Huecker Martin, Harris Zach, Yazel Eric
Department of Emergency Medicine, University of Louisville School of Medicine, Louisville, Kentucky.
Private Practice Emergency Medicine, Louisville, Kentucky.
J Emerg Med. 2017 Oct;53(4):558-562. doi: 10.1016/j.jemermed.2017.04.037.
Achondroplastic dwarfism is associated with anatomic abnormalities that can predispose to occult injury and challenges in trauma management. Airway anatomy is problematic due to macrocephaly, midface hypoplasia, and a narrow nasopharynx. Manipulation of the neck is very dangerous due to the high likelihood of preexisting cervicomedullary stenosis. Restrictive lung disease and obstructive sleep apnea may complicate respiratory status. Peripheral and central venous access can be difficult to obtain. Orthopedic and metabolic comorbidities can lead to a prolonged hospital course.
A 17-year-old male patient with achondroplasia presented to the Emergency Department after a high-speed motor vehicle collision. Despite a negative computed tomography scan of the cervical spine and absence of neck pain, a magnetic resonance imaging evaluation was obtained due to severe neurologic deficits; it revealed disruption of the anterior longitudinal ligament at C2/3 and spinal cord contusion from C3-C6. The patient had a difficult intubation and prolonged weaning from the ventilator after his operation. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Emergency physicians must maintain preparedness for all patients and situations, no matter how rare. Prior knowledge of key differences in management of the ABCDs (airway, breathing, circulation, neurological deficit) in patients with achondroplasia will reduce morbidity and mortality.
软骨发育不全性侏儒症与解剖学异常相关,这些异常易导致隐匿性损伤,并给创伤管理带来挑战。由于巨头畸形、面中部发育不全和鼻咽部狭窄,气道解剖结构存在问题。由于存在颈椎脊髓狭窄的高可能性,颈部的操作非常危险。限制性肺病和阻塞性睡眠呼吸暂停可能使呼吸状况复杂化。外周和中心静脉通路可能难以建立。骨科和代谢合并症可能导致住院时间延长。
一名17岁软骨发育不全男性患者在高速机动车碰撞后被送往急诊科。尽管颈椎计算机断层扫描结果为阴性且无颈部疼痛,但由于严重的神经功能缺损,仍进行了磁共振成像评估;结果显示C2/3处前纵韧带断裂以及C3 - C6脊髓挫伤。该患者插管困难,术后脱机时间延长。
急诊医生为何应了解此情况?:急诊医生必须对所有患者和情况做好准备,无论多么罕见。事先了解软骨发育不全患者在气道、呼吸、循环、神经功能缺损(ABCD)管理方面的关键差异将降低发病率和死亡率。