treatment should be applied if symptoms deteriorate or do not improve.
