Naik B Naveen, Sujith John, Kajal Kamal
Department of Anaesthesia and Critical Care, JIPMER, Puducherry, India.
Department of Anaesthesia and Intensive Care, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Indian J Anaesth. 2019 May;63(5):400-402. doi: 10.4103/ija.IJA_779_18.
Maffucci syndrome is an extremely rare, sporadic, and nonhereditary disease characterized by enchondromatosis, bony abnormalities, and vascular malformations. We report a successful anaesthetic management of an adult patient with Maffucci syndrome scheduled for surgical excision of parathyroid adenoma, who had numerous enchondromas, haemangiomas, and skeletal deformities involving different parts of the body and posing significant challenge during positioning, securing intravenous access, and insertion of endotracheal tube. Awareness about this syndrome is of paramount importance because similar patients may be encountered with increasing frequency for incidental or corrective surgeries.
马富西综合征是一种极其罕见的、散发的、非遗传性疾病,其特征为内生软骨瘤病、骨骼异常和血管畸形。我们报告了一例成年马富西综合征患者成功的麻醉管理案例,该患者计划接受甲状旁腺腺瘤手术切除,其患有大量内生软骨瘤、血管瘤以及涉及身体不同部位的骨骼畸形,这在体位摆放、确保静脉通路以及插入气管导管过程中带来了重大挑战。认识这种综合征至关重要,因为在进行偶然或矫正手术时,可能会越来越频繁地遇到类似患者。