Department of Anesthesia and Perioperative Care, Rutgers New Jersey Medical School, 538 E-MSB, 185 South Orange Ave, Newark, NJ, 07103, USA.
BMC Pediatr. 2024 Jul 5;24(1):434. doi: 10.1186/s12887-024-04907-8.
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.
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.
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.
大田原综合征是一种进行性发育性和癫痫性脑病，主要表现为婴儿早期。这种罕见的疾病以难治性癫痫发作、精神运动发育迟缓及预后不良为特征。迄今为止，仅有少数关于大田原综合征患儿麻醉管理的病例报告。然而，目前关于大田原综合征伴气道困难的病例报告有限。本报告描述了我们对一名大田原综合征患儿气道的发现及全身麻醉管理，该患儿因严重吸气性喘鸣行诊断性支气管镜检查。
一名 14 个月大、9 公斤重的男性大田原综合征患儿，因长达 1 年的严重吸气性喘鸣而就诊，拟行支气管镜检查及灌洗。体格检查时，患儿呼吸声粗重、不能言语且发育迟缓，头控差，有明显的中枢性低张力。患儿以氯胺酮诱导麻醉，以丙泊酚维持全身麻醉。支气管镜检查顺利完成，诊断为喉气管软化症。整个过程中患儿均自主呼吸，未出现癫痫发作。在麻醉后恢复室，患儿呼吸和心血管功能稳定。
本报告记录了一例 14 个月大的大田原综合征患儿出现严重吸气性喘鸣的罕见表现，以及我们在诊断性支气管镜检查期间的麻醉管理。目前，大田原综合征患儿复杂气道病理的文献记录有限，需要进一步评估。这将有助于小儿麻醉医生为这些患儿提供术前评估、气道管理及备用手术方案。
