Department of Pediatric Anesthesiology, Medical College of Wisconsin, Milwaukee, WI, USA.
Department of Pediatric Anesthesiology, Children's Wisconsin, Milwaukee, WI, USA.
Paediatr Anaesth. 2021 Apr;31(4):419-428. doi: 10.1111/pan.14131. Epub 2021 Feb 28.
Trisomy 18 or Edwards syndrome is the second most common aneuploidy with a prevalence between 1/3000 and 1/10 000 live births. The syndrome encompasses malformations of the central nervous, cardiac, respiratory, gastrointestinal, and genitourinary systems. Trisomy 18 carries a poor prognosis with 90% of patients not surviving beyond 1 year of age; however, the current trend toward more aggressive supportive care may prolong survival. The limited anesthesia literature highlights the abnormal airway anatomy but generally describes uneventful airway management and perioperative course.
Our goal was to review all anesthesia encounters recorded for eleven trisomy 18 patients treated at Children's Wisconsin during the study period to explore the frequency of anesthesia encounters and to improve our understanding of the perioperative risks.
We performed a retrospective chart review of all patients with trisomy 18 who were treated at our institution between 2012 and 2017. Records were screened for anesthesia encounters, perioperative critical events and complications, enrollment in palliative care, code status, and time of death.
Eleven children were identified. Children were born between 2001 and 2016. Two children never required anesthesia care. Nine patients had a total of 84 anesthesia encounters for 121 surgical or diagnostic procedures or emergent intubations. Critical events and perioperative complications included difficult mask ventilation (n = 7), difficult intubation (n = 15), and mechanical or pharmacological cardiopulmonary resuscitation (n = 6). Five patients presented with difficult peripheral intravenous access. One patient died in the immediate postoperative period. On five occasions, patients required emergent intubation outside of the operating room.
Difficult airway management and respiratory compromise were critical concerns during the perioperative period in our patient population, and the inability to ventilate could lead to cardiorespiratory arrest. This case series provides a comprehensive, longitudinal view of complete trisomy 18 patients in the perioperative period and adds information for counseling families and care providers.
18 三体综合征或爱德华兹综合征是第二常见的常染色体三体异常，发病率为每 3000 至 10000 例活产儿中有 1 例。该综合征包括中枢神经系统、心脏、呼吸、胃肠道和泌尿生殖系统的畸形。18 三体综合征预后不良，90%的患者活不过 1 岁；然而，目前更积极的支持性治疗趋势可能延长生存时间。有限的麻醉文献强调了异常气道解剖结构，但通常描述了气道管理和围手术期过程平稳。
我们的目标是回顾在研究期间在威斯康星儿童医院接受治疗的 11 例 18 三体综合征患者的所有麻醉记录，以探讨麻醉次数的频率，并加深我们对围手术期风险的认识。
我们对 2012 年至 2017 年在我院治疗的所有 18 三体综合征患者进行了回顾性病历审查。记录筛查了麻醉经历、围手术期关键事件和并发症、姑息治疗登记、代码状态和死亡时间。
确定了 11 名儿童。这些儿童出生于 2001 年至 2016 年。两名儿童从未接受过麻醉护理。9 名患者共进行了 84 次麻醉，用于 121 次手术或诊断程序或紧急插管。关键事件和围手术期并发症包括困难面罩通气（n=7）、困难插管（n=15）和机械或药物心肺复苏（n=6）。5 名患者外周静脉穿刺困难。1 名患者在术后即刻期死亡。有 5 次患者需要在手术室外紧急插管。
在我们的患者群体中，围手术期期间气道管理困难和呼吸功能障碍是关键关注点，无法通气可能导致心肺骤停。本病例系列提供了 18 三体综合征患者围手术期的全面、纵向观察结果，并为咨询家属和护理人员提供了信息。
