Berl M, Dubois L, Belkacem H, Dailland P, Carli P
Département d'anesthésie et de réanimation chirurgicale, unité de la maternité, groupe hospitalier Necker-Enfants-Malades, 149, rue de Sèvres, 75743 cedex 15, Paris, France.
Ann Fr Anesth Reanim. 2003 Apr;22(4):359-62. doi: 10.1016/s0750-7658(03)00060-1.
Von Hippel-Lindau (VHL) disease is a rare autosomal dominant genetic disorder with retinal and nervous system haemangioblastomas, phaeochromocytomia, kidney, pancreas and endolymphatic tumors. The management of childbirth, including epidural anaesthesia is controversial. The possible presence of vascular malformations (retinal and central nervous system and spinal haemangioblastomas) increases the risk of disrupting central nervous system haemangioblastoma during delivery and when epidural anaesthesia is initiated. We report 3 cases of patient with VHL disease with successful epidural anaesthesia 2 for delivery and 1 for Caesarean section.
冯·希佩尔-林道（VHL）病是一种罕见的常染色体显性遗传病，可出现视网膜和神经系统血管母细胞瘤、嗜铬细胞瘤、肾、胰腺及内淋巴囊肿瘤。分娩管理，包括硬膜外麻醉，存在争议。血管畸形（视网膜、中枢神经系统和脊髓血管母细胞瘤）的可能存在增加了分娩期间及开始硬膜外麻醉时破坏中枢神经系统血管母细胞瘤的风险。我们报告3例VHL病患者，其中2例成功进行硬膜外麻醉分娩，1例成功进行硬膜外麻醉剖宫产。
