Singavarapu Joshua, Yoo Sunny, Borodulin Vitaliy, Borodulina Galina
Anesthesiology, SUNY Downstate Medical Center, Brooklyn, USA.
Anesthesiology, Private Practice, New York, USA.
Cureus. 2025 May 7;17(5):e83687. doi: 10.7759/cureus.83687. eCollection 2025 May.
Pierre Robin Sequence (PRS) is a rare congenital disorder characterized by a triad of micrognathia, glossoptosis, and airway obstruction. This case report presents a rare instance of PRS marked by congenital airway obstruction, which significantly complicated endotracheal intubation with vocal cord motion and a smaller airway to navigate. It will describe what can be expected from such airways and the approaches taken to manage them. The patient was a five-month-old female with PRS, undergoing removal of bilateral mandibular distractors for treatment of micrognathia. Preoperative evaluation revealed a well-nourished, full-term, American Society of Anesthesiologists (ASA) Class II infant in no distress, breathing spontaneously on room air with previously placed bilateral mandibular distractors. Anesthesia induction utilized 8% sevoflurane in 100% oxygen, maintaining spontaneous ventilation. Video laryngoscopy showed a large U-shaped cleft palate and a Cormack-Lehane class II view. The uneventful procedure included dexamethasone, acetaminophen, ketorolac, propofol, and local anesthesia (lidocaine with epinephrine and marcaine). Total IV fluids were 350 ml, blood loss was minimal, and extubation was successful with stable recovery. Video documentation of the intubation sequence is included within this report. This report demonstrates effective airway management strategies tailored to PRS-related anatomical challenges and highlights the vital role of anesthesiologists in clinical decision-making teams for the management of complex airway scenarios.
皮埃尔·罗宾序列征(PRS)是一种罕见的先天性疾病,其特征为小下颌、舌后坠和气道阻塞三联征。本病例报告呈现了一例罕见的以先天性气道阻塞为特征的PRS病例,该病例使声带运动的气管插管及较小气道的操作显著复杂化。报告将描述此类气道的情况以及处理这些气道所采取的方法。患者为一名5个月大患有PRS的女性,正在接受双侧下颌骨牵张器取出术以治疗小下颌。术前评估显示这是一名营养良好、足月的美国麻醉医师协会(ASA)Ⅱ级婴儿,无痛苦表现,在室内空气中自主呼吸,此前已放置双侧下颌骨牵张器。麻醉诱导采用8%七氟醚与100%氧气混合,维持自主通气。视频喉镜检查显示有一个大的U形腭裂以及Cormack-LehaneⅡ级视野。手术过程顺利,使用了地塞米松、对乙酰氨基酚、酮咯酸、丙泊酚和局部麻醉(含肾上腺素的利多卡因和布比卡因)。总静脉输液量为350毫升,失血量极少,拔管成功,恢复平稳。本报告中包含插管过程的视频记录。本报告展示了针对PRS相关解剖学挑战制定的有效气道管理策略,并突出了麻醉医师在复杂气道情况管理的临床决策团队中的重要作用。