Cerebral Vasospasm Following Endoscopic Transsphenoidal Hypophysectomy in the Treatment of IGG-4 Related Lymphocytic Hypophysitis

Objectives: To describe the first case of cerebral vasospasm following transsphenoidal hypophysectomy (TSH) in the treatment of IgG4-related lymphocytic hypophysitis. Discuss clinical course and management options for cerebral vasospasm in this setting. Disseminate knowledge of this potential complication following endoscopic endonasal surgery. Discuss IgG4-related lymphocytic hypophysitis.

Design: Case Report.

Setting: Tertiary care hospital.

Participants: Single Case.

Main Outcome Measures: NIH stroke scale. Modified rankin score. Clinical course of cerebral vasospasm following TSH for IgG4-related lymphocytic hypophysitis.

Results: Endoscopic Endonasal TSH was performed with pathological diagnosis of IgG4-related lymphocytic hypophysitis. Intraoperative CSF leak was repaired with multi-layered closure using nasal septal flap. No arterial damage or significant bleeding occurred intraoperatively. The patient was discharged on postoperative day 4 but re-presented with altered mental status and slurred speech on postoperative day 9. Imaging revealed a small left basal ganglia infarct and catheter angiography demonstrated vasospasm involving the supraclinoidal carotid artery and it's major divisions. The patient was treated with volume expansion, calcium channel blockers, and multiple sessions of intra-arterial vasodilator infusions. Endovascular balloon angioplasty was considered but not performed due to small vessels caliber and recent adjacent surgery. Despite treatment she ultimately went on to develop global aphasia and right hemiplegia secondary to patchy dominant hemisphere infarction. Her course was further complicated by idiopathic thrombocytopenia purpura requiring plasmapheresis. At 8 month follow up she has residual hemiparesis and improving residual expressive aphasia. She can communicate fully with text messages. She is able to ambulate independently and continues to undergo speech therapy. Work up for systemic IgG4 - related disease was negative.

Conclusions: Cerebral vasospasm is a well-recognized cause of morbidity following aneurysmal subarachnoid hemorrhage but is rarely reported after tumor surgery. The clinical course of vasospasm in this setting parallels that of the post subarachnoid hemorrhage patient and typically peaks 5–10 days after the event. However, because this entity is rare in the setting of tumor resection, patients are typically not monitored for its occurrence nor treated prophylactically for its prevention. They often present to the hospital with a fulminant form that is difficult to treat. With the increased popularity and expanding indications for endoscopic endonasal surgery of the sella and parasellar regions, it is important to recognize this potential complication. IgG4-related lymphocytic hypophysitis is a rare but recently established subtype which may have systemic features involving the head and neck. This is the first description of vasospasm in this setting. Further investigation will be necessary to establish a possible correlation between the two entities.