The management of Cesarean delivery in a parturient with paroxysmal nocturnal hemoglobinuria complicated by severe preeclampsia

Abstract

Paroxysmal nocturnal hemoglobinuria is associated with an increased risk of venous thromboembolism, and so anticoagulation therapy assumes primary importance. The use of LMWH for prophylaxis in combination with thrombocytopenia may contraindicate neuraxial anesthesia. General anesthesia should be aimed at preventing or exacerbating complement mediated intravascular hemolysis.

Anaesthesia and orphan disease

“…11 For maintenance of anaesthesia, we chose sevoflurane because it is associated with a 70% reduction in C 3 and C 4 levels and significantly reduces activation of the complement pathway. 12 Analgesia was achieved with remifentanil TCI, as compared with intermittent fentanyl, as there is an association between the reduction in stress response and complement factors. 13 Altogether, this kept BIS monitoring values between 30 and 50.…”

Anaesthesia and orphan disease

“…We used propofol in lipid emulsion at the induction of anaesthesia because it has no association with complement activation. 12 Analgesia was achieved with remifentanil TCI, as compared with intermittent fentanyl, as there is an association between the reduction in stress response and complement factors. 12 Analgesia was achieved with remifentanil TCI, as compared with intermittent fentanyl, as there is an association between the reduction in stress response and complement factors.…”