PDF(Pubmed)
Abstract：
BACKGROUND: Anesthesia for spinal muscular atrophy (SMA) patients undergoing spinal deformity surgery is challenging. We report an unusual case of an SMA girl who developed severe intraoperative hypoxemia and hypotension during posterior spinal fusion related with surgical positioning.
METHODS: A 13-yr-old girl diagnosed with SMA type 2, severe kyphoscoliosis and thoracic deformity was scheduled for elective posterior spinal fusion. She developed severe hypoxemia and profound hypotension intraoperatively in the prone position with surgical table tilted 45° to the right. Though transesophageal echocardiography (TEE) could not be performed due to limited mouth opening, her preoperative computed tomography revealed a severely distorted thoracic cavity with much reduced volume of the right side. A reasonable explanation was when the surgeons performed surgical procedure with the tilted surgical table, the pressure was directly put on the shortest diameter of the significantly deformed thoracic cavity, causing severe compression of the pulmonary artery, resulting in both hypoxemia and hypotension. The patient stabilized when the surgical table was tilted back and successfully went through the surgery in the leveled prone position.
CONCLUSIONS: Spinal fusion surgery is beneficial for SMA patients in preventing scoliosis progression and improving ventilation. However, severe scoliosis and thoracic deformities put them at risk of both hemodynamic and respiratory instability during surgical positioning. When advanced monitoring like TEE is not practical intraoperatively, preoperative imaging may help with differential diagnosis, and guide the surgical positioning to minimize mechanical compression of the thoracic cavity, thereby helping the patient complete the surgery safely.
METHODS: A 13-yr-old girl diagnosed with SMA type 2, severe kyphoscoliosis and thoracic deformity was scheduled for elective posterior spinal fusion. She developed severe hypoxemia and profound hypotension intraoperatively in the prone position with surgical table tilted 45° to the right. Though transesophageal echocardiography (TEE) could not be performed due to limited mouth opening, her preoperative computed tomography revealed a severely distorted thoracic cavity with much reduced volume of the right side. A reasonable explanation was when the surgeons performed surgical procedure with the tilted surgical table, the pressure was directly put on the shortest diameter of the significantly deformed thoracic cavity, causing severe compression of the pulmonary artery, resulting in both hypoxemia and hypotension. The patient stabilized when the surgical table was tilted back and successfully went through the surgery in the leveled prone position.
CONCLUSIONS: Spinal fusion surgery is beneficial for SMA patients in preventing scoliosis progression and improving ventilation. However, severe scoliosis and thoracic deformities put them at risk of both hemodynamic and respiratory instability during surgical positioning. When advanced monitoring like TEE is not practical intraoperatively, preoperative imaging may help with differential diagnosis, and guide the surgical positioning to minimize mechanical compression of the thoracic cavity, thereby helping the patient complete the surgery safely.
摘要：
背景：脊髓性肌萎缩症（SMA）患者接受脊柱畸形手术的麻醉具有挑战性。我们报告了一例SMA女孩的不寻常病例，该女孩在与手术定位相关的后路脊柱融合术中出现严重的术中低氧血症和低血压。
方法：一名13岁的女孩被诊断为SMA2型，严重的脊柱后凸和胸椎畸形，计划进行选择性后路脊柱融合术。她在俯卧位术中出现严重的低氧血症和深度低血压，手术台向右倾斜45°。尽管由于张口受限而无法进行经食管超声心动图（TEE），她的术前计算机断层扫描显示胸腔严重扭曲，右侧体积大大减少。一个合理的解释是当外科医生用倾斜的手术台进行外科手术时，直接将压力施加在明显变形的胸腔的最短直径上，导致肺动脉严重受压,导致低氧血症和低血压。当手术台向后倾斜时，患者稳定下来，并以水平的俯卧位成功进行了手术。
结论：脊柱融合术有利于SMA患者预防脊柱侧凸进展和改善通气。然而,严重的脊柱侧凸和胸廓畸形使他们在手术定位过程中面临血流动力学和呼吸不稳定的风险。当像TEE这样的高级监测在术中不实用时，术前成像可能有助于鉴别诊断，并引导手术定位以最大程度地减少胸腔的机械压缩，从而帮助患者安全地完成手术。
方法：一名13岁的女孩被诊断为SMA2型，严重的脊柱后凸和胸椎畸形，计划进行选择性后路脊柱融合术。她在俯卧位术中出现严重的低氧血症和深度低血压，手术台向右倾斜45°。尽管由于张口受限而无法进行经食管超声心动图（TEE），她的术前计算机断层扫描显示胸腔严重扭曲，右侧体积大大减少。一个合理的解释是当外科医生用倾斜的手术台进行外科手术时，直接将压力施加在明显变形的胸腔的最短直径上，导致肺动脉严重受压,导致低氧血症和低血压。当手术台向后倾斜时，患者稳定下来，并以水平的俯卧位成功进行了手术。
结论：脊柱融合术有利于SMA患者预防脊柱侧凸进展和改善通气。然而,严重的脊柱侧凸和胸廓畸形使他们在手术定位过程中面临血流动力学和呼吸不稳定的风险。当像TEE这样的高级监测在术中不实用时，术前成像可能有助于鉴别诊断，并引导手术定位以最大程度地减少胸腔的机械压缩，从而帮助患者安全地完成手术。
