Spinal dural arteriovenous fistula rupture after Rathke’s cleft cyst endoscopic resection: Case report and literature review

BACKGROUND: Spinal dural arteriovenous fistula (SDAVF) is the most frequent vascular malformation of the spine and accounts for approximately 70% of all vascular spinal malformations. In rare cases, SDAVF rupture and subsequent subarachnoid hemorrhage or intramedullary hematoma may occur. The aim of this article is to present a fatal case of SDAVF rupture after a Rathke’s cleft cyst (RCC) endoscopic resection. CASE DESCRIPTION: An 80-year-old female was referred to our hospital with a clinical presentation of bilateral reduction in visual acuity, bitemporal hemianopsia, and sellar magnetic resonance imaging (MRI) highly suggestive of RCC. After the first endonasal endoscopic surgery, the cyst was partially removed and vision improved. No signs of cerebrospinal fluid (CSF) leak were observed. After 1 year, the patient returned because of RCC recurrence and decreased visual acuity. In the second procedure, the lesion was totally resected and CSF leak was observed. A nasoseptal flap was rotated to cover the skull base defect. The patient developed subtle paraparesis followed by paraplegia on the 4(th) postoperative day. The dorsal spine MRI revealed a T3-T4 intramedullary hematoma. A dorsal laminectomy was performed and a SDAVF was observed. During microsurgery, at the right T3 nerve root level, an arteriovenous shunting point was identified, coagulated, and divided. The intramedullary hematoma was evacuated. The patient developed neurogenic and septic shock and died. CONCLUSION: Venous hypertension, venous wall fragility, and venous thrombosis seem to be the main factors involved in SDAVF rupture. In this particular case, reduction of the extravascular pressure and sudden variation in the pressure gradient caused by sustained CSF leak, also appeared to play an important role in SDAVF rupture. It may represent one more complication related to radical resection of RCC.