Dogan Zafer, Yildiz Huseyin, Coskuner Ismail, Uzel Murat, Garipardic Mesut
Department of Anesthesiology and Intensive Care, Bezmialem Vakif University Medical School, Istanbul, Turkey.
Department of Anesthesiology and Intensive Care, Kahramanmaras Sutcu Imam University, Medical School, Kahramanmaras, Turkey.
Braz J Anesthesiol. 2014 May-Jun;64(3):201-4. doi: 10.1016/j.bjane.2012.12.006. Epub 2013 Oct 23.
Fanconi anemia is a rare autosomal recessive inherited bone marrow failure syndrome with congenital and hematological abnormalities. Literature regarding the anesthetic management in these patients is limited. A management of a developmental dislocation of the hip was described in a patient with fanconi anemia. Because of the heterogeneous nature, a patient with fanconi anemia should be established thorough preoperative evaluation in order to diagnose on clinical features. In conclusion, we preferred caudal anesthesia in this patient with fanconi anemia without thrombocytopenia, because of avoiding from N2O, reducing amount of anesthetic, existing microcephaly, hypothyroidism and elevated liver enzymes, providing postoperative analgesia, and reducing amount of analgesic used postoperatively.
范可尼贫血是一种罕见的常染色体隐性遗传性骨髓衰竭综合征，伴有先天性和血液学异常。关于这些患者麻醉管理的文献有限。本文描述了一名范可尼贫血患者髋关节发育性脱位的处理。由于其异质性，对于范可尼贫血患者应进行全面的术前评估，以便根据临床特征进行诊断。总之，对于这名无血小板减少的范可尼贫血患者，我们选择骶管麻醉，原因是避免使用氧化亚氮、减少麻醉药物用量、患者存在小头畸形、甲状腺功能减退和肝酶升高、提供术后镇痛以及减少术后镇痛药物用量。
