A case of dural arteriovenous fistula at the craniocervical junction presenting with hiccups and Horner's syndrome due to medullary edema

<p>  Craniocervical junction dural arteriovenous fistula (CCJ‒dAVF) is a rare disease. We report the case of a patient with CCJ‒dAVF who presented with hiccups, single leg paralysis, and Horner’s syndrome due to medullary edema. A 72‒year‒old Japanese male was admitted to our hospital with nausea, hiccups, and weakness of the left lower extremity. An MRI FLAIR image showed edema on the dorsal side of the right medulla oblongata, and a T2‒weighted image showed many flow voids on the anterior surface of the brainstem between the vertebral arteries (VAs). Cerebral digital subtraction angiography (DSA) showed an arteriovenous (AV) shunt near the transdural junction of the right VA and a dilated drainer vein ascending to the anterior surface of the brainstem. A craniotomy for shunt point obliteration was performed on the 13th day of hospitalization. After surgery, the patient’s preoperative symptoms improved, and postoperative DSA showed disappearance of the AV shunt. The patient was transferred on postoperative day 30. In cases with myelopathy/brain stem disorder due to venous congestion of the spinal cord/brain stem, the disease often progresses. Early intervention is thus necessary, and direct surgery can be considered as the first choice. To the best of our knowledge, the present report is the first published description of a patient with CCJ‒dAVF, hiccups, and Horner’s syndrome, and this combination is extremely rare.</p>