Atalay Yunus Oktay, Kaya Cengiz, Ustun Yasemin Burcu, Sahinoglu Ali Haydar
Radiology Department, Outpatient Anesthesia Service, Faculty of Medicine, Ondokuz Mayis University, Samsun, Turkey.
Anesthesiology and Reanimation Department, Faculty of Medicine, Ondokuz Mayis University, Samsun, Turkey.
Med Arch. 2014 Oct;68(5):359-60. doi: 10.5455/medarh.2014.68.359-360. Epub 2014 Oct 15.
The aim of this case was to describe the anesthetic approach to a patient with Kabuki syndrome.
A patient with Kabuki syndrome had revision surgery for scoliosis. On physical examination, shown were long palpebral fissures, large, prominent fissures with an eversion of the lateral third of the lower eyelids, large, prominent malformed ears with low implantation, a short nasal septum, micrognathia, thoracolumbar scoliosis, a depressed left shoulder, a low-set occipital hairline and a short neck. The skin was elastic, and joints were lax. The laryngoscopy showed a Grade II Cormack and Lehane view of the larynx. The trachea was intubated easily. The patient was positioned carefully. Vital signs remained stable during surgery. The patient was extubated and transported to the post-anesthetic care unit.
Anesthesiologists should be aware of possibly difficult tracheal intubation cardiac lesions, respiratory problems, neurological and musculoskeletal disorders, and a latex allergy when managing anesthesia for a patient with Kabuki syndrome.
本病例的目的是描述对一名歌舞伎综合征患者的麻醉方法。
一名歌舞伎综合征患者接受了脊柱侧弯翻修手术。体格检查发现，患者存在睑裂长、睑裂大且突出伴下眼睑外侧三分之一外翻、耳朵大且畸形突出伴低位植入、鼻中隔短、小颌畸形、胸腰椎脊柱侧弯、左肩凹陷、枕后发际线低和颈部短。皮肤有弹性，关节松弛。喉镜检查显示为Cormack和Lehane二级喉镜视野。气管插管顺利。患者体位摆放谨慎。手术过程中生命体征保持稳定。患者拔管后被转运至麻醉后护理单元。
麻醉医生在为歌舞伎综合征患者实施麻醉管理时，应意识到可能存在的气管插管困难、心脏病变、呼吸问题、神经和肌肉骨骼疾病以及乳胶过敏。
