Baidya Dalim Kumar, Khanna Puneet, Kumar Anil, Shende Dilip
Department of Anaesthesia and Intensive Care, All India Institute of Medical Sciences, New Delhi, India.
J Anaesthesiol Clin Pharmacol. 2011 Oct;27(4):550-2. doi: 10.4103/0970-9185.86607.
Blepharophimosis syndrome is an autosomal dominant disorder characterized by eyelid malformation, involvement of reproductive system and abnormal facial morphology leading to difficult airway. We report a rare association of blepharophimosis syndrome and atrial septal defect in a 10-year-old girl who came for reconstruction surgery of eyelid. The child had dyspnea on exertion. Atrial septal defect was identified preoperatively by clinical examination and echocardiography. Anesthesia management was complicated by failure in laryngeal mask airway placement and Cobra perilaryngeal airway was subsequently used.
睑裂狭小综合征是一种常染色体显性疾病,其特征为眼睑畸形、生殖系统受累以及导致气道困难的面部形态异常。我们报告了一名10岁前来进行眼睑重建手术的女孩,她患有罕见的睑裂狭小综合征合并房间隔缺损。该患儿运动时出现呼吸困难。术前通过临床检查和超声心动图确诊为房间隔缺损。麻醉管理因喉罩置入失败而变得复杂,随后使用了Cobra喉周气道。