Department of Internal Medicine, White River Medical Center, Batesville, Arkansas, USA.
Rutgers New Jersey Medical School, New Jersey, Newark, USA.
BMC Anesthesiol. 2021 Apr 15;21(1):117. doi: 10.1186/s12871-021-01339-0.
Adams-Oliver syndrome is characterized by the combination of congenital scalp defects and terminal transverse limb defects. In some instances, cardiovascular malformations and orofacial malformations have been observed. Little is written with regards to the anesthetic management and airway concerns of patients with Adams-Oliver syndrome.
A five-year-old female with Adams-Oliver syndrome presented for repeat lower extremity surgery. Airway exam was significant for dysmorphic features, such as hypertelorism, deviated jaw, and retrognathia. Video laryngoscope was utilized for intubation due to the patients retrognathic jaw, cranial deformities, and facial dysmorphism. A vein finder with ultrasound guidance was needed to place the peripheral intravenous line due to her history of difficult intravenous access. The patient was successfully intubated with slight cricoid pressure applied to direct the endotracheal tube smoothly. Surgery and recovery were both unremarkable.
Due to varying presentations of Adams-Oliver syndrome, anesthetic and airway management considerations should be carefully assessed prior to surgery. Anesthesiologists must take into consideration possible orofacial abnormalities that may make intubation difficult. Amniotic band syndrome and other limb defects could potentially impact intravenous access as well.
Adams-Oliver 综合征的特征是先天性头皮缺损和末端横断肢体缺陷的组合。在某些情况下，还观察到心血管畸形和口面畸形。关于 Adams-Oliver 综合征患者的麻醉管理和气道问题的文献记载很少。
一名五岁的女性患有 Adams-Oliver 综合征，因下肢手术再次就诊。气道检查显示存在明显的发育异常特征，如眼球突出、下颌偏斜和小下颌。由于患者的小下颌、颅面畸形和面部发育不良，使用视频喉镜进行插管。由于她有静脉穿刺困难的病史，因此需要使用带有超声引导的静脉寻找器来放置外周静脉通路。通过对环状软骨施加轻微压力，成功地将气管内导管直接引导插入。手术和恢复过程均无异常。
由于 Adams-Oliver 综合征的表现各异，在手术前应仔细评估麻醉和气道管理的注意事项。麻醉师必须考虑可能导致插管困难的口面异常。羊膜带综合征和其他肢体缺陷也可能影响静脉通路。
