Consolo D, Ouardirhi Y, Wessels C, Girard C
Département d'anesthésie-réanimation, CHU le Bocage, 2, boulevard Maréchal-de-Lattre-de-Tassigny, 21000 Dijon, France.
Ann Fr Anesth Reanim. 2005 Apr;24(4):428-31. doi: 10.1016/j.annfar.2005.01.012.
Acute hepatic porphyrias are genetic diseases, characterized by acute neurological symptoms, sometimes fatal, triggered by different factors, in particular by many anaesthetic drugs, and also by pregnancy. We report here the experience of three porphyric patients'deliveries, allowing us to consider a proposition of management in this context. After discussion between anaesthesiologist, obstetrician and porphyria specialist, two types of management of such patients can be foresee. Asymptomatic patients, or in long remission, can benefit from locoregional anesthesia techniques with bupivacaine for both labour analgesia and Caesarean section. Spinal anaesthesia is then the technique of choice, allowing using smaller quantity of local anaesthetic than epidural anaesthesia. For symptomatic patients, or in crisis, we have rather choose intravenous narcotics for labour analgesia, and general anaesthesia for Caesarean section. The hypnotic agent of choice for both induction and maintenance of such anaesthesia is then propofol.
急性肝卟啉病是遗传性疾病，其特征为急性神经症状，有时会致命，由不同因素引发，尤其是多种麻醉药物，还有妊娠。我们在此报告三例卟啉病患者分娩的经验，使我们能够在此背景下考虑一种管理建议。经过麻醉医生、产科医生和卟啉病专家的讨论，可以预见对此类患者有两种管理方式。无症状患者或处于长期缓解期的患者，可受益于使用布比卡因的局部区域麻醉技术用于分娩镇痛和剖宫产。此时脊髓麻醉是首选技术，与硬膜外麻醉相比可使用较少量的局部麻醉药。对于有症状患者或处于发作期的患者，我们更倾向于选择静脉麻醉药用于分娩镇痛，剖宫产则选择全身麻醉。此类麻醉诱导和维持的首选催眠药为丙泊酚。
