一例Alport综合征患儿在俯卧位手术中使用喉罩的麻醉挑战：病例报告

文献信息

中文摘要

引言： Alport综合征是一种由IV型胶原异常引起的罕见遗传性疾病，以进行性肾脏疾病为特征，并伴有可变的肾外表现。指导麻醉管理，尤其是儿科患者麻醉管理的证据仍然有限。
病例介绍： 我们报告一例临床诊断为X连锁Alport综合征的12岁女孩的麻醉管理，该诊断基于镜下血尿和家族性致病性COL4A5变异的专性杂合性，她接受择期俯卧位下肢手术。尽管肾功能保留，围手术期管理包括谨慎选择药物以及使用第二代喉罩、不进行神经肌肉阻滞的丙泊酚全静脉麻醉。俯卧位手术期间通气保持稳定，恢复顺利，术后疼痛控制有效。
结论： 本病例为儿科Alport综合征麻醉管理有限的文献增添了资料，并强调了关于气道管理、麻醉技术和肾脏保护性护理的实际围手术期考虑。

英文摘要

INTRODUCTION: Alport syndrome is a rare hereditary disorder caused by type IV collagen abnormalities and characterized by progressive kidney disease with variable extrarenal manifestations. Evidence guiding anesthetic management, particularly in pediatric patients, remains limited.
CASE PRESENTATION: We report the anesthetic management of a 12-year-old girl with a clinical diagnosis of X-linked Alport syndrome based on microscopic hematuria and obligate heterozygosity for a familial pathogenic COL4A5 variant undergoing elective lower-leg surgery in the prone position. Despite preserved renal function, perioperative management included careful drug selection and a propofol-based total intravenous anesthesia using a second-generation laryngeal mask airway without neuromuscular blockade. Stable ventilation was maintained throughout prone surgery, and recovery was uncomplicated with effective postoperative pain control.
CONCLUSIONS: This case contributes to the limited literature on anesthetic management in pediatric Alport syndrome and highlights practical perioperative considerations regarding airway management, anesthetic technique, and renal-protective care.