Barde Saurabh M, Deshmukh Pratibha U, Laddha Mukesh, Rathi Dilip
Anaesthesia, Datta Meghe Medical College, Datta Meghe Institute of Higher Education and Research, Nagpur, IND.
Orthopaedics, RNH Hospital Pvt. Ltd., Nagpur, IND.
Cureus. 2024 Jun 7;16(6):e61882. doi: 10.7759/cureus.61882. eCollection 2024 Jun.
Alkaptonuria is a rare hereditary condition in which homogentisic acid is deposited in collagenous tissues, leading to blackish discoloration, degenerative changes, restricted mobility, and pain in the affected part. The skeletal system is commonly affected, resulting in the stiffening of the vertebral spine, shoulders, knees, hip joints, and thoracic cage. Additionally, the degenerative process involves heart valves, endocardium, and kidneys, with associated pathophysiological changes. These patients present significant challenges in neuraxial anesthesia, airway management, and postoperative pain relief. In this report, we present the anesthetic management of a case of alkaptonuria undergoing total knee arthroplasty and discuss the encountered difficulties. We conclude that the perioperative anesthesia management of alkaptonuria patients requires thorough planning to effectively address the various challenges associated with the administration of anesthesia.
黑尿症是一种罕见的遗传性疾病，其中尿黑酸沉积在胶原组织中，导致受影响部位出现黑色变色、退行性变化、活动受限和疼痛。骨骼系统通常会受到影响，导致脊柱、肩部、膝盖、髋关节和胸廓僵硬。此外，退行性过程涉及心脏瓣膜、心内膜和肾脏，并伴有相关的病理生理变化。这些患者在神经轴麻醉、气道管理和术后疼痛缓解方面面临重大挑战。在本报告中，我们介绍了一例接受全膝关节置换术的黑尿症患者的麻醉管理，并讨论了遇到的困难。我们得出结论，黑尿症患者的围手术期麻醉管理需要全面规划，以有效应对与麻醉给药相关的各种挑战。
