Cesarean section in a patient with severe preeclampsia and vertebral artery aneurysm, secondary to neurofibromatosis—A rare case

Abstract

Neurofibromatosis (NF1) is a relatively common multi-system genetic disorder. NF1 increases the risk of hypertension, and vasculopathy is a common finding. Extracranial vertebral artery aneurysm, though rare, can rupture and cause fatal hemorrhage. Pregnancy in NF1 is known to be associated with hypertension, preeclampsia, Hemolysis, elevated liver enzymes, low platelets (HELLP) syndrome. We report anesthesia management of a primigravida with 30 weeks of gestation with neurofibromatosis presenting with severe preeclampsia and acute onset vertebral artery aneurysm, who underwent urgent cesarean section. We opted for general anesthesia over regional due to the risk of aneurysm rupture and technical difficulty with vigilant hemodynamic control. Postoperative course was complicated with HELLP and sepsis but was successfully managed. Knowledge, careful preoperative evaluation, appropriate technique, and management of anesthesia, and close postoperative monitoring are of utmost importance.

Keywords

• neurofibromatosis 1 (nf1)
• severe pre-eclampsia
• vertebral artery aneurysm