Anesthesia management for dental procedures in a patient with aromatic L-amino acid decarboxylase deficiency: a case report.

Edvin Bihorac, Akkan Mahmud, Ahmed Uslu, Nedim Çekmen
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Abstract

Aromatic L-amino acid decarboxylase deficiency (AADCD) is a rare autosomal recessive neurometabolic synthesis disease with sympathetic autoregulation deficiency. Perioperative management of AADCD presents unique challenges due to autonomic dysfunction, impaired sympathetic regulation of heart rate, and potential airway complications. Herein, we report the successful anesthetic management of a 14-year-old male patient with AADCD under total intravenous anesthesia during extensive dental procedures. We applied the rapid sequence induction and intubation method with cricoid pressure to minimize the risk of pulmonary aspiration due to the potential difficulty in mask ventilation and intubation, limited mouth opening, microcephaly, micrognathia, and poor cooperation. Intubation was performed without any complications. Due to the autonomic dysfunction, potential airway complications, and developmental, mental, and motor delays in this disorder, perioperative management requires a multidisciplinary approach with comprehensive preoperative evaluation.

芳香族 L-氨基酸脱羧酶缺乏症患者牙科手术的麻醉管理:病例报告。
芳香l -氨基酸脱羧酶缺乏症(AADCD)是一种罕见的常染色体隐性神经代谢性合成疾病,伴有交感神经自调节缺陷。由于自主神经功能障碍、心率交感调节受损以及潜在的气道并发症,AADCD的围手术期管理面临着独特的挑战。在此,我们报告一名14岁男性AADCD患者在广泛的牙科手术中,在全静脉麻醉下成功的麻醉管理。我们采用环状压迫快速顺序诱导插管方法,以尽量减少因面罩通气和插管困难、张嘴受限、小头畸形、小颌畸形和配合不良而导致肺部误吸的风险。插管无任何并发症。由于自主神经功能障碍,潜在的气道并发症,以及发育,精神和运动迟缓,围手术期管理需要多学科方法和全面的术前评估。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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