Cardiopulmonary complication as a pitfall of the perioperative management of moyamoya syndrome with atherosclerosis: Conflict to counteract with cerebral hyperperfusion

A 42-year-old man with a history of hypertension and obesity presented with transient dysesthesia in his left upper and lower extremities and was found to have moyamoya syndrome associated with atherosclerosis. He underwent superficial temporal artery-middle cerebral artery anastomosis with pial synangiosis in the right hemisphere 1 month after the onset of symptoms. Prophylactic blood pressure lowering (< 130 mmHg) as well as minocycline administration was introduced immediately after surgery to prevent symptomatic cerebral hyperperfusion, but he developed pulmonary edema due to congestive heart failure several hours after surgery. We subsequently allowed his systolic blood pressure to be under 140 mmHg, which dramatically improved his cardiopulmonary condition. The neurologic status of the patient was unremarkable, but ¹²³I-IMP-SPECT the day after surgery demonstrated an intense increase in the cerebral blood flow at the site of the anastomosis. Moreover, postoperative magnetic resonance angiography demonstrated the bypass as thick, high signal. Together, these results led us to the diagnosis of cerebral hyperperfusion. The patient did not demonstrate any neurological sign during the entire perioperative period, but CT scan performed 7 days after surgery revealed a delayed intra-cerebral hemorrhage in the right temporal lobe due to the cerebral hyperperfusion. We continued to mildly lower his blood pressure, and neither ischemic nor hemorrhagic events were subsequently observed ; he was discharged without neurological deficit 2 weeks after surgery. In conclusion, congestive heart failure and pulmonary edema are potential complications of the perioperative management of moyamoya syndrome with atherosclerotic background. Moreover, cardiopulmonary complications should be mentioned as a potential pitfall of the intensive perioperative management of moyamoya disease to counteract with cerebral hyperperfusion.