背景:Ohtahara综合征是一种进行性发育性和癫痫性脑病,表现在婴儿早期。这种罕见疾病的特征是顽固性癫痫发作,精神运动性迟钝,预后不良。迄今为止,关于Ohtahara综合征儿童的麻醉管理的病例报道很少。然而,存在困难气道的Ohtahara综合征患者的报告有限。本报告描述了我们对患有Ohtahara综合征的儿科患者的气道发现和全身麻醉管理,该患者正在接受诊断支气管镜检查以治疗严重的吸气性喘鸣。
方法:14个月大,9公斤,Ohtahara综合征的男性患者有一年的严重吸气喘鸣病史,并计划进行支气管镜检查并进行灌洗。在考试中,病人呼吸嘈杂,是非语言发育迟缓的,头部控制不佳,有明显的中枢张力减退。患者用氯胺酮诱导,全身麻醉用丙泊酚维持。支气管镜检查顺利完成,并诊断为喉气管软化症。患者的呼吸在整个过程中保持自发,没有发现癫痫发作。在麻醉后护理室,患者的呼吸和心血管功能稳定。
结论:本报告记录了一名14个月大的儿童被诊断为Ohtahara综合征的严重吸气喘鸣的异常发现,以及我们在其诊断支气管镜检查期间的麻醉管理。目前,Ohtahara综合征患者存在的复杂气道病理学的文献有限,应进一步评估。这将有助于儿科麻醉师,因为这些患者可能需要仔细的术前评估,周到的气道管理,和手术替代品待命。
BACKGROUND: Ohtahara syndrome is a progressive developmental and epileptic encephalopathy that manifests in the early infantile period. This rare condition is characterized by intractable seizures, psychomotor retardation, and poor prognosis. To date, there are a handful of
case reports regarding the anesthetic management of children with Ohtahara syndrome. However, limited
reports exist of patients with Ohtahara syndrome who present with difficult airways. This report describes our airway findings and general anesthetic management of a pediatric patient with Ohtahara syndrome undergoing diagnostic bronchoscopy for severe inspiratory
stridor.
METHODS: A 14-month-old, 9 kg, male patient with Ohtahara syndrome presented with a year-long history of severe inspiratory
stridor and was scheduled for bronchoscopy with lavage. On exam, the patient had noisy breathing, was non-verbal with developmental delay, and had poor head control with significant central hypotonia. The patient was induced with ketamine and general anesthesia was maintained with propofol. Bronchoscopic evaluation was completed uneventfully and revealed a diagnosis of laryngotracheomalacia. The patient\'s breathing was maintained spontaneously throughout the procedure and no seizures were noted. In the post anesthesia care unit, the patient\'s respiratory and cardiovascular function were stable.
CONCLUSIONS: This report documents the unusual finding of severe inspiratory
stridor in a 14-month-old child diagnosed with Ohtahara syndrome and our anesthetic management during their diagnostic bronchoscopy. Currently, documentation of complex airway pathology present in patients with Ohtahara syndrome is limited and should be further evaluated. This will assist pediatric anesthesiologists as these patients may require careful preoperative assessment, thoughtful airway management, and surgical alternatives on standby.