Demir Ibrahim, Kılıç Ebru Tarıkçı, Akdemir Mehmet Salim
Department of Mardin Anesthesiology and Reanimation, Kiziltepe State Hospital, Kiziltepe, Turkey.
Department of Anesthesiology and Reanimation, Ümraniye Research and Health Sciences University, Istanbul, Turkey.
Anesth Essays Res. 2018 Jan-Mar;12(1):273-275. doi: 10.4103/aer.AER_222_17.
Neurofibromatosis type 1 (NF1) is an autosomal-dominant neuroectodermal hereditary disorder, in which spinal skeletal deformities are one of the manifestations. Pectus carinatum, temporomandibular joint dysfunction, and kyphoscoliosis can be seen with this genetic disorder which can lead to cardiorespiratory system problems and can cause difficulty in airway management. We present the anesthetic management of a NF1 patient with a sharp thoracolumbar dystrophic kyphoscoliosis who have been posted for emergency cesarean section as a result of fetal distress. The operation was successfully performed with subarachnoid block. This case highlights the anesthetic challenges and the significance of early planning anesthetic technique.
1型神经纤维瘤病（NF1）是一种常染色体显性神经外胚层遗传性疾病，脊柱骨骼畸形是其表现之一。鸡胸、颞下颌关节功能障碍和脊柱后凸侧弯可见于这种遗传性疾病，该疾病可导致心肺系统问题，并可引起气道管理困难。我们介绍了一名患有严重胸腰椎营养不良性脊柱后凸侧弯的NF1患者的麻醉管理情况，该患者因胎儿窘迫接受急诊剖宫产手术。手术在蛛网膜下腔阻滞下成功完成。该病例突出了麻醉挑战以及早期规划麻醉技术的重要性。
